Anda di halaman 1dari 51

JAN/FEB 2012

Jul/Aug 2013 Vol.


Vol. 38
39 No.
No. 14 Your partner in paediatric
and O&G practice
JOURNAL OF PAEDIATRICS, OBSTETRICS & GYNAECOLOGY

ISSN 1016-0124
CME ARTICLE
(INDONESIA)

5 SK
P
Management of
Pregnancies With Previous
Caesarean Section

JOURNAL WATCH

PAEDIATRICS

Management of Hearing
Loss in Children
Constipation in Infants
and Children

Gynaecology

Ovarian Cancer:
Current Management
and Future Directions

OBSTETRICS

Management of
Early Pregnancy
Complications
www.jpog.com

Get your copy of JPOG today and earn SKP IDI

JPOG_JulAug13_CVR_FINAL.indd 5 8/5/13 2:00 PM


JOURNAL OF PAEDIATRICS, OBSTETRICS & GYNAECOLOGY

J UL/AUG 2013
Vol. 39 No. 4

Journal Watch

133 Calcium intake and mortality in Swedish women


Outcome of extreme preterm birth in England, 19952006
134 New anti-interleukin therapies for systemic JIA
CPAP vs surfactant and higher vs lower oxygen saturation for
extremely preterm infants: Outcomes at 1822 months
135 Paromomycin for cutaneous leishmaniasis
HMPV infection in young children in the US
133 136 Risk factors for stillbirth
Fetal macrosomia in developing countries

136

Editorial Board Professor Biran Affandi Dr Tak-Yeung Leung Dr Raman Subramaniam


University of Indonesia Chinese University of Hong Kong Fetal Medicine and Gynaecology Centre, Malaysia
Board Director, Paediatrics Dr Karen Kar-Loen Chan Professor Tzou-Yien Lin Professor Walfrido W Sumpaico
Professor Pik-To Cheung The University of Hong Kong Chang Gung University, Taiwan MCU-FDT Medical Foundation, Philippines
Associate Professor Professor Oh Moh Chay Professor Somsak Lolekha Professor Cheng Lim Tan
Department of Paediatrics KK Womens and Childrens Hospital, Ramathibodi Hospital, Thailand KK Womens and Childrens Hospital, Singapore
and Adolescent Medicine Singapore Professor Lucy Chai-See Lum Professor Kok Hian Tan
The University of Hong Kong
Associate Professor Anette Jacobsen University of Malaya, Malaysia KK Womens and Childrens Hospital, Singapore
Board Director, Obstetrics and Gynaecology KK Womens and Childrens Hospital, Singapore Professor SC Ng Professor Surasak Taneepanichskul
Professor Pak-Chung Ho Professor Rahman Jamal National University of Singapore Chulalongkorn University, Thailand
Head, Department of Universiti Kebangsaan Malaysia Professor Hextan Yuen-Sheung Ngan Professor Eng-Hseon Tay
Obstetrics and Gynaecology The University of Hong Kong Thomson Women Cancer Centre, Singapore
The University of Hong Kong Dato Dr Ravindran Jegasothy
Hospital Kuala Lumpur, Malaysia Professor Carmencita D Padilla Professor PC Wong
University of the Philippines Manila National University of Singapore
Associate Professor Kenneth Kwek
KK Womens and Childrens Hospital, Singapore Professor Seng-Hock Quak Adjunct Professor George SH Yeo
National University of Singapore KK Womens and Childrens Hospital, Singapore
Dr Siu-Keung Lam
Dr Tatang Kustiman Samsi Professor Hui-Kim Yap
Prestige Medical Centre, Hong Kong
University of Tarumanagara, Indonesia National University of Singapore
Professor Terence Lao Professor Alex Sia Professor Tsu-Fuh Yeh
Chinese University of Hong Kong KK Womens and Childrens Hospital, Singapore China Medical University, Taiwan
Dr Kwok-Yin Leung
The University of Hong Kong

JPOG JUL/AUG 2013 i

JPOG_JulAug_2013_TOC_Final.indd 1 8/5/13 1:51 PM


Journal of Paediatrics, Obstetrics & Gynaecology

MIMS.com now comes with


a brand new look and mobile app!
Along with a redesign that helps enhance ease of access to crucial
clinical information, MIMS.com can now be accessed through a
FREE mobile application, available on the iTunes store.
With MIMS available across multiple platforms and devices, you can
now easily and conveniently find the most up-to-date and relevant drug
information anytime, anywhere.

CONNECT WITH MIMS:

www.mims.com MIMS mobile/tablet app facebook.com/mimscom

MIMScomNew206x276_0613.indd 1 6/4/13 3:45 PM


JOURNAL OF PAEDIATRICS, OBSTETRICS & GYNAECOLOGY

J UL/AUG 2013
Vol. 39 No. 4

Review Article
Paediatrics

137 The Management of Hearing Loss in Children


Universal neonatal hearing screening aims to detect the 1 in 1,000 babies born in the UK with a permanent
hearing loss detectable at birth. However, children may present later to the paediatrician with hearing
difficulties. This article aims to discuss the clinical assessment of hearing and provides an overview of the
management options available in the treatment of hearing loss.
Marianne D Elloy, Andrew H Marshall

Review Article
137 Obstetrics

146 Management of Early Pregnancy Complications


Complications of early pregnancy are common, including pregnancy loss, threatened miscarriage,
ectopic pregnancy, molar pregnancy and hyperemesis. This review discusses the different presentations,
diagnoses and management of the common problems complicating early pregnancy.
Harriet Pugsley, Judith Moore

146

Publisher
Ben Yeo
Enquiries and Correspondence PUBLISHER: Journal of Paediatrics, Obstetric & Gynaecology (JPOG) is
published 6 times a year by MIMS Pte Ltd. CIRCULATION: JPOG is a controlled
circulation for medical practitioners in South East Asia. It is also available on
Publication Manager China Malaysia Vietnam subscription to members of allied professions. SUBSCRIPTION: The price per
Marisa Lam annum is US$42 (surface mail, students US$21) and US$48 (overseas airmail,
Yang Xuan Meera Jassal, Lee Pek Lian, Nguyen Thi Lan Huong, students US$24); back issues US$8 per copy. EDITORIAL MATTER published
Managing Editor Tel: (86 21) 6157 3888 Sheena Subash, Grace Yeoh Nguyen Thi My Dung herein has been prepared by professional editorial staff. Views expressed are
Greg Town Email: enquiry.cn@mims.com Tel: (60 3) 7954 2910 Tel: (84 8) 3829 7923 not necessarily those of MIMS Pte Ltd. Although great care has been taken in
compiling and checking the information given in this publication to ensure that
Associate Editor Email: enquiry.my@mims.com Email: enquiry.vn@mims.com
Grace Ling Hong Kong it is accurate, the authors, the publisher and their servants or agents shall not
be responsible or in any way liable for the continued currency of the information
Designers Kristina Lo-Kurtz, Jacqueline Cheung, Philippines Europe/USA or for any errors, omissions or inaccuracies in this publication whether arising
Agnes Chieng, Sam Shum Marisa Lam, Miranda Wong Marian Chua, Kims Pagsuyuin, Kristina Lo-Kurtz from negligence or otherwise howsoever, or for any consequences arising
therefrom. The inclusion or exclusion of any product does not mean that the
Production Tel: (852) 2559 5888 Rowena Belgica, Philip Katipunan Tel: (852) 2116 4352 publisher advocates or rejects its use either generally or in any particular field
Edwin Yu, Ho Wai Hung, Email: enquiry.hk@mims.com Tel: (63 2) 886 0333 Email: kristina.lokurtz@mims.com or fields. COPYRIGHT: 2013 MIMS Pte Ltd. All rights reserved. No part of
Steven Cheung Email: enquiry.ph@mims.com this publication may be reproduced, stored in a retrieval system or transmitted
India in any form or by any means, electronic, mechanical, photocopying, recording
Circulation Monica Bhatia Singapore or otherwise, in any language, without written consent of copyright owner.
Christine Chok Permission to reprint must be obtained from the publisher. ADVERTISEMENTS
Tel: (91 80) 2349 4644 Jason Bernstein, Carrie Ong, are subject to editorial acceptance and have no influence on editorial content or
Accounting Manager Email: enquiry.in@mims.com Josephine Cheong, Melanie Nyam presentation. MIMS Pte Ltd does not guarantee, directly or indirectly, the quality
Minty Kwan
Tel: (65) 6290 7400 or efficacy of any product or service described in the advertisements or other
Advertising Coordinator Korea Email: enquiry.sg@mims.com
material which is commercial in nature. Philippine edition: Entered as second-
class mail at the Makati Central Post Office under Permit No. PS-326-01 NCR,
Rachael Tan Choe Eun Young dated 9 Feb 2001. Printed by Fortune Printing International Ltd, 3rd Floor, Chung
Tel: (82 2) 3019 9350 Thailand On Industrial Building, 28 Lee Chung Street, Chai Wan, Hong Kong.
Published by: Email: inquiry@kimsonline.co.kr Wipa Sriwijitchok
MIMS Asia Pacific Tel: (66 2) 741 5354
27th Floor, OTB Building Indonesia Email: enquiry.th@mims.com
160 Gloucester Road, Hafta Hasibuan, Sri Damayanti,
Wan Chai, Hong Kong
Tel: (852) 2559 5888 Ritta Pamolango
Email: enquiry@jpog.com Tel: (62 21) 729 2662
Email: enquiry.id@mims.com

JPOG JUL/AUG 2013 ii

JPOG_JulAug_2013_TOC_Final.indd 2 8/5/13 1:51 PM


Indonesia

JOURNAL OF PAEDIATRICS, OBSTETRICS & GYNAECOLOGY

J UL/AUG 2013
Vol. 39 No. 4

Review Article
Gynaecology

155 Ovarian Cancer: Current Management and Future Directions


Ovarian cancer has the highest mortality of all the gynaecological malignancies. Treatment of advanced
epithelial ovarian cancer usually involves debulking surgery and chemotherapy. Treatment may prolong life
and palliate symptoms but it is rarely curative. New treatments are constantly being developed and offer
the hope of improved outcomes.
Sin E Taylor, John M Kirwan

Review Article
155 Paediatrics

164 Constipation in Infants and Children


Constipation is a common problem in children and is usually functional, related to stool-withholding.
Successful management requires parent education, behavioural strategies, laxative agents (often long
term) and ongoing review.
Taya Dowling, Scott Nightingale

P
Continuing Medical Education 5 SK

169 Management of Pregnancies With Previous Caesarean Section


This article reviews the recommendations on vaginal birth after previous caesarean section (VBAC) by
different professional societies, the favourable and unfavourable factors in considering VBAC, the associated
169 maternal and fetal benefits and risks, as well as the non-medical concerns that play a role in the decision-
making process.
Yung WK, Lau WL, Leung WC

Review Articles Case Studies


Comprehensive reviews Interesting cases seen in general
providing the latest clinical practice and their management.
information on all aspects The Cover:
of the management of medical Pictorial Medicine Early Pregnancy Complications
conditions affecting children Vignettes of illustrated cases 2013 MIMS Pte Ltd
and women. with clinical photographs.
For more information, please refer to the Instructions for Authors on our website www.jpog.com, or contact:
The Editor
MIMS Pte Ltd, 6 Shenton Way, #15-08 Tower 2, Singapore 068809 Lisa Low, Illustrator
Tel: (65) 6290 7400 Fax: (65) 6290 7401 E-mail: enquiry@jpog.com

JPOG JUL/AUG 2013 iii

JPOG_JulAug_2013_TOC_Final.indd 6 8/5/13 1:51 PM


Peer Reviewed Journal Watch

cardiovascular mortality, and 114% in coronary cerebral ultrasound. In 2006, compared with 1995,
GYNAECOLOGY disease mortality, and no significant change in 44% more infants born alive at 2225 weeks were
stroke mortality, compared with a calcium intake of admitted to neonatal intensive care, and survival
6001,000 mg a day. After further statistical analy- of infants born at 23, 24, and 25 weeks increased
Calcium intake and mortality in sis, low intakes of calcium (< 600 mg/day) were by 9.5%, 12%, and 16%, respectively. Overall, the
Swedish women no longer significantly associated with increased proportion treated for retinopathy increased from
mortality. Among people taking calcium tablets and 12% in 1995 to 22% in 2006.
with a dietary calcium intake of > 1,400 mg/day, Neurodevelopmental outcomes at ages 23
all-cause mortality was increased 2.6-fold. years were assessed for 1,031 survivors in the 2006
High-calcium intake is associated with in- cohort. The prevalence of moderate or severe im-
creased all-cause and cardiovascular mortality. pairment was 45% among survivors born at 2223
weeks, 30% at 24 weeks, 25% at 25 weeks, and
Michalson K et al. Long-term calcium intake and rate of all cause and
cardiovascular mortality: community-based prospective longitudinal 20% at 26 weeks. Overall, one in seven (14%) had
cohort study. BMJ 2013; 346: 14 (f228).
cerebral palsy, usually mild or moderate. Mean
predicted adjusted mental development index quo-
tients (Bayley scales) were 80 (2223 weeks), 87
(24 weeks), 88 (25 weeks), and 91 (26 weeks). In

PAEDIATRICS the 2006 cohort, a greater absolute number of chil-


dren than in 1995 will need lifelong special care. It
is calculated that of every 100 infants born at 24

Outcome of extreme preterm weeks, 60 will die despite intensive care and 12
birth in England, 19952006 of the 40 survivors will have serious impairments.
Meta-analyses of randomized studies have shown Further follow-up of the 2006 cohort is planned.
that taking calcium supplements is associated with Two successive papers in the BMJ have examined Between 1995 and 2006 the survival of ex-
increased risk of coronary disease and stroke. A short-term and long-term outcomes for extremely tremely preterm infants in England improved, but
Swedish cohort study has confirmed the increased preterm births in England in 1995 and 2006.
risk for cardiovascular disease in general but not The prospective national cohort studies pro-
for stroke. vided short-term data about 666 babies born at 22
The Swedish mammography cohort was set 25 weeks gestation in England in March to Decem-
up in 1987 and included 61,433 women born be- ber 1995 and all 3,133 babies born at 2226 weeks
tween 1914 and 1948. National registries provided gestation in 2006. In 2006, 56% of infants born at
data about all-cause and cardiovascular mortality 22 weeks and 98% of those born at 26 weeks were
over a mean follow-up of 19 years. Food frequency born alive. Active care at birth was withheld from
questionnaires in 1987 and 1997 provided data 73% of infants born at 22 weeks, 16% at 23 weeks,
about dietary intake and use of calcium supple- and < 2% at 24 weeks or later. Survival rates for
ments for 38,984 women. The relationship between live-born infants were 2% at 22 weeks, 19% at 23
calcium intake and all-cause mortality took the weeks, 40% at 24 weeks, 66% at 25 weeks, and
form of a J-shaped curve, with higher mortality 77% at 26 weeks. More than two-thirds (68%) of
at both extremes of intake. An intake of 1,400 mg survivors had bronchopulmonary dysplasia, 16%
a day of calcium was associated with significant were treated (laser treatment) for retinopathy of
increases of 40% in all-cause mortality, 49% in prematurity, and 13% had a serious abnormality on

JPOG JUL/AUG 2013 133

JPOG_JulAug_2013_COMBINE_Final.indd 133 8/5/13 2:18 PM


more survived with disability. The writers of an The humanized, antihuman interleukin-6-re- De Benedetti F et al. Randomized trial of tocilizumab in systemic juvenile
idiopathic arthritis. NEJM 2012; 367: 238595; Ruperto N et al. Two
editorial point out that in the Netherlands, infants ceptor monoclonal antibody, tocilizumab, was as- randomized trials of canakinumab in systemic juvenile idiopathic arthritis.
Ibid: 2396406; Sandborg C, Mellins ED. A new era in the treatment of
born before 24 completed weeks are not routinely sessed in a placebo-controlled trial at 43 centres systemic juvenile idiopathic arthritis. Ibid: 243940 (editorial).

offered neonatal intensive care. in Europe North America, and South America. The
trial included 112 children aged 217 years with
Costeloe KL et al. Short term outcomes after extreme preterm birth
in England: comparison of two birth cohorts in 1995 and 2006 (the active, treatment-resistant systemic JIA. Random-
EPICure studies). BMJ 2012; 345: 14 (e7976); Moore T. Neurological and CPAP vs surfactant and higher
developmental outcome in extremely preterm children born in England
ization (2:1) was to intravenous tocilizumab or vs lower oxygen saturation for
in 2006 and 1995: the EPICure studies. Ibid: 15 (e7961), Groenendaal F,
Uiterwaal C. Long-term follow-up of extremely preterm neonates. Ibid:10
(e8252) (editorial).
placebo every 2 weeks for 12 weeks. At 12 weeks, extremely preterm infants:
an improvement of at least 30% on the American Outcomes at 1822 months
College of Rheumatology JIA score (JIA ACR 30)
was achieved by 85% (tocilizumab) vs 24% (pla-
cebo), a highly significant difference. At week 52
New anti-interleukin therapies
a JIA ACR 70 response (at least 70% improvement)
for systemic JIA
was achieved by 80% in the tocilizumab group and
a JIA ACR 90 response by 59%. Steroid therapy had
been stopped by 52% in this group, and 48% had no
active arthritis. Common adverse events with tocili-
zumab included infections, neutropenia, and raised
aminotransferase levels.
The fully human, anti-interleukin-1 mono-
clonal antibody, canakinumab, was assessed in two
international trials reported together, including 84
and 100 patients. In the first trial, randomization
was to subcutaneous canakinumab or placebo, and
an adapted JIA ACR 30 response was achieved by
84% (canakinumab) vs 10% (placebo). In the sec-
ond trial, 100 patients who had responded to 32
weeks of canakinumab were randomized to con- The Surfactant, Positive Pressure, and Pulse Oxim-
tinued canakinumab or to placebo. A disease flare etry randomized trial was a multicentre, random-
occurred in 74% (canakinumab) vs 25% (placebo). ized, controlled trial with a 2 2 multifactorial
Systemic juvenile idiopathic arthritis (systemic JIA) The median time to disease flare was incalculable design in which 1,316 extremely preterm infants
frequently leads to joint damage and disability and in the canakinumab group and 236 days in the pla- (born at 24 weeks 0 days to 27 weeks 6 days) were
is accompanied by systemic features such as fever, cebo group. The disease became inactive in 62% randomized at 20 US centres to early continuous
rash, hepatosplenomegaly, and serositis. High- vs 34%. One in three patients on canakinumab was positive airway pressure (CPAP), or early surfactant
dose steroid treatment may lead to severe toxicity, able to discontinue steroid therapy. Infections were via an endotracheal tube and to a target oxygen
and treatments such as methotrexate and tumour frequent with canakinumab and five patients (ver- saturation of 8589% or of 9195%. Early assess-
necrosis factor inhibitors may be ineffective. The sus two in the placebo group) developed the mac- ment (at 36 weeks postmenstrual age) showed
effectiveness of antibodies to the interleukin-6 rophage activation syndrome. similar rates of death or bronchopulmonary dyspla-
receptor and to interleukin-1 has been shown in Both tocilizumab and canakinumab were ef- sia with either CPAP or surfactant, and the lower
successive papers in the New England Journal of fective treatment for systemic JIA but more data target range for oxygen saturation was associ-
Medicine. are needed about toxicity. ated with less retinopathy of prematurity but more

JPOG JUL/AUG 2013 134

JPOG_JulAug_2013_COMBINE_Final.indd 134 8/5/13 2:18 PM


Peer Reviewed Journal Watch

deaths. Now, surviving infants have been assessed Leishmaniasis is prevalent in Eurasia, Africa, and
at 1822 months. the Americas, and although cutaneous leishmani-
Neurodevelopmental status was assessed asis eventually resolves without treatment it is the
at 1822 months in 990 of 1,058 surviving infants cause of much morbidity. Cutaneous leishmaniasis
(94%). Death or neurodevelopmental impairment due to Leishmania major is prevalent in Tunis, and
occurred in 27.9% (CPAP) vs 29.9% (surfactant), a trial there has shown topical paromomycin to be
a non-significant difference, and in 30.2% (lower effective treatment.
oxygen saturation target) vs 27.5% (higher oxygen A total of 375 patients aged 565 years (half
saturation target), also a non-significant differ- of them children < 17 years old) were randomized
ence. There was a significant increase in mortality to three groups: 15% paromomycin, 15% paromo-
with the lower oxygen saturation target (22.1% vs mycin plus 0.5% gentamicin, or vehicle alone (pla-
18.2%). cebo), all applied as topical creams for 20 days to
These researchers conclude that outcomes all ulcerated skin lesions including an index lesion
are similar with early CPAP and with early surfac- (15 cm diameter with leishmania demonstrated).
tant, but the lower oxygen saturation target should Cure of the index lesion was achieved in 82% (par-
not be used in the care of extremely premature omomycin), 81% (paromomycin/gentamicin), and
babies. 58% (placebo). Only seven patients (five in the pla-
cebo group) had any persisting lesions after cure of York. It included inpatients and outpatients in No-
Vaucher YE et al. Neurodevelopmental outcomes in the early CPAP and
pulse oximetry trial. NEJM 2012; 367: 2495504. the index lesion. Mild to moderate local reactions vember to May each year between 2003 and 2009
occurred with paromomycin. with children presenting with an acute respiratory
Topical treatment with paromomycin cream, illness or fever. The rate of HMPV detection (using
Paromomycin for cutaneous with or without gentamicin, was effective treat- reverse transcriptasepolymerase chain reaction
leishmaniasis ment for cutaneous leishmaniasis due to L major. assay on nose and throat swabs) was 200/3,490
(6%) among children in hospital, 222/3,257 (7%)
Salah AB et al. Topical paromomycin with or without gentamicin for
cutaneous leishmaniasis. NEJM 2012; 368: 52432. among children in outpatient clinics, 224/3,001
(7%) among children in the emergency department,
and 10/770 (1%) among healthy children in well-
HMPV infection in young children child primary care clinics. Rates of hospital admis-
in the US sion with HMPV infection among children aged < 5
years were 1 in 1,000 (3 in 1,000 at age < 6 months,
Human metapneumovirus (HMPV) is a paramyxovi- and 2 in 1,000 at age 611 months). Among chil-
rus discovered in 2001 as a cause of acute respi- dren admitted to hospital with an acute respiratory
ratory illness in infants and young children world- illness or fever, those with HMPV infection were
wide. It also affects old people and people with older, more likely to be diagnosed as pneumonia or
debilitating illnesses. A study at three US sites asthma, to need supplemental oxygen, and to stay
has provided more data about the epidemiology of longer in intensive care, compared with children
HMPV in children under the age of 5 years. testing negative for HMPV. It was estimated that
The survey by the Centers for Disease Con- among 1,000 children of this age, HMPV would,
trol and Prevention (CDC) New Vaccine Surveillance each year, cause 55 clinic visits and 13 visits to the
network took place at three sites, in Cincinnati, emergency department. Among children admitted
Ohio; Nashville, Tennessee; and Rochester, New to hospital, coexisting high-risk conditions (pre-

JPOG JUL/AUG 2013 135

JPOG_JulAug_2013_COMBINE_Final.indd 135 8/5/13 2:18 PM


mature birth, immunodeficiency, chronic disease of Indian, or Pakistani), maternal obesity (BMI 30 or
lungs, heart, or kidneys, cancer, or sickle-cell dis- greater), smoking, pre-existing diabetes, history of
ease) were present in 40% (HMPV-positive) vs 30% mental health problems, and pregnancy complica-
(HMPV-negative). tions (antepartum haemorrhage, fetal growth re-
In the US, HMPV is an important cause of striction). The greatest risk factor was fetal growth
acute respiratory illness and fever among young restriction which increased the risk of stillbirth by a
children. factor of 7.8 in non-smoking mothers, 5.7 in smok-
ing mothers, and 10.0 in mothers only exposed to
Edwards MK et al. Burden of human metapneumovirus infection in young
children. NEJM 2013; 368: 63343. passive smoking. The population attributable risk
from fetal growth restriction was 6.2% if detected
antenatally and 32.0% when not detected antena-
tally. Antenatal detection of fetal growth restric-
OBSTETRICS
tion was associated with delivery 10 days earlier
on average. The stillbirth rate (per 1,000 births)
was 4.2 overall, 2.4 in pregnancies with no fetal
Risk factors for stillbirth
growth restriction, 9.7 when fetal growth restric-
tion was detected antenatally, and 19.8 when fetal
growth restriction was not detected antenatally.
This study identifies fetal growth restriction using country-specific data) for infants born in
as the main risk factor for stillbirth. Antenatal de- hospital in 23 developing countries in Africa and
tection of fetal growth restriction and appropriate Latin America (20042005) and Asia (20072008),
early delivery might prevent 600 stillbirths each including 276,436 singleton live births or fresh still-
year in the UK. births. The 90th percentile for birth weight varied
from 3,250 g in India to 4,050 g in Algeria, and the
Gardosi J et al. Maternal and fetal risk factors for stillbirth: population
based study. BMJ 2013; 346: 15 (f108); McCowan LME, Groom KM. prevalence of a birth weight of 4,000 g or greater
Identifying risk factors for stillbirth. Ibid: 7 (f416) (editorial).
was 0.5% in India and 14.9% in Algeria. Factors
found to be significantly associated with macroso-
mia included higher maternal age (2034 years),
Fetal macrosomia in developing higher maternal height, higher parity, higher mater-
countries
nal BMI, maternal diabetes, post-term pregnancy,
and male fetus. Macrosomia was associated with
Fetal macrosomia may lead to perinatal death, increased risk of caesarean section and of adverse
perinatal asphyxia, shoulder dystocia, caesarean maternal outcomes. The risk of adverse perinatal
section, maternal haemorrhage, prolonged labour, outcome was increased in Asia.
There has been little improvement in stillbirth rates and perinatal trauma. In the developed world, the The increase in obesity and diabetes in
in recent years, and the importance of risk factors prevalence of macrosomia has increased along women of reproductive age might have led to an
has been uncertain. A study in the West Midlands with maternal obesity and diabetes. Little is increase in fetal macrosomia worldwide. Research
region of England has included 91,829 live births known, however, about fetal macrosomia in devel- into ways of controlling these factors is needed.
and 389 stillbirths. oping countries. Now, the WHO Global Survey on
Koyanagi A et al. Macrosomia in 23 developing countries: an analysis
Risk factors for stillbirth included parity Maternal and Perinatal Health has provided data of a multicountry, facility-based, cross-sectional survey. Lancet 2013;
381: 47683; Dennedy M, Dunne F. Macrosomia: defining the problem
(para 0 and 3+), ethnicity (African, Afro-Caribbean, about macrosomia (birth weight > 90th percentile worldwide. Ibid: 4356 (comment).

JPOG JUL/AUG 2013 136

JPOG_JulAug_2013_COMBINE_Final.indd 136 8/5/13 2:18 PM


MIMS
Current Opinion in
Video Series

BY DOCTORS
FOR DOCTORS Gastroenterology
By Doctors For Doctors

Professor David Lieberman Professor Nimish Vakil Dr Markus Cornberg


shares his perspective on the present and talks about management of patients with discusses the management of chronic
future of colorectal cancer screening. refractory GERD. hepatitis B.
There is a lot of potential to prevent Successful treatment of refractory GERD The aim of therapy should be the cure
many cancers if we can improve the rate requires thorough investigation of the patient or control of HBV infection without the
of CRC screening. situation. need for life-long treatment.

MIMS Video Series features


interviews with leading experts.
In this Series, find out what these medical experts have to say about latest
updates in the management of refractory GERD, the management of chronic
hepatitis B and the present & future of colorectal cancer screening.

Got a spare 5 minutes?


Go to www.mims.asia/video_series SCAN TO WATCH VIDEO

Brought to you by MIMS

MIMSVideo2013_GERD_ad_206x276.indd 1 1/8/13 12:42 PM


PAEDIATRICS
PAEDIATRICS II Peer
Peer Reviewed
Reviewed

Imaging Paediatric
The Management of
Brain
Hearing Tumours
Loss in Children
Tang Phua Hwee, MBBS, FRCR, MMed Diagnostic Radiology
Marianne D Elloy, MBBS, MRCS, DOH-NS; Andrew H Marshall, FRCS(ORL-HNS)

INTRODUCTION

Prompt identification and management of hearing loss in childhood is essential to en-


sure optimal speech and language development in the early years of life and optimal
school performance for older children. The implementation of universal neonatal hear-
ing screening has facilitated earlier identification of congenital losses, with 1 in 1,000
babies being born in the UK with a permanent hearing loss detectable at birth. However,
for more common acquired otological conditions of childhood such as otitis media with
effusion (OME) or hearing loss in the presence of complex medical needs, the paediatri-
cian may be the first port of call for parents or concerned allied health professionals,
and an understanding of the presentation, assessment and management options can
ensure optimal outcomes for this group of children. The aim of this review is to discuss
the clinical assessment of hearing loss and provide an overview of the management
options available.

Types of Hearing Loss


Hearing loss is classified by both type and severity. The types of hearing loss include
conductive, sensorineural, or mixed hearing loss, which can be subdivided into congeni-
tal or acquired. A differential diagnosis is included in Table 1. The severity of hearing
loss in decibels hearing level (dBHL) is based on a pure tone average, which is the
mean hearing threshold at four different sound frequencies (0.5, 1, 2, and 4 kHz). This
allows classification of the patients hearing as normal (< 25 dBHL) or determines the
extent of the loss; mild (2550 dBHL), moderate (5170 dBHL), severe (7190 dBHL),
profound (91110 dBHL), or total (> 110 dBHL). Management of the hearing loss thus
depends on its aetiology and the extent of the loss.

JPOG JUL/AUG 2013 137

JPOG_JulAug_2013_COMBINE_Final.indd 137 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed

Table 1. Differential diagnosis of paediatric hearing loss

Inheritance/aetiology Sensorineural HL Conductive HL


Congenital Non- Autosomal dominant DFNA chromosome loci: approx
syndromic Autosomal recessive 40 gene loci identified. Most
X-linked common is GJB2 (connexin 26)
DFNB chromosome loci: approx
35 gene loci identified. Most
common is GJB2 (connexin 26)
DFN chromosome loci: approx 5
gene loci identified
Congenital ossicular
anomalies/fixation
Syndromic Autosomal Dominant Branchio-oto-renal syndrome
Craniosynostosis syndromes (Crouzon, Apert,
Muenke, Pfeiffer)
DiGeorge syndrome
Noonan syndrome
Osteogenesis imperfecta
Marshall syndrome Treacher Collins syndrome
Neurofibromatosis type 2
Saethre-Chotzen syndrome
Stickler syndrome
Waardenburg syndrome
Autosomal recessive Albers-Schonberg disease
Pendred syndrome
Enlarged vestibular aqueduct
syndrome
Usher syndrome
X-linked Alport syndrome
Mohr-Tranebjaerg syndrome
Trisomy
Down syndrome
Sporadic, autosomal
CHARGE syndrome
dominant or autosomal
Goldenhar syndrome
recessive
Alport syndrome
Acquired Prenatal Intrauterine infections
Intrauterine exposure to
ototoxic drugs
Postnatal Infectious AOM and complications of
AOM Meningitis
Viral: measles, mumps, CMV
Inflammatory Chronic suppurative otitis media
OME
Trauma Tympanic membrane
perforation
Temporal bone fracture
AOM = acute otitis media; CMV = cytomegalovirus; OME = otitis media with effusion.

JPOG JUL/AUG 2013 138

JPOG_JulAug_2013_COMBINE_Final.indd 138 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed
PAEDIATRICS

CLINICAL ASSESSMENT audiological referral criteria are the same as for


the well baby protocol; in addition, if either test
Detection is missed or the tests provide inconsistent results,
The detection of hearing loss either occurs as a re- then audiological review is recommended. A poten-
sult of a universal neonatal hearing screening pro- tial pitfall using OAEs as a screening tool is the po-
gramme or as a result of parental or professional tential failure to identify a condition called auditory
concern. neuropathy. In these children, OAEs are present but
Universal neonatal hearing screening is now their auditory brainstem response and functional
well established in the United Kingdom, and its aim hearing can be very poor. This condition is more
is for early identification of hearing loss as early prevalent in babies that would be identified by the
intervention results in better speech and language NICU/SCBU protocol in particular due to prematu-
outcomes. rity or neonatal infection.
The programme aims for babies to be screened
by the gestational age of 44 weeks but preferably History
before discharge from hospital. Premature babies The history in neonates should explore the risk fac-
should not be screened before the gestational age tors identified in the neonatal hearing screening
of 34 weeks. Two screening pathways exist: the pathway which includes parental or professional
well baby protocol and the neonatal intensive care concern regarding the infants hearing or develop-
unit/special care baby unit (NICU/SCBU) protocol ment of auditory or vocal behaviour, high risk of
(for those babies admitted to NICU/SCBU for more chronic middle ear problems, for example, Down
than 48 hours). The exclusions for hearing screen- syndrome or cleft palate, craniofacial anomalies,
ing are ear atresia, microtia, or meningitis, with a family history of permanent sensorineural hearing
recommendation of direct referral for audiological loss from early childhood (in parents or siblings),
assessment in this high-risk group. intermittent positive pressure ventilation, on NICU
A dedicated hearing screening team under- or SCBU for more than 5 days, jaundice or hyperbili-
takes the assessments. The first step of the well rubinaemia requiring exchange transfusion, proven
baby protocol comprises otoacoustic emissions or possible congenital infections, TORCH (Toxoplas-
(OAEs) testing. If the baby passes this test and mosis Other: syphilis Rubella CMV Herpes), neuro-
there is no history of risk factors, then the baby is degenerative or neurodevelopmental disorders, and
discharged from the programme. If the baby fails exposure to ototoxic drugs with monitored levels
the test, it can be repeated on a second occasion; outside the therapeutic range. However, it is im-
however, if the test is failed again, an automated portant to be aware that 50% of newborns born
auditory brainstem response (AABR) test is per- with permanent bilateral congenital hearing loss
formed. If this is passed and there are no risk fac- do not have any known risk factors. Other risk fac-
tors, the baby can be discharged; however, if the tors which have been identified include bacterial
AABR is missed or incomplete, or if there are no meningitis, whereby 10% of children will develop
clear responses in one or both ears, or risk factors a subsequent sensorineural hearing loss, Apgar
are identified, further audiological assessment will scores (04 at 1 minute and 06 at 5 minute), birth
be arranged. The NICU/SCBU protocol differs with weight less than 1,500 g, and consanguinity.
all babies undergoing an OAE and AABR test. The The history in older children should focus on

JPOG JUL/AUG 2013 139

JPOG_JulAug_2013_COMBINE_Final.indd 139 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed

the impact of the hearing loss on the childs speech ophthalmological anomalies and all children with
and language development, and school perfor- a moderate or greater sensorineural hearing loss
mance including social interaction with their peers. should be reviewed by an ophthalmologist.
The aforementioned risk factors should be explored In older children, the most common cause of
and, in addition, enquiry about previous head injury childhood hearing loss is OME, and a number of dif-
and temporal bone fracture. ferent characteristic otoscopic appearances have
been identified including a dull tympanic membrane,
loss of the light reflex, flattening of the handle of
the malleus, a golden or blue hue of the tympanic
membrane, and indeed sometimes an air fluid me-
niscus or retrotympanic bubbles. Otosocopy should
also assess for the presence impacted cerumen, a
Syndromic hearing foreign body, infection, congenital cholesteatoma,
tympanosclerosis (the presence of calcification in
loss can occur in up
the tympanic membrane which can sometimes also
to 30% of children affect the ossicular chain), perforation (including
the site, extent, and status of the middle ear mu-
with bilateral
cosa), or an attic defect with the possibility of cho-
permanent hearing lesteatoma.
loss and other The facial nerve should also be assessed par-
ticularly in the presence of pathology.
associated features
may be noted Investigations

Audiological Assessments
The objective audiological assessments can be per-
formed for children of any age as no contribution to
the testing process is required by the patient.
Otoacoustic emissions: the principle of
OAEs is that objective sounds are emitted from the
Examination outer hair cells of a normally functioning cochlea.
In neonates with hearing loss, a full clinical exami- OAEs can be spontaneous or occur in response to
nation should be undertaken as syndromic hearing acoustic stimulus. Transient-evoked OAEs are used
loss can occur in up to 30% of children with bilat- in neonatal hearing screening, whereby broadband
eral permanent hearing loss and other associated clicks are delivered to the ear by a handheld probe
features may be noted. which also contains a microphone to detect the
Otoscopy may be normal. Examination for emissions. The presence of OAEs indicates a hear-
craniofacial anomalies, preauricular pits, sinuses, ing threshold of 2040 dBHL. The test is quick and
and branchial pits should be undertaken. Some syn- easy to administer, is not affected by sleep, and has
dromes with hearing loss can be associated with a high sensitivity (97%), making it a useful screen-

JPOG JUL/AUG 2013 140

JPOG_JulAug_2013_COMBINE_Final.indd 140 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed
PAEDIATRICS

ing tool. The limitations include inability to esti- response to the sound, a visual reward is triggered
mate hearing thresholds or assess specific frequen- by the audiologist conducting the test, for example,
cies. Specificity is low and patients can fail if they an illuminated moving toy. The advantages are that
have impacted wax or OME. In contrast, a patient hearing thresholds can be determined; however,
can pass this test but still have hearing problems children can tire and lose interest which reduces
due to an auditory neuropathy. accuracy.
Auditory brainstem response: Auditory Conditioning/play audiometry: this can be
brainstem response can be undertaken as an auto- used for children aged above 23 years who can
mated test or a manually interpreted test to gain in- obey simple commands. The child is instructed to
formation about frequency-specific hearing thresh- perform a task each time they hear a sound pre-
olds including bone conduction thresholds and sented by headphones or sound field, for example,
aided thresholds, which is useful in the presence put a wooden man into a wooden boat. This is easy
of ear canal atresia and microtia. The test takes to perform and can establish hearing thresholds but
longer to administer and may require sedation or is dependent on the compliance of the child.
general anaesthesia. The test records the activity Pure tone audiometry: this is the same test
of the eighth cranial nerve and auditory pathways in as used for testing the hearing in adults, requiring
response to acoustic stimulus (via headphones) by the patient to press a button each time they hear
adhesive scalp electrodes. It has a high specificity a sound. This can be used in children aged 4 years
and sensitivity (> 90%). and above, depending on their compliance. Parents
Tympanometry: this test does not assess and siblings should be investigated with pure tone
hearing but is used to assess the compliance of audiometry.
the tympanic membrane and is particularly useful Imaging: imaging is indicated in patients with
in the assessment of OME. The shape of the graph a bilateral severe to profound hearing loss, severe
produced gives information about middle ear com- to profound unilateral hearing loss, or a progres-
pliance, and a normal peak (type A) suggests nor- sive loss. Local protocols vary and a combination of
mal middle ear function, a flattened peak (type B) computed tomography or magnetic resonance imag-
is suggestive of OME (or perforation in the presence ing (MRI) scanning can be utilized. The anatomical
of a high ear canal volume), and a peak shifted to a features or variations of the cochlear nerves and
more negative pressure (type C) suggests Eustachi- structure of the cochlea are well demonstrated by
an tube dysfunction. MRI. However, the presence of ossification within
the cochlea, in particular, following meningitis is
Behavioural Testing optimally imaged using computed tomography.
Visual reinforcement audiometry: this can be
used for children age 6 months to 3 years. It is un- Other Investigations
dertaken in a specially adapted audiology room us- Electrocardiogram (ECG): all children identified
ing either sound field speakers or ear inserts. The to have a bilateral permanent hearing loss should
child must be able to sit on a parents lap and be undergo an ECG to assess for a prolonged QT inter-
able to turn their head to the sound. A distracter en- val which is typically found in Jervell and Lange-
tertains the child with toys and sounds are played Neilsen syndrome, and if anomalies are identified
via the speakers; when the child turns correctly in referral to a cardiologist should be undertaken.

JPOG JUL/AUG 2013 141

JPOG_JulAug_2013_COMBINE_Final.indd 141 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed

Figure 1. Soft band hearing aid. geneticists to investigate aetiology of hearing loss
and undertake genetic counselling with the childs
family.
The childs family will require a significant
amount of support and input from the allied health
professionals. The audiologists not only assess
the childs hearing thresholds but also are the key
professional group in the provision of adjuncts to
hearing rehabilitation including the management
of hearing aids, bone anchored hearing aids, and
cochlear implant programming. The teachers of
the deaf provide support in the learning environ-
ment before children even start school. They spend
time in the classroom to assess what adjuncts may
be required and train the teachers in mainstream
schools on how to support children with hearing im-
pairment. The speech and language therapists in-
put is essential to optimize language development,
Blood tests and urinalysis: screening for and they often work both in the community and the
congenital infections can be undertaken by test- school to provide input. At the initial consultation,
ing for antibodies to toxoplasmosis, syphilis, ru- simple measures to enhance a childs hearing abili-
bella, cytomegalovirus, and herpesvirus hominis. A ties should be discussed with the parents, including
blood test for connexin 26, the most common non- classroom placement strategies.
syndromic genetic cause of hearing loss, can be
undertaken. Urea and electrolytes accompanied by Conservative Management
urinalysis for haematuria should be undertaken as Children who are making satisfactory progress de-
part of a screen for Alport syndrome and branchio- spite a mild to moderate hearing loss may be man-
oto-renal syndrome. Thyroid function tests may be aged with supportive measures. The majority of
normal in the early stages of Pendred syndrome, children with OME are managed conservatively as a
and the classical investigation described is the per- significant proportion resolve spontaneously.
chlorate discharge test. In those children requiring intervention, the
use of hearing aids for amplification is the main-
MANAGEMENT stay of auditory rehabilitation. Most children will
require behind-the-ear digital hearing aids, with
For optimal outcomes, the management of paedi- multiple brands and models available. These can be
atric hearing loss requires a multidisciplinary ap- programmed to the childs hearing loss and a mould
proach. This can involve a host of medical speciali- can be made to fit specifically in their ear, which
ties, in addition to ear, nose and throat surgeons, can be personalized with the logo of their favourite
the general practitioner, paediatricians, ophthal- football team being popular for boys and glitter be-
mologists, to manage ocular anomalies and the ing a hit with girls! For children with ear canal atre-

JPOG JUL/AUG 2013 142

JPOG_JulAug_2013_COMBINE_Final.indd 142 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed
PAEDIATRICS

sia, microtia and other conductive deficits, a soft Figure 2. Cochlear implant.
band hearing aid a bone conductor hearing aid
attached to a soft head band is particularly useful
(Figure 1). Adjunctive devices such as FM systems
for the classroom can also be valuable in hearing
rehabilitation.

Surgical Management
Grommets (ventilation tubes): grommets are
small (plastic or titanium) tubes that are inserted
into the tympanic membrane to aerate the middle
ear. Multiple types of grommets are available with
the design impacting on the duration that the grom-
met is maintained in position. The main indication
for grommet insertion is persistent OME with hear-
ing loss. The National Institute for Clinical Excel-
lence (NICE) guidance (2008) advocates a 3-month
period of active observation as during this period
the effusion will resolve in 60% of children. For How a cochlear implant works
persistent bilateral OME with hearing in the better 1. The sound processor (A) captures sound and converts it
into digital code.
ear of 2530 dBHL or worse, grommets can be of-
2. The sound processor transmits the digitally coded sound
fered, or if the hearing is less than 2530 dBHL but through the coil (B) to the implant (C) just under the skin.
is significantly impacting the childs development 3. The implant converts the digitally coded sound to electrical
or education. Children with Down syndrome often signals and sends them along the electrical signals and
have persistent problems with OME, and grommet sends them along the electrode array, which is positioned
in the cochlea.
placement can be technically difficult owing to nar-
4. The implants electrodes stimulate the cochleas hearing
row ear canals and children often require multiple nerve fibres, which relay the sound signals to the brain to
sets of grommets. As such, a separate pathway is produce hearing sensations.
provided for children with Down syndrome, with
more emphasis on utilization of hearing aids than 1-week course of topical antibiotic ear drops is ad-
grommets. A third pathway for children with cleft vocated as the first-line treatment, on rare occa-
palate advocates the use of grommets in the pres- sions, for refractory infections grommets have to be
ence of persistent OME and hearing loss. surgically removed.
The advantages of grommet placement are Bone-anchored hearing aid (BAHA): the
instantaneous improvement in hearing. This quick indications for BAHA in children include congeni-
procedure is carried out under general anaesthetic tal aural atresia and microtia, chronic suppurative
in children and often preferred by parents to the otitis media, persistent OME, chronic otitis externa,
alternative; a hearing aid as compliance is not an unilateral profound hearing loss, failure with con-
issue. The risks of surgery include bleeding, infec- ventional aids, and trauma to the external ear ca-
tion, and perforation. In the event of infection, a nal. A BAHA is a titanium screw inserted into the

JPOG JUL/AUG 2013 143

JPOG_JulAug_2013_COMBINE_Final.indd 143 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed

Figure 3. Cochlear implant. The risks of surgery include failure of osse-


ointegration, soft tissue complications, trauma,
fixture displacement, and extradural haematoma.
Outcomes measures include favourable audiologi-
cal outcomes in comparison to conventional bone
conductor aids and good patient usage and satis-
faction after up to 10 years after fitting.
Cochlear implant: A cochlear implant is
a surgically implantable device which bypasses
damaged hair cells in the cochlea and directly
stimulates the cochlear nerve (Figures 2 and 3). It
comprises a microphone (which captures speech
and sound), processor (which converts the sound
into an electronic signal), transmitting coil (which
transmits the electronic signal), internal receiver/
stimulator (which receives the electronic signal and
converts it to electrical impulses), and an electrode
array (which delivers the electrical impulses to the
cochlear nerve). Modern electrodes are multichan-
nel with the common brands having 16 and 22 chan-
nels. Each channel stimulates a specific frequency.
Insertion of an electrode into the cochlear risks
destroying any residual hearing and makes the ear
reliant on the cochlear implant. However, hybrid
cochlear implants have been recently developed
for use in patients with serviceable low-frequency
hearing but a profound high-frequency loss, where-
by a custom made shortened electrode is inserted
with the aim of preserving existing hearing in the
low frequencies.
NICE guidance introduced in 2009 recom-
calvarial bone behind the ear. Osseointegration mended that simultaneous bilateral cochlear im-
occurs, resulting in bony growth into the titanium plantation was indicated in children with severe
screw. Once this has occurred, typically after 36 to profound hearing loss who did not receive ad-
months, a bone conduction hearing aid system can equate benefit from conventional hearing aids. The
be attached, providing the child with amplification. guidelines defined the severe to profound hearing
Typically, children cannot undergo implantation un- loss as only hearing sounds louder than 90 dBHL
til the age of 4 years; until a child is old enough to at the frequencies of 2 and 4 kHz without hearing
undergo implantation, a soft band hearing aid can aids and recommended the utilization of speech,
be used. language, and listening skills appropriate for age,

JPOG JUL/AUG 2013 144

JPOG_JulAug_2013_COMBINE_Final.indd 144 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed
PAEDIATRICS

combined with development stage and cognitive Practice points


ability as factors to consider when assessing the
benefit of hearing aids. The main contraindications
to cochlear implantation include lack of a cochlear Universal neonatal hearing screening identifies 1 in 1,000
babies with a permanent hearing impairment at birth.
nerve and complete ossification of the cochlea.
Management of hearing loss is a multidisciplinary approach.
Patients who are candidates for cochlear im-
Hearing loss can be associated with a syndrome, and associated
plantation will be assessed by a specialist multi-
features should be identified and investigated.
disciplinary cochlear implant team including au- Early identification and intervention of hearing loss improves
diological investigations, imaging, trial of hearing speech and language outcomes.
aids, review by the speech and language therapists, Conventional digital hearing aids or soft band bone conductor
and teachers of the deaf to assess for suitability. hearing aids can be used for children.
Confirmation that children are vaccinated against If hearing aids provide inadequate response to sound, patients
pneumococcus will be sought preoperatively from can be referred for assessment for either bone-anchored
the general practitioner. hearing aid or cochlear implants, depending on their hearing
thresholds and pathology.
The risks of surgery include altered taste, in-
fection, meningitis, device failure, and facial nerve
injury. It is also important to note that children with
cochlear implants cannot undergo MRI. http://hearing.screening.nhs.uk/; 2011 (accessed 5 Mar 2011).
Outcome measures have been used to assess NICE. Cochlear implants for children and adults with severe to
profound deafness: NICE technology appraisal guidance 166. Avail-
the efficacy of cochlear implantation, and there is
able online at http://guidance.nice.org.uk/TA166; 2009 (accessed 5
a wealth of literature supporting the success of Mar 2011).
hearing rehabilitation and these include sound lo- NICE. Surgical management of otitis media with effusion in children:
NICE clinical guideline 60. Available online at http://guidance.nice.
calization and speech recognition in noise meas- org.uk/CG60; 2008 (accessed 5 Mar 2011).
ures and quality of life. Reports have revealed that Papsin B, Gordon K. Bilateral cochlear implants should be the stan-
dard for children with bilateral sensorineural deafness. Curr Opin
many children who received cochlear implants at an Otolaryngol Head Neck Surg 2008;16:6974.
early age are achieving age-appropriate academic Phillips J, Yung M, Burton M, Swan I. Use of aminoglycoside contain-
ing ear drops in the presence of a perforation evidence review and
performance.
ENT UK consensus statement. Available online at http://www.
entuk.org/news/news/attachments/eardrops; 2007 (accessed 5
FURTHER READING Mar 2011).
US Preventative Services Task Force. Universal screening for hearing
loss in newborns: US Preventative Services Task Force recommen-
Browning G. Clinical otology & audiology. London: Arnold, 2001.
dation statement. Pediatrics 2008;122:143148.
Gerber S. The handbook of pediatric audiology. Washington: Gallau-
det, 1996.
Graham J, Scadding G, Bull P. Pediatric ENT. Heidelberg: Springer,
2007.
Johnston J, Durieux-Smith A, OConnor A, Fitzpatrick E. Bilateral 2011 Elsevier Ltd. Initially published in Paediatrics and Child
cochlear implants: a critical review. Int J Audiol 2009;48:601617. Health 2011;22(1):1318.
Kral A, ODonoghue G. Profound deafness in childhood. N Engl J Med
2010;363:14381450.
Kunst D, Kremer H, Cremers C. Genetics for ENT specialists. London: About the Authors
Remedica, 2005. Marianne D Elloy is Specialist Registrar in Otolaryngology at the
McDermott A-L, Sheehan P. Bone anchored hearing aids in children. Royal Derby Hospital, Derby, UK. Andrew H Marshall is Consul-
Curr Opin Otolaryngol Head Neck Surg 2009;17:488493. tant in Otolaryngology at the Queens Medical Centre, Notting-
NHS. Newborn Hearing Screening Programme. Available online at ham, UK.

JPOG JUL/AUG 2013 145

JPOG_JulAug_2013_COMBINE_Final.indd 145 8/5/13 2:18 PM


PAEDIATRICS
OBSTETRICS
OBSTETRICS II Peer
Peer Reviewed
Reviewed

Imaging
Management of Paediatric
Early Pregnancy
Brain Tumours
Complications
Tang Phua Hwee, MBBS, FRCR, MMed Diagnostic Radiology
Harriet Pugsley, MB ChB, MRCOG; Judith Moore, MRCOG

INTRODUCTION

Most women presenting with complications in early pregnancy are assessed, diagnosed
and managed at early pregnancy assessment units (EPAUs). These units aim to provide
thorough assessments, access to specialist investigations (scan, human chorionic gon-
adotrophin [hCG]), a rapid turnaround of results, and co-ordination of further manage-
ment.
The EPAU enables continuity of care, fewer admissions, and planned follow-up. It
is beneficial in the provision of open access for GPs, and ideally patients particularly
following a previous pregnancy loss. By streamlining investigations and treatment, this
system is also more cost-effective.
For women who have had previous pregnancy complication, a familiar setting and
ongoing support in a future pregnancy is a valued service.
A downside of the EPAU system is that it is often only available at limited times,
thus for complications occurring outside of these hours, patients require ward contact
numbers and more frequent inpatient based care.

HYPEREMESIS GRAVIDARUM

Over 50% of women suffer from nausea in pregnancy. Hyperemesis gravidarum is the
inability to maintain hydration, resulting in dehydration and ketonuria as a result of
nausea and vomiting in pregnancy. It affects between 0.1% and 1% of women. Patients
become dehydrated and ketonuric, develop an electrolyte imbalance (hyponatraemia and
hypokalaemia), and in severe untreated cases a nutritional (thiamine) deficiency culmi-
nates in Wernickes encephalopathy.

JPOG JUL/AUG 2013 146

JPOG_JulAug_2013_COMBINE_Final.indd 146 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

Symptoms develop around 68 weeks of gesta- Practice points I


tion and are directly related to levels of hCG, peak-
ing towards the end of the first trimester before Hyperemesis
settling in the second trimester. Women present 50% of women suffer from nausea in pregnancy
with nausea, weakness, vomiting, and occasionally 0.11% of women suffer from hyperemesis gravidarum
ptyalism (inability to swallow saliva). On examina- Symptoms peak at the end of the first trimester
tion, there are signs of dehydration and tachycardia In severe cases, there is a risk of Wernickes encephalopathy re-
with or without hypotension. Those who have suf- sulting from a nutritional imbalance of reduced thiamine
fered with hyperemesis in a previous pregnancy are Treatment includes intravenous rehydration, electrolyte monitor-
more likely to develop similar symptoms in subse- ing and restitution, antiemetics, thiamine supplementation and,
quent pregnancies. Nausea and vomiting present- in non-resolving cases, steroid therapy
ing after 12 weeks of gestation is not hyperemesis Molar and multiple pregnancies should be excluded
gravidarum.
The proposed pathophysiology behind hy-
peremesis is related to the hCG. The hCG molecule nous rehydration with normal saline or Hartmanns
has common alpha subunit with thyroid-stimulating with potassium supplementation and monitoring of
hormone and is thought to exert its effect via a electrolytes and ketonuria should be employed. In-
temporary physiological thyrotoxicosis; there may fusions containing dextrose should be avoided as
be evidence of a raised free thyroxine and a low they may precipitate Wernickes encephalopathy.
thyroid-stimulating hormone. This accounts for the Women requiring admission are intravascularly de-
timing of the onset and settling of symptoms in cor- hydrated posing an increased thrombotic risk, thus
relation with levels of hCG. There may also be psy- thromboprophylaxis should be considered. Rarely,
chological and cultural factors. intractable cases may require treatment with ster-
In all presentations, a multiple or molar preg- oids to relieve symptoms.
nancy should be excluded as these conditions also Hyperemesis is not uncommon; the majority of
result in an increased hCG level. cases can be successfully treated as a day admis-
Many women with mild symptoms are managed sion to an EPAU. The outcome for the both preg-
in the community; once women are ketotic and un- nancy and patient are excellent.
able to maintain hydration, admission is necessary.
For the majority of women, intravenous rehydration MISCARRIAGE
with antiemetic medication, on a day case basis
without admission, is sufficient to break the cycle Fifteen percent to 20% of pregnancies end in mis-
and allow the patient to re-establish oral intake. carriage.
Many units have managed to expand the role of the Miscarriage was traditionally classified as
EPAU to incorporate this service and release capac- threatened, inevitable, complete, incomplete, or
ity on the admitting wards. However, more severe missed. Most women present with pain and bleed-
cases do require an inpatient admission, further ing in early pregnancy. Alternatively, miscarriage
rehydration, antiemetics, thiamine supplements, may be diagnosed at the early dating scan with
daily electrolyte analysis, and a gradual resumption no prior warning symptoms, this is classified as a
of oral intake and monitoring of ketonuria. Intrave- missed miscarriage.

JPOG JUL/AUG 2013 147

JPOG_JulAug_2013_COMBINE_Final.indd 147 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

Practice points II appropriate contact numbers and advised to return


if their bleeding becomes heavy.
If there are symptoms (or with minimal symp-
Miscarriage
toms), treatment of miscarriage is based on three
Affects up to 1 in 5 pregnancies
alternatives: expectant, medical, or surgical. Ex-
Treatment options are expectant, medical, and surgical
pectant management employs awaiting the natural
Expectant management aims to avoid surgery, may result in
course of events, for the products to pass sponta-
prolonged follow-up with a risk of heavier bleeding and failed
neously. Medical management involves combina-
treatment
tions of oral or vaginal prostaglandins to induce the
Medical management aims to avoid surgery, may be uncomfort-
completion of miscarriage. Surgical management
able with heavier bleeding and risk of later surgery
involves an operation, usually vacuum aspiration,
Surgical management allows early completion of treatment with
to remove any remaining products of pregnancy.
the risk of surgical and anaesthetic complications
There are risks and benefits associated with
There is no difference in post-treatment conception rates regard-
all approaches:
less of method of management
1. Expectant management requires a longer fol-
low-up, often multiple visits, and is associated
with more prolonged and heavier bleeding.
An episode of pain or bleeding in an early Owing to the unpredictable of length of follow-
pregnancy subsequently demonstrated to be a vi- up and number of visits associated with expect-
able pregnancy is termed a threatened miscarriage. ant management, many women veer away from
Traditionally, classifications of complete, in- this option. There is a higher risk of treatment
complete and inevitable miscarriage have been failure and an increased need for later surgery.
used. Diagnosis and management are based on This method of treatment may be more effec-
clinical findings: (1) examination of vaginal loss tive for those who are symptomatic of pregnan-
for products of conception, and (2) examination cy loss (ie, those presenting with an incomplete
of the cervical, to determine whether it is open or miscarriage) because the process of spontane-
closed. More recently, there has been a tendency ous loss will have already begun. The timescale
to be increasingly conservative in management of for completion of expectant management can
miscarriage with routine use of ultrasound to aid extend over 26 weeks with the emphasis on
management. Once an intrauterine pregnancy is planned follow-up at specified intervals for
demonstrated by ultrasound, with uncertain viabil- assessments usually with ultrasound scans
ity, a follow-up scan will be arranged 1014 days to ensure all products have passed. Although
later to determine whether the pregnancy is ongo- figures differ depending on the length and type
ing or not. If initial symptoms are of heavy bleeding, of follow-up that patients have (clinical versus
or if they worsen between appointments, then med- ultrasound), around 80% complete without
ical intervention in terms of a surgical evacuation intervention. When counselling women, the
may be necessary at an earlier stage. Patients with possibility of a period of prolonged follow-up
a pregnancy of unknown viability, who are waiting and of the risk of incomplete loss with the
for follow-up, must be informed of the risk of pain subsequent need for surgery should be stressed.
Medical management also aims to avoid
and bleeding between appointments and be given 2. 

JPOG JUL/AUG 2013 148

JPOG_JulAug_2013_COMBINE_Final.indd 148 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

surgery and is an accelerated method of Practice points III


conservative management with a more predict-
able timescale of completion. It can be carried
Recurrent miscarriage
out as an inpatient or outpatient procedure,
Defined as three or more consecutive miscarriages
provided support is available for women at
Affects 1% of couples
home if needed. If managed in the community,
For the majority of patients, all investigations are normal
follow-up is necessary once again, to ensure
A minority of patients will have antiphospholipid syndrome,
completion and that ongoing complications are
which can be treated with heparin and aspirin to improve the
recognized and treated.
chance of an ongoing pregnancy
3. 
The benefit of surgical management is to
limit the need for prolonged follow-up,
reduce the number of symptomatic days, and
for early closure of treatment. Complications, It is recommended that investigations into
although infrequent, include uterine perforation, why women have miscarried should be implement-
cervical trauma, incomplete evacuation, the ed following the third consecutive miscarriage.
risk of the anaesthetic, and a slightly higher For the majority of women, the results are normal.
infection rate when compared with expectant Prognosis for the future worsens with increasing
management. numbers of pregnancy losses, advancing maternal
The miscarriage treatment trial and subse- age, normal histopathology, and a normal parental
quent Cochrane reviews (2006, 2010) have con- karyotype. Recurrent miscarriage is particularly
cluded that there is no superior method of man- devastating for couples as they may be able to
agement and have recommended that the womans conceive relatively easily but not carry an ongoing
preferences are taken into account when planning pregnancy; they may thus need increased support
care; treatment should therefore be patient-guided, and counselling. EPAUs arrange specialist follow-
based on an informed decisions. up and offer a patient educational role with the
Importantly, the long-term follow-up of the provision of information combined with access to
miscarriage treatment trial concluded that there support groups and counselling.
is no difference in later conception rates follow- Following recurrent miscarriage, investiga-
ing the different approaches to management. It has tions may include screening for antiphospholipid
also been suggested that empowering patients by syndrome, karyotyping the products of conception,
choice in their management reduces subsequent diagnostic imaging for structural abnormalities, and
anxiety and depression rates. Women who have parental karyotyping looking for balanced translo-
been involved in the decision-making process have cations. The incidence of controlled diabetes and
subsequently scored more favourably on quality-of- thyroid disease are no different in this population
life outcome questionnaires. when compared with that of the general public.
As stated, for the majority of patients, inves-
Recurrent Miscarriage tigations will be normal and no cause is identified.
Recurrent miscarriage is defined as three or more In a minority, there may be a treatable haemato-
consecutive miscarriages and affects 1% of couples logical factor such as antiphospholipid syndrome.
trying to conceive. In the case of recurrent miscarriage, positive serum

JPOG JUL/AUG 2013 149

JPOG_JulAug_2013_COMBINE_Final.indd 149 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

Practice points IV no fetal tissue.


Partial moles are triploid in nature, may con-
tain fetal parts, and are the result of dispermic fer-
Molar pregnancy
tilization.
Affects 1 in 700 conceptions
The concern with molar conceptions is the
The concern is the risk of progression to neoplasia if unrecog-
risk of progression to neoplasia if left untreated.
nized or untreated
Molar pregnancies can present as a miscarriage
All cases must be registered at a tertiary referral centre and re-
or, more rarely, after a normal pregnancy. They are
quire long-term follow-up
associated with characteristic appearances on an
The risk of recurrence is low (12%)
ultrasound scan (snow storm/placental vacuoles).
Cases which have progressed to neoplasia are responsive to
Molar pregnancies are usually detected in the
treatment; 56% require chemotherapy with a 98% cure rate
first trimester because women either present with
bleeding, resulting in an early ultrasound scan, or
are suspected on the dating ultrasound scan. They
blood results on two separate occasions 12 weeks are also associated with a raised hCG level, and the
apart with a history of three consecutive pregnancy condition should be excluded in women presenting
losses before 10 weeks are necessary to confirm with hyperemesis.
antiphospholipid syndrome. (Positive bloods in as- Diagnosis is sometimes retrospective follow-
sociation with a single pregnancy loss above 10 ing histological examination of products of concep-
weeks or a poor obstetric outcome before 34 weeks tion removed following a surgical evacuation. In
secondary to placental disease also fulfil the crite- cases of miscarriage, products should be screened
ria for antiphospholipid syndrome.) For these wom- for gestational trophoblastic disease.
en, treatment with aspirin and heparin will improve Once suspected, the majority are easily treat-
the chance of an ongoing pregnancy. Without treat- ed by surgical evacuation alone, with histological
ment, the likelihood of a term pregnancy may be as confirmation. However, owing to the low but poten-
low as 10%; with treatment, the miscarriage rate tially life-threatening risk of ongoing trophoblastic
may be reduced by up to 54%. tissue and neoplasia, registration and referral to
specialist tertiary care is crucial.
MOLAR PREGNANCY Due to the nature of hydatidiform gestational
trophoblastic disease/neoplasia (GTN) with the po-
Molar pregnancies are rare, affecting roughly 1 in tential for progressive disease, long-term follow-up
700 conceptions. They are the result of an abnor- is necessary. This involves prolonged measurement
mal conception and can be either complete moles of urine hCG levels to ensure disease-free status,
or partial moles. They are more common at the ex- avoidance of future pregnancy until hCG levels have
tremes of reproductive ability and among people been normal for 6 months, and further monitoring
living in Asia. The risk of recurrence is low: 12%. after all future pregnancies.
Complete moles are diploid, contain only pa- Treatment with surgical evacuation and pro-
ternal chromosomes, and are either the result fer- longed monitoring of hCG levels are usually all that
tilization of an empty ovum with duplication of a is required, with repeat monitoring of levels in sub-
single sperm or dispermic fertilization. They contain sequent pregnancies. All UK cases of gestational

JPOG JUL/AUG 2013 150

JPOG_JulAug_2013_COMBINE_Final.indd 150 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

trophoblastic disease are monitored via a national Practice points V


registration programme consisting of three centres
based in Sheffield, Dundee, and Charring Cross.
hCG is a marker of ongoing trophoblastic dis- Ectopic pregnancy
ease. If levels are persistently elevated, further Affects 1 in 50 pregnancies
treatment is required; this is usually medical with The main concern is the risk of life-threatening bleeding
methotrexate and, in rare cases, chemotherapy; a Management options depend largely on presentation and include
repeat evacuation is not usually helpful. GTN is mainly surgery or methotrexate (rarely, expectant management
extremely responsive to treatment. Chemotherapy may be employed in the case of resolving trophoblast)
is necessary in only 56% of cases with a greater Salpingotomy is only recommended during surgical treatment if
than 98% cure rate and is more commonly required there is a concern that the contralateral tube is non-functional
following complete rather than partial molar preg-
nancies.
Following diagnosis and treatment, women blood vessel, resulting in massive pelvic haemor-
are advised to delay further conception until 6 rhage; this can happen with or without rupture of
months following normalization of the hCG levels the Fallopian tube.
and a year after completion of chemotherapy. Bar- Presentation, as with most early pregnancy
rier contraception is advised; hormonal contracep- complications, is with pain with or without vaginal
tion is an option once hCG levels have normalized. bleeding, or as collapse with associated concern-
If contraception has been commenced before diag- ing symptoms of dizziness and fainting. Diagno-
nosis, there may be a slight increase in the risk of sis is based on history, clinical examination, and
gestational trophoblastic disease progressing to investigations, including transvaginal ultrasound
neoplasia with combined preparations, but there scan, serial hCG measurements, and laparoscopy.
is no evidence of detriment from single agent pro- In a normal pregnancy, the serum hCG level should
gestogens. rise by at least 60% in 48 hours, although anything
Thankfully, the recurrence risk is low, so wom- above 30% can be consistent with normal early
en who continue to have a future pregnancy can be pregnancy. The hCG levels in an ectopic pregnancy
reassured that 98 out of 100 conceptions will not be may initially rise at a normal rate, but by the time
a further molar pregnancy. In the women in whom a of presentation the rise is usually suboptimal. Once
second molar pregnancy does occur, it is usually of suspected, ectopic pregnancies might be suggested
the same histological type as the first. by ultrasound evidence of gestational tissue out-
side of the body of the uterus with an empty uter-
ECTOPIC PREGNANCY ine cavity. However, laparoscopy remains the gold
standard for confirmation of diagnosis and allows
Ectopic pregnancy complicates approximately 1 in concurrent treatment.
50 pregnancies. Risk factors include previous ec- Although diagnosis can be suggested by pel-
topic pregnancy, a history of pelvic infection, or vic ultrasound and hCG, confirmation can often
past pelvic surgery. If not recognized or managed take several days because of presenting features
inappropriately, it can become a life-threatening in common with other early pregnancy complica-
condition as a result of invasion and rupture of a tions, uncertainty on ultrasound scan, and the need

JPOG JUL/AUG 2013 151

JPOG_JulAug_2013_COMBINE_Final.indd 151 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

For the majority of patients with recurrent miscarriage, is for the hCG level to fall). Laparoscopic surgery
investigations are normal and no cause is identified.
may be initially diagnostic to confirm the ectopic
pregnancy as the cause of pain and then therapeu-
tic by salpingectomy or salpingotomy as definitive
treatment.
Any surgery performed will have implications
for future fertility potential. At the time of surgery,
both Fallopian tubes should be assessed as the
health of the unaffected Fallopian tube is also rele-
vant. The surgical recommendation is for salpingec-
tomy to be performed if the contralateral tube is
deemed healthy and that salpingotomy should only
be performed if there is doubt about fertility with
the remaining tube. This is because preservation of
the Fallopian tube following an ectopic pregnancy
retains the risk of a subsequent ectopic pregnancy
in the same tube, whereas a salpingectomy removes
this risk. The fertility rate from a single functional
tube should be sufficient to allow conception.
If there is a diseased contralateral Fallopian
tube (such as a hydrosalpinx) and a salpingectomy
for serial blood levels. In the stable patient in this is performed, future options for conception are like-
situation, the 48-hour hCG trend and symptoms are ly to depend on in vitro fertilization. The limitation
crucial factors in determining timing and options of visual assessment of the tube should be remem-
for treatment. In cases of haemodynamic insta- bered as it will give an indication of gross disease
bility secondary to a suspected bleeding ectopic but not assess tubal patency for which a dye test is
pregnancy, emergency surgery is necessary either more accurate.
by laparotomy or laparoscopy, in order to stem the Owing to the risk of life-threatening bleeding
bleeding and remove the ectopic as rapidly and from an ectopic pregnancy and the need to be ab-
safely as possible. solutely certain that a viable intrauterine pregnancy
Once diagnosed, there are several options for has been excluded, there are rigid criteria to be met
the management of an ectopic pregnancy, depend- before medical management becomes an option.
ing mainly on presentation and individual circum- It is recommended that ultrasound findings
stances. For example, in the acute emergency due should show a suspected ectopic mass less than 3
to a bleeding ectopic, urgent surgery is required cm in size with minimal free fluid and no visible
as a lifesaving procedure. However, the majority fetal heart. There should be minimal pain on ex-
of ectopic pregnancies are treated by scheduled amination and a suboptimal rise in hCG which is
laparoscopic surgery or by medical management initially less than 3,000 IU/L. (The Royal College of
with methotrexate. Conservative management is Obstetricians and Gynaecologists disseminated na-
also an option with resolving trophoblast (the trend tional recommendation, from which individual units

JPOG JUL/AUG 2013 152

JPOG_JulAug_2013_COMBINE_Final.indd 152 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

set their own treatment pathways). These criteria decreasing and women must be able to seek appro-
are employed to enable patient selection so that priate help and attend hospital easily should they
medical management is an option for appropriate need to. Follow-up needs to be rigorous to ensure
patients who are unlikely to have spontaneous that the hCG titres have returned to non-pregnant
bleeding whilst undergoing treatment. Success of levels and complications are not missed.
treatment is assessed by subsequent hCG estima- For women who have experienced an ectopic
tions. pregnancy, fears for the future include the risk of
The main benefit of methotrexate is the avoid- a recurrent ectopic pregnancy, concerns regarding
ance of surgery. The downside includes the low risk the ability to conceive again, and fear relating to
of failure of treatment in cases in which ongoing the health risks of a recurrent ectopic pregnancy.
trophoblast results in bleeding from the ectopic It may be particularly worrying for women to plan a
site and the need for surgical intervention. further pregnancy if they have already experienced
Methotrexate has antifolate properties and is life-threatening emergency surgery from a ruptured
teratogenic, so patients must be suitably advised ectopic or a previous pregnancy complication. If one
to delay conception following treatment, and care Fallopian tube is diseased, it is likely that the other
must be taken to encourage folic acid prior to and tube is also affected, and spontaneous conception
during subsequent conceptions. may be delayed or impossible. Therefore fertility
may well depend on in vitro fertilization which may
not be an option for many women (limited National
Health Service funding, personal finances, and
emotional reasons).
Women who are informed
of the long-term effects CONCLUSION
of treatment... and who are
For the majority of women, conception, pregnancy,
given an informed choice and birth are straightforward, resulting in an uncom-
regarding their management, plicated confinement, delivery, and healthy baby.
For women who do experience complications,
may find it easier to come
explanation, advice, follow-up, and necessary in-
to terms with their loss vestigations will help when planning for the future
and have less anxiety and and deciding whether to try for a further pregnancy.
Women who are informed of the long-term effects of
depression long-term.
treatment, for example, that surgical/medical man-
agement following a miscarriage does not convey
differences in conception rates, and who are given
an informed choice regarding their management,
Expectant management can be employed for may find it easier to come to terms with their loss
cases of resolving trophoblast, which may either be and have less anxiety and depression long-term. It
a resolving ectopic pregnancy or an early pregnan- must be remembered that each case is individual,
cy miscarriage. For safety, levels of hCG must be all circumstances are different and women need

JPOG JUL/AUG 2013 153

JPOG_JulAug_2013_COMBINE_Final.indd 153 8/5/13 2:18 PM


OBSTETRICS I Peer Reviewed

to be informed of the risks and benefits of all the FURTHER READING


available treatment options.
Royal College of Obstetricians and Gynaecologists. The manage-
The role of the EPAU, with open access and ment of early pregnancy loss. Green-top Guideline No. 25.
support in future pregnancies in terms of regular London: RCOG, 2006.
Trinder J, Brocklehurst P, Porter R, Read M, Vyas S, Smith L.
scanning and as a point of contact for the individual,
Management of miscarriage: expectant, medical or surgical?
has been shown to improve the successful outcome Results of randomized controlled trial (miscarriage treatment
of future pregnancies following miscarriage. Open (MIST) trial). BMJ 2006;332:1235.
Smith LFP, Ewings PD, Quinain C. Incidence of pregnancy after
access and choice in care options have also been expectant, medical or surgical management of spontaneous
advocated as helping women to feel more in con- first trimester miscarriage. Long term follow-up of miscar-
riage treatment (MIST) randomized controlled trial. BMJ
trol of their treatment and reducing post-treatment
2009;339:b3827.
rates of anxiety and depression. Nanda K, Peloggia A, Grimes DA, Lopez LM, Nanda G. Expect-
Besides the feeling of loss, it is normal for ant care versus surgical treatment for miscarriage. Cochrane
Database Syst Rev 2006. Issue 2. Art. No.: CD003518. pub. 2.
women to feel guilt, blame, and anxiety. Women Neilson JP, Gyte GML, Hickey M, Vaquez JL, Don L. Medical
will have concern for their own health, particularly treatments for incomplete miscarriage (less than 24 weeks).
Cochrane Database Syst Rev 2010. Issue 1. Art. No.: CD007223.
if they have required emergency treatment. Part-
pub. 2.
ners may also harbour feelings of guilt, responsi- Neilson JP, Hiskey M, Vaquez JL. Medical treatment for early
bility, and helplessness for the situation, and may fetal death (less than 24 weeks). Cochrane Database Syst Rev
2006. Issue 3. Art. No.: CD002253. pub. 3.
have conflicting emotions with concerns regarding Rai RS, Clifford K, Cohen H, Regan L. High prospective fetal
the risk to their loved ones health in a future preg- loss rate in untreated pregnancies of women with recurrent
miscarriage and antiphospholipid antibodies. Hum Reprod
nancy, balanced with their desire to increase their 1995;10:33013304.
family. Royal College of Obstetricians and Gynaecologists. The manage-
ment of recurrrent miscarriage. Green-top Guideline No. 17.
It is important for patients who have had an
London: RCOG, 2011.
ectopic pregnancy to understand that they require Royal College of Obstetricians and Gynaecologists. The manage-
an early ultrasound in subsequent pregnancies to ment of gestational trophoblastic neoplasia. Green-top Guide-
line No. 38. London: RCOG, 2010.
exclude a recurrence and to seek a medical assess- Royal College of Obstetricians and Gynaecologists. The manage-
ment early in pregnancy. ment of tubal pregnancy. Green-top Guideline No. 21. London:
RCOG, 2004.
Previously, in future conceptions, early open
access and regular specialist follow-up have been
available, enabling reassurance, investigations,
early detection of further complications, along with
support throughout subsequent pregnancies. Unfor-
2012 Elsevier Ltd. Initially published in Obstetrics, Gynaecol-
tunately as healthcare services are put under ever ogy and Reproductive Medicine 2012;22(3):7680.
increasing pressure to reduce costs, open access
may no longer be deemed a financially viable ser-
vice.
It is important that appropriate investigations
are arranged and results collated and explained. It About the Authors
is also necessary that patients are given appropri- Harriet Pugsley is Specialist Registrar in Obstetrics and Gynae-
cology. Judith Moore is Consultant Obstetrician and Gynaecolo-
ate counselling, information, and links to support gist at Nottingham United Hospitals NHS Trust, Nottingham City
groups should they require additional care. Hospital, Nottingham, UK.

JPOG JUL/AUG 2013 154

JPOG_JulAug_2013_COMBINE_Final.indd 154 8/5/13 2:18 PM


GYNAECOLOGY
GYNAECOLOGY II Peer
Peer Reviewed
Reviewed

Ovarian Cancer:
Current Management
and
Autism Future Directions
Spectrum Disorders
Sin E Taylor, BSc, MB ChB, MRCOG, MD; John M Kirwan, MB ChB, MRCOG
Patricia Howlin, BA MSc PhD FBSP, Professor of Clinical Child Psychology

INTRODUCTION

The lifetime prevalence of ovarian cancer in the developed world is 12%. It is often
described as a silent killer; however, the majority of women frequently experience symp-
toms in the months leading to diagnosis. The majority of ovarian cancers are diagnosed
at an advanced stage. In England and Wales, ovarian cancer kills more women than all
of the other gynaecological malignancies combined.
Pregnancy, breastfeeding, and use of the oral contraceptive pill all appear to pro-
tect against the development of epithelial ovarian cancer; the lower incidence seen in
less developed countries may be related to a higher birth rate.
Patients with ovarian cancer are best managed by multidisciplinary teams. These
usually include nurse specialists, medical oncologists, histopathologists, radiologists,
palliative care specialists, and gynaecological oncologists, in collaboration with the
patients and their families.

TYPES OF OVARIAN CANCER

Primary ovarian tumour types include epithelial, sex cordstromal and germ cell tu-
mours. Tumours not specific to the ovaries also occur, such as sarcomas and lymphomas.
Metastatic tumours from breast, stomach, and endometrial primaries are not uncommon.

Epithelial Tumours
More than 80% of ovarian cancer is epithelial in origin. The most common subtype
is serous, accounting for about 50%, followed by endometrioid, mucinous, clear cell,
transitional (Brenner), mixed, and undifferentiated tumours. These have previously been

JPOG JUL/AUG 2013 155

JPOG_JulAug_2013_COMBINE_Final.indd 155 8/5/13 2:18 PM


GYNAECOLOGY I Peer Reviewed

The histological subtypes of epithelial ovarian cancer are Primary peritoneal cancer is histologically
distinct entities, requiring different treatment strategies.
indistinguishable from metastatic serous ovarian
cancer. It is diagnosed in the absence of any clear
ovarian primary. Treatment is the same as for ovar-
ian cancer, although, as there is often no mass to
debulk, chemotherapy is more often used as the
primary treatment.
Borderline ovarian tumours are not truly can-
cers but are termed borderline because they show
histological features that are intermediate between
benign and malignant tumours. They are staged in
exactly the same way and sometimes spread be-
yond the ovary to produce non-invasive implants in
the omentum and the peritoneum. They can recur
after long periods; cases have been documented
with disease returning over 30 years after the initial
presentation. They are typically found in a younger
population compared with epithelial cancers, one-
third occur in women under the age of 40. The main
treatment is surgical excision, and opinions differ
in the extent of surgery required. It is probable that
they represent premalignant disease for low-grade
ovarian carcinomas.

Sex CordStromal Tumours


Sex cordstromal tumours account for approximate-
ly 7% of all malignant ovarian tumours. They arise
from a combination of the hormone-producing cells
treated as essentially the same disease with dif- of the ovary and stromal fibroblasts. Seventy per-
fering histology; however, it is becoming increas- cent of all malignant sex cordstromal tumours are
ingly evident that they behave as distinct entities. granulosa cell tumours. The majority occur in wom-
For example, endometrioid and clear cell cancers en in their sixth decade, although a small proportion
are strongly linked with endometriosis, whilst many arises in young women and prepubertal girls. Gran-
mucinous cancers originate in the appendix and evi- ulosa cell tumours may secrete sex hormones; most
dence increasingly points to serous tumours arising secrete oestrogen (although androgen-secreting va-
from dysplastic endometrium in the distal fallopian rieties do occur) potentially leading to endometrial
tube. Response to chemotherapy varies; serous tu- hyperplasia and carcinoma. Presenting symptoms
mours tend to be highly chemosensitive, but clear include abdominal distension, acute abdominal
cell and mucinous tumours are more resistant to pain, and abnormal vaginal bleeding. As most pre-
conventional chemotherapy. sent at an early stage, the prognosis is good. Treat-

JPOG JUL/AUG 2013 156

JPOG_JulAug_2013_COMBINE_Final.indd 156 8/5/13 2:18 PM


GYNAECOLOGY I GYNAECOLOGY
Peer Reviewed

ment is principally surgical with platinum-based Table 1. Approximate lifetime percentage risk of developing
ovarian cancer
chemotherapy for advanced disease. Surgical treat-
ment is as for epithelial ovarian cancer, although in
young women with early disease, fertility preserva-
tion is an option. Other stromal tumours are rare General population 1.6%
and include thecomas, fibromas, Sertoli-Leydig cell One first-degree relative affected under 55 years 5.2%
One first-degree relative affected over 55 years 3.4%
tumours, and gynandroblastomas.
Two first-degree relatives affected 7%
BRCA1 carrier 2844%
Malignant Germ Cell Tumours BRCA2 carrier 27%
Malignant germ cell tumours occur chiefly in girls HNPCC carrier 12%
and young women. The most common variety is the
HNPCC = hereditary non-polyposis colorectal cancer.
dysgerminoma, the counterpart to the seminoma in
the male. Other types include the yolk sack tumour,
embryonal carcinoma, polyembryoma, non-gesta- may indicate the presence of one of several pos-
tional choriocarcinoma, and teratoma. They usually sible BRCA1 or BRCA2 gene mutations. The gene
present with abdominal pain, which is sometimes products are involved in DNA repair. These gene
acute, and a palpable pelvic mass. Treatment for mutations are particularly common amongst the
early-stage disease is surgical. As over 60% are Ashkenazi Jewish population. Men carrying these
confined to one ovary at diagnosis, fertility-sparing genes are at increased risk of pancreatic cancer
surgery is usual with unilateral salpingo-oophorec- as well as male breast cancer. Endometrial, colon,
tomy or even ovarian cystectomy in selected cases ovarian, and other cancers cluster in families with
with otherwise normal ovaries. Dysgerminomas are the Lynch II or hereditary non-polyposis colorectal
highly radiosensitive, but platinum-based chemo- cancer (HNPCC) syndrome. This is caused by a vari-
therapy is currently the preferred option as radio- ety of defects in the DNA mismatch repair system.
therapy usually results in premature ovarian failure. BRCA1/2 and HNPCC are inherited in an autosomal
Non-dysgerminoma tumours are treated with the dominant fashion; further mutation or epigenetic
chemotherapy combination of bleomycin, etopo- silencing of the second allele results in malignancy.
side, and cisplatin. Response rates are excellent Prophylactic risk-reducing surgery is recom-
with cure rates approaching 100% in early-stage mended for BRCA1 and BRCA2 carriers when they
disease and up to 75% in advanced disease. reach the age of 35 or have completed their fami-
lies. This usually consists of a laparoscopic bilateral
FAMILIAL OVARIAN CANCER salpingo-oophorectomy. The risk of ovarian cancer
under 35 years in BRCA patients is low, and remov-
Approximately 510% of all ovarian cancer is as- al of the ovaries has the dual advantage of reducing
sociated with a genetic predisposition. Individuals the risks of both ovarian and breast cancer. HNPCC
carrying these gene defects have a significantly carriers are offered hysterectomy and bilateral sal-
higher risk of developing ovarian cancer than the pingo-oophorectomy when their family is complete.
general population (Table 1). Until patients are ready to undergo surgery, annual
A strong family history of breast and/or ovar- screening is commonly offered with cancer antigen
ian cancer, especially at a relatively young age, 125 (CA 125) and transvaginal ultrasound; however,

JPOG JUL/AUG 2013 157

JPOG_JulAug_2013_COMBINE_Final.indd 157 8/5/13 2:18 PM


GYNAECOLOGY I Peer Reviewed

Screening the anxious low-risk patient for ovarian cancer may not be beneficial because of the risk of false-
positive result.

there is no evidence that this is effective. Even if when the disease has already spread beyond the
the ovaries are removed, there is a small continuing ovary. Other tumour subtypes, especially mucinous,
risk of developing primary peritoneal cancer. often produce a more modest elevation.
The lack of one of these specific conditions Other markers are also used. Germ cell ovarian
does not exclude an increased cancer risk, as not all cancers often secrete highly specific tumour mark-
inherited ovarian cancer syndromes have had their ers which are useful in diagnosis and monitoring,
genetic origin fully characterized. eg, -fetoprotein, -hCG, and lactate dehydroge-
nase. These should be tested, in addition to CA
BIOMARKERS AND SCREENING 125, in women under the age of 40 years with a
suspicious pelvic mass. In addition, inhibin may be
The most commonly used marker for ovarian cancer of use as a marker for mucinous and granulosa cell
is CA 125. This is a glycoprotein that is released tumours.
into the bloodstream by any condition that disturbs Novel markers are currently under development
the peritoneum, including any peritoneal cancer, both as panels and individually. Human epididymis
cirrhosis, congestive cardiac failure, endometrio- protein 4 has been suggested as a biomarker with
sis, and pelvic inflammatory disease. Pregnancy equal or greater sensitivity and specificity than CA
also causes a variable increase in serum levels. It 125, however clinical trials are awaited.
is therefore notoriously non-specific at low levels. The PLCO (prostate, lung, colon, ovary) trial,
However, serous ovarian cancer can cause dramatic a huge US-based randomized controlled trial of
increases in CA 125. This is only likely to occur cancer screening, has recently reported. It con-

JPOG JUL/AUG 2013 158

JPOG_JulAug_2013_COMBINE_Final.indd 158 8/5/13 2:18 PM


GYNAECOLOGY I GYNAECOLOGY
Peer Reviewed

cluded that screening the general population with Table 2. FIGO staging of ovarian cancer
annual CA 125 and transvaginal ultrasound does
not reduce ovarian cancer mortality. Indeed, a sig- Stage Description
nificant number of women had major complications
I Confined to ovaries
from surgery performed because of a false-positive
Ia One ovary, no ascites present containing malignant
screening test.
cells, no tumour on external surface, capsule intact
There are also two large UK-based multicentre
Ib Both ovaries, no ascites present containing malignant
trials investigating ovarian cancer screening. The cells, no tumour on external surfaces, capsule intact
first is in postmenopausal women without a signifi- Ic Tumour limited to one or both ovaries with any of
cant family history of ovarian cancer and also uses a the following: tumour on the surface on one or both
combination of transvaginal ultrasound and CA 125 ovaries, capsule ruptured, ascites present with malig-
(UK Collaborative Trial of Ovarian Cancer Screen- nant cells or positive peritoneal washings
ing [UKCTOCS]). The second is in women with a II Growth involving one or both ovaries with pelvic exten-
sion
significant family history and is testing a panel of
IIa Extension and/or metastases to uterus and/or fallopian
biomarkers in addition to CA 125 and transvaginal
tubes
ultrasound (UK Familial Ovarian Cancer Screening
IIb Extension to other pelvic tissues
Study). Both have now finished recruitment and are
IIc Tumour stage IIa or IIb but with tumour on surface of
due to report in the next few years.
one or both ovaries, capsule ruptured, ascites pres-
Screening with a combination of CA 125, ul- ent containing malignant cells or positive peritoneal
trasound, and pelvic examination is commonly per- washings
formed for anxious patients who desire screening III Tumour involving one or both ovaries with microscopi-
for ovarian cancer. If the findings of the PLCO trial cally confirmed peritoneal implants outside the pelvis
are duplicated in the UKCTOCS trial, this practice is and/or regional lymph node metastasis

likely to become hard to defend in low-risk women. IIIa Microscopic peritoneal metastasis beyond the pelvis
IIIb Macroscopic peritoneal metastasis beyond the pelvis, 2
cm or less in greatest dimension
INVESTIGATION
IIIc Abdominal implants greater than 2 cm in diameter and/
or regional lymph nodes metastasis
The first symptoms of ovarian cancer usually
IV Distant metastasis beyond the peritoneal cavity.
emerge some time before diagnosis. These com-
Includes liver parenchymal metastasis and/or pleural
monly include early satiety, changes in bowel effusion with positive cytology
habit, bloating, urinary frequency, and pelvic and
FIGO = International Federation of Gynecology and Obstetrics.
abdominal pain. Patients with advanced cancer of-
ten complain of abdominal swelling and discomfort
due to ascites with or without a large abdomin- therefore difficult to detect.
opelvic mass. Eating is often difficult, and patients Recent National Institute for Clinical Excel-
may notice weight loss, apart from the distended lence (NICE) guideline recommend that women,
abdomen. It is not uncommon for patients to pre- especially those over 50 years old, who experience
sent with a swollen leg secondary to a deep vein symptoms persistent or frequent symptoms as de-
thrombosis. However, most small ovarian cancers scribed above, or new-onset symptoms suggestive
are asymptomatic when confined to the ovaries and of irritable bowel syndrome, should have CA 125

JPOG JUL/AUG 2013 159

JPOG_JulAug_2013_COMBINE_Final.indd 159 8/5/13 2:18 PM


GYNAECOLOGY I Peer Reviewed

Advanced epithelial ovarian cancer is difficult to cure. clinically indicated) should be performed, prior to
surgery, to assess the extent of the disease.

STAGING

Staging is performed to guide treatment and to


provide information on prognosis. Traditionally, this
has been achieved by performing a staging lapa-
rotomy. Information can also be gleaned from ra-
diological investigations, guided biopsy, and cytol-
ogy of ascitic or pleural fluid. The current staging
system was devised by International Federation of
Gynecology and Obstetrics (FIGO) (see Table 2).

TREATMENT

Treatment in ovarian cancer depends upon the stage


at presentation and the histological subtype. Non-
epithelial cancers are discussed earlier. In general,
epithelial ovarian cancers are treated with a combi-
nation of surgery and chemotherapy. Except in very
measured. If the level is > 35, an ultrasound of the early disease, treatment is rarely curative but it can
abdomen and pelvis should be performed. provide symptom relief and prolong life.
The ultrasound and CA 125 together are used Traditionally, ovarian cancer is treated with a
to calculate the risk of malignancy index (RMI): RMI staging and debulking laparotomy followed by six
= U M CA 125, where U = ultrasound score (1 cycles of chemotherapy. Second-look laparotomy,
point for each of multilocular cysts, solid areas, where a second surgical debulking procedure is
ascites, bilateral lesions, metastases. U = 0 for 0 performed after completion of chemotherapy, is not
points, U = 1 for 1 point, U = 3 for 25 points); M = beneficial. Data from the EORTC 55971 trial, which
menopausal status (premenopausal = 1, postmeno- compared interval debulking surgery performed
pausal = 3); CA 125 = serum CA 125 level. midway between six cycles of chemotherapy with
A score of 250 has been chosen by NICE to the traditional laparotomy and six cycles of post-
guide triage to either surgery in a cancer centre un- operative chemotherapy, suggested that neoadju-
der the care of a specialist multidisciplinary team, vant chemotherapy in bulky stage IIIc/IV disease
including subspecialist gynaecological oncologists did not adversely affect prognosis and that interval
( 250), or to care under a general gynaecologist debulking is associated with a lower post-operative
with an interest in gynaecological oncology in a morbidity and mortality. The CHORUS (CHemother-
cancer unit (< 250). apy OR Upfront Surgery) trial also addresses this
If ovarian cancer is suspected, a computed to- question and its publication is awaited.
mography of the abdomen and pelvis (and thorax if In light of this, interval debulking surgery, per-

JPOG JUL/AUG 2013 160

JPOG_JulAug_2013_COMBINE_Final.indd 160 8/5/13 2:18 PM


GYNAECOLOGY I GYNAECOLOGY
Peer Reviewed
The decision to adopt palliation in patients with ovarian cancer can
be challenging.

formed midway through chemotherapy, is gaining The decision to adopt palliation in patients with ovarian cancer
can be challenging.
popularity.

SURGERY

Surgical treatment, whether primary or as an inter-


val debulking procedure, involves a midline laparot-
omy, sampling of ascitic fluid or peritoneal wash-
ings for cytology, full assessment by palpation of
all peritoneal surfaces and biopsy of any suspicious
areas, removal intact of any encapsulated masses
or debulking of tumour, sampling of suspicious
pelvic and para-aortic lymph nodes, omentectomy,
total abdominal hysterectomy, and bilateral salpin-
go-oophorectomy. The aim is to completely remove
all visible disease. This is thought to promote an
optimum response to chemotherapy. Surgery also
provides ample material for diagnostic histological
assessment. The evidence for debulking improving
chemosensitivity is not absolute. Many argue that
the ability to perform optimal cytoreduction is a
more a reflection of favourable tumour biology with Recent NICE guidance recommends that lym-
an intrinsically better prognosis than the surgery phadenectomy is restricted to removal of clinically
itself influencing outcome. However, it has been involved nodes. Full pelvic and para-aortic node dis-
demonstrated that survival in stage III disease is section in suspected stage I disease is not recom-
improved by the primary surgery being performed by mended.
a specialist gynaecological oncologist, rather than Less aggressive surgery is preferred in early
an obstetrician/gynaecologist or a general surgeon. epithelial ovarian cancer in young women who wish
Surgical aggressiveness varies considerably to preserve their fertility. About 8% of stage I epi-
between continents, countries, and individual units. thelial ovarian cancers occur in women under the
Some studies have shown improvements in survival age of 35. A proportion of these will not have com-
with radical surgery, including liver resection, bowel pleted childbearing and may wish to consider fertil-
resection, and splenectomy. Published case series ity-sparing surgery. Suitable patients include those
show small or no increases in mortality and morbid- with stage IA, grade 1 or possibly grade 2 disease.
ity. However, these studies usually involve highly se- Such conservative surgery would typically consist
lected cases from single institutions and may not be of peritoneal fluid cytology, unilateral salpingo-
directly applicable to wider practice. Some disease oophorectomy, omental biopsy, and careful inspec-
remains unresectable to even the most adventurous tion of the contralateral ovary and nodal chain. One
surgeon; this includes small bowel mesenteric dis- case series describes 282 women treated conserva-
ease and disease of the portal triad. tively for epithelial ovarian cancer. Just over 30%

JPOG JUL/AUG 2013 161

JPOG_JulAug_2013_COMBINE_Final.indd 161 8/5/13 2:18 PM


GYNAECOLOGY I Peer Reviewed

Practice points The current standard first-line chemotherapy


regimen for ovarian cancer involves intravenous ad-
ministration of a platinum-based drug with a taxa-
Ovarian cancer is best managed within a cancer centre by a ne, usually paclitaxel, given 3 weekly for six cycles.
multidisciplinary team
Most units prefer carboplatin to cisplatin as it has
Women with symptoms suggestive of ovarian cancer should
have a CA 125 performed as an initial screen, followed by a less toxic side effect profile. Evidence for the use
ultrasound if abnormal of paclitaxel is drawn from its efficacy in relapsed
Ovarian cancer screening with ultrasound and CA 125 does not ovarian cancer. However, paclitaxel significantly in-
appear to be helpful in the low-risk population
Treatment involves a combination of surgery and platinum- creases the risk of neuropathy when compared with
based chemotherapy carboplatin alone, and some patients have anaphy-
Fertility-sparing surgery is possible in women with early-stage lactic reactions to taxanes. For these reasons, it
disease
is not universally used. Chemotherapy can also be
given via the intraperitoneal route; however, cur-
rently in the UK its use is not recommended outside
subsequently went on to have term deliveries. Four of clinical trials.
percent died of conditions related to their disease. Epithelial ovarian cancer is one of the more
Surgery also plays a role in palliative care of chemosensitive solid tumours, and complete clini-
patients with ovarian cancer. Bowel obstruction is cal and radiological response occurs in up to 50%
common in the end stages of the disease; post-mor- with the above regimen. Conversely, 2030% will
tem studies of women dying with ovarian cancer show no evidence of response. Unfortunately, the
revealed bowel obstruction in almost 50%. Before majority of patients with advanced ovarian cancer
contemplating such surgery, consideration must be will relapse. Chemotherapy for recurrent disease is
given as to whether it is appropriate. Surgery should determined in part by the length of time before re-
only be performed if it has a reasonable chance of lapse occurs. If it is more than 6 months afterwards,
success, and risks need to be carefully balanced it is potentially platinum-sensitive and, unless con-
against potential symptom relief. Contraindications traindicated, a regimen containing platinum will be
to surgery include patient refusal, rapidly accumu- used again. A taxane is likely to be included, espe-
lating acsites, high obstruction, multiple levels of cially if not used initially. Paclitaxel is sometimes
obstruction, and poor nutritional status. Decisions used at a lower dose at more frequent intervals;
involving palliative surgery should involve the mul- this appears to reduce the adverse effects experi-
tidisciplinary team and careful discussion with the enced. Response rates are in the order of 30%. If
patient and her relatives. relapse occurs within 6 months, second-line drugs,
such as liposomal doxorubicin and topotecan, may
CHEMOTHERAPY be considered. As response rates are low, approxi-
mately 1020%, the choice of drug is made bearing
Patients should have histological confirmation of in mind side effect profiles and ease of administra-
their ovarian cancer prior to starting chemotherapy. tion.
This may be obtained through image-guided percu- Novel chemotherapeutic agents are constantly
taneous biopsy, or where this is not possible or the being developed, some of the most promising effect
results are inadequate, by laparoscopic biopsy. the functioning of vascular endothelial growth fac-

JPOG JUL/AUG 2013 162

JPOG_JulAug_2013_COMBINE_Final.indd 162 8/5/13 2:18 PM


GYNAECOLOGY I GYNAECOLOGY
Peer Reviewed

tor. Interim analysis of the ICON 7 trial of standard delivered by syringe driver are useful as they permit
therapy with or without bevacizumab (a monoclo- a steady concentration of drug and oral medications
nal antibody to vascular endothelial growth factor) may be poorly absorbed. Several hospices publish
shows a sustained improvement in progression-free their guidelines for managing symptoms in pallia-
survival in a subgroup of women with advanced dis- tive care on the internet, which can be very helpful.
ease and suboptimal surgical debulking. Finally, the physical needs of a seriously ill pa-
Hormonal therapies are occasionally used; tient are only one facet of their care. Social, spiritu-
these probably act by reducing oestrogen activity al, and emotional needs also need to be addressed,
and include tamoxifen, aromatase inhibitors, and both for the patient and their relatives. Specialist
gonadotropin-releasing hormone analogues. Re- oncology nurses and palliative care input is essen-
sponse rates of 1015% have been achieved in re- tial, as are discussions on resuscitation status and
lapsed disease. Their main advantage is their mini- preferred place of death. These aspects of care are
mal side effects when compared with conventional easily overlooked but can make the difference be-
chemotherapy. tween a peaceful and a difficult death.

PALLIATIVE CARE FURTHER READING

Agarwal R, Linch M, Kaye SB. Novel therapeutic agents in ovarian


Palliation is an integral part of the care of pa- cancer. EJSO 2006;32:875886.
tients with ovarian cancer. Maintaining a balance Aletti GD, Gallenberg MM, Cliby WA, Jatoi AJ, Hartmann LC.
Current management strategies for ovarian cancer. Mayo Clin Proc
between optimism and pragmatism, together with
2007;82:751770.
knowing when the emphasis of care should tilt to- Blagden S, Gabra H. Future directions in the management of epithelial
wards palliation, are some of the most challenging ovarian cancer. Future Oncol 2008;4:403411.
Cannistra SA. Cancer of the ovary. N Engl J Med 2004;351:25192529.
aspects of caring for this group of patients. Colombo N, Parma G, Zanagnolo V, Insinga A. Management of ovarian
Almost all patients with advanced ovarian stromal cell tumours. J Clin Oncol 2007;25:29442951.
Gershenson DM. Management of ovarian germ cell tumours. J Clin
cancer, whether in the terminal phase or not, will
Oncol 2007;25:29382943.
have distressing symptoms that require treatment. Goff BA, Mandel L, Muntz HG, Melancon CH. Ovarian carcinoma
These symptoms may be due to the disease itself diagnosis, results of a national ovarian cancer survey. Cancer
2000;89:20682075.
or secondary to their treatment. Common symptoms Markman M. New, expanded, and modified use of approved antineo-
include nausea, pain, loss of appetite, constipation, plastic agents in ovarian cancer. The Oncologist 2007;12:186190.
NICE clinical guideline 122, Ovarian Cancer.
and abdominal distension. This is too small a space
Nickles Fader A, Rose PG. Role of surgery in ovarian carcinoma. J Clin
to describe all the possible treatment strategies Oncol 2007;25:28732883.
involved, but it is worth emphasizing a few princi- Rao G, Crispens M, Rothenberg ML. Intraperitoneal chemotherapy
for ovarian cancer: overview and perspective. J Clin Oncol
ples. It is important to take a history, examine the 2007;25:28672872.
patient, and review the drug chart and case notes.
2011 Elsevier Ltd. Initially published in Obstetrics, Gynaecology
Then consider what investigations may be helpful
and Reproductive Medicine 2011;22(2):3437.
and what drug or intervention will best treat the
most likely cause of her symptoms. These patients About the Authors
are usually complex with multiple problems; how- Sin E Taylor is Subspecialty Trainee in the Department of Gynae-
cologial Oncology at Liverpool Womens Hospital, Liverpool, UK.
ever, a logical and systematic approach will help to John M Kirwan is Consultant Gynaecological Oncologist at Liverpool
identify the best treatment. Subcutaneous infusions Womens Hospital, Liverpool, UK.

JPOG JUL/AUG 2013 163

JPOG_JulAug_2013_COMBINE_Final.indd 163 8/5/13 2:18 PM


PAEDIATRICS
PAEDIATRICS II Peer
Peer Reviewed
Reviewed

Constipation in
Imaging Paediatric
Infants and Children
Brain Tumours
Taya Dowling, BMedSci, MB BS; Scott Nightingale, BMed(Hons), MClinEpid, FRACP
Tang Phua Hwee, MBBS, FRCR, MMed Diagnostic Radiology

REMEMBER

Constipation is a common paediatric presentation.


Constipation is defined based on the frequency of stooling (which varies widely
depending on the age of the child), and more importantly the consistency, size and
difficulty with which stools are passed (see the box on page 165).1
C onstipation arising beyond the neonatal period is most often functional (ie, does not
result from any identifiable organic pathology), and may be perpetuated by voluntary
withholding of stool to avoid painful defaecation.
There are several red flags that should prompt further investigation for a contrib-
uting medical or surgical condition, but these are uncommon (see the box on page
165).2,3
Faecal incontinence is thought to be related to overdistension of the rectum with
stool, shortening of the anal canal and resulting impairment of normal continence
mechanisms. Incontinence is not deliberate or caused by laziness on the part of the
child.
Untreated constipation and faecal incontinence (encopresis) can have a significant
psychosocial impact on a child.
Management of constipation is often a long-term process that requires the com-
plementary approaches of careful education of the child and parents, behavioural
4
techniques, laxative agents and review.

ASSESSMENT

T he focus should be on identifying the rare child with an organic cause for consti-

JPOG JUL/AUG 2013 164

JPOG_JulAug_2013_COMBINE_Final.indd 164 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed
PAEDIATRICS

Rome III diagnostic criteria for functional constipation in tinence, withholding behaviour and any symp-
children1
toms associated with defaecation, such as pain,
bleeding and straining.
Important aspects of the history include age at
At least two of the following features have been present for at least 2 onset, growth trends, diet history and the pres-
months in a child aged 4 years or older (developmental age):
ence of red flags (see the box on this page).
two or fewer defaecations in the toilet per week
at least one episode of faecal incontinence per week A thorough physical examination should be per-
history of retentive posturing or excessive volitional stool retention formed, particularly focusing on growth param-
history of painful or hard bowel movements eters, palpable abdominal faecal masses, in-
presence of a large faecal mass in the rectum spection of the perianal and lumbosacral regions
history of large diameter stools that obstruct the toilet and lower limb neurological examination. Poor
growth may occur with Hirschsprung disease,
hypothyroidism and coeliac disease.
D igital rectal examination should be avoided in
Red flag feature2,3 primary health care as it rarely contributes to the
clinical assessment and can be particularly dis-
tressing for the child.
History
Impaction is suggested by faecal incontinence or
Constipation from the neonatal period
Failure to pass meconium by the age of 48 hours a palpable faecal mass (preferably determined
Ribbon stools suggesting anorectal stricture or stenosis via abdominal palpation).
Abdominal distension and vomiting If the likely diagnosis is functional constipation
Poor weight gain or weight loss then no further investigation is needed. Abdomi-
Leg weakness or delayed gross motor development nal X-r ays are not needed to diagnose constipa-
tion or to determine response to therapy.5
Examination
If a pathological cause for constipation is sus-
Gross abdominal distension
pected then appropriate investigations should be
Abnormal appearance, position or patency of anus fistulae, bruis-
performed in consultation with a paediatrician or
ing, multiple fissures or fissures away from the midline, tight or
patulous anus, anteriorly placed anus, absent anal wink paediatric surgeon.
Lumbosacral abnormalities evidence of sacral agenesis, discol-
oured skin, naevi, sinus, hairy patch, lipoma, central pit, scoliosis MANAGEMENT
Gluteal asymmetry or wasting
Absent cremasteric reflex A combination of management approaches that
Abnormal results on lower limb neurological examination de- complement each other are almost always re-
formity such as talipes, abnormal reflexes quired in the management of childhood consti-
pation. Individual elements in isolation (eg, di-
simpaction without maintenance laxatives) are
pation, and determining whether the child has unlikely to be unsuccessful.
faecal impaction. E ducation of parents and caregivers about the
T he history should include a detailed descrip- relationship between behavioural aspects (eg,
tion of the childs stool, stool frequency, incon- fear of pain and withholding) and functional

JPOG JUL/AUG 2013 165

JPOG_JulAug_2013_COMBINE_Final.indd 165 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed

Parents and caregivers are to be educated to encourage the child with faecal incontinence develop a healthy
stooling habit.

constipation is vital. They should be informed after disimpaction and often needs to continue
that this is usually a chronic problem, requiring for many months after normalization of stool-
long-term management. The rationale behind the ing. A plan should be made to restart laxatives
various aspects of management should be made promptly on signs of relapse.
clear, and education should be reviewed on sub- T he objectives of maintenance therapy are that:
sequent visits.  the child passes regular soft stools (eg, 1 to

Faecal disimpaction, if necessary, should be 2 per day) without discomfort or excessive ef-
achieved using laxatives (see the Table). Except fort
in infants, oral therapy should generally be tried 
t he rectum remains empty to prevent re
first. In children, rectal therapy should usually impaction.
be reserved for those with more severe or un- T here are many options available for mainte-
responsive constipation, leading to persistent nance laxative therapy (see the Table), with
rectal discomfort or unproductive straining. Oral choice influenced by the age of the child, pre-
macrogol 3350 (polyethylene glycol) was found vious experience, ease of administration and
to be equally effective to enema therapy for dis- palatability to the child:
6
impaction in a randomized study of 90 children. L iquid paraffin should be avoided in infants
If multiple enemas or nasogastric lavage are re- and those at risk of aspiration because of the
quired for disimpaction then a paediatrician or risk of lipoid pneumonia, and should not be
paediatric surgeon should be involved. given within 2 hours of sleep.
Maintenance laxative therapy should be started In children, the osmotic laxative macrogol


JPOG JUL/AUG 2013 166

JPOG_JulAug_2013_COMBINE_Final.indd 166 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed
PAEDIATRICS

Table. Initial laxative options for infants and children

Infants (< 12 months) Children


Disimpaction Glycerol suppository 700 mg M
 acrogol 3350 1 to 1.5 g/kg/day (max 52 g for
children aged 2 to 5 years and 78 g for children
aged 6 years and over) until disimpacted4*

Maintenance S orbitol-containing fruit juices, such as prune, M acrogol 3350 0.4 to 0.8 g/kg daily up to 17 g* or
pear, apple (50 to 100 mL/day) or P araffin oil 1 to 3 mL/kg daily (start 10 to 20 mL
Lactulose 5 mL daily daily) or
Lactulose 10 to 15 mL daily
* Macrogol 3350 (polyethylene glycol) is available in Australia in a variety of formulations, in scoop packs or sachets.

Doses are a guide only and should be titrated to effect every 2 to 3 days as required, with consideration of the maximum recommended dose.

Not recommended in infants, or in children with gastro-oesophageal reflux or risk of aspiration.

was found to result in more stools per week vantage of the gastrocolic reflex. Positive rein-
and less need for additional therapy when forcement for sitting (eg, using reward charts)
compared with lactulose in a meta- analysis of should be encouraged. Children should never be
7
four studies. Macrogol is not currently recom punished for being constipated or incontinent.
mended for children younger than 2 years in  A healthy diet should be encouraged rather than
Australia because of lack of safety data; how- a high-f ibre diet, as there is little evidence that
ever, these data are accumulating. increasing dietary fibre is an effective treat-
Stimulant laxatives such as bisacodyl, sen- ment for childhood constipation. 2 There is lim-
nosides and sodium picosulfate are effective ited evidence that avoiding cow milk may result
adjuncts to osmotic laxatives when necessary. in improvement in some children with chronic
There are few data to support widely held con- constipation, particularly those with atopic ten-
cerns regarding long-term use of laxatives, in dencies.9,10 Any trial of such an elimination diet
particular stimulant laxatives. Clinical studies should be limited to 2 to 4 weeks, and the child
show that long-term use of osmotic laxatives is should be re-c hallenged to confirm any effect.
7
safe and well tolerated. Caregivers should be Prolonged elimination diets require supervision
educated about this safety and the need for long by a qualified dietitian to prevent any nutritional
term maintenance therapy. deficiencies and there should be repeated at-
B ehavioural measures combined with laxative tempts to normalize the diet.
therapy are superior to either therapy alone in A side from situations of clinical dehydration,
8
children with faecal incontinence. It is impor- there is no evidence that increasing water intake
tant that behavioural measures are applied con- is beneficial in constipation. However, ensuring
sistently with the aim of developing a healthy adequate fluid intake is important when using
stooling habit. The child should be encouraged to osmotic laxatives to avoid dehydration.
sit on the toilet for 5 minutes, two or three times R egular review is required to monitor response
a day, within 30 minutes after meals to take ad- to therapy, adjust laxative dose, reinforce educa-

JPOG JUL/AUG 2013 167

JPOG_JulAug_2013_COMBINE_Final.indd 167 8/5/13 2:18 PM


PAEDIATRICS I Peer Reviewed

tion and support the family through what is often M anagement of constipation is often a long-
a long and frustrating period. term process that requires the complementary
Lack of response to management should prompt approaches of careful education of the child and
review of all aspects of the management plan. parents, behavioural techniques, laxative agents
Persisting failure to respond may prompt recon- and review.
sideration of pathological causes and investiga-
2013 Medicine Today Pty Ltd. Initially published in Medicine Today
tion for these. July 2013;14(7):7173. Reprinted with permission.

CONCLUSION About the Authors


Dr Dowling is a Paediatric Registrar, Hunter New England Local Health
District, Newcastle. Dr Nightingale is a Paediatric Gastroenterolo-
Constipation is a common paediatric presenta-
gist, John Hunter Childrens Hospital; and Conjoint Lecturer, School
tion and is usually functional. of Medicine and Public Health, University of Newcastle, Newcastle,
Further investigation or referral is guided by the NSW, Australia.
presence of red flags. Series Editor: Associate Professor Simone Strasser, MD, FRACP, Clini-
cal Associate Professor, Central Clinical School (Medicine), University
Untreated constipation and faecal incontinence
of Sydney; and Senior Staff Specialist, AW Morrow Gastroenterol-
(encopresis) can have a significant psychosocial ogy and Liver Centre, Royal Prince Alfred Hospital, Sydney, NSW,
impact on a child. Australia.

REFERENCES
1. Drossman DA. Rome III: the functional gastro- dations of the North American Society for Pediatric lonic transit time, and rectal ultrasound scanning in 8. Brazzelli M, Griffiths PV, Cody JD, Tappin D. Be-
intestinal disorders. 3rd ed. McLean, VA: Degnon Gastroenterology, Hepatology and Nutrition. J Pedi- the diagnosis of idiopathic constipation in children: havioural and cognitive interventions with or with-
Associates; 2006. atr Gastroenterol Nutr 2006;43:e1-e13. a systematic review. J Pediatr 2012;161:44-50, out other treatments for the management of faecal
2. National Institute for Health and Care Excellence 4. North American Society for Pediatric Gastroen- e41-42. incontinence in children. Cochrane Database Syst
(NICE). Constipation in children and young people: terology, Hepatology and Nutrition. Evaluation and 6. Bekkali NL, van den Berg MM , Dijkgraaf MG, et Rev 2011;12:CD002240.
diagnosis and management of idiopathic childhood treatment of constipation in children: summary of al. Rectal fecal impaction treatment in childhood 9. Iacono G, Cavataio F, Montalto G, et al. Intoler-
constipation in primary and secondary care: quick updated recommendations of the North American constipation: enemas versus high doses oral PEG. ance of cows milk and chronic constipation in chil-
reference guide. London: NICE; 2010. Society for Pediatric Gastroenterology, Hepatol- Pediatrics 2009;124:e1108-E1115. dren. N Engl J Med 1998;339:1100-1104.
3. Constipation Guideline Committee of the North ogy and Nutrition. J Pediatr Gastroenterol Nutr 7. Gordon M, Naidoo K, Akobeng AK, Thomas AG. 10. Irastorza I, Ibanez B, Delgado-Sanzonetti L,
American Society for Pediatric Gastroenterology, 2006;43:405-407. Osmotic and stimulant laxatives for the manage- Maruri N, Vitoria JC. Cows milkfree diet as a
Hepatology and Nutrition. Evaluation and treatment 5. Berger MY, Tabbers MM, Kurver MJ, Boluyt N, ment of childhood constipation. Cochrane Database therapeutic option in childhood chronic constipa-
of constipation in infants and children: recommen- Benninga MA. Value of abdominal radiography, co- Syst Rev 2012;7:CD009118. tion. J Pediatr Gastroenterol Nutr 2010;51:171-176.

JPOG JUL/AUG 2013 168

JPOG_JulAug_2013_COMBINE_Final.indd 168 8/5/13 2:18 PM


Essential
Reference
Medical
FOR

Professionals

ide ns
armacy Gu l ecialty Editio
nnua
MI MS Ph MIMS A MIMS Sp MIMS

The Complete Knowledge Solution

Join over a million MIMS members who have incorporated MIMS into
their daily workflow. Connect with MIMS today.

www.mims.com MIMS mobile/tablet app facebook.com/mimscom


Continuing Medical Education

P
5 SK

Management of Pregnancies With Previous


Caesarean Section
Yung WK, MBBS, MRCOG, FHKAM (O&G); Lau WL, MBBS, FRCOG, FHKAM (O&G); Leung WC, MBBS, MD, FRCOG, FHKAM (O&G), Cert RCOG (Maternal and Fetal Med)

INTRODUCTION

Over the past few decades, there has been


widespread concern about the increasing
proportion of births born by caesarean de-
livery. The rising rate of primary caesarean
section has led to the increased number of
obstetric population with a history of prior
caesarean delivery. Although this group
of women may be offered planned vagi-
nal birth after previous caesarean section
(VBAC) or elective repeat caesarean sec-
tion (ERCS), the VBAC rate is generally low
particularly in well-developed countries.
In the United States, the VBAC rate has
decreased to 8.5% by 2006, while the total
caesarean rate has increased to 31.1%.1
In this article, we review the recom-
mendations on VBAC by different profes-
One of the obstetricians roles is to identify women who are most likely to achieve
sional societies, the favourable and unfa- successful vaginal birth after previous caesarean section.
vourable factors in considering VBAC, the
associated maternal and fetal benefits
and risks, as well as the non-medical ERCS. International authorities have limited by several factors: (1) there is no
concerns that play a role in the decision- published their guidelines to provide randomized controlled trial on VBAC ver-
making process. evidence-based obstetric care. Examples sus ERCS; (2) adverse maternal and peri-
are guidelines from the American College natal outcomes rarely occur; (3) there was
WHO ARE SUITABLE TO of Obstetrics and Gynecology (ACOG) in selection bias in the published reports, as
ATTEMPT VBACWHAT 2010, the Royal College of Obstetrics and a womans decision to attempt VBAC re-
DOES THE EVIDENCE SAY? Gynecology (RCOG) in 2007, and the Society lies on the counselling by the health-care
of Obstetricians and Gynaecologists of provider and local resources.2
There have been numerous studies evalu- Canada (SOGC) in 2005. All three organi- The three societies share similar
ating the risks and benefits of VBAC and zations emphasized that their data were opinion on patient selection undergoing

JPOG JUL/AUG 2013 169

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 169 8/5/13 2:09 PM


planned VBAC. Previous uterine rupture RCOG and ACOG suggest that planned ery.2,6,7 Peaceman et al 8 evaluated the ef-
and high vertical classical caesarean sec- VBAC is contraindicated in women with fect of fetal size on VBAC. If prior caesar-
tion are the contraindications, as they are more than two previous caesarean sec- ean delivery was performed for dystocia
associated with a substantial risk of scar tions. 1,5
In clinical practice, most women defined as failed induction, cephalopelvic
rupture. For the other scar variants such undergoing planned VBAC have one prior disproportion or failure to progressor
as prior inverted T or J incision, lower ver- caesarean delivery. The quoted success failed instrumental delivery, the VBAC
tical incision, previous myomectomy and rates of planned VBAC by the ACOG, RCOG success rate was reduced to 54% com-
prior complex uterine surgery, the avail- and SOGC are 6080%, 7276%, and 50 pared with 67% for other indications. If
able data are, however, insufficient and 85%, respectively. fetal weight of the current pregnancy ex-
conflicting owing to the limited number ceeded the prior one by 500 g, the success
of studies. Planned VBAC in such circum- rate was only 38%. 8 Other retrospective
stances should be assessed by experi- analysis showed that infants with birth
enced obstetricians on an individual basis. weight of more than 4 kg were less likely
Maternal age alone
The risk of uterine rupture for women to be delivered vaginally. However, obste-
with one previous caesarean section of a should not be used to tricians should be aware that so far there
low transverse incision is low. The risk fig- discourage women is a lack of reliable methods in estimat-
ures by ACOG, RCOG and SOGC are < 1%, ing fetal weight with accuracy. Therefore,
from attempting a
0.20.7%, and 0.21.5%, respectively. clinical suspicion of fetal macrosomia
In some patients, the type of uterine vaginal delivery. alone should not be a contraindication to
incision of prior caesarean delivery can- offering planned VBAC.
not be confirmed. Although some have Womens age might be considered
questioned the safety of offering VBAC during the counselling on planned VBAC.
under these circumstances, two case se- One of the obstetricians roles is to Although there were studies showing that
ries reported a similar rate of successful identify women who are most likely to the success rate decreased with advanced
VBAC and uterine rupture to known low achieve successful VBAC. Prior vaginal age,9 maternal age alone should not be
transverse scar. 3,4 It is based on the fact birth, spontaneous onset of labour, prior used to discourage women from attempt-
that nowadays most caesarean incisions caesarean section for a non-recurring ing a vaginal delivery.
are low transverse. Therefore, the ACOG condition and preterm labour are the fa- Maternal obesity adversely influenc-
and SOGC guidelines suggest that VBAC vourable factors for successful VBAC. 1,5
es the success rate.10 The available evi-
is not contraindicated in women with one On the contrary, recurrent indication for dence consistently demonstrates a gradu-
previous caesarean delivery with unknown prior caesarean section (eg, labour dys- al reduction of vaginal delivery rate with
type of scar unless there is a high clinical tocia), increased maternal age, maternal increasing body mass index (BMI). Juhasz
suspicion of a previous classical uterine obesity, gestation greater than 40 weeks, et al11 evaluated 1,213 women in their
incision. The RCOG states that the opera- increased neonatal birth weight, and short study. The VBAC rate fell from 83.1% for
tive notes of previous operation should be pregnancy interval are the negative pre- BMI < 19.8, to 79.9% for BMI 19.826, fur-
reviewed where possible. dictor factors. 1
ther to 69.3% for BMI 26.129, and down
Although the risk of uterine rupture Macrosomia has been recognized to 68.2% for BMI > 29 (P < 0.001). Of inter-
increases with the number of previous as a significant factor leading to failed est, retrospective studies by Durnwald et
caesarean sections, the chance of achiev- VBAC, with a higher risk of uterine rupture al 12 found that women who gained weight
ing VBAC appears to be similar. Both the
1
in women with no previous vaginal deliv- between pregnancies had a lower VBAC

JPOG JUL/AUG 2013 170

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 170 8/5/13 2:09 PM


Continuing Medical Education

Table. Experience of Kwong Wah Hospital, Hong Kong, in the management of women with one prior caesarean
delivery

Year

2007 2008 2009 2010


Total no. of maternities 5,395 5,446 5,682 5,945
Women with prior caesarean 9.1 9.9 9.7 9.4
deliveries (%)
Successful planned VBAC rate (%) 94.3 88.5 86.3 87.2
Total VBAC rate (%) 21.1 21.9 22.9 26.9
Uterine rupture (%) 0 0.8 0 0
VBAC = vaginal birth after previous caesarean section.

rate. Women with normal BMI who turned should be conducted in a suitably staffed the fetal presenting part.5
overweight (BMI 2529.9) before their and equipped delivery suite, with avail- For women attempting VBAC, mater-
second pregnancy had a 56.6% success able resources for immediate laparotomy nal and fetal condition should be regularly
rate; which was in contrast to the 74.2% and neonatal resuscitation. assessed once labour begins. The likeli-
for women whose BMI remained normal (P Early diagnosis of uterine scar rup- hood of vaginal delivery may be modified
= 0.006). 12
ture followed by expeditious laparotomy by intrapartum conditions. Studies report-
Some authorities consider short and resuscitation is essential to reduce ed that women admitted with a more fa-
inter-delivery interval as a risk factor for adverse maternal and fetal outcome. vourable cervical status (eg, cervical dila-
failed VBAC and uterine rupture. As uter- Although there is no single pathognomon- tation > 4 cm, > 75% effacement) in spon-
ine rupture is an uncommon complication, ic clinical feature of the rupture, an abnor- taneous labour have a twofold increase in
evidence from RCOG based on three small mal tracing on cardiotocography is present success rate.1315
observational studies suggested a two- in 5587% of the cases. 5 The RCOG and Induction of labour for maternal or
to threefold increased scar rupture risk SOGC recommend continuous electronic fetal condition is increasingly common in
with inter-delivery interval below 1224 fetal monitoring for all women attempting obstetric practice. Labour induction and
months.5 VBAC. augmentation in women with previous
Other associated features of uterine caesarean delivery is associated with an
THE DELIVERY rupture include acute onset of scar ten- increased risk of uterine rupture. The risk
INTRAPARTUM derness, severe abdominal pain persisting is highest with misoprostol (6%), followed
MANAGEMENT between contractions, chest pain or shoul- by prostaglandin E2 (2%) and lowest with
der tip pain, sudden onset of shortness of oxytocin (1.1%). 16 All the three organiza-
Slight variations in opinion exist among breath, abnormal vaginal bleeding or hae- tions discouraged the use of misoprostol
authorities on the standard of an institu- maturia, cessation of previously efficient and prostaglandins in most women with
tion offering VBAC. Nevertheless, they uterine activity, maternal tachycardia, hy- previous caesarean delivery.
are all in the direction that planned VBAC potension or shock, and loss of station of Studies on mechanical cervical ripen-

JPOG JUL/AUG 2013 171

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 171 8/5/13 2:09 PM


es must be considered in all cases. For ex- increases the risk of long-term problems.
ample, if a patient, who may not otherwise Chronic pain is a common condition expe-
be a candidate for planned VBAC, presents rienced by some women after caesarean
in advanced labour, obstetricians may judge delivery. The risk appears to be higher
it best to proceed with vaginal delivery. with increasing number of operations
performed. A group in Denmark followed
MATERNAL BENEFIT AND up 244 women for chronic pain after cae-
RISK sarean section. Of those women, 18.6%
had pain after 3 months and 12.3% still
Both planned VBAC and ERCS carry mater- suffered from pain at 10 months after de-
nal and neonatal risks. The risks of either livery. Furthermore, 5.9% of the women
approach include maternal haemorrhage, characterized their pain as being present
infection, operative injury, thromboembo- daily or almost daily.19 One of the causes
lism, hysterectomy, and death. The risk of moderate to severe pelvic pain was
of uterine rupture is essentially limited neuropathic pain caused by entrapment of
to the VBAC group. As successful VBAC iliohypogastric or ilioinguinal nerves.20
is associated with the least complications As with chronic pain, pelvic adhe-
while a failed one with more complica- sions increase with the number of cae-
tions, careful selection of women who sarean sections. Dense adhesions make
Both planned vaginal birth after would most likely deliver vaginally is the subsequent operation more difficult, and
previous caesarean section and elective
key to management of pregnancy after increase the operating time, blood loss
repeat caesarean section carry maternal
and neonatal risks. prior caesarean section. and the risk of injury to the surrounding or-
gans.20 Adhesions were assessed in a co-
Short term hort study involving 3,190 women in Saudi
ing and labour induction with transcervical Successful VBAC offers several distinct, Arabia. 21 Adhesions were described as se-
catheter were limited and retrospective. The consistently reproducible short-term ad- vere if they were dense or causing fusion
risk on uterine rupture was inconclusive. 1
vantages compared with ERCS, such as of uterus to the abdominal wall or blad-
Induced labour is also associated fewer hysterectomies, fewer thromboem- der. In that study, severe adhesions were
with lower success rate of VBAC com- bolic events, lower blood transfusion rate, present in 0.2%, 11.5%, 26.0%, 44.8%,
pared with spontaneous labour. If oxytocin and shorter hospital stay. However, when 54.5% and 50.6% of women having their
was used alone for induction or augmen- VBAC fails, emergency caesarean section first, second, third, fourth, fifth, and sixth
tation, the vaginal delivery rate was 62% is associated with increased uterine rup- or more caesarean sections, respectively.
(95% confidence interval, CI, 5370%) and ture, hysterectomy, operative injury, blood A strong association exists between
68% (95% CI, 6472%), respectively. 17
transfusion, endometritis, and longer hos- previous caesarean delivery and abnormal
Although induction of labour is not pital stay. 18 At present, clinical prediction placentation. A meta-analysis performed
contraindicated in women with prior cae- of the optimal candidate for planned VBAC by Ananth et al 22 showed that a woman
sarean delivery, the fact of lower success remains imprecise. with at least one previous caesarean
rate together with higher rupture risk was at 2.6 times greater risk of placenta
should be considered in the counselling. Long term previa in her subsequent pregnancy. The
Nonetheless, individual circumstanc- It is well-known that caesarean delivery risk increased with the number of prior

JPOG JUL/AUG 2013 172

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 172 8/5/13 2:09 PM


Continuing Medical Education

caesarean sections. fetal deaths per 1,000 live births compared 95% CI, 2.46.5), 38 weeks gestation (OR,
The most significant long-term risk of with expectant management. 3.0; 95% CI, 2.14.3) and 39 weeks gesta-
caesarean delivery is placenta accreta oc- As with perinatal death, asphyxia- tion (OR, 1.9; 95% CI, 1.23.0). A same
curring in subsequent pregnancies. As the related injury in VBAC usually occurs af- pattern was observed for risk of serious
placenta does not properly separate from ter uterine rupture. In a study by Landon respiratory morbidity at an even higher
the uterus after delivery, it may result in et al,25 the incidence of hypoxic ischemic odds ratio at 37 weeks gestation (OR 5.0;
massive bleeding, leading to disseminated encephalopathy was 0.08% in the VBAC 95% CI, 1.616.0). This is the reason why
intravascular coagulopathy, multi-organ group compared with 0% in the ERCS most authorities recommend that ERCS be
failure and maternal mortality. Even if cae- group, at a background uterine rupture rate performed after 39 weeks gestation. Other
sarean hysterectomy is to be performed as of 0.7%. potential benefits of vaginal delivery are
the life-saving procedure, the operation Sepsis is a frequent indication for the lower risk of hypothermia and hypoten-
could be difficult with its own set of com- admission to the neonatal intensive care sion at birth, higher likelihood of success-
plications and results in permanent loss of unit. Both maternal fever and prolonged ful breastfeeding, and lower incidence of
fertility. 20 As with previa, it is certain that rupture of membrane greater than 18 hours asthma in childhood. 28
the risk of placental accreta increases with are more common in the VBAC group.
the number of prior caesarean sections. Neonates who were born after failed VBAC NON-MEDICAL FACTORS
The combination of placenta previa and requiring emergency caesarean delivery
previous caesarean delivery dramatically had a significantly greater rate of suspect- Despite the numerous research-based evi-
increases the risk further. In a cohort study ed sepsis.26 dence on the safety of trial of labour after
of 723 women with placenta previa, ac- Although the evidence suggests an caesarean section, the VBAC rate remains
creta occurred in 3%, 11%, 40%, 61% and increased perinatal risk for women un- low in some developed countries such as
67% of those having their first, second, dergoing VBAC, the absolute numbers of the United States (8.5% in 2006). It is ap-
third, fourth, and fifth or more caesarean serious morbidity and mortality remain parent that some non-clinical factors play
sections, respectively. 23 low. One should also note that a large a role in the decision-making.
proportion of women undergoing VBAC The factors which might affect VBAC
FETAL BENEFIT AND RISK would deliver successfully. In fact, there rate in an institution include administrative
are numerous beneficial effects of labour policies, medicolegal pressures, profes-
Perinatal morbidity and mortality are other and vaginal delivery to the newborn. Even sional society guidelines, and patient and
concerns when considering the option of at term gestation, babies born vaginally obstetrician preferences.
delivery. The largest population-based are at lower risk of respiratory morbidity According to a survey by Wells30 in the
evaluation of perinatal mortality was per- (respiratory distress syndrome or transient United States, reasons for obstetricians
formed by Smith et al.24 The rate of deliv- tachypnoea of newborn) compared with not offering VBAC included (1) them be-
ery-related perinatal death was signifi- those born by prelabour caesarean sec- ing unwilling to accept the risk of adverse
cantly greater in the VBAC group than in the tion.27,28 This finding was demonstrated by outcome, (2) them not believing that VBAC
ERCS group (12.9 vs 1.1 per 10,000 births). a large cohort study29 in Denmark involving is safe, (3) medico-legal liability concerns,
The marked excess of perinatal deaths was 34,458 babies over 9 years. The risk of res- and (4) lack of immediate availability of fa-
mainly due to uterine rupture in the VBAC piratory morbidity for infants delivered by cilities for laparotomy.
group. What further complicated the rela- elective caesarean section was increased The decision may also be affected
tive risk is that elective caesarean delivery compared with that by the vaginal route at by non-clinical patient priorities, such as
at 39 weeks gestation would prevent two 37 weeks gestation (odds ratio, OR, 3.9; patients desire for a vaginal delivery, her

JPOG JUL/AUG 2013 173

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 173 8/5/13 2:09 PM


wish to experience (or avoid) labour, her in early pregnancy, planned VBAC could be are therefore not influenced by personal
need for scheduling the delivery, and her encouraged with the flexibility of a later financial gain. Women with history of
positive or negative feeling about the pre- change of plan. A local study has been prior caesarean deliveries are assessed by
vious delivery. It is important to discuss
31
conducted to assess the psychological im- obstetricians at the booking visit. The pre-
with the women their risk perception and pact of women being assigned to the mode vious operation records are traced via the
priorities when considering the mode of of delivery. It involved 298 women, who
33
electronic system of the public hospitals
delivery. showed no preference for VBAC or ERCS, or by an enquiry letter to the correspond-
being randomly assigned to either option ing obstetricians/hospitals. Planned VBAC
COUNSELLING AND with flexibility. Women in the planned is offered to women with one previous
DECISION-MAKING VBAC group achieved high success rate of uncomplicated transverse lower segment
vaginal delivery (73%) without an increase caesarean delivery. Information pamphlets
Depending on the clinical situation, either in the psychological stress and morbidity. on planned VBAC and ERCS are given out
planned VBAC or ERCS is usually appropri- during antenatal visits. The pregnancy
ate for most women with prior caesarean conditions are continuously reviewed. The
delivery. Understanding the womens be- mode of delivery is discussed before 37
liefs and values of the process and out- weeks gestation and documented in the
come is essential in providing evidence- record. Women who have no strong pref-
Successful VBAC carries
based, patient-centred care. Discussing
32
erence for either option are encouraged to
the options in early pregnancy allows the the lowest risk, consider planned VBAC when the pregnan-
women and their family to evaluate the followed by ERCS, cy conditions are favourable. Women who
risks and benefits based on their own intend to undergo ERCS are forewarned of
and the risk is highest
perception. While patient pamphlets play a possible re-evaluation of the mode of
an important role in general information- with failed VBAC, delivery if they present themselves in an
giving, the obstetricians advice should requiring caesarean advanced stage of labour. All women at-
be more specific to the individuals condi- tempting VBAC receive electronic continu-
delivery.
tion. The decision made in early pregnancy ous fetal heart monitoring during labour.
should never be considered final, as medi- Labour progress is assessed regularly by
cal and social circumstances continue to the obstetricians. Facilities and expertise
evolve. For instance, women who intend to for emergency operation are available in
deliver by ERCS might present with spon- EXPERIENCE FROM A PUBLIC the delivery suite. The authors experience
taneous labour before the scheduled date. OBSTETRIC UNIT over a 4-year period is shown in the Table.
If labour progresses well and the chance
of a successful VBAC is high, she might Kwong Wah Hospital is one of the eight CONCLUSION
change her preference to vaginal delivery. public hospitals in the Hong Kong terri-
According to the international guidelines, tory providing obstetric service. There are At present, there is no reliable formula
ERCS should be scheduled after 39 weeks almost 6,000 deliveries annually. Women to calculate the best mode of delivery
gestation in an otherwise uncomplicated receive obstetric service at minimal per- in women with prior caesarean section.
pregnancy. 1
sonal cost, as the service is largely funded This should be a shared decision made by
If a woman does not show any strong by the government. Obstetricians and mid- the obstetricians and the women, in-
preference regarding the mode of delivery wives are salaried, and the advices given corporating medical factors, social

JPOG JUL/AUG 2013 174

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 174 8/5/13 2:09 PM


Continuing Medical Education

circumstances and patients preferences. (< 1%) in UK, where the rate of primary turn women away from attempting VBAC
Successful VBAC carries the lowest risk, caesarean section has increased over the without balancing the short- and long-
followed by ERCS, and the risk is high- past decade. Moreover, the resulting seri- term complications of ERCS.
est with failed VBAC, requiring caesarean ous adverse outcome after uterine rupture
delivery. is very low in absolute number.
Although uterine rupture increases Given the high success rate of VBAC
the risk of adverse outcome, it is still a rare in carefully selected pregnancies, obste- About the Authors
Dr Yung is Associate Consultant, Dr Lau is Consultant, and
complication in high-resource settings. tricians should not overstate the risk and Dr Leung is Chief of Service at the Department of Obstet-
The incidence has remained the same consequence of uterine rupture so as to rics and Gynaecology, Kwong Wah Hospital, Hong Kong.

References
1. American College of Obstetricians and Vaginal birth after caesarean delivery: does ma- report/Technology Assessment No. 191. ery. N Engl J Med 2004;351:25812589.
Gynecologists. ACOG Practice bulletin no. 115: ternal age affect safety and success? Paediatr Rockville, MD: Agency for Healthcare Research 26. Hook B, Kiwi R, Amini SB, Fanaroff A, Hack
vaginal birth after previous cesarean delivery. Perinat Epidemiol 2007;21:114120. & Quality; 2010. M. Neonatal morbidity after elective repeat
Obstet Gynecol 2010;116:450463. 10. Shanks AL, Cahill AG. Delivery after 18. Lydon-Rochelle MT, Cahill AG, Spong CY. cesarean section and trial of labor. Pediatrics
2. Bujold E. Evaluating professional soci- prior cesarean: success rate and factors. Clin Birth after previous cesarean delivery: short- 1997;100:348353.
ety guidelines on vaginal birth after cesarean. Perinatol 2011;38:233245. term maternal outcome. Semin Perinatol 27. Patel RM, Jain L. Delivery after previous ce-
Semin Perinatol 2010;34:314317. 11. Juhasz G, Gyamfi C, Gynmfi P, Tocce K, 2010;34:249257. sarean: short-term perinatal outcomes. Semin
3. Pruett KM, Kirshon B, Cotton DB, Poindexter Stone J. Effect of body mass index and ex- 19. Nikoajsen L, Sorensen HC, Jensen TS, Perinatol 2010;34:272280.
AN 3rd. Is vaginal birth after two or more cessive weight gain on success of vagina Kehlet H. Chronic pain following Caesarean 28. Sinha A, Bewley S, McIntosh T. Myth: ba-
cesarean sections safe? Obstet Gynecol birth after cesarean delivery. Obstet Gynecol section. Acta Anaesthesiol Scand 2004;48: bies would choose prelabour caesarean section.
1988;72:163165. 2005;106:741746. 111116. Semin Fetal Neonatal Med 2011;16:247253.
4. Beall M, Eglinton GS, Clark PL, Phelan JP. 12. Durnwald CP, Ehrenberg HM, Mercer BM. 20. Silver RM. Delivery after previous cesarean: 29. Hansen AK, Wisborg K, Uldbjerg N,
Vaginal delivery after cesarean section in wom- The impact of maternal obesity and weight gain long-term maternal outcomes. Semin Perinatol Henriksen TB. Risk of respiratory morbidity in
en with unknown types of uterine scar. J Reprod on vaginal birth after cesarean delivery. Am J 2010;34:258266. term infants delivered by elective caesarean
Med 1984;29:3135. Obstet Gynecol 2004;191:954957. 21. Makoha FW, Felimban HM, Fathuddien section: cohort study. BMJ 2008;336:8587.
5. Royal College of Obstetricians and 13. Eden KB, McDonagh M, Denman MA, et MA, Roomi F, Ghabra T. Multiple cesarean sec- 30. Wells CE. Vaginal birth after cesarean deliv-
Gynaecologists. Birth after previous caesarean al. New insights on vaginal birth after ce- tion morbidity. Int J Gynaecol Obstet 2004;87: ery: views from the private practitioner. Semin
section (Green-top 45). Available at http:// sarean: can it be predicted? Obstet Gynecol 227232. Perinatol 2010;34:345350.
www.rcog.org.uk. Published February 1, 2007. 2010;116:967981. 22. Ananth CV, Smulian JC, Vintzileos AM. The 31. Sharma PS, Eden KB, Guise JM, Jimison
6. Elkousy MA, Sammel M, Steven E, Peipert JF, 14. Landon MB, Leindecker S, Spong CY, et association of placenta previa with history of HB, Dolan JG. Subjective risk vs. objective risk
Macones G. The effect of birth weight on vagi- al. The MFMU Cesarean Registry: factors af- cesarean delivery and abortion: a meta-analy- can lead to different post-cesarean birth deci-
nal birth after cesarean delivery success rates. fecting the success of trial of labor after pre- sis. Am J Obstet Gynecol 1997;177:10711078. sions based on multiattribute modeling. J Clin
Am J Obstet Gynecol 2003;188:824830. vious cesarean delivery. Am J Obstet Gynecol 23. Silver RM, Landon MB, Rouse DJ, et al. Epidemiol 2011;64:6778.
7. Jastrow N, Roberge S, Gauthier RJ. Effect 2005;193:10161023. Maternal morbidity associated with mul- 32. Kaimal AJ, Kuppermann M. Understanding
of birth weight on adverse obstetric outcomes 15. Flamm BL, Geiger AM. Vaginal birth after ce- tiple cesarean deliveries. Obstet Gynecol risk, patient and provider preferences, and
in vaginal birth after cesarean delivery. Obstet sarean delivery: an admission scoring system. 2006;107:12261232. obstetrical decision making: approach to
Gynecol 2010;115:338343. Obstet Gynecol 1997;90:907910. 24. Smith GC, Pell JP, Cameron AD, Dobbie R. delivery after cesarean. Semin Perinatol
8. Peaceman AM, Gersnoviez R, Landon MB, 16. Cheng YW, Eden KB, Marshall N, Pereira L, Risk of perinatal death associated with labor af- 2010;34:331336.
et al. The MFMU Cesarean Registry: impact of Caughey AB, Guise JM. Delivery after prior ce- ter previous cesarean delivery in uncomplicated 33. Law LW, Pang MW, Chung TKH. Randomised
fetal size on trial of labor success for patients sarean: maternal morbidity and mortality. Clin term pregnancies. JAMA 2002;287:26842690. trial of assigned mode of delivery after a previ-
with previous cesarean for dystocia. Am J Perinatol 2011;38:297309. 25. Landon MB, Hauth JC, Leveno KJ, et al. ous cesarean sectionimpact on maternal psy-
Obstet Gynecol 2006;195:11271131. 17. Guise JM, Eden K, Emeis C, et al. Vaginal Maternal and perinatal outcomes associated chological dynamics. J Matern Fetal Neonatol
9. Srinivas SK, Stamilio DM, Sammel MD, et al. Birth After Cesarean: New Insights. Evidence with a trial of labor after prior cesarean deliv- Med 2010;23:11061113.

JPOG JUL/AUG 2013 175

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 175 8/5/13 2:09 PM


CME
CME Questions
Questions

Program pendidikan kedokteran berkelanjutan ini dipersembahkan oleh MIMS, bekerjasama dengan
Ikatan Dokter Indonesia.
Setelah membaca artikel Management of Pregnancies With Previous Caesarean Section, jawab
pertanyaan berikut kemudian kirimkan dengan menggunakan formulir jawaban yang sudah disediakan ke
CME Journal of Paediatrics, Obstetrics & Gynaecology, untuk mendapatkan 5 SKP.

P
Artikel CME: 5 SK

Management of Pregnancies With Previous Caesarean Section


Jawab pertanyaan di bawah ini dengan Benar atau Salah

Please indicate on your answer sheet whether the following statements are True or False.

1. Previous classical caesarean section is a contraindication to planned vaginal birth after previous caesarean section
(VBAC).
2. Patients with unknown type of previous caesarean scar should not be offered planned VBAC.
3. Obese patients (body mass index, BMI, > 30) should not be offered planned VBAC, as high BMI is associated with a low
success rate.
4. Spontaneous labour increases the likelihood of successful VBAC.
5. Continuous electronic fetal monitoring should be offered to all women attempting VBAC.
6. Labour induction and augmentation by oxytocin are associated with increased risk of uterine rupture in patients with prior
caesarean delivery.
7. The risk of placenta previa/placenta accreta is directly related to the number of previous caesarean deliveries.
8. Perinatal mortality and asphyxia-related fetal injury are more significant in the VBAC group compared with the elective
repeat caesarean section (ERCS) group.
9. Babies who are born vaginally are at lower risk of respiratory morbidity in both the short and long term.
10. Women who decline planned VBAC should be offered ERCS at 37 weeks gestation in order to avoid spontaneous labour
before the scheduled operation.

JPOG JUL/AUG 2013 176

JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 176 8/5/13 2:09 PM

Anda mungkin juga menyukai