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PAEDIATRIC SURGERY

INTRODUCTION TO CONGENITAL SURGICAL ANOMALITIES OF


SURGICAL INTEREST
E.N. SAYI
Lecture note consists of the following brief topics:1.

Congenital abnormalities of the MOUTH. i.e. Lip and Palate.

II.

Oesophageal atresia (OA) and Tracheoesophageal Fistula (T&F).

III.

Anorectal Malformations (ARM)


-

1.

Imperforate Anus

CONGENITAL ANOMALIES OF MOUTH.


A.

CLEFT LIP.
It is an abnormality involving the upper or lower lip.
(i)

Embrology:
The face develops from 5 processes:Frontonasal process.
Maxillary processes (2)
Mandibular processes (2)
Incomplete union of these processes may result in variety of
clefts.

(ii)

Aetiological factors:Hereditary.
Nutrition during pregnancy.
Mechanical factors.
Tumours, amniotic bands and adhesions.
Infection and injuries during pregnancy.

(iii)

Epidemiology:
Frequency varies from 1:500 to 1:1000. Live births
Boys are more affected than girls.
The left side is more affected than the right side.

B.

CLEFT PALATE:
(i)

Pre-palate (primary or labial palate)


-

(ii)

C.

Palatal development is related to the development of the


face, lips, premaxilla and 4 incisor teeth.
It is the region anterior to the INCISIVE FORAMEN.

Palate proper (Secondary palate or oval).


It is related to the development of the hard palate, soft
palate, uvula and maxillary teeth.
It is the region posterior to the INCISIVE FORAMEN.

CLASSIFICATION OF LEFT LIP AND PALATE ANOMALIES


There are many classifications.
Commonest is THE RITCHIE DAVIS VEAUS anatomic
classification:(i)

GROUP I
Pre alveolar clefts (lip only).
Unilateral, bilateral or median.
Partial or complete clefts.

II.

(ii)

GROUP II
Post alveolar, clefts (palate only).
Unilateral or bilateral clefts.

(iv)

GROUP III
Complete Alveolar.
Unilateral.
Bilateral.

OESOPHAGEAL ATRESIA + TRACHEOESOPHAGEAL FISTULA (OA/TOF)


A.

EMBROLOGY:
-

The trachea and oesophagus first appear as a ventral diverticulum


of the FOREGUT at the gestational age of 20th 23 days.
As the diverticulum elongates, masses of endodermal cells form
ridges of tissues that ultimaltely divide the foregut into tracheal
and oesophageal channels.

By the 26th day of gestation the oesophagus and trachea have


become completely separated.

It is during this period of separation when abnormalities may


occur.

The explanation of development of OA and TOF is less clear.

There are theories which may contribute such as:(i)


(ii)

B.

Vascular deficiency.
Genetic transmission e.g. patients who survived repair
produce off- springs with the same abnormality.

CLINICAL/ANATOMICAL CLASSIFICATION (TYPE)


TYPE 1:

ISOLATED OESOPHAGEAL ATRESIA (OA)


In this abnormality both the upper and lower pouches of
oesophagus end blindly. There is no oegophageal
continuity.
The incidence of this type is about 5%.
It is the second commonest.

TYPE 2:

OESOPHAGEAL ARTRESIA (OA) WITH DISTAL TRACHEOESOPHAGEAL FISTULA (TOF)


-

TYPE 3:

The upper oesophageal segment ends blindly.


The lower oesophageal segment ends into the trachea or
bronchus.
The type can be subdivided into:
(i)
Short gap.
(ii)
Log gap.
The incidence of this type is 85 90% of all cases.
It is the most common type.

ISOLATED TRACHEOESOPHAGEAL FISTULA WITHOUT


OESOPHAGEAL ATRESIA. (H-TYPE).
There is no true oesophageal atresia.
A trachoesophageal fistula (TOF) is the only
abnormality.
-

It is difficulty to diagnose.

Swallowing is normal.

TYPE 4:

Commonest presentation is chronic chest infection and


choking on breast feeding.

The incidence of this type is 2 to 6%. Average 3%.

It is the 3rd commonest type.

OESOPHAGEAL ATRESIA WITH PROXIMAL TOF.


-

The upper oesophageal segments ends into the either


trachea or bronchus.
The lower oesophageal segments ends blindly
The incidence is less than 1%.
It is one of the rare varieties.

GENERAL SYMPTOMATOLOGY:
III.

Failure to swallow even saliva.


Respiratory distress immediately after birth.
There is foam formation in the month due to
accumulation of saliva and pharyngeal secretions.
Chest infection.

ANORECTOMALFORMATIONS (IMPERFORATE ANUS)


1.

DEFINITION.
It is the congenital disturbances in the normal developmental process of
the anus and rectum.

2.

AETIOLOGY.
-

3.

Poly aetiological in nature, i.e. various harmful agents


acting in the critical period of embryo development and
in the period of organogenesis.

INCIDENCE:
The incidence in the US is 1: 5000 live births.

4.

CLASSIFICATION

As described at the WINGSPREAD CONFERENCE U.S.A in


1994.

The anomalies in both sexes are divided as:


(a)
(b)
(c)

High.
Intermediate.
Low anomalies.

This is in relationship to the level of the puboretalis portion of the


levator ani muscle.

Also whether there is a fistula to the urinary tract in males or the


vagina in females.

The following table summarizes the classifications:CLASSIFICATION


(a) HIGH ANOMALIES

MALES
ANORECTAL AGENESIS
with
- Recto urethral fistula
- without fistula
- rectal atresia alone

FEMALES
ANORECTAL AGENESIS
with
- Recto vaginal fistula
- without fistula
- rectal atresia alone

(b) INTERMEDIATE
ANOMALIES

With
- Rectobulbar fistula
- Anal agenesis without
fistula.
With
- Anocutaneous fistula
- Anal stenosis
PERSISTENT CLOACA

With
- Rectovestibular fistula
- Rectovaginal fistula
- Anal agenesis.

LOW ANOMALIES
(D) RARE FORM

5.

- Ano vestibular fistula


- Anocutaneous fistula.
PERSISTENT CLOACA

INVESTIGATIONS.
(a) Radiology.
-

Plan abdominal X-ray including INVERTOGRAM.


Colostogram.
Abdominal Ultrasound Scan.
Examination under anaesthesia.

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