Current Anaesthesia & Critical Care 21 (2010) 153e155

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Current Anaesthesia & Critical Care

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CASE REPORT

A case of atypical HELLP (haemolysis, elevated liver enzymes and low platelet
count) syndrome presenting as bleeding from the epidural puncture site
during labour
S. Roopa, Harihar V. Hegde*, Rohini Bhat Pai, Vijay G. Yaliwal, P. Raghavendra Rao
Department of Anaesthesiology, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka 580 009, India

s u m m a r y
Keywords: HELLP (haemolysis, elevated liver enzymes and low platelet count) syndrome has been recognised as
Atypical HELLP syndrome a life threatening complication of pregnant women for many years. The majority of women with HELLP
Epidural labour analgesia
syndrome have hypertension and proteinuria which may be absent in 10e20% of casesereferred to as
Thrombocytopenia
atypical HELLP. A primigravida received epidural labour analgesia and she continued to bleed from the
skin puncture site. Evaluation for the cause of bleed established the diagnosis of atypical HELLP
syndrome. Although rarely encountered, an atypical HELLP may have dangerous anaesthetic conse-
quences when clinicians are caught unawares. We report one such case with atypical presentation.
Ó 2010 Elsevier Ltd. All rights reserved.

1. Introduction showed traces of albumin. Bleeding time and clotting time

HELLP (haemolysis, elevated liver enzymes and low platelet
count) syndrome has been recognized as a life threatening compli-
cation of pregnant women for many years. Weinstein coined the
term HELLP in 1982 and since then, there have been controversies
regarding diagnosis and management of this syndrome.1 Diagnosis
of the complete form of the HELLP syndrome requires the presence
of all 3 major components (haemolysis, elevated liver enzymes and
low platelet count). The majority of women with HELLP syndrome
have hypertension and proteinuria which may be absent in 10e20%
of casesereferred to as atypical HELLP.2 When clinicians are caught
unawares, such a manifestation may have dangerous anaesthetic
consequences. We report one such atypical case.
2. Case report
A 35-year-old primigravida at 40 weeks of gestation with
uneventful antenatal follow-up was admitted for safe
confinement. On examination, her weight was 63 kg, pulse rate
was 100 beats/min and blood pressure measured in the right arm,
in supine position was 110/70 mmHg. There was pedal oedema.
Systemic examination was unremarkable. The Obstetrician
deemed her pelvis to be adequate for vaginal delivery. Her hae-
moglobin was 10.2 g/dl and blood group Aþve. Urine examination
* Corresponding author. Tel.: þ91 836 2477755; fax: þ91 836 2461651.
E-mail address: drharryhegde@yahoo.co.in (H.V. Hegde).
estimated were 3 min 30 s and 5 min 30 s respectively. Platelet
count in the third trimester was 231 000/mm3.
Labour was induced with misoprostol (synthetic prostaglandin
analogue). While she was in active labour, an epidural catheter was
inserted in L3e4 interspace after locating the space by loss of
resistance to air technique using an 18 G Tuohy needle without any
difficulty. A test dose of 3 ml of 2% lignocaine with adrenaline 15 mg
was used to exclude intrathecal/intravascular placement. A bolus of
8 ml of 0.125% bupivacaine was administered epidurally followed
by an infusion of 0.0625% bupivacaine with 2 mg/ml fentanyl at
8 ml/h. She was comfortable and analgesia was adequate. Heart
rate, blood pressure, oxygen saturation, sensory and motor
functions were regularly monitored.
Thirty minutes following epidural catheter insertion, active
oozing of blood was noted from the catheter insertion site. The
catheter was examined and the dressing was changed with sterile
dry gauze. No blood or blood tinged fluid was aspirated through the
epidural catheter. She remained pain free which confirmed the
correct position of the epidural catheter, and the epidural infusion
was continued. However, the catheter insertion site continued to
bleed for which she was investigated further. Coagulation profile,
peripheral smear, liver function tests and renal function tests were
requested. She had retention of urine and a Foley's catheter (14 Fr)
was passed without any difficulty which revealed haematuria.
Blood pressure was in the range of 120/70e130/70 mmHg
throughout the intra-partum period.
Investigations revealed haemoglobin-9.6 g/dl, platelet count-
35 000 cells/mm3 with prolonged activated partial thromboplastin

0953-7112/$ e see front matter Ó 2010 Elsevier Ltd. All rights reserved.
doi:10.1016/j.cacc.2010.02.004
154 S. Roopa et al. / Current Anaesthesia & Critical Care 21 (2010) 153e155

time (aPTT) and prothrombin time (PT) (Table 1). Total bilirubin was
2.5 mg/dL, direct bilirubin-0.6 mg/dL, aspartate aminotransferase
(AST)-200 U/L, alanine aminotransferase (ALT)-140 U/L and lactate
dehydrogenase (LDH)-860 U/L. Peripheral smear showed
normocytic-normochromia, a few schizocytes, reticulocyte count
>2%, thrombocytopenia and leucocytosis. The diagnosis of atypical
HELLP syndrome was established.
She delivered a healthy female baby with an episiotomy after 4 h
and 30 min with adequate analgesia following which the epidural
infusion was discontinued. The epidural catheter was not removed
and a vigilant neurological monitoring was continued. There was
oozing of blood from the episiotomy wound even after the repair.
She developed post-partum haemorrhage and shock (pulse rate-
136 beats/min and blood pressure-72/40 mmHg) which required
oxytocin infusion, methylergometrine, prostaglandin F2-a and
uterine massage, resuscitation with crystalloids (1.5 L), colloids
(gelatin, 500 mL), fresh whole blood (2 Units) and platelets (2 Units).
The estimated blood loss was 1500 ml. She also had one episode of
haematemesis.
Eight hours post-partum, her haemodynamics stabilised. There
was no further post-partum haemorrhage. Oozing from the
epidural catheter insertion site also had stopped. An ultrasonog-
raphy did not reveal any abnormalities of liver or other organs.
Fibrin degradation product (FDP) performed on the 2nd post-par-
tum day was 40 m/ml (normal value <5 m/ml).
On the 2nd post-partum day, she developed pulmonary oedema
which was managed with O2, propped-up position, furosemide, and
fluid restriction. The epidural catheter was removed on 3rd post-
partum day when the platelet count was 90 000 cells/mm3. Acute
renal failure was managed conservatively. She was discharged on
15th post-partum day.
3. Discussion
HELLP syndrome occurs in about 0.5e0.9% of all pregnancies
and in about 10e20% of cases with severe preeclampsia. The
syndrome manifests itself usually between 32 and 34 weeks of
gestation, even though 30% of the cases occur in the post-par-
tum period. Various diagnostic criteria have been defined. In the
Tennessee classification system, diagnostic criteria for HELLP are
haemolysis with increased LDH (>600 U/L), AST (70 U/L), and
platelet count of (<100$109/L). The Mississippi tripleeclass
HELLP system further classifies the disorder by the nadir platelet
count.3
An early detection of HELLP syndrome seems to be difficult, as
its onset, presentation and course vary from patient to patient.
Classical symptoms of HELLP include right upper quadrant or
epigastric pain, nausea and vomiting. About 30e90% of women
have headache and 20% visual symptoms. Most patients give
history of malaise for a few days before presentation and some give
nonspecific viral syndrome like symptoms. A majority of patients
with HELLP syndrome have hypertension and proteinuria, which
may be absent in 10e20% of cases.2 Segal et al4 have reported two
atypical cases of HELLP syndrome which lacked usual signs of
preeclampsia, such as hypertension and proteinuria. Koenen et al5
have noted diurnal pattern in the clinical symptoms of HELLP
syndrome with exacerbation during the night and recovery during
the day.
Thrombocytopenia complicates 10% of all pregnancies.6 Three
most common causes are incidental gestational thrombocytopenia
(74%), preeclampsia and HELLP (21%), and immune thrombocyto-
penic purpura (4%). Other rare causes include thrombotic throm-
bocytopenic purpura, hemolytic uremic syndrome, disseminated
intravascular coagulation etc. In gestational thrombocytopenia, the
platelet count is rarely <100 000/mm3. Although platelet count is
stable and their function is preserved in many conditions, platelet
count may fall within a short period of time and their function can
be impaired in patients with preeclampsia.7
Currently, there is no consensus over the ‘safe’ platelet count to
administer regional anaesthesia in parturients even though most
consider the lower limit of platelet count for regional anaesthesia as
50  109/L in non-preeclamptic parturients.8 There are isolated case
reports9 of uneventful epidural labour analgesia in patients with
platelet count well below this level. In a recent review of neuraxial
techniques in obstetric and non-obstetric patients with common
bleeding diatheses,10 the authors concluded that the minimum
“safe” factor levels and platelet count for neuraxial techniques
remain undefined. Epidural catheters have safely been removed in
living liver donors11 with platelet count >82  109/L. It would be
reasonable to perform regional anaesthesia in pre-eclamptic
patients if the platelet count is >75  109/L considering the
possibility of additional platelet functional abnormality in these
patients.
Martin et al12 have studied the pattern of disease progression
and regression. They have noted that most gravid women with
HELLP syndrome had decreasing platelet count until 24e48 h after
delivery, and in all patients who recovered, a platelet count
>100 000/mm3 was spontaneously achieved within 72 h or by 6th
post-partum day. Our patient also had increasing trend in platelet
count in the post-partum period.
Thrombotic thrombocytopenic purpura - Hemolytic uremic
syndrome (TTP-HUS) in pregnancy has a spectrum of presentations
which are similar to preeclampsia, eclampsia and HELLP syndrome
and are indistinguishable.13 However, the HELLP syndrome
improves following delivery. Hence the diagnosis of HELLP
syndrome was made. Some authors suggest that subclinical (or
compensated) DIC is present in all women with HELLP
syndrome.14,15
The HELLP syndrome is associated with both maternal and
foetal complications. More common and serious maternal compli-
cations are abruption-placenta, disseminated intravascular coagu-
lation and post-partum bleeding.2

Table 1
Laboratory parameters.

Ante-partum Day 1 Day 2 Day 3 Day 4 Day 5 Day 6

Haemoglobin (g/dl) 9.6 7.8 7.1 8.2 9.0 9.0 9.2
Platelet count (cells/mm3) 35000 60000 77000 90000 130000 150000 231000
PT-test/control (s) 17.3/15.3 15.3/11.9
aPTT-test/control (s) 48.9/25.8 33.4/25.8
INR 1.13 0.78
Blood urea (mg/dL) 37 58 77 89 95 56 40
Serum creatinine (mg/dL) 2.6 3.2 4.0 5.3 4.7 2.1 1.8
Sodium (mmol/L) 134 131 126 126 127 130 132
Potassium (mmol/L) 5.7 5.1 4.8 4.8 4.4 4.1 3.9
Urine output (ml/24 h) 800 2450 2200 1800 1450 2000 2550

PT ¼ prothrombin time, aPTT ¼ activated partial thromboplastin time, Day 1e6 ¼ post-partum days.
 S. Roopa et al. / Current Anaesthesia & Critical Care 21 (2010) 153e155
Life threatening neurological complications of the HELLP
syndrome includes cerebral oedema, thrombosis and haemorrhage.
One of the catastrophic complications occurring in approximately
2% of patients is spontaneous bleeding into liver parenchyma
accompanied by rupture of the organ with a maternal mortality of
40e60%.16 Other known serious complication includes acute renal
failure and pulmonary oedema. Development of the HELLP
syndrome in the post-partum period increases the risk of renal
failure and pulmonary oedema.2
Thromboelestogram (TEG) may have some role in the evaluation
of coagulation in parturients with thrombocytopenia. A significant
correlation between the platelet count and the TEG variables,
maximum amplitude (MA) and k time has been shown.17,18 TEG was
not available in our institute.
We made the diagnosis of atypical HELLP syndrome only after
the patient was evaluated for the cause of thrombocytopenia.
Bleeding from the epidural catheter insertion site gave the first clue
that led to the diagnosis, which might otherwise have been made
significantly later. Apart from the pedal oedema and traces of
albumin in urine, our patient did not have any of the diagnostic
indicators such as hypertension, proteinuria, or typical symptoms.
A careful decision on the timing of removal of the epidural catheter
was also necessary as the risk of haemorrhage versus the risk of
infection if the catheter was left in-situ for prolonged period were
considered. Although rarely encountered, an atypical presentation
as in our patient can result in potentially catastrophic events.
Implications of this to the anaesthesiologist are multiple with
regard to the administration of labour analgesia as well as
anaesthesia. The aggressive resuscitation may have contributed to
the pulmonary oedema.
American Society of Anesthesiologists task force on obstetric
anaesthesia practice guidelines has not recommended a routine
platelet count in a healthy parturient before inserting an epidural
catheter.19 An unanticipated bleeding diathesis in a patient
receiving epidural catheter may result in an epidural haematoma
with lasting consequences and medico-legal implications although
we were fortunate in this patient. If such a patient undergoes
caesarean section, the caregivers might be caught unawares with
an excessive blood loss and post-partum haemorrhage. Vaginal
delivery can lead to an unanticipated post-partum haemorrhage
and shock as occurred in our patient.
To conclude, although evaluation of the coagulation profile in
every parturient is not justified, Anaesthesiologists and Obstetri-
cians need to have low threshold for evaluating such patients for
the cause of bleeding and manage the patient appropriately.
Financial support
There are no financial interests.
155
Conflicts of interest
There are no conflicts of interest involved in this study.
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