An extrahepatic biliary atresia
(EHBA) mimic- a case report
Hemlata Singh1,
Bhavika Y
M2, Ramkesh Meena3, Vivek
Dewan4
1Dr. Hemlata Singh, Senior Resident, Paediatrics, 2Dr. Bhavika
Y.M,PG Resident, 3Dr. Ramkesh Meena, Senior
Resident, 4Dr. Vivek Dewan, Professor, MD paediatrics, all authors are
affiliated with Department of Paediatrics, PGIMER Dr RML
hospital, New Delhi, India
Address for
Correspondence: Dr Hemlata Singh, Department of
Pediatrics, PGIMER and Dr RML Hospital, Baba Kharak Singh Marg, New
Delhi, India. E-mail: drhema.rml@gmail.com
Abstract
Extra hepatic biliary obstruction is surgically correctable cause of
neonatal cholestasis, with good outcome if operated within 6 weeks. We
report a case mimicking biliary atresia clinically and on imaging
(HIDA, MRCP) and had complete resolution after exploratory laparotomy.
Per-operatively, bile ducts were found to be patent; subsequently
patient’s bilirubin status and liver functions improved and
child has been thriving well. Preoperative liver biopsy showed patent
ducts with findings of cholestasis.
Key words:
neonatal cholestasis, MRCP. bile plug, EHBA
Manuscript received:
2nd June 2017, Reviewed:
12th June 2017
Author Corrected:
21st June 2017, Accepted
for Publication: 28th June 2017
Introduction
EHBA is one of the most commonly encountered causes of neonatal
cholestasis with a prevalence of 1 in 15000-20000. With latest imaging
techniques it is relatively easier to differentiate it from neonatal
hepatitis and confirm with liver biopsy. Most patients (85%-90%) have
progressive postnatal obliteration of bile ducts requiring early
surgical correction. We report a case presenting as EHBA clinically,
biochemically, sonographically and on imaging, however, preoperatively
on saline flushing, had a patent biliary system with relief of
mechanical obstruction.
Case
Report
A two months male child admitted with progressively increasing jaundice
since 10 days of life and passing white/pale stools since birth. There
was no fever, vomiting, irritability, seizures, bleeding
manifestations. He was born at term with uneventful antenatal and
perinatal period. Child was hemodynamically stable with deep icterus
and hepatomegaly with liver span of 7 cm, but had no features of liver
cell failure or facial dysmorphism. Fundus was normal, spleen was not
palpable and other system examination within normal limits.
Investigations revealed conjugated hyperbilirubinemia (total bilirubin-
13, direct- 5 mg/dl) and deranged liver enzymes (SGOT-258 /SGPT- 168
U/L), total alkaline phosphatase (369 U/L) and GGT level (178 U/L).
Serum proteins (5.5 mg/dl), S. albumin (3.9 mg/dl), and coagulation
profile was normal. Other investigations showed negative TORCH profile,
absence of reducing substances in urine, non-reactive viral markers
(HBsAg/HAV/HCV/HEV), normal TMS screen, normal levels of alpha-1
antitrypsin and sweat chloride, normal lipid and thyroid profiles.
Ultrasonography showed normal liver echotexture with contracted gall
bladder, normal portal vein size and distal CBD was not visualised.
HIDA scan was suggestive of biliary outflow obstruction. MRCP showed a
diffusely narrowed common bile duct in mid-portion. Inevitably, EHBA
was considered as diagnosis. Exploratory laparotomy and open liver
biopsy was done but preoperatively, after flushing of biliary tract,
findings were inconsistent with biliary atresia and cholestasis; biopsy
was taken for confirmation. Liver biopsy showed ballooning feathery
degeneration and giant cell canalicular cholestasis, rosette formation,
focal bile lakes, focal sinusoidal dilation with prominence of kupffer
cells with few showing lipofuschin pigment and few acidophilic bodies
consistent with neonatal cholestasis. Postoperatively, patient had
improving trends of hyperbilirubinemia and gradual resolution of
bilirubin was documented within few days of laparotomy. At 3 month
follow up, a consistent weight gain was observed along with normal LFT
(Total- 0.4 mg/dl; Direct-0.1 mg/dl; SGOT-22 U/L; SGPT-28 U/L; GGT- 20
U/L).
Discussion
Biliary atresia (BA) is an ascending inflammatory process of biliary
tree leading to biliary cirrhosis. Hence rapid identification of BA is
crucial [1]. HIDA scan (hepatobiliary sequence scintigraphy) has a high
sensitivity (83–100%) but lacks specificity in many cases
(33–80%), limiting its usefulness to differentiate between BA
and other non-surgical conditions [2]. Magnetic resonance
cholangiopancreatography (MRCP) is a widely accepted method of imaging
the biliary system. The diagnostic value of MRCP for EHBA is
82–98%, with sensitivity of 90–100%, and
specificity of 77–96% [3]. In our patient, on the basis of
HIDA and MRCP a diagnosis of biliary atresia was kept and patient was
referred for surgery to avoid further delays but paradoxically no
evidence of atresia was found peroperatively. The plausible explanation
could be dislodgement of a bile plug or breaking of any thin membrane
and drainage via the bile duct. We could not ascertain an alternative
diagnosis for this presentation. Child is on follow up in special
clinic and is thriving well with no jaundice and normal liver
functions. This kind of presentation of biliary atresia mimic as a
result of mechanical block possibly due to mucus plug or membrane has
never been reported in the literature to the best of our knowledge.
Conclusion
EHBA is a surgical emergency but atypical presentation such as in this
case where spontaneous remission was seen, builds up the curiosity to
identify additional pathogenetic mechanism that might explain such
presentations and defer radical surgeries.
Abbreviations: EHBA-
extrahepatic biliary obstruction; HIDA- hepatobiliary iminodiacetic
acid; MRCP- magnetic resonance cholangiopancreaticography
Funding:
Nil, Conflict of interest:
Nil
Permission from IRB:
Yes
References
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Hadchouel M, Niaudet P. Renovascular hypertension and vascular
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3. Yang J, Ma D, Peng Y, Song L, Li C. Comparison of different
diagnostic methods for differentiating biliary atresia from idiopathic
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How to cite this article?
Hemlata Singh, Bhavika Y M, Ramkesh Meena, Vivek Dewan. An extrahepatic
biliary atresia (EHBA) mimica case report. J
PediatrRes.2017;4(06):361-362.doi:10.17511/ijpr.2017.06.02.