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.
Highlights
EHBA is one of the most commonly encountered causes of neonatal cholestasis with a prevalence of 1 in 15000-20000
We report a case presenting as EHBA clinically, biochemically, sonographically and on imaging, preoperatively on saline flushing, had a patent biliary system with relief of mechanical obstruction
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
Summary
EHBA is one of the most commonly encountered causes of neonatal cholestasis with a prevalence of 1 in 15000-20000. Most patients (85%-90%) have progressive postnatal obliteration of bile ducts requiring early surgical correction. 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). 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. 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)
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