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Semin Liver Dis 2000; Volume 20(Number 03): 385-390
DOI: 10.1055/s-2000-9425
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

DIAGNOSTIC PROBLEMS IN HEPATOLOGY

A 7-Month-Old Male Infant with Extra- and Intrahepatic Biliary CystsMARGRET. S. MAGID1,4 , SUKRU. EMRE2 , KAREN. I. NORTON3,4 , KATYA. IVANOV1 , BENJAMIN. L. SCHNEIDER2,4
  • 1Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai School of Medicine, New York, New York
  • 2Recanati-Miller Transplant Institute, Mount Sinai School of Medicine, New York, New York
  • 3Department of Radiology, Mount Sinai School of Medicine, New York, New York and
  • 4Department of Pediatrics, Mount Sinai School of Medicine, New York, New York
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Publication History

Publication Date:
31 December 2000 (online)

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CASE REPORT

A 4-month-old former 30-week premature infant presented with jaundice. Upon discharge from the newborn intensive care unit at 6 weeks of age, his direct bilirubin was 0.5 mg/dL (normal = < 0.3 mg/dL). Evaluation at 4 months of age included standard biochemical screening assays and an abdominal ultrasound, which demonstrated hepatosplenomegaly, ascites, and reversal of blood flow in the portal vein. A percutaneous liver biopsy revealed cirrhosis, cholestasis, and bile ductular proliferation. Given his advanced age and the presence of cirrhosis, a portoenterostomy was not performed. Supportive care, including fat soluble vitamins and a medium chain triglyceride containing formula, was administered.

He failed to thrive and was referred for possible liver transplant evaluation at 7 months of age. External examination was remarkable for jaundice and normal facial features. The liver was firm, nodular and palpable 8 cm below the right costal margin, and the spleen was palpable 2 cm below the left costal margin. No cardiac murmur was appreciated. There were ascites, and diminished muscle mass and subcutaneous adipose tissue. Laboratory evaluations revealed a total bilirubin of 12.7 mg/dL (normal ≤ 1.0 mg/dL), an albumin of 3.1 g/dL (normal = 3.9-5.0 g/dL), alkaline phosphatase of 724 U/L (normal = 145-420 U/L), gamma-glutamyl transpeptidase of 643 U/L (normal = 5-32 U/L), and a prothrombin time of 13 seconds (control = 11-15 seconds). Magnetic resonance cholangiopancreatography (MRCP) demonstrated focal areas of cystic dilatation, as well as some beading of the intrahepatic biliary system. The entire extrahepatic biliary system was massively dilated. The gallbladder was normal (Fig. [1]). There was no evidence of renal disease, including normal creatinine, urine analysis, renal ultrasonography, and renal magnetic resonance imaging. Living-related left lateral segment orthotopic liver transplantation was performed 3 weeks after initial evaluation for indications including intractable ascites, failure to thrive, and cholangitis. Post-operative chylous ascites was treated conservatively and the patient was discharged 23 days after the transplant. Follow-up at 6 months has been unremarkable with tacrolimus- based primary immunosuppression.

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