부산대학교 도서관

Background: Neonatal hydrops of the gall bladder is a rare entity, resolving conservatively most of the times, with requirement of surgery only in few cases. It is usually seen in association with septicemia, jaundice, and inborn errors of metabolism. Diagnostic laparoscopy happens to be a useful tool in guiding the management of these babies. Clinical description: Case 1: A 12-day-old neonate presented with refusal to feeds, jaundice, and right upper abdominal mass. Serum ferritin was markedly raised. Radiological work-up revealed a distended gall bladder and on MRI, there were diffuse hypo-intensities in the liver suggestive of iron deposition. A diagnostic laparoscopy was performed which was suggestive of an over-distended, tense, and shiny gall bladder with normal biliary tree and hepatomegaly. In view of clinical deterioration and large liver, an exploratory laparotomy with cholecystectomy and liver biopsy was performed. Histopathology revealed non-inflamed gall bladder and showed iron overload in the liver biopsy. Case 2: A 2-month-old baby presented with jaundice, clay-colored stool, abdominal distension, and a palpable mass. Sonography and MRCP were suggestive of distended gall bladder with patent distal CBD and dilated IHBR. Serum GGT was raised. USG-guided aspiration revealed white clear transparent fluid. A diagnostic laparoscopy was performed and showed atretic biliary tree with cirrhotic liver and massively distended gall bladder. As the laparoscopic findings were consistent with the biliary atresia, an exploratory laparotomy and Kasai’s portoenterostomy were performed. Conclusion: Neonatal hydrops usually is secondary to underlying medical or surgical conditions. Apart from medical conditions, rarely it can also occur secondary to neonatal hemosiderosis as seen in gestational alloimmune liver disease and biliary atresia. We thought of proceeding for diagnostic laparoscopy to confirm the rare association before embarking upon the definitive repair.