Sawsan Drebeka1, Bashier Elgharmool2, Rabia Gwedar3, and Fauzi Sagher3
1-Department of Histopathology. 2-Department of Paediatric surgery. 3-Department of Paediatric Gastroenterology. Hepatology and Nutrition. T M C. Tripoli- Libya
JMJ Vol.6 No.1 (Autumn) 2006: 73-75
Nine months old Libyan boy presented with prolonged jaundice since the age of 15 days. He was born preterm, had septicaemia at early neonatal period presented to our department at the age of 45 days with prolonged neonatal jaundice and hepatomegaly, no ascites, no dilated veins, weight and length on 3rd centile, OFC at 25th centile. Laboratory investigations showed anaemia, leucocytosis, high ESR, and high eosinophilia, high total and direct bilirubin, alkaline phosphatase, GGTP and alpha feto-protein, PT was prolonged. TORCH and hepatitis screen were negative. Ultrasound showed homogenous echo-free cystic lesion “suspected choledocal cyst” and dilated right renal pelvis, (MCU) confirmed grade III right vesicoureteral reflux. HIDA scan: no intestinal excretion. Laparotomy done at the age of 66 days, a retroperitoneal large cystic lesion removed and cholecystectomy and liver biopsy were carried out. Histopathologically the mass consistent with Cystic Neuroblastoma. The liver histology showed giant cell hepatitis. Neuroblastoma associated with several conditions but, this is the first report of giant cell hepatitis, vesicoureteric reflux and cystic neuroblastoma.
Keywords: Cystic neuroblastoma, Giant cell hepatitis, Cholestatic jaundice, Conjugated hyperbilirubinaemia, Vesicoureteric reflux