RISHI BOLIA UJJAL PODDAR SURENDER KUMAR YACHHA ANSHU SRIVASTAVA Correspondence: Dr. Ujjal Poddar Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow - 226014, India Email: ujjalpoddar@hotmail.com References 1. Giefer MJ, Murray KF, Colletti RB. Pathophysiology, diagnosis, and management of pediatric ascites. J Pediatr Gastroenterol Nutr. 2011;52:50313. 2. Redman JF, Seibert JJ, Arnold W. Urinary ascites in children owing to extravasation of urine from the bladder. J Urol . 1979;122:40911. 3. Vasdev N, Coulthard MG, De la hunt MN, Starzyk B, Ognjanovic M, Willetts IE. Neonatal urinary ascites secondary to urinary bladder rupture. J Pediatr Urol. 2009;5:1004. 4. Kuwata T, Matsubara S, Nakamura S, Nakai H. Urinary ascites in a fetus with posterior urethral valve: antenatal diagnosis. Pediatr Int. 2011;53:2812. 5. Murphy D, Simmons M, Guiney EJ. Neonatal urinary ascites in the absence of urinary tract obstruction. J Pediatr Surg. 1978;13:52931. 6. Kato A, Yoshida K, Tsuru N, Ushiyama T, Suzuki K, Ozono S, et al. Spontaneous rupture of the urinary bladder presenting as oliguric acute renal failure. Intern Med. 2006;45:8158. 7. Saliba W, Grant ME. Conservative management of spontaneous bladder rupture. Kansas J Med. 2011;4:446. 8. Stebbing J, Ezra DG, Cackett PD, Greenstein AS. Ascites and apparent renal failure treated with a Foley catheter. J R Soc Med. 1999;92:5823. Congenital hepatic fibrosis with extra-hepatic porto-systemic shunt and hepatopulmonary syndrome successfully managed with living related liver transplantation Introduction Congenital hepatic fibrosis (CHF) is a rare condition in which portal hypertension may present without overt signs or symptoms of liver disease. Association of CHF with hepatopulmonary syndrome (HPS) has been well described in literature, 1 however there are no reports of CHF with extra- hepatic porto-systemic shunt presenting with HPS. We report here such a patient of CHF with extra-hepatic porto-systemic shunt presenting with HPS, who was successfully managed with liver transplantation. Case report A 14-year-old boy presented to us with two years history of exertional dyspnea and easy fatigability. He also gave history of bluish discoloration of hands and lips for the last three months. There was no history of jaundice, ascites, gastrointestinal bleed or easy bruisability. Clinical examination revealed cyanosis, grade II clubbing and hepatomegaly. There was no jaundice and the remaining systemic examination was normal. Investigations revealed a hemoglobin of 11.8 gm%, a platelet count of 146,000/cu mm and a normal coagulogram. Liver and kidney function tests were normal except for hypoalbuminemia. Arterial blood gas analysis showed an oxygen saturation of 68% and a PaO 2 of 37 mmHg. The patient Figure 1: 99 Tc MAA scan showing patchy perfusion in both lungs with reduced perfusion especially in apices of both lung fields. Significant uptake (73%) in the brain is visible and suggests intrapulmonary shunting. Tropical Gastroenterology 2014;35(2):116–118