Etiological spectrum of esophageal varices due to portal hypertension in Indian children: Is it different from the West?

Background and Aim: Information about portal hypertension (PHT) in children is meagre. We therefore studied the spectrum and outcome of PHT in children (<= 14 years of age) over a period of 9 years. Methods: PHT was diagnosed on endoscopy (presence of varices) in 517 cases during the study period. The diagnosis of extrahepatic portal venous obstruction (EHPVO) and Budd-Chiari Syndrome (BCS) were made on the basis of ultrasound examination. Cirrhosis was diagnosed on the basis of clinical, biochemical, ultrasound, and liver biopsy (whenever feasible). Noncirrhotic portal fibrosis (NCPF) and congenital hepatic fibrosis (CHF) were diagnosed on liver biopsy. Endoscopic sclerotherapy (EST) was done in all the patients who presented with variceal bleeding and surgery was performed whenever indicated. Results: Causes of PHT included EHPVO in 54%, cirrhosis in 39%, CHF in 3%, NCPF in 2%, and BCS in 2%. Of these, 279 (54%) patients presented with upper gastrointestinal bleeding and this group comprised of EHPVO in 85%, cirrhosis in 10%, CHF in 2.5%, NCPF in 2%, and BCS in 1%. Bleeding was the presenting feature in 85% of EHPVO cases and in 13% of cirrhosis cases. In EHPVO cases, variceal eradication was achieved in 95% of cases with a mean 5 +/- 2.4 EST sessions. Surgery was required in 24 cases of EHPVO. Mortality due to bleeding was 1.7% in EHPVO and 30% in cirrhosis. Conclusions: EHPVO and cirrhosis are the two major causes of PHT in children. However, predominant cause of variceal bleeding is EHPVO. EST is an effective method of treatment in EHPVO.