Revisiting extra hepatic portal vein obstruction in children from the north Indian gangetic plain.

OBJECTIVES

To study the demographic features, etiology, clinical, radiology and laboratory findings in children with Extra hepatic portal vein obstruction (EHPVO) from the North Indian Gangetic Plain.

METHODS

A prospective analysis of 53 patients of EHPVO (<14 y of age) was done. Data for clinical presentation, laboratory workup, endoscopic procedures, growth and development, management and outcome were analyzed.

RESULTS

A total of 53 patients (32 male, 21 female) with mean age of 8.66 ± 3.32 y at presentation were included. Growth retardation was present in 30 (56.60 %) patients at time of diagnosis. The most common presenting symptoms were upper gastrointestinal bleeding (86.80 %) and an incidental finding of splenomegaly (13.2 %). Hematological parameters of hypersplenism were present in 9 (17 %) patients. Protein C (PC), Protein S (PS), antithrombin III (AT) deficiency were found in 8 (20 %), 4 (10 %) and 6 (15 %) of the patients respectively in 40 patients tested. On first endoscopy, esophageal varices were present in all patients. Endoscopic sclerotherapy (EST)/band ligation (EBL) was performed in 46 bleeder patients and success rate was 83.3 % for sclerotherapy and 90 % for band ligation.

CONCLUSIONS

The etiology of EHPVO in the majority of patients remains elusive. It results in impaired somatic growth. Sclerotherapy and endoscopic banding are effective means for treatment for bleeding varices. It is still not clear whether deficiency of anticoagulant proteins is a primary event or secondary to disease process.