Farzana Aziz, Pervez Ashraf, Ziad Sophie.
Non-Cirrhotic Portal Hypertension in a Young Adult.
J Coll Physicians Surg Pak Nov ;10(8):306-7.

Portal vein thrombosis is a major cause of non-cirrhotic portal hypertension and variceal hemorrhage may be the presenting feature. This is a case report of a 17 years old boy presenting with variceal hemorrhage thrice over a span of 18 months, underwent band ligation and sclerotherapy sessions. Abdominal ultrasound was suggestive of cirrhosis with negative hepatitis serology, liver biopsy showed focal portal fibrosis with intact liver architecture and CT Scan showed portal vein thrombosis but etiology could not be determined. Since the patient presented many years after the onset of portal fibrosis and due to repeated episodes of GI hemorrhage with massive splenomegaly as well as persistent hypersplenism with preserved liver functions, the patient was referred for vascular surgery and proximal splenorenal shunt along with splenectomy was performed which was followed by successful recovery.

CASE REPORT: A 17 years old boy from Interior Sind with no known comorbids presented at the emergency room for the first time in November, 1998 with 4 days history of hemetemesis and melena. He had similar complaints one month before presenting to us and had gastroscopy done at his native town, which showed esophageal varices. There was no history of jaundice, blood transfusions or any other illness except frequent episodes of diarrhea he had during childhood which is a recognised cause of portal vein thrombosis in children.On examination, he was fully oriented, had tachycardia, was hypotensive, appeared anemic and dehydrated. On systemic examination, there was massive splenomegaly and ascites. His investigations showed pancytopenia, normal liver functions and mildly deranged clotting profile. (Hb; 5.4 g/dl, TLC;1.8 X10^9/1 platelet; 37 x10^9, total bilirubin; 0.98, direct; 0.27 SGPT; 16 u/l alkaline phosphatase; 46 a/1 total protein; 6.55 g/dl, albumin; 3.4 g/dl globulin 3.14 g/dl, A/G ; 1.09, PT;16/13, APTT;34/32).Patient underwent emergency endoscopy and band ligation of grade III esophageal varices. His ultrasound of liver was suggestive of cirrhosis with negative serology for hepatitis B and C viruses. He refused liver biopsy and further work up so discharged on symptomatic treatment and did not come for follow up. A year later, he was readmitted with upper GI hemorrhage. His investigations again revealed pancytopenia but liver function tests remained in the normal range. He underwent sclerotherapy for grade -III esophageal varices and was again discharged on request without further work up.

Six months later, in June 1999, he was readmitted for the third time with same complaints and underwent sclerotherapy for grade - III esophageal varices. This time he agreed to have liver biopsy which showed good core of tissue, with `intact lobular architecture and mild focal portal fibrosis.There was no evidence of granuloma, active inflammation, iron deposition or malignancy. CT scan of abdomen showed small thrombi in right and left radicles of portal vein due to recanal isation of portal vein thrombus. Hence the diagnosis of non-cirrhotic portal hypertension was made.The patient was referred for splenectomy and proximal splenorenal shunt due to recurrent esophageal variceal hemorrhage, despite regular sessions of sclerotherapy, and persistent splenomegaly with hypersplenism. Postoperatively,he improved uneventfully. Follow-up endoscopy performeda month after surgery, revealed grade -III esophageal varices whereas gastroscopy on second follow-up visit in December 1999, showed regression of esophageal varices from grade-III to grade -I with significant improvement in the cell count. He is currently symptom-free and enjoying good health.

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