EXTRAHEPATIC PORTAL VEIN OBSTRUCTION IN CHILDREN: A CASE REPORT.

Background: Extrahepatic portal vein obstruction (EHPVO), although rare in children, is one of the most important causes of noncirrhotic portal hypertension (NCPH) among children. The incidence of EHPVO is estimated to be 0,72/million. The initial clinical manifestation can be represented by upper gastrointestinal bleeding, splenomegaly or ascites. In children, identified risk factors of EHPVO are neonatal vein catheterization, umbilical cord infections, blood transfusions, sepsis, dehydration and coagulation abnormalities. Objective: To present the case of a 9 years old female patient who was hospitalized in a regional hospital with abdominal pain and diarrhea. Two days later she was transferred to our hospital with the suspicion of a malignant hematologic disorder due to important splenomegaly. Material and methods: At the admission in our hospital the clinical exam revealed ponderal hypotrophy and splenomegaly. The patient history determined: small for gestational age, acute fetal distress, perinatal asphyxia, neonatal sepsis, resuscitated cardiac arrest at 2 days of life, cerebral hemorrhage and umbilical vein catheterization. Laboratory findings revealed signs of mild microcytic normochromic anemia associated with thrombocytopenia and leukopenia. The abdominal ultrasonography and color Doppler showed portal vein cavernous transformation, important splenomegaly (123mm), accessory spleen and normal hepatic parenchyma. The upper endoscopy determined 1st grade esophageal varices. The investigations for thrombophilia revealed deficiency of Protein C. Results: The diagnosis of EHPVO was based on splenomegaly, thrombocytopenia due to hypersplenism and the portal cavernoma. Sepsis and umbilical vein catheterization in neonatal period associated with deficiency of protein C were the risk factors for EHPVO in our patient. Conclusions: EHPVO represents a rare disorder in children having similarities with the malignant hemopathies. NCPH represents an uncommon cause of portal hypertension in children and the main cause is represented by EHPVO. The major risk factor is represented by the umbilical vein catheterization, therefore close follow up should be conducted in these cases. [ABSTRACT FROM AUTHOR]