Background/purpose: A retrospective study was performed to evaluate risk factors, clinical features, and treatment modalities of portal vein thrombosis (PVT) after splenectomy in pediatric hematologic disease.

Methods: Sixty-eight patients who underwent splenectomy for various hematologic diseases were evaluated with regard to age, sex, blood count, and splenic mass. Patients who developed PVT were also reviewed for clinical features, treatment modalities, and outcome.

Results: Patients with PVT (n = 4, 5.88%) and without PVT (n = 64, 94.2%) had a mean age and female/male ratio of 13.2 years (range, 10-16 years) and 4:0, and 10.2 years (range, 1-16 years) and 29:35, respectively. Postoperative thrombocyte levels and splenic mass with and without PVT was 804 x 10(3)/mm(3) and 752.5 g, and 465.2 x 10(3)/mm(3) and 441g, respectively. Three patients with PVT presented with abdominal pain, fever, and vomiting. The diagnosis of PVT was made by Doppler ultrasonography in all patients including the asymptomatic case. Protein C, protein S, and antithrombin III levels were mostly decreased and/or normal and di-dimer levels were increased and/or normal after the development of PVT. Antiplatelet (acetylsalicylic acid) and antithrombotic therapy (low molecular weight heparin) were treatment agents. None of the patients needed surgery. During a mean follow-up period of 55.5 months, by Doppler ultrasonography, 1 patient was found to be free of thrombosis, whereas 1 had partial thrombosis. Two patients developed cavernomatous transformation leading to portal hypertension.

Conclusions: Portal vein thrombosis is a rare but significant complication of splenectomy done for hematologic diseases. According to our results, female gender and decreased levels of coagulation inhibitors seem to be risk factors in addition to previously mentioned thrombocytosis and greater splenic mass. Doppler ultrasonography may be performed in all patients after splenectomy to screen PVT. In the presence of well-known risk factors, prophylactic antiplatelet and antithrombotic therapy should be considered after splenectomy.