Portal vein thrombosis after splenectomy for hereditary stomatocytosis in childhood

Portal vein thrombosis is a rare but potentially lethal complication in chi ldren requiring splenectomy. We report on a 15-year-old boy with a dehydrat ed hereditary stomatocytosis, who underwent splenectomy and presented a pos toperative partial portal vein thrombosis. With prompt heparin therapy, nei ther propagation of the thrombus nor further cavernous transformation in th e following occurred 6 years. Conclusion Recent data suggest that hereditary stomatocytosis carries a hig h risk of thrombotic complications, especially after splenectomy. This proc edure, the benefit of which is limited in this condition, should therefore be strongly-avoided.