An 8-year-old girl presented with a history of pain in the right hypocondrium, multiple petechiae in the skin, and ecchimoses at sites of minor trauma. Laboratory investigations showed severe thrombocytopenia. Doppler ultrasonography and magnetic resonance imaging showed portal and splenic vein cavernomatous transformation and splenomegaly. The patient underwent laparoscopic subtotal splenectomy with lower pole preservation and esophagogastric devascularization. The postoperative course was uneventful. No gastrointestinal bleeding occurred within the first 34 months after surgery.
Conclusions: Thrombocytopenia associated with splenomegaly is a rare form of presentation in portal cavernoma. Preserving the spleen immune function must be a goal in surgical management, especially in children. Laparoscopic subtotal splenectomy combined with esophagogastric devascularization is a difficult procedure, but it can be useful in patients with portal cavernoma and severe thrombocytopenia without gastrointestinal bleeding.