Purpose: This report describes life-threatening spontaneous splenic rupture in two brothers with congenital afibrinogenemia.
Patients: Two brothers, aged 11 and 14 years, had intra-abdominal bleeding due to splenic rupture confirmed by ECHO ultrasonography and computed tomography scans.
Results: Splenectomy was performed after administration of heat- and solvent-detergent treated fibrinogen concentrates.
Conclusions: Hemostatic treatment for splenic rupture using heat- and solvent detergent-treated fibrinogen concentrates was effective. Careful attention must be paid to the risk of splenic rupture during the growth spurt in physically active children with this rare coagulation disorder.