Congenital afibrinogenemia and splenic rupture: A case report

Congenital afibrinogenemia is an extremely rare coagulation disorder affecting 1-2 individuals per million. We report the case of a 15-year-old male with known congenital afibrinogenemia who developed hemorrhagic shock following minor soccer ball trauma to the abdomen. Clinical examination revealed hemodynamic instability with hypotension (90/40 mmHg) and tachycardia (140 bpm), associated with abdominal defense. Laboratory tests showed severe anemia (Hemoglobin 7 g/dL), undetectable fibrinogen (<0.2 g/L), and prolonged coagulation times. Abdominal computed tomography revealed massive hemoperitoneum with splenic involvement and a hematoma of the greater gastric curvature. Emergency exploratory laparotomy with gastrosplenic disconnection and surgical hemostasis was performed. The patient received blood transfusions, fresh frozen plasma, and 9 grams of fibrinogen concentrate, with full recovery and discharge on day 7. Splenic rupture occurs disproportionately in afibrinogenemia patients despite the rarity of the condition. Management requires prompt diagnosis, fibrinogen replacement therapy, and surgical intervention when indicated, along with preventive education regarding high-risk activities.