Hepatic rupture in a neonate with hemophilia B: a case report

Hepatic rupture in neonates is extremely rare and often fatal. Its association with hemophilia B has scarcely been reported. A male neonate was born via cesarean section at 39 weeks of gestation with a birth weight of 3.6 kg and Apgar scores of 10 and 10. The prenatal course was without complications and there was no family history of bleeding disorders. At 59 hours of life the patient developed with abrupt abdominal distension, which progressed to hemodynamic shock by 61 hours of life. Bloodwork revealed a hemoglobin level of 4.5 g/dL and severe metabolic acidosis (pH 6.57, lactate 27 mmol/L). A bedside ultrasound combined with abdominal paracentesis confirmed hemoperitoneum. Emergency laparotomy was performed at 63 hours of life. We found a 3-cm fissure on the right hepatic lobe, which was surgically repaired. The patient had a postoperative uneventful recovery and was discharged home in stable condition 2 weeks later. Coagulation function assays confirmed factor 9 activity of 2%, confirming the diagnosis of hemophilia B. At 6-month of follow-up he has had no recurrent bleeding episodes and remains clinically stable on regular factor replacement therapy. Neonatal hepatic rupture may be the initial manifestation of an underlying bleeding disorder such as hemophilia B. Neonates with spontaneous hepatic rupture should undergo testing to rule out coagulation disorders.