Managing massive gastrointestinal and abdominal haemorrhage in inherited bleeding disorders: experience from a...

Massive gastrointestinal (GI), intraperitoneal, and pelvic hemorrhage in inherited bleeding disorders (IBDs) is rare but potentially life-threatening. Pediatric data remain limited.

We retrospectively reviewed patients ≤ 18 years with hemophilia A/B, von Willebrand disease (VWD), or rare bleeding disorders admitted with massive abdominal hemorrhage (September 2017-August 2025). Massive GI bleeding was defined as overt bleeding with estimated loss > 70 mL/kg/day or bleeding resulting in shock or transfusion. Intraperitoneal and pelvic hemorrhage required imaging confirmation. Demographics, interventions, and outcomes were analyzed and compared with the pediatric IBD cohort.

Of 788 pediatric IBD patients, 10 (1.2%) developed massive abdominal hemorrhage: GI (n = 5), intraperitoneal (n = 3), and pelvic hematoma (n = 2). The GI bleeding cohort was older than the pediatric IBD cohort (median 16 vs. 8 years, p < 0.001). Diagnoses included hemophilia A (n = 4; 75% inhibitor-positive), hemophilia B (n = 1), VWD (n = 4), and Glanzmann thrombasthenia (n = 1). Seven required transfusions; four met massive transfusion criteria. Endoscopy identified a bleeding source in 80% of GI bleeds. Diagnostic delays were longer for intraperitoneal hemorrhage than for GI bleeds (p < 0.05).

Massive abdominal haemorrhage causes significant morbidity. Early imaging, endoscopy, and aggressive hemostatic therapy resulted in 100% survival. Improved access to prophylaxis may prevent such events.