Acute intestinal obstruction due to intramural haemorrhage in small intestine in a patient with severe haemophilia A and inhibitor.
Patients with severe haemophilia A usually present with joint, gastrointestinal and urinary tract haemorrhage. Bleeding elsewhere is often precipitated by pre-existing pathology or trauma. We report a patient with severe haemophilia A, who presented with symptoms of acute intestinal obstruction. He has a factor VIII inhibitor and receives recombinant factor VIIa on demand at home. The CT scan of abdomen showed dilated small intestine with fluid filled loops and a long segment in the jejunum with marked transmural thickening. There was no other pathology in the small intestine. These appearances were consistent with intramural haemorrhage in the small intestine as the cause of acute obstruction. He was managed conservatively with recombinant factor VIIa and this resulted in resolution of his symptoms. This case highlights an unusual presentation of bleeding in a haemophilia patient. Intestinal obstruction due to haemorrhage in the small intestinal wall is extremely rare and only previously reported in a few haemophilia patients. It also highlights the effectiveness of conservative management with recombinant factor VIIa as opposed to immediate exploratory surgery.
Haemophilia Centre in Northern Ireland, Belfast City Hospital, Belfast, UK. kramadan4@email.com
Khaled M A
KM
Ramadan
John P
JP
Lowry
Alan
A
Wilkinson
Orla
O
McNulty
Mary F
MF
McMullin
Francis G C
FG
Jones
D000208:Acute Disease; D000328:Adult; D000906:Antibodies / Q000097:blood; D005169:Factor VIII / Q000276:immunology; D015942:Factor VIIa / Q000627:therapeutic use; D006471:Gastrointestinal Hemorrhage / Q000150:complications* / Q000209:etiology; D006467:Hemophilia A / Q000150:complications* / Q000276:immunology; D006801:Humans; D007410:Intestinal Diseases / Q000150:complications / Q000209:etiology; D007415:Intestinal Obstruction / Q000209:etiology*; D007421:Intestine, Small; D008297:Male
D000906:Antibodies; D005169:Factor VIII; D015942:Factor VIIa