Massive retroperitoneal pseudotumour in a patient with type 3 von Willebrand disease.
Formation of destructive haemorrhagic pseudocysts or pseudotumours thought to arise from unresolved, encapsulated haematomas is a well-recognized, rare complication of severe haemophilia A or B, and has been reported in a single patient with von Willebrand disease (vWD). We report a 41-year-old patient with type 3 vWD who underwent incomplete resection of a large retroperitoneal pseudocyst in 1995 and presented with a recurrent, extensive right abdominal and flank mass and signs and symptoms of large bowel obstruction. He required emergency partial colectomy for bowel ischaemia and removal of his right kidney, which was hydronephrotic due to prolonged ureteral obstruction by the pseudocyst. Following repeat partial resection of the pseudotumour, he developed persistent bleeding into the operative site despite aggressive administration of von Willebrand factor (vWF)-rich factor VIII concentrates, resulting in retroperitoneal haematomas and abscesses, which resolved after 13 months of percutaneous drainage, extended supplementation of vWF and antibiotic therapy.
Eby
C S
CS
Department of Pathology and Department of Internal Medicine, Center for Bleeding and Thrombotic Disorders, Saint Louis University School of Medicine, MO 63110, USA. eby@labmed.wustl.edu
Caracioni
A A
AA
Badar
S
S
Joist
J H
JH
D000328:Adult; D005500:Follow-Up Studies; D006406:Hematoma / Q000209:etiology; D006801:Humans; D007415:Intestinal Obstruction / Q000209:etiology; D008297:Male; D012008:Recurrence; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000209:etiology*; D014842:von Willebrand Diseases / Q000150:complications*