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World J Gastrointest Surg. 2015 Mar 27;7(3):43-6. doi: 10.4240/wjgs.v7.i3.43.

Successful emergency resection of a massive intra-abdominal hemophilic pseudotumor.

World journal of gastrointestinal surgery

Julie Frezin, Lancelot Marique, Laurent Coubeau, Catherine Hubert, Catherine Lambert, Cédric Hermans, Nicolas Jabbour

Affiliations

  1. Julie Frezin, Lancelot Marique, Laurent Coubeau, Catherine Hubert, Nicolas Jabbour, Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, 1200 Brussels, Belgium.

PMID: 25848492 PMCID: PMC4381156 DOI: 10.4240/wjgs.v7.i3.43

Abstract

An intra-abdominal pseudotumor is a rare complication of hemophilia. Surgical treatment is associated with high morbidity and mortality rates and reported cases are scarce. We present a 66-year-old Caucasian male suffering from severe hemophilia type A treated for 10 years with Factor VIII. Major complications from the disease were chronic hepatitis B and C, cerebral hemorrhage and disabling arthropathy. Twenty-three years ago, retro-peritoneal bleeding led to the development of a large intra-abdominal pseudotumor, which was followed-up clinically due to the high surgical risk and the lack of clinical indication. The patient presented to the emergency department with severe sepsis and umbilical discharge that had appeared over the past two days. Abdominal computed tomography images were highly suggestive of a bowel fistula. The patient was taken to the operating room under continuous infusion of factor VIII. Surgical exploration revealed a large infected pseudotumor with severe intra-abdominal adhesions and a left colonic fistula. The pseudotumor was partially resected en bloc with the left colon leaving the posterior wall intact. The postoperative period was complicated by septic shock and a small bowel fistula that required reoperation. He was discharged on the 73(rd) hospital day and is well 8 mo after surgery. No bleeding complications were encountered and we consider surgery safe under factor VIII replacement therapy.

Keywords: Colonic fistula; Factor VIII replacement therapy; Hemophilia A; Hemophilic pseudotumor; Surgery in hemophilic patient

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