Successful emergency resection of a massiveintra-abdominal hemophilic pseudotumor

An intra-abdominal pseudotumor is a rare complicationof hemophilia. Surgical treatment is associated withhigh morbidity and mortality rates and reported casesare scarce. We present a 66-year-old Caucasian malesuffering from severe hemophilia type A treated for10 years with Factor Ⅷ. Major complications fromthe disease were chronic hepatitis B and C, cerebralhemorrhage and disabling arthropathy. Twenty-threeyears ago, retro-peritoneal bleeding led to the developmentof a large intra-abdominal pseudotumor, whichwas followed-up clinically due to the high surgical riskand the lack of clinical indication. The patient presentedto the emergency department with severe sepsis andumbilical discharge that had appeared over the past twodays. Abdominal computed tomography images werehighly suggestive of a bowel fistula. The patient wastaken to the operating room under continuous infusion offactor Ⅷ. Surgical exploration revealed a large infectedpseudotumor with severe intra-abdominal adhesionsand a left colonic fistula. The pseudotumor was partiallyresected en bloc with the left colon leaving the posteriorwall intact. The postoperative period was complicatedby septic shock and a small bowel fistula that requiredreoperation. He was discharged on the 73rd hospital dayand is well 8 mo after surgery. No bleeding complicationswere encountered and we consider surgery safe underfactor Ⅷ replacement therapy.