摘要
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.
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.
