Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Mar 27, 2015; 7(3): 43-46
Published online Mar 27, 2015. doi: 10.4240/wjgs.v7.i3.43
Successful emergency resection of a massive intra-abdominal hemophilic pseudotumor
Julie Frezin, Lancelot Marique, Laurent Coubeau, Catherine Hubert, Catherine Lambert, Cédric Hermans, Nicolas Jabbour
Julie Frezin, Lancelot Marique, Laurent Coubeau, Catherine Hubert, Nicolas Jabbour, Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, 1200 Brussels, Belgium
Catherine Lambert, Cédric Hermans, Department of Hematology, Cliniques Universitaires Saint-Luc, 1200 Brussels, Belgium
Author contributions: Coubeau L, Hubert C and Frezin J performed the surgery; Frezin J and Marique L reviewed current literature and wrote the paper; Lambert C, Hermans C and Jabbour N coordinated the paper elaboration and revised the article.
Ethics approval: The study was reviewed and approved by the Institutional Review Board of the Université catholique de Louvain.
Informed consent: The patient gave his verbal consent for reporting this case.
Conflict-of-interest: None.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Julie Frezin, MD, Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Avenue Hippocrate, 10, 1200 Brussels, Belgium. juliefrezin@gmail.com
Telephone: +32-2-7641401
Received: October 19, 2014
Peer-review started: October 21, 2014
First decision: December 17, 2014
Revised: January 16, 2015
Accepted: February 9, 2015
Article in press: February 11, 2015
Published online: March 27, 2015
Processing time: 162 Days and 21.3 Hours
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 73rd 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: Hemophilia A; Hemophilic pseudotumor; Colonic fistula; Factor VIII replacement therapy; Surgery in hemophilic patient

Core tip: We present a patient suffering from hemophilia A complicated by a large intra-abdominal hemophilic pseudotumor. This condition is rare and there is no consensus for treatment. Emergency resection was required because of bowel complications and septic shock. Based on our experience, we recommend elective surgery prior to complications under appropriate factor VIII replacement therapy.