Giant haemophilic pseudotumour of the pelvis: case report and literature review

Haemophilia A is an inherited disorder caused by a deficiency in factor VIII (FVIII) that results in spontaneous and trauma-related bleeding. Pseudotumours, encapsulations of blood that can develop following repeated haematomas in bone or soft tissue, are a rare, severe complication of haemophilia [1]. Multiple case reports have described the management of large haemophilic pseudotumours (e.g. pseudotumours of the abdomen, chest, pelvis, gluteus maximus, leg and iliopsoas) in patients with haemophilia A or B. We describe the successful surgical resection of two giant haemophilic pseudotumours destroying both iliac wings and reconstruction of the pelvis by compound osteosynthesis in a patient with severe haemophilia A. A 37-year-old white male with severe haemophilia A started treatment for haemophilia at age 4 years and had received on-demand FVIII therapy for most of his life. The patient’s medical history included haemophilic arthropathy, liver cirrhosis, splenomegaly, HIV infection, chronic hepatitis C and elevated lactate dehydrogenase levels. The patient underwent three major surgeries; written informed consent was provided for publication of this case report and accompanying images. In 2004, when the patient was aged 30 years, pelvic pseudotumours associated with complete destruction of the right ilium and partial destruction of the left ilium were diagnosed (Fig. 1a). Both pseudotumours likely originated from inadequately treated iliopsoas muscle bleeding episodes. In 2011, a large pseudotumour (~75-cm circumference) in the right pelvic area with retroperitoneal spread reaching the diaphragm and a smaller pseudotumour (~60-cm circumference) on the left side with retroperitoneal extension to the pelvic vessels, were observed (Fig. 1b). Both pseudotumours were septated; the right pseudotumour consisted of four sections (a basketball-sized lateral part, a handball-sized cranial part, a medial part and a small caudal part), and the left pseudotumour consisted of two sections (a cranial/abdominal part and a part extending through the inguinal canal into the thigh muscles). A preoperative photograph of the patient’s abdomen is shown (Fig. 1c). On admission to the hospital, the patient required a wheelchair because of severe right pelvic pain. He experienced dyspnoea at rest owing to compression of the right lung by the pseudotumour and pronounced venous collateral circulation of the liver (caput medusae). Concomitant medications taken before the first surgery included lopinavir/ritonavir (Kaletra ; Abbott Laboratories, North Chicago, IL, USA; initiated in 2006), abacavir sulphate/lamivudine (Kivexa ; GlaxoSmithKline, Research Triangle Park, NC, USA; initiated in 2006) and plasma-derived FVIII (Octanate ; Octapharma AG, Lachen, Switzerland; initiated in 2009). One day before surgery, the right-side pseudotumour was embolized. The first surgery was performed in August 2011 via an extended ilioinguinal approach. Thin abdominal muscles attached to the former iliac crest, lateral attachments of the gluteal fascia and the gluteus medius muscle were separated. Because of its large size, the pseudotumour was difficult to separate from adjacent abdominal structures, and the mass was decompressed to improve exposure and avoid injuring abdominal organs and vessels. The right-side pseudotumour was opened, and several litres of browngreyish liquid were emptied. Remnants of an ossified haematoma were found within the pseudotumour capsule. Intralesional resection of the pseudotumour was performed in the pelvis and abdomen. The right iliac crest was custom-fit with a 20-hole reconstruction plate, which was attached to the remnants of the posterior superior iliac spine and the anterior acetabulum with screws and 3.0-mm K-wires. Dead space between the wires and plate was filled with bone cement. An initial dose of 4250 IU recombinant FVIII (rFVIII) was used before surgery; total rFVIII dose on the day of surgery was 12 000 IU. Total blood loss during surgery was 1000 mL, but no haemostasisrelated complications occurred. Duration of surgery was 6 h and 56 min. Four units each of red blood cells and fresh frozen plasma concentrate were Correspondenec: Peter H. Pennekamp, Department of Orthopedics and Trauma Surgery, University of Bonn, Sigmund-FreudStr. 25, 53127 Bonn, Germany. Tel.: +49 (0)228 287 15328; fax: +49 (0)228 287 14175; e-mail: peter.pennekamp@ukb.uni-bonn.de