Management of pseudo tumour in haemophilia patients

ABSTRACT

A hemophilic pseudotumor is one of the rare complications of hemophilia that results from repetitive bleeding, forming an expanding destructive encapsulated hematoma and necrosed tissue. It has a considerable amount of morbidity. These have become rare over the years with better treatment modalities like factor replacement. Presently, excision is the preferred treatment by many authors. There are instances where surgical excision is not feasible. In such situations, radiotherapy and arterial embolization should be considered either alone or as an adjunct to surgery. A 32 years old male patient, with severe hemophilia A diagnosed with an abdominal tumour 7 years ago during routine screening, that progressively grew to encompass the lower abdominal area. A 44 years old male patient with hemophilia A presented with slow growing swelling over the left gluteal region since, 10 years associated with ulceration and bleeding since, 3 days. The management of a patient with a haemophilic pseudo tumour is complex, with a high rate of potential complications. Surgical excision is the treatment of choice but can only be carried out by a multidisciplinary surgical team. The main postoperative complications are rebleed, infection, fistula and pathological fractures. Pelvic pseudo tumours can even become complicated by fistula formation to the large bowel and by obstruction of the ureters. Untreated pseudo tumours will ultimately destroy soft tissues, erode bone, and may produce neurovascular complications. The hemophilic pseudo tumour is a rare entity which is slow growing painless tumour, with few reports worldwide in the management of this rare complication.