Treatment of pelvic haemophilic pseudotumour: A retrospective study

Abstract

A pseudotumour is a very rare but serious complication of haemophilia. The prevalence of a pseudotumour in severe haemophilia is 1%-2% but can be up to 10% in haemophilic patients with coagulation inhibitors.. 1 Pelvic haemophilic pseudotumour is essentially chronic fibroproliferative inflammation of the pelvic and abdominal cavity. It initially manifests in soft tissue and then gradually erodes bone tissue. Pelvic haemophilic pseudotumour is characterized by multiple arterial supplies, is expansive, painless and adheres to deep tissues, resulting in poor mobility. Spontaneous rupture, infections, pathological fractures or fatal bleeding may occur as the lesions progress. 2 Treatment of a pelvic pseudotumour depends on its location, size, growth rate and impact on surrounding tissues. Current treatments

A Novel Surgical Classification for Extremity and Pelvic Hemophilic Pseudotumors

“…Therefore, pelvic MRI or CT urography is recommended to clarify the anatomical relationship between the pseudotumor and the visceral organs, nerves, and vessels. Reconstruction with a pedicle screw and rod system or plates combined with Kirschner wires or bone cement can be used for an HPT in the ilium with pelvic discontinuity 30 . When the HPT was located in the retroperitoneal region, it should be excised with preservation of the capsule to reduce the risk of injury to the surrounding viscera 7,16,31 .…”

Giant pelvic haemophilic pseudotumor

“…Pseudotumor, a rare and serious complication of haemophilia, arises from repeated episodes of bleeding within the musculoskeletal system 1 . Pelvic haemophilic pseudotumor poses a risk of spontaneous rupture, infections, pathological fractures, or fatal haemorrhage due to the abundant arterial supply, strong expansile growth, and adherence to deep tissues 2 . The main treatment is the replacement of clotting factor concentrates to control bleeding and prevent further growth of the pseudotumor, and surgery may be necessary to remove the pseudotumor and repair any damage to the surrounding tissues 3 …”

Absence of femur induced by haemophilic pseudotumour

“…2 The rupture of haemophilic pseudotumour creates cutaneous openings that serve as pathways for pathogens to enter the body, thereby increasing the risk of infection and potentially posing a life-threatening condition. 3…”

“…Prolonged presence of a pseudotumour, along with the accompanying inflammatory response, leads to the destruction and dissolution of surrounding bone tissues, particularly in weight‐bearing joints and vulnerable areas 2 . The rupture of haemophilic pseudotumour creates cutaneous openings that serve as pathways for pathogens to enter the body, thereby increasing the risk of infection and potentially posing a life‐threatening condition 3 …”