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Chinese Scholars Propose Pelvic Hemophilic Pseudotumor Classification and Surgical Strategies for the First Time

ZhangSaiWei Thu, Mar 21 2024 11:12 AM EST

Recently, a team led by Professor Xi'an Weng and Professor Bin Feng, chief physicians of orthopedics at Peking Union Medical College Hospital, and a team led by Professor Bin Chen, chief physician of the Affiliated Southern Hospital of Southern Medical University, published an original paper in Lancet's sub-journal eClinicalMedicine. This study reports on the epidemiological characteristics, pseudotumor characteristics, surgical data, and midterm follow-up results of pelvic hemophilic pseudotumor in China. Combining the team’s years of experience, the paper also, for the first time internationally, proposes a classification and surgical strategy for pelvic hemophilic pseudotumor, providing important references for surgical treatment and perioperative management for global peers. 65fa8ae2e4b03b5da6d0b82c.png Pelvic Hemophilic Pseudotumors: A Comprehensive Analysis of 21 Cases

Hemophilic pseudotumors (HPTs) are rare and severe complications in hemophiliacs, affecting only 1–2% of patients. They are encapsulated cysts in the musculoskeletal system outside joints, resulting from recurrent bleeding in hemophiliacs. HPTs can lead to life-threatening bleeding, bone erosion, and compression of adjacent organs and neurovascular structures. Current treatment options include local aspiration, radiotherapy, interventional embolization, and surgical excision, with surgery considered the optimal approach.

Pelvic hemophilic pseudotumors (PHPTs) arise from the pelvis, iliopsoas muscle, and retroperitoneum. PHPTs are extremely rare and difficult to surgically excise, resulting in limited reports on outcomes after PHPT surgery. There is currently no consensus on the optimal treatment strategy for PHPT, and large-scale follow-up studies are lacking.

This study included 21 patients with primary PHPT and a mean follow-up of 7.1 years (maximum 19.3 years). Five patients experienced PHPT recurrence after surgery, and three underwent a second operation. Based on the pseudotumor characteristics and required surgical approach, the research team proposed a classification system and corresponding surgical strategies for PHPT:

  1. PHPTs confined to soft tissue: Excision of PHPT + Hemostatic material filling + Primary closure.
  2. PHPTs involving the bony pelvis but not causing pelvic discontinuity: Excision of PHPT + Debridement of pelvic lesions + Routine drainage + Hemostatic material filling + Primary closure.
  3. PHPTs causing pelvic discontinuity: Excision of PHPT + Debridement of pelvic lesions + Pelvic reconstruction + Routine drainage + Hemostatic material filling + Primary closure.
  4. Infected PHPTs: Excision of PHPT + Debridement + Antibiotic-loaded bone cement filling + Vacuum-sealed drainage + Secondary closure.

The orthopedics team at Peking Union Hospital has a long-standing focus on surgical treatment of hemophilic pseudotumors. This study extends their previous findings, demonstrating that surgical treatment of PHPT is feasible and relatively safe. The research team recommends that PHPT patients with relevant symptoms or progressive pseudotumor enlargement undergo surgical intervention as soon as possible. Surgeons should be cautious about using excessive gelatin sponges during PHPT surgery due to the risk of severe embolic complications.

Reference: https://doi.org/10.1016/j.eclinm.2024.102497