PB1253 - Uncommon orthopedic complications in haemophilia

Tuesday, June 25, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

CU

Cristina Emilia Ursu, MD, PhD

MD, PhD
Romanian Academy of Medical Sciences, Onco-Hematology Research Unit, Children Emergency Hospital "Louis Turcanu”, European Haemophilia Treatment Center, Timisoara, Romania
Timisoara, Timis, Romania

Co-Author(s)

MS

Margit Serban

Professor Doctor
Romanian Academy of Medical Sciences, Onco-Hematology Research Unit, Children Emergency Hospital "Louis Turcanu”, European Haemophilia Treatment Center, Timisoara, Romania
Timisoara, Timis, Romania
JP

Jenel Marian Patrascu

Professor Doctor
Department of Orthopaedics, “ Victor Babes ” University of Medicine and Pharmacy Timisoara, Romania
Timisoara, Timis, Romania
CV

Costel Vlad

MD, PhD, Medical Director
Paediatric Orthopaedic Department, Children Hospital "Dr. Victor Gomoiu", Bucharest, Romania
Bucharest, Bucuresti, Romania
VU

Valentina Uscatescu

Professor Doctor
Hemostasis Laboratory, Fundeni Clinical Institute Bucharest, Romania
Bucharest, Bucuresti, Romania
AT

Adina Traila

MD
Medical Centre for Education and Rehabilitation of Children and Young Adults, European Hemophilia Treatment Centre, Buzias, Romania
Buzias, Timis, Romania
II

Ioana Ionita

MD, PhD, Associate Professor
Department of Hematology, “Victor Babes” University of Medicine and Pharmacy Timisoara, 300041 Timisoara, Romania
Timisoara, Timis, Romania
IV

Ines Vaide

MD
Institute of Clinical Medicine, University of Tartu, Estonia
Tartu, Tartumaa, Estonia
TA

Teodora Smaranda Arghirescu

MD, PhD, Associate Professor
Department of Pediatrics, Division of Onco-Hematology, “ Victor Babes ” University of Medicine and Pharmacy Timisoara, Romania
Timisoara, Timis, Romania

Background: Avascular bone necrosis (AVN) is a rare uncommon pathological event in haemophilia. Its silent onset and evolution are challenging and responsible for delayed diagnosis.

Aims: We would like to present two such cases and bring AVN to our attention.

Methods: Case presentations.

Results: Case 1. A 21-year-old male patient, with severe haemophilia B, was treated on-demand with factor IX (FIX) concentrates until the age of 14 when he accessed continuous prophylactic substitution. During its clinical course, he developed bleeding episodes and target joints (right knee, ankles). He was referred to us for persistent claudication, pain, and limited motion of the left tibiotalar joint, without swelling. The Magnetic Resonance Imaging (MRI) revealed a talus necrosis, with heterogeneous STIR hypersignal, T1 hyposignal that included almost the entire bone, and similar changes were visualized partially in the calcaneus. The patient stopped demanding activities and attended regular physiotherapy and hyperbaric oxygen therapy, and eventually, the outcome became favorable. Case 2. A 7-year-old boy, with moderate haemophilia B, treated only on-demand, after two years of bleeding-free time, was addressed for a spontaneous limp, right hip joint pain, and limited movement. Ultrasonography excluded haemarthrosis or soft tissue bleeding. Native pelvic MRI confirmed the idiopathic AVN of the right femoral head and incomplete subchondral linear fracture. After 11 months of continuous FIX prophylaxis, immobilization, and joint containment, the MRI examination showed unfavorable evolution, with more than 50% impairment of the femoral epiphysis. Right proximal femoral varus osteotomy with a triple pelvic osteotomy for femoral head coverage and containment was performed under rigorous hemostatic control. After long-term monitoring, a rehabilitation program, and prophylactic replacement, the patient gains complete gait recovery.

Conclusion(s): Timely diagnosis and therapeutic approach are decisive for patients with haemophilia and AVN. A precocious diagnosis and appropriate treatment will help to avoid locomotor disabilities in adults and early arthroplasty.