P033
La technique de Masquelet pourrait-elle être une bonne option thérapeutique pour la pseudarthrose tibiale congénitale chez les patients neurofibromateux ? A propos d'un cas.
Benjamin
Dubois
(Saint-Etienne, France),
Martin
Bail
(Saint-Etienne, France),
Béatrice
Bayle
(Saint-Etienne, France),
Hassan
Al-Khoury Salem
(Saint-Etienne, France),
Bérénice
Zagorda
(Saint-Etienne, France),
Vincent
Gautheron
(Saint-Etienne, France),
Hugo
Bessaguet
(Saint-Etienne, France)
Objective :
Congenital tibial pseudarthrosis (CTP) is a common affection in patients with neurofibromatosis type 1 (NF1). This situation is a therapeutic challenge in terms of consolidation and correction of axial defects, which are the guarantees of a good functional result. As such, Masquelet technique may represent an interesting surgical strategy to reconstruct the missing bone. The objectives of this paediatric case report are to show functional risks and benefits we encountered during the management.
Material / Patients and Methods :
We report a case of a 7 years-old male, presenting a CTP due to NF1. He underwent a 1st surgical stage of Masquelet technique in March 2020, and a 2nd stage 2 months later. First radiological control 6 month later showed the persistence of CTP, requirring multiple bones grafting operations. Due to multiple complications,he was finally admitted in PRM department 2 years later for functionnal evaluation.
Results :
Clinically he presented with left lower limb muscular stiffness and distal muscular weakness. We observed an associated painful limp walking. As bone healing was not effective, he still required tibial external fixator. Moreover, he had not gone to school for 2 years, had triplicate his class (primary education) due to absenteism. He suffered from social withdrawal.
Discussion - Conclusion :
This case challenges the surgical strategies and complications that can arise in the management of PTC. Indeed, the iterative surgeries and relapses had a significant impact on the psychosocial development of this young child. With his future likely to be oriented toward amputation, should the physicians have decided on an amputation plan earlier?
Keywords :
Congenital Tibial Pseudarthrosis, NF1, Masquelet technique, Amputation