부산대학교 도서관

Introduction: Wolf-Hirschhorn syndrome (WHS) is a genetic disorder from a chromosomal deletion on chromosome 4, leading to systemic pathologies including spinal deformities like scoliosis. Aim: The objective of this publication is to present a case of scoliosis treatment in a patient with WHS, where the primary goal was to halt the progression of the curvature and achieve the most effective correction of the deformity, despite the guardians' refusal to extend the stabilization system to the pelvis. Case study: Preoperative radiographs revealed a single-curve scoliosis of 64°, as measured by the Cobb method between Th11 and L5. Prior to the scheduled procedure, a magnetic resonance imaging examination of the spinal column was conducted to potentially visualize any spinal cord pathology. A corrective procedure, posterior spinal fusion, was performed using cobalt-chrome rods. A postoperative posturographic control examination was conducted, and follow-up outpatient visits were recommended. Results and discussion: Despite not implementing the stabilization system to the pelvis, a reduction in deformation by 22° was achieved, resulting in 42° measured between Th11 and L5. In a follow-up examination 18 months post-operation, the degree of deformity remained stable, maintaining the curvature angle of 42° measured between Th11 and L5. Conclusions: Treating scoliosis in patients with WHS requires a tailored approach, considering the specifics of their condition and utilizing optimal spinal stabilization methods. A multidisciplinary approach, integrating various therapeutic modalities, is crucial for enhancing the quality of life of WHS patients. [ABSTRACT FROM AUTHOR]