重点手术病例报告:沃尔夫-赫希霍恩综合征脊柱侧凸的治疗

Q4 Medicine Polish Annals of Medicine Pub Date : 2024-02-27 DOI:10.29089/paom/177551
Artur Fabijan, B. Polis, Agnieszka Zawadzka-Fabijan, Michal Kolejwa, Kacper Wróbel, J. Kujawa, Remigiusz Kozłowski, Michał Marczak, Krzysztof Zakrzewski, E. Nowosławska
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摘要

沃尔夫-赫希霍恩综合征(Wolf-Hirschhorn Syndrome,WHS)是一种遗传性疾病,由4号染色体上的染色体缺失引起,可导致包括脊柱侧弯等脊柱畸形在内的全身性病变。尽管监护人拒绝将稳定系统延伸至骨盆,但治疗的首要目标是阻止脊柱侧弯的发展,并对畸形进行最有效的矫正。术前X光片显示,Th11和L5之间采用Cobb法测量的单曲线脊柱侧弯为64°。在预定手术前,医生对脊柱进行了磁共振成像检查,以确定脊髓是否存在病变。使用钴铬合金棒进行了脊柱后路融合术。尽管没有在骨盆上安装稳定系统,但变形还是减少了 22°,Th11 和 L5 之间的测量值为 42°。在术后18个月的随访检查中,畸形程度保持稳定,在Th11和L5之间测量的弯曲角度维持在42°。综合各种治疗方法的多学科方法对于提高 WHS 患者的生活质量至关重要。
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A focused surgical case report: Managing scoliosis in Wolf–Hirschhorn syndrome
Wolf–Hirschhorn syndrome (WHS) is a genetic disorder from a chromosomal deletion on chromosome 4, leading to systemic pathologies including spinal deformities like scoliosis.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.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.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.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.
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来源期刊
Polish Annals of Medicine
Polish Annals of Medicine Medicine-Medicine (all)
CiteScore
0.40
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发文量
28
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