CASE REPORT
A focused surgical case report: Managing scoliosis in Wolf–Hirschhorn syndrome
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1
Department of Neurosurgery, Polish-Mother’s Memorial Hospital Research Institute, Lodz, Poland
2
Department of Medical Rehabilitation, Faculty of Health Sciences, Medical University of Lodz, Poland
3
Department of Gastroenterology, Allergology and Paediatrics, Polish-Mother’s Memorial Hospital Research Institute, Lodz, Poland
4
Department of Management and Logistics in Healthcare, Medical University of Lodz, Poland
5
Collegium of Management, WSB Merito University of Warsaw, Poland
These authors had equal contribution to this work
Submission date: 2023-10-19
Final revision date: 2023-12-22
Acceptance date: 2023-12-22
Online publication date: 2024-02-27
Pol. Ann. Med. 2024;31(1):34-40
KEYWORDS
TOPICS
ABSTRACT
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.
ACKNOWLEDGEMENTS
Many thanks to Agnieszka Strzała for the linguistic proofreading and Robert Fabijan for help in preparing the figures.
FUNDING
This research was supported by the project ‘InterDoktorMen—Building new quality and effectiveness of education in the formula of doctoral studies for healthcare managers at the Health Sciences Faculty at the Medical University in Lodz’ (POWR.03.02.00-00-1027/16-00) and co-financed by the European Union: resources from the European Social Fund as a part of the Operational Pro-gramme Knowledge Education Development.
CONFLICT OF INTEREST
The authors declare no conflict of interest.
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