Treatment of Severe Kyphoscoliosis in Children with Mucopolysaccharidosis Type I (Pfaundler-Hurler Syndrome) Using the Growing Rod Technique: A Case Series with Mid-Term Results.
Growing rod technique
Kyphoscoliosis
Kyphosis
Mucopolysaccharidoses
Scoliosis
Journal
World neurosurgery
ISSN: 1878-8769
Titre abrégé: World Neurosurg
Pays: United States
ID NLM: 101528275
Informations de publication
Date de publication:
07 2020
07 2020
Historique:
received:
11
03
2020
accepted:
07
04
2020
pubmed:
21
4
2020
medline:
8
9
2020
entrez:
21
4
2020
Statut:
ppublish
Résumé
Patients with mucopolysaccharidosis type I (MPS I) have a good life expectancy due to early therapeutic options, such as stem cell therapy. Stem cell therapy can prevent the progression of some skeletal malformations. In contrast, the progression of thoracolumbar kyphoscoliosis, genua vara, and hip dysplasia cannot be influenced. We present 3 cases of children with MPS I with thoracolumbar kyphosis/kyphoscoliosis treated with a growing rod system. The medical records and radiologic imaging of 3 children with a diagnosis of MPS I and kyphosis/kyphoscoliosis of the lumbar spine treated between 2007 and 2019 were retrospectively analyzed. Two children presented with a kyphoscoliosis, and 1 child had a combination of severe anterolisthesis and kyphoscoliosis. Surgery to correct the kyphosis and dorsal stabilization was performed in all patients after exhausted conservative treatment. There were no neurologic complications. Postoperative treatment and aftercare included a corset for 4 months and physical therapy. In all 3 patients, distraction surgery of the lumbar stabilization was done twice at a mean interval of 1 year. If conservative treatment fails and surgery is necessary, an individual approach is needed. Dorsal stabilization with pedicle screws using a growing rod technique is an option for the correction of thoracolumbar/lumbar kyphosis in children with MPS I. However, fusion should be prevented initially or should be kept as short as possible. We achieved acceptable correction of the spinal deformity using the growing rod technique. Finally, surgery with correction and fusion is necessary after exhausted correction potential.
Sections du résumé
BACKGROUND
Patients with mucopolysaccharidosis type I (MPS I) have a good life expectancy due to early therapeutic options, such as stem cell therapy. Stem cell therapy can prevent the progression of some skeletal malformations. In contrast, the progression of thoracolumbar kyphoscoliosis, genua vara, and hip dysplasia cannot be influenced. We present 3 cases of children with MPS I with thoracolumbar kyphosis/kyphoscoliosis treated with a growing rod system.
CASE DESCRIPTION
The medical records and radiologic imaging of 3 children with a diagnosis of MPS I and kyphosis/kyphoscoliosis of the lumbar spine treated between 2007 and 2019 were retrospectively analyzed. Two children presented with a kyphoscoliosis, and 1 child had a combination of severe anterolisthesis and kyphoscoliosis. Surgery to correct the kyphosis and dorsal stabilization was performed in all patients after exhausted conservative treatment. There were no neurologic complications. Postoperative treatment and aftercare included a corset for 4 months and physical therapy. In all 3 patients, distraction surgery of the lumbar stabilization was done twice at a mean interval of 1 year.
CONCLUSIONS
If conservative treatment fails and surgery is necessary, an individual approach is needed. Dorsal stabilization with pedicle screws using a growing rod technique is an option for the correction of thoracolumbar/lumbar kyphosis in children with MPS I. However, fusion should be prevented initially or should be kept as short as possible. We achieved acceptable correction of the spinal deformity using the growing rod technique. Finally, surgery with correction and fusion is necessary after exhausted correction potential.
Identifiants
pubmed: 32311562
pii: S1878-8750(20)30762-2
doi: 10.1016/j.wneu.2020.04.055
pii:
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
169-174Informations de copyright
Copyright © 2020 Elsevier Inc. All rights reserved.