ArticleSurgical neurology international2017
Chiari I malformation and syringomyelia in mucopolysaccharidosis type I (Hurler syndrome) treated with posterior fossa decompression: Case report and review of the literature.
Article in Surgical neurology international, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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3 citing papers in PubMed, 5 citations in OpenAlex.
- Posterior fossa decompression in syndromic children with chiari-like posterior fossa crowding: a nationwide US-based study.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Mucopolysaccharidosis Type 1 among Children-Neuroradiological Perspective Based on Single Centre Experience and Literature Review.Metabolites · 2023Article
- Treatment of thoracolumbar kyphosis in patients with mucopolysaccharidosis type I: results of an international consensus procedure.Orphanet journal of rare diseases · 2019Review
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Authors and funding
4 authors at 2 institutions in 1 country.
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Abstract
backgroundHurler Syndrome is the most severe phenotype of mucopolysaccharidosis type I. With bone marrow transplant and enzyme replacement therapy, the life expectancy of a child with Hurler syndrome has been extended, predisposing them to multiple musculoskeletal issues most commonly involving the spine. CASE DESCRIPTION: This is the case report of a 6-year-old male with Hurler syndrome who was diagnosed with Chiari I malformation and cervicothoracic syringomyelia on a preoperative magnetic resonance imaging (MRI) for his thoracolumbar kyphosis. This report details the successful management of a Chiari I malformation and syringomyelia with posterior fossa decompression in a child with Hurler syndrome.
conclusionChildren born with MPS I can have complex spine issues that require surgical management. The most common orthopedic spinal condition for these patients, thoracolumbar kyphosis, requires evaluation with an MRI before performing surgery. This resulted in the diagnosis of a Chiari I malformation and syringomyelia in our patient with Hurler syndrome. This was successfully treated with decompression of the posterior fossa.
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