Delayed Onset Post-Operative Neurologic Deficit in a Patient With Mucopolysaccharidosis type VI: A Case Report

Christopher Lindsay; Joshua Holt; Stuart Weinstein

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Delayed Onset Post-Operative Neurologic Deficit in a Patient With Mucopolysaccharidosis type VI: A Case Report

Journal article

Open access

Peer reviewed

Delayed Onset Post-Operative Neurologic Deficit in a Patient With Mucopolysaccharidosis type VI: A Case Report

Christopher Lindsay, Joshua Holt and Stuart Weinstein

The Iowa orthopaedic journal, Vol.42(2), pp.122-127

2022

PMCID: PMC9769351

PMID: 36601232

Appears in Diamond Open Access

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https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9769351/View

Published (Version of record) Open Access

Abstract

Mucopolysaccharidoses (MPS) are lysosomal storage disorders characterized by abnormal deposition of glycosaminoglycans (GAGs) in tissues. In type VI MPS, otherwise known as Maroteaux-Lamy syndrome, the defect is in the enzyme N-acetylgalactosamine-4-sulfatase. Thoracolumbar kyphosis results from GAG deposition, leading to incompetence of posterior ligamentous structures as well as poor trunk control. Though neurologic symptoms from canal compression due to deformity and hypertrophy of tissues have been described, occasionally requiring surgical decompression, there has not been a prior report of late onset of symptoms in a previously neurologically intact patient following surgery to correct spine deformity. The case reviewed is a 14 year old girl with mucopolysaccharidosis type VI underwent anterior release and posterior instrumentation for correction of severe progressive lumbar kyphosis. Postoperatively she developed delayed onset of profound lower extremity weakness and underwent urgent wide laminectomies and resection of thickened ligamentum flavum. At 1 year follow-up, she had near complete neurologic recovery. Patients with mucopolysacchari-doses are at significant risk for neurologic compromise both as part of the natural history of the disease, and as a risk of deformity correction. The surgeon must consider the pathologic thickening of tissues surrounding the spinal cord when planning surgery. .

Adolescent

Female

Humans

Kyphosis - etiology

Kyphosis - surgery

Mucopolysaccharidosis VI - complications

Mucopolysaccharidosis VI - surgery

N-Acetylgalactosamine-4-Sulfatase

Details

Title: Subtitle: Delayed Onset Post-Operative Neurologic Deficit in a Patient With Mucopolysaccharidosis type VI: A Case Report
Creators: Christopher Lindsay - Department of Orthopedics and Rehabilitation, University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
Joshua Holt - University of Iowa
Stuart Weinstein - University of Iowa
Resource Type: Journal article
Publication Details: The Iowa orthopaedic journal, Vol.42(2), pp.122-127
Publisher: Dept. of Orthopaedics, The University of Iowa; United States
PMID: 36601232
PMCID: PMC9769351
ISSN: 1541-5457
eISSN: 1555-1377
Language: English
Date published: 2022
Academic Unit: Stead Family Department of Pediatrics; Orthopedics and Rehabilitation
Record Identifier: 9984355053302771

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