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Published in: Child's Nervous System 9/2018

01-09-2018 | Original Paper

Spinal cord issues in adult patients with MPS: transition of care survey

Authors: Kemel A. Ghotme, Fernando Alvarado-Gomez, Christina Lampe, Klane K. White, Martha Solano-Villareal, Roberto Giugliani, Paul R. Harmatz

Published in: Child's Nervous System | Issue 9/2018

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Abstract

Purpose

This study aims to raise awareness of the need for research and appropriate guidelines for managing spinal cord issues in adult patients with mucopolysaccharidosis (MPS) and transition of these patients from pediatric to adult care.

Methods

Pediatric/adult neurosurgeons, orthopedic spine surgeons, and treating physicians with expertise in metabolic disorders and spinal cord issues were invited to complete a survey to assess their experience with spinal cord problems in MPS and their opinion on transitioning routes from pediatric to adult care.

Results

Twenty specialists completed the survey; 16 had treated spinal cord issues in patients with MPS. Foramen magnum and cervical stenosis (87%), atlanto-axial instability (67%), and lumbar spine instability (33%) were the main spinal cord issues encountered; 28% had treated adult patients for one or more spinal cord issues. In 40% of cases, this concerned an intervention or procedures performed during childhood. The main specialist responsible for the care of adult patients with MPS differed considerably between institutions and included both pediatric and adult specialists (30% pediatric neurosurgeons, 10% pediatric spine orthopedic surgeons, 30% adult spine neurosurgeons, 20% general adult surgeons). The preferred option (> 50%) for the transition of care was an interdisciplinary team of pediatric and adult specialists.

Conclusions

Further work needs to be done to address problems of managing spinal cord issues in adult patients with MPS. Currently, the responsibility for the care of patients with MPS with spinal cord issues is inconsistent. The best strategy for transitioning these patients from pediatric to adult care is likely an interdisciplinary approach.
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Metadata
Title
Spinal cord issues in adult patients with MPS: transition of care survey
Authors
Kemel A. Ghotme
Fernando Alvarado-Gomez
Christina Lampe
Klane K. White
Martha Solano-Villareal
Roberto Giugliani
Paul R. Harmatz
Publication date
01-09-2018
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 9/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-018-3834-6

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