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European Spine Journal

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01-07-2018 | Case Report

Ten year follow-up of Jarcho–Levin syndrome with thoracic insufficiency treated by VEPTR and MCGR VEPTR hybrid

Authors: Kenny Yat Hong Kwan, Jason Pui Yin Cheung, Karen Kar Lum Yiu, Kenneth Man Chee Cheung

Published in: European Spine Journal | Special Issue 3/2018

Abstract

Purpose

Jarcho–Levin syndrome patients commonly suffer from repeated respiratory infections and become ventilator dependent due to an insufficient thoracic volume. Multiple congenital vertebral anomalies are associated with this genetic disorder and results in stunted spine growth. The purpose of this case report is to present the results of a hybrid vertical expandable prosthetic titanium rib (VEPTR) and magnetically controlled growing rod (MCGR) construct for the management of a patient with Jarcho–Levin syndrome.

Methods

A boy with Jarcho–Levin syndrome undergoes a hybrid VEPTR–MCGR construct to treat his thoracic insufficiency syndrome and spinal deformity.

Results

The patient could wean off ventilator and had reduced chest complications with the construct. He was also able to achieve some spine length gain with the distraction device. However, there were limitations in prolonged treatment as no spine height was gained once he reached 12 years old. Once no further growth can be achieved, the MCGR led to progressive kyphosis. Nevertheless, at latest follow-up after the removal of all implants, the patient could maintain satisfactory correction of both thoracic and spine deformities.

Conclusions

A hybrid construct is necessary for managing Jarcho–Levin syndrome as the VEPTR deals with the thoracic cage deformity while the MCGR deals with the spine deformity.

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Title: Ten year follow-up of Jarcho–Levin syndrome with thoracic insufficiency treated by VEPTR and MCGR VEPTR hybrid
Authors: Kenny Yat Hong Kwan
Jason Pui Yin Cheung
Karen Kar Lum Yiu
Kenneth Man Chee Cheung
Publication date: 01-07-2018
Publisher: Springer Berlin Heidelberg
Published in: European Spine Journal / Issue Special Issue 3/2018
Print ISSN: 0940-6719
Electronic ISSN: 1432-0932
DOI: https://doi.org/10.1007/s00586-017-5164-x

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Ten year follow-up of Jarcho–Levin syndrome with thoracic insufficiency treated by VEPTR and MCGR VEPTR hybrid

Abstract

Purpose

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusions

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