• Kenny Yat Hong Kwan, Cheung Jason Pui Yin JPY Department of Orthopaedics and Traumatology, Li Ka Shing Faculty of Medicine, Queen Mary Hospital, The University of Hong Kong, Pokfulam Road, Ho, Karen Kar Lum Yiu, and Cheung Kenneth Man Chee KMC Department of Orthopaedics and Traumatology, Li Ka Shing Faculty of Medicine, Queen Mary Hospital, The University of Hong Kong, Pokfulam Road,.
• Department of Orthopaedics and Traumatology, Li Ka Shing Faculty of Medicine, Queen Mary Hospital, The University of Hong Kong, Pokfulam Road, Hong Kong, SAR, Hong Kong.
• Eur Spine J. 2018 Jul 1; 27 (Suppl 3): 287-291.

PurposeJarcho-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.MethodsA boy with Jarcho-Levin syndrome undergoes a hybrid VEPTR-MCGR construct to treat his thoracic insufficiency syndrome and spinal deformity.ResultsThe 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.ConclusionsA 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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