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CC BY 4.0 · Surg J (N Y) 2016; 02(03): e91-e95
DOI: 10.1055/s-0036-1593354
Case Report

Poland Syndrome: Use of Vertical Expandable Prosthetic Titanium Rib System before Walking Age—A Case Report

Rosen S. Drebov
1   Department of Pediatric Surgery, University Emergency Hospital “N.I. Pirogov”, Sofia, Bulgaria
,
Atanas Katsarov II
2   Department of Children's Orthopaedics, University Emergency Hospital “N.I. Pirogov”, Sofia, Bulgaria
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Abstract

Aim To present a new therapy for Poland syndrome (PS) using a novel surgical approach: the vertical expandable prosthetic titanium rib (VEPTR) system.

Methods The VEPTR system rib-to-rib variant was used to enhance the chest wall and vertebral column support in a young patient before walking age.

Case Report We present a 12-month-old infant diagnosed with left-sided PS at the age of 6 months associated with missing ribs, scoliosis, and absence of the left pectoral muscles. Because of four missing ribs, paradoxical breathing was present. In addition, the left scapula was protruding into the chest due to the missing rib support. Scoliosis was caused by a left-sided nonsegmented bar of the thoracic spine.

Results We decided to use the VEPTR system before the patient reached walking age to prevent progression of column deformation and future pulmonary problems. To improve the spinal deformity, to stabilize the thorax, and to improve thoracic function, we performed the operation at 1 year of age. At 10-month follow-up, the patient was reevaluated. The construction was still stable and scoliosis had not deteriorated.

Conclusion The VEPTR system is a choice of treatment in young patients with PS to prevent late complications after a child reaches walking age.

Keywords

Poland syndrome - VEPTR - vertical expandable prosthetic titanium rib


Publication History

Received: 12 May 2016

Accepted: 11 August 2016

Publication Date:
14 September 2016 (online)

© 2016. Thieme. All rights reserved.

Thieme Medical Publishers
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