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

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.

• Authors: Rosen S Drebov, Atanas Katsarov 2nd
• Journal: Surgery journal (New York, N.Y.) (Surg J (N Y)) Vol. 2 Issue 3 Pg. e91-e95 (Jul 2016) ISSN: 2378-5128 [Print] United States
• PMID: 28824998 (Publication Type: Case Reports)