Techniques of surgical correction for the keeled chest in Marfan’s syndrome

Authors

  • Yakov Fishchenko
  • Vitaliy Fishchenko

DOI:

https://doi.org/10.15674/0030-59872010383-88

Keywords:

Marfan’s syndrome, keeled chest, surgical correction

Abstract

Marfan’s syndrome is an autosomal-dominant genetic disease of the connective tissue, caused by mutation of 15q21.1 gene with a resultant disorder in the synthesis of fibrillin-1, which is a component of collagen. Keeled chest is one of the commonest skeletal manifestations of Marfan’s syndrome. Materials and methods: The research was based on the study of treatment results in 42 patients with Marfan’s syndrome, aged 7 to 35, who underwent surgical treatment of their keeled chest at the hospital of this Institute. The correction of a symmetrically keeled chest was achieved by means of parasternal chondrotomy of the deformed cartilaginous part of the ribs with a subsequent fixation of the sternal rib end to the breastbone. Correction of an asymmetrically keeled chest on the convex side was similar to the previous one; correction on the concave side was achieved by making numerous notches on the cartilaginous part of the ribs deformed. Results: Good results of treating patients with a symmetrical deformity were traced in 19 cases (79.1 %), satisfactory results were in 5 patients (20.9 %), any negative results were not observed. Good results of treatment in patients with an asymmetrical deformity were achieved in 10 cases (55.5 %), satisfactory results in 7 patients (39.0 %), and a bad one in 1 case (5.5 %). Conclusions: Degrees 1–3 of progressing keeled chests in patients with Marfan’s syndrome are medical indications for corrective thoracoplasty. Analysis of the surgical treatment results demonstrated sufficient efficacy of the deformity correction technique, used by the authors.

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How to Cite

Fishchenko, Y., & Fishchenko, V. (2010). Techniques of surgical correction for the keeled chest in Marfan’s syndrome. ORTHOPAEDICS TRAUMATOLOGY and PROSTHETICS, (3), 83–88. https://doi.org/10.15674/0030-59872010383-88

Issue

No. 3 (2010)

Section

ORIGINAL ARTICLES

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Copyright (c) 2014 Yakov Fishchenko, Vitaliy Fishchenko

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