Operative management of acquired Jeune's syndrome

Maria Grazia Sacco Casamassima, Seth D. Goldstein, Jose H. Salazar, Dominic Papandria, Kimberly H. McIltrot, David E. O'Neill, Fizan Abdullah, Paul M. Colombani*

*Corresponding author for this work

Research output: Contribution to journalArticle

6 Scopus citations

Abstract

Background Acquired Jeune's syndrome is a severe iatrogenic deformity of the thoracic wall following a premature and aggressive open pectus excavatum repair. We report herein our technique and experience with this rare condition. Methods From 1996 to 2011, nineteen patients with acquired Jeune's syndrome were retrospectively identified in a tertiary referral center. The technique used to expand and reconstruct the thoracic wall consisted of 1) release of the sternum from fibrous scar tissue, 2) multiple osteotomies along the lateral aspect of the ribs with anterior advancement of costal-cartilages to protect the heart, 3) stabilization of the thorax by placing a curved bar for retrosternal support and, 4) restoration of the sterno-costal junction by wiring the lower cartilages to the edge of the sternum. Results Major complications observed in this series were: bar displacement (seven cases), postoperative death from cardiac arrest following bronchoscopy (one case), late cardiac tamponade from migration of wire suture fragment (one case), and need for multiple reoperations (one case). Long-term cosmetic results and improvement in daily quality of life were reported as positive in the majority of cases. Conclusions Anterior chest wall reconstruction successfully treated our series of patients with acquired Jeune's syndrome. This multifaceted technique is an effective procedure that allows expansion of the thoracic cavity and improvement of aerobic activity.

Original languageEnglish (US)
Pages (from-to)55-60
Number of pages6
JournalJournal of pediatric surgery
Volume49
Issue number1
DOIs
StatePublished - Jan 1 2014

Keywords

  • Asphyxiating thoracic dystrophy
  • Jeune's syndrome
  • Recurrent Pectus excavatum

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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  • Cite this

    Sacco Casamassima, M. G., Goldstein, S. D., Salazar, J. H., Papandria, D., McIltrot, K. H., O'Neill, D. E., Abdullah, F., & Colombani, P. M. (2014). Operative management of acquired Jeune's syndrome. Journal of pediatric surgery, 49(1), 55-60. https://doi.org/10.1016/j.jpedsurg.2013.09.027