Lateral thoracic expansion for Jeune’s syndrome: midterm results

• Published in: The Annals of thoracic surgery Vol. 72; no. 3; pp. 872 - 877
• Main Authors: Davis, J.Terrance, Heistein, Jonathan B, Castile, Robert G, Adler, Brent, Mutabagani, Khaled H, Villalobos, Rafael E, Ruberg, Robert L
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Inc 01.09.2001
• Subjects: Adolescent; Bone Lengthening - methods; Bone Plates; Child; Child, Preschool; Female; Humans; Lung Volume Measurements; Male; Osteochondrodysplasias - complications; Osteochondrodysplasias - diagnostic imaging; Osteochondrodysplasias - pathology; Osteochondrodysplasias - surgery; Radiography, Thoracic; Respiratory Insufficiency - etiology; Ribs - abnormalities; Ribs - surgery; Syndrome; Thoracic Surgical Procedures - methods; Tomography, X-Ray Computed
• ISSN: 0003-4975; 1552-6259
• DOI: 10.1016/S0003-4975(01)02841-7

Background. In 1995, we reported the use of lateral thoracic expansion in a patient with symptomatic Jeune’s asphyxiating thoracic dystrophy. We have subsequently used lateral thoracic expansion 16 times on 10 patients during 7 years. This article reports our outcomes and provides surgical details. Methods. Charts of all patients undergoing lateral thoracic expansion were reviewed. Eight of the 10 patients had symptomatic Jeune’s syndrome. The other 2 had similar thoracic deformities limiting thoracic capacity. In half of the patients the procedures were performed bilaterally. Results. All patients older than 1 year of age were symptomatically benefited by lateral thoracic expansion. Functional and anatomic measurements documented thoracic enlargement in several patients who had comparable preoperative and postoperative studies. However, 2 infants with significant underlying airway disease did not improve and went on to succumb to that aspect of their disease despite enlargement of the thorax. Fracture of the titanium ministruts has been a recurrent problem, and we now use larger struts. Conclusions. Lateral thoracic expansion is a safe and effective procedure in selected patients with Jeune’s syndrome older than 1 year of age as judged by short-term and midterm follow-up. More experience and longer follow-up are required to discern the place of the lateral thoracic expansion in the overall management of these patients.