AffiliationDepartments of Paediatric Orthopaedic Surgery, Children's University Hospital, Temple Street, Dublin, Ireland. eimearconroy04@eircom
MeSHEllis-Van Creveld Syndrome
MetadataShow full item record
CitationSternoplasty and rib distraction in neonatal Jeune syndrome. 2010, 30 (6):527-30 J Pediatr Orthop
JournalJournal of pediatric orthopedics
AbstractA 12-week-old boy with Jeune syndrome (asphyxiating thoracic dystrophy) was referred to the orthopaedic unit with progressive respiratory failure, recurrent respiratory tract infections, and recurrent admissions to the intensive care unit for ventilatory support. His chest x-ray revealed a small and narrow thoracic cage with short broad ribs and abnormal costal cartilages. His chest expansion was impaired by the short, horizontally positioned ribs resulting in alveolar hypoventilation. Without surgical intervention to expand his thoracic cage, he would die of respiratory failure.