Sternoplasty and rib distraction in neonatal Jeune syndrome.
BACKGROUND
A 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.


METHODS
Using the technique of distraction osteogenesis, we split his sternum and slowly expanded this split to a total of 3 cm using a Leibinger mid-face distractor allowing adequate wound healing and bone formation between the 2 sternal edges. He returned to the theater 4 weeks after his initial surgery to have the distractor removed and 2 Leibinger plates inserted to hold the sternum out to length. Sternal distraction was repeated and the sternum was then supplemented with a Leibinger mesh. At 8 months, he had successful expansion of his ribs bilaterally using this technique of distraction osteogenesis.


RESULTS
Presently, the patient is 30 months old and is living at home, is not oxygen dependent, and continues to thrive.


CONCLUSIONS
Jeune syndrome presenting with respiratory failure in the neonate is fatal without surgical intervention to expand the thoracic cage. No successful surgical techniques have been described in the literature for neonates. This is the first case in which distraction osteogenesis has been used to distract both sternum and ribs in an infant with Jeune syndrome. Hence, distraction osteogenesis in thoracic dystrophy is a novel approach in the neonate.


METHODS
Case report.
Conroy
Eimear
E
Departments of Paediatric Orthopaedic Surgery, Children's University Hospital, Temple Street, Dublin, Ireland. eimearconroy04@eircom
Eustace
Nicholas
N
McCormack
Damian
D
D004613:Ellis-Van Creveld Syndrome / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D019857:Osteogenesis, Distraction / Q000379:methods*; D012131:Respiratory Insufficiency / Q000209:etiology / Q000601:surgery*; D012272:Ribs / Q000002:abnormalities / Q000601:surgery; D013249:Sternum / Q000002:abnormalities / Q000601:surgery; D035441:Thoracic Wall / Q000002:abnormalities / Q000601:surgery