Barnes syndrome
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(thoraco-laryngo-pelvic dysplasia)
Very rare: prevalence < 1/106. Association of a thoraco-pelvic dysostosis with a laryngeal stenosis.
Clinical features:
- small, narrow chest with short ribs; unlike Jeunes syndrome, the chest is bell-shaped
- small and narrow pelvis
- laryngeal stenosis: the laryngeal involvement may be secondary to intubation in the neonatal period.
Major neonatal respiratory distress. The combination of a narrow thorax with a narrow pelvis give the impression of a protruding abdomen.
Anesthetic implications:
restrictive respiratory syndrome, subglottic stenosis
References :
- Miller TL, Cox T, Blackson T, Paul D et al.
Pulmonary function assessment in an infant with Barnes syndrome : proactive evaluation for surgical intervention.
Pediatrics 2006, 118: e1264-7.
- Wood E., Kearns D.
Laryngotracheal stenosis in thoracolaryngopelvic dysplasia: Barnes syndrome.
Otolaryngol, Head & Neck Surg 1995; 113: 807-9
- Patel SH, Banzali FM, Post RJ, Nguyen CV, Benoit RM, Tieu DD, Stranc DS, Conte AH, Rudikoff AG.
Parturient with Barnes syndrome (thoracolaryngopelvic dysplasia) undergoing cesarean delivery of a neonate with Barnes syndrome : a case report.
A&A Practice 2018 ; 11 : 151-4.
Updated: October 2018