CONGENITAL SUBGLOTTIC STENOSIS OF THE LARYNX IN 2 BROTHERS WITH CHONDRODYSPLASIA

Background: Keutel-Gabriel syndrome (chondrodysplasia) is a rare autos omal recessive disease. The patients have characteristic malformations such as midfacial hypoplasia, brachytelephalangia, and hearing loss a s leading symptoms. Patients: We report about two brothers with clinic al and radiological features of Keutel-Gabriel syndrome. Congenital su bglottic laryngeal stenosis was also present in both. In the younger b rother an emergency tracheotomy had to be performed. In a staged proce dure the stenosis was successfully treated with laryngotracheoplasty a ccording to Cotton. Conclusions: This is the first description of a co ngenital subglottic laryngeal stenosis with Keutel-Gabriel syndrome. T o avoid long-term tracheotomy, a tracheoplasty with autologous cartila ge should be performed.