[Congenital subglottic laryngeal stenosis in 2 brothers with chondrodysplasia syndrome (Keutel-Gabriel syndrome)].
BACKGROUND
Keutel-Gabriel syndrome (chondrodysplasia) is a rare autosomal recessive disease. The patients have characteristic malformations such as midfacial hypoplasia, brachytelephalangia, and hearing loss as leading symptoms.


METHODS
We report about two brothers with clinical and radiological features of Keutel-Gabriel syndrome. Congenital subglottic laryngeal stenosis was also present in both. In the younger brother an emergency tracheotomy had to be performed. In a staged procedure the stenosis was successfully treated with laryngotracheoplasty according to Cotton.


CONCLUSIONS
This is the first description of a congenital subglottic laryngeal stenosis with Keutel-Gabriel syndrome. To avoid long-term tracheotomy, a tracheoplasty with autologous cartilage should be performed.
Buchsteiner
I
I
HNO-Klinik, Medizinischen Hochschule Hannover.
Kempf
H G
HG
Arslan-Kirchner
M
M
Schulze-Florey
T
T
D000293:Adolescent; D000328:Adult; D002356:Cartilage / Q000637:transplantation; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D007829:Laryngostenosis / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D008297:Male; D010009:Osteochondrodysplasias / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D013577:Syndrome; D014135:Tracheal Stenosis / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D014140:Tracheotomy; D014182:Transplantation, Autologous