Malformation and stenosis of the cricoid cartilage in association with Larsen's syndrome.
Three patients with the typical features of Larsen's syndrome are described. All three developed severe respiratory symptoms caused by a congenital subglottic stenosis. Tracheotomy and treatment of the stenosis by means of laryngotracheoplasty resulted in complete collapse of the cricoid cartilage and the proximal tracheal skeleton. Lack of rigidity of the laryngeal and tracheal cartilages in patients with Larsen's syndrome could well be responsible for this failure. Surgical treatment consisted of resection of the stenotic and collapsed areas and end-to-end anastomosis. This therapy was eventually successful in all three patients.
Hoeve
H J
HJ
Department of Otorhinolaryngology, Sophia Children's Hospital, Rotterdam, The Netherlands.
Joosten
K F
KF
Bogers
A J
AJ
Hazebroek
F W
FW
Pfenninger
J
J
van der Voort
E
E
Leijala
M
M
D000015:Abnormalities, Multiple; D000402:Airway Obstruction / Q000209:etiology; D000714:Anastomosis, Surgical; D003251:Constriction, Pathologic; D003413:Cricoid Cartilage / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D019066:Facies; D005260:Female; D006801:Humans; D007223:Infant; D004204:Joint Dislocations / Q000150:complications*; D007829:Laryngostenosis / Q000150:complications / Q000151:congenital / Q000601:surgery*; D008297:Male; D013577:Syndrome; D014132:Trachea / Q000601:surgery