Tracheal cartilaginous sleeve association with syndromic midface hypoplasia.
OBJECTIVE
We report 2 new cases of tracheal cartilaginous sleeve and perform a systematic literature review of all documented cases of this condition. We aim to increase awareness of this anomaly and provide recommendations for both intraoperative and postoperative management of patients with tracheal cartilaginous sleeve undergoing tracheostomy.


METHODS
We studied the clinical records of 2 children with tracheal cartilaginous sleeves and short tracheas treated at our institution. One of these patients had Beare-Stevenson syndrome, and the other had Crouzon syndrome. Both patients required tracheostomy for persistent upper airway obstruction, and both required custom-length tracheostomy tubes. Bronchoscopy and needle localization were beneficial in performing tracheostomy on these patients. All documented cases of tracheal cartilaginous sleeve in the literature were found to be associated with midface hypoplasia secondary to syndromic craniosynostosis. Seventy-five percent of cases required tracheostomy.


CONCLUSIONS
Tracheal cartilaginous sleeve can be associated with Beare-Stevenson syndrome. In infants with midface hypoplasia associated with a craniosynostosis syndrome undergoing tracheostomy, the surgeon must be prepared to encounter and manage tracheal cartilaginous sleeve intraoperatively. Bronchoscopy and needle localization can be beneficial when performing tracheostomy in these patients. Customized, shorter-length tracheostomy tubes should be considered for these patients.
Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Weill Cornell Medical College, New York, New York.
Brian J
BJ
Stater
Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, University Medical Center Utrecht-Wilhelmina Children's Hospital, Utrecht, the Netherlands.
Karin P Q
KP
Oomen
Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Weill Cornell Medical College, New York, New York.
Vikash K
VK
Modi
D000015:Abnormalities, Multiple; D002356:Cartilage / Q000002:abnormalities*; D003394:Craniofacial Dysostosis; D003398:Craniosynostoses / Q000150:complications; D005145:Face / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013577:Syndrome; D014132:Trachea / Q000002:abnormalities*; D014139:Tracheostomy / Q000379:methods