title: Otolaryngologic surgery in children with trisomy 18 and 13.
abstract: OBJECTIVE
  Trisomy 18 and 13 are the most common autosomal trisomy disorders after Down syndrome. Given the high mortality rate (5-10% one-year survival), trisomy 18 and 13 were historically characterized as uniformly lethal and palliation was the predominant management approach. Management strategy has shifted with recognition that through medical and surgical intervention, children with trisomy 18 and 13 can achieve developmental milestones, live meaningful lives, and exhibit long-term survival. Otolaryngologic surgery in children with trisomy 18 and 13 has not been described. The objective of this article is to describe the role of the otolaryngologist in the management of children with trisomy 18 and 13.
  
  
  METHODS
  Retrospective cohort analysis of the surgery registry for the Support Organization for Trisomy 18, 13 and Related Disorders for otolaryngologic surgeries reported from 1988 through June 1, 2014.
  
  
  RESULTS
  In the database of approximately 1349 children, 1380 procedures were reported, 231 (17%) of which were otolaryngologic. The most common otolaryngologic procedures were tympanostomy tube placement (57/231, 25%), cleft lip repair (40/231, 17%), tracheostomy (38/231, 16.5%), tonsillectomy and/or adenoidectomy (37/231, 16%), and cleft palate repair (30/231, 13%). Of the ten most common procedures reported, four were otolaryngologic.
  
  
  CONCLUSIONS
  Seventeen percent of procedures performed in children with trisomy 18 and 13 were otolaryngologic, highlighting the significant role of the otolaryngologist in the treatment of these patients. Surgical intervention may be considered as part of a balanced approach to patient care.
authors.affiliation: Saint Louis University School of Medicine, Department of Otolaryngology-Head and Neck Surgery, 3635 Vista Avenue, St. Louis, MO 63110, United States. Electronic address: kkarimne@slu.edu.
authors.forename: Kaveh
authors.initials: K
authors.lastname: Karimnejad
authors.affiliation: Saint Louis University School of Medicine, Department of Otolaryngology-Head and Neck Surgery, 3635 Vista Avenue, St. Louis, MO 63110, United States; SSM Cardinal Glennon Children's Medical Center, 1465 South Grand Avenue, Room B-826, St. Louis, MO 63104, United States. Electronic address: costadj@slu.edu.
authors.forename: Dary J
authors.initials: DJ
authors.lastname: Costa
mesh_terms: D002648:Child; D002675:Child, Preschool; D025063:Chromosome Disorders / Q000601:surgery*; D002882:Chromosomes, Human, Pair 13; D002887:Chromosomes, Human, Pair 18; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D013517:Otorhinolaryngologic Surgical Procedures / Q000706:statistics & numerical data*; D012042:Registries; D012189:Retrospective Studies; D014314:Trisomy; D000073839:Trisomy 13 Syndrome; D000073842:Trisomy 18 Syndrome
keywords: Otolaryngology; Pediatric; Surgical management; Trisomy