Otolaryngologic surgery in children with trisomy 18 and 13

Area of Science:

• Genetics and Genetic Disorders
• Pediatric Surgery
• Otolaryngology

Background:

• Trisomy 18 and 13 are significant autosomal trisomy disorders with historically high mortality rates.
• Management paradigms have shifted towards interventions to improve quality of life and long-term survival.
• The role of otolaryngologic surgery in these children remains undescribed.

Purpose of the Study:

• To describe the role and scope of otolaryngologic surgery in the management of children with Trisomy 18 and 13.
• To highlight the frequency and types of otolaryngologic procedures performed in this population.

Main Methods:

• Retrospective cohort analysis.
• Utilized the surgery registry from the Support Organization for Trisomy 18, 13 and Related Disorders.
• Data collected from 1988 through June 1, 2014.

Main Results:

• Of 1349 children, 1380 procedures were reported; 231 (17%) were otolaryngologic.
• Most common procedures included tympanostomy tube placement (25%), cleft lip repair (17%), and tracheostomy (16.5%).
• Four of the ten most common procedures were otolaryngologic.

Conclusions:

• Otolaryngologic procedures constitute a significant portion (17%) of surgical interventions for children with Trisomy 18 and 13.
• Surgical management by otolaryngologists is integral to a comprehensive care approach for these patients.
• These interventions support improved outcomes and quality of life.