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Otolaryngologic surgery in children with trisomy 18 and 13
Kaveh Karimnejad1, Dary J Costa2
1Saint Louis University School of Medicine, Department of Otolaryngology-Head and Neck Surgery, 3635 Vista Avenue, St. Louis, MO 63110, United States.
Insights
Otolaryngologic (ENT) surgeries are crucial for children with Trisomy 18 and 13, improving their quality of life. Seventeen percent of all procedures were ENT-related, demonstrating their significant role in managing these genetic disorders.
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.
Objectives:
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 And Materials:
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.
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