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Fraser Syndrome: A Stumbling Block for the Anaesthesiologist.

Sapna Bathla¹, Wahaja Karim¹, Anil Kumar¹

¹Department of Anesthesia, VMMC and Safdarjung Hospital, New Delhi, Delhi India.

Indian Journal of Otolaryngology and Head and Neck Surgery : Official Publication of the Association of Otolaryngologists of India

|February 6, 2023

Summary

Fraser syndrome, a rare genetic disorder, causes complex airway malformations. This study details airway management strategies, including emergency tracheostomy, for affected children facing intubation challenges.

Keywords:

Difficult intubation Fraser syndrome Paediatric Subglottic stenosis

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Area of Science:

Genetics
Pediatrics
Anesthesiology

Background:

Fraser syndrome is a rare autosomal recessive disorder characterized by a spectrum of congenital malformations.
Airway abnormalities are a significant feature, including high arched palate, laryngeal atresia, hypoplasia, and stenosis.
Difficulties with laryngeal intubation are frequently documented in affected individuals.

Purpose of the Study:

To discuss the challenges and decision-making processes involved in airway management for children with Fraser syndrome.
To highlight the critical role of emergency tracheostomy in managing severe airway compromise.

Main Methods:

Review of clinical literature pertaining to Fraser syndrome and airway management.
Discussion of anesthetic and surgical approaches for airway control in affected pediatric patients.

Main Results:

Laryngeal intubation can be exceedingly difficult in Fraser syndrome patients due to anatomical variations.
Emergency tracheostomy is often a necessary intervention for securing the airway in these complex cases.

Conclusions:

Effective airway management in Fraser syndrome requires careful planning and a low threshold for tracheostomy.
Multidisciplinary collaboration is essential for optimizing outcomes in these rare pediatric cases.