The Complication of Endotracheal Intubation in a Patient with Mucopolysaccharidosis Type IIIA

Ademhan Tural, Dilber; Emiralioglu, Nagehan; Ozsezen, Beste; Cıkı, Kismet; Ozcan, Nursun; Ardicli, Burak; Sivri, Serap

doi:10.22038/ijp.2020.47300.3837

The Complication of Endotracheal Intubation in a Patient with Mucopolysaccharidosis Type IIIA

Authors

Dilber Ademhan Tural ¹

Nagehan Emiralioglu ²

Beste Ozsezen ²

Kismet Cıkı ³

Nursun Ozcan ⁴

Burak Ardicli ⁵

Serap Sivri ³

¹ Department of Pediatric Pulmonology, Ihsan Dogramaci Children’s Hospital, Hacettepe University Faculty of Medicine, Ankara, Turkey.

² Department of Pediatric Pulmonology, Ihsan Dogramaci Children’s Hospital, Hacettepe University Faculty of Medicine, Ankara, Turkey.

³ Department of Pediatric Metabolism, Ihsan Dogramaci Children’s Hospital, Hacettepe University Faculty of Medicine, Ankara, Turkey.

⁴ Department of Radiology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

⁵ Department of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey

10.22038/ijp.2020.47300.3837

Abstract

Mucopolysaccharidosis type III (MPS III), also known as Sanfilippo syndrome, is an autosomal recessive, neurodegenerative lysosomal storage disorder. Substantial challenges for airway management and endotracheal intubation are predictable due to the MPS patients' specific phenotypic, facial, and airway characteristics. In studies concerning various types of MPS, the incidence of difficult endotracheal intubation ranges between 28%-44%. This study intends to present a case with MPSIIIA, who aspirated her tooth during endotracheal intubation due to acute respiratory failure. This study reports a 12.5 year-old girl who presented with MPSIIIA and aspirated her tooth during endotracheal intubation due to acute respiratory failure resulting in lung lobe segmentectomy. The majority of MPS patients’ intubation and airway maintenance are hard and get even more difficult by age because of their mental retardation and oral health deteriorating, which may require tracheostomy.

Keywords

Aspiration

Child

Difficult Airway Maintenance

Intubation

MPS Type IIIA