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

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.

[1]  S. Sivri,et al.  Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II , 2019, Turkish Journal of Pediatric Emergency and Intensive Care Medicine.

[2]  J. Cravero,et al.  Airway management in patients with mucopolysaccharidoses: The progression toward difficult intubation , 2019, Paediatric anaesthesia.

[3]  M. Bosatra,et al.  Anesthesiological risks in mucopolysaccharidoses , 2018, Italian Journal of Pediatrics.

[4]  A. Zanella,et al.  Safety of anesthesia for children with mucopolysaccharidoses: A retrospective analysis of 54 patients , 2018, Paediatric anaesthesia.

[5]  J. Sprung,et al.  Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management. , 2017, Bosnian journal of basic medical sciences.

[6]  K. Flanigan,et al.  General anesthesia with a native airway for patients with mucopolysaccharidosis type III , 2017, Paediatric anaesthesia.

[7]  M. Cohen,et al.  Delivery of anesthesia for children with Mucopolysaccharidosis Type III (Sanfilippo syndrome): a review of 86 anesthetics , 2017, Paediatric anaesthesia.

[8]  C. Whitley,et al.  Anesthetic care and perioperative complications in children with Sanfilipo Syndrome Type A , 2016, Paediatric anaesthesia.

[9]  T. Coşkun,et al.  Mucopolysaccharidosis: Otolaryngologic findings, obstructive sleep apnea and accumulation of glucosaminoglycans in lymphatic tissue of the upper airway. , 2014, International journal of pediatric otorhinolaryngology.

[10]  M. Poe,et al.  Natural history of Sanfilippo syndrome type A , 2014, Journal of Inherited Metabolic Disease.

[11]  J. E. Wraith,et al.  Tracheostomy in mucopolysaccharidosis type II (Hunter's Syndrome). , 2013, International journal of pediatric otorhinolaryngology.

[12]  I. Bruce,et al.  Anaesthesia and airway management in mucopolysaccharidosis , 2012, Journal of Inherited Metabolic Disease.

[13]  H. Sivri,et al.  Oral health status in patients with mucopolysaccharidoses. , 2018, The Turkish journal of pediatrics.

[14]  O. Addison,et al.  The oral health needs of children, adolescents and young adults affected by a mucopolysaccharide disorder. , 2012, JIMD reports.

[15]  Anthony O. Fedele.  The Application of Clinical Genetics Dovepress Sanfilippo Syndrome: Causes, Consequences, and Treatments , 2022 .