Anesthethic approach of mucopolysaccharidoses

Authors

  • Margarida Marcelino Serviço de Anestesiologia – Instituto Português de Oncologia de Lisboa, Francisco Gentil
  • Luísa Marote  Serviço de Anestesiologia – Hospital de Santa Maria (HSM), Centro Hospitalar Lisboa Norte (CHLN)
  • Maria Domingas Patuleia  Serviço de Anestesiologia – Hospital de Santa Maria (HSM), Centro Hospitalar Lisboa Norte (CHLN)

DOI:

https://doi.org/10.25751/rspa.8855

Keywords:

Mucopolysaccharidoses, General anaesthesia, Perioperative Period, Airway Management

Abstract

Mucopolysaccharidoses (MPS) are a group of hereditary disorders caused by a deficiency of glycosaminoglycan (GAG) metabolism enzymes, with subsequent incomplete breakdown of GAG, which accumulate in organs. They are chronic and progressive disorders. Cardiac and respiratory involvements are the principal causes of mortality. 

Patients with MPS are frequently submitted to surgical procedures. Nowadays, these patients’ increase in life expectancy causes their perioperative management to be a concern for all anaesthetists. Despite breakthroughs in the anaesthetic and surgical approach, perioperative mortality is still high among patients.

Preoperative evaluation includes a thorough clinical history and note of prior surgical procedures or anaesthesias. Airway evaluation should include diagnostic imaging to identify odontoid dysplasia or atlantoaxial subluxation.

Locoregional techniques should be chosen whenever possible, in order to avoid airway management. However, this may not always be possible due to the patient’s age, comorbidities and surgical procedure.

Airway management may be achieved with orotracheal intubation, either by laryngoscopy or fiberoptic techniques, laryngeal mask or tracheostomy.

In these patients postoperative management should take place in an Intensive Care Unit.

Downloads

Download data is not yet available.

Downloads

Published

2016-03-22

How to Cite

Marcelino, M., Marote, L., & Patuleia, M. D. (2016). Anesthethic approach of mucopolysaccharidoses. Journal of the Portuguese Society of Anesthesiology, 21(3), 19–22. https://doi.org/10.25751/rspa.8855

Issue

Vol. 21 No. 3 (2012): Journal of the Portuguese Society of Anesthesiology

Section

Narrative Reviews

License

Articles are freely available to be read, downloaded and shared from the time of publication.

The RSPA reserves the right to commercialize the article as an integral part of the journal (in the preparation of reprints, for example). The author should accompany the submission letter with a declaration of copyright transfer for commercial purposes.

Articles are published under the terms of the Creative Commons Attribution Non-Commercial License (CC BY-NC).

After publication in RSPA, authors are allowed to make their articles available in repositories of their home institutions, as long as they always mention where they were published.