Anesthesiological risks in mucopolysaccharidoses

Abstract

BackgroundPatients suffering from mucopolysaccharidosis are among the most complex from the anesthesiological point of view, especially regarding the management of the airway. The evidence base for anesthesia management is often limited to case reports and small case series.AimsTo identify useful information about experience with each subtype of mucopolysaccharidosis reported in the literature and propose a guide on the best options for airway management to the anesthesiologists who take care of these patients.MethodsA query of the PubMed database specific for “anesthesia” and “mucopolysaccharidosis” and a further query specific for “mucopolysaccharidosis and difficult airway management” was conducted. We looked for those items that offered practical guidance to anesthesiological management. We did not exclude case reports, especially those that reported a specific technique, because of their practical suggestions.ResultsWe identified 15 reviews, 17 retrospective case series, 5 prospective studies, and 28 case reports that focused on airway managements in anesthesia or had practical suggestions for preoperative evaluation and risk assessment. An accurate preoperative evaluation and the need for an experienced team are emphasized in all the reviewed articles and for each type of mucopolysaccharidosis. Many suggestions on how to plan the perioperative period have been highlighted. Insertion of a laryngeal mask airway generally improves ventilation and facilitates intubation with a fiberoptic bronchoscope. Furthermore, the videolaryngoscope is very useful in making intubation easier and facilitating bronchoscope passage.ConclusionsPatients with mucopolysaccharidosis are at high risk for anesthesia-related complications and require a high level of attention. However, a multidisciplinary approach, combined with expertise in the use of new techniques and new devices for airway management, makes anesthesiological management safer. Further research with prospective studies would be useful.Electronic supplementary materialThe online version of this article (10.1186/s13052-018-0554-1) contains supplementary material, which is available to authorized users.

Disease Manifestations in Mucopolysaccharidoses and Their Impact on Anaesthesia-Related Complications—A Retrospective Analysis of 99 Patients

“…To exemplify this, 44% of MPSI patients have undergone at least two surgeries by four years of age [4]. Simultaneously, MPS patients experience a high perioperative risk of morbidity and mortality [5][6][7]. Registry data on MPSI patients revealed a perioperative morbidity rate of up to 30% [8] and a risk of death of 0.7% within 30 days after surgery [9].…”

Early Recurrent Carpal Tunnel Syndrome in Patients with Mucopolysaccharidoses

“… 4 However, surgeries expose this patient population to the risks of general anesthesia, which are compounded by difficult airways and other medical comorbidities. 9 Due to their young age and common developmental delays, IV sedation or local/regional anesthesia is often not an option, and emergent intubation can be highly dangerous in these patients.…”

New recommendations for the care of patients with mucopolysaccharidosis type I

“…Large airway obstruction is a serious problem. 14 The hypertrophy of palatine tonsils and adenoids results in snoring, obstruction, and obstructive sleep apneas. They should be studied with sleep polysomnography and lateral neck X-ray in sniffing position.…”