Anesthetic Management in Mucopolysaccharidoses

Abstract

Mucopolysaccharidoses (MPSs) are a group of uncommon genetic diseases of connective tissue metabolism. It is well established that the elective treatment of subjects affected by MPS is multidisciplinary and must be carried out by experienced personnel in highly specialist centers. However, there is the possibility to perform an anesthesia in a peripheral center, where anesthesiologists might not have a large experience of MPS. Various attempts to produce guidelines have been made for MPS. There has been an increasing interest in the topic as it is demonstrated by the number of scientific studies published in the last few years (peak in 2011). We want to provide a summary of anesthetic management, reviewing the current literature on the topic in a procedural algorithm for these high-risk patients, who require surgical procedures and diagnostic examinations under sedation with a higher frequency than the general population.

Management of a Difficult Airway Scenario in a Case of Hurler’s Syndrome with a D-Blade Video Laryngoscope

“…Children with Hurler's syndrome can present with abnormal upper airway anatomy, poor thoracic compliance, and atlantoaxial instability. 1,3 Unsuccessful intubation attempts have also resulted in airway-related deaths of children having mucopolysaccharidoses. 4 Dealing with such a challenging scenario at a remote location outside operating rooms is a trying ordeal for an anesthesiologist.…”

“…Hurler's syndrome, a type 1 mucopolysaccharidosis (MPS 1H), is an uncommon genetic disease caused by deficiency of a specific lysosomal enzyme, a-1-iduronidase, which is required for a normal degradation of glycosaminoglycans and characterized by progressive multiorgan accumulation of the same. 1 It is typically associated with progressive craniofacial, skeletal, and cardiac involvement, which increases the risk of anesthesia. 1,2 We report a case of a difficult airway scenario of a child with Hurler's syndrome, leading to an apparent cannot ventilate cannot intubate scenario, managed successfully with the help of a C-MAC video laryngoscope and pediatric D-blade.…”

“…1 It is typically associated with progressive craniofacial, skeletal, and cardiac involvement, which increases the risk of anesthesia. 1,2 We report a case of a difficult airway scenario of a child with Hurler's syndrome, leading to an apparent cannot ventilate cannot intubate scenario, managed successfully with the help of a C-MAC video laryngoscope and pediatric D-blade. Informed consent was obtained from the parents prior to submission for publication.…”

Anaesthesia management in craniovertebral junctional anomalies

“…During the transoral procedures, intubation is done by a reinforced ETT. [8] An alternative intubation technique is preferred, a video laryngoscope,[14] (angled video intubation laryngoscope); the distal blade has a 25° angle to guarantee a greater visualization or fibreoptic intubation. Good preparation of the nose using a vasoconstrictor is essential to avoid bleeding into the airway (which could make the intubation more difficult).…”

“…Results of the cardiac consultation should be communicated to both surgeon and anesthesiologist. [131415] The cardiac consultation should include assessment of current hemodynamic stability, provide recommendations on the need for additional medications or tests, suggest the level of postoperative care and may even uncover a need to defer the procedure. Preoperative cardiac assessment should identify any cardiac disease that would place the patient in the surgical high-risk category.…”