Airway management and perioperative adverse events in children with mucopolysaccharidoses and mucolipidoses: A retrospective cohort study

Abstract

Background Children suffering from mucopolysaccharidoses (subtypes I, II, III, IV, VI, and VII) or mucolipidoses often require anesthesia, but are at high risk for perioperative adverse events. However, the impact of the disease subtype and the standard of care for airway management are still unclear. Aims This study aimed to assess independent risk factors for perioperative adverse events in individuals with mucopolysaccharidoses/mucolipidoses and to analyze the interaction with the primary airway technique implemented. Methods This retrospective study included individuals with mucopolysaccharidoses/mucolipidoses who underwent anesthesia at two high‐volume centers from 2002 to 2016. The data were analyzed in a multivariate hierarchical model, accounting for repeated anesthesia procedures within the same patient and for multiple events within a single anesthesia. Results Of 141 identified inpatients, 67 (63 mucopolysaccharidoses and 4 mucolipidoses) underwent 269 anesthesia procedures (study cases) for 353 surgical or diagnostic interventions. At least one perioperative adverse event occurred in 25.6% of the cases. The risk for perioperative adverse events was higher in mucopolysaccharidoses type I (OR 8.0 [1.5‐42.7]; P = .014) or type II (OR 8.8 [1.3‐58.6]; P = .025) than in type III. Fiberoptic intubation through a supraglottic airway was associated with the lowest risk for perioperative adverse events and lowest conversion rate. Direct laryngoscopy was associated with a significantly higher risk for airway management problems than indirect techniques (estimated event rates 47.8% vs 10.1%, OR 24.05 [5.20‐111.24]; P < .001). The risk for respiratory adverse events was significantly higher for supraglottic airway (22.6%; OR 31.53 [2.79‐355.88]; P = .001) and direct laryngoscopy (14.8%; OR 14.70 [1.32‐163.44]; P = .029) than for fiberoptic intubation through a supraglottic airway (2.1%). Conclusions The disease subtype and primary airway technique were the most important independent risk factors for perioperative adverse events. Our findings indicate that in MPS/ML children with predicted difficult airway indirect techniques should be favored for the first tracheal intubation attempt.

Perioperative management of patients with Mucolipidosis II and III: Lessons from a case series

“…This patient was then successfully intubated using a GlideScope® videolaryngoscope. Again, our findings support the conclusion of Dohrmann 9 that a specific type of indirect intubation technique cannot be recommended in all children with MPS/ML and thus anesthetists caring for these complex children must be skilled in a range of advanced pediatric airway management techniques.…”

“…Thus, a fiber‐optic or videolaryngoscope technique are often utilized first‐line for potentially difficult airways. Our practice is supported by the findings of a recent cohort study of airway management and perioperative adverse events in children with MPS and ML where Dohrmann et al found that direct laryngoscopy was associated with a much higher probability for airway management problems and respiratory adverse events compared to indirect techniques 9 …”

“…These include the typical “coarse” facial dysmorphism of frontal bossing, a flattened wide nasal bridge, gingival hypertrophy, and macroglossia. Difficulty with airway management has been widely reported in the literature 3–10 and is multifactorial; storage material deposits in the airway of MLII patients have been described causing thickening of the epiglottis, larynx, trachea, and tongue base 3,11 . One series of patients with MLII found that 100% studied patients had sleep‐disordered breathing 12 .…”

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

“…A high rate of failed direct laryngoscopy and respiratory events prove an especially difficult airway and call for advanced airway management in these patients [5,10]. We recently published that the anaesthetic risk is higher in patients with MPS types I or II than with MPSIII and suggested using indirect intubation techniques as the first-line airway approach in individuals with MPS [11].…”

Aspects of Surgical Treatment of Spinal Pathology in the Patient with Gurler Syndrome

“…Мукополисахаридоз (МПС) -орфанное заболевание, относящееся к группе лизосомных болезней накопления. Известно [1][2][3][4][5][6]. Накопление гликозаминогликанов (ГАГ) вызывает задержку физического развития, умственную отста-На фоне бурно прогрессирующей кифотической деформации наблюдалось нарастание неврологического дефицита.…”