Anaesthetic implications of mucolipidosis

Baines, David; Street, Neil; Overton, John H.

doi:10.1111/j.1460-9592.1993.tb00092.x

Cited by 6 publications

(10 citation statements)

References 6 publications

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“…Although case numbers for very rare disease are too low to draw clear conclusions, we found a high rate of perioperative adverse events in children with ML. Current knowledge concerning anesthesia in ML is based on only very few reports …”

Section: Discussionmentioning

confidence: 99%

“…MPS III was associated with the lowest rate of perioperative adverse events in our study. Airway management was usually [24][25][26] Male gender was found to be an independent risk factor for perioperative adverse events. For the interpretation of this finding, the genetic pathway of MPS/ML should be taken into account.…”

Section: The Disease Subtype Is An Independent Risk Factormentioning

confidence: 99%

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

Dohrmann

Muschol

Sehner

et al. 2020

Pediatric Anesthesia

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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.

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Section: Discussionmentioning

confidence: 99%

Section: The Disease Subtype Is An Independent Risk Factormentioning

confidence: 99%

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

Dohrmann

Muschol

Sehner

et al. 2020

Pediatric Anesthesia

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“…These issues may be further magnified by musculoskeletal malformations with limited neck movement. Mucopolysaccharide deposits in the supraglottis and anterolateral displacement of the larynx have led to difficulty with mask ventilation and other attempts at direct laryngoscopy, flexible endoscopy, and indirect laryngoscopy including indirect video laryngoscopy using the Glidescope ® [13][14][15][16]. A previous study which looked at the practical challenges faced in securing airways revealed that the ML II group of children required an average of 3.6 attempts to achieve endotracheal intubation compared to 1.2 in the control group [13,17].…”

Section: Progressive Gingival Hypertrophy Gives Rise To An Open Bitementioning

confidence: 99%

“…To date, there are only two previous reports of anesthetic care in patients with ML II (Table 1 [14,15]). When considering the perioperative challenges of such patients, many issues parallel those of patients with MPS including airway, respiratory and cardiac involvement.…”

Section: Progressive Gingival Hypertrophy Gives Rise To An Open Bitementioning

confidence: 99%

Anesthetic Management of a 13-Year-Old Adolescent With Mucolipidosis Type II for Total Hip Arthroplasty

et al. 2017

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Mucolipidosis II (ML II) or inclusion cell disease is a rare lysosomal storage disorder, inherited as an autosomal recessive trait. Deficiency of the lysosomal transport enzyme, N-acetylglucosamine-1-phosphotransferease, results in the intracellular accumulation of macromolecules (mucopolysaccharides and mucolipids) in the lysosome which leads to cellular dysfunction and a multi-system disorder. Manifestations are present at birth including muscle hypotonia, a weak cry, and failure to thrive. Additional physical signs include hip dislocation, inguinal hernia, hepatomegaly, limitation of joint movement, and cutaneous changes. Coarse facial features and skeletal abnormalities become more conspicuous with time. A rapidly progressive psychomotor deterioration, developmental delay and growth failure are often noted with a limited life span of 10 years. Due to the aforementioned physical abnormalities, surgical and anesthetic care may be required. We present a 13-year-old girl with ML II who required anesthetic care for a total hip arthroplasty due to chronic right hip dislocation. Previous reports of anesthetic care for these patients are reviewed, end-organ involvement is discussed, and options for anesthetic care are presented.

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“…The important point in managing patients with syndromes or congenital abnormalities is to be aware of possible problems so that one is prepared to deal with them if they occur. Anaesthetic implications of mucolipidosis SIR-We read the case report Anaesthetic implications of mucolipidosis (Baines, Street & Overton 1993) with great interest, especially because only few institutions are able to report about broad experience related to anaesthesia for patients with a specific but rare disease.…”

Section: Authors' Replymentioning

confidence: 99%

Correspondence

1994

Pediatric Anesthesia

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Anaesthetic implications of mucolipidosis

Cited by 6 publications

References 6 publications

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

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

Anesthetic Management of a 13-Year-Old Adolescent With Mucolipidosis Type II for Total Hip Arthroplasty

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