1993
DOI: 10.1111/j.1460-9592.1993.tb00092.x
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Anaesthetic implications of mucolipidosis

Abstract: The anaesthetic implications of mucolipidosis in three siblings is reported. Two of the three children e>;hibit marked difficulty with their airways under anaesthesia and all three have narrow tracheas. Surgery and anaesthesia should be undertaken with caution in patients with a mucolipidosis.

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Cited by 6 publications
(10 citation statements)
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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%
See 1 more Smart Citation
“…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%
“…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%
“…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%