High rate of postoperative mortality in patients with mucopolysaccharidosis I: findings from the MPS I Registry

Arn, Pamela; Whitley, Chester B.; Wraith, J. Edmond; Webb, Hueldine; Underhill, Lisa; Rangachari, Lakshmi; Cox, Gerald F.

doi:10.1016/j.jpedsurg.2011.09.042

Cited by 32 publications

(28 citation statements)

References 34 publications

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“…The interpretation of these neuroimaging findings is somehow challenging, because a clear relationship with cognitive insufficiency in MPS patients is lacking [30,31], although mental retardation has been reported in the more severe forms of MPS 1 [32][33][34][35][36][37].…”

Section: Discussionmentioning

confidence: 99%

Characterization of Hearing Loss in Children with Mucopolysaccharidosis

Zanetti¹,

Vezzani²,

Berardino³

et al. 2018

An Excursus Into Hearing Loss

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Hearing impairment is common in patients with mucopolysaccharidoses (MPS) in the preschool age. Conductive or mixed hearing loss is the most frequent occurrence while the involvement of the inner ear or central auditory pathways may occur in more severe forms. A retrospective review of 82 children with MPS admitted at the Pediatric Department of the University of Milano Bicocca was performed to determine the incidence of otological symptoms. We focused particularly on audiological investigations in a subgroup of 47 children diagnosed before 6 years of age (MPS I, n = 11 patients; MPS II, n = 10; MPS III, n = 7; MPS IV, n = 14; MPS VI, n = 5). In 37 children, a magnetic resonance imaging (MRI) of the brain and cervical spine was also performed in order to correlate the audiological findings with the imaging of the middle and inner ear. A total of 40 out of 47 children (86%) showed some degree of hearing impairment: sensorineural or mixed hearing loss in 23 cases (48.93%) and retrocochlear in 4 (8.51%). MRI ascertained multiple CNS abnormalities in 13 (35.3%): dilated perivascular spaces in 5 (38.5%); dilated ventricular cavities in 5 (38.5%); demyelinated and gliotic areas in 3 (23.0%). Conversely, one-fourth of the children's inner ears showed some morphological anomaly (24.3%).

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Section: Discussionmentioning

confidence: 99%

Characterization of Hearing Loss in Children with Mucopolysaccharidosis

Zanetti¹,

Vezzani²,

Berardino³

et al. 2018

An Excursus Into Hearing Loss

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“…A study comprising 932 patients enrolled in the MPS I registry that underwent a total of 4,762 procedures showed 30-day risk of death/procedure and death/patient rates of 0.7 % and 4.2 % (Arn et al 2012). Most serious anaesthetic complications occurring during surgery in MPS patients are associated with airway obstruction, with accompanying difficulty in ventilation and oxygenation, resulting in significant cardiovascular compromise.…”

Section: Anaesthetic Complications and Surgical Mortality In Mps Patimentioning

confidence: 99%

Anaesthesia and airway management in mucopolysaccharidosis

Walker

Belani

Braunlin

et al. 2012

J of Inher Metab Disea

129

139

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This paper provides a detailed overview and discussion of anaesthesia in patients with mucopolysaccharidosis (MPS), the evaluation of risk factors in these patients and their anaesthetic management, including emergency airway issues. MPS represents a group of rare lysosomal storage disorders associated with an array of clinical manifestations. The high prevalence of airway obstruction and restrictive pulmonary disease in combination with cardiovascular manifestations poses a high anaesthetic risk to these patients. Typical anaesthetic problems include airway obstruction after induction or extubation, intubation difficulties or failure [can’t intubate, can’t ventilate (CICV)], possible emergency tracheostomy and cardiovascular and cervical spine issues. Because of the high anaesthetic risk, the benefits of a procedure in patients with MPS should always be balanced against the associated risks. Therefore, careful evaluation of anaesthetic risk factors should be made before the procedure, involving evaluation of airways and cardiorespiratory and cervical spine problems. In addition, information on the specific type of MPS, prior history of anaesthesia, presence of cervical instability and range of motion of the temporomandibular joint are important and may be pivotal to prevent complications during anaesthesia. Knowledge of these risk factors allows the anaesthetist to anticipate potential problems that may arise during or after the procedure. Anaesthesia in MPS patients should be preferably done by an experienced (paediatric) anaesthetist, supported by a multidisciplinary team (ear, nose, throat surgeon and intensive care team), with access to all necessary equipment and support.Electronic supplementary materialThe online version of this article (doi:10.1007/s10545-012-9563-1) contains supplementary material, which is available to authorized users.

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“…[7][8][9] However, perioperative morbidity and mortality are dramatically higher among children with MPS/ ML. [4][5][6][9][10][11] In MPS type I, a perioperative morbidity of up to 30% 5,6 and a 30-day risk of death per procedure of approximately 0.7% 12,13 have been reported.…”

Section: Introductionmentioning

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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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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High rate of postoperative mortality in patients with mucopolysaccharidosis I: findings from the MPS I Registry

Cited by 32 publications

References 34 publications

Characterization of Hearing Loss in Children with Mucopolysaccharidosis

Characterization of Hearing Loss in Children with Mucopolysaccharidosis

Anaesthesia and airway management in mucopolysaccharidosis

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

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