Mucopolysaccharidosis type I (Hurler syndrome) and anesthesia: the impact of bone marrow transplantation, enzyme replacement therapy, and fiberoptic intubation on airway management

Kirkpatrick, Katherine; Ellwood, James; Walker, Robert

doi:10.1111/j.1460-9592.2012.03897.x

Cited by 44 publications

(52 citation statements)

References 14 publications

(16 reference statements)

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“…These therapies have a positive impact on pulmonary function, which may also reduce the anaesthetic risk. HSCT in MPS I patients before the age of 2 years appears to reduce airway complications and improve mask ventilation and intubation (Frawley et al 2012; Kirkpatrick et al 2012). However, the use of HSTC is limited in MPS types other than MPS IH (Rovelli 2008).…”

Section: Future Of Mps Management and Implications For The Anaesthetistmentioning

confidence: 99%

Anaesthesia and airway management in mucopolysaccharidosis

Walker

Belani

Braunlin

et al. 2012

J of Inher Metab Disea

124

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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Section: Future Of Mps Management and Implications For The Anaesthetistmentioning

confidence: 99%

Anaesthesia and airway management in mucopolysaccharidosis

Walker

Belani

Braunlin

et al. 2012

J of Inher Metab Disea

124

139

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“…Registry data show that almost half of children with MPS I undergo two or more surgeries until the age of four . Many clinical manifestations of MPS/ML, especially progressive airway obstruction, restrictive lung disease, thorax deformity, adenotonsillar hypertrophy, obstructive and central sleep apnea, macroglossia, cervical spinal canal stenosis, dens hypoplasia, atlantoaxial instability, spinal cord compression, and skeletal dysplasia, have important implications for anesthesia …”

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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“…For certain disorders, such as the MPS physical and occupational therapy are components of a comprehensive program to facilitate performance of activities of daily living. Special precautions are necessary in children with MPS who need anesthesia, because of risks associated with cardiopulmonary disease and cervical manipulation in cases with odontoid hypoplasia (40, 41). Pulmonary assistance (BiPAP or CPAP) provides significant relief for patients with MPS and sleep apnea, and for those with both infantile and late onset Pompe disease who suffer from intercostal and diaphragmatic muscle weakness (42, 43).…”

Section: Evaluation and Managementmentioning

confidence: 99%

Clinical Neurogenetics

Pastores

Maegawa

2013

Neurologic Clinics

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The lysosomal storage disorders (LSDs) are a clinically heterogeneous group of inborn errors of metabolism, associated with the accumulation of incompletely degraded macromolecules within several cellular sites. Affected individuals present with a broad range of clinical problems, including hepatosplenomegaly and skeletal dysplasia. Onset of symptoms may range from birth to adulthood. The majority are associated with neurological features, including developmental delay, behavioral/psychiatric disturbances, seizures, acroparesthesia, motor weakness, cerebrovascular ischemic events and extra-pyramidal signs. It should be noted that later-onset forms are often misdiagnosed as symptoms, which might include psychiatric manifestations, are slowly progressive and may precede other neurologic or systemic features. Inheritance is primarily autosomal recessive. For all subtypes, diagnosis can be confirmed using a combination of biochemical and/or molecular assays. In a few LSDs, treatment with either hematopoietic stem cell transplantation, enzyme replacement or substrate reduction therapy is available. Genetic counseling is important, so patients and their families can be informed of reproductive risks, disease prognosis and therapeutic options. Investigations of disease mechanisms are providing insights into potential therapeutic approaches. Symptomatic care, which remains the mainstay for most subtypes, can lead to significant improvement in quality of life.

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Mucopolysaccharidosis type I (Hurler syndrome) and anesthesia: the impact of bone marrow transplantation, enzyme replacement therapy, and fiberoptic intubation on airway management

Abstract: Managing the MPS1 patient continues to be a challenge but with treatment and newer forms of airway management it is improving.

Cited by 44 publications

References 14 publications

Anaesthesia and airway management in mucopolysaccharidosis

Anaesthesia and airway management in mucopolysaccharidosis

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

Clinical Neurogenetics

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