Mucopolysaccharidosis (Hunters and Hurlers)
Background
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The mucopolysaccharidoses (MPS) are the result of a deficiency of lysosomal enzymes that cleave mucopolysaccharides (glycosaminoglycans)
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An accumulation of mucopolysaccharides occurs in the brain, heart, liver, bone, cornea, & tracheobronchial tree. There are seven types & several subtypes of MPS, each with various clinical presentations
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Multisystem diseases but airway is the main concern:
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difficult intubation 53%
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failed intubation 23%
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Considerations
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Review the patient's particular type of mucopolysaccharidoses & clinical features
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Airway:
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Difficult to impossible airway (BMV & DL) that worsens with time:
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Macroglossia
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Hyperplasia of adenoids, tonsils, pharyngeal tissue
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Friable tissues
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↓ TMJ
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Possible atlantoaxial instability (only for Morquio’s or MPS-IV)
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Difficult surgical airway (short neck, retrosternal trachea)
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Respiratory:
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RLD: recurrent pulmonary infections, pectus excavatum & kyphoscoliosis
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OSA, pulmonary hypertension
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Possible need for post-operative ventilatory support
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Cardiac:
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Cardiomyopathy
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Diffuse CAD from coronary artery deposition
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Valvulopathy: AI, MR
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Neuro:
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Potential developmental delay, uncooperative
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Dural thickening can result in compressive myelopathy
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Developmental delay
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Hydrocephalus
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Others:
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Hepatosplenomegaly
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Hepatic dysfunction
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Periop risk of hypoglycaemia
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Metabolic acidosis due to inability to convert lactic acid to glycogen (avoid ringer’s)
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Hemorrhagic diathesis due to platelet dysfunction
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Goals
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Safe establishment of airway:
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Consider awake fiberoptic intubation
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If uncooperative: spontaneous breathing fiberoptic intubation
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Surgical backup for rigid bronchoscopy & tracheostomy immediately available
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Avoid/minimize resp-depressants if possible
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Perioperative monitoring of of serum glucose, minimization of fasting times when possible
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Pre-operative identification & optimization of cardiac system