Airway management in Hurler's syndrome: A persistent challenge for anaesthesiologists

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From: Indian Journal of Anaesthesia(Vol. 60, Issue 8)
Publisher: Medknow Publications and Media Pvt. Ltd.
Document Type: Clinical report
Length: 1,393 words
Lexile Measure: 1470L

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Byline: Sukhyanti. Kerai, Vandana. Saith, Rakesh. Kumar, Saipriya. Tewari


The difficulty in airway management of Hurler's syndrome or mucopolysaccharidosis (MPS) type I patients has been described as the worst. [sup][1] These patients may develop serious complications under anaesthesia including airway obstruction leading to severe hypoxaemia, inability to ventilate or intubate and post-extubation problems. [sup][2] Despite many potential benefits of using supraglottic devices for primary airway management in these patients over endotracheal tube (ETT) intubation, their usage has been limited. We at our centre decided to insert a Proseal laryngeal mask airway (PLMA) in awake state in a patient of Hurler syndrome to circumvent the problem of the difficult airway.


A 15-year-old male child diagnosed as a case of Hurler's syndrome with bilateral corneal haze was scheduled for keratoplasty of the right eye. He exhibited characteristic features of a severe form of MPS type I including short stature, facial dysmorphism, protruded abdomen and multiple joint deformity of limbs and complaint of obstructive sleep apnoea for which he was using nasal bilevel positive airway pressure (BiPAP) device for the last 5 years. There was no history of any other significant illness or anaesthetic exposure.

On examination, the child was found to be of normal intelligence, cooperative and with stable vital parameters. His airway examination revealed reduced mouth opening of 2.5 cm, large tongue, receding chin, thyromental distance of 4.5 cm, modified Mallampati class 4 and adequate range of neck movements [Figure 1] and [Figure 2]. On systemic examination, significant findings were systolic murmur in aortic area on auscultation and hepatosplenomegaly on abdominal palpation. Routine laboratory haematological examinations were unremarkable. Chest X-ray showed cardiomegaly and on echocardiogram, thick calcified aortic valve with mild mitral regurgitation was found. The X-ray of cervical spines ruled out atlantoaxial instability.{Figure 1}{Figure 2}

During pre-anaesthetic visit, the patient was explained about the necessity to perform awake supraglottic device insertion or awake fibreoptic bronchoscopy (FOB) guided intubation to which he agreed. He was also asked to bring nasal BiPAP machine to the hospital on the day of surgery.

In the morning of surgery after...

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Gale Document Number: GALE|A460355826