Anesthetic management in children with Hurler's syndrome undergoing emergency ventriculoperitoneal shunt surgery

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Date: April-June 2012
From: Saudi Journal of Anaesthesia(Vol. 6, Issue 2)
Publisher: Medknow Publications and Media Pvt. Ltd.
Document Type: Article
Length: 1,761 words
Lexile Measure: 1490L

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Byline: Nidhi. Gupta, Girija. Rath, Renu. Bala, Bapura. Reddy, Arvind. Chaturvedi

Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans. Patients with MPS I (Hurler's syndrome) present as one of the most difficult airway problems to be managed by anesthesiologists. Hydrocephalus with increased intracranial pressure is a frequent complication seen in these patients. The rarity of the syndrome accounts for the small number of case reports and anecdotal information on the intracranial manifestations of this syndrome. We describe the successful anesthetic management of 2 children with Hurler's syndrome who underwent ventriculoperitoneal shunt surgery for acute hydrocephalus.


Mucopolysaccharidoses (MPSs) often present with difficulties in managing the airway. [sup][1],[2] Among the various subtypes, Hurler's (severe MPS I) and Hunter (MPS II) syndromes tend to be most challenging in this regard. [sup][3] Hydrocephalus with increased intracranial pressure (ICP) is a frequent complication seen in patients with Hurler's phenotype. [sup][2],[4],[5],[6],[7] These patients may require emergency ventriculoperitoneal (VP) shunt surgery to reduce an increased ICP. Here, we describe successful anesthetic management in 2 such children who underwent insertion of VP shunt for acute hydrocephalus and the literature in this context has been reviewed.

Case Reports

Case 1

A 3-year-old boy, weighing 11 kg, was admitted with complaints of headache and vomiting for 2 days. He had a history of delayed developmental milestones and head banging. The child was diagnosed as a case of Hurler's syndrome and was on enzyme replacement therapy. A computed tomographic (CT) scan showed communicating hydrocephalus with dilatation of ventricles. The child had coarse facial features, large tongue, short neck, frontal bossing with a dolicocephalic head [Figure 1]a, dorsolumbar kyphosis, and short stubby fingers with wide wrist [Figure 1]b. Hepatospleenomegaly was ascertained on abdominal examination. The head circumference was 49 cm. Airway examination revealed modified Mallampati class III. The child was premedicated with atropine 0.04 mg/kg orally 30 min prior to induction of anesthesia. Anesthesia was induced with fentanyl 2 [micro]g/kg and propofol 2 mg/ kg maintaining spontaneous ventilation. After ensuring adequate mask ventilation, rocuronium 1 mg/ kg was given. A Cormack-Lehane (CL) grade IV was achieved in first laryngoscopy. The child was intubated in second attempt with the help of gum elastic bougie with an endotracheal tube (ETT) of 4.5 mm ID. Correct position of the ETT was confirmed. The intraoperative course was uneventful at the end of which trachea was extubated.{Figure 1}

Case 2

A 4-year-old girl, weighing 11 kg, a known case of Hurler's syndrome was admitted with complaints of vomiting and decreased appetite for 5 days. A CT scan of head revealed communicating hydrocephalus with periventricular ooze. The child was...

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Gale Document Number: GALE|A293468240