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

Gupta N, Rath GP, Bala R, Reddy BK, Chaturvedi A - Saudi J Anaesth (2012)

Bottom Line: Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans.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.

Affiliation: Department of Neuroanaesthesiology, All India Institute of Medical Sciences (A.I.I.M.S.), New Delhi, India.

ABSTRACT

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.

Child of Hurler syndrome with (a) frontal bossing (dolicocephalic) of head, and (b) short stubby fingers with wide wrist
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Figure 1: Child of Hurler syndrome with (a) frontal bossing (dolicocephalic) of head, and (b) short stubby fingers with wide wrist

Mentions: 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 1a], dorsolumbar kyphosis, and short stubby fingers with wide wrist [Figure 1b]. 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 μg/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.

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

Gupta N, Rath GP, Bala R, Reddy BK, Chaturvedi A - Saudi J Anaesth (2012)

Child of Hurler syndrome with (a) frontal bossing (dolicocephalic) of head, and (b) short stubby fingers with wide wrist
© Copyright Policy
Figure 1: Child of Hurler syndrome with (a) frontal bossing (dolicocephalic) of head, and (b) short stubby fingers with wide wrist
Mentions: 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 1a], dorsolumbar kyphosis, and short stubby fingers with wide wrist [Figure 1b]. 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 μg/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.

Bottom Line: Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans.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.

Affiliation: Department of Neuroanaesthesiology, All India Institute of Medical Sciences (A.I.I.M.S.), New Delhi, India.

ABSTRACT

Background: 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.

View Similar Images In: Results  - Collection
View Article: Pubmed Central -  PubMed
Show All Figures - Show MeSH
getmorefigures.php?pmc=3385265&rFormat=json&query=null&req=5