Skip to main content

04-04-2012 | Genetics | Article

Airway management remains critical in children with Hurler syndrome


Free abstract

MedWire News: Children with mucopolysaccharidosis 1, or Hurler syndrome, remain difficult to intubate during anesthesia despite advances in treatment and airway-management techniques, show results from a retrospective case review.

Children with Hurler syndrome, a rare genetic disorder, have specific physiologic features that make airway management difficult, such as having a narrow nasal airway, large tonsils, a short and immobile neck, a large tongue, and stiff perilaryngeal tissue.

As drugs, monitoring, airway-management strategies, and devices have all improved in recent years, Thomas Harendza (Hannover Medical School, Germany) and colleagues carried out a retrospective case review to evaluate whether airway management of these children during anesthesia has improved since 1996, when the last retrospective review was published.

The researchers reviewed 41 anesthetic charts covering the 2004-2010 period of 10 children (seven male) with Hurler syndrome to assess whether airway problems occurred. The children were aged 5 years on average (range 6 months to 12 years) when anesthetized and all the children had stem cell transplantation to treat their disorder at the age of 2 years or younger.

The main reason for anesthesia was for diagnostic imaging such as magnetic resonance imaging or computed tomography scanning.

The team reports that mask ventilation was difficult in five of 41 anesthetics given (three of 10 children).

In total, 29 intubations took place. Direct laryngoscopy was described as difficult (Cormack and Lehane grade of at least 3) in 11 of 41 anesthetics (five of 10 children), and three direct laryngoscopy intubations failed and had to be resolved using laryngeal mask airway insertion or by using a videolaryngoscope.

The anesthesiologists used a laryngeal mask airway (LMA) on 11 occasions in order to avoid managing the airway invasively, and once when direct laryngoscopy was not deemed possible.

"The airway management of children with mucopolysaccharidosis 1 remains critical, despite advances in both treatment and airway management techniques," write Harendza et al in the European Journal of Anesthesiology, noting that problems with airway management did not appear to increase in older children.

They concede that "limitations due to the retrospective non-randomised and non-controlled design of our trial have to be considered," although larger studies are difficult to carry our due to the rare nature of the condition.

However, they suggest: "Alternative airway management devices have a place in the management of children with… [Hurler syndrome] particularly the LMA, which can be used instead of conventional intubation."

MedWire ( is an independent clinical news service provided by Springer Healthcare Limited. © Springer Healthcare Ltd; 2012

By Helen Albert

Related topics