Management of a difficult airway in Hunters syndrome

Dear Editor,

We would like to report a 10-year old, 21 kg boy with a recurrent umbilical hernia scheduled for surgery. He was a diagnosed case of mucopolysaccharidosis (MPS) type 2 – Hunter’s syndrome. Preoperative evaluation revealed dysmorphic face, flattened nasal bridge, macroglossia, limited mobility of the mandible, and short neck [Figure 1]. Airway imaging revealed hypertrophy with narrowed airway passages.

Figure 1.

Preoperative image of the child illustrating macroglossia

We decided on general anesthesia as the contents of the hernia involved bowel loops whose manipulation increased the risk of aspiration. Sedative premedication was avoided. The airway was nebulized with 4% xylocaine. Inhalational induction with preservation of spontaneous ventilation followed by supraglottic airway device (SGAD) placement and fiberoptic bronchoscope (FOB)–guided intubation was planned. A video laryngoscope (VL) (C-Mac) and a tracheostomy were kept as a backup. After induction with sevoflurane, nasopharyngeal airway insertion of sizes 4 and 5 was attempted but failed; thus, nasal fiberoptic was not attempted. A reasonable chest-rise and capnography tracing were obtained using a jaw-thrust maneuver. Neither I-gel nor Proseal- laryngeal mask airways (LMA) could be seated properly. Laryngoscopy with VL revealed that the epiglottis could be visualized but not lifted. We then decided to attempt oral FOB under C-MAC guidance. The FOB was loaded and its tip was maneuvered into the trachea. During airway manipulation, passive oxygen insufflation was maintained. After securing the tube, the anesthesia was maintained with fentanyl, atracurium, and 1% isoflurane. The maneuvere took 7 min, and the minim saturation recorded was 84%. Extubation was performed after the child was fully awake, over an airway exchange catheter [Figure 2]. The perioperative course was uneventful.

Figure 2.

Postextubation image with airway exchange catheter in situ

MPS type 2 is a rare inherited disorder in which lysosomal storage defect leads to accumulation of glycosaminoglycans in soft tissue and the central nervous system.[1] which increase with age.

Pediatric patients are unwilling candidates for awake intubation. In this case, the nasopharyngeal airway could not be maneuvered because of probable hypertrophied adenoids, and we did not force the same to avoid trauma and loss of visualization of the airway. Our patient had three of the four risk factors described by Saito et al.[2] for difficult ventilation via SGAD. This is in contrast to results quoted by Madoff et al.[3] who documented easy placement of SGADs in patients of MPS. This highlights that no single strategy is failsafe for the management of such a case.

Combined FOB along with VL has been used previously in Cormack-Lehane grade 3 and 4 airways in adults.[4] There are a few case reports in children describing their combined use.[5] The VL acts as an airway adjunct for providing airway space for manipulation of FOB and a more panaromic view. The advantage offered by the fiberscope is its increased maneuverability. Simultaneous utilization of these two devices helps combine their advantages for securing the airway.

We would like to highlight that although the plan for airway management should be premediated upon, changing or unanticipated circumstances might need a change in their order or might warrant a combination of techniques for optimum results.

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Conflicts of interest

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