Sir,Mucopolysaccharidoses (MPS) are lysosomal storage disorders caused by the deficiency of enzymes required for the stepwise breakdown of glycosaminoglycans (GAGs), also known as mucopolysaccharides. Patients with MPS may develop complications during general anaesthesia (GA) due to the presence of airway obstruction, excessive secretions, a large tongue and an abnormal airway anatomy. Patients with MPS type IV, also known as Morquio syndrome, pose even greater challenge because of additional problems such as neck instability, restrictive pulmonary disease and end-organ damage.A 16-year-old male patient (weight: 27 kg; height: 135 cm) diagnosed with Morquio-Brailsford syndrome was posted for valgus osteotomy of femur. He was found to have short stature, small barrel chest with pectus carinatum, large head with small neck and kyphoscoliosis of lower thoracic and lumbar vertebrae [Figure 1a]. He was operated for congenital bilateral dislocation of hip at the age of 3 and 4 years, respectively, under GA for which no records were available. He had normal intellectual development and had cleared secondary school examination recently. Airway examination revealed Grade 2 macroglossia, an enlarged uvula and tonsils, Mallampati Grade II and adequate neck flexion with restricted extension [Figure 1b]. Examinations of the rest of the systems were normal. As the patient refused to receive spinal anaesthesia, awake fibreoptic intubation (AFOI) was planned. Both the nares were prepared and the patient was pre-medicated with glycopyrrolate 0.2 mg, midazolam 1 mg and fentanyl 50 μg. Attempt at inserting the fibreoptic bronchoscope (FOB) resulted in trauma leading to blood tinged secretions, which precluded the view. After two failed AFOI attempts, GA was induced with propofol 60 mg and Air-Q Intubating Laryngeal Airway™ (Cook gas LLC, Mercury Medical, Clearwater, FL, USA) #2.5 was inserted with manual inline stabilisation of cervical spine and its position was confirmed with FOB, capnograph and auscultation. A 5.5 mm ID cuffed endotracheal tube (ETT) was railroaded over FOB through the Air-Q ILA, and both the devices were secured in place [Figure 1c]. Surgery lasted for more than 2 h with stable intraoperative vitals. At the end of surgery, the ETT was removed and ventilation was continued via Air Q ILA. Neuromuscular blockade was reversed and Air Q ILA was removed once the patient was fully awake.
Figure 1
(a) Body habitus of the patient; (b) restricted neck extension and large Tongue with Mallampati Grade II (c) Air-Q ILA™ and endotracheal tube in place
(a) Body habitus of the patient; (b) restricted neck extension and large Tongue with Mallampati Grade II (c) Air-Q ILA™ and endotracheal tube in placeMPS type IV also known as “Morquio syndrome” is an autosomal recessive disorder caused by the deficiency of n-acetylgalactosamine-6-sulphate leading to deposition of large amount of keratan sulphate and chondroitin sulphate in various tissues of body. At birth, child with Morquio syndrome may appear healthy; however, as they grow, progressive mesenchymal deposition of GAG results in appearance of manifestations of the syndrome. The incidence of difficult intubation in patients with Morquio syndrome varies from 0% to 50%, and airway difficulty progresses as age advances due to accumulation of keratan sulphate.[1] Hence, previous anaesthesia records of surgery with no difficulty in airway could be misleading. In our case, the patient had previous history of anaesthetic exposure, but the record was not available. Case reports concerning the airway management in patient with Morquio syndrome have been reported in the literature.[2] Most authors recommended AFOI- or FOB-guided intubation after induction. However, FOB-guided intubation without any airway conduit may be extremely difficult as these patients may have a “hanging epiglottis” making passage of fibrescope toward the glottis impossible.[3] Dhanger et al. reported successful I-gel-guided fibreoptic intubation in patient with Morquio syndrome.[2] However, I-gel™ requires greater mouth opening for insertion and one may face difficulty in its removal after intubation.[4] In our patient, we decided to intubate as there was a possibility of change in the patient position to lateral decubitus during intraoperative period. After the failed AFOI attempt, our choice of Air-Q ILA™ was advantageous because of its ability to be inserted without any head extension, to be left in situ during ventilation through ETT and to be used as conduit for ventilation after ETT removal. The Air-Q ILA™ has proved to be a safe supraglottic airway device in children with difficult airway as it has low potential for trauma of the airway and it can be used as a facilitator for blind endotracheal intubation.[5] The special feature of Air-Q ILA include a larger diameter and shorter length of airway tube allowing intubation with a standard ETT of age appropriate size and removal of the device following intubation does not require aid of stabilizing rod.We used Air-Q ILA™-guided intubation as an alternative to AFOI in patients with Morquio syndrome. Insertion of Air-Q ILA™ did not require neck extension and was left in place to serve as an additional conduit for ventilation after extubation. Thus, Air-Q ILA™ provided safe intubation and extubation.
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