Safe intubation in Morquio-Brailsford syndrome: A challenge for the anesthesiologist
A 3-year-old child weighing 9kg diagnosed with Morquio-Brailsford syndrome was posted for MRI and CT of cervical spine after she had developed sudden weakness of the upper and lower limbs with inability to walk. She was diagnosed as Morquio syndrome at the age of 1 year, with a delay in achieving developmental milestones. She had short neck, pectus carinatum thoracolumbar kyphoscoliosis with a suspected unstable cervical spine based on clinical deterioration and cervical spine X-ray []. Cardiovascular, abdominal, and hematological examinations were unremarkable. Airway assessment could not be done as the child was uncooperative. A high-risk informed consent was taken in view of difficult airway and possible cervical instability which could worsen cord compression during the procedure.\nIntubation was done inside the operation theatre (OT) in presence of senior anesthesiologists and then shifted to the MRI suite. Different sizes of endotracheal tubes (ETTs), laryngeal mask airways (LMAs), proseal LMA, and pediatric fibreoptic bronchoscope were kept in the difficult airway cart. An intravenous (IV) access was secured and child was shifted to OT after administration of 0.1mg IV glycopyrrolate and 0.5mg IV midazolam. Baseline vitals were heart rate of 150/min, blood pressure 94/60mmHg and oxygen saturation (SpO2) of 99%. Anesthesia was induced with titrating doses of propofol along with 3% sevoflurane in 100% oxygen; ensuring spontaneous ventilation was maintained while monitoring SpO2 and end tidal carbon dioxide (EtCO2). A total of 10μg IV fentanyl was given and after adequate depth of anesthesia, a size 2 classic LMA which was shaped like a proseal LMA using stylet was gently inserted with manual in-line stabilization (MILS) []. LMA position was confirmed by adequate chest expansion and presence of capnogram. A right-angle tracheal tube connector with seal was attached to the LMA []. This allowed fibreoptic bronchoscope to be inserted through the LMA while the child was ventilated with 100% oxygen and 2%-3% sevoflurane.\nA 4.0 mm uncuffed portex ETT was railroaded over the 2.4 mm Outer Diameter (OD) pediatric bronchoscope. Then the bronchoscope was introduced through the lumen of LMA into the trachea and the ETT was advanced over the bronchoscope. On connecting the breathing circuit to the ETT, though a typical capnogram was present but visualization through the fibreoptic scope showed that the ETT tip was just above the glottis, even on introducing the ETT to the maximum depth possible in the presence of right-angle tracheal tube connector. Eventually, the right-angle tracheal tube connector was removed and reintubation was done using similar technique, now ensuring that the tip of ETT was placed about 2cm above carina by fibreoptic visualization.\nThe ETT with LMA was fixed as a single unit after confirming the ETT tip position by visualization and auscultation. Care was taken to avoid kinking of the ETT at the point of exit from LMA. Throughout the procedure, saturation was maintained between 96% and 100%. A total of 3mg IV atracurium was then given and child was shifted to MRI suite on oxygen trolley with monitors. Anesthesia during the MRI and CT was uneventful, after which child was shifted to pediatric intensive care unit and was extubated in ICU. MRI showed cord compression involving the cervicomedullary junction, hypoplastic split atlas, and posterior atlantooccipital fusion []. CT confirmed hypoplasia of C1 vertebra, split atlas, and nonossification of the dens []. Child was then put on a cervical collar and eventually discharged.