P045: ANESTHETIC CONSIDERATIONS FOR A PATIENT WITH MORQUIO SYNDROME
Laura Valente, MD1,2; Keith Newton, MD1,2; Viet Sean Nguyen, BS3; Lu Zhang, MD1,2; Ettore Crimi, MD1,2; 1Department of Anesthesiology, College of Medicine, University of Central Florida, Orlando, FL, USA; 2 Ocala Health, Ocala, FL, USA; 3University of Central Florida College of Medicine, Orlando FL, USA
Introduction: Morquio syndrome is a lysosomal storage disease that affects 1 in 210,000 births, characterized by the inability to metabolize keratin sulfate that results in skeletal spondyloepiphyseal dysplasia (1). Abnormalities such as macroglossia, coarse facial features, prognathism, and atlantoaxial instability present challenges to the delivery of safe anesthesia (2).
Case Report: A 56-year-old male with a history of Morquio syndrome, pulmonary hypertension, COPD, smoking presented for an elective right ankle surgery. He had prior quadruple CABG and aortic valve replacement. The primary anesthetic plan was spinal anesthesia, with general anesthesia with video laryngoscopy as back up plan. A fiber optic bronchoscope was immediately available in the event no view was obtained with video laryngoscopy.
Preoperative medications included albuterol and ipratropium nebulizer treatment. While patient in sitting position, a spinal anesthesia was performed at L3-L4 level using a 24-gauge spinal needle with injection of 1.4 mL 2% bupivacaine. However, no adequate sensory block upon testing was achieved.
The patient was placed supine and airway topicalization with topical 4% lidocaine spray and blocks of the glossopharyngeal nerve and superior and recurrent laryngeal nerves were performed with 2% lidocaine. Midazolam and ketamine were used to maintain spontaneous ventilation. Video laryngoscopy showed a grade 2a Cormack view. Once a view of the vocal cords was established, general anesthesia was induced, a short acting muscle relaxant was administered, and the trachea was intubated with a 7.0mm cuffed endotracheal tube. General anesthesia was maintained with sevoflurane. Pressure control was used as the mode of ventilation. Emergence was uneventful. Patient was discharged home the same day.
Discussion: Atlantoaxial instability, restrictive respiratory disease, and kyphoscoliosis in patients with Morquio syndrome may present major challenges to the anesthesiologists in terms of airway, mechanical ventilation management, and placement of spinal anesthesia.
Spinal anesthesia can be a reasonable choice due to superb sensory and motor block while maintaining spontaneous ventilation in known difficult airway. Though, kyphoscoliosis can contribute to difficulty when administering. Regional anesthesia can avoid a challenging spinal and also reduce the risk of post operative pulmonary complications. However, the patient refused. When using regional or spinal anesthesia, the anesthesia provider must be ready to manage a difficult airway with general anesthesia in the event of failure.
Abnormal airway anatomy and limited neck extension can make the endotracheal intubation difficult and lead to potential cervical spine injury. We used a series of cranial nerve blocks to maintain spontaneous ventilation, while minimizing movement and trauma, until a view is confirmed with video laryngoscopy. Video laryngoscopy further reduces the likelihood of cervical spine injury. A lighted intubating stylet can be used to minimize neck extension (3), but is not available at our institution.
Restrictive lung pathology secondary to scoliosis, COPD, and smoking increase the risk of intraoperative barotrauma. Pressure control ventilation was selected to maintain lower intrathoracic pressure.
Conclusion: We would conclude that these patients require adequate preparation for difficult airway and video laryngoscopy should be used also to minimize risk of cervical trauma.