Boy with Coffin-Lowry syndrome associated with spinal cord injuries
Kawana, Y.; Okamura, K.; Kurahashi, K.
Masui. Japanese Journal of Anesthesiology 63(2): 203-205
2014
ISSN/ISBN: 0021-4892 PMID: 24601120 Document Number: 677238
A 12-year-old male patient with Coffin-Lowry syndrome was scheduled for posterior cervical decompression and fusion for cervical spinal injuries. The patient had features of Coffin-Lowry syndrome including mental retardation, prominent forehead, a short nose with a wide tip, a wide mouth with full lips, short stature, microcephaly, and kyphoscoliosis. We anticipated major troubles related to anesthesia such as difficult ventilation and intubation, communication difficulty during induction and extubation, and difficulty in using a naso-pharyngeal airway. In addition, we had to stabilize neck alignments during intubation because cervical vertebrae were unstable and spinal cord has already been injured. Therefore, we scheduled slow induction with sevoflurane maintaining spontaneous respiration. As we found the full mouth opening of the patient after the induction, we inserted an intubating laryngeal mask, through which ventilation was successfully maintained. A tracheal tube was inserted through the intubating laryngeal mask. When the surgery was completed, we extubated using a tube introducer in the trachea. As we found that the patient's airway was open, we removed the introducer. In conclusion, with a thorough planning of the anesthetic management, we successfully managed anesthesia for cervical spinal surgery in a patient with Coffin-Lowry syndrome.