A Case of Ohtahara Syndrome-related False Median Cleft Lip with Ventilation Difficulty after Palatoplasty

Abstract

False median cleft lip is often associated with dysplastic brain, epilepsy, and metabolic endocrine abnormalities. The condition is often associated with a poor life expectancy. However, several recent reports indicated good results from invasive surgical treatments. When general anesthesia is possible in cooperation with the pediatric department, surgery is implemented for epilepsy treatment and improvement of the patient's general condition. In this study, we reported our experience with a patient who underwent palatoplasty for a false median cleft lip and developed frequent epileptic seizures immediately after extubation, which caused glossoptosis and difficulty in mask ventilation. The patient was reintubated and required intensive care treatment.
A 1-year 6-month-old male patient had had epileptic seizures from birth and false median cleft lip. Rhinocheiloplasty was performed at 4 months old. At the age of 1 year 10 months when palatoplasty was performed, he developed apnea after the operation. He was reintubated and required intensive care treatment, and subsequently underwent tracheostomy to keep his breathing stable.