The anesthetic management of a child with ohtahara syndrome and severe stridor: a case report.

BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory stridor. CASE PRESENTATION: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient's breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient's respiratory and cardiovascular function were stable. CONCLUSIONS: This report documents the unusual finding of severe inspiratory stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.

The effects of thoracoplasty on immediate post-operative recovery in adolescent idiopathic scoliosis.

PURPOSE: The purpose of this study was to evaluate differences in pain, opiate utilization and oxygen (O2) consumption during the immediate post-operative course for patients undergoing posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) with and without thoracoplasty. METHODS: Patients aged 10-21 years old that had undergone PSF for AIS were identified from 1/1/16-8/1/18. A cohort of patients that did not have a thoracoplasty group was used as a control group. A standardized pain protocol (rapid response pathway) was implemented on all patients. A retrospective chart review was performed. Pre-operative, operative and post-operative data and SRS scores were analyzed. RESULTS: Forty-six patients (38 girls, 8 boys) were included, 23 in each group. The average age was 15.2 years (range 12.0-19.3) and BMI was 20.9 (range 13.1-37.6). Differences in visual analog scores, post-operative oxygen and narcotic consumption were not statistically significant on any POD. Total opiate utilization was 146.06 morphine milligram equivalents (MME) in the control group and 149.41 MME in the thoracoplasty group (p = 0.78). One-year SRS self-image scores were higher in the thoracoplasty group (4.24 vs 3.96) (p = 0.007). There was no difference in length of hospitalization (3.6 vs 3.5 days), levels fused (12.9 vs 12.9) or blood loss (334.1 vs 413.5 mL). There was one pleural effusion and no instances of intercostal neuralgia or pneumothorax in the thoracoplasty group. CONCLUSION: There is no increase in immediate post-operative pain, narcotic use or oxygen consumption when a thoracoplasty is performed with a PSF for AIS. Improved SRS self-image scores were seen after thoracoplasty.