1032 Mixed sleep apnea management in a pediatric patient with Arnold Chiari type 1 and septo-optic dysplasia leading to hypopituitarism

Abstract

Abstract Introduction Mixed Sleep apnea and Central sleep apnea are commonly noted in a patient with Arnold Chiari Malformation and definitive treatment of Arnold Chiari Malformation is surgical decompression. Report of case(s) The patient at the time of diagnosis of Mixed sleep apnea was 7 years old. She had already undergone tonsillectomy and adenoidectomy at the age of 4 years. Due to continued Sleep-disordered breathing, PSG was done in 2016 which showed predominantly mixed and central events. MRI was done which revealed Chiari 1 malformation with cerebellar tonsils extending 6 mm below the foramen magnum. The patient also due to septo-optic dysplasia and hypopituitarism followed with pediatric endocrinology and was on supplementation of Desmopressin, Hydrocortisone, Levothyroxine, and Somatropin. The mother at the time of pregnancy was a substance abuser, was incarcerated and the primary care was assumed by the patient’s legal guardian. The legal guardian was not interested in surgery for Arnold Chiari Malformation as she feared long-term outcomes and requested alternate treatment options. The patient underwent another split study and started on CPAP. The patient did not tolerate the CPAP and was transitioned to BiPAP. During her last clinic visit, the patient’s compliance had improved and she had achieved therapeutic AHI. The patient was also counseled on the effects of GH supplementation and untreated sleep apnea and is regularly followed by Sleep Medicine and Pediatric Endocrinology. The patient after transitioning to BiPAP has responded well to treatment for Mixed sleep apnea. Despite being on GH supplementation, with careful monitoring of hormone levels by Pediatric Endocrinology and symptom control by Sleep Medicine, the patient has shown remarkable improvement. Conclusion Mixed and central sleep apnea secondary to Arnold Chiari Malformation responds well to surgical decompression with improvement in sleep-disordered breathing. In a pediatric patient with complicated medical history with needing stress dose steroids, not a candidate for surgery, and intolerant to CPAP, BiPAP as a treatment modality showed good control of Sleep Apnea. Support (if any)