Neurodevelopmental outcome and respiratory management of congenital central hypoventilation syndrome: a retrospective study [post]

Tomomi Ogata, Kazuhiro Muramatsu, Kaori Miyana, Hiroshi Ozawa, Motoki Iwasaki, Hirokazu Arakawa
2020 unpublished
Background: Congenital central hypoventilation syndrome (CCHS) is a rare disease characterized by sleep apnea. Anoxia often occurs soon after birth, and it is important to prevent anoxia-mediated central nervous system complications; however, data on the relationship between respiratory management and the prognosis for intellectual development of patients with CCHS is not well yet investigate. Methods: We performed a retrospective chart review cohort study of patients with CCHS in Japan. We
more » ... HS in Japan. We investigated the risk and prognostic factors for developmental outcomes and examined the disease in terms of its symptoms, diagnosis, complications, and treatment. Results: Of the 123 patients with CCHS included in the survey, 88 patients were 6 years old and older. They were divided into two group based on their intelligence quotient. Those treated using positive-pressure ventilation via tracheostomy in the first three months of life had a better developmental prognosis than those managed via tracheostomy after three months of age and those treated by ventilation using mask (OR = 3.80; 95% CI: 1.00–14.37, OR = 4.65; 95% CI: 1.11–19.37). There was no significant difference in physical development (P = 0.64).Conclusions: The best respiratory treatment for patients with CCHS is ventilation via tracheostomy, initiated ideally before the age of three months.
doi:10.21203/ fatcat:zwyaxudsbvc5ljsdsf7seb3qce