CONGENITAL CENTRAL HYPOVENTILATION SYNDROME - OCULAR FINDINGS IN 37 CHILDREN

Background: Congenital central hypoventilation syndrome (CCHS) is a ra re cause of central sleep apnea. Although ophthalmic abnormalities hav e been reported, the ocular findings have not been discussed in detail . Methods: We examined or obtained the records of 37 children with CCH S. Results: Twenty-seven patients were found to have abnormal pupils, most of which were miotic and reacted poorly to light. In 18 cases, th e anterior surface of the iris was unusually smooth. Ten of the childr en with abnormal pupils also demonstrated light-near dissociation. Twe nty had strabismus of various types, and 18 showed evidence of converg ence insufficiency. Conclusions: The high incidence of strabismus, pup illary abnormalities, and convergence insufficiency may be a result of neurologic defects in the midbrain.