Tonic pupil aociated with congenital neuroblatoma, hirchprung dieae, and central hypoventilation yndrome

PURPOE: To report the aociation of tonic pupil, congenital neuroblatoma, Hirchprung dieae, and central hypoventilation yndrome. METHOD: Cae report. REULT: Two infant with central hypoventilation yndrome and Hirchprung dieae were evaluated for dilated, nonreactive pupil preent ince birth. In both cae, pharmacologic teting with dilute pilocarpine confirmed denervation uperenitivity conitent with tonic pupil. The diagnoi of congenital neuroblatoma wa ubequently etablihed in both infant. CONCLUION: The cloe aociation of thee condition in thee two young children upport the concept of a common neural cret abnormality being preent. A careful ytemic evaluation to rule out congenital neuroblatoma hould be performed in any young child who preent with tonic pupil in the etting of central hypoventilation yndrome and Hirchprung dieae