[Bilateral congenital mydriasis with accommodation failure].

In the literature congenital mydriasis is described as a very rare condition and explained as a result of isolated aplasia of the iris sphincter muscle. Aplasia of the ciliary muscle was assumed to cause congenital accommodation insufficiency. A case of congenital mydriasis with lack of accommodation is presented. The first ophthalmological check-up was 2 weeks after surgery for a persistent ductus arteriosus Botalli. The girl was 15 weeks old. Her parents had watched her dilated pupils since birth. The diameter of both pupils was 6.5 mm. They did not react to light, lid closure, or conjunctival administration of pilocarpine solutions up to 1%. A refractive error of OD +3.0 D and OS +2.5 D was measured by retinoscopy. The hypermetropia was also uninfluenced by topical locarpine 1%. Two drops of pheylephrine 2.5% caused additional pupillary dilatation of 0.5 mm. Besides the lack of accommodation and pupillary constriction, all ocular findings were regular. No chromosomal abnormalities were found. No further cases of pupillary disorders are known in the family. These findings can only result from the lack of cholinergic sensitivity or aplasia of the pupillary sphincter and ciliary muscle. The infant was supplied with bifocals and sunglasses. The near correction was spontaneous. At the age of 15 months there was a grating acuity of 20/80, which is in the normal range, as measured by preferential looking.