Extreme hydrocephalus syndrome (kleeblattschdel syndrome; cloverleaf skull syndrome; hydrocephalus; chondrodystrophicus congenita)

General: Secondary obstruction of cerebrospinal fluid circulation caused by some primary disease such as maternal rubella, Rh incompatibility, or hydramnion; Arnold- Chiari syndrome has similar associated findings; almost all affected children are born dead.

Ocular: Exophthalmos with downward placement and downward rotation of the globes; propulsion of globes; upper lid retraction and lower lids covering almost half of the downwardly rotated cornea; nystagmus; strabismus; exposure keratitis; optic nerve atrophy.

Clinical: Extreme hydrocephalus; low-set ears; thin and spastic extremities with digital anomalies; convulsions; spina bifida.

Holtermuller K, Wiedemann HR. Kleeblattschadel syndrome. Med Wschr 1960; 14:439.

Resnick DK, et al. Surgical management of the cloverleaf skull deformity. Pediatr Neurosurg 1995; 22:29-37.

Rogers GL, et al. The management of the Kleeblattschadel syndrome. Ann Ophthalmol 1981; 13: 1173-l175.

Shiroyama Y, et al. The relationship of cloverleaf skull syndrome to hydrocephalus. Child Nerv Syst 1991; 7:382-385.

Walters EC, et al. Cloverleaf skull syndrome. Am J Ophthalmol 1973; 76:716.