Biomedical subjects
Publications and source records attributed to G A Chrousos.
Head turning in patients with spasmus nutans seems to transiently dampen the nystagmus. This was evident in two children by clinical observation and electrooculographic recording. The suppression of the nystagmus may have been mediated by the vestibular system.
To our knowledge this is the first reported case of cyclopentolate induced bilateral acute angle-closure glaucoma associated with the Weill-Marchesani syndrome without lens subluxation. The diagnosis of Weill-Marchesani syndrome was not initially suspected, and Pilocarpine was used in an attempt to break the acute attack. Treatment with Pilocarpine-induced myosis only worsened the glaucoma by causing a severe pupillary block. This case is important because it demonstrates that mid-dilatation and cycloplegia can induce acute glaucoma in patients with the Weill-Marchesani syndrome and points out the importance for considering the diagnosis of Weill-Marchesani syndrome in any case of acute glaucoma in childhood.
Near-evoked nystagmus was evident in two children with spasmus nutans by clinical observation and electro-oculographic recording. In one child the nystagmus appeared to be evoked by fusional convergence and in the other by convergence-accommodation. These cases represent an atypical form of spasmus nutans in which the nystagmus is modulated by centers controlling visuomotor changes with near viewing.
Ten patients, ages 10 to 19, with accommodation insufficiency are reported. All patients were in good health and asymptomatic, except for asthenopia during near visual activities. Each patient's amplitude of accommodation was measured and found to be considerably below the minimal normal for their respective ages (an average of 6 diopters). Only three patients had associated convergence insufficiency. No etiology for the diminished accommodation was suggested by history or could be identified by careful examination. All patients were successfully managed optically with bifocals or reading glasses, although three required the addition of base-in prisms for the near exodeviation. Near vision testing and determination of the near point of accommodation should be part of the pediatric ophthalmologic examination in all patients with complaints referable to their reading and visual performance at near.
The malformations in the amniotic band syndrome (ABS) are due to entrapment of fetal parts by fibrous band in the amniotic sac. Limbs are most commonly affected followed by craniofacial defects in one third of patients. Ocular defects include corneal leukomas and lid colobomas often contiguous with facial clefts, strabismus, hypertelorism, and microphthalmos. Unilateral chorioretinal defects or lacunae are rare findings in the ABS. We report a female infant with such a lacunar defect along with central nervous abnormalities, and discuss the differential diagnosis and the embryopathic implications.