The visual function of 26 patients with a diagnosis of primary infantile glaucoma was studied. Quantitative visual acuities were determined in 15 patients. Sixteen eyes in these patients achieved 20/20 to 20/50 visual acuity and five additional eyes had 20/60 to 20/200. Thirteen eyes in young patients were noted to have central and steady fixation and four eyes exhibited eccentric fixation. Seven eyes were considered to have decreased visual acuity on the basis of anatomical defects. The remainder of the patients had decreased visual acuities secondary to amblyopia or large refractive errors. Amblyopia responded to treatment in six of 12 patients.

Amblyopia↗

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Hyperplastic persistent pupillary membrane--surgical management.

Two infants, aged three days and six weeks, respectively, presented with dense extensive persistent pupillary membranes as sporadic isolated findings. A clear central pupillary aperture was unable to be achieved with mydriatic agents. The patients were treated by surgical removal of the membranes at age five days and six months respectively. Surgery was without complication and visual development has been normal. We suggest that early intervention in the first weeks or months of life is necessary to prevent the development of amblyopia. If mydriasis is unsuccessful, surgery is recommended followed by optical correction and occlusion therapy as necessary.

Anterior Chamber↗

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Ocular abnormalities in terminal deletion of the long arm of chromosome seven.

Patients with distal deletions of the long arm of chromosome 7q have been reported with a variety of non-specific findings. Ocular findings have been limited to hypertelorism, palpebral fissure abnormalities, strabismus and speckled irides and no intraocular pathology with the exception of one case with unilateral optic nerve coloboma. We report a patient with terminal deletion of the long arm of chromosome 7 with marked ocular abnormalities including severe microphthalmus and large retinal colobomas.

Chromosome Deletion↗

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Epibulbar osseous choristoma and ectopic lacrimal gland underlying a dermolipoma.

A 6-month-old girl underwent surgical excision of an epibulbar mass which had the clinical appearance of a dermolipoma. Intraoperatively, a separate underlying mass was uncovered which was firmly adherent to the sclera. Histopathologic examination confirmed the superficial lesion to be a typical dermolipoma, and the underlying lesion to be an epibulbar osseous choristoma with associated adherent ectopic lacrimal gland tissue. The clinical features, management, and histopathology of this new association are reviewed.

Choristoma↗

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Management of posterior lenticonus.

Posterior lenticonus is associated with progressive lens opacification. We report a series of 40 consecutive patients (41 eyes) with posterior lenticonus who were treated by three pediatric ophthalmologists between 1974 and 1988. Prior to cataract surgery, appropriate patients were treated with atropine dilatation, spectacle correction including bifocals for refractive errors, and amblyopia occlusion therapy. Indications for surgery were a measured decrease in visual acuity, the loss of a central fixation reflex, or the onset of strabismus. The age at which cataract surgery was performed ranged from 2 months to 12 years (mean 4 years, 6 months). Nineteen eyes (49%) achieved postoperative acuities in the 20/20 to 20/40 range, 7(18%) eyes achieved 20/50 to 20/100, 4 (10%) eyes achieved 20/200, and 4 (10%) eyes achieved less than 20/200. Two (5%) young patients had central, steady, and maintained visual fixation reflexes and 3 (8%) additional patients had central, steady, but not maintained reflexes. After cataract surgery and aphakic optical correction, amblyopia was present in 84% of patients and strabismus was present in 51% of patients. The patients in this study had sufficient optical distortion to produce amblyopia. Cataract removal and optical correction alone did not correct the vision; occlusion therapy for amblyopia was required. Earlier surgery may be indicated to prevent visual deprivation amblyopia in patients with posterior lenticonus.

Amblyopia↗

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Idiopathic tractional corectopia.

Four infants were referred for congenital unilateral corectopia. In each case, the abnormal position of the pupil was caused by a fibrous structure that tethered the iris pupillary margin to the peripheral cornea. No patients showed characteristics of intrauterine infection, Rieger anomaly, ectopia lentis et pupillae, or iris coloboma. Amblyopia was not present in any of the patients. Three children demonstrated progression of the corectopia in the first 6 months of life. Two who developed shallow anterior chambers were treated surgically, one with an Nd:YAG laser and the other with incisional surgery. The third was treated with medical mydriasis. All four children have had favorable visual outcomes to date. The origin of the tethering strands is unclear but may be related to incomplete regression of vessels from the embryologic vascular system. We recommend medical or surgical intervention for unilateral corectopia when the pupillary aperture is displaced peripheral to the central visual axis or when the position of the iris threatens angle structures. Prophylactic occlusion therapy may also be indicated.

Female↗

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Ocular pathology of Lowe's syndrome in a female infant.

The ocular pathologic findings are presented in a case of a female infant with clinical and biochemical evidence of Lowe's syndrome. A small but increasing number of reports suggest that the mode of inheritance is not always sex-linked. The ocular histopathologic findings in the present case parallel those previously described for this syndrome. While the pathologic findings in other systems are variable, it is thought that the constellation of lens and anterior segment changes are sufficiently characteristic to identify cases of Lowe's syndrome.

Anterior Chamber↗

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Massive congenital ocular toxoplasmosis.

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Brain↗

PubMedDate not supplied

The surgery and results following traumatic cataracts in children.

Ninety eyes undergoing surgery for the removal of traumatic cataracts have been evaluated. In this group, the visual results relate to the coincident complications and amblyopia. Most commonly, they may be related to the extent of the preoperative trauma to the injured eye or to the age of the patient at the time of the injury. Forty-eight eyes (54%) achieved an acuity of 20/20 to 20/80 with 38 (42%) eyes achieving acuity of 20/20 to 20/30. A visual result of 20/80 or better was obtained in 12 eyes of the 29 patients six years of age or less.

Adolescent↗