Brown's syndrome.
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Cranial Nerve Diseases↗
Biomedical subjects
R M Robb
Publications and source records attributed to R M Robb.
Outcome of treatment for bilateral congenital cataracts.
The outcome of treatment for bilateral congenital cataracts was studied retrospectively in a group of 51 patients. Two major categories of lens opacities were identified. In the first category, the opacities were extensive and visual impairment was evident early in the first year. These cataracts often occurred in eyes with small corneal diameters and poorly dilating pupils. Postoperative strabismus was nearly universal; nystagmus developed in over 50%; and late onset open-angle glaucoma developed in 8 of the 29 patients studied. Early surgery did not seem to abort the development of nystagmus in this group of patients. In the second category, the lens opacities were partial, often lamellar in configuration, and visual impairment was less severe. Surgery was usually performed after 3 years of age, with good visual results if the opacities were symmetrical and there was no nystagmus. No deprivation amblyopia developed in this group, even when surgery was delayed into the second decade. Strabismus developed postoperatively in about a third, but so far, no delayed open-angle glaucoma has been identified.
Adolescent↗
Outcome of treatment for bilateral congenital cataracts.
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Adolescent↗
Pinealoblastoma metastatic to the optic nerve.
We report a 10-year-old white girl who developed a pinealoblastoma 2 years before presenting to us with metastatic spread to the left optic nerve. She was treated for the original tumor with irradiation and chemotherapy. She presented to us with decreased vision in the left eye, unilateral disk edema, and diffuse left optic nerve swelling without increased intracranial pressure. Biopsy of the posterior optic nerve sheath was required for diagnosis. Vision was lost in the left eye, but the optic nerve swelling diminished after chemotherapy and additional radiation therapy.
Biopsy↗
Ocular dermoids.
The following types of ocular dermoids were seen in a retrospective study of 50 children with these lesions: 34 epibulbar dermoids, 12 lipodermoids, and 4 dermoid cysts. Forty-six percent had hemifacial microsomia, and one-half of these had additional stigmata of the Goldenhar variant of the syndrome. Ninety-seven percent of the dermoids were found in the temporal half of the globe; of these, 76 percent were in the inferolateral quadrant, straddling the corneoscleral limbus. The majority of children had an oblique astigmatism in the involved eye. Epibulbar dermoids may be removed by careful superficial keratectomy, but caution should be exerted in excising lipodermoids because of the risks of affecting ocular motility and tear secretion.
Adolescent↗
Special diagnostic and therapeutic modalities in pediatric ophthalmology.
Ophthalmology is a specialty rich in instrumentation used for special diagnostic and therapeutic functions. Many of these special modalities are appropriate to pediatric ophthalmology, and this article discusses them in summary fashion for those who may wish to know about the procedures but who may not be involved directly in their use.
Child↗
Probing and irrigation for congenital nasolacrimal duct obstruction.
I reviewed the results of probing for congenital nasolacrimal duct obstruction in a series of 107 patients, with special reference to age at the time of probing. Relief of tearing and discharge was achieved in 90% of patients with the first probing, and an additional 6% were cured after a second probing. Altered nasolacrimal duct anatomy seemed to account for probing failures rather than any delay in probing. Primary probing continued to be an effective treatment well after 2 years of age and was successful in two 5-year-old patients. Unsuccessful probings were usually apparent at the time of the initial probing and were characterized by difficulty passing the probe and subsequent inability to irrigate saline through the nasolacrimal system into the nose. Dacryocystorhinostomy was an effective secondary procedure in the few patients in whom probing was unsuccessful.
Age Factors↗
The broad clinical spectrum of early infantile esotropia.
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Amblyopia↗
Regional changes in retinal pigment epithelial cell density during ocular development.
The density of retinal pigment epithelial (RPE) cells in various parts of the posterior segment of developing human eyes was examined. The material for study consisted of 19 autopsy eyes, ranging in age from the sixth gestational month to 6 postnatal years. Whereas RPE cell density gradually increased in the macular area up to 6 months of age, cell density dropped in all other areas of the posterior segment through the first 2 postnatal years. The decrease in cell density was especially marked near the ora serrata. Mitotic figures were rarely seen and only found in the eyes of preterm infants. The author concludes that the increase in total surface area of the RPE during the period studied is primarily accounted for by changes in the regional density of the existing cell population. A centripetal shifting of cells toward the macular area from more peripheral areas would account for the increasing density in the posterior pole.
Child↗
An autopsy study of eye involvement in acute leukemia of childhood.
The eyes of 60 children who had died of acute leukemia between 1968 and 1977 at the Children's Hospital Medical Center have been examined pathologically. An attempt has been made to relate eye findings to the state of the systemic disease at the time of death. Eight of the 60 patients had leukemic retinal infiltrates and all eight had fulminant disease with terminal leukocyte counts over 100,000 per cubic millimeter and a high percentage of "blast" cells. Twenty-six patients (43%) had leukemic infiltration of the choroid that was inapparent clinically. Choroidal involvement was not correlated with high terminal leukocyte counts but was invariably associated with widespread infiltration of other organs. Four patients had optic nerve involvement; all four had coexistent meningeal leukemia. Isolated retinal hemorrhages could not be correlated with other parameters of the leukemic process.
Acute Disease↗
Studies on the development of the eye cup and optic nerve in normal mice and in mutants with congenital optic nerve aplasia.
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Animals↗
Internal ophthalmoplegia following inferior oblique myectomy: a report of three cases.
Three patients are reported in whom paralysis of accommodation and mydriasis (internal ophthalmoplegia) developed following inferior oblique myectomy. In all three patients, accommodation eventually returned to normal; in two, mild anisocoria persisted; in one patient, pupillotonia was noted after 2 1/2 years. The mechanism of this previously unreported surgical complication is thought to be excessive stretching of the nerve to the inferior oblique muscle with secondary trauma to the ciliary ganglion.
Accommodation, Ocular↗
Cyclic nucleotide phosphodiesterase activity in normal mice and mice with retinal degeneration.
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2',3'-Cyclic-Nucleotide Phosphodiesterases↗
Pathology of the Lens in Down's syndrome.
A pathological study of the lenses of 21 patients with Down's syndrome disclosed no distinctive abnormalities of the lens capsule, as had been reported previously. Five patients, 15 years of age or older, had focal cortical changes consistent with the characteristic flake-like opacities seen in patients with Down's syndrome after the first decade of life. Three patients with more extensive, visually significant lens opacities showed nonspecific cataractous changes. No abnormalities other than pathological artifacts were seen in the lenses of patients younger than 15 years of age.
Adolescent↗
Ocular manifestations of group A Niemann-Pick disease.
Four infants with Group A Niemann-Pick disease had similar ocular abnormalities secondary to this systemic disease. Each child demonstrated corneal opacification, brown discoloration of the anterior lens capsule, and retinal opacification with a macular cherry-red spot. These abnormalities were seen in each child during the first year of life and appeared stable. Recognition of this combination of ocular defects facilitates early identification of patients with Group A infantile Niemann-Pick disease.
Cornea↗
Histochemical demonstration of cyclic guanosine 3',5'-monophosphate phosphodiesterase activity in retinal photoreceptor outer segments.
A technique for the histochemical demonstration of cyclic guanosine monophosphate phosphodiesterase in retina is described. Enzyme activity was identified on photoreceptor outer segment lamellae, a finding in agreement with previous biochemical data on isolated outer segment preparations. The distribution of phosphodiesterase activity for cyclic guanosine monophosphate was similar to that found previously in rod outer segments for cyclic adenosine monophosphate, suggesting that the same enzyme may hydrolyze both nucleotides.
3',5'-Cyclic-GMP Phosphodiesterases↗
A pathological study of eye involvement in acute leukemia of childhood.
The eyes of 60 children dying of acute leukemia between 1968 and 1976 at the Children's Hospital Medical Center have been examined pathologically. An attempt has been made to relate eye findings to the state of the systemic disease at the time of death. Eight of the 60 patients had leukemia retinal infiltrates and all eight had fulminant disease with terminal leukocyte counts over 100,000 per cubic millimeter and a high percentage of "blast" cells. Twenty-six patients (43%) had leukemic infiltration of the choroid which was not apparent clinically, but which would require therapy in any effort to eradicate leukemic cells from the body. Five of six patients with optic nervic involvement had coexistant meningeal leukemia. Isolated retinal hemorrhages could not be correlated with other parameters of the leukemic process.
Acute Disease↗
Ocular retardation (or) in the mouse.
The ocular retardation (or) mutation in mice has been studied morphologically in serial 1 mu sections. This recessively inherited, fully penetrant mutation is characterized by an early arrest of retinal development, aplasia of the optic nerve, cataractous degeneration of the lens, and microphthalmia. We describe early alterations of normally occurring morphogenetic cell death in the optic cup and aberrations of optic fissure formation which appear to precede the arrest of retinal and optic nerve development. The subsequent disappearance of central retinal vessels and cataract formation are interpreted as secondary phenomena.
Animals↗