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Biomedical subjects

A H Weiss

Publications and source records attributed to A H Weiss.

10 recordsLinked to original sources
PubMed1992

Ankyloblepharon filiforme adnatum.

We treated 4 infants with ankyloblepharon filiforme adnatum (AFA), an uncommon anomaly in which the apposing eyelid margins are connected by abnormal tissue strands. One infant had AFA alone, one had Hay-Wells syndrome, characterized by ectodermal dysplasia, and the other 2 had chromosome abnormalities, trisomy 18, and complex chromosome rearrangement, with visceral malformations. Despite heterogeneity and phenotypic variability, these developmental abnormalities shared (1) involvement of tissues growing in apposition and (2) temporal overlap of their occurrence. This suggests a common defect in the mechanism(s) that regulate tissue fusion at multiple sites during development.

Abnormalities, Multiple↗
PubMed1992

Axial myopia in eyes with optic nerve hypoplasia.

Given that abnormal visual experience during post natal development interferes with emmetropization, we proposed that eyes with hypoplastic optic nerves were predisposed to the development of refractive errors. Six of 14 patients with unilateral optic nerve hypoplasia and 5 of 22 patients with bilateral involvement had at least 4 D of myopia. Nine of the 11 patients with asymmetric bilateral involvement had relative myopia in the eye with the more abnormal optic nerve; none of the patients with symmetric bilateral involvement had a significant interocular refractive difference. Analysis of axial length measurements obtained in 10 of the 11 patients with high myopia showed a significant increase in total axial length. The presumed normal eye of patients with unilateral involvement was significantly smaller than the mean value for age-adjusted normals. We suspect that visual input to the central nervous system is one of the feedback signals involved in the regulation of ocular growth.

Adolescent↗
PubMed1989

Adrenal suppression after corticosteroid injection of periocular hemangiomas.

Two infants with symptomatic periocular hemangiomas received 1-ml intralesional injections of a 50:50 mixture of triamcinolone acetonide (40 mg/ml) and betamethasone (8 mg/ml). Both developed adrenal suppression as evidenced by the immediate depression of their serum cortisol and adrenocorticotrophic hormone levels. One patient remained adrenal suppressed for five months and, concurrently, her growth rate and weight dropped from normal to below the fifth percentile. Adrenal suppression should be added to the growing list of complications caused by the corticosteroid injection of periocular hemangiomas.

Adrenal Cortex Hormones↗
PubMed1989

Complex microphthalmos.

Forty patients were diagnosed as having complex microphthalmos on the basis of a malformed globe with a total axial length measurement at least 2 SDs below the mean for age-similar controls. Three had anterior segment dysgenesis; 4, congenital lens abnormalities; 14, chorioretinal colobomas; 12, persistent hyperplastic primary vitreous; 4, retinal dysplasia; and 3, complex malformations due to ipsilateral facial malformations. Measurements of total axial length indicated that complex microphthalmos was congenital and that postnatal growth of the malformed eye was similar to that of normal eyes. In most patients the anterior segment length was normal, while in all patients the posterior segment length was at least 2 SDs below the mean. Corneal diameter correlated significantly with total axial length (r2 = .57) and decreased linearly as total axial length decreased. In most patients in whom measurements were obtained, the lens and corneal power were increased, thereby compensating for decreased total axial length. We propose that inadequate production of secondary vitreous is the cause of the microphthalmos, given that the posterior segment was disproportionately reduced in size and the secondary vitreous is its predominant component. Evidence that each of the various ocular malformations can influence the production of secondary vitreous is presented.

Abnormalities, Multiple↗
PubMed1989

Simple microphthalmos.

Simple microphthalmos was diagnosed in 22 patients on the basis of a normal-appearing eye and a total axial length at least 2 SDs below the mean for age. Anterior segment length was normal in most patients while posterior segment length was at least 2 SDs below the mean in all patients, indicating that disproportionate reduction in posterior segment length accounted for the microphthalmos. The normal values for total axial length, anterior segment length, and posterior segment length were determined from the analysis of axial length measurements obtained from age-similar controls. Ten patients had isolated microphthalmos. One of them was diagnosed as having nanophthalmos on the basis of microcornea, total axial length less than 18 mm, and absence of systemic disease. Twelve patients had associated systemic disorders, such as fetal alcohol syndrome, myotonic dystrophy, and achondroplasia, which implicated decreased size of the optic cup, altered vitreous proteoglycans, low intraocular pressure, and abnormal release of growth factors in the pathogenesis of microphthalmos.

Adult↗
PubMed1989

Visual sensory disorders in congenital nystagmus.

Congenital nystagmus (CN) is a common disorder indicative of a primary disturbance of the ocular motor or visual sensory systems. The authors prospectively evaluated 81 patients with CN, structurally normal eyes, and minimal or no abnormalities of the optic nerve, macula, and retinal pigment epithelium (RPE). Seventy-four (91%) patients were found to have a disorder of the visual sensory system. Thirty-four patients had albinism, 37 had a congenital or early onset disorder of the retinal photoreceptors, and 3 had abnormalities of the optic nerve. The remaining seven (9%) patients had motor CN. Most patients presenting with CN have visual loss and should be evaluated for an underlying disorder of the visual sensory system.

Adolescent↗
PubMedDate not supplied

Primary and secondary orbital teratomas.

Two patients with primary teratomas of the orbit and a third patient with a teratoma invading the orbit from the maxillary sinus are presented. The clinical presentation of each patient was spectacular. In the primary teratomas, the globe was displaced out of the orbit by the attached tumor, causing extreme proptosis. Computed tomography was virtually diagnostic, showing a variegated orbital mass with solid and cystic components. Histologic examination showed multiple tissues derived from two or three germinal layers. The surgical removal of the tumors and reasons for the poor visual outcome are discussed. The appearance of teratomas in the orbit and other cephalic structures appears to arise from the survival and proliferation of germ cells deposited there during embryogenesis.

Female↗
PubMedDate not supplied

Neuroretinitis in childhood.

We report the clinical features and results of laboratory testing of seven children with neuroretinitis, diagnosed on the basis of reduced visual acuity, relative afferent pupillary defect, and distinctive appearance of the fundi. Disc swelling was segmental in five patients with unilateral disease and diffuse in two patients with bilateral disease. Retinal infiltrates were transiently present in two patients. Swelling of the optic disc and peripapillary retina, along with the inflammatory vitritis resolved by 2 to 6 weeks but macular exudates persisted for 3 to 12 months. Two patients developed optic atrophy with permanent visual loss and two patients had visual acuity reduction detected only under low contrast conditions. Cat-scratch disease was implicated as the cause of the neuroretinitis in two patients; the etiology of the remaining cases was not established.

Adolescent↗
PubMedDate not supplied

Blue cone monochromatism.

Blue cone monochromatism (BCM) is a subtype of achromatopsia in which the blue cone mechanism predominates. Each of the four patients in this study had BCM proven by their having peak spectral sensitivities in the blue region of the visible spectrum (near 440 nm). Clinically, the diagnosis was suspected because of x-linked inheritance, the presence of acuities better than 20/200 in two patients and myopia ranging from -1.75 to -15.00 diopters in three patients. Congenital nystagmus was the presenting sign in three of the four patients. Examination of the fundi was uniformly normal. The distinctive spectral properties of BCM were demonstrated by the American Optical H-R-R and the Panel D-15 tests. All affected patients correctly identified three of the four blue-yellow plates and a variable number of the red-green plates in the American Optical H-R-R test. The study patients consistently made errors oriented along the protan and deutan axes but they made none along the tritan axis. The authors conclude that the results of these two color discrimination tests are useful in diagnosing BCM.

Child↗