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Biomedical subjects

G R Beauchamp

Publications and source records attributed to G R Beauchamp.

At least 19 recordsLinked to original sources

Ocular findings associated with neurofibromatosis type II.

BACKGROUND: Neurofibromatosis has been recently acknowledged as consisting of a number of different diseases. Neurofibromatosis (NF) type I and NF type II are the most clearly defined. Type II is characterized by bilateral acoustic neuromas and is rare (its incidence is 1/50,000). The previously reported ocular associations of NF type II are posterior subcapsular cataracts, Lisch nodules, and combined hamartomata of the retinal pigment epithelium and retina. In this study, the authors attempt to define further the ocular manifestations of NF type II. METHODS: The authors prospectively examined 9 patients who met the diagnostic criteria for NF type II (age, 18 to 38 years; mean, 25 years). RESULTS: Seven of nine patients had epiretinal membranes in the posterior pole. None of these epiretinal membranes were visually significant. In addition, five patients had central posterior cortical cataracts and five had peripheral wedge-shaped cortical cataracts. CONCLUSION: The presence of epiretinal membranes in young patients may represent another clinical finding associated with NF type II. Epiretinal membranes, central posterior cataracts, peripheral cortical cataracts, or combined hamartoma of the retinal epithelium and retina in young patients should alert the ophthalmologist to include NF type II in the differential diagnosis in patients with stigmata of NF type II.

Adolescent

Clinicopathological findings in a growing optic nerve melanocytoma.

We present an unusual case of a melanocytoma of the optic disc that showed documented progressive growth over a period of six years. It reached the largest size of any reported optic nerve melanocytoma and the eye was enucleated because of the possibility of malignant degeneration. Optic nerve melanocytomas are locally invasive but are not known to undergo transformation into malignant melanoma. Follow-up should remain the primary approach in the management of patients with optic nerve melanocytomas, as previously advocated by Zimmerman.

Adult

Recession and anterior transposition of the inferior oblique for treatment of superior oblique palsy.

We evaluated the effectiveness of inferior oblique recession with anterior transposition in treating 12 patients with superior oblique palsy. Mean decreases of hypertropia measured 17 prism diopters in the primary position, 24 prism diopters in adduction, and 21 prism diopters on ipsilateral head tilt. Head tilt and diplopia were uniformly eliminated. No surgical complications were encountered. Postoperative deviations were mild and infrequent. Only one patient demonstrated postoperative underaction of the recessed inferior oblique.

Adolescent

Ring stromal opacity in lens-corneal adhesion.

Severe congenital corneal clouding may be associated with abnormal anterior segment development (dysgenesis). Often, congenital corneal opacities preclude visualization of intraocular anatomy. In this report, five eyes of four patients with congenital corneal clouding and lens-corneal adhesion (LCA) are described. All had a ring- or doughnut-shaped haze within the stroma surrounding a central clear corneal zone delineating the adhesion. Recognition of LCA is pivotal in planning surgical therapy for eyes with congenital corneal clouding. As a clue to recognition of this anomaly, the ring-shaped stromal opacity may aid in diagnosis of this phenomenon and in avoidance of surgical complications.

Cornea

Learning disabilities: update comment on the visual system.

The role of the eye in vision and its relationship to learning disabilities is discussed in this very timely article. A historical overview is included, as well as a consideration of the more recent neuroanatomic research in this area.

Brain

Accommodative esotropia: review of current practices and controversies.

The treatment of accommodative esotropia requires a thorough evaluation at first presentation to elucidate all factors involved. The degree of hypermetropia, character of the esodeviation (i.e., intermittent or constant), presence or absence of amblyopia, and the AC/A ratio are all important in the initial management of these patients. In addition, the binocular status can be of prognostic value. Close follow-up is needed to ensure maintenance of binocularity and appropriate early intervention to modify the course before bifixation is lost.

Accommodation, Ocular

Ocular findings in juvenile nasopharyngeal angiofibroma.

Juvenile nasopharyngeal angiofibroma (JNA) is the most common benign neoplasm of the nasopharynx. While histologically benign, it has the propensity for aggressive local growth. This highly vascular tumor predominantly occurs in adolescent males. The literature fails to provide a thorough description of ocular complications and their incidence in JNA. This report summarizes the data from those clinical series detailing ocular findings in a total of 218 JNA cases. Exophthalmos was found in 14% of all cases. Decreased visual acuity and partial ophthalmoplegia occurred in 5% and 2% respectively. Recognition of ocular involvement in JNA is of the utmost importance, for it is often a manifestation of orbital or intracranial extension or both. We describe the diagnosis and management of a case of JNA in a five-year-old white male. The patient developed ocular findings of marked exophthalmos and optic atrophy. Early multidisciplinary diagnostic evaluation (otolaryngological, neurosurgical, and ophthalmological) followed by a team surgical approach to excision is most likely to yield efficacious results.

Child, Preschool

Inheritance of Fuchs' combined dystrophy.

The inheritance pattern of Fuchs' combined corneal dystrophy is not confirmed. Published pedigrees fail to demonstrate a 50% segregation and sex ratio. They include no more than two generations of affected individuals and indicate a strong, female predilection. The pedigree we will present shows 16 affected persons in four generations. The ratio of affected to unaffected and men to women is 1:1. Penetrance is apparently 100%. Nine of the affected are under 50 years of age; four are subteen age. Light and electron micrographs of corneal tissue from three patients in three different generations are consistent with the diagnosis of Fuchs' dystrophy. Fuchs' dystrophy can therefore be established as a classic autosomal dominant pattern.

Adolescent

Filtering surgery in children: barriers to success.

The use of trabeculectomy in advanced pediatric glaucoma is reviewed. Intraocular pressure was controlled in only 50% of the patients, and the best visual results was 20/200. Complications of vitreous loss, scleral collapse, ectasia, retinal detachment, and endophthalmitis were encountered. No evidence was found to support the claim that in the seriously compromised buphthalmic eyes, trabeculectomy is safer than other filtering operations since it filters under a scleral flap.

Adolescent

Pediatric keratoplasty: problems in management.

A summary of experience in pediatric keratoplasty is given by adding ten recent cases to a previous report. A review of keratoplasty in infants and children from the literature generally recommends the procedure as safe and effective. Recent reports, however, cast some doubt on the effectiveness of the procedure for certain conditions. An update of experience is given as a focal point for a discussion of problems in clinical management of children who undergo keratoplasty. These problems are arbitrarily separated by pre-, intra-, and postoperative time frames.

Adolescent

Glycoconjugates, cellular differentiation, and congenital glaucoma.

Studies of congenital glaucoma have described and debated the disease process in cellular and structural terms, yet the functional aspects of the disease remain poorly understood. This paper presents an attempt to look at the disease from a different perspective: the role of glycoconjugates and their influence on morphogenesis of the corneoscleral angle. Extracellular and cell-surface complex carbohydrates and trabecular meshwork development were observed in normal C-57 BL/6J mice with light and electron microscopy. Lectin-stained fixed tissues were evaluated by fluorescein light microscopy and with computer-controlled scanning microspectrophotometry. The appearance and type of specific glycoconjugates was related to morphogenesis and differentiation, with specific reference to the development of the trabecular meshwork. Our results suggest that glycoconjugates are: (1) important in the morphogenesis of the corneoscleral angle, and (2) provide some of the required signals for the differentiation of the cells of the trabecular meshwork. These findings emphasize the possibility that a complex series of morphological and biological events in the normal development of the trabecular meshwork ultimately leads to a functionally competent trabecular meshwork, that is, capacitation.

Animals

Congenital glaucoma and retinal dysplasia.

The differential diagnosis of leukocoria (pseudoglioma) in the neonate includes multiple conditions, including malformations with retinal dysplasia as a component. Typically bilateral, retinal dysplasia is characteristically seen in microphthalmic eyes. Certain chromosomal defects have been described. The case reported herein presented in the first month of life with an enlarged eye, elevated intraocular pressure, prominent iris vasculature, and leukocoria. Family history was positive in one respect: this is the second child of a Viet Nam veteran exposed to Agent Orange. The first child, from a different mother, also had birth defects. Other than his left eye, the child is completely normal. Ultrasonography showed posterior vitreous opacities of indeterminate configuration. CT scan suggested a posterior intraocular mass. Histologically, the principal features were an anomalous, largely unformed corneoscleral angle, intraocular hemorrhage, and retinal dysplasia. Light microscopic studies were performed. The corneoscleral angle revealed an anteriorly inserted iris with an absence of trabecular meshwork and Schlemm's canal. This case is considered unique on the basis of the association of retinal dysplasia with congenital glaucoma and larger-than-normal eye. The significance of reported paternal exposure to Agent Orange in this instance is unknown.

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