Status of postdoctoral dental education: clinical training.
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Biomedical subjects
Publications and source records attributed to R G Weaver.
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Direct carotid-cavernous sinus fistulas that present with signs and symptoms contralateral to the arterial supply of the fistulas are not uncommon. We present a thoroughly documented case of a dural-cavernous sinus fistula with symptoms exclusively contralateral to the arterial source, a rarer entity. The patient presented with a red, proptotic right eye and a history of transient horizontal diplopia and a "feeling of fullness" in that eye. Magnetic resonance imaging (MRI) of the brain and orbits performed at another hospital had shown no abnormalities. Carotid angiography performed on the right side was normal; carotid angiography performed on the left side showed a dural-cavernous sinus fistula, with shunting from branches of the left external carotid artery directly to the right cavernous sinus. Orbital duplex color-flow sonography showed reverse flow in a dilated right superior ophthalmic vein. This unusual manifestation of a dural-cavernous sinus fistula offers insight into the pathophysiology of arteriovenous fistulas involving the cavernous sinus, and is a reminder that bilateral injections are required when performing carotid angiography to characterize these disorders.
We report on a child with blepharophimosis, ptosis, and epicanthus inversus (BPES), developmental delay and an interstitial deletion of band q22 of chromosome 3. A review of chromosome 3q anomalies associated with eye abnormalities, specifically blepharophimosis and ptosis, strongly suggests that a locus for eyelid development is present at the interface of bands 3q22.3 and 3q23.
Twenty-two patients (36 eyes) are reported with Brown-McLean syndrome, which consists of peripheral corneal edema associated with peripheral endothelial pigment deposits, usually after intracapsular cataract extraction. This group, the largest reported to date, had a spectrum of corneal alterations, those at the more severe end of the spectrum being both progressive and symptomatic. Some patients required medical and surgical treatment, including keratoplasty. Four corneas (two obtained surgically, two postmortem) were examined by light and electron microscopy (EM). Centrally, the corneas were relatively normal, but peripherally there were disintegrated endothelial cells with an abnormal posterior collagenous layer of Descemet's membrane. Scanning EM showed a somewhat distinct junction between the normal central endothelium and the diseased peripheral endothelium.
Although decreased vision from corneal epithelial basement membrane dystrophy (CEBMD) usually is associated with pain from corneal epithelial erosion, it may be an overlooked cause of painless, sometimes sudden, visual disturbance. We report a series of eight patients referred to our department with visual deficits for whom various diagnoses had been made. A total of 15 neurologic and ophthalmologic tests had been ordered before their referral. All patients had CEBMD as a cause for their visual deficit. Retroillumination through a dilated pupil and examination of the fluorescein tear film were the best methods to demonstrate the CEBMD. Six of the eight patients underwent epithelial debridement with complete resolution of their symptoms. We recommend that all patients with unexplained visual disturbances be examined closely for CEBMD.
We report on a patient with bilateral microphthalmia and unusual cataracts with a de novo pericentric inversion of chromosome (2)(p21q31). A literature review of previous associations of eye abnormalities and anomalies of chromosome 2 suggests probable gene locations for eye development.
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Following enucleation or evisceration, the wearing of a prosthetic eye may be impractical, impossible, or undesirable. We describe herein 10 patients for whom a prosthesis was not feasible and who therefore underwent removal of the eye with extirpation of the conjunctiva, tarsi, and lid margins, followed by lid closure. The procedure as originally described is suitable for a wide range of disorders. Modifying the technique to incorporate simultaneous removal of the conjunctiva, tarsi, and lid margin together with enucleation of the globe permitted the use of the operation in the treatment of patients with ocular and conjunctival surface malignant neoplasms. Although the procedure does not replace simple enucleation of the globe or exenteration of the orbit, it is useful under certain circumstances.
A macular branch retinal artery occlusion developed in the right eye of a 25-year-old woman when she was 38 weeks pregnant. She subsequently presented 5 days postpartum with a branch retinal artery occlusion in her left eye. Although her initial work-up did not reveal a source for her occlusions, subsequent studies have documented a deficiency of protein S.
Statistically controlled studies substantiating the current widespread acceptance of intravitreal air injection as an adjunct to traditional or standard scleral buckling techniques in the repair of rhegmatogenous retinal detachment are unavailable. The surgical success rate of a prospectively chosen group of patients who received intravitreal air was compared with that of a retrospectively chosen but comparable group of patients who did not receive air injection in their detachment repair. The 100% retinal reattachment rate in the air group at six weeks was found to be statistically significant for comparable configurations of retinal detachment and superiorly located breaks in patients without significant proliferative vitreoretinopathy.
A father and daughter with isolated aniridia were observed to have an apparently balanced, reciprocal translocation involving chromosomes 5 and 11 [t(5;11)(q13.1;p13)]. No other clinical characteristics often associated with the deletion of 11p13 were observed in this family. This finding, in association with 3 other instances of single breaks at 11p13 and aniridia, supports the assignment of AN2 to 11p13.
The ophthalmic literature of the past half century contains relatively few reports of true exfoliation of the lens capsule, reflecting a reduction in the occupation-related occurrence of "glassblower's cataract." The authors report 11 eyes (7 patients) with a diaphanous membrane arising from the anterior lens capsule, which they have identified by slit-lamp biomicroscopy. None of the seven patients had had extended exposure to an infrared-emitting heat source or ocular trauma; one patient with bilateral true exfoliation of the lens capsule had a history of unilateral herpes simplex keratitis. The average age of these patients at the time of diagnosis was 85.4 years. Identification of these 11 eyes by two ophthalmologists during a 6-year period suggests that idiopathic true exfoliation of the lens capsule has been underdetected, under-reported, or both.
The current classification of cavitary optic disc anomalies including the morphologically related entities--optic nerve pit, morning glory disc anomaly, coloboma of the optic nerve, and retinochoroidal coloboma involving the optic nerve--is inexact and confusing. Traditionally, these disc abnormalities have been regarded as distinct morphologic anomalies. Thirty-five members of a five-generation kindred with autosomal dominantly inherited optic disc anomalies were examined. Observed abnormalities in this pedigree comprised a spectrum of morphologic variants ranging from large anomalous discs to typical pits and colobomas. The findings in this family suggest a variable expression of a single autosomal dominant defect rather than the chance occurrence of three separate, distinct, but morphologically similar entities occurring in a single pedigree.
Optic nerve colobomas can occur as sporadic abnormalities, may be inherited as an autosomal dominant defect, occur as part of syndromes, and are rarely associated with cardiac malformations and midline encephaloceles. Karcher [1979] described a father and son with the "morning glory" optic disc anomaly and renal disease as a new association. We report on two brothers with optic nerve colobomas associated with renal disease. The ophthalmologic findings and renal histopathology are presented. This second familial occurrence suggests that the association of optic nerve coloboma and renal disease is a newly recognized syndrome.
The spontaneous release of epiretinal membranes is a rare clinical phenomenon, the mechanism of which is poorly understood. The authors present three patients with epiretinal macular membranes associated with peripheral retinal lesions, in whom spontaneous release of the membrane occurred in conjunction with detachment of the posterior vitreous. In each patient, the membrane remained attached to the posterior vitreous face, and in each eye there was a decrease in retinal striae and an improvement in vision. The authors postulate that posterior vitreous separation was the mechanism by which these membranes detached from the macula.
Three patients had documented fundus changes conforming to those of the recently described multiple evanescent white dot (MEWD) syndrome. All three patients were unilaterally affected with variously sized, soft, single, and coalescent white lesions at the level of the RPE and the deep retina. Fluorescein angiography demonstrated early staining and hyperfluorescence of the white dots and delayed staining around the optic disc. Some degree of optic disc edema could be seen in all three eyes, two of which had corresponding field defects. In all three eyes, characteristic "stippling," or granularity, of the affected macula developed rapidly and vitreal cells were observed. One eye had signs of previous perivascular inflammation. ERG studies performed on one patient indicated a reduction in the a-wave and depression of the ERP, findings that correlated with the clinical observations of RPE affectation.
Two sibs are reported with Walker-Warburg syndrome including hydrocephalus, agyria, anterior chamber dysgenesis, and encephalocele. In addition, both had cleft lip and cleft palate and intrauterine growth retardation, findings not previously noted in this condition.
Thirty-four long-term survivors of childhood acute lymphoblastic leukemia (ALL) underwent comprehensive ophthalmic examinations to detect retinopathy or other ocular sequelae. Sixteen of the 34 patients received whole brain radiation (greater than or equal to 2400 rad). All 18 patients in the non-radiated group had normal eye examinations, while 4 of 16 in the radiated group had ocular abnormalities. None of the ocular abnormalities could be definitely attributed to radiation and all patients had normal visual acuity. No radiation retinopathy was found in either group.