Richard E. Goldberg

Retinal arterial obstruction in children and young adults.

The records of 27 patients who developed retinal arterial obstruction (RAO) prior to the age of 30 years were studied to ascertain associated systemic and ocular findings as possible etiologic factors. A history of migraine was found in approximately one third of the patients, and coagulation abnormalities wer also common. Trauma, sickle cell hemoglobinopathies, cardiac disorders, use of oral contraceptives, pregnancy, systemic lupus erythematosus and intravenous drug abuse were less frequently encountered. Ocular abnormalities included increased intraocular pressure, subtle buried drusen of the optic nerve head and a congenital prepapillary arterial loop. In contrast to older patients with RAO, there was no clinical evidence of atheromatous disease. In most patients, one or more systemic or ocular etiologic factors could be discerned. Whereas etiologic relationships may be multifactorial and generally differ from those commonly found in older patients with RAO, the visual prognosis in younger and older patients appears to be similar.

Congenital Pits of the Optic Nerve Head: II. Clinical Studies in Humans

The clinical characteristics of 75 eyes with congenital pits of the optic nerve head were reviewed, particularly in relation to associated serous retinal detachment. Retinal detachment was found in 52% of all eyes with pits and 63% of eyes with temporally located pits. Of 20 untreated eyes with a pit and coexistent macular retinal detachment followed for more than one year, 55% (11/20) had visual acuity less than or equal to 6/30 and 75% (15/20) had subretinal fluid at the most recent visit. Visual fields and intravenous fluorescein angiographic characteristics of pits are discussed and clinical evidence is presented supporting the theory that the associated subretinal fluid is derived from liquified vitreous.

Arterial Obstruction and Ocular Neovascularization

A series of 12 patients (13 eyes) with neovascular glaucoma in association with arterial obstructive disease is presented. Appropriately characterized as having an ocular ischemic syndrome, the anterior segment findings in each included aqueous flare and rubeosis iridis. Posterior segment manifestations included midperipheral intraretinal hemorrhages (venous stasis retinopathy), narrowed retinal arteries, often a cherry red spot, and neovascularization of the disc and/or retina. Most eyes with the ocular ischemic syndrome have either ipsilateral common carotid artery obstruction or severe bilateral obstruction of the internal carotid arteries.

Presumed Acquired Retinal Hemangiomas

This paper presents the clinical findings in 12 patients with a peculiar vascular mass of the sensory retina. On the basis of ophthalmoscopy, fluorescein angiography, ultrasonography and other studies, the authors presume that the described lesion is a primary acquired benign vascular tumor, most likely a variant of capillary hemangioma. The features that seem to differentiate clinically this lesion from von Hippels retinal angiomatosis, other ocular tumors, and pseudotumors are discussed and therapeutic recommendations are presented.

Cilioretinal artery obstruction.

The cases of 23 patients with untreated cilioretinal artery obstruction were reviewed. Three distinct groups were found: (1) isolated cilioretinal artery obstruction, (2) cilioretinal artery obstruction associated with central retinal venous obstruction, and (3) cilioretinal artery obstruction in conjunction with ischemic optic neuropathy. In the first group 90% of eyes achieved 6/12 or better vision and there was a high incidence of associated atherosclerotic carotid disease. In the second group, 70% of eyes improved to 6/12 or better, while in the last group no eye reached better than 6/120 vision.

The Use of Perfluorophenanthrene in the Removal of Intravitreal Lens Fragments

We used perfluorophenanthrene as an aid during pars plana vitrectomy in removing lens fragments dislocated posteriorly into the vitreous cavity. We reviewed the records of all patients (nine patients, nine eyes) referred with dislocated lenses who underwent pars plana vitrectomy with perfluorophenanthrene for removal of the lens material at Wills Eye Hospital from July 1, 1991, through Nov. 30, 1991. The lens was dislocated during cataract extraction in eight eyes. One eye had a history of nonpenetrating trauma and lens dislocation. In one eye a retinal tear was noted intraoperatively and treated. Six eyes had postoperative visual acuity of 20/50 or better. In all eyes the lens was removed without further complications. Perfluorocarbon liquids such as perfluorophenanthrene may enhance current techniques by allowing easier and safer removal of displaced lens fragments through the pars plana.

Classification and management of hereditary retinal angiomas

Two distinct types of retinal angiomas are currently recognized. Capillary hemangiomas occur most characteristically as part of the von Hippel-Lindau syndrome. The retinal capillary hemangiomas typically appear as globular red-orange tumors with dilated and tortuous afferent arterioles and efferent venules. Cavernous hemangiomas typically appear as grape-like clusters of dilated vascular sacs without pronounced alteration in the adjacent arterioles and venules. The spectrum of clinical features of these two types of hemangiomatosis and current approaches to management of patients with these disorders is reviewed.

Changing appearance of retinal arteriovenous malformation

Retinal arteriovenous malformations (racemose angiomas) are usually described as non-changing congenital vascular anomalies. The authors describe prominent retinal vascular changes that occurred in the fundus of a patient with a complex retinal arteriovenous malformation as part of the Wyburn-Mason syndrome during a follow-up period of 17 years. Retinale, arteriovenöse Mißbildungen (Angioma racemosom) werden normalerweise als unveränderliche, angeborene Gefäßanomalien beschrieben. Die Autoren beschreiben auffällige Veränderungen der Netzhautgefäße am Augenhintergrund eines Patienten mit komplexen arteriovenösen Mißbildungen als Teilsymptom eines Wyburn-Mason-Syndroms über eine Nachuntersuchungszeit von 17 Jahren.

Traumatic Central Retinal Artery Occlusion with Sickle Cell Trait

An 8-year-old black boy with sickle cell trait struck his left brow and globe on the edge of a table and had immediate blurring of vision. Six days later visual acuity was light projection, and traumatic iritis with secondary glaucoma and perimacular edema were present. Fluorescein angiography indicated obstructed peripheral and perimacular arterioles and dye leakage from the disk.

OCULAR MANIFESTATIONS OF RELAPSING POLYCHONDRITIS

The clinical, ultrasonographic, fluorescein angiographic, and immunological features of a case of relapsing polychondritis in a 46-year-old female are described. Acute bilateral sclerouveitis with multiple retinal pigment epithelial defects and sensory retinal detachment were present on initial examination. Antibodies to type II collagen were present in serum samples taken from the patient during the acute and convalescent phase of the disease. A possible relationship of this disorder to the phylogenetic development of the eye is discussed.