Corkscrew retinal vessels in neurofibromatosis type 1: report of 12 cases.

AIM: To describe a distinctive spectrum of retinal microvascular abnormalities in 12 patients with neurofibromatosis type 1 (NF-1). METHODS: This is an observational prospective study of the ocular fundus evaluated by direct ophthalmoscopy with or without fluorescein angiography, to investigate retinal microvascular abnormalities in 32 patients with NF-1 and in 30 control subjects. The evaluation included a complete general and neurological physical examination and in some cases computed tomography, magnetic resonance imaging with gadolinium-DTPA, or both. RESULTS: The occurrence of a distinctive spectrum of retinal microvascular abnormalities is described in 12 patients with NF-1 (37.5%). At the lower end of the spectrum, present in 10 patients, the anomaly consisted of minuscule second or third order tortuous venules, which were called "corkscrew retinal vessels." These were usually isolated but in a few cases multiple. They flow towards the superior or inferior temporal veins. They had a length of one to two disc diameters. They ended either in a minute tuft or vanished on the retinal surface. The upper end of the spectrum was seen in only two patients. One of them had an exceptionally large venous anastomosis on the nasal retina and the other had an arteriovenous malformation extending over one retinal quadrant. None of the patients in the control group had such retinal microvascular abnormalities. CONCLUSION: The "corkscrew" retinal vessels described in this report constitute a broad spectrum of microvascular markers in NF-1 patients.

Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). METHODS: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.

Retinal findings in Takayasu's arteritis.

PURPOSE: To describe unusual as well as typical ocular findings in eight patients with Takayasu's disease. METHODS: The ophthalmic manifestations and clinical courses of eight patients with Takayasu's disease were evaluated. RESULTS: All patients had clinical and angiographic evidence of pulseless disease. Typical findings included retinal venous congestion, fluorescein staining, capillary drop-out, microaneurysms and arteriovenous shunting. Unusual findings included cotton wool spots, anterior ischemic neuropathy and retinal emboli. In one case microaneurysms disappeared after carotid bypass surgery. Several patients had profound, transient, visual loss with changes in head posture. CONCLUSION: Takayasu's disease can cause a variety of retinal manifestations. Less common findings include cotton wool spots, anterior ischemic optic neuropathy and retinal emboli.

Optociliary veins in optic nerve sheath meningioma. Indocyanine green videoangiography findings.

OBJECTIVE: To report the indocyanine green videoangiography (ICG-V) characteristics of optociliary veins (OV) in optic nerve sheath meningioma (ONSM). DESIGN: Case series. PARTICIPANTS: Four eyes (patients) with OV and ONSM were examined. INTERVENTION: Indocyanine green videoangiography and intravenous fluorescein angiography (IVFA) were prospectively performed. MAIN OUTCOME MEASURES: The course of OV in ONSM cases was observed with ICG-V and IVFA. RESULTS: On ICG-V, the course of OV was followed from their origin at small tributaries of the central retinal vein, their junction with choroidal veins, and finally to their drainage in the vortex venous system. The authors found an inverse relationship between the degree of optic disc edema and the development and ease of visualization of the optociliary veins and their draining course through the choroidal circulation. The mean time from the injection of indocyanine green to the visualization of OV's draining choroidal vessels was 35.4 seconds (range, 25.4-50 seconds). Intravenous fluorescein angiography allowed visualization of OV only at the margins of the optic disc in all cases. CONCLUSION: Indocyanine green videoangiography provides important and new information in cases with OV and ONSM that is not obtained with IVFA. Such information may provide important knowledge regarding the hemodynamics of the choroidal circulation in normal and pathologic states.

Epidemic optic neuropathy in Cuba. Eye findings.

OBJECTIVE: To characterize and establish a clinical definition of the optic neuropathy that appeared in epidemic form in Cuba in 1992 and 1993. METHODS: At the invitation of the Cuban Ministry of Health, Havana, members of ORBIS International and the Pan American Health Organization, assembled teams that traveled to Cuba in May 1993. We were initially briefed by Cuban national experts in the areas of virology, nutrition, toxicology, ophthalmology, neurology, and public health. We then examined 20 patients on our own. Thirteen of these patients underwent a comprehensive neuro-ophthalmologic examination, including neurologic examination, ophthalmologic examination, visual fields, optic nerve function studies, contrast sensitivity studies, and funduscopy. We returned 4 months later to perform an additional 12 comprehensive neuro-ophthalmologic and follow-up examinations. RESULTS: Only seven of the 13 patients who were alleged to have the optic form of the epidemic and who were rigorously and systematically examined on the first visit demonstrated a bilateral optic neuropathy. These seven patients had several features that included decreased visual acuity, poor color vision, central scotomas, decreased contrast sensitivity, saccadic eye movements, and most prominent and distinctive of all, nerve fiber layer wedge defects of the papillomacular bundle. Our clinical definition was then implemented by the Cuban ophthalmologists and epidemiologists. On returning 4 months later, we found that all newly presented patients were correctly diagnosed to have the epidemic disease. With the new case definition and the application of a few simple psychophysical tests, the false-positive rate of diagnosis became much lower. After vitamin therapy, we reexamined the patients seen on our initial visit, and all showed marked improvement. CONCLUSIONS: The Cuban epidemic was characterized by an optic neuropathy with features that were similar to those of tobacco/alcohol amblyopia and Leber's optic atrophy. Recent political, economic, and social changes in Cuba may have contributed to the nutritional and/or toxic compromise of mitochondrial function of an acquired nature, which led to selective retinal ganglion cell damage. We have termed this condition Cuban epidemic optic neuropathy.

Optic neuritis in children with poor recovery of vision.

We reviewed the records of 10 children with optic neuritis in whom recovery of vision was poor or incomplete. Our cases were otherwise similar to those described in previous studies in that they were always bilateral, often accompanied by a viral prodrome (seven of 10), and usually associated with disc oedema (seven of 10). Seven of twenty eyes had a final visual acuity of 6/60 or worse and only one patient regained 6/6 vision in either eye. In three patients the best vision in either eye was 6/60 or worse. Recovery of vision was often slow, taking up to six years. Five of 10 patients have developed multiple sclerosis (MS), and one child had acute disseminated encephalomyelitis (ADEM) with optic neuritis. Optic neuritis in children does not always carry a good prognosis for recovery of vision; however, the failure of vision recovery in a short period of time does not necessarily indicate a poor outcome. Some children with optic neuritis develop MS, which can develop even when optic neuritis follows a viral illness.

Disappearing optociliary shunt vessels and neonatal hydrocephalus.

This is a report of two cases of optociliary shunt vessels in patients with neonatal hydrocephalus. Complete disappearance of the optociliary shunt vessels occurred after surgical procedures to normalize intracranial pressure. The clinicopathological significance, prognosis, and treatment of patients with acquired optociliary shunt vessels is discussed. This is also the first report of the association of optociliary shunt vessels and neonatal hydrocephalus.

El deffecto pupilar aferente (pupila de Marcus-Gunn). El gran ignorado del examen oftalmologico. / The afferent pupillary defect (Marcus-Gunn pupil).The most unknown in ophthalmologic test

El defecto pupilar aferente o pupilar de Marcus Gunn, es un 'pequeno gran' signo semiologico que evidencia en forma objetiva la existencia de una lesion organica en la via pre-quiasmatica del mismo lado donde ha sido observado. Es el signo pupilar mas importante en medicina clinica. Consideramos que en razon de la rapidez y facilidad con que puede ser investigado, y la informacion que aporta cuando presente, se hace mandatoria su inclusion entre los parametros a ser evaluados en todo examen clinico integral. Como signo pupilar preeminente, propugnamos el que su ensenanza se inicie desde un nivel de pre-grado, cuando se trate de la semiologia pupilar

Retinal fluorescein angiographic evidence for atheromatous microembolism. Demonstration of ophthalmoscopically occult emboli and post-embolic endothelial damage after attacks of amaurosis fugax.

There is evidence that microemboli responsible for amaurosis fugax may be atheromatous but it can elude ophthalmoscopic confirmation, because such emboli quickly fragment and disappear from view in the retinal vessels. This report documents 2 patients in whom fluorescein angiography of the retina provided evidence of microembolization after an attack of amaurosis although the fundus appeared normal. In one patient the angiogram revealed intraluminal material; in the other it showed fluorescein leakage from patent arteriolar bifurcations.

[Stenosis and intestinal ulcer due to chronic ingestion of a combination of a thiazidic diuretic with potassium]. / Estenósis y ulceración intestinal asociada a ingestion crónica de una combinación de diurético tiazídico con potasio.

Reports from recent years shown evidence of the existence of a type of primary ulceration of the small bowel associated with stenosis, relatively frequent and possibly iatrogenic. The primary characteristics of the studied cases were that it was present in patients over 50 years old, a good number of them had cardiorenal diseases or were hypertensives, and ingestion of a thiazide diuretic supplemented with potassium. Clinical and experimental investigations suggest that potassium, with or without a diuretic, could cause ulceration and stenosis of the small bowel. Presented in this report the case of a 62-year-old patient with cardiovascular disease and chronic ingestion of a thiazide diuretic with potassium chloride (Diclotride-K (R) who developed an intestinal obstruction that merited surgical intervention which showed a circumscribed stenosis of jejunum accompanied by ulceration.