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Vitreous hemorrhage complicating retinal astrocytic hamartoma.

Tuberous sclerosis classically presents a triad of mental deficiency, seizures and adenoma sebaceum. Among the ocular manifestations are astrocytic hamartomas in the retina. This paper describes the case of a 24-year-old man with tuberous sclerosis who had recurrent vitreous hemorrhage from a large epipapillary astrocytic hamartoma in the left eye. Pars plana vitrectomy cleared the vitreous and bimanual bipolar coagulation of tumor bleeding points was done. Fragments of the friable tumor were obtained with the vitrectomy instrument and were prepared for tissue culture, histology, and electron microscopy. Although pars plana vitrectomy offers certain advantages over other methods of biopsy, it does present a danger of extraocular seeding of malignant cells; therefore, it is not recommended in cases with probable intraocular malignancy.

Adolescent↗

Picture resembling hemicentral retinal vein occlusion in the acquired immunodeficiency syndrome: is it related to cytomegalovirus?

Three HIV-seropositive subjects presented with diffuse inferior retinal hemorrhages. The first patient had an inferior hemicentral vein occlusion with subsequent development of an inferior cytomegalovirus retinitis. Two other patients had an inferior 'hemorrhagic' cytomegalovirus retinitis mimicking a venous occlusion, with the retinitis being subtle in 1 patient. We speculate that: (1) venous occlusion may predispose to cytomegalovirus infection of the retina by stasis and ischemic loss of endothelium; (2) early cytomegalovirus infection of the large retinal veins may give the picture of a hemorrhagic retinopathy in a subset of patients with cytomegalovirus retinitis. The differential diagnoses of hemorrhagic retinopathy in HIV-seropositive subjects include: cytomegalovirus retinitis, retinal vein occlusion, syphilitic retinitis and retinopathy of severe anemia.

AIDS-Related Opportunistic Infections↗

[Abnormal findings in the ocular fundi of colony-born cynomolgus monkeys].

The ocular fundi of 1,151 apparently healthy colony-born cynomolgus monkeys (Macaca fascicularis) ranging in age from newborn to 19 years were examined using an ophthalmoscope. Two hundred and thirty-eight abnormal findings were recorded in 219 of the 1,151 monkeys. Of these, 23 were related to the optic disc and 115 to the retinal vessels. Of the remaining 100 abnormal findings, 91 were retinal degenerations and 9 were retinal hemorrhages. The 23 optic disc abnormalities consisted of 3 cases of micropapilla, 4 of ectasia and 16 of myelination of the retinal nerve fibers. Of the 115 retinal vascular abnormalities, 87 were arterial tortuosity, one was venous tortuosity, 2 were tortuosity of both artery and vein, 2 were artery-vein crossing, 20 were copper-wire artery, one was inosculation of the artery, one was vascularization of the vein and one was persistent hyaloid artery. Of the 91 retinal degenerations, one was degeneration of the periphery of the macular and the optic disc, 8 were macular degeneration and 82 were peripheral degeneration. Nine cases of retinal hemorrhages appeared under 6 years of age.

Age Factors↗

A new staging system for idiopathic retinal periphlebitis.

PURPOSE: To develop and standardize a universally acceptable new staging system for idiopathic retinal periphlebitis (Eales disease). METHODS: A new staging system was established and standardized based on standard terminology and features. Idiopathic retinal periphlebitis was classified as peripheral and central types. Peripheral disease consisted of four stages. Stage 1 is periphlebitis of small (1a) and large (1b) caliber vessels with superficial retinal hemorrhages. Stage 2a denotes capillary nonperfusion and 2b neovascularization elsewhere/of the disc. Stage 3a is classified as fibrovascular proliferation and 3b vitreous hemorrhage. Stage 4a is traction/combined rhegmatogenous retinal detachment whereas 4b is rubeosis iridis, neovascular glaucoma, complicated cataract, and optic atrophy. A total of 253 cases of idiopathic retinal periphlebitis (mean age, 24.7 +/- 4.7 years, all male) presenting at this tertiary care center were classified prospectively according to the new staging system, by two independent observers (interobserver correlation = 0.7). RESULTS: The new staging system was consistent, simple, and easy to recall. Peripheral and central types of idiopathic retinal periphlebitis were found in 94.07% and 5.93% of cases, respectively. The new staging system also defined the severity of the disease. Vitreous hemorrhage was found to be the commonest presenting feature (51.68%), whereas traction/combined rhegmatogenous detachment was found in 5.88% of cases. CONCLUSIONS: The new staging system is useful in classifying and assessing the severity of disease. Management strategy can also be defined according to the stage of the disease. It is designed to promote the use of standard assessment with applications to clinical management and research.

Adolescent↗

Recurrent unilateral frosted branch angiitis.

In the left eye of a 27-year-old man we found perivascular creamy sheathing of retinal veins with retinal hemorrhages and, on fluorescein angiography, delayed filling of veins with late leakage. Dramatic recovery of visual acuity and healing of retinal lesions followed intravenous corticosteroid therapy. However, the condition recurred several times within a few months. Fluorescein angiography showed delayed filling of arteries and veins and arteriovenous anastomoses with a widespread capillary nonperfusion area. Eventually, neovascular glaucoma resulted. It is suggested that frosted branch angiitis is related to vascular occlusion. Systemic corticosteroid therapy seems to affect the course of this disease.

Adult↗

Episcleral plaque thermoradiotherapy of posterior uveal melanomas.

Episcleral plaque radiotherapy is a widely applied treatment for selected patients with uveal melanomas. This treatment is well tolerated but may produce severe late radiation complications resulting in decreased visual acuity that reduces the attractiveness of conservative therapy. The purpose of this study was to access if the addition of episcleral hyperthermia decreases late radiation complications through radiation dose reduction while maintaining high incidence of local tumor control. In a 3-year period, episcleral plaque thermoradiotherapy was given to 25 patients with uveal melanoma in a Phase I study. The mean tumor height was 6.2 mm and the mean tumor basal area was 173 mm(2). The mean radiation dose given to the tumor apex was 72.2 Gy and the mean hyperthermia temperature, given once for 45 min, was 43.5 degrees C. Of the 25 patients treated, 22 (88%) showed tumor height reduction, 2 (8%) showed no change, and 1 (4%) had an increase in tumor height. At the last follow-up (range, 20-68 months; mean, 31.2 months), a 43% mean tumor height reduction was recorded (p = 0.0002). Of the 22 patients initially showing tumor regression, 2 (9%) had subsequent tumor progression. At least ambulatory vision (>5/200) was maintained by 20 (80%) patients. Severe complications, including hemorrhagic retinal detachment and a large vitreous hemorrhage, were seen in 2 (8%) patients early in this Phase I study. The treatment program was well tolerated by the study patients. Severe late treatment toxicity was sharply reduced by limiting the mean scleral temperature to < or equal to 44 degrees C. This study employing 30% lower radiation doses, showed tumor regression in the majority of patients. Longer follow-up is needed to assess long-term treatment efficacy and late treatment complications.

Adolescent↗

Correlation between retinal microcirculation and blood viscosity in patients with hyperviscosity syndrome.

Hyperviscosity syndrome leads to vascular disturbances in different organs. In the retina typical ophthalmoscopic changes can be found including dot and blot hemorrhages, retinal and optic nerve head edema, and increased diameter of retinal veins. In this study we examined the retinal microcirculation in patients with hyperviscosity syndrome. Nineteen patients (14 patients with Waldenstroem's macroglobulinemia, two patients with kryoglobulinemia, three patients with plasmacytoma) were examined. All patients underwent a video fluorescein angiography. In all angiograms the arteriovenous passage time (AVP) and the arm retina time (ART) were quantified. In addition, hematocrit (Hct) and plasma viscosity (ETA) were measured. In patients with hyperviscosity syndrome AVP was significantly prolonged in comparison to healthy volunteers (AVP: 2.5+/-1.3 s vs. 1.5+/-0.4 s; p < 0.01). The ART showed no significant differences. Plasma viscosity was doubled in patients as compared with reference values (ETA: 2.57+/-1.5 mPa s vs. 1.24+/-0.08 mPa s; p < 0.01). In this study we showed an increase in plasma viscosity as well as an increase in arteriovenous passage time. This may result in retinal circulatory disturbances and may cause the typical fundus changes in patients with hyperviscosity syndrome.

Adult↗

Symptoms and risk factors of ocular complications following dengue infection.

BACKGROUND: Dengue infection can result in severe ocular complications. We prospectively studied the range of ocular symptoms in a cohort of patients with dengue infection. METHODS: We included adult patients with serologically confirmed dengue infection who received clinical care at the National University Hospital, Singapore. They were assessed for demographic characteristics, ocular symptoms, laboratory parameters and dengue severity. Patients with two or more ocular symptoms were referred to an ophthalmologist and underwent full ophthalmologic examination that included visual acuity, slit-lamp examination and indirect fundoscopic examination. Descriptive and logistic regression analyses were performed and statistical significance was set at p<0.05. RESULTS: One hundred and fifty-six patients, aged (S.D.) 33.94 (12.7) years, consisting of 89 (57.1%) males and 67 (42.9%) females, participated in this study. They reported ocular symptoms, such as eye strain (30%), retro-ocular pain (20%), blurring of vision (10%), double vision (3%), foreign body sensation (3%), eye flashes (2%) and floaters (1%). Multivariate analysis identified white cell count (odds ratio, OR 0.531; 95% CI 0.292, 0.963) and serum albumin (OR 0.758; 95% confidence intervals, CI 0.618, 0.928) as significant predictors of clinically significant ocular symptoms. The triad of symptoms (eye flashes, floaters and blurring of vision) was highly predictive for the development of retinal hemorrhages (positive predictive value 100%). CONCLUSIONS: Dengue virus results in a spectrum of ocular manifestations, ranging from non-specific symptoms to severe retinal hemorrhages. Further studies are needed to validate the triad of ocular symptoms as a screening tool for severe ocular complications following dengue infection.

Adult↗

Retinal microvascular signs and risk of stroke and stroke mortality.

OBJECTIVE: The purpose of this study was to assess the relation of retinal microvascular signs and incident stroke and stroke mortality in an older population. METHODS: The authors took retinal photographs on baseline participants (3,654 patients aged 49+ years) of the Blue Mountains Eye Study (1992 to 1994). They assessed the presence of retinopathy (microaneurysms, retinal hemorrhages) in participants without diabetes and retinal arteriolar signs in all participants using standardized grading protocols. Incident stroke/TIA/cerebrovascular death (combined stroke events) were identified at follow-up examinations during 1997 to 1999. RESULTS: During a 7-year period, 859 participants died, 97 (11.3%) of which died of cerebrovascular causes. Of survivors, 24 had confirmed incident stroke, and 11 had incident TIA. Combined stroke events were more frequent in participants with retinopathy (5.7%), with moderate/severe arteriovenous nicking (4.2%), or with focal arteriolar narrowing (7.2%) compared with those without (1.9%). After controlling for age, sex, systolic blood pressure, smoking, and self-rated health, retinopathy was significantly associated with combined stroke events (relative risk [RR] 1.7, 95% CI 1.0 to 2.8) in persons without diabetes. This association was stronger in those without severe hypertension (RR 2.7, CI 1.2 to 6.2) or in persons with two or more retinal microvascular signs (RR 2.7, CI 1.5 to 5.2). Generalized or focal arteriolar narrowing or arteriovenous nicking was not independently associated with combined stroke events after multivariate adjustment. CONCLUSIONS: In older Australians without diabetes, retinopathy signs predict stroke or stroke-related death independent of traditional stroke risk factors.

Age Factors↗

Occult ocular damage as a leading sign in the battered child syndrome.

A three-month old, premature female infant is presented with severe bilateral occult ocular damage comprised of cataracts, lenses dislocation, retinal hemorrhages and total funnel-shaped retinal detachment without external signs such as bruises. This severe eye trauma is the result of child abuse. We would like to alert pediatricians as well as ophthalmologists that any ocular injury should raise the possible diagnosis of battered child syndrome even in the absence of other supportive evidence of external trauma.

Battered Child Syndrome↗

Interferon-associated retinopathy in a uniform regimen of natural interferon-alpha therapy for chronic hepatitis C.

BACKGROUND/AIMS: The development of retinal lesions induced by a uniform regimen of interferon-alpha therapy for chronic hepatitis C was prospectively investigated. METHODS: Eighty-one patients received 6 mega units of natural interferon-alpha intramuscularly daily for 2 weeks and then 3 times a week for 22 weeks. The total dose of interferon-alpha administered was uniformly 478 mega units per patient. Two expert ophthalmologists prospectively examined the patients for retinal lesions before, during and after the therapy. RESULTS: Retinopathy was not found in comparison groups or any of the patients before treatment. In total 34.6% (28/81) of the patients showed cotton-wool spots or minor retinal hemorrhage, or both lesions, during therapy, but these lesions were reversed during or after interferon therapy. The occurrence rates of cotton-wool spots alone, retinal hemorrhage alone and both lesions were 13.6% (11/81), 6.2% (5/81), and 14.8% (12/81), respectively. The appearance of retinopathy did not correlate with patients' background including viral loads and response to the therapy, but was more frequently found in older patients and patients with hypertension and/or diabetes mellitus; disappearance of retinopathy was more prolonged than in patients without these complications. Almost all the lesions appeared 2-4 months after the start of the therapy, and the severity of the lesions did not differ between patients with and without hypertension and/or diabetes mellitus. CONCLUSION: Although it is not clear if interferon-associated retinopathy occurs in a dose-dependent manner, the present study shows a standard pattern of the occurrence of retinopathy in patients with chronic hepatitis C receiving a uniform dosage of natural interferon-alpha.

Age Distribution↗

[Purtscher's retinopathy: a rare complication of acute non-alcoholic pancreatitis].

Purtscher's retinopathy is a rare complication of acute alcoholic pancreatitis that is characterized by the development of cotton-wool exudates and retinal hemorrhages located around the optic disk. Its development is due to ischemic phenomena in the posterior pole of the retina due to microemboli in the retinal arterioles and capillaries. This complication is not related to a poorer prognosis of acute pancreatitis and the retinopathy usually has a favorable outcome with disappearance of the ophthalmic lesions and restoration of visual acuity after 4-6 weeks in most cases. However, some patients may show residual lesions, especially if optical atrophy occurs. We present a 40-year-old woman with loss of visual acuity due to Purtscher's retinopathy during the course of mild acute idiopathic pancreatitis.

Acute Disease↗

[Myambutol (ethambutol)-induced retinoneuritis].

A retinoneuritis due to ethambutol (Myambutol) occurs with Myambutol doses of over 20 mg/kg per day after 3 weeks to 15 months. Visual acuity can deteriorate within the following 6 months even after immediate discontinuation of treatment with Myambutol. It may recover 2 months (though in some cases only 16 months) after the onset of the deterioration; in cases of optic atrophy a more severe loss of vision usually remains. If there was loss of vision to 1/10 or less there was in most cases no change in the visual acuity. On the other hand, a primary loss of vision to only 0.3 or 0.4 usually ended with recovery of full vision. The occasionally observed clinical picture of a reddish optic disk, retinal hemorrhages, a very fine granular pigment alteration of the macular region, and loss of vision for more than a year without optic disk pallor suggests a toxic retinitis or retinoneuritis rather than neuritis. This is confirmed by electrophysiological observations.

Adult↗

Disk edema in an overweight woman.

A 46-year-old extremely obese black woman presented with headaches, blurred vision, and visual obscurations. Her exam was notable for bilateral severe papilledema, retinal hemorrhages, and lethargy. Her CAT scan was normal, and a spinal tap revealed a very high opening pressure. Although this patient's presentation mimicked pseudotumor cerebri, the lethargy and retinal hemorrhages were atypical. Her hospital evaluation was notable for elevation of the serum bicarbonate level, and she was subsequently found to have hypoxia and hypercapnia on a blood gas. The patient was diagnosed as Pickwickian syndrome, with obstructive sleep apnea. Treatment of the pulmonary problem resulted in dramatic improvement in her eye findings and her lethargy, and optic nerve sheath fenestration was not necessary.

Female↗

Retinal changes in Himalayan climbers.

Changes in the fundus of the eye were studied in 15 members of a mountaineering expedition to Dhaulagiri, Nepal (elevation, 8,167 meters [26,795 ft]). Retinal photographs were taken at sea level and at 5,883 meters (19,300) after each climber had descended from his highest point. Five Nepali Sherpas and an additional American climber who came to the base camp late in the climb were also studied. Vascular engorgement with tortuosity, a 24% increase in arterial diameter, and a 23% increase in venous diameter were observed. Retinal hemorrhages were seen in five American climbers (33%), but in none of the Sherpas. The high incidence is striking, but the cause is unknown. We suggest that the hypoxic vasodilatation makes retinal vessels more vulnerable to sudden rises in intravascular pressure.

Adult↗

Retinopathy associated with acute pancreatitis.

Two patients (a 28-year-old woman and a 23-year-old man) with acute pancreatitis developed severe visual loss. The acute stage of retinopathy consisted of retinal edema, cotton-wool patches, and retinal hemorrhages, predominantly in the posterior pole of both eyes. During the five-year follow-up period, visual acuity improved and sequential fluorescein angiography of both patients demonstrated reperfusion of previously occluded retinal vessels. After resolution of the cotton-wool patches, the previously edematous areas were replaced by foci of retinal thinning which created irregularities in the internal limiting membrane light reflex. Visual fields demonstrated scotomas corresponding to the areas of the previous cotton-wool patches.

Acute Disease↗

Intravitreal cysticercosis.

Examination of a 36-year-old man with naked visual acuity of 20/20 revealed a floating, conspicuous cyst of Cysticercus cellulosae in the vitreous cavity of the right eye. A vitreous traction band from the vitreous base and the optic disc was connected to the lodging bulb of the cyst. In the superonasal area, an ovoid retinal break surrounded by a white retinal lesion with two elliptical retinal hemorrhages was found, and this seems to be the previous lodging site of the cyst. A pars plana vitrectomy was performed to remove the parasite, and laser photocoagulation was carried out around the retinal break. Four months after the operation, the patient was satisfied with naked visual acuity of 25/20 without any complication in the affected eye.

Adult↗

Wernicke-Korsakoff syndrome associated with hyperemesis gravidarum.

PURPOSE: The authors hereby describe a case of Wernicke-Korsakoff syndrome with accompanying ocular findings that is caused by hyperernesis gravidarum. METHODS: We experienced a 27-year-old female at 12 weeks of pregnancy, who visited our clinic because of weight loss, gait disturbance, decreased mentality and dizziness after prolonged vomiting for 2 months. Neurological examination demonstrated ataxia of gait and loss of orientation. Ophthalmologic examination showed decreased visual acuity, upbeat nystagmus, diplopia and retinal hemorrhage. RESULTS: We report a relatively rare case of Wernicke-Korsakoff syndrome with ophthalmic symptoms induced by hyperemesis gravidarum. CONCLUSIONS: If a pregnant women has symptoms of severe vomiting along with other ocular findings such as retinal hemorrhage or restricted extraocular movement, one must suspect the diagnosis of Wernicke-Korsakoff syndrome, and should start appropriate treatment immediately.

Adult↗