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Retinopathy secondary to anemia from myeloid metaplasia in polycythemia vera.

BACKGROUND: Polycythemia vera is a chronic clonal disorder associated with excessive proliferation of erythrocytes, leukocytes, and thrombocytes, as well as an accompanying splenomegaly. Ocular manifestations of polycythemia vera include occipital cortex transient ischemic attacks, transient monocular blindness, vaso-occlusive disease, and retinal hemorrhages. CASE REPORT: A 56-year old man with longstanding polycythemia vera sought treatment for a chief symptom of blurred vision in the left eye and a red tinge to things first noticed on awakening that morning. He had preretinal and intraretinal hemorrhages and was subsequently found to be severely anemic as a result of postpolycythemic myeloid metaplasia after years of phlebotomy. Splenectomy controlled his anemia and thrombocytopenia, allowing transient improvement of the retinal hemorrhages. Acute leukemia subsequently developed and the patient died 7 weeks after initial examination. CONCLUSION: In this case, preretinal and intraretinal hemorrhages were found in a patient with longstanding polycythemia vera. The exact origin of these hemorrhages is uncertain. They are probably secondary to anemia, but the possibility that they are sites of extramedullary hematopoiesis must be considered. The appearance of retinal hemorrhages warrants careful investigation to rule out diabetes, hypertension, and anemia, as well as the various other blood dyscrasias.

Anemia↗

Prognostic indicators for vision and mortality in shaken baby syndrome.

OBJECTIVE: To study ocular and nonocular signs of patients diagnosed as having "shaken baby syndrome" and determine prognostic indicators for vision and mortality. METHODS: Medical records of child abuse cases involving bilateral retinal hemorrhages were reviewed. Particular attention was paid to visual function and pupillary light reaction at the time of admission as well as the location of retinal hemorrhages, neuroimaging findings, ventilatory requirement, and associated skeletal injuries. These findings were correlated with visual prognosis and mortality. RESULTS: Thirty consecutive cases met the criteria for review. At the initial visit, mean age of the children was 9.3 months (range, 1-39 months) and 12 children (40%) had at least fix-and-follow vision. Preretinal and intraretinal hemorrhages (93% [n = 28] and 100% [n = 30]) were more common than vitreous hemorrhage (10% [n = 3]). Subdural hematomas were detected in 21 patients (70%). Twenty children (67%) had seizures and 16 (53%) required ventilatory support; bruises and long bone fractures were seen in 14 (47%) and 4 (13%) children, respectively. Eight patients died. All patients with nonreactive pupils on presentation died, while all patients with a pupillary light reaction lived (P<.001). Six (86%) of 7 patients with midline shift died, whereas 21 (91%) of 23 with no midline shift lived (P<.001). At follow-up, retinal hemorrhages had resolved in nearly all children by 4 months, and 16 children (73%) had at least fix-and-follow vision. Ventilatory requirement was associated with poorer vision (P<.01). CONCLUSIONS: Nonreactive pupils and midline shift of the brain structures correlate highly with mortality. Ventilatory requirement, but not visual acuity on presentation, predicts visual outcome.

Battered Child Syndrome↗

A 12-year ophthalmologic experience with the shaken baby syndrome at a regional children's hospital.

PURPOSE: To examine the ophthalmologic experience with the shaken baby syndrome (SBS) at one medical center, including clinical findings, autopsy findings, and the visual outcome of survivors. METHODS: One hundred sixteen patients admitted from 1987 to 1998 for subdural hematomas of the brain secondary to abuse were included. RESULTS: Retinal hemorrhages were detected in 84% of the children, but this important finding had been missed often by nonophthalmologists. Poor visual response, poor pupillary response, and retinal hemorrhage correlated strongly with demise of the child. One child who died had pigmented retinal scars from previous abuse, a condition not previously observed histopathologically. The clinical and autopsy findings varied somewhat, probably because of the differing conditions for examination. No correlation could be made between computerized tomography scans done during life and the subdural hemorrhage of the optic nerve found on autopsy. Half of the surviving patients were known to have good vision. One fourth of the patients had poor vision, largely due to cerebral visual impairment from bilateral injury posterior to the optic chiasm. Severe neurologic impairment correlated highly with loss of vision. CONCLUSION: This series provides information on the frequency of eye findings in SBS patients. No fundus finding is pathognomonic for SBS. When retinal hemorrhages are found in young children, the likelihood that abuse occurred is very high. The difficulty that nonophthalmologists have in detecting retinal hemorrhage may be an important limiting factor in finding these children so they may be protected from further abuse.

Battered Child Syndrome↗

Correlation of fundus lesions and hematologic findings in leukemic retinopathy.

This prospective study evaluated the relationship between the fundus findings in leukemic retinopathy and hematologic parameters. Seventy-four newly diagnosed consecutive patients with acute leukemia were included, 49 with acute myelocytic leukemia (AML), and 25 acute lymphocytic leukemia (ALL). Blood parameters were based on data obtained before starting any therapeutic modalities. Leukemic retinopathy was detected in 32 patients (43%). Patients with ALL and retinal hemorrhages had significantly lower hemoglobin and hematocrit levels than those without hemorrhages (p = 0.004 and 0.018 respectively). AML patients with white-centered hemorrhages had a significantly higher leukocyte count than those without (p = 0.0002). ALL patients with cotton-wool spots had significantly lower hemoglobin levels and hematocrit than patients without such lesions (p = 0.044 and 0.05 respectively). AML patients with cotton wool spots had significantly lower leukocyte and platelet counts than those without (p = 0.019 and 0.003 respectively). Our results suggest that anemia is related to the findings of retinal hemorrhage and cotton-wool spots in ALL patients, that high leukocyte count is associated with white centered hemorrhage in AML patients, and that thrombocytopenia is not associated with retinal hemorrhage in this group of patients.

Acute Disease↗

Predicting subsequent visual field loss in glaucomatous subjects with disc hemorrhage using retinal nerve fiber layer polarimetry.

PURPOSE: To predict progression of visual field loss after an episode of disc hemorrhage in glaucoma patients on the basis of retinal nerve fiber layer (RNFL) GDx polarimetry measurements analyzed by wavelet-Fourier analysis (WFA). METHODS: Retrospective GDx data from 16 subjects (10 progressors and 6 non-progressors based on visual fields) obtained near the time of disc hemorrhage were analyzed to predict which patients would have visual field progression. Polarimetry scans throughout a follow-up period (31 months average) were also analyzed to compare field progression to RNFL thickness change after the hemorrhage. Mean RNFL thickness inferred from the polarimetry data at sixteen 22.5 degrees sectors at distances of 1.6, 1.7, and 1.8 disc diameters were used. Data were analyzed by applying to appropriate regions of disc hemorrhage patients a structural analysis (WFA) we had developed previously. A linear discriminant function (Fischer) was produced and a leave-one-out method using separate training and test data was used to assure validity of the results. RESULTS: Patients who subsequently progressed were successfully predicted with moderate success (sensitivity / specificity was 0.77 / 0.88 with ROC area = 0.858). A separate analysis comparing pre- and post-hemorrhage RNFL sector thickness revealed clear evidence of RNFL thinning at the inferior and superior sectors before progression of visual field. The thinning of RNFL thickness was not restricted to regions corresponding to the location of the hemorrhage. CONCLUSION: Wavelet-Fourier analysis can differentiate progressors from non-progressors with moderate accuracy. Comparison to a prior study of this same cohort emphasizes that relatively small regions must be considered (as opposed to larger quadrants) to see these significant changes in RNFL.

Aged↗

Head injury in the infant and toddler. Coma scoring and outcome scale.

This is a retrospective review of all closed-head injured children, ages 1-36 months, admitted to the Children's Memorial Hospital from 1959 to 1978. Injuries ranged from trivial to those producing deep coma. A coma scoring system (to correlate level of consciousness with age and outcome) was devised. Lateralizing neurological signs, fontanelle status, retinal hemorrhages, seizures, and skull fractures were correlated with age, outcome, and late onset of seizures. Children of 1 year and younger were more prone to have a poor outcome in all coma grades except the deepest, where the trend apparently reversed. Of the three components of the coma score (ocular, motor, verbal) the ocular score more constantly reflected neurological damage. Subdural hematomas, which were largely responsible for poorer outcomes in infants, occurred almost exclusively in children under 1 year of age. Lateralizing neurological signs, which were not found to correlate directly with a poor outcome, were unilateral Babinski, ataxia, and the combination of ocular deviation and hemiparesis (which is probably a seizure variant). Hemiparesis alone, however, did correlate directly with a poor outcome, as did full, and especially tense, fontanelle, and split sutures or diastatic fractures. Linear fractures, when unilateral, were not associated with a poor outcome, although bilateral linear and depressed fractures were. Bilateral retinal hemorrhages were found to be both quantitatively and qualitatively different from unilateral retinal hemorrhages, and to be statistically more often associated with subdural hematoma.

Age Factors↗

[Terson syndrome associated with a carotid-ophthalmic giant aneurysm].

UNLABELLED: PURPOSE/MATERIAL AND METHOD: A case of a 42 year-old woman presenting headache, vomiting and loss of consciousness of sudden onset. Upon testing right eye presented amaurosis while left eye showed a visual acuity of 20/50. RESULTS/CONCLUSION: Brain angiography showed a right sided giant carotid aneurysm at the ophthalmic artery area. Globe fundus in left eye showed vitreal and intra-retinal hemorrhage appearing in retinal posterior pole and periphery of the retina. Subarachnoidal hemorrhage accompanied by intra-vitreal bleeding is known as Terson's Syndrome. Peculiarities of this clinical case are discussed in this paper.

Adult↗

Fatal pediatric head injuries caused by short-distance falls.

Physicians disagree on several issues regarding head injury in infants and children, including the potential lethality of a short-distance fall, a lucid interval in an ultimately fatal head injury, and the specificity of retinal hemorrhage for inflicted trauma. There is scant objective evidence to resolve these questions, and more information is needed. The objective of this study was to determine whether there are witnessed or investigated fatal short-distance falls that were concluded to be accidental. The author reviewed the January 1, 1988 through June 30, 1999 United States Consumer Product Safety Commission database for head injury associated with the use of playground equipment. The author obtained and reviewed the primary source data (hospital and emergency medical services' records, law enforcement reports, and coroner or medical examiner records) for all fatalities involving a fall. The results revealed 18 fall-related head injury fatalities in the database. The youngest child was 12 months old, the oldest 13 years. The falls were from 0.6 to 3 meters (2-10 feet). A noncaretaker witnessed 12 of the 18, and 12 had a lucid interval. Four of the six children in whom funduscopic examination was documented in the medical record had bilateral retinal hemorrhage. The author concludes that an infant or child may suffer a fatal head injury from a fall of less than 3 meters (10 feet). The injury may be associated with a lucid interval and bilateral retinal hemorrhage.

Accidental Falls↗

Role of the indigenous drug saptamrita lauha in hemorrhagic retinopathies.

A clinical study was done on 48 eyes to assess the role of an indigenous drug, Saptamrita Lauha, in the absorption of hemorrhages and prevention of their recurrence in retinopathic eyes. Rapid absorption of retinal hemorrhages was observed in both treated groups: diabetic and hypertensive (mean durations, 16 days and 17.83 days, respectively). In three eyes, the hemorrhage cleared completely; partial clearance was observed in the rest of the eyes. Recurrence of a hemorrhagic episode was less in treated eyes (25% in each group) compared with control eyes (60% in diabetic and 50% in hypertensive patients). This study suggests that Saptamrita Lauha may hasten the process of retinal hemorrhage absorption and prevent its recurrence. The beneficial effect could be attributed to the flavanoid-like properties of one of the ingredients of this natural product.

Adult↗

Intraretinal hemorrhage associated with leishmaniasis.

A 39-year-old man presented with sudden loss of visual acuity caused by two retinal hemorrhages with no choroidal neovascularization (confirmed by fluorescein angiography). The patient was hospitalized for malaise, progressive pancytopenia, hepatosplenomegaly, progressive anemia, and perianal inflammation. Positive serologies were obtained for toxoplasmosis (IgG) and Leishmania (1/160). A diagnosis of visceral leishmaniasis was made, and the patient was treated with pentavalent antimonials. Two months later, best-corrected visual acuity was 20/25, with no residual scotoma. Indirect ophthalmoscopy showed complete resolution of the hemorrhages. This patient was an otherwise healthy immunocompetent adult who presented frank visceral leishmaniasis and retinal hemorrhages as the only ocular or systemic hemorrhagic findings, with spontaneous resolution after improvement of platelet levels. This rare cause of macular hemorrhage should be considered in areas where Leishmania is endemic.

Adult↗

Correlation between retinal abnormalities and intracranial abnormalities in the shaken baby syndrome.

PURPOSE: To report correlation between retinal and intracranial abnormalities and to evaluate pathogenesis of retinal hemorrhages in the shaken baby syndrome (SBS). DESIGN: Observational case series. METHODS: Seventy-five children with apparent nonaccidental head trauma consistent with SBS had complete physical examination, complete ophthalmologic examination, neuroimaging by CT or MRI, or both, and skeletal radiographic survey. In this retrospective review, ophthalmoscopic and intracranial abnormalities were correlated. RESULTS: The age of patients ranged from 2 to 48 months (mean - SD, 10.6 +/- 10.4 months). Neuroimaging was abnormal in all 75 cases. Findings included subdural hematoma (70 children, 93%), cerebral edema (33 children, 44%), subarachnoid hemorrhage (12 children, 16%), vascular infarction (nine children, 12%), intraparenchymal blood (six children, 8%), parenchymal contusion (six children, 8%), and epidural hemorrhage (one child, 1%). Sixty-four (64/75, 85%) children had retinal abnormalities, mostly (53/64, 82%) confluent multiple hemorrhages that were subretinal, intraretinal, and preretinal in 47/64 (74%) and bilateral in 52/64 (81%). No association was found between anatomic site (left, right, or bilateral) of intracranial and retinal findings (McNemar test kappa = -0.026-0.106) or between any of the intracranial findings mentioned above and the following retinal findings: normal or abnormal retinal examination, multiple (>10) or few retinal hemorrhages (< or =10), symmetric or asymmetric retinal findings, or retinoschisis (kappa = -0.127-0.104). Signs of possible increased intracranial pressure were not correlated with any retinal abnormality (kappa = -0.03-0.073). There was no correlation between evidence of impact trauma to the head and retinal hemorrhages (kappa = 0.058). Total Cranial Trauma Score and Total Retinal Hemorrhage Score, both indicating the severity of injury, were correlated (P =.032). CONCLUSIONS: Our study supports previous observations that the severity of retinal and intracranial injury is correlated in SBS. We cannot support the suggestions that in most children with SBS retinal bleeding is caused by sustained elevated intracranial, elevated intrathoracic pressure, direct tracking of blood from the intracranial space, or direct impact trauma. The correlation in severity of both eye and head findings may suggest, however, that retinal abnormalities are the result of mechanical shaking forces.

Battered Child Syndrome↗

Visual impairment following epidural fluid injections and epiduroscopy: a review.

INTRODUCTION: Visual impairment apparently is a rare but significant complication following epidural fluid injection and epiduroscopy. We report a review of cases reported in the literature with the aim of gaining an understanding of how the complication occurs as well as how to avoid it. MATERIALS AND METHODS: A literature review was performed using PubMed to find 1) case reports and 2) factors that contribute to the development of visual loss in the cases. RESULTS: Twelve cases of visual impairment following epiduroscopy or epidural fluid injection have been reported in the literature. The average age of the patients was approximately 50 years, 83.3% of the patients were female, 16.7% were male. Bolus injection of fluid with or without epiduroscopy was considered to be the precipitating event. The volume of solution injected epidurally varied from 20 mL to 120 mL (average = 59.4 mL). The common finding was retinal hemorrhage, with 58.3% occurring bilaterally. Recovery occurred in 79.2% of the cases. CONCLUSION: Retinal hemorrhage following epidural fluid injections/epiduroscopy apparently is due to an increase in cerebrospinal fluid pressure proportional to the rate and the amount of fluid injected. A sudden increase in epidural pressure is transmitted into the subarachnoid space to the optic nerve sheath, compressing the optic nerve and its vasculature. The vasculature compression ruptures retinal blood vessels.

Endoscopy↗

[Fundus hemorrhage in patients with intracranial hemorrhage caused by cerebrovascular disease--its clinical significance].

To clarify the significance of fundus hemorrhage in the acute stage of intracranial hemorrhage due to cerebrovascular disease (HCVD), the incidence of such hemorrhage (retinal, preretinal, and subhyaloid hemorrhage) was examined and its correlations with the severity of the disturbance of consciousness and the prognosis were investigated. Two hundred sixteen HCVD patients with intracranial hemorrhage were the subjects of this study, including 87 cases of subarachnoid hemorrhage (SAH), 60 cases of hypertensive intracerebral hemorrhage (HICH), 54 cases of arteriovenous malformation (AVM), and 15 cases of Moyamoya disease. Fundus hemorrhage was recognized in 41 cases of SAH (47%), 5 cases of HICH (8%), 3 cases of AVM (6%), and 2 cases of Moyamoya disease (13%). The incidence of fundus hemorrhage was significantly higher in SAH than in the other type of HCVD associated with intracranial hemorrhage. Among patients with severe disturbance of consciousness (JCS 100-300), fundus hemorrhage was recognized in 69% with SAH, 13% with HICH, 17% with AVM, and none with Moyamoya disease. Therefore, patients with both fundus hemorrhage and severe disturbance of consciousness in the acute stage are most likely to have SAH, but HICH and AVM should be also taken into consideration. In patients with slight disturbance of consciousness (JCS 0-30), fundus hemorrhage was only noted in SAH (27%) and not in other types of HCVD associated with intracranial hemorrhage. Thus, SAH is highly likely to be the primary HCVD associated with intracranial hemorrhage, if we recognize fundus hemorrhage in patients with slight disturbance of consciousness in the acute stage.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral Hemorrhage↗

[Effect of Panax notoginseng saponins combined isovolumic haemodilution on the retinal microcirculation of patients with retinal vein occlusion].

OBJECTIVE: To study the effect of Panax notoginseng saponins (PNS) or isovolumic haemodilution (IHD) and combination of PNS with IHD on retinal microcirculation of patients with retinal vein occlusion (RVO). METHOD: Seventy three patients with RVO were allocated at random to three groups which were treated with PNS, IHD, or PNS + IHD respectively. The retinal circulation time (RCT) and retinal hemorrhage, edema, leakage of capillary, cystoid macular edema (CME), and nonperfusion area of capillary in retina were observed before and after treatment. RESULT: RCT of patients with RVO was shortened by three treating methods. RCT in the patients who treated by PNS + IHD was the shortest, especially in the patients with nonischemic RVO and branch retinal vein occlusion(BRVO). The retinal hemorrhage, edema, leakage of capillary, and CME in the patients treated by PNS + IHD disappeared quicker than those treated by PNS or IHD. At the same time, the incidence of nonperfusion area in patients whose course of disease was shorter than fourteen days was decreased. The perfusion of capillaries was increased at the edge of nonperfusion area which existed before the treatment. But the effect for nonperfusion area which existed before the treatment in the type of ischemic RVO was not obvious. CONCLUSION: The treatment of PNS + IHD can both shorten RCT of patients with RVO, and promote absorption of retinal hemorrhage, edema, leakage of capillary, and CME. It is possible that PNS + IHD treatment also has an effect to decrease the forming of nonperfusion area in patients whose course of disease is at early stage.

Female↗

Tocopherol efficacy and safety for preventing retinopathy of prematurity: a randomized, controlled, double-masked trial.

To test the efficacy and safety of vitamin E in preventing retinopathy of prematurity, 287 infants with birth weights of less than 1.5 kg or gestational ages of less than 33 weeks were enrolled within 24 hours of birth in a randomized, double-masked trial of IV, followed by oral, placebo v tocopherol (adjusted to plasma levels of 3 to 3.5 mg/dL). In the 196 infants completing ophthalmic follow-up, tocopherol did not prevent retinopathy of prematurity of any stage (28% placebo treated v 26% tocopherol treated) or moderately severe retinopathy of prematurity (8% placebo treated v 11% tocopherol treated). Cicatricial sequelae were not significantly different (1/97 placebo treated v 3/99 tocopherol treated), with one placebo-treated infant and one tocopherol-treated infant having retinal detachments. Among all 232 infants examined, those treated with tocopherol had more retinal hemorrhage than placebo-treated infants (8/121 placebo treated v 16/111 tocopherol treated), and retinal hemorrhage correlated positively (P less than .01) with plasma levels of tocopherol after the first 2 weeks of age. Prospective monitoring of morbidity including late-onset sepsis, necrotizing enterocolitis, etc revealed no differences between groups except that grades 3 and 4 intraventricular hemorrhage occurred more frequently in infants weighing less than 1 kg at birth who had received tocopherol (14/42, 33%) v those who had received placebo (4/43, 9%) (P less than .02). Our data do not support the use of tocopherol for prophylaxis against retinopathy of prematurity in premature infants and suggest that IV tocopherol treatment starting on day 1 may increase the incidence of hemorrhagic complications of prematurity, particularly in infants with birth weights of less than 1 kg.

Birth Weight↗

Terson syndrome: CT evaluation in 12 patients.

PURPOSE: Terson syndrome may be overlooked in the acute setting and often requires ophthalmologic intervention to prevent long-term visual loss. In this syndrome, vitreous or retinal hemorrhage results from an abrupt rise in intracranial pressure, leading to retinal venous hypertension and intraocular hemorrhage. Our objective was to determine whether imaging findings could be discovered that might facilitate an earlier diagnosis. METHODS: Our inpatient medical record data base for 1991-1996 listed 11 patients with Terson syndrome. The medical records of these 11 patients were reviewed retrospectively and compared with their noncontrast head CT scans and with scans of 10 control subjects. One additional case was discovered prospectively, for a total of 12 patients. Three radiologists unaware of the patients' history evaluated CT scans of the orbits for evidence of intraocular hemorrhage. RESULTS: CT findings in eight patients were suggestive of retinal hemorrhage manifested by a retinal crescent or nodule that was slightly hyperdense relative to the vitreous humor. There was a high degree of concordance between the retrospective and independent reviews. CONCLUSION: Retinal nodularity and crescentic hyperdensities are evident on CT scans in the majority of patients with Terson syndrome. Although findings are subtle and not present in all cases, in the setting of subarachnoid hemorrhage they suggest retinal hemorrhage and warrant detailed fundoscopic evaluation.

Adolescent↗