On retinal hemorrhages in general:
"The cause is most likely VENOUS CONGESTION. The fetal head is compressed two to four times more forcefully than other fetal parts during the second stage of labor. Retinal hemorrhage is more common in promiparus deliveries and after forceps or vacuum extraction; it is rare after cesarean section. It may occur in normal deliveries."
 

 


http://www.sciencedaily.com/releases/2006/02/
060226114611.htm

Researchers Say Criterion For Diagnosing Child Abuse Not Always Accurate
ScienceDaily (Feb. 26, 2006) — When it comes to looking for damage to the eyes to prove child abuse, new research shows that things aren't always as they seem, according to Patrick Lantz, M.D., a forensic pathologist from Wake Forest University Baptist Medical Center.

"Contrary to what many doctors have been taught, we found that number and location of hemorrhages of the eye's retina aren't always proof of child abuse," said Lantz, who reported the results today at the 58th annual meeting of the American Academy of Forensic Sciences in Seattle. "Retinal hemorrhages occur more often than most doctors think are associated with a wide variety of conditions."

Lantz found that about 16 percent of the 700 individuals he examined during autopsy had hemorrhages of the retina, which is light-sensitive nerve tissue at the back of the eye. The bleeding occurs when tiny blood vessels on the retina's surface rupture. Lantz found the hemorrhages in individuals who had died from ruptured aneurysms, falls, car wrecks, gunshot wounds, meningitis and even drug overdose.

"Our research shows that you see the hemorrhages in a lot of different situations," Lantz said. "Retinal hemorrhages occur in child abuse, but they don't always mean a child was abused. Unfortunately, many pathologists, pediatricians and ophthalmologists have been taught that retinal hemorrhages are diagnostic of child abuse unless the child was involved in a high-speed car crash or fell more than two stories."

Currently, when child abuse is suspected, doctors conduct an eye exam to look for retinal hemorrhages and other eye changes that are considered proof of child abuse. Lantz got the idea to question this common assumption after he found that another eye condition, a buckling of the retina, is not always diagnostic for shaken baby syndrome. He reported those results in the British Medical Journal.

To test his theory that retinal hemorrhages also may not always be indicative of child abuse, Lantz decided to look for the condition during autopsies to learn more about when they occur.

Previously, the only way to look for the hemorrhages during an autopsy was to remove the eyes. Lantz came up with an alternative -- performing eye exams during autopsies using a surgical headlight and a handheld lens. This simple technique is sometimes used by ophthalmologists when more sophisticated equipment is not available, but no one had ever reported using it during autopsies.

The 700 deaths were in people ranging in age from birth to 96. Causes of death or conditions associated with retinal hemorrhages included suffocation, sudden infant death syndrome, meningitis, blunt trauma to the head, ruptured cerebral aneurysms, hemorrhagic strokes, cancer that had spread to the brain, high blood pressure, bleeding disorders, diabetes and gunshot wounds to the head.

"Many doctors have been taught to look for the hemorrhages when they suspect child abuse and often will diagnose child abuse without considering other possibilities," Lantz said. "Our research shows that you see the hemorrhages in a variety of different situations in infants, children and adults."

According to medical literature, retinal hemorrhages in infants are rare except in cases of abuse. "We're finding just the opposite," said Lantz. "We've found more retinal hemorrhages in non-abuse cases than in abuse cases, but most doctors don't look in the eyes of children unless they suspect child abuse."

Retinal hemorrhages were found in 30 children under age 14, yet only 6 cases were associated with child abuse.

As one of the first pathologists to routinely look at the back of the eye during autopsies, Lantz has learned that the technique can help diagnose hypertension, glaucoma, Marfan syndrome and even diabetes. He has taught residents and medical students to conduct the examinations and published an article in the Journal of Forensic Science (Nov. 2005) on the technique.

Lantz's co-researcher was Constance A. Stanton, M.D., neuropathologist, from Wake Forest University Baptist Medical Center.



--------------------------------------------------------------------------------

Adapted from materials provided by Wake Forest University Baptist Medical Center, via EurekAlert!, a service of AAAS.
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Wake Forest University Baptist Medical Center (2006, February 26). Researchers Say Criterion For Diagnosing Child Abuse Not Always Accurate. ScienceDaily. Retrieved October 24, 2008, from http://www.sciencedaily.com­ /releases/2006/02/060226114611.htm

 

 

Perimacular retinal folds from childhood head trauma
BMJ  2004;328:754-756 (27 March), doi:10.1136/bmj.328.7442.754
P E Lantz, associate professor1, S H Sinal, professor2, C A Stanton, associate professor1, R G Weaver, Jr, associate professor3
 Department of Pathology, Wake Forest University School of Medicine, Winston-Salem, NC 27157, USA, 2 Department of Paediatrics, Wake Forest University School of Medicine, 3 Department of Ophthalmology, Wake Forest University School of Medicine

No abstract available but in summary it is a case report of a 14month old child with retinal changes often described as pathognomonic for NAI, but where the cause was clearly documented to be accidental (A television fell on the child's head).  This publication comes at a time when the expert medical evidence for child abuse is coming under increasing scrutiny following the publicity around Roy Meadows cases in the UK. The Editorial (below) is worth reading if ever you might find yourself in the witness stand. MIKE


Editorial -  The evidence base for shaken baby syndrome
BMJ  2004;328:719-720 (27 March)

We need to question the diagnostic criteria

The phrase "shaken baby syndrome" evokes a powerful image of abuse, in which a carer shakes a child sufficiently hard to produce whiplash forces that result in subdural and retinal bleeding. The theory of shaken baby syndrome rests on core assumptions: shaking is always intentional and violent; the injury an infant receives from shaking is invariably severe; and subdural and retinal bleeding is the result of criminal abuse, unless proved otherwise.1 These beliefs are reinforced by an interpretation of the literature by medical experts, which may on occasion be instrumental in a carer being convicted or children being removed from their parents. But what is the evidence for the theory of shaken baby syndrome?

Retinal haemorrhage is one of the criteria used, and many doctors consider retinal haemorrhage with specific characteristics pathognomonic of shaking. However, in this issue Patrick Lantz et al examine that premise (p 754) and conclude that it "cannot be supported by objective scientific evidence."2 Their study comes hard on the heels of a recently published review of the literature on shaken baby syndrome from 1966 to 1998, in which Mark Donohoe found the scientific evidence to support a diagnosis of shaken baby syndrome to be much less reliable than generally thought.3

Shaken baby syndrome is usually diagnosed on the basis of subdural and retinal haemorrhages in an infant or young child,1 although the diagnostic criteria are not uniform, and it is not unusual for the diagnosis to be based on subdural or retinal haemorrhages alone.w1 The website of the American Academy of Ophthalmology states that if the retinal haemorrhages have specific characteristics "shaking injury can be diagnosed with confidence regardless of other circumstances."4 Having reviewed the evidence base for the belief that perimacular folds with retinal haemorrhages are diagnostic of shaking, Lantz et al were able to find only two flawed case-control studies, much of the published work displaying "an absence of... precise and reproducible case definition, and interpretations or conclusions that overstep the data."2 Their conclusions are remarkably similar to those of Donohoe, who found that "the evidence for shaken baby syndrome appears analogous to an inverted pyramid, with a very small database (most of it poor quality original research, retrospective in nature, and without appropriate control groups) spreading to a broad body of somewhat divergent opinions."3 His work entailed searching the literature, using the term "shaken baby syndrome" and then assessing the methods of the articles retrieved, using the tools of evidence based inquiry. Reviewing the studies achieving the highest quality of evidence rating scores, Donohoe found that "there was inadequate scientific evidence to come to a firm conclusion on most aspects of causation, diagnosis, treatment, or any other matters," and identified "serious data gaps, flaws of logic, inconsistency of case definition."3

The conclusions of Lantz et al and of Donohoe make disturbing reading, because they reveal major shortcomings in the literature relating to a field in which the opportunity for scientific experimentation and controlled trials does not exist, but in which much may rest on interpretation of the medical evidence.5

If the concept of shaken baby syndrome is scientifically uncertain, we have a duty to re-examine the validity of other beliefs in the field of infant injury. The recent literature contains a number of publications that disprove traditional expert opinion in the field. A study of independently witnessed low level falls showed that such falls may prove fatal, causing both subdural and retinal bleeding.6 w2 A biomechanical analysis validates that serious injury or death from a low level fall is possible and casts doubt on the idea that shaking can directly cause retinal or subdural haemorrhages.7 w3 An important lucid interval may be present in an ultimately fatal head injury in an infant.8 Neuropathological studies have shown that abused infants do not generally have severe traumatic brain injury and that the structural damage associated with death may be morphologically mild.9 10 What is the relevance of the craniocervical injuries to corticospinal tracts, dorsal nerve roots, and so on that have been described?10 11 We do not know. What is the force necessary to injure an infant's brain? Again, we do not know.

While most abused children indisputably show the signs of violence, not all do. No one would be surprised to learn that a fall from a two storey building or involvement in a high speed road traffic crash can cause retinal and subdural bleeding, but what is the minimum force required? "It is one thing clearly to state that a certain quantum of force is necessary to produce a subdural hematoma; it is quite another to use examples of obviously extreme force... and then suggest that they constitute the minimum force necessary."12

Research in the area of injury to infants is difficult. Quality evidence may need to be based on finite element modelling from data on infants' skulls, brains, and neck structures, rather than living animals. Any studies on immature animal models, if performed, will need to be validated against the known mechanical properties of the human infant. Pending completion of such studies, the reviews by Lantz and Donohoe are a valuable contribution and provide a salutary check for anyone wishing to cite the literature in support of an opinion. Their criticisms of lack of case definition or proper controls can be levelled at the whole literature on child abuse. If the issues are much less certain than we have been taught to believe, then to admit uncertainty sometimes would be appropriate for experts. Doing so may make prosecution more difficult, but a natural desire to protect children should not lead anyone to proffer opinions unsupported by good quality science. We need to reconsider the diagnostic criteria, if not the existence, of shaken baby syndrome.

J F Geddes, retired (formerly reader in clinical neuropathology, Queen Mary, University of London)

London (j.f.geddes@doctors.org.uk)

J Plunkett, forensic pathologist

 

Central Retinal Vein Occlusion

Last Updated: June 26, 2001

 
Synonyms and related keywords: nonischemic central vein occlusion, partial, incomplete, imminent, threatened, incipient, impending, perfused, venous statis retinopathy, ischemic central vein occlusion, complete, hemorrhagic, nonperfused

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Author: Lakshmana M Kooragayala, MD, Staff Physician, Assistant Professor of Ophthalmology, Department of Ophthalmology, Louisiana State University Health Sciences Center at Shreveport

 

Lakshmana M Kooragayala, MD, is a member of the following medical societies: Louisiana State Medical Society

 

Editor(s): Vytautas A Pakainis, MD, Chief of Ophthalmology, Dorn Veterans Administration Medical Center, Professor of Ophthalmology, Ophthalmology, University of South Carolina School of Medicine; Donald S Fong, MD, MPH, Assistant Clinical Professor of Ophthalmology, UCLA School of Medicine; Consulting Physician, Department of Ophthalmology, Southern California Permanente Medical Group; Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Lance L Brown, OD, MD, Ophthalmologist, Regional Eye Center, Affiliated With Freeman Hospital and St John's Hospital, Joplin, Missouri; and Hampton Roy, Sr, MD, Clinical Associate Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
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Background: Central retinal vein occlusion (CRVO) is a common retinal vascular disorder. Clinically, CRVO presents with variable visual loss; the fundus may show retinal hemorrhages, dilated tortuous retinal veins, cotton-wool spots, macular edema, and optic disc edema. In view of the devastating complications associated with the severe form of CRVO, a number of classifications were described in the literature. All these classifications take into account the area of retinal capillary nonperfusion and development of neovascular complications.

Broadly, CRVO can be divided into 2 clinical types, ischemic and nonischemic. In addition, a number of patients may have an intermediate in presentation with variable clinical course. On initial presentation, it may be difficult to classify a given patient into either category, since CRVO may change with time.

A number of clinical and ancillary investigative factors are taken into account for classifying CRVO, including vision at presentation, presence or absence of relative afferent pupillary defect, extent of retinal hemorrhages, cotton-wool spots, extent of retinal perfusion by fluorescein angiography, and electroretinographic changes.

Nonischemic CRVO is the milder form of the disease. It may present with good vision, few retinal hemorrhages and cotton-wool spots, no relative afferent pupillary defect, and good perfusion to the retina. Nonischemic CRVO may resolve fully with good visual outcome or may progress to the ischemic type.

Ischemic CRVO is the severe form of the disease. CRVO may present initially as the ischemic type, or it may progress from nonischemic. Usually, ischemic CRVO presents with severe visual loss, extensive retinal hemorrhages and cotton-wool spots, presence of relative afferent pupillary defect, poor perfusion to retina, and presence of severe electroretinographic changes. In addition, patients may end up with neovascular glaucoma and a painful blind eye.

 

Pathophysiology: The exact pathogenesis of the thrombotic occlusion of the central retinal vein is not known. Various local and systemic factors play a role in the pathological closure of the central retinal vein.

Central retinal artery and vein share a common adventitial sheath, as they exit the optic nerve head and pass through narrow opening in the lamina cribrosa. Because of this narrow entry in the lamina cribrosa, vessels are in a tight compartment with limited space for displacement. This anatomical position predisposes formation of thrombus in the central retinal vein by various factors, including slowing of blood stream, changes in the vessel wall, and changes in the blood.

Arteriosclerotic changes in the central retinal artery transforms the artery into a rigid structure and impinges upon the pliable central retinal vein, causing hemodynamic disturbances, endothelial damage, and thrombus formation. This mechanism explains the fact that there will be an associated arterial disease with CRVO. However, this association has not been proven consistently, and various authors disagree on this fact.

Thrombotic occlusion of the central retinal vein can occur as a result of various pathologic insults, including compression of the vein (mechanical pressure due to structural changes in lamina cribrosa, eg, glaucomatous cupping, inflammatory swelling in optic nerve, orbital disorders); hemodynamic disturbances (associated with hyperdynamic or sluggish circulation); vessel wall changes (eg, vasculitis); and changes in blood (eg, deficiency of thrombolytic factors, increase in clotting factors).

Whatever the mechanism of the occlusion of the central retinal vein, it leads to backup of blood in the retinal venous system and increased resistance to venous blood flow. This increased resistance causes stagnation of blood and ischemia of inner retinal layers. It has been postulated that ischemic damage to the retina produces angiogenesis factors, which stimulates abnormal vascularization of the posterior and anterior segment. In addition, increased blood pressure in the venous system causes break down of inner retinal barrier at the retinal capillary endothelium, leading to abnormal leakage of fluid in the retinal layers causing macular edema.

Prognosis of CRVO depends upon reestablishment of patency of the venous system by recanalization, dissolution of clot, or formation of optociliary shunt vessels.

 

Frequency:
 

bulletIn the US: CRVO and branch retinal vein occlusion constitute the second most common retinal vascular disorder. The nonischemic type is more common than the ischemic type.
bulletInternationally: A large population-based study in Israel reported a 4-year incidence of retinal vein occlusion of 2.14 cases per 1000 of general population older than 40 years and 5.36 cases per 1000 of general population older than 64 years.

In Australia, prevalence of vein occlusion ranges from 0.7% in patients aged 49-60 years to 4.6% in patients older than 80 years.

Mortality/Morbidity: CRVO is not associated directly with increased mortality.

bulletNonischemic CRVO may resolve completely without any complications in about 10% of cases. In about 50% of patients, vision may be 20/200 or worse. One third of patients may progress to the ischemic type, commonly in the first 6-12 months after presentation.
bulletIn more than 90% of patients with ischemic CRVO, final visual acuity may be 20/200 or worse. Anterior segment neovascularization with associated neovascular glaucoma develops in more than 60% of cases. This can happen within a few weeks and up to 1-2 years afterward.
bulletIt has been reported that the fellow eye may develop retinal vein occlusion in about 7% of cases within 2 years. In another report, the 4-year risk of developing second venous occlusion is 2.5% in the same eye and 11.9% in the fellow eye. Neovascular glaucoma may result in a painful blind eye.

Race: CRVO does not have any particular racial preference.

Sex: CRVO occurs slightly more frequently in males than in females.

Age: More than 90% of CRVO occurs in patients older than 50 years, but it has been reported in all age groups.

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History: Direct review of systems toward various systemic and local factors predisposing the CRVO.

bulletSignificant history includes the following:
bulletHypertension
bulletDiabetes mellitus
bulletCardiovascular disorders
bulletBleeding or clotting disorders
bulletVasculitis
bulletAutoimmune disorders
bulletUse of oral contraceptives
bulletClosed-head trauma
bulletAlcohol consumption

 

bulletAmount of physical activity

 

bulletPrimary open-angle glaucoma or angle-closure glaucoma
bulletOcular symptoms at initial presentation
bulletAsymptomatic
bulletDecreased vision
bulletVisual loss can be sudden or gradual, over a period of days to weeks. Visual loss ranges from mild to severe. Patients can present with transient obscurations of vision initially, later progressing to constant visual loss.

 

bulletPhotophobia

 

bulletPainful blind eye

 

bulletRedness of eyes
bulletOcular symptoms in later stages
bulletDecrease of vision
bulletPain in the eyes
bulletDiscomfort
bulletRedness
bulletWatering

Physical: Patients should undergo a complete eye examination, including visual acuity, pupillary reactions, slit lamp examination of the anterior and posterior segments, undilated examination of the iris, gonioscopy, fundus examination with indirect ophthalmoscope, and fundus contact lens.

bulletVisual acuity: Best-corrected vision always should be obtained. It is one of the important indicators of final visual prognosis.
bulletPupillary reactions may be normal and may present with relative afferent pupillary reflex. If iris has abnormal blood vessels, pupil may not react.
bulletConjunctiva: Advanced stages may show congestion on conjunctival and ciliary vessels.
bulletCornea: Advanced stages may show diffuse corneal edema obscuring the visibility of internal structures.
bulletIris may be normal. Advanced stages may show neovascularization. These vessels are detected best on an undilated iris. Initially, vessels may be seen around pupillary margins and peripheral iridectomy openings if present.
bulletAnterior chamber angle is examined by gonioscopy. This is examined best in an undilated iris. Initially, it may show neovascularization with open angles and later show total peripheral anterior synechia and closed angles.
bulletFundus examination
bulletRetinal hemorrhages may present in all 4 quadrants.
bulletHemorrhages can be superficial, dot and blot, and/or deep.

 

bulletIn some patients, hemorrhages may be seen in peripheral fundus only.

 

bulletHemorrhages can be mild to severe, covering the whole fundus giving a "blood and thunder appearance."
bulletDilated tortuous veins: Veins may be dilated and tortuous.
bulletOptic disc edema: Optic disc may be swollen during early stage disease.
bulletCotton-wool spots are more common with nonischemic CRVO. Usually, they are concentrated around the posterior pole. Cotton-wool spots may resolve in 2-4 months.
bulletNeovascularization of the disc
bulletFine abnormal neovascularization on the disc (NVD) or within 1 disc diameter from the disc may be present.
bulletNVD indicates severe ischemia of the retina.
bulletSometimes NVD is difficult to differentiate from optociliary shunt vessels.
bulletNVD can lead to preretinal or vitreous hemorrhage.
bulletNeovascularization elsewhere

 
bulletNeovascularization elsewhere (NVE) is not as common as NVD.

 

bulletNVE indicates severe ischemia of the retina.

 

bulletNVE can lead to preretinal or vitreous hemorrhage.

bulletOptociliary shunt vessels are abnormal blood vessels on the disc, directing blood from retinal circulation to choroidal circulation, which indicate good compensatory circulation.

 

bulletPreretinal or vitreous hemorrhage

 

bulletMacular edema with or without exudates

 

bulletCystoid macular edema

 

bulletLamellar or full-thickness macular hole

 

bulletOptic atrophy

 

bulletPigmentary changes in the macula

Causes: Central retinal vein obstruction has been associated with various systemic pathological conditions, although the exact cause and effect relationship has not been proven. Some of the conditions in which CRVO has been associated include the following:

bulletSystemic vascular disease
bulletHypertension
bulletDiabetes mellitus
bulletCardiovascular disease
bulletBlood dyscrasias
bulletPolycythemia vera
bulletLymphoma
bulletLeukemia
bulletClotting disorders
bulletActivated protein C resistance
bulletLupus anticoagulant
bulletAnticardiolipin antibodies
bulletProtein C

 

bulletProtein S

 

bulletAntithrombin III
bulletParaproteinemia and dysproteinemias
bulletMultiple myeloma

 

bulletCryoglobulinemia
bulletVasculitis

 
bulletSyphilis

 

bulletSarcoidosis

bulletAutoimmune disease - Systemic lupus erythematosus
bulletOral contraceptive use in women

 

bulletOther rare associations

 
bulletClosed-head trauma

 

bulletOptic disc drusen

 

bulletArteriovenous malformations of retina

bulletThe Eye Disease Case-Control Study Group reported that risk of CRVO is decreased in men with increasing level of physical activity and increasing levels of alcohol consumption. The same study group reported decreased risk of CRVO with use of postmenopausal estrogens and increased risk with higher erythrocyte sedimentation rates in women.
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Branch Retinal Vein Occlusion
Ocular Ischemic Syndrome
 


Other Problems to be Considered:

Hypertensive retinopathy
Retinopathy due to anemia
Retinopathy due to thrombocytosis

 
 
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Lab Studies:
 

bulletNo laboratory studies are indicated routinely in the diagnosis of CRVO.
bulletIn older patients, laboratory testing should be directed toward identifying systemic vascular problems.
bulletIn young patients, laboratory testing may be tailored depending upon individual findings, to include the following:
bulletComplete blood count
bulletGlucose tolerance test
bulletLipid profile
bulletSerum protein electrophoresis
bulletChemistry profile
bulletHematologic tests
bulletSyphilis serology
bulletIn addition, thrombophilia screening, activated protein C resistance, lupus anticoagulant, anticardiolipin antibodies, protein C, protein S, and antithrombin III may be completed.

Imaging Studies:
 

bulletColor Doppler imaging is a noninvasive quantitative method of assessing the retrobulbar circulation. Detection of low venous velocities has been used to predict the onset of iris neovascularization. At present, this is performed as an investigational procedure in large facilities.

Other Tests:
 

bulletFluorescein angiography is the most useful test for the evaluation of retinal capillary nonperfusion, posterior segment neovascularization, and macular edema.
bulletFluorescein angiography is one of the tests used in the classification of CRVO. Areas of capillary nonperfusion are visualized as hypofluorescence, but hemorrhages can block fluorescence and give a similar picture. Therefore, in early stages of the disease process, due to extensive hemorrhages, fluorescein angiography gives little information regarding the perfusion status of the retina. Once the hemorrhages clear, areas of capillary nonperfusion can be detected as hypofluorescence in the fluorescein angiography.
bulletVarious studies have reported different criteria for defining ischemic versus nonischemic CRVO based on extent of capillary nonperfusion of the retina. The amount of retinal nonperfusion ranges from 10-30 disc areas.
bulletIn addition, fluorescein angiography may show delayed arteriovenous transit, staining along the retinal veins, microaneurysms, arteriovenous collaterals, NVD, NVE, and dilated optic nerve head capillaries.
bulletIn a nonischemic central retinal vein obstruction, angiography may show minimal or absent retinal capillary nonperfusion, staining along the retinal veins, microaneurysms, and dilated optic nerve head capillaries. Resolved CRVO may be completely normal.
bulletMacular edema may be detected as leakage from perifoveal capillaries, leakage from microaneurysms, or diffuse leakage on fluorescein angiography. If extensive edema is present, fluorescein angiography may show pooling of dye in large cystoid spaces. In addition, capillary nonperfusion around the fovea may indicate macular ischemia. If macular edema persists, pigmentary changes become evident.
bulletElectroretinography (ERG) is another useful test to evaluate functional status of the retina and to classify CRVO.
bulletIn ERG waveform, b wave and a wave are produced by the inner and outer retina, respectively.
bulletIn central retinal vein obstruction, perfusion of inner retina is affected, so that amplitude of the b wave is decreased relative to the a wave; the ratio of b to a has been shown to be reduced. Some studies indicate that a b-to-a ratio of less than 1 suggests an ischemic central retinal vein obstruction.

Histologic Findings: Not many histopathologic reports exist in the literature. A report of histologic sections of 29 eyes with central retinal vein obstruction showed a fresh or recanalized thrombus at or just posterior to the lamina cribrosa. Within the thrombi, a mild lymphocytic infiltration with prominent endothelial cells was seen. Loss of the inner retinal layers consistent with inner retinal ischemia also was seen.

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Medical Care: No known effective medical treatment is available for either prevention or the treatment of CRVO. Identifying and treating any systemic medical problems to reduce further complications is important. Because the exact pathogenesis of the CRVO is not known, various medical modalities of treatment have been advocated by multiple authors with varying success in preventing complications and preserving vision.

bulletThe following is a list of those advocated treatments:
bulletAspirin
bulletSystemic anticoagulation with coumadin, heparin, alteplase
bulletLocal anticoagulation with intravitreal injection of alteplase
bulletFibrinolytic agents
bulletSystemic corticosteroids

 

bulletAnti-inflammatory agents

 

bulletIsovolumic hemodilution

 

bulletPlasmapheresis

Surgical Care: Laser photocoagulation is the known treatment of choice in treatment of various complications associated with retinal vascular diseases (eg, diabetic retinopathy, branch retinal vein occlusion). Panretinal photocoagulation (PRP) has been used in the treatment of neovascular complications of CRVO for a long time. However, no definite guidelines exist regarding exact indication and timing of PRP. A National Eye Institute sponsored multicenter prospective study, the Central Vein Occlusion Study (CVOS) gave guidelines for treatment and follow-up care of patients with CRVO.

bulletNeovascularization: CVOS evaluated the efficacy of prophylactic PRP in eyes with 10 or more disc areas of retinal capillary nonperfusion, confirmed by fluorescein angiography, in preventing development of 2 clock hours of iris neovascularization or any angle neovascularization or whether it is more appropriate to apply PRP only when iris neovascularization or any angle neovascularization occurs. CVOS concluded that prophylactic PRP did not prevent the development of iris neovascularization and recommended to wait for the development of early iris neovascularization and then apply PRP.
bulletArgon green laser usually is used. Laser parameters should be about 500-mm size, 0.1-0.2 second duration, and power should be sufficient to give medium white burns. Laser spots are applied around the posterior pole, extending anterior to equator. They should be about 1 burn apart and total 1200-2500 spots.
bulletIf ocular media is hazy for laser to be applied, cryoablation of the peripheral fundus is performed. About 16-32 transscleral cryo spots are applied from ora serrata posteriorly.
bulletMacular edema: CVOS evaluated the efficacy of macular grid photocoagulation in preserving or improving central visual acuity in eyes with macular edema due to central vein occlusion (CVO) and best-corrected visual acuity of 20/50 or poorer. Macular grid photocoagulation was effective in reducing angiographic evidence of macular edema, but it did not improve visual acuity in eyes with reduced vision due to macular edema from CVO. At present, the results of this study do not support a recommendation for macular grid photocoagulation for macular edema.
bulletChorioretinal venous anastomosis is performed by creating an anastomosis to bypass the site of venous occlusion in the optic disc. In this procedure, retinal veins are punctured, either using laser or by surgery, through the retinal pigment epithelium and the Bruch membrane into the choroid, thereby developing anastomotic channels into the choroid. This reduces macular edema and may improve vision in nonischemic CRVO. Only a few groups of researchers have produced good results, and other investigators have not reproduced results yet. Major complications have been reported, and further work is necessary prior to widespread use of this technique.

Consultations: A general ophthalmologist should consult a retinal specialist for management of CRVO complications. They also should consult an internist for proper evaluation and management of any systemic medical problems. If patients develop neovascular glaucoma, a glaucoma specialist should be consulted.

Diet: Diet should be tailored to systemic medical problems.

Activity: No restrictions usually exist. If patients develop vitreous hemorrhage, they are advised to avoid strenuous activities, sleep with few pillows, and avoid bending and lifting heavy weights.

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Further Outpatient Care:
 

bulletSince neovascular complications and development of second venous occlusions can develop after CRVO, all these patients need follow-up care for long periods of time.
bulletCVOS recommended careful observation with frequent follow-up examinations in the early months for detection of iris neovascularization and prompt treatment.
bulletPatients with poor initial visual acuity should be monitored every month during the first few months and spaced thereafter, depending on course of the disease. These criteria apply more for patients with ischemic CRVO than with patients with nonischemic CRVO.
bulletWith any associated complications, follow-up care should be individualized.

Deterrence/Prevention:
 

bulletOptimal control of associated systemic diseases may reduce the incidence of similar occlusions in the fellow eye.
bulletEven though controversial, good control of intraocular pressure in patients known to have glaucoma may prevent CRVO.

Complications:
 

bulletOcular neovascularization
bulletAnterior segment neovascularization leading to neovascular glaucoma
bulletPosterior segment neovascularization leading to vitreous hemorrhage
bulletMacular edema
bulletMacular edema is the common cause of decreased vision in CRVO, more so in the nonischemic type.
bulletMay resolve with good visual return
bulletMay develop permanent degenerative changes with poor visual prognosis
bulletMay develop cystoid macular edema leading to lamellar or full-thickness macular hole
bulletCellophane maculopathy and macular pucker
bulletOptic atrophy

Prognosis:
 

bulletNonischemic CRVO
bulletComplete recovery with good visual recovery occurs only in about 10% of cases.
bulletFifty percent of patients will have 20/200 or worse vision.
bulletAbout one third of patients convert to ischemic CRVO. The CVOS group noted that of 547 eyes initially diagnosed to have nonischemic central retinal vein obstructions, 185 (34%) progressed to become ischemic central retinal vein obstructions within 3 years; 15% converted within the first 4 months.
bulletIschemic CRVO
bulletMore than 90% of patients will have 20/200 or worse vision.
bulletAbout 60% of patients develop ocular neovascularization with associated complications.
bulletAbout 10% of patients can develop CRVO or other type of vein occlusions either within the same eye or contralateral eye within 2 years.

Patient Education:
 

bulletGood control of systemic medical problems
bulletRegular medical and ophthalmologic checkups
  MISCELLANEOUS Section 8 of 10   Click here to go to the previous section in this topic Click here to go to the top of this page Click here to go to the next section in this topic
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Medical/Legal Pitfalls:
 

bulletIn view of multiple systemic problems these patients can have, it is important to refer patients to an appropriate specialist to avoid any legal ramifications.
bulletInitially, these patients should be referred to an internist.
bulletPatients may be referred to a retinal and glaucoma specialist as required to treat advanced problems.
  PICTURES Section 9 of 10   Click here to go to the previous section in this topic Click here to go to the top of this page Click here to go to the next section in this topic
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Caption: Picture 1. Recent onset central retinal vein occlusion, showing extensive hemorrhages in the posterior pole giving the "blood and thunder appearance"
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Caption: Picture 2. Peripheral fundus view of the same patient in Picture 1, showing hemorrhages extending all over the fundus
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Caption: Picture 3. Fluorescein angiograph of the same patient in Picture 1, showing hypofluorescence due to blockage from hemorrhages in the retina. It is not useful to perform a fluorescein angiogram in acute stages of the disease.
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Caption: Picture 4. Fundus picture of the same patient in Picture 1, showing resolving neovascularization of the disc and panretinal photocoagulation scars
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Caption: Picture 5. Fluorescein angiogram of the same patient in Picture 1, taken more than 1 year later, showing persistent cystoid macular edema with good laser spots
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Caption: Picture 6. Patient with nonischemic central retinal vein occlusion presented with dilated, tortuous veins and superficial hemorrhages
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Caption: Picture 7. Fundus picture of the same patient in Picture 6, showing resolved hemorrhages and pigmentary changes in the macula several months later
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Caption: Picture 8. Central retinal vein occlusion showing significant disc edema with dilated tortuous veins and scattered retinal hemorrhages
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Caption: Picture 9. Fluorescein angiogram of the same patient in Picture 8, showing leakage from disc, staining of retinal veins
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Caption: Picture 10. Fundus of a patient with nonischemic central retinal vein occlusion, showing few scattered peripheral fundus hemorrhages
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Caption: Picture 11. Scattered retinal hemorrhages in a patient with central retinal vein occlusion
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Caption: Picture 12. Fluorescein angiogram of a patient with nonischemic central retinal vein occlusion, showing staining of dilated tortuous veins with leakage into macula in a cystoid pattern
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Caption: Picture 13. Fluorescein angiogram of the same patient in Picture 12, showing perifoveal capillary leakage in a cystoid pattern in late phases of angiogram
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Caption: Picture 14. Arteriovenous phase of fluorescein angiograph showing perifoveal capillary leakage in a patient with nonischemic central retinal vein occlusion
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Caption: Picture 15. Late phase of fluorescein angiograph of the same patient in Picture 13, showing cystoid pattern of leakage from perifoveal dilated leaking capillary network
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Caption: Picture 16. Fundus picture of a well-compensated, old central retinal vein occlusion showing optociliary shunt vessels
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Caption: Picture 17. Red-free photo of the same patient in Picture 16, showing prominent optociliary shunt vessels
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  BIBLIOGRAPHY Section 10 of 10   Click here to go to the previous section in this topic Click here to go to the top of this page
Author Information Introduction Clinical Differentials Workup Treatment Follow-up Miscellaneous Pictures Bibliography
 

bulletBaxter GM, Williamson TH: Color Doppler flow imaging in central retinal vein occlusion: a new diagnostic technique? Radiology 1993 Jun; 187(3): 847-50[Medline].
bulletBreton ME, Quinn GE, Keene SS, Dahmen JC, Brucker AJ: Electroretinogram parameters at presentation as predictors of rubeosis in central retinal vein occlusion patients. Ophthalmology 1989 Sep; 96(9): 1343-52[Medline].
bulletBrowning DJ, Rotberg MH: Vitreous Hemorrhage complicating laser-induced chorioretinal anastomosis for central retinal vein occlusion. Am J Ophthalmol 1996 Oct; 122(4): 588-9[Medline].
bulletCentral Vein Occlusion Study: Baseline and early natural history report. Arch Ophthalmol 1993 Aug; 111(8): 1087-95[Medline].
bulletCentral Vein Occlusion Study: Evaluation of grid pattern photocoagulation for macular edema in central vein occlusion. The Central Vein Occlusion Study Group M report. Ophthalmology 1995 Oct; 102(10): 1425-33[Medline].
bulletCentral Vein Occlusion Study Group: A randomized clinical trial of early panretinal photocoagulation for ischemic central vein occlusion. The Central Vein Occlusion Study Group N report. Ophthalmology 1995 Oct; 102(10): 1434-44[Medline].
bulletCentral Vein Occlusion Study Group: Natural history and clinical management of central retinal vein occlusion. [published erratum appears in Arch Ophthalmol 1997 Oct;115(10):1275]. Arch Ophthalmol 1997 Apr; 115(4): 486-91[Medline].
bulletCentral Vein Occlusion Study Group: A randomized clinical trial of early panretinal photocoagulation for ischemic central vein occlusion. The Central Vein Occlusion Study Group N report. Ophthalmology 1995 Oct; 102(10): 1434-44[Medline].
bulletCentral Vein Occlusion Study Group.: Central vein occlusion study of photocoagulation therapy. Baseline findings. Online J Curr Clin Trials 1993 Oct 14; Doc No 95:[Medline].
bulletDavid R, Zangwill L, Badarna M, Yassur Y: Epidemiology of retinal vein occlusion and its association with glaucoma and increased intraocular pressure. Ophthalmologica 1988; 197(2): 69-74[Medline].
bulletEccarius SG, Moran MJ, Slingsby JG: Choroidal neovascular membrane after laser-induced chorioretinal anastomosis. Am J Ophthalmol 1996 Oct; 122(4): 590-1[Medline].
bulletGreen WR, Chan CC, Hutchins GM: Central retinal vein occlusion: a prospective histopathologic study of 29 eyes in 28 cases. Retina 1981; 1: 27-55.
bulletHayreh SS, Zimmerman MB, Podhajsky P: Incidence of various types of retinal vein occlusion and their recurrence and demographic characteristics. Am J Ophthalmol 1994 Apr 15; 117(4): 429-41[Medline].
bulletHayreh SS: Retinal vein occlusion. Indian J Ophthalmol 1994 Sep; 42(3): 109-32[Medline].
bulletHayreh SS: Classification of central retinal vein occlusion. Ophthalmology 1983 May; 90(5): 458-74[Medline].
bulletLuttrull JK: Epiretinal membrane and traction retinal detachment complicating laser- induced chorioretinal venous anastomosis. Am J Ophthalmol 1997 May; 123(5): 698-9[Medline].
bulletMcAllister IL, Constable IJ: Laser-induced chorioretinal venous anastomosis for treatment of nonischemic central retinal vein occlusion. Arch Ophthalmol 1995 Apr; 113(4): 456-62[Medline].
bulletMitchell P, Smith W, Chang A: Prevalence and associations of retinal vein occlusion in Australia. The Blue Mountains Eye Study. Arch Ophthalmol 1996 Oct; 114(10): 1243-7[Medline].
bulletWilliamson TH: Central retinal vein occlusion: what's the story? Br J Ophthalmol 1997 Aug; 81(8): 698-704[Medline].