Extensive Retinal Hemorrhages Research Articles

Retinal Vasculopathy and Choroiditis after Pegcetacoplan Injection: Clinicopathologic Support for a Drug Hypersensitivity Reaction

PurposeTo report the detailed histopathology of two enucleated eyes from two patients who developed severe visual loss associated with retinal hemorrhages, vessel sheathing and vascular nonperfusion after administration of an initial dose of intravitreal pegcetacoplan, and to propose, with supportive histopathology, the pathogenesis of the clinical syndrome previously termed hemorrhagic occlusive retinal vasculitis (HORV). DesignCase series. SubjectsTwo enucleated eyes from two patients treated with intravitreal pegcetacoplan. MethodsRetrospective, multicenter consecutive clinical-pathologic analysis. Main Outcome MeasuresHistopathologic review and immunophenotypic characterization. ResultsBoth patients presented with inflammation and significant vision loss nine days following the initial injection of pegcetacoplan with no subsequent improvement and underwent enucleation for pain control. Histologic examination of the enucleated eyes (patient one at 4 months post-injection and patient two at 40 days) revealed extensive vascular thrombosis, retinal hemorrhages and necrosis, and a dense inflammatory infiltrate in the uvea and variably the optic nerve, episclera, and muscle tendons composed of predominantly of T-cells, macrophages, and eosinophils. Notably, the inflammatory infiltrate was absent from the retina. In addition, one eye demonstrated multiple foci of glomerular-like vascular proliferations in the uveal tract and thrombosis with focal recanalization of vessels in the optic nerve. ConclusionDrug-induced, immune-mediated, retinal vasculopathy and choroiditis (DIRVAC) is a rare complication following pegcetacoplan injection. Although some limitations arise in interpretation of histopathologic findings due to compensatory changes in the eyes over time (prior to enucleation), the authors propose that the combined clinical, histopathologic, and immunohistochemical findings suggest a mixed-type, delayed hypersensitivity reaction as mechanism of initial injury.

Ophthalmic Findings of Abusive Head Trauma in Korea from a Single-large Trauma Center, 2011-2022

Purpose: To investigate the ophthalmic findings associated with abusive head trauma (AHT) at a major trauma center in Korea.Methods: We retrospectively reviewed the medical records of 14 children with AHT referred for an ophthalmologic examination at a large regional trauma center from January 2011 to December 2022. We analyzed pertinent findings for AHT and its sequelae on brain computed tomography, skeletal surveys obtained using X-ray, external photographs, and fundus photographs taken with a portable fundus camera.Results: Among 14 patients (mean age, 7.4 months) with subdural hematoma from AHT, nine (64.3%) had retinal hemorrhages. Eight of these patients (88.9%) required neurosurgery, while only one child (20.0%) among the five without retinal hemorrhages needed drainage for brain hemorrhages. All patients with retinal hemorrhages (mean age, 6.0 months) presented with numerous multilayered hemorrhages across the entire retina including dome-shaped hemorrhages (six patients), perimacular ridges (one patient), and retinal hemorrhages at different stages of healing (one patient). The retinal hemorrhages resolved within an average of 2.4 months without necessitating retinal surgery. In five patients followed for an average of 28.2 months, there were instances of optic disc pallor in three, cortical visual impairment in one, and fovea scarring in one.Conclusions: More than half of the Korean children with AHT had extensive retinal hemorrhages leading to ophthalmic sequelae in the majority. These findings support the need for long-term active ophthalmic follow-up in AHT cases.

Hyperviscosity Syndrome-related Retinopathy in a Patient with Waldenström Macroglobulinemia

Purpose: We report a case of retinopathy related to bilateral hyperviscosity syndrome in a patient with Waldenström macroglobulinemia.Case summary: A 77-year-old male presented with reduced visual acuities of both eyes 2 months in duration. Fundus examination revealed extensive flame-shaped retinal hemorrhages and venous congestion. Optical coherence tomography (OCT) evidenced macular edema and OCT angiography (OCTA) indicated damage to the superficial and deep capillary plexi of retina and choriocapillaris and dilated large choroidal vessels. We diagnosed central retinal vein occlusion with macular edema and prescribed intravitreal bevacizumab and posterior subtenon triamcinolone injections, however, the edema did not improve. As retinopathy associated with hematological disease was thus suspected, we referred the patient to our department of hematology and oncology for further assessment. He was diagnosed with Waldenström macroglobulinemia. After initiation of chemotherapy, the retinal hemorrhage in and macular edema of both eyes decreased. After six chemotherapy cycles, the retinal hemorrhages resolved and the macular edema improved in both eyes. OCTA revealed that the choroidal vessel dilation also improved.Conclusions: In elderly patients presenting with central retinal vein occlusions and macular edema of both eyes, it is important to assess whether the retinopathy is associated with a hyperviscosity syndrome linked to a hematological disease.

Multimodal Imaging Characteristics and Risk Factors Analysis of Waldenström Macroglobulinemia Retinopathy

To summarize the multimodal imaging features and analyze the risk factors of Waldenström macroglobulinemia retinopathy (WMR). Retrospective, cross-sectional study. Patients diagnosed with Waldenström macroglobulinemia and underwent ophthalmic examination in Peking Union Medical College Hospital in the last decade were included. Multimodal imaging characteristics of WMR were summarized. Univariate and multivariate logistic regression analysis of WMR and potential systemic and ocular factors was performed. A total of 50 WM patients were included in this study, and 28 patients had WMR in at least one eye. WMR was found to have worse LogMAR visual acuity (VA) (0.52±0.54 vs. 0.21±0.18, p=0.009), and was characterized by tortuous retinal vessels, extensive retinal hemorrhage, and distinctive shape of macular edema, etc. In univariate analysis, the presence of WMR was significantly associated with the mean VA (LogMAR), serum RBC counts, serum PLT counts, HGB level, serum M protein, serum IgM level, and LDH (with p<0.05). In multivariate analysis, WMR was significantly correlated with M protein (adjusted OR=1.127, 95% CI 1.052-1.209, p= 0.001) and serum IgM (adjusted OR=1.059, 95% CI 1.023-1.095, p= 0.001) with the predicted AUC of 0.820 and 0.859, respectively. The optimal cut-off values were 26.2g/L for M protein, and 51.0g/L for IgM, which accounts for a sensitivity of 95.4% and 95.4%, and specificity of 64.3% and 60.7%. Retinopathy of WM has specific characteristics in ophthalmic examinations. Serum IgM levels and M protein are good predictors of WMR, which could attach important value of fundus examinations for WM patients.

Frosted Branch Angiitis; Case Series and Literature Review

ABSTRACT Purpose Frosted branch angiitis (FBA) is a rare form of retinal vasculitis. Four case series and an extensive literature review of 236 cases were conducted to clarify the characteristics of this rare condition. Methods Case series and literature review. Results An analysis of the reported cases revealed that a majority (54.6%) developed FBA in the presence of an underlying disease, with recurrence and complications requiring surgical intervention being rare. The frequency of bilateral occurrence (55.0%) and prevalence in female patients (45.0%) were noted to be lower than previously reported. Conclusion In general, idiopathic FBAs are more likely to be bilateral and diagnosed at a younger age than secondary FBAs. In idiopathic FBA, fundus involvement is more generalized, exudates tend to be more translucent, and extensive retinal hemorrhage tends to be less frequent. However, there were no differential characteristic fundus features that clearly distinguished idiopathic FBA from secondary FBA.

Malignant Idiopathic Intracranial Hypertension

A 24-year-old woman with a body mass index of 28 presented with 2 weeks of progressive vision loss and 2 months of headache. Visual acuity was 20/400 in both eyes, and examination showed Frisén grade 5 papilledema with extensive retinal hemorrhage and exudates (Figs A[OD = right eye] and B [OS = left eye]). Imaging did not show evidence of mass lesions or dural venous sinus thrombosis, and lumbar puncture opening pressure was 46 cm H2O with normal cerebrospinal fluid contents, consistent with idiopathic intracranial hypertension.

Computer Algorithms Support Physician Decisions in Traumatic Head Injury.

Computer Algorithms Support Physician Decisions in Traumatic Head Injury.

Haemorrhagic Retinopathy Associated with Zieve Syndrome

Zieve syndrome, a scarcely reported case is a triad of jaundice, haemolytic anaemia and hyperlipidaemia that develops secondary to alcohol-induced liver injury. Herein, a case of 23-year-old young male with history of steatohepatitis is reported who presented with diminution of vision in both eyes. On fundoscopy, there were extensive retinal haemorrhages involving fovea. Workup was consistent with haemolytic anaemia with no source of active bleeding. The patient was managed with supportive treatment. No specific ocular treatment was given. However, the vision gradually improved during follow-up. A rare association of zieve syndrome with haemorrhagic retinopathy is described here. Moreover, zieve syndrome has been described in the literature, mostly in non-English language case studies and is still under-recognised and under-reported. Diagnosis should be made quickly to avoid unnecessary invasive diagnostic interventions.

Granulicatella adiacens endophthalmitis after phacoemulsification cataract surgery.

Two cases of acute postoperative Granulicatella adiacens endophthalmitis following uneventful phacoemulsification surgery are reported. The cases had intracameral cefuroxime as antibiotic prophylaxis, visual acuity (VA) was perception of light and hand movements, respectively, and there was severe anterior chamber inflammation and dense vitritis with no fundal view. Vitreous biopsy identified G adiacens using genome sequencing. Intraoperative findings included extensive retinal hemorrhages, abscesses, occluded retinal vessels, and retinal necrosis. Because of the ischemic retinal appearance, pan-retinal photocoagulation endolaser was performed. Postoperatively both cases developed rhegmatogenous retinal detachment (RRD) anterior to the laser marks and were successfully treated. VA improved to 6/18 and 6/60, respectively. Hyperreflective dots and Henle fiber layer hemorrhage are reported for the first time, to the authors' knowledge, in association with endophthalmitis. The successful outcome suggested that intraoperative pan-retinal photocoagulation could be considered in such ischemic cases to prevent rubeosis and to reduce the risk for postoperative RRD or limit its extent if it recurs.

Successful treatment of endogenous endophthalmitis with extensive subretinal abscess: a case report

BackgroundEndogenous endophthalmitis could lead to a devastating outcome without a prompt and appropriate management. We report a case of advanced endogenous Klebsiella pneumoniae endophthalmitis with extensive subretinal abscess that was successfully treated with a vitrectomy.Case presentationA systemically well 61-year-old man complained of ocular pain and visual decrease in his right eye for eighteen days. Ophthalmic examination showed dense inflammation in the anterior chamber and vitreous body. Systemic investigations discovered diabetes and no specific site of systemic infection was found after hospitalization. The inflammation continued to worsen after the intravitreal antibiotic injection. Therefore, a pars plana vitrectomy combined with phacoemulsification was performed. Intraoperatively, a white elevated, fluffy mass with the overlying retinal whitening and necrosis was revealed in superior periphery. In addition to this, extensive retinal hemorrhages and five adjacent subretinal whitish masses with exudative retinal detachment were observed in the posterior pole and inferior quadrants, which were suggestive of extensive subretinal abscess with intense overlying retinal inflammation. The excision of white fluffy mass superiorly was performed without retinotomy and aspiration of extensive subretinal abscess. The polymerase chain reaction of vitreous samples was positive for Klebsiella pneumonia. Intravitreal 2 mg/0.1 ml ceftazidime were repeated. Nine days after the surgery, the inflammation significantly subsided and the retina reattached. The patient was in a stable condition at subsequent visit eight months later.ConclusionThe delay in an accurate diagnosis and treatment caused extensive subretinal abscess combined with endogenous endophthalmitis. The treatment modality of subretinal abscess is typically individualized to the patient’s presentation. If the retina overlying the abscess is not necrotic, the extensive subretinal abscess can quickly absorbed after vitrectomy, retinotomy with aspiration of the abscess should be avoided to decrease the risk of retinal detachment.

Is rotational trauma the only relevant pathogenic mechanism in hemorrhagic retinopathy of shaken baby syndrome?

The pathogenesis of ocular hemorrhage in abusive head trauma/shaken baby syndrome (AHT/SBS) is complex. Whereas severe hemorrhagic retinopathy has a robust association with AHT/SBS, mild disease is less specific. We report the ocular and cerebral findings in an 8-month-old girl with spastic torticollis who presented following confessed, violent sagittal plane acceleration-deceleration with impact. Autopsy revealed subdural hemorrhage over the convexities, ischemic brain injury, and preexisting polymicrogyria. Ocular examination showed extensive but unilateral retinal hemorrhage, extending broadly to the ora serrata, with perimacular retinal folds and retinoschisis. The retina of the right globe was entirely normal. The findings in this case, suggest that movement of the globe within the orbit with acceleration-deceleration, in this case greater on one side due to torticollis, may be the primary factor for severe hemorrhagic retinopathy associated with AHT/SBS. The findings argue against putative global mechanisms, such as intracranial hypertension.

Minor injuries… major implications: Watching out for sentinel injuries.

An otherwise healthy two-month-old boy presented to his general paediatrician for follow-up of gastroesophageal reflux disease. His mother reported that since starting ranitidine two weeks earlier, his discomfort following feeds and previously reported excessive crying had both improved. She had no new concerns about the child’s health but reported that she had observed a small amount of blood on the infant’s crib sheet in the past week, which she could not explain. On examination, he appeared alert with normal vital signs and had adequate weight gain since his last visit. Cardiovascular, respiratory, abdominal and neurological examinations were within normal limits. The paediatrician made note of a solitary, bluegreen bruise on the baby’s left anterior thigh, which measured approximately 2 cm in diameter. He inquired about its origin; however, the child’s mother stated that she had not noticed it and was unsure of the causal mechanism. There were no other skin findings, bruises or petechiae noted. Examination of the oral cavity was unremarkable. The paediatrician prescribed a three-month supply of ranitidine and recommended routine follow-up with the family physician for well-baby care and immunizations. One month later, the child was brought to the local emergency department by ambulance after his mother found him unresponsive in his crib, with jerking of his arms and legs. There was no history of recent illness or trauma. On examination, he was pale and lethargic. His heart rate was 118 beats/min and his blood pressure was 86/58 mmHg. He was afebrile, with shallow respirations and an oxygen saturation of 96% on room air. He exhibited tonicclonic movement of his right upper and lower extremities. His pupils were equal and reactive; however, intermittent nystagmus was noted. An area of soft tissue swelling was palpable at the base of the skull and there were two green bruises on the left cheek. The child was admitted to the paediatric medical unit. Computed tomography of the head revealed bilateral areas of subdural haemorrhage. His coagulation profile, complete blood count and electrolytes were all normal. Ophthalmological examination demonstrated bilateral extensive and multilayered retinal haemorrhages. Three posterior rib fractures with callous formation were present on skeletal survey. A report was made to the local child welfare authority because of suspected physical abuse and nonaccidental head injury.

Anatomic and functional outcome of eyes with massive submacular hemorrhage secondary to retinal macroaneurysm submitted to vitrectomy

Purpose: To report the anatomic and functional outcome in patients with severe visual loss after acute massive submacular hemorrhage secondary to retinal arterial macroaneurysm submitted to vitrectomy and subretinal recombinant tissue plasminogen activator injection. Methods: Retrospective, observational, case-series of 4 eyes of 4 patients submitted to pars plana posterior vitrectomy (ppV) combined with internal limiting membrane (ILM) removal and subretinal recombinant tissue plasminogen activator (rtPA-12.5 mg/0.1 ml) injection with dilute (20%) sulfur hexafluoride (SF6) gas in the vitreous cavity of eyes with recent onset (≤7 days) massive macular hemorrhage due to retinal arterial macroaneurysm (RAMA). Optical coherence tomography (OCT) was obtained both at presentation and during follow up. Results: Patients ranged in age from 63 to 78 years and all had systemic arterial hypertension. Visual acuity at presentation ranged from hand motions to count fingers at 50 cm. All eyes showed extensive retinal hemorrhage involving more than two-thirds of macular area. The time between the onset of symptoms and the surgery ranged from 3 to 7 days. After a mean postoperative follow-up of 15.5 ± 5.19 months (range, 10-22 months), all eyes showed visual acuity improvement and final visual acuity ranged from 20/30 to 20/80. All had complete displacement of the subretinal hemorrhage from the fovea after the surgery. OCT images showed neurosensory retina thinning and disruption of the reflective line that represents the junction between inner and outer photoreceptors segments (IS/OS line) beneath the macular area and absence of the external limiting membrane (ELM). Conclusions: ppV associated with subretinal rtPA injection with intravitreal gas seems to be a safe and effective technique to promote visual improvement in patients with multilevel macular hemorrhage secondary to RAMA. Despite functional improvement, OCT images demonstrate that submacular hemorrhage leads to permanent structural damage to the neurosensory retina, especially to the outer photoreceptors layers.

Blinding bilateral hyperviscosity retinopathy in a 43-year-old nigerian male with lymphoplasmacytic lymphoma: a case report and management challenges.

Lymphoplasmacytic lymphomas are rare and may present with uncommon and devastating symptoms. We report a case of a 43-year-old male who presented with bleeding gums and sudden onset of bilateral blindness but was not on anticoagulants and had no family history of bleeding disorder. He had bilateral hyperpigmented infraorbital skin lesions, visual acuities (VA) of hand motion in both eyes (blindness), round and sluggish pupils, and bilateral diffuse and extensive retinal haemorrhages obliterating the retinal details with central visual field defects. The optical coherence tomography revealed retinal haemorrhage, oedema, detachment, and diffuse photoreceptors damage. Investigations revealed elevated ESR and β 2 microglobulin, monoclonal peak on serum protein electrophoresis, high IG with lambda restriction on serum, and urine immunofixation with increased lymphocytes and plasma cells in the bone marrow. A diagnosis of lymphoplasmacytic lymphoma complicated by blinding hyperviscosity retinopathy was made. In the absence of an aphaeresis machine, he received four cycles of manual exchange blood transfusion (EBT) and commenced with chlorambucil/prednisolone due to difficulty in obtaining blood for continued EBT. His general condition and VA has improved and he is stable for more than six months into treatment.

Sudden onset bilateral deafness as a presentation of chronic myeloid leukaemia

A young adult male presented with a 3-week history of tinnitus and vertigo and a 3-day history of ataxia and bilateral deafness. An ataxic gait, staccato speech and bilateral sensorineural deafness were noted on examination, together with massive splenomegaly and extensive bilateral retinal haemorrhages. The blood leucocyte count was 605 × 109/l, with karyotype and blood film morphological features consistent with chronic phase chronic myeloid leukaemia. He was not significantly thrombocytopaenic. A magnetic resonance imaging scan demonstrated leucostasis-induced bilateral intracerebral haemorrhages involving the primary auditory cortex (known as Heschl's gyri), with a further haemorrhage in the right lobe of the cerebellum (indicated by arrows). Haematological remission was induced with leucapheresis, hydroxycarbamide and then imatinib. The deafness has not resolved. Cortical deafness is rare and usually occurs following damage to the bilateral temporal or temporoparietal lobes, including the primary auditory cortex. This results in the interruption of the projection fibres from the medial geniculate bodies to the auditory-related areas, resulting in severe, persistent hearing loss (Tanaka, Y., Kamo, T., Yoshida, M. & Yamadori, A., 1991, So called cortical deafness: clinical, neurophysiological and radiological obervations. Brain, 114, 2385-2401).

Retinal Hemorrhages in Children

OBJECTIVE To evaluate the role of intracranial pressure (ICP) in the production of retinal hemorrhage in young children. DESIGN Review of published clinical, postmortem, and experimental research findings worldwide pertinent to our review objective. We used PubMed, MEDLINE, and Ovid Evidence-Based Medicine Reviews as well as references found in other published articles to conduct searches. MAIN EXPOSURES Increased ICP from various etiologies. MAIN OUTCOME MEASURE Hemorrhagic retinopathy, in particular with extension to the periphery, multiple layers, and too-numerous-to-count hemorrhages. The review also considers additional intraocular findings such as retinoschisis and perimacular folds. RESULTS In general, elevated ICP does not cause extensive hemorrhagic retinopathy. Papilledema may be associated with a small number of hemorrhages on or around the optic disc. There are isolated case reports that severe hyperacute ICP elevation, unlike the subacute pressure increase in abusive head injury, in children may rarely result in extensive retinal hemorrhage. These diagnoses are readily distinguished from child abuse. CONCLUSIONS In the absence of the few readily recognizable alternate scenarios, extensive retinal hemorrhage in very young children is not secondary to isolated elevated ICP.

Do true preretinal (subhyaloid) hemorrhages occur in infants?

The recent educational paper by Taylor [4] uses the ambiguous descriptor “preretinal” to describe intraocular hemorrhages. Clinically, this term has been used to describe both “true” preretinal (subhyaloid) hemorrhages and subinternal limiting membrane (subILM) hemorrhages. Emerson et al. [1] uses neither subhyaloid nor preretinal in his pathological description of intraocular hemorrhage and indeed finds the blood to be subILM in all 41 of his cases with macular hemorrhage. Why this is so may be explained by Sebag’s [2] demonstration of a “sheetlike” adherence of the vitreous that encompasses the macula as well as the peripapillary posterior pole in individuals 20 years of age or younger. Thus, although as Srinivasan and Kyle’s [3] picture of a subhyaloid hemorrhage in the macula overlying a subILM hemorrhage in the same eye in a 46 year old proves hemorrhage can occur in either space in an adult, it is possible that in infants the adhesive force between the vitreous and ILM exceeds the cohesive force of the vitreous and thus the blood cannot dissect between the vitreous and the ILM but instead breaks into the vitreous as seen in almost 40% of Emerson’s cases. Taylor also references a piglet study by Coats et al. This study used rotational accelerations over 50 times those achieved by human shaking yet produced only microscopic retinal hemorrhage at the retina/ciliary body junction and anterior chamber hemorrhage (hyphema) and ciliary body disruption. These are findings associated with direct eye trauma. They are not the extensive posterior retinal hemorrhage associated with abusive head trauma. Thus, this study would suggest that the eye findings of abusive head trauma are not produced by rotational acceleration.

Central Retinal Vein Occlusion AssociatedWith Sildenafil (Viagra)

Objectives To report the occurrence of a severe, blinding and irreversible adverse effect following use of the drug sildenafil citrate (Viagra). Methods This is a case report of a 60 year old man who presented with unilateral, suddenonset, painless, visual loss following the use of sildenafil citrate 100mg twice weekly, prescribed for his erectile dysfunction. Examination revealed a visual acuity of 6/6 in the right eye and counting fingers left eye. His left fundus showed marked, diffuse disc edema, extensive retinal hemorrhages in all quadrants, engorged retinal veins and cotton wool spots on the macula. His intraocular pressures were 27mmHg right eye and 25mmHg left eye. Results Fundus fluoresceine angiography confirmed left ischemic central retinal vein occlusion. Conclusion Central retinal vein occlusion is a possible adverse effect of sildenafil use. Physicians should be vigilant while prescribing thismedication and avoid its use in patients with elevated intraocular pressure. KeyWords: Sildenafil, Central retinal vein occlusion, Adverse effects, Erectile dysfunction

Ocular Findings in Raised Intracranial Pressure

We present the case of a 7-month-old female infant who was found crying and limp. She was transported to a hospital where a possible subarachnoid hemorrhage was diagnosed radiologically. Before further studies could be pursued, her condition worsened and she died. The autopsy demonstrated diffuse subarachnoid hemorrhage of the brain and along the spinal cord. The brain, spinal cord, and eyes were retained and examined postfixation. An aneurysm of the middle cerebral artery was identified. Examination of the eyes demonstrated bilateral optic nerve sheath hemorrhage and extensive retinal hemorrhages extending to the ora serrata. A rapid increase in intracranial pressure secondary to subarachnoid hemorrhage following rupture of an aneurysm can result in sequelae similar to those found in inflicted traumatic brain injury. In this case, the rise in intracranial pressure resulted in marked hemorrhage within the optic nerve sheaths as well as intra- and preretinal hemorrhages. Patients with subarachnoid hemorrhage, or other causes of rapidly increased intracranial pressure, may develop ocular hemorrhage (Terson syndrome). This case illustrates the importance of ruling out natural disease before attributing the autopsy findings to trauma, as well as the importance of postmortem fixation of pediatric brains and eyes prior to examination.

Ischaemic change after intravitreal bevacizumab (Avastin®) injection for macular oedema secondary to non‐ischaemic central retinal vein occlusion

Editor, A 65-year-old male patient with a 12-year history of diabetes presented with decreased visual acuity in his right eye of 3 months duration. Best-corrected visual acuity (BCVA) was 0.4 in the right eye (RE) and 1.0 in the left eye (LE). A slit-lamp examination showed grade two bilateral nucleosclerosis with no further abnormalities of the anterior segment. Fundus examination and fluorescein angiography showed flame-shaped retinal haemorrhages in four quadrants and optocillary shunt vessels. These ocular findings were consistent with non-ischaemic central retinal vein occlusion (CRVO). Optical coherence tomography (OCT) showed submacular fluid and macular elevation with a central macular thickness of 533 μm in the RE (Fig. 1). An intravitreal off-label bevacizumab injection was recommended. The patient was informed about the treatment beforehand and provided written informed consent. The injection was performed in a sterile setting and prophylactic topical antibiotics and carbonic anhydrase inhibitor were given for 1 week pre- and post-injection. The intravitreal dosage of bevacizumab was 1.25 mg (0.05 ml). Repeat examinations of visual acuity, intraocular pressure and fundus examinations were scheduled at 1 day, 1 week and 1 month after injection. Three weeks after injection, the patient reported a deterioration of visual acuity in the RE. His visual acuity in the RE was 0.02 and a fundus examination showed disc oedema with extensive flame-shaped retinal haemorrhages in the four retinal quadrants (Fig. 2). A fluorescein angiogram of the RE demonstrated severe non-perfusion including the fovea with blocked hypofluorescence. OCT imaging showed a central macular thickness of 560 μm (Fig. 2). Six months after the injection, his visual acuity in the RE was 0.02 and panretinal photocoagulation was performed. Fundus photography (A), fluorescein angiography (B) and optical coherence tomography (OCT) (C) images at initial presentation. (A) Fundus photograph showing multiple intraretinal haemorrhages and dilated retinal veins with optociliary shunt vessels. (B) Fluorescein angiograph in the late phase showing blocked hypofluorescence with patelloid pattern of dye leakage in the fovea in the right eye. (C) A vertical OCT image of the right eye showing cystoid macular oedema with a central macular thickness of 533 μm. Fundus photography (A), fluorescein angiography (B) and optical coherence tomography (OCT) (C) images 3 weeks after intravitreal bevacizumab administration. (A) Fundus photograph showing extensive flame-shaped retinal haemorrhages and disc swelling. (B) Fluorescein angiograph in the late phase showing severe non-perfusion and dye leakage at the optic disc. (C) Vertical OCT image showing subretinal fluid with a central macular thickness of 560 μm. Bevacizumab represents a new treatment option for early intervention against the formation of macular oedema in various disease conditions. Several short-term results have been published on intravitreal bevacizumab for macular oedema secondary to retinal vein occlusion with CRVO (Iturralde et al. 2006; Spandau et al. 2006; Pai et al. 2007). These reports suggest that intravitreal bevacizumab may reduce macular oedema and increase visual acuity in CRVO. But because a physiological level of vascular endothelial growth factor (VEGF) may be necessary for retinal homeostasis, precautions may have to be taken to avoid the possible negative effects of a complete blockade of VEGF, particularly in primarily ischaemic diseases. However, no report has been issued regarding complications associated with intravitreal bevacizumab except for endophthalmitis. Here, we present a case of non-ischaemic CRVO conversion to ischaemic CRVO after injecting bevacizumab intravitreally. The formation of a new blood flow balance is presumably supported by the formation of collateral vessels with a new drainage route. Spandau et al. (2006) suggested the possibility of complete blockage of VEGF by intravitreal bevacizumab. Although 11–34% of non-ischaemic CRVO cases convert to ischaemic CRVO over a natural course of 3–5 years, we suggest that intravitreal bevacizumab injection affected the outcome in this case (TCVOS 1997). This is because ischaemic CRVO occurred 3 weeks after the injection, when bevacizumab has its peak effect and relatively good initial visual acuity and perfusion status. There were no other patient factors that might cause the ischaemic conversion of CRVO except diabetes. The causes and mechanisms of treatment failure associated with bevacizumab administration must be elucidated further in vitro and by clinical studies. Furthermore, the possibility that bevacizumab has a negative long-term effect on collateral vessel formation because of its anti-VEGF action requires investigation.