Multimodal Imaging Findings Research Articles

Predominant polarity as a neurobiological specifier in bipolar disorder: Evidence from a multimodal neuroimaging study

BackgroundWhile predominant (PP) and onset polarity (OP) have considerable clinical and treatment implications in bipolar disorder (BD), the neurobiological underpinnings of PP and OP from a radiological perspective remain largely unknown. The main objective of this study is to investigate the neuroanatomical profile of polarity subphenotypes (PP and OP) in euthymic BD patients, using a standardized multimodal neuroimaging protocol to evaluate regional gray matter (GM) volumes, cortical thickness, as well as white matter (WM) integrity of major projection, commissural and association tracts. MethodsForty-two euthymic BD patients stratified for PP and OP and 42 healthy controls (HC) were included in this computational neuroimaging study to comprehensively characterize gray and white matter alterations. Univariate analyses of covariance (ANCOVAs) were conducted with Bonferroni corrections for each MRI modality and Cohen's d effect sizes were calculated for group comparisons. ResultsPhenotype-associated cortical thickness abnormalities and volumetric alterations were identified, but no WM changes ascertained. Specifically, we found a main effect of OP on GM volume of left middle frontal gyrus and of OP and PP (either or both) on cortical thickness of various regions previously implicated in BD, i.e. inferior frontal gyrus-pars opercularis (left) and pars orbitalis (bilateral), left lateral orbitofrontal gyrus, bilateral medial segment of the superior frontal gyrus, left planum polare, right anterior cingulate gyrus, left anterior and posterior insula, bilateral frontal operculum (both OP and PP); left anterior and posterior orbitofrontal gyrus, left transverse temporal gyrus, right posterior insula (only OP); and right medial frontal cortex (only PP). Based on the magnitude of differences on pairwise comparisons, we found a large effect of OP on cortical thickness in a single region (left anterior orbitofrontal gyrus) (OP-M > OP-D), while PP subgroups showed large or medium effect size differences in cortical thickness (PP-M > PP-D) in a wider array of regions (right medial frontal cortex, left frontal operculum, left inferior frontal gyrus-pars opercularis, bilateral medial segment of the superior frontal gyrus). For most regions, PP-D patients showed the greatest decreases in cortical thickness compared to HC while PP-M showed the smallest, with PP-U showing an “unspecified” pattern mostly lying in-between PP-D and PP-M. ConclusionsOur multimodal imaging findings suggest specific polarity BD subgroups with compromised cortical thickness; we recorded a greater impact of PP on brain structure compared to OP, which provides additional evidence that PP can be considered as a neurobiological specifier in BD.

Multimodal Imaging Findings of Cerebral Amyloid Angiopathy Related Inflammation With Unusual Clinical Manifestation: A Case Report

Multimodal Imaging Findings of Cerebral Amyloid Angiopathy Related Inflammation With Unusual Clinical Manifestation: A Case Report

Breast Lymphoma: A Report of 5 Cases and Literature Review

Breast lymphoma may occur as either a primary or a secondary lesion and the secondary form is more common than the primitive form. We report five cases of breast lymphoma who underwent multimodal breast imaging studies in our institute between March 2018 and June 2020, one was primary and the others were secondary. The multimodal imaging consisted of mammography, ultrasonography (US) and dynamic computed tomography (CT). The objective of this paper was to illustrate the multimodal imaging findings in the primary and secondary forms of breast lymphoma and to describe the key clinical and radiological findings that allow it to be distinguished from other breast malignancies.

Clinical features and multimodal imaging findings in primary vitreoretinal lymphoma

AbstractPurpose: To describe clinical features and multimodal imaging findings in primary vitreoretinal lymphoma (PVRL).Methods: Medical records and imaging findings of 4 patients (4 eyes) diagnosed with PVRL were retrospectively reviewed. Detailed ophthalmic examination, fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), optical coherence tomography (OCT) and OCT angiography (OCTA) were analysed in all patients. All patients underwent vitreous biopsy for diagnosis confirmation. Brain magnetic resonance imaging (MRI) was performed in all cases.Results: There were 2 women and 2 male patients with a mean age of 68.75 years. Visual blurring was the presenting symptom in all patients (100%). Clinical findings at presentation included vitritis in 4 eyes (100%), vitreous clumps in 2 eyes (50%), subretinal deposits in 3 eyes (75%) and exudative retinal detachment in one eye (25%). FAF showed granular hyperautofluorescence and hypoautofluorescence lesions in 3 eyes (75%) and blockage by mass lesion was seen in 1 eye. The most common pattern on FA was hypofluorescent lesions with a “leopard spot” appearance (75%). OCT revealed hyperreflective sub retinal lesions between retinal pigment epithelium and Bruch's membrane with an undulating shape (seasick sign) in 3 eyes (75%) and epiretinal membrane in one eye. OCTA showed areas of decreased signal on choriocapillaris slab in 2 eyes (50%). MRI of the globes and brain showed no abnormality in all cases. Vitreous cytology revealed atypical mononuclear cells consisting with the diagnosis of PVRL in all cases. The patients were referred to oncology unit.Conclusions: The diagnosis of PVRL is challenging. Multimodal imaging provides novel insights into features of PVRL.

Multimodal imaging features in chorioretinitis sclopetaria

AbstractPurpose: To describe the multimodal imaging findings in a case of chorioretinitis sclopetaria (CS).Methods: Single case report.Results: A 26‐year‐old male reported a history of ocular injury to the right eye (OD) in childhood caused by a palm tree branch after which he underwent cataract surgery. On examination, his best‐corrected visual acuity was light perception in OD. Anterior segment examination revealed the presence of an anterior chamber intraocular lens. Intraocular pressure was within the normal range. Dilated funduscopy showed an area of bare sclera in the inferonasal quadrant adjacent to a white fibroglial proliferation that extended to the nasal and temporal periphery consistent with CS. A large area of chorioretinal atrophy involving the macular region with a nasal demarcation line and scattered hyperpigmented patches was also noted. The left eye examination was unremarkable. Fundus autofluorescence showed hypoautofluorescence of the scar tissue and in the posterior pole. Swept‐source optical coherence tomography scans confirmed the rupture of the choroid underneath a full‐thickness hyperreflectivity in the inferonasal quadrant and revealed disruptions in the macular RPE and outer retinal layers.Conclusions: CS is caused by a shock wave from a high‐velocity projectile resulting in the tearing of the choroid, retinal pigment epithelium (RPE), and neurosensory retina which later induces fibrous scarring, RPE hyperplasia, and photoreceptor loss. Multimodal imaging findings of CS are consistent with these features.

Multimodal imaging in persistent fetal vasculature

AbstractPurpose: To describe the clinical manifestations of the persistence of the fetal vascular system and to show multimodal imaging findings in this disease.Methods: We presented two cases of persistent fetal vasculature. Thorough clinical examination and full multimodal imaging have been undertaken.Results: The first case is a 12‐year‐old girl who consulted for a visual impairment evolving for few years. Fundoscopy revealed a unilateral whitish vascular membrane attached to the optic disc towards the vitreous associated with retinal folds in interpapillomacular region as well as chorio‐retinal atrophy and nasal pigment migrations. B‐mode ultrasonography confirmed the presence of this membrane attached to the optic disc but does not reach the lens. Optical coherence tomography (OCT) showed a deficit of RNFL, especially in superotemporal and hyaloid condensation in the optic disc with macular folds. The second case is a 42‐year‐old patient who consulted for a presbyopia. Fundoscopy revealed membranous short band‐like lesion attached to the optic disc associated with retinal folds, better seen in the anerythral photograph which showed an alternation of grey and white bands.Conclusions: Multimodal imaging is of a paramount importance in diagnosing the fetal vascular system. It also allows the recognition of the form of this disease and thus the way to manage it.

Clinical and Multimodal Imaging Features of Choroidal Nevi in the Korean Population.

Choroidal nevus is a precursor of choroidal melanoma. Multimodal imaging has become vital in predicting the malignant transformation of choroidal nevi. This single-center, retrospective study analyzed clinical characteristics and multimodal imaging findings of 168 choroidal nevi (164 patients) of the Korean population. The mean age at presentation was 50 ± 15 (range, 13-85) (women, n = 88 [53.7%]). Choroidal nevi (melanotic, n = 164 [97.6%]; postequatorial, n = 160 [95.2%]) were densely located between the optic disc and foveola (65.5%). The mean maximum linear basal diameter on fundus photography and thickness on optical coherence tomography were 2.97 ± 1.51 mm and 521 ± 297 μm, respectively. On ultrasonography, the mean thickness was 0.87 ± 0.60 mm. Choroidal nevi in women were associated with a higher maximum linear basal diameter (3.23 ± 1.65 vs. 2.68 ± 1.21 mm; p = 0.033) and age at diagnosis (52 ± 14 vs. 47 ± 16 years; p = 0.046) than those in men. Choroidal nevi with associated subretinal fluid (23.2%) presented with larger basal diameter (p = 0.049) and thickness on B-scan and optical coherence tomography (p < 0.001), but a younger age at diagnosis (p < 0.001) than those of dry nevi. This multimodal imaging study of choroidal nevi revealed some distinct characteristics, including topographic distribution, sex-related differences, and a younger age at diagnosis of nevi with fluid.

LOCATIONAL AGREEMENT OF NEAR-INFRARED AUTOFLUORESCENCE WITH CHOROIDAL VASCULAR HYPERPERMEABILITY IN CENTRAL SEROUS CHORIORETINOPATHY.

To characterize the findings of eyes with choroidal vascular hyperpermeability (CVH), a hallmark of central serous chorioretinopathy, using multimodal imaging and investigate the locational agreement between these findings and CVH. Among patients with central serous chorioretinopathy in either eye, eyes with CVH identified using indocyanine green angiography without exudative changes were included. All eyes were examined using funduscopy, fluorescein angiography, spectral-domain optical coherence tomography, and short-wavelength or near-infrared autofluorescence (SWAF or NIRAF). The locational agreement between CVH and imaging findings was evaluated for each modality. The relative index on how they overlapped was calculated as the overlapping index. Binarized images, particularly for NIRAF, were also evaluated. This study included 69 CVH sites in 33 eyes of 28 patients. Pachydrusen was detected in 36% of CVH sites. Fluorescein angiography revealed hyperfluorescent areas in 39% of CVH sites. Optical coherence tomography findings identified 65% of CVH sites, but the overlapping index was 5%. Short-wavelength or near-infrared autofluorescence imaging identified 89% of CVH sites, but they exhibited variable autofluorescence. NIRAF imaging revealed hypoautofluorescence findings in all CVH sites. When binarized, near-infrared autofluorescent dark dots were observed in all CVH sites. Overlapping indices before and after binarization were 78% and 98%, respectively. The near-infrared autofluorescent dark dots area corresponded well with that of CVH (intraclass correlation coefficient, 0.987 [95% confidence interval, 0.952-0.995]). Hypoautofluorescent findings revealed on NIRAF imaging, especially after binarization processing, corresponded well with CVH sites. These multimodal imaging results may help investigate the anatomical or locational characteristics of CVH in patients with central serous chorioretinopathy.

Multimodal Retinal Imaging Findings in Two Cousins With VCAN-Related Vitreoretinopathy or Wagner Disease.

Wagner disease is a rare, nonsyndromic vitreoretinopathy caused by autosomal dominant variants in the versican (VCAN) gene. It is associated with abnormalities of the vitreoretinal interface that can lead to peripheral traction and retinal detachments, which also occur in other vitreoretinopathies such as X-linked retinoschisis (XLRS), familial exudative vitreoretinopathy (FEVR) and Stickler syndrome. There is variability in the clinical phenotype in Wagner disease potentially due to variants in VCAN gene variants. In this article, we report a family harboring the VCAN c.9265+1G>C variant and describe the clinical and retinal findings in two members. [Ophthalmic Surg Lasers Imaging Retina 2022;53:639-643.].

Benign Lobular Inner Nuclear Layer Proliferations of the Retina Associated with Congenital Hypertrophy of the Retinal Pigment Epithelium

To report the clinical and imaging findings of 4 patients with benign intraretinal tumors, 2 of which were associated with retinal pigment epithelium (RPE) hypertrophy. To our knowledge, this condition has not been described previously and should be distinguished from retinoblastoma and other malignant retinal neoplasms. Retrospective case series. Four patients from 3 institutions. Four patients with intraretinal tumors of the inner nuclear layer (INL) underwent a combination of ophthalmic examination, fundus photography, fluorescein angiography, OCT, OCT angiography, and whole exome sequencing. Description of multimodal imaging findings and systemic findings from 4 patients with benign intraretinal tumors and whole exome studies from 3 patients. Six eyes of 4 patients 5, 13, 32, and 27 years of age were found to have white intraretinal tumors that remained stable over the follow-up period (range, 9 months-4 years). The tumors were unilateral in 2 patients and bilateral in 2 patients. The tumors were white, centered on the posterior pole, and multifocal, with some consisting of multiple lobules with arching extensions that extended beyond the central tumor mass. OCT demonstrated these lesions to be centered within the INL at the border of the inner plexiform layer. In addition, 2 patients demonstrated congenital hypertrophy of the RPE (CHRPE) lesions. Three of 4 patients underwent whole exome sequencing of the blood that revealed no candidate variants that plausibly could account for the phenotype. We characterize a novel benign tumor of the INL that, in 2 patients, was associated with separate CHRPE lesions. We propose the term benign lobular inner nuclear layer proliferation to describe these lesions. Proprietary or commercial disclosure may be found after the references.

Multimodal imaging findings of perforated choledochal cyst with biliary lithiasis and secondary pancreatitis in a pediatric patient: A case report and literature review

Multimodal imaging findings of perforated choledochal cyst with biliary lithiasis and secondary pancreatitis in a pediatric patient: A case report and literature review

Macular atrophy following macular hole surgery assisted by Membrane Blue-Dual: A case series.

The aim of this study is to describe the clinical and multimodal imaging findings in patients with macular atrophy following macular hole surgery assisted by Membrane Blue Dual. This study is a monocenter, retrospective, observational case series that included patients who presented macular atrophy following macular hole surgery. Among the patients included in this study, four were operated for idiopathic macular hole and one for total retinal detachment associated with macular hole. In all patients the internal limiting membrane was brittle and adherent and multiple stains were required. One month postoperatively all patients showed a reduced visual acuity excepted the patient with total retinal detachment. At fundus examination all patients showed patchy atrophy with a mottled hypo- and hyperpigmented appearance in the macular region. Optical coherence tomography scans demonstrated a closed macular hole with retinal thinning, disruption of the external retinal layers and irregular retinal pigment epithelium thickening. Fundus autofluorescence showed a well-defined area of both hypo- and hyper-autofluorescence involving the macular area. Macular atrophy following Membrane Blue Dual assisted internal limiting membrane peeling represents a severe complication that vitreoretinal surgeons should be aware of and that should be taken into account in preoperative evaluation and surgical procedure planning. In order to reduce the risk of this complication, we recommend to ensure the best conditions of visibility, to reduce as much as possible the intensity and the distance of the endoillumination from the retina, and to use as little dye as possible.

A Pigmented Fundus Lesion with Pseudohypopyon and Subretinal Fluid Resembling Unilateral Retinal Pigment Epithelium Dysgenesis (URPED).

To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium (RPE). Case report. A 36-year-old female presented with photopsia in her left eye. Funduscopy revealed an 8 × 7 mm, dark brown lesion at the level of the RPE inferior to the macula. The lesion had an irregular, cauliflower-like border and a light grey subretinal pseudohypopyon. On fundus autofluorescence, the lesion was markedly hypoautofluorescent with an irregular hyperautofluorescent margin. It was generally hyopofluorescent on fluorescein angiography and moderately hypofluorescent on indocyanine green angiography. Spectral-domain optical coherence tomography revealed a fine layer of subretinal fluid over the entire lesion, thinning of the outer retinal layers with loss of photoreceptors, and an irregular RPE. Multiple drusen-like subretinal deposits were located along the lesion margin, and inferiorly, the pseudohypopyon appeared as a hyperreflective subretinal mass. During three years of follow-up, her symptoms remained unchanged and fundus photography showed minimal enlargement of the lesion. Multimodal imaging findings of a solitary pigmented RPE lesion with pseudohypopyon and subretinal fluid are shown. The lesion may represent an atypical variant of unilateral retinal pigment epithelium dysgenesis.

A Pigmented Fundus Lesion with Pseudohypopyon and Subretinal Fluid Resembling Unilateral Retinal Pigment Epithelium Dysgenesis (URPED).

To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium (RPE). Case report. A 36-year-old female presented with photopsia in her left eye. Funduscopy revealed an 8 × 7 mm, dark brown lesion at the level of the RPE inferior to the macula. The lesion had an irregular, cauliflower-like border and a light grey subretinal pseudohypopyon. On fundus autofluorescence, the lesion was markedly hypoautofluorescent with an irregular hyperautofluorescent margin. It was generally hyopofluorescent on fluorescein angiography and moderately hypofluorescent on indocyanine green angiography. Spectral-domain optical coherence tomography revealed a fine layer of subretinal fluid over the entire lesion, thinning of the outer retinal layers with loss of photoreceptors, and an irregular RPE. Multiple drusen-like subretinal deposits were located along the lesion margin, and inferiorly, the pseudohypopyon appeared as a hyperreflective subretinal mass. During three years of follow-up, her symptoms remained unchanged and fundus photography showed minimal enlargement of the lesion. Multimodal imaging findings of a solitary pigmented RPE lesion with pseudohypopyon and subretinal fluid are shown. The lesion may represent an atypical variant of unilateral retinal pigment epithelium dysgenesis.

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH SERPIGINOUS-LIKE PERIPAPILLARY CHORIORETINAL ATROPHY.

To report nine cases of multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. A retrospective observational case series of eyes with multifocal choroiditis with serpiginous-like peripapillary chorioretinal atrophy. Multimodal imaging findings were reviewed and presented. Fifteen eyes of 9 patients (6 women and 3 men), with a mean age of 48.1 years (median, 46 years; range, 23-74 years), presented with multifocal choroiditis serpiginous-like peripapillary chorioretinal atrophy. All 15 eyes presented with serpiginoid peripapillary changes and had discrete patches of atrophy or punched-out scars in the posterior pole or periphery. Eleven eyes (73.3%) had cone-shaped retinal pigment epithelium elevations on optical coherence tomography, 10 eyes (66.7%) had mild vitritis, and 4 eyes (26.7%) had peripheral curvilinear streak lesions. Three eyes (20%) had choroidal neovascularization. All patients responded well to treatment with systemic immunosuppression, local corticosteroid injections, and/or intravitreal anti-vascular endothelial growth factor injections. Multifocal choroiditis may present with peripapillary chorioretinal changes resembling a serpiginous-like choroiditis in addition to the classic findings of patches of atrophy or punched-out scars in the posterior pole or periphery, cone-shaped retinal pigment epithelium elevated on optical coherence tomography and peripheral curvilinear streak lesions.

THE TARGET SIGN: A Near Infrared Feature and Multimodal Imaging in a Pluri-Ethnic Cohort with RDH5-Related Fundus Albipunctatus.

Retinol dehydrogenase 5 (RDH5)-related fundus albipunctatus can present with phenotypic variability. Our purpose was to investigate new clinical characteristics and multimodal imaging findings in patients from different ethnic origins, carrying different mutations. Multicenter international retrospective case series of 18 patients with genetically confirmed RDH5-related fundus albipunctatus. Patients' files were reviewed for fundus images, visual acuity, macular optical coherence tomography scans, near-infrared images, fundus autofluorescence, electroretinogram, and genetic mutations. Imaging and electroretinogram findings. All eyes (n = 36, 100%) showed small circular findings seen on near-infrared images, termed as the "target sign," correlating to the yellowish dots seen clinically and to the distinct hyperreflective linear lesions on optical coherence tomography at the level between external limiting membrane and retinal pigment epithelium. Perifoveal atrophy with foveal sparing was seen in 4 eyes of 2 patients (both RDH5-c.160C>T, p.R54X mutation). Fundus autofluorescence revealed small hyperautofluorescent dots (n = 16, 44.4%). Scotopic electroretinograms were significantly reduced in all cases with an electronegative pattern, 66.7% displayed cone dysfunction. Our results show distinct imaging findings present in all patients with fundus albipunctatus independent of ethnicity or genetic mutation. Our results can facilitate the current algorithm to diagnose RDH5-related fundus albipunctatus and allow for targeted genetic testing.

Multimodal imaging findings in Purtscher-like retinopathy after retrobulbar anesthesia

Introduction: This is a case of Purtscher-like retinopathy with an unusual etiology of retrobulbar anesthesia, evidenced by optical coherence tomography-angiography (OCT-A) images. Patient and Clinical Findings: After cataract surgery with a retrobulbar block, a 65-year-old woman experienced sudden dramatic visual reduction with superficial white retinal patches on fundus examination and corresponding capillary bed infarcts on fluorescein angiography and OCT-A. Diagnosis, Intervention, and Outcomes: Using advanced OCT-A, fluorescein angiography imaging, and fundus photography, the patient was diagnosed with Purtscher-like retinopathy. The patient was treated with topical steroids, nonsteroidal anti-inflammatory drops, and antibiotic drops, with gradual and nearly complete resolution of visual acuity. Conclusions: This case represents a rare but possible complication of retrobulbar anesthesia associated with cataract surgery. Despite dramatic changes on OCT-A, and in contrast to other reports with similar characteristic findings, our patient regained significant vision.

Clinical and Morphologic Characteristics of Perivenular Fernlike Leakage on Ultrawide-field Fluorescein Angiography

To analyze the spectrum of the perivenular fernlike leakage on ultrawide-field fluorescein angiography (UWFA) and discuss its potential implications in the current understanding of the retinal venous outflow. Retrospective, observational case series. Eyes presenting with fernlike patterns of dye leakage on UWFA were included in this study. Analysis of the clinical characteristics and multimodal imaging findings using UWFA and wide-angle swept-source OCT-angiography (SS-OCTA). The disease spectrum, anatomic origin, and clinical implications of this fernlike leakage. Multimodal retinal images from 40 eyes of 29 patients with fernlike leakage on UWFA were studied. The underlying etiologies included a wide range of inflammatory disorders, including pars planitis (18 eyes) and central retinal vein occlusion (2 eyes). On UWFA, the fernlike leakage originated from the retinal capillaries and venules directly adjacent to the veins and spared the periarterial zone. This perivenular fernlike leakage involved the far periphery in all cases and progressed more diffusely and centripetally in cases with more severe intraocular inflammation. On wide-angle SS-OCTA, the impairment of deep capillary plexus (DCP) flow signals precisely colocalized with the perivenular fernlike leakages identified on UWFA. The fernlike leakage on UWFA refers to the distinctive perivenular dye leakage that originates from the retinal capillaries and venules. Multimodal imaging correlation suggests that the predominant impairment is at the level of the DCP. The axial symmetry of the fernlike leakage with the veins and sparing of the periarterial zone may support the dominant venous role of the DCP.

ACQUIRED VITELLIFORM LESIONS: A Novel Finding in Eyes with Peripapillary Pachychoroid Syndrome.

To describe a novel finding of acquired vitelliform lesions in eyes with peripapillary pachychoroid syndrome. A retrospective, multicenter, observational case series including seven patients with peripapillary pachychoroid syndrome and concomitant acquired vitelliform lesions. Medical records and multimodal imaging findings were comprehensively reviewed. Six of seven patients were men, with a mean age of 72.6 years. Mean baseline best-corrected visual acuity was 0.11 ± 0.07 (Snellen equivalent 20/25), and all patients presented with baseline best-corrected visual acuity of 20/30 or better. Mean follow-up time was 24.4 months (range 1-54 months). At last follow-up, best-corrected visual acuity was stable in 13/14 eyes, and deteriorated in one eye because of subfoveal atrophy.Fundus examination revealed peripapillary retinal pigment epithelium alterations and pigment migration in all eyes. Fundus autofluorescence showed mottled hyperautofluorescence and hypoautofluorescence in the peripapillary region, and hypoautofluorescent lesions corresponding to the pigmentary changes. Optical coherence tomography showed nasal choroidal thickening and pachyvessels with overlying retinal pigment epithelium irregularity. All eyes presented with peripapillary intraretinal fluid. Subretinal fluid was present in five eyes of three patients. Acquired vitelliform lesions were present in 11 eyes. The novel association between peripapillary pachychoroid syndrome and acquired vitelliform lesions is characterized by the classic findings of both entities, and visual prognosis is accordingly good.

Henle Fiber Layer Hemorrhage in Macular Telangiectasia Type 2: Is Right Eye Dominance Coincidence or Consequence?

To summarize all reported cases of Henle fiber layer (HFL) hemorrhage in the absence of subretinal neovascularization (SRNV) in patients with macular telangiectasia type 2 (MacTel2) and to propose a mechanism for the right-sided predominance of this unique presentation. Perspective. Collection, review, and analysis of all cases in the literature and in the authors' databases of HFL hemorrhage in MacTel2, including analysis of baseline and follow-up multimodal retinal imaging findings of selected cases. Elucidation of the complex interplay of systemic venous pressure with the deep retinal capillary plexus and hypothesis regarding the right-sided predilection of HFL hemorrhage complicating MacTel2. Ten patients presented with a unilateral, characteristic radial macular hemorrhage within the HFL that affected only the right eye in all cases. Absence of SRNV was confirmed by fluorescein angiography and/or optical coherence tomography angiography. The hemorrhage resolved spontaneously in at least 7 of the 10 eyes. The HFL hemorrhage may plausibly be explained by dysfunction of the deep capillary plexus in MacTel2 combined with an acute rise in central venous pressure, for which the right side may be at increased risk. HFL hemorrhage can complicate MacTel2 in the absence of SRNV, and the radial pattern of blood affecting only the right eye is remarkable. The right eye predominance may be multifactorial in etiology. Related factors may include the right-sided predilection of MacTel2 and/or increased right-sided dural sinus drainage related to normal anatomical variation.