PURPOSE
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.
DESIGN
Perspective.
METHODS
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.
RESULTS
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.
CONCLUSIONS
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.
Macular telangiectasia type 2 (MacTel2) is a slowly progressive neurodegenerative disease caused by Müller cell dysfunction with secondary retinal vascular alterations. , Recent studies have shown that mutations in genes involved in serine and lipid metabolism may play a pathogenic role in MacTel2, especially in the hereditary forms associated with neuropathy. The disease is characterized by the loss of macular luteal pigment and attenuation of the ellipsoid zone that can progress to cavitation of the retina, and even full-thickness macular hole.
Secondary telangiectasia within the deep retinal capillary plexus (DCP) is also a typical finding and is best identified with optical coherence tomography (OCT) angiography. Progression from the nonproliferative to the proliferative stage can lead to more sudden vision loss due to macular hemorrhage and edema associated with subretinal neovascularization (SRNV).
Macular hemorrhage can also complicate MacTel2 in the absence of SRNV. Patients typically present with decreased vision or a central scotoma and a characteristic radial or petaloid pattern of paracentral blood that appears as hyperreflectivity in the Henle fiber layer (HFL) on cross-sectional OCT. HFL hemorrhage can complicate a wide spectrum of diseases, although local and systemic venous disorders are a common underlying association.
Although previous studies have described HFL hemorrhages in MacTel2, this report includes an aggregate analysis of 10 cases (the largest prior report was 3 cases) and proposes more plausible explanations for the very unique right-sided predilection of disease. Specifically, we summarize all known cases of HFL hemorrhages secondary to MacTel2 in the absence of SRNV and describe a systemic pathway that may explain the right-sided dominance of this complication and the right-sided predilection of this disease.
ILLUSTRATIVE CASES
Demographic data for the 10 cases are summarized in Table 1 . All 10 cases were notable for the sudden onset of unilateral HFL hemorrhage in the right eye secondary to MacTel2. We could identify no cases in the literature or in our databases of HFL hemorrhage secondary to MacTel2 that affected the left eye as confirmed with retinal examination and/or multimodal retinal imaging. However, all cases showed characteristic multimodal imaging findings of MacTel2 in the fellow left eye, including foveal thinning and cavitation with OCT and temporal hyperautofluorescence with fundus autofluorescence (FAF).
| Case | Age | Sex | Eye | Valsalva? | Blood Thinners? | Medical History | Symptoms | Follow-up Interval | Baseline Vision | Final Vision | Treatment |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 60 | F | OD | Emesis | None | None | Central scotoma | 6 mo | 20/400 | 20/70 | Bevacizumab × 6 |
| 2 | 67 | F | OD | None | None | DM, anemia, HTN | Decreased vision | 4 wk | 20/70 | 20/30 | None |
| 3 | 58 | M | OD | None | Aspirin, Clopidogrel | DM, HTN, CAD s/p PCI | Central scotoma | 6 mo | 20/250 | 20/200 | PPV+TPa |
| 4 | 43 | M | OD | None | Aspirin | HTN, HLD, prior smoker | Scotoma | 7 mo | CF | 20/50 | Bevacizumab × 5 |
| 5 | 70 | M | OD | None | Aspirin, Rivaroxaban | OSA, DM, HTN, Pulmonary Embolism | Decreased vision | 6 wk | 20/200 | 20/40 | None |
| 6 | 50 | M | OD | None | None | Obesity, DM, HLD | Decreased vision | 1.5 yr | 20/70 | NA | None |
| 7 | 60 | M | OD | None | None | s/p CABG | Decreased vision | 5 mo | 20/400 | 20/60 | None |
| 8 | NA | NA | OD | NA | NA | NA | NA | 10 mo | NA | NA | None |
| 9 | 42 | M | OD | None | None | None | Central scotoma | 2 mo | 20/100 | 20/20 | None |
| 10 | 75 | F | OD | Bowel movement | None | NA | Decreased vision | 1 yr | 20/50 | 20/20 | None |
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