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We report a case of polypoidal choroidal vasculopathy (PCV) presented with massive submacular hemorrhage (SMH) and macular hole (MH). Spectral domain optical coherence tomography confirmed the presence of MH along with SMH and also revealed an extrafoveal-notched pigment epithelium detachment (PED) suggestive of PCV. Urgent pneumatic displacement with perfluoropropane (C3F8) was done. Indocyanine green angiography done 3 weeks later showed polyps in the superonasal macula corresponding to PED. Extrafoveal polyps were treated with laser photocoagulation. Inverted flap MH surgery was planned but the patient was lost to follow-up.
Polypoidal choroidal vasculopathy (PCV) commonly presents as multiple recurrent serosanguinous detachment of the neurosensory retina and pigment epithelium detachment (PED). Spontaneous massive submacular hemorrhage (SMH) from rupture of thin-walled choroidal vessels is not an infrequent presentation of PCV requiring prompt pneumatic displacement. We report a rare case of macular hole (MH) secondary to PCV-related SMH.
A 60-year-old female presented with sudden loss of vision in oculus dexter (OD) for 4 days. Her best-corrected visual acuity was 20/200 OD and 20/20 in oculus sinister (OS). Intraocular pressure was within normal limits in oculus uterque (OU). Anterior segment OU was unremarkable. Fundus OD revealed massive SMH with an overlying MH. Fundus OS was normal. Spectral domain optical coherence tomography (Cirrus HD-OCT, Carl Zeiss Meditec, Dublin, CA, USA) OD showed SMH and FTMH at fovea and a notched PED with subretinal fluid (SRF) at superonasal macula suggestive of PCV [Figure 1]. A provisional diagnosis of PCV with SMH and FTMH was made, and an emergency pneumatic displacement with intravitreal perfluoropropane gas (C3F8 0.3 ml, 100%) was performed. At 3-week follow-up, SMH was completely resolved and a reddish orange polyp-like lesion was noted clinically in the superonasal macula along with MH. Indocyanine green angiography (TRC-50EX mydriatic camera, Topcon Medical Systems, Tokyo, Japan) confirmed the presence of extrafoveal polyps with branching vascular network (BVN) [Figure 2]. Focal laser photocoagulation was applied to polyps. Inverted flap MH surgery was planned but the patient was lost to follow-up.
Spontaneous massive SMH is a known complication of PCV requiring prompt pneumatic displacement. PCV has been rarely reported to cause massive suprachoroidal hemorrhage and secondary angle closure glaucoma. We report yet another rare presentation of MH secondary to PCV. We hypothesize that the explosive nature of SMH can rupture the relatively thinner fovea resulting in MH. The presence of blood at the base of the MH, bridge of remnant retinal tissue, and breakthrough vitreous hemorrhage are indirect evidence for that. MH has been reported to be associated with SMH following trauma but the cause of MH is probably trauma rather than SMH. Sagara et al. suggested the possibility of MH following subretinal hemorrhage in ruptured retinal arterial macroaneurysm (RAM). MH developing 5 months after SMH has been reported in a 75–year-old female with age-related macular degeneration. Cho et al. reported MH following intravitreal ranibizumab injection in PCV. Bakri et al. noted MH intraoperatively during subretinal injection of tissue plasminogen activator. To the best of our knowledge, spontaneous MH with SMH as a presenting feature of PCV has not been reported in literature. Such a complication is likely to be associated with poor visual outcome.
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There are no conflicts of interest.