Congenital retinal macrovessel in a patient with rhegmatogenous retinal detachment.

A 48-year-old female presented with old inferior rhegmatogenous retinal detachment (RRD) with demarcation line encroaching the macula [Fig. 1a]. Best corrected visual acuity was 20/30. Lattice degeneration with atrophic holes was noted at 6’o clock meridian. A branch of the infero-temporal retinal vein coursed upwards through the fovea and crossed the horizontal raphe. Fundus fluorescein angiogram [FFA, Fig. 1b] confirmed this vessel to be of venous origin and showed absence of foveal avascular zone. Old RRD leading to retinal pigment epithelium (RPE) atrophy showed as hyper fluorescent area while blocked fluorescence was seen in relation to the demarcation line because of RPE hypertrophy. Vertical spectral domain optical coherence tomography scan [Fig. 1c] through macula showed inferior subretinal fluid. The vessel passing through the macula was seen in inner retinal layers with shadowing underneath [blue arrow, Fig. 1c]. The patient was diagnosed with congenital retinal macrovessel (CRM) and inferior RRD. The patient underwent uneventful scleral buckling surgery. Visually acuity was maintained at 20/30 and retina was attached at 6-month follow-up.

Figure 1

Colour fundus photograph (a) showing inferior rhegmatogenous retinal detachment with demarcation line. A branch of the infero-temporal vein passes through the fovea. Fluorescein angiogram (b) shows demarcation line and confirms venous origin of congenital retinal macrovessel. Vertical spectral domain optical coherence tomography scan (c) shows congenital retinal macrovessel (blue arrow) and inferior subretinal fluid

CRM is rare congenital anomaly and consists of aberrant branch of a retinal vessel crossing the macula and horizontal raphe.[1] Commonly, CRM is a vein, but it may arise from an artery as well and is uusually asymptomatic.[1] Visual loss in patients with CRM has been attributed to serous detachment,[2] macro aneurysm,[3] branch retinal artery occlusion,[4] macular haemorrhage, vitreous haemorrhage,[5] foveal cyst, or mere presence of blood vessel.[1] FFA in CRM usually reveals early filling and delayed emptying and may be associated with dilated capillary bed surrounding the vessels.[6] CRM has never been reported in patient with RRD. The association of the two appears to be coincidental though.

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