An unusual pattern of idiopathic choroidal folds.

Choroidal folds are parallel grooves or striae in the fundus, which may be idiopathic or associated with a variety of conditions. They are seen ophthalmoscopically and on angiography as alternating light and dark streaks oriented horizontally at the posterior pole. Less commonly vertical, oblique, or irregular alignment may be seen.[1] We describe an unusual pattern of bilateral idiopathic choroidal folds present circumferentially around the posterior pole.

A 50-year-old nonsmoker female presented with the complaint of blurring in her left eye since 3 weeks. She had no significant ocular or medical history.

Her corrected visual acuity was 20/20 in the right eye and 20/30 in the left eye. She had normal anterior segments and pupillary reactions with intraocular pressure of 14 mm Hg in both the eyes. There was no proptosis or restricted ocular movements.

Dilated fundus examination revealed pigmentary changes inferotemporal to the macula in the right eye [Figure 1a] and neurosensory detachment at the macula in the left eye [Figure 1b]. In addition, choroidal folds were noted, present circumferentially around the arcades, in both eyes [Figures 1a, b]. The optic discs were unremarkable. Fluorescein angiography showed a window defect corresponding to the area of pigmentary disturbance in the right eye [Figure 2a] and an inkblot type of leak subfoveally in the left eye [Figure 2b]. The folds were seen prominently owing to their classic alternating hypo and hyperfluorescence [Figures 2a–c].

Figure 1

(a) Fundus photograph of the right eye showing subtle choroidal folds temporal to the macula, extending circumferentially from the superior to the inferior vascular arcade and pigmentary changes inferotemporal to the macula (b) Fundus photograph of the right eye showing subtle choroidal folds temporal to the macula, extending circumferentially from the superior to the inferior vascular arcade and neurosensory detachment at the macula

Figure 2

(a) Fluorescein angiography of the right eye showing a window defect corresponding to the area of pigmentary disturbance. The choroidal folds are seen prominently owing to their classic alternating hypo and hyperfluorescence in this unusual configuration (b) Fluorescein angiography of the left eye showing an inkblot type of leak subfoveally. The choroidal folds are seen prominently owing to their classic alternating hypo and hyperfluorescence in this unusual configuration (c) Fluorescein angiography of the left eye showing choroidal folds along the superotemporal arcade

Optical coherence tomography revealed a shallow neurosensory detachment in the left eye. B-scan ultrasonography was normal in both eyes, notable for the absence of any mass or flattening of the posterior pole. Axial length measurement by A-scan ultrasonography was 22.56 mm in the right and 22.48 mm in the left eye. Computed tomography (CT) of the orbits showed no evidence of an intraorbital mass or posterior flattening of the globes.

The patient declined a lumbar puncture. She was asked to follow up regularly.

Known causes of choroidal folds include retrobulbar masses, choroidal tumors, scleral diseases, hypotony, papilledema, choroidal neovascularization, or scleral buckle. Though the exact pathogenesis is unknown, it is believed that conditions causing thickening or shortening of the sclera produce wrinkling of Bruch's membrane and the adjacent layers of the choroid and retinal pigment epithelium (RPE).[1]

When no obvious cause is found, the choroidal folds are considered idiopathic. Increased intracranial pressure can present with only choroidal folds; therefore, these patients should have an appropriate work-up that should probably include a lumbar puncture before being labeled as idiopathic.[2]

Though various patterns and orientations of choroidal folds are described, the circumferential arrangement in the absence of any identifiable cause has not been described. The association of central serous chorioretinopathy (CSR) with idiopathic choroidal folds is known.[3]

To conclude, the diagnosis of idiopathic choroidal folds is one of exclusion. The mechanism is poorly understood, however, the visual prognosis is good unless the macular area is involved. Management is to observe visual changes and monitor for the development of choroidal neovascularization.