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Editor,—Central serous chorioretinopathy (CSC) is a common idiopathic condition which predominantly affects middle aged men. We report an atypical case in a women which was unusual on two counts—frequent cyclical episodes governed by the menstrual cycle and the presence of significant cystoid macular oedema. To our knowledge, neither phenomenon has been previously described.
CASE REPORT
A 45 year old premenopausal white female presented with a 1 year history of intermittent blurring of central vision in her right eye. The left eye was blind following a penetrating eye injury as a child. Apart from low grade bronchiectasis with infrequent exacerbations she was otherwise well and took no regular medication. Initial clinical examination revealed a small area of neurosensory retinal detachment inferonasal to the fovea and cystoid macular oedema. There was no evidence of intraocular inflammation and the vitreous was clear. Fundus fluorescein angiography (FFA) taken during a symptomatic period showed three parafoveal areas of intraretinal oedema with small cystic spaces surrounding larger “petaloid” spaces typical of cystoid macular oedema (Fig 1A).
Late fluorescein angiograms of the right macula taken at 13 minutes. During a period of poor subjective vision and before hormone therapy there is significant cystoid macular oedema (CMO) with a typical petaloid pattern surrounded by three areas of outer retinal oedema (A). After 4 months of continuous hormone therapy with Mercilon there is no evidence of CMO and one area of retinal oedema has resolved (B).
The patient had kept a menstrual diary over a year confirming a completely regular menstrual cycle and there was a clear association between poor subjective vision and the first half of each cycle. Vision deteriorated each month with the onset of menstruation and began to improve again around the time of ovulation
Over the subsequent 12 months visual blurring fluctuated with Snellen visual acuity varying between 6/6 and 6/12 when measured at clinic visits.
Retinal examination at times of poor vision revealed a small foveal yellow spot and a small area of parafoveal neurosensory retinal elevation inferior to this. On examination during a period of normal vision during the latter half of the menstrual cycle, these changes had usually resolved leaving only slight perifoveal retinal pigment epithelium pigment disturbance and FFA showed persistence of the three more peripheral areas of retinal oedema but absence of the central petaloid changes. Indocyanine green angiography was normal with no evidence of choroidal vascular anomaly or leakage.
A typical month is illustrated in Figure 2. The subjective quality of vision is superimposed on a graph of levels of the principal female sex hormones oestradiol and progesterone, showing poor vision to be associated with very low progesterone levels during the early follicular half of the cycle. Her vision deteriorated from “good” to “bad” quite rapidly over a 1–2 day period associated with rapidly declining progesterone levels.
Subjective quality of vision recorded by the patient during a typical menstrual cycle superimposed on a graph of levels of the principal female hormones oestradiol (broken line) and progesterone (solid line). Poor vision is associated with very low progesterone levels during the first half of the cycle.
She was treated with the oral contraceptive pill Microgynon (levonorgestrel 150 μg, ethinyloestradiol 30 μg) on a continuous basis. Within 3 days of commencing treatment she experienced an improvement in vision to its best level which was maintained for 49 days followed by return of symptoms associated with a recurrence of clinical and angiographic cystoid macular oedema. Treatment was changed to continuous Mercilon (desogestrol 150 μg, ethinyloestradiol 20 μg), a pill containing an alternative less androgenic progestogen and lower dosage of oestrogen, with further recovery which has been maintained for 4 months. At review the macula was clinically dry and repeat FFA confirmed the absence of central oedema and resolution of one of the peripheral areas of oedema (Fig 1B).
COMMENT
The unusual clinical picture in this patient with cyclical visual blurring in her only good eye is suggestive of central serous chorioretinopathy (CSC) with episodes of localised serous retinal detachment. Clinical and angiographic cystoid macular oedema was a prominent feature not usually described in textbook accounts of the condition. An optical coherence tomography study of CSC suggests, however, that subclinical intraretinal oedema is usually present in detached retina, with retinal thickness returning to normal when serous fluid is resorbed.1 Intraretinal cystic spaces were identified in one patient.
Pathological studies of the condition are limited but microcystic retinal degeneration in the Henlé fibre layer has been observed in eyes with a history of CSC.2 One could therefore postulate that while usually subclinical, retinal oedema with cyst formation became clinically manifest in our patient owing to frequent monthly recurrent episodes of CSC. The angiographic appearance suggested intraretinal fluid to be in the outer retinal layers and probably derived from the choroidal rather than the retinal vasculature—hyperfluorescence appeared in early frames of the angiogram.
Central serous chorioretinopathy appears to have a multifactorial aetiology and personality and hormonal influences may be important.3 CSC has been documented following systemic corticosteroid therapy4 and during pregnancy.5 Gass also described a single case of postpartum “idiopathic” cystoid macular oedema developing 3 days after a full term normal delivery with recovery after 2 months.6 This is relevant to our case, suggesting that rapidly declining hormone levels may rarely have adverse effects on retinal capillary, choriocapillaris, or RPE function. That hormonal fluctuations may influence inflammatory processes in the eye is supported by a report of cyclical premenstrual attacks of severe anterior uveitis responding to suppression of the pituitary-ovarian axis with danazol.7
Little is known about the precise influence of female sex hormones on ocular function and cyclical changes in various ocular and visual variables probably have little clinical significance, perhaps with the exception of fluctuation in contact lens tolerance.8 They appear to have some influence on choroidal circulation in women with significantly lower pulse amplitude and pulsatile ocular blood flow in post-menopausal women relative to age matched men and pre-menopausal women.9 At a molecular level, recent experimental work has identified sex steroid hormone receptor mRNAs in various ocular tissues, including retina, choroid, and retinal pigment epithelium in rats, rabbits, and humans suggesting these represent target tissues for androgens, oestrogens, and progestins.10 From a therapeutic perspective in this case the relative dosage of oestrogen and progestogen may be important as the pill with lower oestrogen but equivalent progestogen dose had a more prolonged and maintained therapeutic effect.
In conclusion, we feel the clinical picture in this patient represents atypical cyclical CSC associated with cystoid macular oedema with monthly attacks precipitated by progesterone deficiency during the early follicular phase of the menstrual cycle. Continuous oral contraceptive treatment has produced significant symptomatic relief. Further study of any relation between attacks of CSC in female patients and the menstrual cycle would be of interest.