Central serous chorioretinopathy (CSC) is believed to be rare in patients who are 25 years or younger. We have observed three cases with features of advanced chronic CSC in young adults retrieved by retrospective review of 385 patients seen at the Glostrup Hospital in Copenhagen and the Macula Vitreous Retina Consultants of New York, between 2007 and 2010.
A 21-year-old woman was referred from her general ophthalmologist with a decline in visual acuity in her left eye and progression in retinal pigment epitheliopathy, for which she had been followed by the referring ophthalmologist, since the age of six. She had used intranasal glucocorticosteroids (Flonase) intermittently because of sinusitis-related problems.
At referral, her best corrected visual acuity (BCVA) was 1.0 in her right eye and 0.5 in her left eye. Examination showed unilateral retinal pigment epitheliopathy with a yellowish discoloration, a small pigment epithelium detachment (PED) and an exudative retinal detachment in the left eye (Fig. 1A). One month after referral, it was decided to treat with full dose of verteporfin and reduced fluence. Complete regression was observed after 2 months and BCVA recovered to 0.7.
A 17-year-old woman was referred with blurred vision in her right eye, slowly progressing over the past 2 years. Best-corrected visual acuity was 0.4 in her right eye and 1.25 in the left eye. Eye examination revealed unilateral retinal pigment epitheliopathy and serous retinal detachment with subretinal debris in her right eye (Fig. 1B and E). Fluorescein and indocyanine green angiography demonstrated two distinct hot spots (Fig. 1C and D). After 1 year of observation, the patient underwent standard treatment with photodynamic therapy (PDT). Complete regression of the serous detachment was observed 5 months after PDT treatment. BCVA increased to 0.7, but the visual symptoms were unchanged.
A 24-year-old male patient was referred with blurred central vision of abrupt onset in his right eye. BCVA was 0.2 in his right eye and 1.25 in the left eye. Eye examination demonstrated extensive retinal pigment epitheliopathy in the macular region in both eyes. In the right eye, a serous detachment of the neurosensory retina and intraretinal cystoid oedemas was seen on optical coherence tomography (OCT) (Fig. 1F). Inferotemporal of the fovea extensive deposits of subretinal fibrin was found that corresponded exactly to the area of angiographic fluorescein leakage. Endogenous levels of catecholamines and cortisol were normal.
The patient was followed without any treatment, and the serous detachment in his right eye regressed spontaneously over 6 months and BCVA increased to 0.5 (Fig. 1G).
A review of the literature indicates that patients with CSC present initially with a neurosensory detachment from a focal leak at the level of the retinal pigment epithelium (RPE) diagnosed generally between the age of 30 and 50 years (Yannuzzi 1986; Wang et al. 2008). Rarely, the presentation of exudative retinal detachments has been reported in younger patients in association with leukaemia (Stewart et al. 1989). These three young patients had all of the typical clinical characteristics of CSC, and to our knowledge, there is no previous report of CSC in patients younger than 25 years, even in large series of consecutive patients (Loo et al. 2002).
Sometimes, CSC can be associated with psychosocial stress, the use of corticosteroids and recreational drugs (Yannuzzi 1986; Gass & Little 1995; Hassan et al. 2001). Only one patient reported intermittent use of glucocorticosteroids, and none reported episodes with psychosocial stress or recreational drugs.
Our observation suggests that the clinical spectrum of CSC is wider than previously described. CSC as seen in our patients is rare, but possibly underreported, because chronic CSC may be unexpected in patients who do not have a history of prior acute CSC, especially if they are younger than 25 years.
The Danish Eye Research Foundation, Danish Eye Health Society, the Velux Foundation and the Macula Foundation Inc.