Article

PDF
Access to the PDF text
Service d'aide à la décision clinique
Advertising


Free Article !

Journal Français d'Ophtalmologie
Volume 38, n° 4
pages e73-e75 (avril 2015)
Doi : 10.1016/j.jfo.2014.08.007
Lettres à l'éditeur

Bilateral central serous chorioretinopathy associated with endogenous Cushing's syndrome
Choriorétinite séreuse centrale bilatérale associée à un syndrome de Cushing endogène
 

T. Buelens a, , b, c , A. Dewachter d
a Department of Ophthalmology, CHU Saint-Pierre and Brugmann, place Arthur-Van-Gehuchten 4, 1020 Brussel, Belgium 
b AZ Sint-Jan Brugge-Oostende, campus Sint-Jan Brugge, Ruddershove 10, 8000 Brugge, Belgium 
c “Vrije Universiteit Brussel”, Laarbeeklaan 103, 1090 Brussels, Belgium 
d Department of Ophthalmology, AZ Sint-Jan Brugge-Oostende, campus Sint-Jan-Brugge, AV, Ruddershove 10, 8000 Brugge, Belgium 

Corresponding author.
Case report

A 65-year-old patient was referred to the eye clinic because of unsatisfactory visual recovery one month after cataract surgery of the left eye. The patient was known with several cardiac risk factors including arterial hypertension, hypercholesterolemia and insulin dependent diabetes mellitus complicated by chronic renal insufficiency.

Best corrected visual acuity (BCVA) at presentation was 7/10 in the left and 9/10 in the right eye, anterior segment examination showed no signs of intra-ocular inflammation, intra-ocular pressure was not raised and posterior segment evaluation showed signs of mild diabetic retinopathy in both eyes. Fluorescein angiography demonstrated retinal pigment epithelial (RPE) alterations in both eyes and multiple sites of leakage in the left eye (Figure 1). Macular OCT showed serous macular detachment and a small pigment epithelial detachment in the left eye (Figure 2); these findings were initially attributed to the topical post-operative treatment with corticosteroid drops and it was opted to await spontaneous resolution. However, because six months later BCVA in the left eye dropped to 5/10 and fluorescein angiography and macular OCT confirmed chronic active disease, it was decided to perform focal laser photocoagulation to the RPE-leaks (photodynamic therapy with verteporfin was contra-indicated because of renal failure). At this time, macular OCT also showed some distant subretinal fluid in the right eye without macular involvement. Nevertheless BCVA kept decreasing (RE 4/10, LE 3/10) with fluorescein angiography and OCT showing signs of continuous active macular disease in both eyes despite additional sessions of laser treatment.



Figure 1


Figure 1. 

Mid-phase fluorescein angiography of the left eye at initial presentation, showing several areas of hyperfluorescence.

Zoom



Figure 2


Figure 2. 

Ocular coherence tomography of the left eye at initial presentation, showing accumulation of fluid beneath the neurosensory retina resulting in a serous detachment.

Zoom

Because of this chronicity with resistance to focal treatment, and the development of a moon shaped facial appearance (Figure 3), the patient was sent to his endocrinologist to rule out any cause of endogenous hypercortisolism. First line screening tests showed failure to suppress morning cortisol secretion after an overnight low dose of dexamethasone, and a 24-hour collection of urinary free cortisol excretion showed raised values. Serum adrenocorticotropic hormone levels were normal associated with a raised morning serum cortisol level (Table 1). Additional MRI-imaging of the pituitary gland revealed a mass of 3.5 by 4mm, hyperintens on T2- and slightly hypo-intens on T1-weighted images, with late contrast hypercaptation; suggestive for a pituitary micro-adenoma. The neurosurgical team was consulted and a transphenoidal tumor resection was performed. Ophthalmological examination seven weeks later showed a visual recovery up to 8/10 in the right and 6/10 in the left eye as well as a clear regression of the CSCR.



Figure 3


Figure 3. 

Facial appearance of our patient approximately one year after initial presentation, suggesting moon facies.

Zoom

Discussion

Central serous chorioretinopathy (CSCR) is the fourth most common retinopathy [1] and, in contrast to our 65-years old patient, it is classically a disease of young otherwise healthy adults. However, it has been noted that CSCR is associated with different conditions, characterized by exposure to increased levels of endogenous or exogenous glucocorticoids.

All forms of exogenous corticosteroid administration appear to be associated with an increased risk [2, 3, 4], with no apparent lower limit on the dose required [5]. In some cases, CSCR was the presenting symptom of endogenous hypercortisolism as a result of stress conditions or Cushing's disease, such as the pituitary adenoma in our case [6, 7] but also adrenocortical adenoma [8] and carcinoma [9], and pregnancy [3]. The rate of CSCR in patients with endogenous hypercortisolism is reported to be approximately 5% [7].

Active CSCR is characterized by detachment of the neurosensory retina caused by accumulation of serous fluid between the photoreceptor outer segments and the RPE in combination with monofocal or multifocal changes in the RPE. Inactive disease, with the retina being fully attached, may be diagnostically challenging because the residual abnormalities of the RPE may resemble those of other conditions, but often a previous history characteristic of acute CSCR can be elicited [1]. It was also shown that RPE-abnormalities are present in nearly all asymptomatic, fluid-free contralateral eyes of CSCR patients [10]. In retrospect, alterations of the RPE-layer were found in both eyes of our patient at baseline examination but without reported episodes of symptomatic visual loss. These events – if any – may have been masked by and/or be attributed to the opacification of the lens.

The high spontaneous remission rate favours conservative management, lifestyle counselling and discontinuation of glucocorticoid medication as first-line therapeutic options. The pituitary micro-adenoma found in our patient could be considered as a form of steroid exposure with resistance to local treatment while present and spontaneous resolution after its (surgical) removal.

Conclusion

CSCR can be the presenting symptom of endogenous hypercortisolism. Cushing's syndrome should be considered as a possible underlying etiology in patients with chronic and/or therapy resistant cases.

Disclosure of interest

The authors declare that they have no conflict of interest concerning this article.

References

Wang M., Munch I.C., Hasler P.W., Prünte C., Larsen M. Central serous chorioretinopathy Acta Ophthalmol 2008 ;  86 (2) : 126-145 [cross-ref]
Tarabishy A.B., Ahn E., Mandell B.F., Lowder C.Y. Central serous retinopathy Arthritis Care Res 2011 ;  63 (8) : 1075-1082 [cross-ref]
Bouzas E.A., Karadimas P., Pournaras C.J. Central serous chorioretinopathy and glucocorticoids Surv Ophthalmol 2002 ;  47 (5) : 431-448 [cross-ref]
Liew G., Quin G., Gillies M., Fraser-Bell S. Central serous chorioretinopathy: a review of epidemiology and pathophysiology Clin Experiment Ophthalmol 2013 ;  41 (2) : 201-214 [cross-ref]
Song E., Wakakura M., Ishikawa S. Central serous chorioretinopathy induced by corticosteroids Nihon Ganka Gakkai Zasshi 1997 ;  101 : 257-264
Harada T., Harada K. Six cases of central serous choroidopathy induced by systemic corticosteroid therapy Doc Ophthalmol 1985 ;  60 : 37-44 [cross-ref]
Bouzas E.A., Scott M.H., Mastorakos G., Chrousos G.P., Kaiser-Kupfer M.I. Central serous chorioretinopathy in endogenous hypercortisolism Arch Ophthalmol 1993 ;  111 : 1229-1233 [cross-ref]
Iannetti L., Spinucci G., Pesci F.R., Vicinanza R., Stigliano A., Pivetti-Pezzi P. Central serous chorioretinopathy as a presenting symptom of endogenous Cushing syndrome: a case report Eur J Ophthalmol 2011 ;  21 : 661-664 [cross-ref]
Thoelen A.M., Bernasconi P.P., Schmid C., Messmer E.P. Central serous chorioretinopathy associated with a carcinoma of the adrenal cortex Retina 2000 ;  20 : 98-99 [cross-ref]
Gupta P., Gupta V., Dogra M.R., Singh R., Gupta A. Morphological changes in the retinal pigment epithelium on spectral-domain OCT in the unaffected eyes with idiopathic central serous chorioretinopathy Int Ophthalmol 2010 ;  30 (2) : 175-181



© 2015  Elsevier Masson SAS. All Rights Reserved.
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Close
Article Outline
You can move this window by clicking on the headline