Pituitary macroadenomas are well-recognised to cause visual field disturbances by mass effect. Central Serous Retinopathy (CSR) is a rare cause of reversible vision loss in Cushing’s Syndrome (CS).
A 54yo previously well Caucasian female was referred for investigation of possible CS after presenting to a vitreoretinal ophthalmologist with a 3 week history of sudden onset of right eye monocular partial vision loss caused by CSR, which deteriorated with over the following 3 weeks. Examination revealed an anxious well lady with no somatic features of CS. BP 182/84 supine, 170/84 standing. Baseline 23.00 salivary cortisols were borderline high (7, 9nmol/L; N <9.0), and dexamethasone suppression was undertaken. Visual fields, fundus photographs and Optical Coherence Tomograms (OCT) (to be presented) were all consistent with CSR. 24hr monitoring showed mean awake BP 167/100. Enalapril was commenced.
Pre- and post- 1mg dexamethasone suppression showed 08.00 ACTH of 48 and 43pg/ml, and cortisol 519 and 372nmol/L. Suppression tests revealed:
|
|
8am (post “low” dose 0.5mg Dex qid 48 hours) |
8am (post “high” dose 2mg Dex qid 48 hours) |
|
ACTH (pg/ml) |
43 |
14 |
|
Cortisol (nmol/L) |
289 |
68 |
Pituitary MRI demonstrated a 4mm microadenoma in the left side with no suprasellar extension or optic chiasm impingement. She underwent successful transsphenoidal adenomectomy. Intercurrent headaches post-operatively resolved with sinus washout surgery. BP 120/70 on Ramipril 10mg daily. Post-surgical MRIs demonstrated essentially a normal pituitary. She required hydrocortisone 27mg daily. Her vision improved gradually with progressive normalisation of OCT and by 1 year after presentation her vision returned to almost normal.
CSR is a localised serious detachment of the posterior retinal pole from the Retinal Pigment Epithelium (RPE), due to a focal RPE defect. CSR has been reported in 5% of patients with endogenous CS (1) and may complicate treatment with high-dose glucocorticoids (2). The pathogenesis of CSR in CS remains unclear.