Phaeochromocytoma revealed by a stellate neuroretinitis

Abstract

A 28-year-old woman presented with bilateral progressive decrease in visual acuity for 1 month. She had a history of headache, palpitations, profuse sweating, and abdominal pain for 3 months. Visual acuity was 20/800 in both eyes. On funduscopy (fi gure A) we saw a prominent bilateral optic disc swelling (black arrowheads) with marked macular hard exudates in a star shape (white arrowheads). Several hyperpigmented lesions (Siegrist streaks; white arrows) were scattered in the temporal midperipheral retina, suggesting marked ischaemia over choroidal arteries. We also noted a mild and diff use arterial narrowing with some intraretinal haem orrhages. This presentation was consistent with a stellate neuroretinitis, which is defi ned by optic disc swelling and macular hard exudates in a star shape. Fluorescein angiography (fi gure B) showed, a bilateral papillary hyperfl uorescence (black arrowheads) with some areas of delayed choroidal fi lling (white arrows) and several well-defi ned hypofl uorescent lesions (Elschnig fl ecks; white arrowheads) suggestive of a hypertensive choroidopathy. Optical coherence tomography (fi gure C) revealed a bilateral mild macular oedema (white arrows) with many perimacular exudates (white arrowheads). Blood count, erythrocyte sedimentation rate, serum lysozyme concentration, serum angiotensin-converting enzyme concentration, and fasting glucose concentration showed no abnormal results. Serology for Bartonella, syphilis, Lyme disease, and leptospirosis, quantiferon gold for tuberculosis, and cerebral CT were all normal. The patient’s blood pressure was 240/130 mm Hg. Abdominal ultra sonography showed a right adrenal mass that was hyperechoic and highly vascularised, measuring 50 mm by 52 mm and displacing the kidney (fi gure D). An abdominopelvic CT scan showed a hypodense right adrenal lesion with areas of necrosis, no intratumoural calcifi cations, and measuring 60 mm in diameter. Urinary catecholamine concentrations were high (metanephrine 107·1 μmol/24 h; normetanephrine 17·22 μmol/24 h). Neck ultrasonography, calcitonin concentration, parathyroid hormone concentration, blood and urinary calcium concentration and phosphorus concentration were normal, discounting medullary thyroid carcinoma and primary hyperparathyroidism. We did a right laparoscopic adrenalectomy; pathological examination confi rmed Phaeochromocytoma. At 3-month follow-up, blood pressure was normal (100/80 mm Hg) and visual acuity was 20/63 (right eye) and 20/200 (left eye). In our patient, the ocular symptoms were related to the high blood pressure, with a subsequent breakdown of the blood–retinal barrier leading to stellate neuroretinitis (which is a grade 4 hypertensive retinopathy). Presentation with bilateral stellate neuroretinitis should lead to measurement of blood pressure. Additional presence of Menard’s triad (headache, palpitations, and sweating) should be recognised as suggestive of phaeochromocytoma.