SUN-392 Severe Idiopathic Intracranial Hypertension with Papilledema after Surgical Treatment of Cushing's Due to an Adrenal Adenoma

Abstract

Background: It has been rarely reported that patients may develop idiopathic intracranial hypertension (IIH) after treatment of Cushing’s disease. We present a first known case of a patient with IIH after treatment of Cushing’s due to an adrenal adenoma. Clinical case: A 29-year-old female presented with 50 lb weight gain over the prior 2 years. She also had facial acne and hirsutism. Menses were regular. Her only medicine was norelgestromin-ethinyl estradiol 50-35 mcg/24hr patch for contraception. On exam she was normotensive with a BMI of 36kg/m2, generalized obesity, facial acne scars, and dark abdominal striae. Pelvic ultrasound revealed polycystic ovaries and small myomas. Extensive blood testing was normal including DHEAS, IGF-1, 17-hydroxyprogesterone, prolactin, TSH, testosterone and HgbA1C. However, her 24-hour urinary free cortisol (UFC) and two late night salivary cortisols were elevated with a suppressed ACTH (UFC 85.9 with normal range 4-50 mcg/24hr, salivary cortisols 0.24 and 0.18 with normal range 0.01-0.09 mcg/dl). Low dose dexamethasone suppression test revealed non suppressibility of cortisol (cortisol 16 mcg/dl). CT abdomen demonstrated a 3.6 x 3.2 x 3.0 cm left adrenal mass with benign characteristics. The patient underwent left adrenalectomy. Pathology confirmed adrenal cortical adenoma. At her one month post-op visit the patient was tearful and fatigued. Her cortisol level was 1.9mcg/dl after holding hydrocortisone for 24 hours. An additional 5 mg hydrocortisone was added midday to her discharge dose of 10 mg in the AM and 5 mg in the PM. She felt well immediately after the dose increase. Three months post-op her weight had decreased by 10 lbs. The hydrocortisone dose was decreased by 2.5 mg. Several days later she noticed daily episodes of transient vision loss with postural changes. She waited 1 month before seeking medical attention and then saw an ophthalmologist who diagnosed papilledema, confirmed by MRI and MRV of the brain, which were otherwise normal. A neurologist prescribed Diamox 500mg tid for presumed intracranial hypertension and referred her for lumbar puncture. The hydrocortisone was increased back to 10mg/5mg/5mg daily. She stopped the Diamox due to unremitting symptoms and inability to tolerate the medication. Three weeks later she presented to ED with progressive visual loss. Lumbar puncture had an increased opening pressure of 32 mm of H2O with normal CSF analysis. She was restarted on Diamox at a lower dose and improved 1 month later. Conclusion: There have been less than a dozen cases of IHH reported in patients treated for Cushing’s disease, 2 after withdrawal from inhaled glucocorticoids, and 2 with adrenal insufficiency. The cause is believed to be multifactorial, likely related to relative decrease in cortisol after surgical resection. This case is the first report of a patient with IIH after treatment for Cushing’s due to an adrenal adenoma.