Idiopathic intracranial hypertension following surgical treatment of Cushing disease: case report and review of management strategies

Abstract Background Idiopathic intracranial hypertension (IIH) in Cushing’s disease (CD) patients, following treatment, is rarely described, in adults. Etiology is thought to be multifactorial, potentially related to a relative decrease in cortisol following surgical resection or medical treatment of a corticotroph pituitary adenoma. We investigated our center’s CD database (140 surgically and 60 medically (primary or adjunct) treated patients) for cases of IIH, describe our center’s experience with symptomatic IIH, and review treatment strategies in adults with CD after transphenoidal resection. Case Description We present the case of a 22-year-old female who presented with worsening headache, nausea, vomiting, blurry vision, diplopia, visual loss and facial numbness 14 weeks after surgical resection of adrenocorticotropic hormone-positive pituitary adenoma. Her CD had been in remission since surgery with subsequent adrenal insufficiency (AI), which was initially treated with supraphysiologic glucocorticoid (GC) replacement, tapered down to physiologic doses at the time the IIH symptoms developed. Conclusion Symptomatic IIH is very rare in adult patients, but can be severe and result in permanent vision loss. A high index of suspicion should be maintained and a fundus exam is necessary to exclude papilledema, whenever there are suggestive symptoms that initially may overlap with AI. It is possible that some cases of mild IIH are misdiagnosed as GC withdrawal and/or AI, however further studies are needed. Treatment consists of re-initiation of higher steroid doses together with acetazolamide with or without cerebrospinal fluid diversion and the priority is to preserve vision and reverse any visual loss.