title: Idiopathic Intracranial Hypertension After Surgical Treatment of Cushing Disease: Case Report and Review of Management Strategies.
abstract: BACKGROUND
  Idiopathic intracranial hypertension (IIH) in patients with Cushing disease (CD), after treatment, is rarely described, in adults. The cause is believed to be multifactorial, potentially related to a relative decrease in cortisol after surgical resection or medical treatment of a corticotroph pituitary adenoma. We investigate 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 transsphenoidal resection.
  
  
  METHODS
  We present the case of a 22-year-old woman 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 replacement, tapered down to physiologic doses at the time the IIH symptoms developed.
  
  
  CONCLUSIONS
  Symptomatic IIH is 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 examination 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 or AI; however, further studies are needed. Treatment consists of reinitiation 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.
authors.affiliation: Department of Neurological Surgery and Northwest Pituitary Center, Oregon Health & Science University, Portland, Oregon, USA.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Wagner
authors.affiliation: Department of Neurological Surgery and Northwest Pituitary Center, Oregon Health & Science University, Portland, Oregon, USA.
authors.forename: Cara M
authors.initials: CM
authors.lastname: Fleseriu
authors.affiliation: Department of Neurological Surgery and Northwest Pituitary Center, Oregon Health & Science University, Portland, Oregon, USA.
authors.forename: Aly
authors.initials: A
authors.lastname: Ibrahim
authors.affiliation: Department of Neurological Surgery and Northwest Pituitary Center, Oregon Health & Science University, Portland, Oregon, USA. Electronic address: cetasj@ohsu.edu.
authors.forename: Justin S
authors.initials: JS
authors.lastname: Cetas
mesh_terms: D000086:Acetazolamide / Q000627:therapeutic use; D000237:Adenoma, Basophil / Q000601:surgery*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D019586:Intracranial Hypertension / Q000209:etiology* / Q000628:therapy*; D047748:Pituitary ACTH Hypersecretion / Q000601:surgery*; D010911:Pituitary Neoplasms / Q000601:surgery*; D011183:Postoperative Complications / Q000209:etiology* / Q000628:therapy*; D017287:Ventriculoperitoneal Shunt; D055815:Young Adult
chemical_list: D000086:Acetazolamide; D006854:Hydrocortisone
keywords: Cushing disease; Idiopathic intracranial hypertension; Pseudotumor cerebri; Transsphenoidal resection