Erin N. Kiehna, Meg Keil, Maya Lodish, Constantine Stratakis and Edward H. Oldfield
Department of Neurosurgery (E.N.K., E.H.O.), University of Virginia, Charlottesville, Virginia 22908-0212; and Section on Endocrinology and Genetics (M.K., M.L., C.S.), Program in Developmental Endocrinology and Genetics, and Pediatric Endocrinology Program, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892
Address all correspondence and requests for reprints to: Edward H. Oldfield, M.D., Department of Neurological Surgery, University of Virginia, P.O. Box 800212, Charlottesville, Virginia 22908-0212.
Context: Pseudotumor cerebri has only been described after successful surgery for Cushing’s disease (CD) in case reports. We sought to establish the incidence and timing of its occurrence, identify predisposing factors, characterize the clinical presentations and their severity, and examine the effects of treatment in patients who underwent surgery for CD.
Setting: This study was conducted at two tertiary care centers: The University of Virginia and the National Institutes of Health.
Patients: We conducted a retrospective review of 941 surgeries for CD (723 adults, 218 children) to identify patients who developed pseudotumor cerebri after surgery for CD and examine the associated clinical features.
Results: Seven children (four males, three females; 3%), but no adults, developed pseudotumor cerebri postoperatively. All underwent resection of an ACTH-secreting adenoma, and postoperative serum cortisol reached a nadir of less than 2 µg/dl. After surgery, all were placed on tapering hydrocortisone replacement therapy. Within 3–52 wk, all seven patients experienced symptoms of pseudotumor cerebri and had ophthalmological examination demonstrating papilledema. One patient had diplopia from a unilateral VIth nerve palsy. Six patients were still on steroid replacement at onset of symptoms. In three patients, a lumbar puncture demonstrated elevated opening pressure. Four patients were treated successfully with a lumbar puncture, steroids, and/or Diamox. Three patients did not receive treatment, and their symptoms resolved over several months. There was no correlation between the degree of hypercortisolism (24-h urinary free cortisol) before surgery and the likelihood of developing pseudotumor cerebri after surgery (P < 0.23).
Conclusions: This series demonstrates a 3% occurrence of pseudotumor cerebri in children after successful surgery for CD, but the absence of the syndrome in adults. Pseudotumor cerebri manifests itself within 1 yr of surgery, often while patients are still undergoing replacement steroid therapy. A patient exhibiting signs of intracranial hypertension after surgery for CD should undergo an evaluation for pseudotumor cerebri. Recognition of the symptoms and treatment should correct and/or prevent ophthalmological sequelae.