title: Single-center experience with pediatric Cushing's disease.
abstract: OBJECTIVE
  Despite ongoing advances in surgical and radiotherapeutic techniques, pediatric Cushing's disease remains a diagnostic and therapeutic challenge. The authors report on the results of a single-center retrospective review of 33 pediatric patients with Cushing's disease, providing details with respect to clinical presentation, diagnostic evaluation, therapeutic course, complications, and outcomes.
  
  
  METHODS
  There were 17 female and 16 male patients whose mean age was 13 years (range 5-19 years) in whom a diagnosis of Cushing's disease was based on clinical and biochemical criteria. Typical symptoms included weight gain (91%), prepubertal growth delay (83%), round facies (61%), hirsutism (58%), headache (45%), abdominal striae (42%), acne (33%), amenorrhea (24%), and hypertension (24%). In 67% of the cases, preoperative magnetic resonance images revealed a pituitary lesion and in 82% of the cases the imaging studies effectively predicted lateralization. Inferior petrosal sinus sampling was performed in seven patients (21%), and in all of these cases lateralization was 100% reliable. Fifty-five percent underwent selective adenomectomies and 45% underwent subtotal hypophysectomies. Complications included one case of diabetes insipidus, one of persistent hypocortisolemia necessitating prolonged glucocorticoid replacement therapy, and one minor vascular injury that did not necessitate postoperative management modification or cause sequelae. There were no surgery-related deaths and no cases of postoperative cerebrospinal fluid leakage or meningitis. During a mean follow-up period of 44 months, clinical remission was ultimately achieved in 91% of patients: 76% after transsphenoidal surgery alone and an additional 15% after adjuvant radiosurgery and/or adrenalectomy following surgical failure. Three patients (12%) experienced disease recurrence and underwent a second surgical procedure at 18, 81, and 92 months, respectively; based on clinical and biochemical criteria a second remission was achieved in all. Three patients (9%) remain with persistent disease.
  
  
  CONCLUSIONS
  Pediatric Cushing's disease is a rare condition, often requiring a multidisciplinary diagnostic and a multimodal therapeutic approach for successful long-term remission.
authors.affiliation: Department of Neurological Surgery, University of Virginia Health System, Charlottesville, Virginia 22908, USA.
authors.forename: Adam S
authors.initials: AS
authors.lastname: Kanter
authors.forename: Alfa O
authors.initials: AO
authors.lastname: Diallo
authors.forename: John A
authors.initials: JA
authors.lastname: Jane
authors.forename: Jason P
authors.initials: JP
authors.lastname: Sheehan
authors.forename: Ashok R
authors.initials: AR
authors.lastname: Asthagiri
authors.forename: Rod J
authors.initials: RJ
authors.lastname: Oskouian
authors.forename: David O
authors.initials: DO
authors.lastname: Okonkwo
authors.forename: Charles A
authors.initials: CA
authors.lastname: Sansur
authors.forename: Mary Lee
authors.initials: ML
authors.lastname: Vance
authors.forename: Alan D
authors.initials: AD
authors.lastname: Rogol
authors.forename: Edward R
authors.initials: ER
authors.lastname: Laws
mesh_terms: D000293:Adolescent; D000311:Adrenal Glands / Q000601:surgery*; D000328:Adult; D000568:Amenorrhea / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006130:Growth Disorders / Q000209:etiology; D006628:Hirsutism / Q000209:etiology; D006801:Humans; D008297:Male; D047748:Pituitary ACTH Hypersecretion / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D012008:Recurrence; D012189:Retrospective Studies; D016896:Treatment Outcome; D015430:Weight Gain