Outcome of Surgical Treatment of 200 Children With Cushing's Disease

• DOI: 10.1210/jc.2012-3604
• Publication Date: 2013-02-01T01:55:58Z
• Authors: Russell R. Lonser; Joshua J. Wind; Lynnette K. Nieman; Robert J. Weil; Hetty L. DeVroom; Edward H. Oldfield
• Keywords: Cushing's disease

Factors influencing the outcome of surgical treatment pediatric Cushing's disease (CD) have not been fully established. The aim this study was to examine features surgery for CD. In prospective observational study, clinical, imaging, endocrinological, and operative outcomes were analyzed in consecutive patients treated at National Institutes Health (NIH) from 1982 through 2010. conducted a tertiary referral center. Two hundred CD (106 females, 94 males) included. Mean age symptom development 10.6 ± 3.6 years (range, 4.0 19.0 y). NIH operation 13.7 3.7 years. Twenty-seven (13%) had prior another institution. Magnetic resonance imaging identified adenomas 97 (50%). When positive, magnetic accurately defined discrete adenoma 96 (99%), which more accurate than use ACTH ratios during inferior petrosal sinus sampling determine lateralization (accurate 72% without surgery). A total 195 200 (98%) achieved remission after (189 [97%] hypocortisolemic; 6 [3%] eucortisolemic postoperatively). associated with initial (P < .05) included identification an surgery, immunohistochemical ACTH-producing adenoma, noninvasive adenoma. Younger age, smaller absence cavernous wall or other dural invasion long-term .05). minimum morning serum cortisol less 1 μg/dl positive predictive value lasting 96%. With rare disorders, such as CD, enhanced are obtained by evaluation centers substantial experience. Resection pituitary that setting can be safe, effective, durable. Early postoperative endocrine testing predicts remission. Because is younger adenomas, lack invasion, early diagnosis should improve outcome.