Dalia L. Batista, Edward H. Oldfield, Margaret F. Keil, and Constantine A. Stratakis*
Section on Endocrinology and Genetics (SEGEN), Program in Developmental Endocrinology & Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), Pediatric Endocrinology Inter-Institute Training Program (PEITP), and Surgical Neurology Branch, National Institute of Neurological Diseases and Stroke (NINDS), National Institutes of Health (NIH), Bethesda, MD 20892, USA
* To whom correspondence should be addressed. E-mail: firstname.lastname@example.org.
Context: Post-operative testing after transsphenoidal surgery (TSS) for Cushing disease (CD) in children and its usefulness in predicting residual disease or recurrence are not well studied.
Objective: To identify which one of three tests that are routinely performed in our institution after TSS performs better in the identification of non-cured patients or predict relapse for CD.
Design: Retrospective review of clinical data of 72 consecutive children who received surgery for CD (age range: 5.8–18.3 years).
Setting: Tertiary care center.
Methods: After TSS plasma ACTH and serum cortisol (at 8 am), urinary free cortisol (UFC) values, and an oCRH stimulation test were obtained. Patients were followed for 24–120 months by a formal protocol.
Results: Of 72 children with CD, 66 (94%) achieved sustained remission after TSS. Two children had persistent disease following TSS, whereas four children appeared cured, at first, but relapsed later. All 4 had low or undetectable UFCs that were not different from cured patients (p>0.1). Children who remained in remission had significantly lower morning ACTH and cortisol levels after TSS compared to those who relapsed (p<0.001). During oCRH stimulation test, ACTH and cortisol values were higher in patients who relapsed vs. those in remission (p<0.001). Lack of histological confirmation of an adenoma, normal serum cortisol or ACTH, a normal response to oCRH, and glucocorticoid replacement for less than 6 months after surgery were associated with relapse.
Conclusion: In pediatric patients with CD, low UFCs after TSS are not good predictors of sustained remission; morning ACTH and cortisol values and/or an oCRH test after TSS predicted patients that recurred.
Key words: Cushing disease • children • cortisol • pituitary tumors • oCRH test • transsphenoidal surgery