Endocrine Abstracts

Introduction: ACTH-secreting pituitary adenomas are the most common cause of endogenous hypercortisolemia in children after 10 years of age. In spite of this pediatric Cushing’s disease (pCD) is a rare medical condition. Transsphenoidal surgery (TSS) remains the treatment of choice but – according to literature date – results of such a procedure could be worse comparatively to adult population. The aim of the study was to evaluate the clinical course, safety, efficacy and complications of TSS for pCD.

Method: Among 312 patients with Cushing’s disease operated on between 2000 and 2011 we identified 11 patients with pCD – five boys and six girls. The mean age was 16.9 years (range: 13–18). The diagnosis was based on commonly adopted hormonal criteria as well as preoperative magnetic resonance imaging. TSS was performed according to the same microsurgical protocol. The remission was assessed based on the early postoperative, subnormal serum cortisol levels (≤2.5) and its dynamics during at least 12 months follow-up.

Results: Growth retardation and overweight or obesity were present in all 11 patients. Hypertension and mental disorders were diagnosed in five of them (45.5%). In all cases, a pituitary microadenoma was precisely visualized in MRI. Based on adopted hormonal criteria nine children (81.8%) were considered to be surgically cured whereas in two patients (18.2%) the persistent CD was confirmed. There were no medical and fatal complications. Transient diabetes insipidus appeared in three patients (27.3%) and syndrome of inappropriate antydiuresis (SIAD) in additional two cases (18.2%). Pituitary insufficiency was observed in one patient (9.1%). There was no case of postoperative cerebral spinal fluid leakage or meningitis.

Conclusions: Clinical manifestation and gender distribution of pCD is different comparatively to adult population. In spite of this TSS is a safe and effective treatment leading to a high rate of biochemical remission, similar to observed in adults.