Background: Due to the rarity of Paediatric Cushings disease (CD) there is limited data on the long-term consequences of treatment.
Objective and hypotheses: We assessed recurrence, anterior pituitary function and psychiatric disorders in a group of paediatric CD patients treated in a single centre.
Method: Retrospective review of 20 patients with CD, mean age 11.75 years (5.7417.8), managed in our centre between 1986 and 2010. Mean follow-up from the first surgical treatment was 10.5 years (5.0127.2). Cure was defined as undetectable postoperative 09:00 cortisol level (<50 nmol/l) and cure after radiotherapy (RT) by mean serum cortisol on 5-point day curve of <150 nmol/l and midnight sleeping cortisol <50 nmol/l.
Results: 14 patients were cured by transsphenoidal surgery (TSS) and five were cured following TSS+radiotherapy (RT). One patient underwent bilateral adrenalectomy (BA) following failed TSS. CD recurrence was seen in 3 (15%) patients: two after TSS (2 years after TSS) and one following TSS+RT (2 years post RT). The former two patients were treated with TSS+RT successfully. The latter was treated by BA. One BA patient developed Nelsons syndrome requiring RT 0.5 years post surgery. GHD was documented in 11 patients (55%) (seven following TSS and four after TSS+RT) and three (15%) had long-term GHD. 15 patients were treated with hGH, nine have reached final height (FH) on treatment. Gonadotropin deficiency causing delayed or slow pubertal development was diagnosed in six patients (30%), only one needed treatment post-pubertally. There were no reproductive problems. two (10%) patients had TSH deficiency. Two (10%) had psychiatric problems and two (10%) have poor memory and concentration after treatment.
Conclusion: Pituitary deficiencies occurred in 60% patients after treatment for CD but long term deficiencies were less common (25% patients). Relapse occurred in 15% of patients after apparent cure of CD and emphasises the importance of continued surveillance.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology