ESPE Abstracts (2015) 84 FC11.6

aMU of Varna, UMHAT St Marina, Varna, Bulgaria; bThe Royal London Hospital, London, UK; cDepartment of Neurosurgery, St Bartholomew’s Hospital, London, UK; dDepartment of Otolaryngology, St Bartholomew’s Hospital, London, UK; eDepartment of Radiotherapy, St Bartholomews Hospital, London, UK; fDepartment of Radiology, St Bartholomew’s Hospital, London, UK; gDepartment of Endocrinology, St Bartholomew’s Hospital, London, UK; hCentre for Endocrinology, Wiliam Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK

Background: Due to the rarity of Paediatric Cushing’s 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 (5.74–17.8), managed in our centre between 1986 and 2010. Mean follow-up from the first surgical treatment was 10.5 years (5.01–27.2). ‘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 Nelson’s 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.

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