Cushing's disease in children: a single-centre experience
No Thumbnail Available
Authors
Elnaggar A.M.
Dattani M.
Gan H.W.
Storr H.L.
MatthewMatson
Drake W.M.
Dorward N.
Grieve J.
Aquilina,K.
Check for full-text access
Issue Date
2025
Type
Article
Language
Keywords
Alternative Title
Abstract
Purpose: To review our institutional experience of paediatric Cushing's disease (CD) over the last 15 years to determine outcomes and complications. Method(s): We reviewed clinical details, neuroradiology and outcomes of all children who underwent surgery for CD in our institution between 2006 and 2023. Result(s): 25 children (14F) age 5-16 years (mean 11.8) underwent surgery for CD. 18 were pre-pubertal at diagnosis. Duration of symptoms to diagnosis was 2 months - 9 years (2.1 years). 70% percent had growth failure. Mean morning, sleeping midnight and 24-h urinary cortisol concentrations were 593, 476 and 652 nmol/L respectively. Inferior petrosal sinus sampling confirmed central ACTH secretion in all cases. MRI revealed an adenoma in 18 children (2.6 - 7 mm diameter). Microscopic and endoscopic transsphenoidal surgery (TSS) was performed in 14 and 11 respectively. Biochemical remission was achieved in 19 patients (76%) after TSS. There was no difference between the two operative approaches. Six failures were treated with revisional surgery (3), radiotherapy (2) or metyrapone / ketoconazole (1). Six children had transient diabetes insipidus post-operatively. CSF rhinorrhoea occurred in three cases and was managed successfully by a lumbar drain. Conclusion(s): CD in children is almost always diagnosed late despite established signs and symptoms, including weight gain and growth failure. TSS is as effective as in adults. A multidisciplinary team is crucial to the safe and effective management of these children. Copyright © The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2025.
Description
Citation
Publisher
License
Journal
Child's Nervous System
Volume
41
Issue
1