Elnaggar, AM;
Dattani, M;
Gan, HW;
Storr, HL;
Matthew Matson;
Drake, WM;
Dorward, N;
... Aquilina, K; + view all
(2025)
Cushing’s disease in children: a single-centre experience.
Child's Nervous System
, 41
, Article 293. 10.1007/s00381-025-06952-9.
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Abstract
Purpose: To review our institutional experience of paediatric Cushing's disease (CD) over the last 15 years to determine outcomes and complications. Methods: We reviewed clinical details, neuroradiology and outcomes of all children who underwent surgery for CD in our institution between 2006 and 2023. Results: 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: 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.
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