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. 2025 Sep 25;41(1):293.
doi: 10.1007/s00381-025-06952-9.

Cushing's disease in children: a single-centre experience

Ahmed Mahmoud Elnaggar  1 , Mehul Dattani  2 , Hoong-Wei Gan  2 , Helen L Storr  3 , Matthew Matson  4 , William M Drake  5 , Neil Dorward  6 , Joan Grieve  6 , Kristian Aquilina  7
Affiliations

Cushing's disease in children: a single-centre experience

Ahmed Mahmoud Elnaggar et al. Childs Nerv Syst. .

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.

Keywords: Inferior petrosal sinus sampling; Pituitary adenoma; Transsphenoidal surgery (TSS).

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Conflict of interest statement

Declarations. Conflicts of interest: The authors have no relevant financial or non-financial interests to disclose.

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