Evidence map›Paper›PMID 40996550›Full record

ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2025

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

Ahmed Mahmoud Elnaggar, Mehul Dattani, Hoong-Wei Gan, Helen L Storr, Matthew Matson, William M Drake, Neil Dorward, Joan Grieve, Kristian Aquilina

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Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

9 authors.

Ahmed Mahmoud ElnaggarDepartment of Neurosurgery, Great Ormond Street Hospital, London, UK. Ahmed.Elnaggar5@nhs.net.
Mehul DattaniDepartment of Endocrinology, Great Ormond Street Hospital, London, UK.
Hoong-Wei GanDepartment of Endocrinology, Great Ormond Street Hospital, London, UK.
Helen L StorrDepartment of Endocrinology, Queen Mary University, London, UK.
Matthew MatsonDepartment of Radiology, St Bartholomew's Hospital, London, UK.
William M DrakeDepartment of Endocrinology, St Bartholomew's Hospital, London, UK.
Neil DorwardDepartment of Neurosurgery, The National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
Joan GrieveDepartment of Neurosurgery, The National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
Kristian AquilinaDepartment of Neurosurgery, Great Ormond Street Hospital, London, UK.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeTo review our institutional experience of paediatric Cushing's disease (CD) over the last 15 years to determine outcomes and complications.

methodsWe reviewed clinical details, neuroradiology and outcomes of all children who underwent surgery for CD in our institution between 2006 and 2023.

results25 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.

conclusionCD 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.

Indexed as

Pituitary ACTH HypersecretionAdolescentChildChild, PreschoolFemaleHumansMagnetic Resonance ImagingMaleRetrospective StudiesTreatment OutcomeInferior petrosal sinus samplingPituitary adenomaTranssphenoidal surgery (TSS)

Identifiers

PMID40996550

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