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Neurosurgical treatment of Cushing disease in pediatric patients: case series and review of literature

Child's Nervous System volume 35, pages 2127–2132 (2019)Cite this article

Abstract

Aim

Pituitary adenomas are rare in childhood in contrast with adults. Adrenocorticotropic hormone (ACTH)-secreting adenomas account for Cushing’s disease (CD) which is the most common form of ACTH-dependent Cushing’s syndrome (CS). Treatment strategies are generally based on data of adult CD patients, although some difficulties and differences exist in pediatric patients. The aim of this study is to share our experience of 10 children and adolescents with CD.

Patients and method

Medical records, images, and operative notes of 10 consecutive children and adolescents who underwent transsphenoidal surgery for CD between 1999 and 2014 in Cerrahpasa Faculty of Medicine were retrospectively reviewed. Mean age at operation was 14.8 ± 4.2 years (range 5–18). The mean length of symptoms was 24.2 months. The mean follow-up period was 11 years (range 4 to 19 years).

Results

Mean preoperative cortisol level was 23.435 μg/dl (range 8.81–59.8 μg/dl). Mean preoperative ACTH level was 57.358 μg/dl (range 28.9–139.9 μg/dl). MR images localized microadenoma in three patients (30%), macroadenoma in four patients (40%) in our series. Transsphenoidal microsurgery and endoscopic transsphenoidal surgery were performed in 8 and 2 patients respectively. Remission was provided in 8 patients (80%). Five patients (50%) met remission criteria after initial operations. Three patients (30%) underwent additional operations to meet remission criteria.

Conclusion

Transsphenoidal surgery remains the mainstay therapy for CD in pediatric patients as well as adults. It is an effective treatment option with low rate of complications. Both endoscopic and microscopic approaches provide safe access to sella and satisfactory surgical results.

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Authors and Affiliations

  1. Department of Neurosurgery, Istanbul Bilim University, Istanbul, Turkey

    Nurperi Gazioglu & Huseyin Canaz

  2. Department of Neurosurgery, Tepecik Research and Training Hospital, University of Health Sciences, Izmir, Turkey

    Mahmut Camlar

  3. Department of Neurosurgery, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey

    Necmettin Tanrıöver

  4. Department of Radiology, Division of Neuroradiology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey

    Naci Kocer & Civan Islak

  5. Pediatric Endocrinology and Adolescent Divisions, Department of Pediatrics, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey

    Olcay Evliyaoglu & Oya Ercan

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  1. Nurperi Gazioglu
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  2. Huseyin Canaz
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Correspondence to Huseyin Canaz.

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Gazioglu, N., Canaz, H., Camlar, M. et al. Neurosurgical treatment of Cushing disease in pediatric patients: case series and review of literature. Childs Nerv Syst 35, 2127–2132 (2019). https://doi.org/10.1007/s00381-018-4013-5

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Keywords

  • Cushing’s disease
  • Endoscopic pituitary surgery
  • Pediatric
  • Transsphenoidal microsurgery