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

  • Nurperi Gazioglu
  • Huseyin CanazEmail author
  • Mahmut Camlar
  • Necmettin Tanrıöver
  • Naci Kocer
  • Civan Islak
  • Olcay Evliyaoglu
  • Oya Ercan
Original Paper

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.

Keywords

Cushing’s disease Endoscopic pituitary surgery Pediatric Transsphenoidal microsurgery 

Notes

Compliance with ethical standards

Conflict of interest

There are no conflicts of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurosurgeryIstanbul Bilim UniversityIstanbulTurkey
  2. 2.Department of Neurosurgery, Tepecik Research and Training HospitalUniversity of Health SciencesIzmirTurkey
  3. 3.Department of NeurosurgeryIstanbul University Cerrahpasa Medical FacultyIstanbulTurkey
  4. 4.Department of Radiology, Division of NeuroradiologyIstanbul University Cerrahpasa Medical FacultyIstanbulTurkey
  5. 5.Pediatric Endocrinology and Adolescent Divisions, Department of PediatricsIstanbul University Cerrahpasa Medical FacultyIstanbulTurkey

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