PNA Medical Corner: Cushing’s Surgery in Children

Gazioglu1This month the PNA Medical Corner spotlights an article co-authored by PNA member Dr. Nurperi Gazioglu, a professor of neurosurgery at Istanbul University Cerrahpasa Medical Faculty. The study looks at surgery for cases of pediatric Cushing’s and concludes that transsphenoidal surgery, both microscopic and endoscopic, is safe for children.

Childs Nerv Syst. 2018 Nov 28. doi: 10.1007/s00381-018-4013-5. [Epub ahead of print]
Neurosurgical treatment of Cushing disease in pediatric patients: case series and review of literature.
Gazioglu N1, Canaz H2, Camlar M3, Tanrıöver N4, Kocer N5, Islak C5, Evliyaoglu O6, Ercan O6.

Author Information

1Department of Human Pathology of Adulthood and Childhood, University of Messina, Messina, Italy. [email protected]
2Department of Human Pathology of Adulthood and Childhood, University of Messina, Messina, Italy.
3Unit of Endocrinology, University Hospital 'G. Martino', Messina, Italy.
4Department of Biomedical Sciences, Dental Sciences and Morpho-functional Imaging, University of Messina, Messina, Italy.
5Department of Economics, University of Messina, Messina, Italy.

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
PMID:30488233
DOI:10.1007/s00381-018-4013-5

 

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