Pituitary Hormonal Abnormalities in Children and Adolescents with Rathke's Cleft Cysts

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 596-630-Pediatric Endocrinology
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-608
Jung Min Ahn*1 and Ho-Seong Kim2
1Severance Chidren's Hospital, SEOUL, South Korea, 2Severance Children's Hospita, Seoul, South Korea
Purpose: Rathke’s cleft cyst (RCC) is benign cystic lesion of the sella that may affect the pituitary function and the visual acuity. The RCC and its clinical manifestations are rarely reported in children and adolescents. This study was conducted to evaluate the endocrinologic disorders in patients with RCC under the age of 20. Methods: One hundred and fifteen patients with RCC younger than 20 years who visited Yonsei University Severance Children’s Hospital between January 2001 and October 2012 were enrolled. The RCC was confirmed by brain magnetic resonance imaging (MRI). The clinical features and hormonal, imaging findings were retrospectively reviewed for evaluating endocrinologic problems.Results: The mean age of 115 patients was 13.6±5.4 years (M=33, F=82, making the M:F ratio of 1:2.48). The chief complaints of patients were precocious puberty (N=38, 33.0%), headache (N=29, 25.2%), short stature (N=17, 14.8%), accidental discovery (N=13, 11.3%), visual disturbances (N=11, 9.6%), polyuria and polydipsia (N=7, 6.1%), and dysmenorrhea (N=7, 6.1%). Especially precocious puberty was the main problem in female (N=35, 42.7%) while short stature was in male (N=11, 33.3%). The most common diagnosis on the basis of endocrinologic examination were growth hormone deficiency (GHD), central precocious puberty (CPP) and thyrotrophin deficiency, respectively 18.3% (N=21), 17.4% (N=20) and 13.0% (N=15). On MRI imaging, location of cyst was almost intrasella (N=92, 80%), and the next was intrasella with suprasellar extension (N=14, 12.2%). However, location of cyst was not associated with hormonal disorder. On sagittal view of MRI, mean cyst size was 8.1±5.0 mm and it was correlated with age (r=0.419, P<0.001). Cyst size of patient with GHD was larger than that of patient without GHD (10.7±7.3 mm vs. 7.4±4.1 mm, p<0.001). However, patient with CPP had smaller cyst than patient without CPP (8.7±5.3 mm vs. 5.2±1.5 mm, p<0.001). Eleven patients were performed surgical management and four of them were recurred. After surgery, headache and visual disturbance were improved, but pituitary insufficiency were not improved.  Conclusion: The RCC is rare in children and adolescents but it can cause many endocrinologic problems. Therefore, patient with pituitary hormonal abnormality should be performed sella MRI to find out structural abnormalities including RCC.

Disclosure: HSK: Investigator, Merck Serono, Planning Group Member, Merck Serono. Nothing to Disclose: JMA

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