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KMID : 1130320100530070759
Korean Journal of Pediatrics
2010 Volume.53 No. 7 p.759 ~ p.765
Risk factor for pituitary dysfunction in children and adolescents with Rathke¡¯s cleft cysts
Lim Han-Hyuk

Yang Sei-Won
Abstract
Purpose: This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke¡¯s cleft cysts.

Methods: Forty-four patients with Rathke¡¯s cleft cysts younger than 19 years who visited Seoul National University Children¡¯s Hospital between January 1995 and September 2009 were enrolled. Rathke¡¯s cleft cysts were confirmed histologically through an operation in 15 patients and by brain magnetic resonance imaging (MRI) in 29 patients. The clinical, hormonal, and imaging features were reviewed retrospectively.

Results: The clinical presentation of symptomatic patients was as follows: headache (65%), endocrinopathy (61%), and visual disturbance (19%). Endocrinopathy included central precocious puberty (18%), diabetes insipidus (14%), general weakness (11%), and decreased growth velocity (7%). After surgery, hyperprolactinemia resolved in all patients, but growth hormone insufficiency, hypothyroidism, and diabetes insipidus did not improve. Pituitary insufficiency except gonadotropin abnormality correlated significantly with severe headache, visual disturbance, general weakness, and cystic size. Suprasellar extension of cysts and high signals in the T2-weighted image on brain MRI were related to hypothyroidism, hypocortisolism, and diabetes insipidus. Multivariable linear regression analysis showed that only general weakness was a risk factor for pituitary insufficiency (R2=0.549).

Conclusion: General weakness is a risk factor for pituitary insufficiency in patients with Rathke¡¯s cleft cysts. When a patient with a Rathke¡¯s cleft cyst complains of general weakness, the clinician should evaluate pituitary function and consider surgical treatment.
KEYWORD
Rathke¡¯s cleft cysts, Pituitary insufficiency
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