ESPE Abstracts (2014) 82 P-D-1-3-196

ESPE2014 Poster Presentations Pituitary (14 abstracts)

Rathke Cleft Cysts and Endocrine Dysfunction in Children

Marie Dupre a , Frederic Brioude b , Laurence Berard b , Blandine Esteva b & Muriel Houang b

aCentre Hospitalier, Valenciennes, France; bHopital Armand Trousseau, Paris, France

Background: Rathke’s cleft cysts (RCC) are benign lesions of the pituitary gland, remnants of Rathke’s pouch. RCC have rarely been reported in children and adolescents and are usually known as asymptomatic but some cases may be associated with hormonal disturbances.

Objective and hypotheses: The aim of the study was to assess the characteristics and frequency of endocrine disturbances in children with RCC.

Method: We retrospectively reviewed the clinical, biological, and magnetic resonance imaging findings in all RCC cases seen in our institution from 2004 to 2014.

Results: We found ten children with RCC. Their age ranged from 14 months to 16 years old (median age at 13.5 years) at diagnosis. They were six males and four females. Common features at presentation were headaches (4/10), growth retardation (3/10), polyuria–polydipsia (1/10), visual disturbances (1/10), and one was asymptomatic. Six children had endocrine dysfunctions and four of them multiple deficiencies. There were five GH deficiencies (three complete GH deficiencies and two partial GH deficiencies), two TSH deficiencies, three ACTH deficiencies, one hypogonadotropic hypogonadism, and two diabetes insipidus. All cysts were in intrasellar position and two of them had a suprasellar portion. Five patients had a cyst longer than 10 mm. Two cases underwent surgical procedure; endocrine deficiencies remained after surgery.

Conclusion: RCC are rare and association with hypopituitarism or diabetes insipidus is higher than expected. This association is not fortuitous since we did not find any RCC in all MRI done for central precocious puberty during the last 10 years. Careful follow up is recommended; surgical procedures are required in case of visual disturbances, persistent headaches, or diagnostic doubt.

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