Background: Rathke cleft cysts are benign, epithelium-lined intrasellar and/or suprasellar cysts believed to originate from remnants of the Rathke pouch. Although its prevalence in adults is rather high, Rathke cleft cysts are rare in children. Often they are asymptomatic findings, however depending on their size and localization they can present with a wide spectrum of symptoms.
Objective and hypotheses: The aim of the study was to analyse the symptoms and surgical outcome of patients with the diagnosis of Ratke cleft cyst based on histopathological examination of postoperative material.
Method: The study is a retrospective analysis of 38 cases of children who underwent a neurosurgical treatment due to Rathke cleft cyst in Childrens Memorial Health Institute in Warsaw, Poland between 19942015.
Results: Sex ratio was 1:0,9 (20 girls and 18 boys), two female patients were twins. At diagnosis patients were between 6 years 11 months and 17 years 10 months old with mean age of 13 years and 8 months. Average diameter of the cyst was 16,7 mm. In six cases calcifications in the lesions were observed either in brain imagining or intraoperatively. Most common symptoms were: headaches (50%), hypothyroidism (50%), short stature and/or decreased growth velocity (47%), delayed puberty and menstrual abnormalities (37%), diabetes insipidus or polydipsia and polyuria (26%), adrenal dysfunction (26%), sleepiness and general weakness (13%), visual disturbances (11%). 29 patients underwent a transsphenoidal operation and in 9 a craniotomy was performed. All but one were successful (one patient died due to postoperative neurosurgical complications). The most common postoperative complications were: diabetes insipidus, adenohypophysis, overweight and obesity.
Conclusion: Although Rathke clefts cysts are considered to be benign, non-neoplastic lesions they can present with serious symptoms that deteriorate significantly patients quality of life. In spite of successful neurosurgical treatment in most of the analysed cases patients required a long-term pharmacological treatment due to endocrinological complications.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology