Symptomatic Rathke cleft cyst in paediatric patients – clinical presentations, surgical treatment and postoperative outcomes – an analysis of 38 cases

Abstract

Abstract Background: Rathke cleft cysts (RCC) are benign, epithelium-lined intrasellar and/or suprasellar cysts believed to originate from the remnants of the Rathke pouch. The aim of this study was to analyse the symptoms and surgical outcome of patients with the diagnosis of RCC verified in a histopathological examination of the postoperative material. Methods: The study is a retrospective analysis of 38 cases of children who underwent a neurosurgical treatment due to RCC at the Children’s Memorial Health Institute in Warsaw, Poland, between 1994 and 2015. Results: At diagnosis, the mean age was 13 years and 8 months (6 years and 11 months–17 years and 10 months, sex ratio was 1:0.9 with a female prevalence). The most common symptoms were the following: headache (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%) and visual disturbances (11%). Due to the gravity of symptoms and size of the lesion, all the patients underwent a surgical treatment. All but one were successful (one patient died due to postoperative neurosurgical complications). The most common postoperative complications were the following: adenohypopituitarism (67%) and diabetes insipidus (45%). Conclusions: RCC can present with serious symptoms that significantly deteriorate patients’ quality of life. Despite a successful neurosurgical treatment in most of the analysed cases, patients required long-term pharmacological treatment.