Background: Rathkes cleft cyst (RCC) is a benign, sellar or suprasellar lesion arising from the remnants of Rathkes pouch that have failed to disappear in the normal development of the embryo. Patients with RCCs are mostly asymptomatic but can also exhibit various symptoms related to pituitary insufficiency. With increasing interest on endocrinology disorders of children or adolescents, the prevalence of RCCs is also on the rise. However, the studies on children or adolescents with RCCs were insufficient so far.
Objective and hypotheses: We investigated clinical manifestations, endocrinology dysfunction, radiological features of patients confirmed RCC and underwent linkage analysis in multiple aspects with sufficient number of enrolled children and adolescents with RCCs.
Method: According to the retrospective review of medical records in the present study, we obtained patients clinical information. We also examined endocrinology function of enrolled cases by basal hormones test or combined pituitary function test or a gonadotropin releasing hormone stimulation test. MRI findings of RCCs were classified with the size, the location and the signal intensities at T1 weighted and T2 weighted settings. Then we tried to investigate association among clinical symptoms, endocrine disorders and radiologic characteristics.
Results: Chief complaints of patients varied depending on the age group. About 80% of patients who performed endocrine function test revealed to have pituitary insufficiencies, most common of which was central precocious puberty. There was no significant difference between the endocrine disorder group and normal group regarding the MRI findings. Seven of ninety-three patients received surgery for various reasons to alleviate symptoms, to check malignancy, or due to continuously increasing size of RCC. It was found that the RCC size of patients who needed operation was significantly larger than those who had no need of the operation. In addition, among those who received the operation, the number of RCCs that were restricted to the suprasella or extended to that area was significantly greater. However, the endocrine disorders in three of seven patients were aggravated after the surgery. Lastly, we discovered that among 66 patients who performed 2-year follow up MRI, about 20% of patients experienced the increase of RCCs.
Conclusion: We can conclude that it is necessary to perform routine following ups of endocrine function test and image study in patients with RCCs.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology