Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update

Abstract

Abstract Central precocious puberty (CPP) is a rare disease. The mean annual incidence in girls is 0.8-1.1/100,000 and in boys 0-0.1/100,000. Intracranial arachnoid cysts (ICACs) are usually congenital and represent 1% of intracranial masses in newborns. Intraventricular location is rare. The objective of this work is to carry out a literature updated review of the coexistence of CPP and intraventricular arachnoid cyst (IVAC). ICACs are usually asymptomatic but can present with CPP in 10-40% of patients. IVACs represents only 0.3-1.4% of ICACs, and most seemed originate from the velum interpositum cistern. CPP in girls is usually idiopathic, while in 30-70% of boys are due to an intracranial lesion. Therefore, the coexistence of PPC and IVAC is very rare in boys and exceptional in girls. The exact mechanism of a cyst´s influence on the hypothalamic-pituitary axis is not completely understood. Theories include increased ventricular volume, associated mass effect on the hypothalamus, and direct compression of portions of the hypothalamic-pituitary axis. Analysis of LH peaks after GnRH testing is the gold standard for the diagnosis of CPP. Brain MRI should be part of the assessment in boys and also in girls since clinical features, including age and sex, are not helpful in predicting those with underlying brain pathology. In cases of CPP with IVAC, surgery does not have any effect on the course of pubertal development. The indication for surgery is the onset of neurological symptoms. The medical treatment selected, safe and effective, is GnRH analog depot preparations. In conclusion, there seems to be a consensus for the diagnosis and management of the coexistence of CPP and IVAC, but the etiopathogenesis is not yet well recognized.