Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update
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 th...
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Emergency Department of Hospital San Pedro (Logroño, Spain)
2020
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oai:doaj.org-article:dc8392758c3e4eb1b785728a6ec617ba2021-12-02T19:14:49ZCoexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update10.5281/zenodo.43232872695-5075https://doaj.org/article/dc8392758c3e4eb1b785728a6ec617ba2020-12-01T00:00:00Zhttps://doi.org/10.5281/zenodo.4323287https://doaj.org/toc/2695-5075Central 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.Gonzalo Oliván-GonzalvoVicente Calatayud-MaldonadoEmergency Department of Hospital San Pedro (Logroño, Spain)articlecentral precocious pubertyintraventricular arachnoid cystchildrenrare diseasesgnrh testbrain mrisurgerygnrh analogsMedicine (General)R5-920ENIberoamerican Journal of Medicine, Vol 3, Iss 1, Pp 65-70 (2020) |
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central precocious puberty intraventricular arachnoid cyst children rare diseases gnrh test brain mri surgery gnrh analogs Medicine (General) R5-920 |
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central precocious puberty intraventricular arachnoid cyst children rare diseases gnrh test brain mri surgery gnrh analogs Medicine (General) R5-920 Gonzalo Oliván-Gonzalvo Vicente Calatayud-Maldonado Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update |
description |
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. |
format |
article |
author |
Gonzalo Oliván-Gonzalvo Vicente Calatayud-Maldonado |
author_facet |
Gonzalo Oliván-Gonzalvo Vicente Calatayud-Maldonado |
author_sort |
Gonzalo Oliván-Gonzalvo |
title |
Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update |
title_short |
Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update |
title_full |
Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update |
title_fullStr |
Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update |
title_full_unstemmed |
Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update |
title_sort |
coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update |
publisher |
Emergency Department of Hospital San Pedro (Logroño, Spain) |
publishDate |
2020 |
url |
https://doaj.org/article/dc8392758c3e4eb1b785728a6ec617ba |
work_keys_str_mv |
AT gonzaloolivangonzalvo coexistenceofcentralprecociouspubertyandintraventriculararachnoidcystabriefliteratureupdate AT vicentecalatayudmaldonado coexistenceofcentralprecociouspubertyandintraventriculararachnoidcystabriefliteratureupdate |
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