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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Autores principales: Gonzalo Oliván-Gonzalvo, Vicente Calatayud-Maldonado
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Publicado: Emergency Department of Hospital San Pedro (Logroño, Spain) 2020
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spelling 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)
institution DOAJ
collection DOAJ
language EN
topic central precocious puberty
intraventricular arachnoid cyst
children
rare diseases
gnrh test
brain mri
surgery
gnrh analogs
Medicine (General)
R5-920
spellingShingle 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
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