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 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 (AU)

Endoscopic surgery for intraventricular arachnoid cysts in children: clinical presentation, radiological features, management, and outcomes over a 12-year period.

PURPOSE: Less than 0.5% of arachnoid cysts are intraventricular in origin. We review our experience with endoscopic surgery for intraventricular arachnoid cysts in children. METHODS: This is a retrospective review of children with intraventricular arachnoid cysts who underwent surgery between 2005 and 2016. Clinical notes and imaging were reviewed. RESULTS: Twenty-nine patients with endoscopically treated intraventricular arachnoid cysts were identified (M/F = 17:12; median age = 1.47 years, range = 7 days-13 years). All had hydrocephalus at presentation, many had symptoms/signs of raised intracranial pressure, and five (17%) were asymptomatic. Cysts were treated with fenestration into the ventricle alone (ventriculocystostomy [VC], n = 14), fenestration into the ventricle and cisternostomy (ventriculocystostomy plus cisternostomy [VC + C], n = 14), or endoscopic third ventriculostomy alone (n = 1). Six (21%) patients experienced transient and/or conservatively managed complications. Further surgery was required in 12 (41%). Revision-free survival was significantly shorter with VC compared to VC + C (log rank p = 0.049), and the majority of VC/VC + C revisions (n = 8 of 11, 73%) were required within 6 months of initial endoscopic surgery. One (3%) patient died during follow-up, from unrelated pathology. After a median follow-up of 67.5 months in survivors (range = 5.5-133.5 months), 24 (83%) cases were clinically and radiologically stable without a shunt in situ. CONCLUSIONS: Endoscopic fenestration is safe and effective in most intraventricular arachnoid cysts. Additional cisternostomy at the time of cyst fenestration into the ventricle significantly improved revision-free survival in our cohort. Endoscopic surgery should be the first-line therapy when considering intervention for symptomatic intraventricular arachnoid cysts and for asymptomatic cysts increasing in size on serial imaging.

Endoscopic Multiple Wide Fenestrations of Intraventricular Arachnoid Cyst Arising in Lateral Ventricle

Intraventricular arachnoid cyst is an uncommon disease and a few surgical experiences have been reported. The authors present an experience of neuroendoscopic surgery in a 4-year-old-male patient with a large arachnoid cyst arising from the right lateral ventricle, who had suffered from generalized tonic clonic seizure. On operation, various portions of the cyst could be easily approached without unwanted parenchymal injury because most of the cystic membrane was movable and not adherent to the ventricular wall except choroid plexus area, which could be considered as origin site of the cyst. Marked shrinkages by electrocoagulation and multiple wide fenestrations of the cyst were obtained through the endoscopic working channel without difficulty. Postoperative brain computed tomography demonstrated decreasing ventricular size with no evidence of cystic recurrence. The postoperative course was uneventful for eight months follow-up period. We suggest that endoscopic procedure has definite advantages as the surgical method of choice for the treatment of intraventricular arachnoid cysts.

Two Cases of Intraventricular Arachnoid Cyst

Two rare cases of intraventricular arachnoid cyst locating in the posterior portion of the third ventricle are presented. A diagnosis was based on the radiological and operative findings and the microscopic features. Cyst wall fenestration by a direct approach resulted in good outcome. The orgin and the management of arachnoid cyst are discussed.

Two Cases of Intraventricular Arachnoid Cyst

Two rare cases of intraventricular arachnoid cyst locating in the posterior portion of the third ventricle are presented. A diagnosis was based on the radiological and operative findings and the microscopic features. Cyst wall fenestration by a direct approach resulted in good outcome. The orgin and the management of arachnoid cyst are discussed.