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Arachnoid cysts are commonly considered congenital lesions, but this has not been proven. With the development of neuroimaging and DNA testing technology, more cases of familial arachnoid cysts have been reported. Herein, we review such cases. The PubMed, Embase, and Web of Science databases were searched for case reports of arachnoid cysts published through April 2018. Case reports were included only if two or more related patients were diagnosed with an arachnoid cyst by neuroimaging or intraoperatively. For each report, the following data were extracted: first author name, date of publication, number of families, number of patients, location of the arachnoid cysts, patient age, patient sex, and genetic mutations and associated disease. Our searches identified 33 case reports involving 35 families and 115 patients. The locations of arachnoid cysts were similar in 25 of the 35 families. Spinal extradural arachnoid cysts were reported most often, followed by arachnoid cysts in the middle fossa and posterior fossa. A left-sided predominance was noticed for arachnoid cysts of the middle fossa. Mutation of the FOXC2 gene was reported most often, and arachnoid cysts may be associated with mutations on chromosome 16. Although the origin of arachnoid cysts is believed to have a genetic component by some researchers, the genes associated with arachnoid cysts remain unknown. Unfortunately, the evidence remains insufficient.

Although middle fossa arachnoid cysts (MFACs) are common, the optimal surgical treatment for these lesions remains controversial. In this study, we present our experiences of treating MFACs by microsurgical fenestration and evaluate its effectiveness.We performed retrospective review of 28 patients who underwent microsurgical fenestration for MFACs between May 2003 and December 2014. We reviewed patient characteristics and treatment outcomes including age, sex, symptoms, complicating hydrocephalus, Gallasi classification, change in cyst size after surgery, complicating subdural hygroma, symptom resolution, regrowth of the cyst, and reoperation (including additional CSF diversion).Twenty-eight MFACs in 28 patients were investigated. The average age at the time of surgery was 61.6 months. The average follow-up duration was 53.5 months. After surgery, 19 (90.5 %) of 21 patients with symptomatic MFACs experienced improvements. The cysts decreased in size in all cases (100 %, 28/28) and disappeared in three cases (11 %, 3/28). None of the cases experienced regrowth of the cyst. Subdural hygroma was identified in 23 cases (82.1 %) at the immediate postoperative period. Hygroma was asymptomatic in all cases but one. Hygroma disappeared in 19 cases (83 %) and decreased in size in three cases (13 %) in the long term. Only one case (4 %) required an additional subdural-peritoneal shunt. The rate of CSF diversion after the fenestration was significantly higher in patients with preoperative ventricular dilation (p = 0.0002).Microsurgical fenestration for pediatric MFACs was considered to be safe and effective. Although subdural hygroma developed in significant number of patients at the immediate postoperative period, it was mostly asymptomatic and disappeared or decreased in size in the long term.

WOS: 000348115200010 PubMed ID: 25142689 The optimal surgical treatment for symptomatic middle fossa arachnoid cyst is still controversial. The most leading therapeutic options include cyst shunting and fenestration (endoscopic, microsurgical). We present our experience on surgical treatments of arachnoid cysts. A retrospective data review of 16 children who underwent keyhole craniotomy for microsurgical fenestration and shunting of middle fossa arachnoid cysts between 1999 and 2012 was performed after institutional review board approval. The average patient age was 6.1 years. The average follow-up period was 36.5 months. There were ten male and six female patients in the series. Indications for surgery included intractable headaches (50 %), increasing in cyst size (18.75 %), and seizures (31.25 %). All patient records were reviewed for their clinical presentation, classification, cyst resolution, symptom resolution, and cyst outcomes. After surgery, all patients underwent assessments of clinical and radiological improvement. Postoperative complications were observed in two cases: progressively resolving monoparesia in one case and resolving epileptic seizure with monotherapy in the other. All patients had a satisfactory clinical outcome, and in 87.5 %, there was either a decrease in the size or a complete disappearance of the MFAC. Nevertheless, three (18.75 %) of all patients needed shunt revision because of shunt dysfunction. Complication related to surgical technique was cerebrospinal fluid leak which spontaneously resolved in one patient. Microsurgical fenestration with keyhole craniotomy to provide passage between cysts to basal cisterns together with cystoperitoneal shunting during the same operation is still an effective and safe method in cases with symptomatic middle fossa arachnoid cysts in children.

The pathogenesis of chronic subdural hematoma (CSDH) associated with an arachnoid cyst (AC) is still not clear. We propose an origin of initial bleeding of CSDH in patients with AC based on our experience and discuss the management of this disease. The total number of operations included in this study was 23. Eleven cases were indicated because of associated CSDH (group 1), and the other cases were due to different reasons (group 2). The relationship of the AC and dura was evaluated in patients who did not have CSDH (group 2) because patients with CSDH would have had structural changes of AC due to hematoma. In group 1, the age distribution was from 8 to 61 years (mean 23.0). The development of CSDH was not related with Galassi types. The hematomas were located outside of the outer cyst membrane in six patients and both inside and outside in three patients. In two patients, the location could not be reviewed. After suspicion of the outer cyst membrane as the origin of the bleeding site, we found small bridging vessels between the dura and outer cyst membrane in three of five consecutive patients in group 2. Based on our surgical experience of AC cyst, we found small bridging vessels between the dura and outer membrane of the AC. We suggested that these small vessels are the source of initial bleeding leading to CSDH in AC.

Arachnoid cysts are benign developmental cysts occurring most commonly in the middle fossa. Posterior fossa arachnoid cysts are less common, with retroclival arachnoid cysts especially in the pediatric age group being rare. We present a case of a retroclival interpeduncular farachnoid cyst in a 10-year-old boy who presented with left-sided ptosis and episodes of hysterical breathlessness. The surgical treatment involved endoscopic assisted microsurgical excision of the cyst and the child made an uneventful and complete recovery with complete cessation of breathlessness. The clinical presentation, etiopathogenesis, imaging characteristics and treatment modalities are discussed and the relevant literature reviewed.

A 14-year-old girl presented with seizures. Radiological examinations revealed an arachnoid cyst in left middle fossa and a cystic mass in the interpeduncular cistern. The cyst was opened and the wall of the cyst and a mass were biopsied. The histological findings were characteristic of an arachnoid cyst and hamartoma, respectively. A hypothalamic hamartoma associated with an arachnoid cyst is comparatively rare; however, such a case may help clarify the genesis of this malformation.

A case of middle fossa arachnoid cyst is presented, which disappeared after head injury. Five days after the trauma, CT scan revealed subdural fluid collection in addition to an arachnoid cyst in the middle fossa on the same side. As the subdural fluid resorbed, the cyst became smaller and disappeared on the follow-up CT scans without surgical intervention.

Two cases of post-traumatic extradural hematoma complicating an arachnoid cyst of the middle cranial fossa in children are described. While subdural and intracystic hemorrhages are well-known complications from this malformation, the association with extradural hematoma has never been previously reported in the literature. The pathogenetic mechanisms are discussed and the particular vulnerability of intracranial arachnoid cysts is stressed.