Management of Pediatric Cavum Cysts: A Scoping Review and a Single Institution Case Series.

INTRODUCTION: Pediatric cavum cysts are a rare yet complicated pathology to manage. The literature is scarce, primarily consisting of case series, and lacking a consensus regarding clear management. In this scoping review, we aimed to compile existing information in the literature regarding the management of pediatric cavum cysts across the last 10 years. We also present our management of 19 patients, the largest case series to date, highlighting knowledge gaps surrounding the management of this salient pathology. METHODS: A literature search using PubMed and SCOPUS was conducted using the following search terms: (pediatric) AND (Cavum septum pellucidum) OR (cavum vergae) OR (cavum velum interpositum) AND (management). Eligibility criteria included peer-reviewed publication published in the last 10 years, pediatric population, cavum cyst, and English language. A retrospective search was conducted for all pediatric cavum cysts between 2013 and 2023 at our institution. Clinical and radiographic characteristics as well as intervention and outcome data were collected for both the scoping review and our cases. RESULTS: 330 total articles were populated using our search. 12 articles met our inclusion criteria. 41.7% (n = 5) of the articles were case series, 33.3% (n = 4) were case reports, 8.3% (n = 1) was a technical article, 8.3% (n = 1) was a systematic review, and 8.3% (n = 1) was a case questionnaire. Resolution of symptoms was noted in all articles of our scoping review, regardless of treatment modality. The average age in our case series was 9.84 years old and average age at diagnosis was 5.53 years old. 6 patients (31.6%) were female and 13 patients (68.4%) were male. 2 out of the 19 patients (10.5%) were surgically treated. CONCLUSION: There is no clear consensus on the management of cavum cysts. A prospective, multicenter study is needed to create standardized pediatric cyst management guidelines. The current thought is that surgical intervention should be saved for those patients with obstructive hydrocephalus and signs of intracranial hypertension.

Idiopathic membranous occlusion of the foramen of Monro: an unusual cause of hydrocephalus and headache in adults.

Occlusion of foramen of Monro is an uncommon clinical entity that usually presents in children. Common causes are obstructing mass, infectious etiologies or vascular malformation. Rarely, it may be an idiopathic stricture or membrane. We report a case of idiopathic membranous obstruction of the foramen of Monro in a 45-year-old male with no past medical or surgical history. He presented with new intermittent dull and burning bifrontal severe headache for 2 d, which was alleviated slightly by non-steroidal anti-inflammatory medication. Imaging showed marked dilation of the lateral ventricles with normal third and fourth ventricles. The patient was discharged initially with conservative medical management and close follows up; however, the headache continued to progress and neurosurgical intervention was offered. The patient underwent endoscopic exploration, fenestration of the septum pellucidum, and right ventriculoperitoneal shunt placement. Bilateral membranous obstruction of foramina of Monro and an auto-fenestrated cavum septum pellucidum were identified intraoperatively. The patient reported resolution of headache post-operatively without recurrence on 1-month follow up. This case is unusual in that the patient presented without any known neurologic history or prior intracranial infections. It became apparent at the time of surgery that chronic obstruction of the bilateral foramina with collapse of the third ventricle had developed, and the safest durable treatment for him was septostomy and cerebrospinal fluid (CSF) shunt placement.

Surgical management of symptomatic cavum septum pellucidum cysts: systematic review of the literature.

Cavum septum pellucidum (CSP) and cavum vergae (CV) cysts are commonly found incidentally. They are usually asymptomatic but may present with symptoms related to obstructive hydrocephalus. There is no consensus about the management of symptomatic CSP and CV cysts. We present, to the best of our knowledge, the first systematic review of the different treatment options for symptomatic CSP and CV cysts. We conducted a literature review using PubMed database, searching for cases of symptomatic CSP and CV cysts managed surgically, and published until April 2019. Preoperative characteristics, surgical procedure, and postoperative outcome were analyzed using SPSS® software (Statistical Package for Social Sciences, IBM®). We found 54 cases of symptomatic CSP and CV cysts managed surgically (34 males, 20 females, 1.7/1 male to female ratio). Mean age was 24.3 ± 20.1 years. The most common presentation was headaches (34 patients, 62%), followed by psychiatric symptoms (27 patients, 49.1%). Preoperative radiological hydrocephalus was present in 30 patients (54.5%). The most common surgical procedure was endoscopic fenestration (39 patients, 70.9%), followed by shunting (10 patients, 18.2%), open surgery (3 patients, 5.5%), and stereotactic fenestration (1 patient, 1.8%). Complete resolution of symptoms was achieved in 36 patients (65.5%) and partial resolution in 7 patients (12.7%), and symptoms were unchanged in 2 patients. The present review suggests that surgical treatment could provide resolution of the symptoms in most of the cases, regardless of the procedure performed. Although mean follow-up was short among the studies, recurrence rate was low.

Pediatric rosette-forming glioneuronal tumor of the septum pellucidum.

Rosette-forming glioneuronal tumor (RGNT) is a rare tumor entity which has been reported mainly occurring in the fourth ventricle. It has been described as a benign lesion with limited extension into surrounding structures, including the cerebellar vermis, midbrain, and cerebral aqueduct. More recently, few cases involving also other midline structures have been documented as well. Here, we report about diagnosis and treatment of RGNT in the septum pellucidum in a pediatric patient which has not been described previously. A 7-year-old boy had a 3-week history of headache. Magnetic resonance imaging showed a solid mass in the septum pellucidum accompanied by hydrocephalus. The tumor was resected via a transcortical approach. Histological examination revealed the typical findings of a RGNT. At 2-year follow-up, there was no tumor recurrence, and clinical outcome was unremarkable. RGNT has to be considered in the differential diagnosis of pediatric midline tumors also outside of the fourth ventricle. Surgical resection is the first-line therapy which may result in beneficial outcome in the long term. The role of adjuvant therapy needs further definition since due to the rarity of this tumor entity, available data is very limited.

[Cysts of septum pellucidum, cavum vergae and cavum veli interpositi. Meta-analysis of 368 cases]. / Kisty prozrachnoi peregorodki, polosti Verge i promezhutochnogo parusa. Metaanaliz 368 klinicheskikh sluchaev.

OBJECTIVE: To review and systematize literature data on the incidence of cysts of septum pellucidum, cavum vergae and cavum veli interpositi, their clinical manifestations, indications for surgical treatment and optimal surgical approach. MATERIAL AND METHODS: An analysis included 72 manuscripts devoted to epidemiology, pathophysiology, clinical symptoms and results of surgical treatment of brain cysts. Case reports, series of cases, reviews and original studies were analyzed. RESULTS: Septum pellucidum cavity is always formed throughout an embryogenesis and persists in 20.34% of adults. Cavum vergae is observed in 2.32% of adults. Cyst of septum pellucidum is detected in 0.04% of adults. Analysis of 368 cases of cysts of septum pellucidum, cavum vergae and cavum veli interpositi has shown that clinical picture consists of headache (50% of cases), convulsive syndrome (23.6%), reduced intelligence (20.1%), behavioural disorders (15.8%), dizziness, nausea and vomiting (10.9%). Hydrocephalus occurs in 16.6% of cases. Endoscopic wall fenestration is preferred for cyst management. CONCLUSION: Brain cysts are rare and characterized by non-specific clinical manifestations. Symptomatic cyst is an indication for surgical treatment. Surgical treatment usually ensures regression of symptoms and low risk of complications.

Cavum Septum Pellucidum Causing Obstructive Hydrocephalus in a Toddler.

Cavum septi pellucidi (CSP) are benign developmental cystic midline cavities that are located between the lateral ventricles through the foramina of Monro. CSP are usually asymptomatic and have no cerebrospinal fluid (CSF) flow. Although their incidence is increased in association with head trauma, as well as psychiatric and behavioural disorders, this increase seldom causes disease. Herein, we discuss the case of a toddler who presented with episodic headaches for 6 weeks with associated vomiting triggered by strenuous activity. His neurological examination was normal. His MRI brain scan revealed large cavum septum pellucidum with obstructive hydrocephalus. He underwent endoscopic fenestration of the cyst with resolution of both hydrocephalus and the symptoms. Although CSP are generally asymptomatic, in rare situations, as in our illustrative case, they may cause obstructive hydrocephalus that requires urgent attention. CSF flow studies are helpful in confirming the diagnosis.

An Analysis of Outcome of Endoscopic Fenestration of Cavum Septum Pellucidum Cyst - More Grey than Black and White?

BACKGROUND: Cavum septum pellucidum (CSP) and cavum vergae are actually fluid-filled, generally communicating midline cavities located between the third ventricle and corpus callosum. There have been various reports of their association with many behavioral and psychiatric disorders. Infrequently, they have been associated with an obstructive hydrocephalus-like picture. Although the structure and management of CSP has long been known, it has been an enigma as far as functional significance and management indications are concerned. The authors of this article try to analyze the significance of a persistent cavum and involvement of the same entity in varied presentations ranging from an incidental imaging finding to acute hydrocephalus, and propose a possible implication on the present surgical intervention paradigm. PURPOSE: To assess the surgical outcome of fenestration of a CSP cyst. METHODS: Retrospective analysis of 3 patients who underwent endoscopic fenestration for CSP with obstructive hydrocephalus between 2012 and 2014 was done, and data were analyzed for symptomatic clinical improvement in particular behavior. RESULTS: Pre- and postoperative brain MRI showed a significant decrease in the size of the cyst as well as the ventricles. There were no recurrences during follow-up. All of the patients improved. CONCLUSIONS: (1) Endoscopic fenestration of symptomatic CSP cysts is a safe treatment option. (2) Neurocognitive assessment is essential in the evaluation and outcome assessment of CSP.

Cavum septi pellucidi cysts: a survey about clinical indications and surgical management strategies.

INTRODUCTION: Cavum septi pellucidi (CSP) cysts have a very low incidence (0.04%). Symptomatic patients usually present aspecific symptoms. For this reason, the management of these patients is still debated. MATERIALS AND METHODS: We selected the case of a ten year old patient, with a clinical history of frontal morning headaches and difficulty in concentration. Brain MRI documented a septum pellucidum cyst and a moderate biventricular dilation. We submitted the case, and a questionnaire concerning indications to surgery and management options to an international group of 54 pediatric neurosurgeons, analyzing the results and comparing them with the current literature. RESULTS: The majority of the participants (50%) indicated as appropriate at the early stage only a clinical observation. In case of persistence of clinical symptoms, 58% opted for intracranial pressure (ICP) monitoring, which, if raised, was considered by 91% as an adequate indication to proceed with surgical treatment. A total of 98% of the participants indicated endoscopic fenestration of the cyst as the preferred surgical strategy. CONCLUSIONS: The management of symptomatic patients with CSP cyst is controversial. Our results suggest that in most of the patients with aspecific symptoms, clinical observation and eventually ICP monitoring are adequate to identify patients for surgery.

Adult idiopatic occlusion of Monro foramina: intraoperative endoscopic reinterpretation of radiological data and review of the literature.

Adult idiopathic occlusion of the foramen of Monro (AIOFM) is a rare condition, with only few cases described in the modern literature. We propose that AIOFM may result from unilateral or bilateral occlusion of Monro foramina, as well as from progression of a monolateral hydrocephalus. Different surgical strategies may be required for effective treatment according to the type of occlusion. To date, only 12 cases of AIOFM have been reported in the literature. We report the cases of two patients, aged 20 and 47 years respectively, who presented with intracranial hypertension secondary to bilateral ventricular dilatation due to obstruction at the level of the foramen of Monro. Both patients were successfully treated with endoscopic fenestration of the primarily obstructed foramen of Monro and, in one patient, fenestration of the septum. We propose that septum pellucidum displacement could play a role in the occlusion of the second foramen of Monro. AIOFM can, therefore, result also from unilateral stenosis of Monro. The difference in AIOFM (i.e. unilateral vs bilateral) will be useful in guiding the most suitable surgical approach in this rare condition.

Operative nuances of excision of colloid cysts in septum pellucidum: a report of three cases.

Colloid cysts are common cysts are often located in the anterior third ventricle and septum pellucidum location is extremely rare. Cysts in septum pellucidum can be missed at surgery because of their unusual location. We describe three patients with colloid cysts in the septum pellucidum, with two in the cavum septum pellucidum. Various surgical implications of this unusual location are enumerated.

Spontaneous Regression of a Symptomatic Cyst of the Cavum Septi Pellucidi and Vergae.

Spontaneous regression of cysts of the cavum septi pellucidi (CSP) and cavum vergae (CV) is rare and little discussed. The authors present their case report of this phenomenon following a severe headache in a 23-year-old woman, in whom magnetic resonance imaging (MRI) had previously confirmed significant thinning of the left lateral cyst wall. We consider this finding to be a possible predisposing factor to rupture and the spontaneous regression of such cysts. In addition to the mechanism of cyst regression, the interrelated causes of their expansion and formation will be discussed.

Lymphoma of the central nervous system originating from the septum pellucidum region: Two case reports with literature review.

RATIONALE: Non-Hodgkin lymphoma affecting the brain, eyes, and cerebrospinal fluid without systemic spread is known as primary central nervous system lymphoma (PCNSL). While intracerebroventricular PCNSL is commonly found in the lateral ventricles and the third and fourth ventricles, the occurrence of PCNSL originating from the septum pellucidum is extremely rare. PATIENT CONCERNS: Two patients presented with recent memory loss and high cranial pressure. DIAGNOSES: Magnetic resonance imaging revealed a clear enhancing lesion in the septum pellucidum region. Pathological examination confirmed that both cases were primary large B-cell lymphoma GCB (germinal center B-cell-like) subtypes located in an "immune-privileged" area. INTERVENTIONS: Both patients underwent total tumor resection, and the procedures were successfully completed without surgical complications. OUTCOMES: Over a 1-year period, treatment included four cycles of high-dose methotrexate combined with temozolomide. During the follow-up period (19-23 months), no recurrence of the lymphoma was observed. LESSONS: In cases of PCNSL in the septum pellucidum, it is crucial to consider it as a potential differential diagnosis for intraventricular tumors. Surgical interventions should focus on maximizing tumor resection while ensuring the protection of critical structures like the fornix and peripheral neural components. The role of surgery compared to biopsy, as well as the long-term complications, necessitates extended follow-up. Additionally, an individualized treatment approach, considering factors such as age, Karnofsky performance score, and organ function assessment, can lead to positive outcomes.

Rosette-forming glioneuronal tumor of the septum pellucidum with extension to the supratentorial ventricles: rare case with genetic analysis.

In the original description, rosette-forming glioneuronal tumors (RGNTs) were restricted to the fourth ventricle and/or posterior fossa. Here, we first report an unusual case of RGNT centered in the septum pellucidum and associated with multiple masses occupying the wall of the bilateral lateral ventricles and the third ventricle. No mass was found in the fourth ventricle. Histological and immunohistochemical examination revealed that the tumor presented biphasic differentiation characterized by predominantly neurocytic rosettes and pilocytic astrocytoma-like components with obvious microvascular proliferation. Chromosome 1p/19q deletions and isocitrate dehydrogenase 1 and 2 (IDH1/2) mutations were not identified. Because this case exhibited a worrisome growth pattern, further studies and long-term follow-up are needed to determine the true nature of these tumors.

Cavum septum pellucidum cyst in children: a case-based update.

BACKGROUND: Cavum septum pellucidum (CSP) cysts are rare lesions which are frequently asymptomatic. Some clinical findings may be associated with CSP cysts, such as headache and other symptoms of increased intracranial pressure, neurological deficit, and mental status changes. There is still controversy in the management of symptomatic cases, especially in children. The main difficulty is to establish a correlation between symptoms and the cyst. When indicated, the treatment is essentially surgical, and the ideal operative technique is also a matter of debate. CASE REPORT: We present a case of a 14-year-old boy with a symptomatic CSP cyst who was successfully treated by neuronavigation-assisted neuroendoscopy with a bilateral fenestration. A literature review is provided with regard to clinical presentation, treatment, and outcome in children. CONCLUSION: The treatment is considered whenever there is an association of a CSP cyst on imaging studies and symptoms attributable to the obstruction of the cerebrospinal fluid flow or direct compression of surrounding structures by the cyst. Endoscopic fenestration is a less invasive and highly effective technique, and is currently the treatment of choice for such lesions in children.

Spontaneous regression of septum pellucidum/forniceal pilocytic astrocytomas--possible role of Cannabis inhalation.

INTRODUCTION: Spontaneous regression of pilocytic astrocytoma after incomplete resection is well recognized, especially for cerebellar and optic pathway tumors, and tumors associated with Neurofibromatosis type-1 (NF1). The purpose of this report is to document spontaneous regression of pilocytic astrocytomas of the septum pellucidum and to discuss the possible role of cannabis in promoting regression. CASE REPORT: We report two children with septum pellucidum/forniceal pilocytic astrocytoma (PA) tumors in the absence of NF-1, who underwent craniotomy and subtotal excision, leaving behind a small residual in each case. During Magnetic Resonance Imaging (MRI) surveillance in the first three years, one case was dormant and the other showed slight increase in size, followed by clear regression of both residual tumors over the following 3-year period. Neither patient received any conventional adjuvant treatment. The tumors regressed over the same period of time that cannabis was consumed via inhalation, raising the possibility that the cannabis played a role in the tumor regression. CONCLUSION: We advise caution against instituting adjuvant therapy or further aggressive surgery for small residual PAs, especially in eloquent locations, even if there appears to be slight progression, since regression may occur later. Further research may be appropriate to elucidate the increasingly recognized effect of cannabis/cannabinoids on gliomas.

Neuroendoscopic treatment of symptomatic cyst of the septum pellucidum in children: A case series.

BACKGROUND: Symptomatic cysts of the septum pellucidum (CSP) are extremely rare in children and surgical indications are not well defined. A very careful clinical and neuroradiologic evaluation is necessary to consider a patient for surgical indication. METHODS: We present a surgical series of 7 pediatric patients. Clinical and radiological features of the patients, including clinical presentation, previous treatment, pre, and post-operative MRI, immediate postoperative, neuropsychiatric assessment, and outcomes were reviewed. RESULTS: There were 5 males and 2 females (mean age 8 yrs). Five patients presented a history of severe intermittent headaches, two of them were admitted with acute symptoms of raised intracranial pressure. One patient presented Epilepsy and ADHD and one patient had severe psychosis. Overall, psychiatric disorders were diagnosed in six patients, three patients had Intellectual Disability (ID). In all cases, the cyst presented a ballooning feature, with a mean volume of 18,36 cm3 (range 10,62-28,5) and significant lateral bulging of both layers. All were operated on endoscopically without complications. After surgery, a very significant decrease in cyst volume was observed (mean volume 5,68 cm3; range 3,18-10,1) with complete disappearance of the ballooning aspect. Headaches resolved in all patients. In two patients operated in emergency papilloedema and vision improved in the first week after surgery. No recurrence of the cysts was noted during follow-up in all patients. CONCLUSIONS: CSP may be associated with behavioral or psychiatric problems also in children. Neuroendoscopic surgery is a safe and effective therapeutic modality to treat CSP presenting with symptoms and signs of intracranial hypertension with good clinical results.

The importance of staged surgery for giant atypical central neurocytoma.

The aim of this article is to discuss the importance of staged surgeries when approaching atypical central neurocytoma in children. Also, we show the preoperative embolization of the lesion as a maneuver to reduce the intraoperative bleeding. Central neurocytomas represent less than 0.5% of all intracranial tumors, and atypical central neurocytomas usually have unfavorable outcome, with high recurrence rate. The intraventricular location is frequent, with a predilection for the lateral ventricles. When completely resected, these lesions have a good prognosis. We report a case of a 12-year old male patient that presented with a history of headache for about 6 months, which worsened for 1 week prior to admission. Magnetic resonance imaging (MRI) brain showed a massive lesion occupying both lateral ventricles. He underwent a microsurgical treatment of a highly vascularized lesion, but the perioperative bleeding required interruption of the surgery. Thus, a preoperative embolization was able to occlude most arterial feeders and allowed subtotal resection in a second surgery. The patient had complete neurological recovery despite immediate post-operative deficits, and the histopathology was suggestive of atypical neurocytoma. Two-stage surgery with preoperative adjuvant embolization is a feasible strategy for treatment of large central neurocytomas in children.

Third Ventricle Cavernous Malformation and Obstructive Hydrocephalus Thought to Be a Colloid Cyst.

BACKGROUND: Third ventricle cavernous malformations (CMs) associated with ventriculomegaly and obstructive hydrocephalus are quite rare in patients. Preoperative surgical planning can be challenging due to the lesion's non-specific appearance on CT and magnetic resonance imaging that can mimic other intraventricular pathologies, such as a colloid cyst. Management of these lesions can be varied in the setting of obstructive hydrocephalus. CASE DESCRIPTION: The patient is a 78-year-old woman who first presented to her primary care provider with balance difficulties and inability to ambulate on her own. She also had bladder incontinence and progressive, severe headaches. Imaging of the brain demonstrated entrapment of the right lateral ventricle and obstructive hydrocephalus due to a lesion in the third ventricle obstructing the right foramen of Monro, thought to be a colloid cyst. A right frontal neuroendoscopic approach with direct visualization, however, confirmed a third ventricle CM. A septal pellucidum fenestration was performed to restore cerebrospinal fluid communication and no resection of the lesion was performed. The patient recovered well after the operation and at clinical follow-up reported no headaches and was walking well without a walker and with no neurological deficits. CONCLUSIONS: A third ventricle CM was discovered after a neuroendoscopic approach for resection of a presumed colloid cyst in a patient with obstructive hydrocephalus. A neuroendoscopic septostomy was performed to treat the obstructive hydrocephalus and no resection was attempted. The patient suffered no complications and is at her neurologic baseline with no deficits.