Cavum septum pellucidum and vergae cyst: A symptomatic case with intracranial hypertension and multiple nerve involvement

Ferreira, Domênica Baroni Coelho de Oliveira; Medeiros, Juliana Weiler; Cwajg, Eduardo; Ferreira-Pinto, Pedro Henrique Costa; Carvalho, Leandro Ururahy de; Nigri, Flavio

doi:10.25259/sni_822_2022

Cited by 5 publications

(4 citation statements)

References 12 publications

(25 reference statements)

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“…Under such circumstances, urgent neurosurgical intervention is necessary to restore CSF equilibrium. Common approaches described in the literature include endoscopic fenestration, shunting, open surgery, and stereotactic fenestration [ 1 , 12 , 13 ]. A recent systematic review reports that surgical treatment provides a resolution of symptoms for most patients, regardless of the choice of procedure [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Cerebral X-linked Adrenoleukodystrophy Presenting As Enlarging Cavum Vergae Cyst: A Case Report

Cheong¹,

Seow²,

Tan³

et al. 2023

Cureus

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The cavum vergae cyst (CVC) is an uncommon brain malformation. Most patients with CVC are asymptomatic and do not require neurosurgical intervention. Separately, cerebral X-linked adrenoleukodystrophy (X-ALD) is one of the phenotypes of a genetic peroxisomal disorder that is seldom managed by neurosurgeons. We report an unusual case of cerebral X-ALD presenting as an enlarging CVC in a child, and discuss its nuances in corroboration with the literature. A previously well six-year-old male presented with confusion and fever. Urgent neuroimaging demonstrated a large CVC with resultant hydrocephalus. Of note, there were symmetrical areas of signal changes in the periventricular white matter bilaterally involving the corpus callosum, thalami, cerebral peduncles, midbrain, and pons in his MRI. Further investigations performed as part of his medical workup reported high plasma concentrations of very long-chain fatty acids (VLCFA). Put together, a diagnosis of cerebral X-ALD was confirmed. Initially, an external ventricular drain was inserted directly into the CVC under stereotaxy to decompress it. Subsequently, endoscopic fenestration of the CVC was performed as the definitive treatment. He recovered uneventfully from the neurosurgical interventions and proceeded for the treatment of his cerebral X-ALD. To our knowledge, this is the first report of cerebral X-ALD presenting as a CVC in a patient. This case adds to the limited literature for both rare conditions and highlights the importance of a multidisciplinary approach to management.

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Section: Discussionmentioning

confidence: 99%

Cerebral X-linked Adrenoleukodystrophy Presenting As Enlarging Cavum Vergae Cyst: A Case Report

Cheong¹,

Seow²,

Tan³

et al. 2023

Cureus

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“…However, if no symptoms are associated with this finding, it is considered an anatomical variation (21). A large CSP cyst obstructing the foramen of Monro has been reported as causing symptomatic obstructive hydrocephalus (22).Furthermore, larger CSP cysts can be linked to acute headaches and multiple cranial nerve palsies (23). A meta-analysis conducted by Wang et al found that individuals with mental disorders had a significantly higher occurrence of CSP of any size compared to healthy comparison subjects.…”

Section: Pathological Associationsmentioning

confidence: 99%

Radiological Anatomy, Variations, and Clinical Significance of Septum Pellucidum: A Mini-Review

Siriwardana,

Pathirage

2023

Preprint

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The septum pellucidum is a thin, translucent double-membrane structure located between the anterior horns of the lateral ventricles. This mini-review explores the gross and radiological anatomy, variations, and clinical significance of the septum pellucidum. The article elaborates on the different types of variations, including cavum septum pellucidum (CSP), cavum vergae (CV), and cavum veli interpositi (CVI). The CSP is a fluid-filled pocket located within the two leaflets of the SP. It forms during fetal development and gradually obliterates before birth. However, the fusion may be prolonged or persist into adulthood in some individuals. The CV is formed when the two commissural plates fail to fuse completely during foetal development. When associated with CSP, the CV is collectively known as the cavum septum pellucidum et vergae (CSPV). However, the isolated CV is rare. The CVI is a CSF-filled space between two tela choroidea layers and communicates with the quadrigeminal cistern, superior to the pineal gland. This article discusses the radiological features of these variations using imaging techniques such as ultrasound, magnetic resonance imaging (MRI), and computed tomography (CT). Ultrasonography is particularly useful for evaluating the SP in fetuses and infants, while MRI and CT scans provide detailed visualisation of the SP and its variations. The SP has the potential to be associated with cerebral anomalies, hydrocephalus, mental disorders, and neurological symptoms. Knowing the related anatomy and detecting variations using imaging modalities are crucial for accurately diagnosing and managing these conditions.

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“…The cavum SP (CSP) and cavum vergae (CV) are enduring membrane structures within the adult brain that arise due to the incomplete closure of the membranous leaves of the septum pylori. CV refers to the posterior extension of the SP [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

“…The majority of these cysts do not exhibit symptoms, although there are rare cases where they may manifest clinical symptoms. The most prevalent symptom is headache, although these lesions can present with a wide range of symptoms such as neurological deficit, ataxia, seizures, syncope, and visual and sensory irregularities [ 1 - 3 ].…”

Section: Introductionmentioning

confidence: 99%

Symptomatic Cavum Vergae Cyst in a Geriatric Patient: A Report of a Rare Case and Conservative Management Approach

Penchev,

Petrov,

Velchev

et al. 2024

Cureus

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Cavum vergae (CV) cysts constitute a small proportion of intracranial cysts, and although generally asymptomatic, there are occasional cases where they might exhibit clinical manifestations. We present a clinical case of a 79-year-old female patient who had a clinical manifestation of headache on the occipital side of the head with irradiation to the shoulder girdle as well as numbness, dizziness, visual impairment, sleep disturbances, and tingling in the hands for three months. Vertigo and rightward staggering had been experienced for two weeks. On physical examination, it was discovered that there was smoothed physiological lordosis, restricted and painful movements, and paravertebral muscle rigidity in the cervical region. The patient had bilaterally reduced biceps and triceps reflexes, painful Erb’s points, and hypesthesia over the C5 and C6 dermatomes on the right side. The patient had decreased coordination and displayed staggered movement to the right. A CT scan discovered dilated subarachnoid spaces of the convexity and a CV cyst. The patient was prescribed conservative therapy consisting of etoricoxib oral at a dosage of 2 × 60 mg for seven days, tolperisone hydrochloride orally at a dosage of 2 × 150 mg for seven days, pregabalin 75 mg, one pill in the evening for seven days, ozoid (a gel containing ozone) for external application, and vinpocetine 2 × 10 mg orally for two months. Following the conservative treatment, the patient exhibited improvement in her symptoms and no longer had challenges carrying out her daily tasks. Furthermore, six months after the therapy, the patient did not experience any symptoms. Long-term follow-up will be conducted in cases of symptom recurrence or cyst enlargement.

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Cavum septum pellucidum and vergae cyst: A symptomatic case with intracranial hypertension and multiple nerve involvement

Cited by 5 publications

References 12 publications

Cerebral X-linked Adrenoleukodystrophy Presenting As Enlarging Cavum Vergae Cyst: A Case Report

Cerebral X-linked Adrenoleukodystrophy Presenting As Enlarging Cavum Vergae Cyst: A Case Report

Radiological Anatomy, Variations, and Clinical Significance of Septum Pellucidum: A Mini-Review

Symptomatic Cavum Vergae Cyst in a Geriatric Patient: A Report of a Rare Case and Conservative Management Approach

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