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|Year : 2015 | Volume
| Issue : 1 | Page : 68-71
Cavernoma of the septum pellucidum in the region of foramen of Monro
Dattatraya Muzumdar1, KM Avinash2, Raghvendra Ramdasi2
1 Department of Surgical Oncology, Prince Aly Khan Hospital, Mumbai; Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Parel, Mumbai, Maharashtra, India
2 Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Parel, Mumbai, Maharashtra, India
|Date of Web Publication||4-Mar-2015|
Prof. Dattatraya Muzumdar
Department of Neurosurgery, King Edward VII Memorial Hospital, Parel, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
A rare case of cavernoma in the region of the septum pellucidum is reported. A 35-year-old female patient presented with chronic headaches. Her neurological exam was normal. Her magnetic resonance (MR) imaging showed a lesion within the inferior aspect of the septum pellucidum extending into the anterior third ventricular region, blocking the foramen of Monro, resulting in moderate supratentorial asymmetrical hydrocephalus. A central neurocytoma or subependymoma was suspected on imaging. Complete excision of the septum pellucidum cavernoma was performed using microneurosurgical techniques through an interhemispheric transcallosal route. The patient had an excellent outcome and is cured. Although rare, septum pellucidum cavernomas should be considered in the differential diagnosis of anterior third ventricular lesions in the region of foramen of Monro. The unusual location, atypical radiological features, differential diagnosis as well as surgical nuances in the management of a cavernoma in the septum pellucidum and anterior third ventricular region are discussed in the light of current literature.
Keywords: Cavernoma; septum pellucidum; surgery; ventricle
|How to cite this article:|
Muzumdar D, Avinash K M, Ramdasi R. Cavernoma of the septum pellucidum in the region of foramen of Monro
. Neurol India 2015;63:68-71
| » Introduction|| |
Cavernous angiomas (cavernomas) are vascular malformations found most commonly in the supratentorial subcortical gray matter. They are well-circumscribed, multilobulated, angiographically occult lesions. The supra- to infratentorial ratio is 74-90% to 10-26%. , Intraventricular cavernomas are rare, comprising 2.5% of all intracranial cavernomas.  They are mostly found in lateral ventricles followed by the third and fourth ventricles.  The occurrence of a cavernoma in the septum pellucidum is rare. The unusual location, radiological features, and surgical principles in the management of cavernomas in this rare location are discussed and the relevant literature is reviewed.
| » Case Report|| |
A 35-year-old female patient presented with progressively increasing headaches and giddiness for the past 3 months. There was no history of convulsions, unconsciousness, trauma, or fever. There was no other significant contributory history. Her neurological examination was unremarkable. Magnetic resonance (MR) imaging of the brain showed a midline anterior third ventricular lesion blocking the foramen of Monro resulting in moderate supratentorial, asymmetrical hydrocephalus. The lesion was iso- to hypointense on T1-, and of mixed intensity on T2-weighted images [Figure 1]. Contrast administration showed no enhancement. The lesion was well-defined, arising from the inferior aspect of the septum pellucidum, splaying the septal leaves, and extending in the anterior third ventricular region causing left lateral ventricular dilatation. The differential diagnosis included a subependymoma or a central neurocytoma. A left frontoparietal parasagittal craniotomy was performed. An interhemispheric transcallosal approach was adopted, and the lesion was completely resected using microsurgical techniques. Intraoperatively, the lesion was found straddling the septum pellucidum and had the classical appearance of a vascular lesion filled with dark brown/black blood with a thin capsule suggestive of a cavernoma. The ipsilateral fornix was safeguarded. Patient had an uneventful postoperative course and was relieved of his headache. MR imaging at 5 years showed no recurrence of the lesion [Figure 2]. The histological examination revealed multiple dilated vascular spaces lined by endothelium with no intervening brain parenchyma confirming the diagnosis of a cavernoma [Figure 3]. At a 5-year follow-up, the patient was asymptomatic and having normal memory.
|Figure 1: (a) T1-weighted axial magnetic resonance (MR) imaging showing a heterogeneous iso- to hypointense well-circumscribed lesion attached to the lower septum pellicidum abutting the foramen of Monro leading to left-sided ventricular dilation. (b and c) T2-weighted coronal and sagittal MR imaging showing a well-circumscribed heterogeneous lesion with few flow voids, occupying the region of foramen of Monro with ventricular dilatation. (d) Post gadolinium T1-weighted MR imaging showing heterogeneous enhancement of the lesion|
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|Figure 2: (a) Follow-up T2-weighted coronal magnetic resonance (MR) imaging showing complete excision of the tumor. Asymmetrical ventricular dilation is still seen. Foramen of Monro is completely open. (b) Follow-up post gadolinium T1-weighted MR imaging showing complete excision of the tumor|
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|Figure 3: (a and b) Photomicrograph (H and E x40 and x100) showing many proliferating dilated and congested vessels, few of them showing fresh fibrin thrombi|
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| » Discussion|| |
Cavernomas are intracranial vascular malformations with a benign histology but may precipitate potentially life-threatening complications. They are more commonly located in the supratentorial (74-90%) than in the infratentorial (10-26%) region. , An intraventricular location is rare and accounts for about 2.5% of all cavernomas.  They are commonly found in the lateral ventricle, followed by the third ventricle and the fourth ventricle in order of the occurrence. In the lateral ventricle, the most common location is the atrium.  There are very few reports of cavernomas in the region of the foramen of Monro. A cavernoma arising in the septum pellucidum is rarer, and only three cases have been reported in literature [Table 1]. ,, In the present case, the cavernoma was present in an unusual location and had atypical characteristics on MR imaging. The lesion was arising from the inferior aspect of the septum pellucidum and was extending into the anterior third ventricular region through the foramen of Monro causing moderate supratentorial asymmetric hydrocephalus.
Cavernomas in the supratentorial region present with seizures, headaches, or focal neurological deficits . They may be detected incidentally. The clinical features largely depend on the location of the cavernoma. Seizure is the most common mode of presentation occurring in 39-79% of patients. Seizures usually arise due to recurrent intratumoral hemorrhages. The annual risk of developing a seizure is approximately 1.5%.  About 45% of the patients become intractable to antiepileptic medications.  Intraventricular cavernomas most often also present either with hemorrhage (14%) or with a seizure (14%).  Cavernomas of the septum pellucidum do not have any specific clinical presentation related to its location. They usually present with features of hydrocephalus due to obstruction of the foramen of Monro.  In the literature, 42% of third ventricular cavernomas developed hydrocephalus usually due to mechanical obstruction.  In the present case, the patient presented with headaches and demonstrated asymmetric hydrocephalus on imaging, which corroborates with a lesion in the septum pellucidum extending into the region of the foramen of Monro.
The most common imaging modalities are computed tomography (CT) and MR imaging. Angiography is not useful as these lesions are angiographically occult. Calcifications may be noted on CT scans. MR imaging is the investigation of choice since cavernomas exhibit their characteristic signal intensity.  The MR imaging features of intraventricular cavernomas are similar to that of parenchymal lesions. However, in majority of the cases, the rarity of these lesion in this location precludes a definitive diagnosis. Hence, conclusive MR evidence of its presence is dependent on the timing at which the MR is performed.  The common differential diagnosis includes an intraventricular meningioma, choroid plexus papilloma, arteriovenous malformation, low-grade astrocytoma, subependymoma, and colloid cyst.  Septum pellucidum cavernomas closely resemble hamartomas, cysts, and lipomas.  In the present case, the cavernoma was confined in between the layers of septum pellucidum. There was evidence of multiple small hemorrhages showing blood in various stages. But a characteristic perifocal hemosiderin ring on T2-weighted image that may suggest a cavernoma was conspicuously absent. Unlike parenchymal cavernomas, intraventricular cavernomas may have atypical features. A high index of suspicion should be maintained and a cavernoma should be included in the differential diagnosis of intraventricular lesions.
The management of intraventricular cavernomas is complex and is a surgical challenge. The lack of authentic prospectively analyzed case series and long-term outcome studies is a limitation. Incorrect preoperative radiological diagnosis has sometimes resulted in inappropriate therapy.  The tendency for rapid growth and extralesional hemorrhage of intraventricular cavernomas may suggest the need to treat these lesions more aggressively. Surgery is advocated when recurrent hemorrhages are frequent and the mass-effect causes progressive neurological deficits. 
Complete surgical excision of the cavernoma is the treatment of choice. Asymptomatic incidentally detected lesions are managed conservatively. In a symptomatic patient, the indications for surgical intervention are intractable seizures, multiple hemorrhages, extralesional hemorrhage, single hemorrhage in a non-eloquent area, focal deficits or mass effect. Patients with medically controlled seizures may still be observed. Although neurendoscopy is being increasingly used for the treatment of intraventricular tumors, its potential for use in the excision of intraventricular cavernomas is still unclear. Cavernomas tend to bleed and hemostasis may be difficult to achieve with an endoscope.  Further problems with an endoscope occur due to wall collapse, bleeding, and ineffectiveness of piecemeal resection consequent to firm consistency of the lesion.  The complex anatomy of the region and its intense vascularization, often makes microsurgical approach the only viable option to ensure a safe and complete resection.  Microsurgical excision is thus the standard treatment and a much safer technique than neuroendoscopy. ,
In the present case, the unusual MR imaging characteristics and the location at septum pellucidum were unique. The occurrence of the cavernoma in the septum pellucidum and extending into the foramen of Monro and in close proximity to the fornices was a surgical challenge. Preserving her memory function was imperative. Circumferential excision of the cavernoma was performed within a small corridor of gliotic tissue surrounding the lesion. The part of the lesion extending into the foramen of Monro was meticulously dissected from the fornices to prevent inadvertent injury. A controlled excision of the lesion is of paramount importance in order to avoid spillage of blood into the ventricles to prevent meningism and obstructive hydrocephalus.
| » Conclusion|| |
Septum pellucidum cavernomas are rare and extension into the region of foramen of Monro is uncommon. The clinical presentation may be innocuous and the MR imaging may be atypical. A high index of clinical and radiological suspicion is warranted. They should be considered in the differential diagnosis of lesions in the region of foramen of Monro. Microsurgical excision is the treatment of choice. Complete extirpation of the lesion is possible and should be performed. The long-term outcome is excellent and curative.
| » References|| |
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[Figure 1], [Figure 2], [Figure 3]
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