Intraventricular cavernomas (IVCs) are rare, and only occur in 2.8 to 10% of patients with cerebral cavernous malformations.
Reports concerning IVC are scarce and are limited mostly to sporadic case reports.
Till 2017, among 136 IVC cases, the mean age of the patients was 36.5 years; the male-to-female ratio was 0.8. The most frequent location was the lateral ventricle (52.6%), and most of the clinical symptoms (74%) were related to mass effects on adjacent brain tissues. Intraventricular hemorrhage occurred in 22.9% of cases 1).
Till 2003, only 10 pediatric cases out of 46 well-documented cases have been published 2).
In a review of literature by Reyns et al., of the 45 cases reported in literature, 44% of intraventricular cavernomas were found to be located in the third ventricle, 27% in the lateral ventricle, 20% in the trigone, and 9% in the fourth ventricle 3).
Third ventricle cavernous malformation
Foramen of Monro
Trigone cavernous malformation
Of intraventricular CM, only about 20% are located in the trigone of the lateral ventricle 4).
The first report of trigonal CM was published in 1977 by Coin 5).
Ohbuchi et al., conducted a PubMed search for trigonal CM and found 17 cases 6).
Although IVCs share some common characteristics with intraparenchymal cavernomas, they also have some distinct features involving structure, clinical symptoms, radiologic appearance, and onset of symptoms 7).
The important differentials of the lesions occurring at the foramen of Monro include colloid cyst, central neurocytoma, giant cell astrocytoma, and oligodendroglioma. Cystic and hemorrhagic metastases, however uncommon, may also occur at the foramen of Monro 8).
Colloid cysts are oval-to-round lesions. Approximately two thirds are homogeneously hyperdense compared to brain on plain scans and one-third of them are isodense. On MRI, the signal characteristics of colloid cysts vary. Most commonly, these appear hyperintense on T1WI and hypointense on T2WI. Central neurocytomas are inhomogeneous, partially calcified, and mildly enhancing lateral ventricular masses. On MRI, these are seen to be inhomogeneously isointense on T1WI with variable appearance on T2WI. Giant cell astrocytomas are seen in association with tuberous sclerosis and appear iso-to- hypodense on CT scans with focal areas of calcification and cyst formation. These demonstrate hypo- to isointense signal on T1WI and are iso- to hyperintense on T2WI. Oligodendrogliomas most commonly show dense areas of calcification. These appear hypo- to isointense on T1WI and hyperintense on T2WI, showing moderate patchy enhancement 9).
Jin et al., report a case of trigonal cavernous malformation (CM) radiologically mimicking meningioma. The computed tomographic (CT) head angiography and magnetic resonance imaging (MRI) showed a partially calcified lesion with slight contrast enhancement located in the area of the left atrium of lateral ventricle. The lesion was completely removed using microsurgery with a parieto-occipital transcortical approach. The resected mass was histologically confirmed as CM. CM should be considered as differential diagnosis in case of the atrial mass lesion due to lack of hemosiderin ring characteristically seen other seated CM 10).
Dey et al., present the first report of an intraventricular encapsulated hematoma, mimicking cavernous malformation on imaging and gross lesion appearance. A 47-year-old female on anticoagulation therapy for atrial fibrillation presented with left upper extremity apraxia, neglect, and mild gait imbalance. Her brain CT scan and MRI revealed multilobulated lesions involving the choroid plexus in the atria of both lateral ventricles. The intraoperative appearance was that of encapsulated mass, with blood clots at different stages of liquefaction and organization, all consistent with the gross appearance of a cavernous malformation. However, histopathologic examination demonstrated hematoma with components at different ages, and normal vessel infiltration without any hint of cavernous malformation histology, or underlying neoplasia. Encapsulated hematoma should be considered in the differential diagnosis of hemorrhagic intraventricular masses 11).
Most of the articles concluded that complete surgical resection is the treatment of choice.The microsurgical approach is currently considered the gold standard for IVC resection 12).
As neuroendoscopy evolves, we see that lesions considered impossible to be managed by neuroendoscopy before today are being treated with the advantages that the minimally invasive surgery offers 13).
However, tumor size and vascular nature are considered restrictive factors.
Till 2013, there are two cases in the literature of intraventricular cavernoma resection done by neuroendoscopy.
Giannetti describe a case of a man who presented with hydrocephalus secondary to a mesencephalic cavernoma and a second cavernous angioma located at the dorsum thalamus. This second lesion was successfully removed using a pure neuroendoscopic technique at the same time as a third ventriculostomy was performed 14).
Using the neuroendoscope and neuronavigational guidance and based on the biological characteristics of the IVC, proper lesion size, and dilated ventricles, Shirvani et al., totally resected the lesion in all his three cases. Neuroendoscopy can be considered as an alternative to microsurgery of IVCs. However, Shirvani et al., believe a larger series of cases is necessary to demonstrate when microsurgery and when neuroendoscopy should be performed for IVC resection 15).
Stavrinou et al., performed a search of the literature of the last 30 years and identified all cases of intraventricular cavernous angiomas. Trigonal cavernomas were separately identified and analysed. Our search yielded a total of 13 trigonal cavernomas.
A total of 61 intraventricular cases, 13 were located in the trigone of the lateral ventricles. The most prominent presenting symptom was intracranial hypertension (68.9%), followed by seizures (18.2%) and hemorrhage (13.1%).The literature review revealed a trend of intraventricular cavernomas to present with intracranial hypertension rather than seizures or focal neurologic deficit, unlike their intraparenchymal counterparts. We feel that this difference has received little attention in the international literature. We discuss a possible pathogenetic mechanism for the presence of intracranial hypertension and address different aspects of diagnosis and treatment of this benign lesion.
Trigonal cavernomas are benign lesions that have an excellent outcome after radical excision. Symptoms and signs of intracranial hypertension and hydrocephalus may be the prominent initial presentation of this rare ailment. 16).
Faropoulos et al., present there experience, consisting of five IVC cases over a period of 11 years. They describe the symptoms leading to hospital admission, the main radiologic findings, the management of each ICV case, and the patients’ clinical status after surgery. They also reviewed the international literature on IVC, presenting the main demographic characteristics, their most common location in the ventricular system, and the main signs and symptoms. Finally, They present the management options according to the current literature, the advantages and disadvantages of every management option, accompanied by a brief follow-up of most IVC cases, whether the cavernoma was treated surgically or conservatively 17).
Kivelev et al., present a series of 12 patients with IVCs that were treated at a single neurosurgical department. In addition, the authors reviewed the literature.
All clinical data were analyzed retrospectively. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale. The authors also conducted a PubMed search and found 77 cases of IVC.
The patients’ median age was 47 years, and the male/female ratio was 2:1. A cavernoma occurred in the lateral ventricle in 6 patients, in another 5 it was in the fourth ventricle, and 1 had a lesion in the third ventricle. Almost all patients presented with acute headache on admission and in more than half, the symptoms were related to cavernoma bleeding. In total, 8 rebleedings occurred in 5 patients during a median of 0.4 years. Three patients with a cavernoma of the fourth ventricle presented with a cranial nerve deficit. In 8 cases, a cavernoma was surgically treated an average of 1.3 years after the diagnosis. Only 1 patient underwent surgery in the acute phase after a major intraventricular/intracerebral hemorrhage. The median follow-up time was 2 years. No patient was lost to follow-up, and no patient died. In total, on follow-up 9 patients improved and 3 had a persistent neurological deficit, of which 2 existed before surgery.
In the present series, the IVCs had a high tendency for rehemorrhage. Surgery is advocated when hemorrhages are frequent, and the mass effect causes progressive neurological deficits. Microsurgical removal of the IVC is safe, but in the fourth ventricle it can carry increased risk for cranial nerve deficits 18).
The clinical and radiographic presentations of 3 patients with intraventricular cavernous hemangioma are described. The accumulated total of 19 cases from the literature are compared to determine whether there is a common clinical and radiographic presentation for this benign intraventricular lesion. The differential diagnosis of intraventricular cavernous hemangioma includes intraventricular meningioma, choroid plexus papilloma, arteriovenous malformation, low grade astrocytoma, and ependymoma 19).
Shirvani et al., describe three IVC cases and briefly review previously documented IVC cases in PubMed. Among 136 IVC cases, the mean age of the patients was 36.5 years; the male-to-female ratio was 0.8. The most frequent location was the lateral ventricle (52.6%), and most of the clinical symptoms (74%) were related to mass effects on adjacent brain tissues. Intraventricular hemorrhage occurred in 22.9% of cases. Most of the articles concluded that complete surgical resection is the treatment of choice.The microsurgical approach is currently considered the gold standard for IVC resection. Using the neuroendoscope and neuronavigational guidance and based on the biological characteristics of the IVC, proper lesion size, and dilated ventricles, we totally resected the lesion in all three cases. Neuroendoscopy can be considered as an alternative to microsurgery of IVCs. However, we believe a larger series of cases is necessary to demonstrate when microsurgery and when neuroendoscopy should be performed for IVC resection 20).
A 64-year-old woman who was evaluated after being found unresponsive. Imaging revealed a foramen of Monro cavernoma resulting in hydrocephalus. Supratentorial cavernomas are most frequently found in the cerebral cortex, and although ventricular cavernomas do occur, they are rarely located in the foramen of Monro. Foramen of Monro cavernomas are extremely dangerous, requiring aggressive management when identified 21).
Bhatia et al., present a case of cavernous hemangioma located at foramen of Monro, with its radiopathological confirmation 22).
A case of trigonal cavernous malformation (CM) with intraventricular hemorrhage. This 67-year-old woman experienced sudden onset of loss of consciousness and her Glasgow Coma Scale (GCS) was 5 points (E1V1M3) on admission. CT scan demonstrated intraventricular hemorrhage and acute hydrocephalus. Angiography did not demonstrate any vascular abnormality. Ventricular drainage was performed for acute hydrocephalus and the postoperative course was good. CT showed a hyperdense lesion in the left trigone, which was contrast-enhanced on T1-weighted MR. Removal of CM was performed via the left middle temporal sulcus 23).
Intraventricular cavernous malformation with superficial siderosis 24).
A case of intraventricular cavernoma in the region of the foramen of Monro with the aim of illustrating the difficulties involved in the diagnosis of this rare lesion 25).
Jin et al., report a case of trigonal cavernous malformation (CM) radiologically mimicking meningioma. The computed tomographic (CT) head angiography and magnetic resonance imaging (MRI) showed a partially calcified lesion with slight contrast enhancement located in the area of the left atrium of lateral ventricle. The lesion was completely removed using microsurgery with a parieto-occipital transcortical approach. The resected mass was histologically confirmed as CM. CM should be considered as differential diagnosis in case of the atrial mass lesion due to lack of hemosiderin ring characteristically seen other seated CM 26).
Muccio et al., report the MRI findings and histological features of an uncommon case of a single giant (maximum diameter: >6 cm) cystic CCM of the left lateral ventricle occurring in a 26-year-old man who had undergone 30 Gy cranial irradiation for acute leukemia at the age of six years. Large cystic CCMs must be included in the neuroradiological differential diagnosis of intraventricular hemorrhagic cystic lesions 27).
A 56 years old patient was admitted with progressive and intractable headache of 10 days of evolution. He was known to suffer familial multiple cavernomatosis. Magnetic resonance imaging (MRI), revealed obstructive hydrocephalus due to a cavernoma located in the area of the left foramen of Monro. Under neuronavigation guidance, complete endoscopic resection of the cavernoma was performed and normal ventricular size achieved. The patient experienced transient recent memory loss that resolved within a month after surgery. In the literature attempted endoscopic resection is reported to be abandoned due to bleeding and ineffectiveness of piecemeal endoscopic resection. In this case, the multiplicity of the lesions made it advisable to resect the lesion endoscopically, to avoid an open procedure in a patient with multiple potentially surgical lesions. Endoscopic resection was uneventful with easy control of bleeding with irrigation, suction, and bipolar coagulation despite dense vascular appearance of the lesion. During the procedure, precise visualization of the vascular structures around the foramen of Monro allowed complete resection with satisfactory control of the instruments. To the best of the authors’ knowledge, this is the first published cavernoma of foramen of Monro successfully resected using an endoscopic approach 28).
A 25-year-old male patient presented with a predominantly intralesional haemorrhage. Neuroimaging led to an accurate preoperative diagnosis although the typical low intensity perilesional ring of gliosis and hemosiderin was not present. The lesion was microsurgically removed using an stereotactically guided posterior temporal transsulcal approach 29).
A eight-year old boy with a rare third ventricular cavernous angioma that hemorrhaged presenting with symptoms of acute hydrocephalus. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) showed a heterogenous ill-defined, solid and cystic intraventricular mass in the third ventricle which was mildly enhanced with contrast and there was associated hydrocephalus. The mass was removed with success and follow up after two years revealed no neurological abnormalities 30).
Longatti et al., report on a patient who presented with an intraventricular mass located at the level of the foramen of Monro. The clinical presentation and neuroimaging appearance of the mass led to an initial diagnosis of colloid cyst. A neuroendoscopic approach offered a direct view of the ventricular lesion, which was found to be a cavernous angioma partially occluding the foramen of Monro. The lesion was then removed using microsurgery. In this report the authors highlight possible pitfalls in the diagnosis of some lesions of the third ventricle, and the possible advantages of using a combined endoscopic and microsurgical technique when approaching such lesions 31).
Three cases of trigonal cavernous angiomas who presented with raised intracranial pressure or seizures and who underwent total excision with a good recovery. We also review the literature and discuss surgical approaches.
On magnetic resonance imaging, intraventricular cavernous angiomas lack the hemosiderin ring characteristically seen around parenchymal cavernous angiomas. This explains why trigonal cavernous angiomas can mimic malignant neoplasm on imaging, and they should be considered in the differential diagnosis of intraventricular masses. Total excision should be the goal of surgery 32).
A 47-year-old woman presented with unilateral ventricular enlargement detected by magnetic resonance imaging during a medical checkup. Neuroendoscopic exploration identified a multilocular lesion in which dark red fluid formed a niveau near the right side of the foramen of Monro. The diagnosis was intraventricular cavernous angioma. Restricted flow of cerebrospinal fluid at the foramen of Monro was observed. Xanthochromia, which seemed to be due to previous bleeding, was observed at the fornix. When the neuroendoscope touched the angioma, the wall collapsed and bled. Endoscopic removal of the angioma was abandoned, and craniotomy and resection of the angioma were performed. No new neurological anomalies were observed after surgery. Preoperative diagnosis of intraventricular cavernous angioma is difficult based on neuroimaging. Neuroendoscopy is effective for diagnosis and the decision-making process regarding treatment 33).
A 51-year-old woman was admitted with obstructive hydrocephalus-related symptoms. The computed tomography (CT) and magnetic resonance imaging (MRI) revealed a partly calcified lesion with slight contrast enhancement located in the area of the right foramen of Monro. The lesion was completely removed by surgical resection with a transfrontal transventricular approach. The resected mass was histologically diagnosed as cavernous hemangioma. The patient’s symptoms resolved immediately after operation. Cavernous hemangioma at the foramen of Monro in the present case had common MRI features as previously reported. Although MRI can provide initial diagnosis for such unusually localized tumor, it should be confirmed histopathologically 34).
Tatsui et al., present two cases with diagnosis made only by histopathologic examination, due to a lack of classic image findings. Cavernous hemangiomas must be included in the differential diagnosis of intraventricular tumors, and total surgical resection is the treatment of choice. Perilesional ring as demonstrated by MRI, must not be expected when dealing with such lesions 35).
A 11-year-old girl operated on for a voluminous cavernoma at the ventricular trigone which was diagnosed after absence seizures.
Interestingly, the case we report showed a hypointense rim on T2-weighted magnetic resonance images which has not been a common finding in the cases previously reported 36).
A clinicopathological review of a giant intraventricular cavernous malformation 37).
Intraventricular cavernoma in the region of the foramen of monro 38).
A 15-years-old female patient, who suffered of mild chronic headache for 8 months, followed by two episodes of sudden intensive headache and stupor with complete recovery after 48 hours. CT scan was performed and revealed a voluminous size, hiperdense mass in the frontal horn of the lateral ventricle. The surgical access to site was through transcallosal interhemisphere approach. The patient had a good recovery without complications.
Although lateral ventricle cavernomas are rare they should be considered in the differential diagnosis of intraventricular tumors. A wrong preoperative diagnosis has sometimes induced a wrong therapy, such as radiotherapy, for these surgically curable benign 39).
A 16-year-old female patient presented with a sudden distal deficit of the left superior limb. She had a voluminous tumor involving the two lateral ventricles, with radiological evidence of recent hemorrhage. A 30-year-old man presented with generalized seizures and a right hemiplegia related to a 4-cm-diameter cavernoma in the two lateral ventricles involving the interhemispheric scissure through the corpus callosum and left centrum ovale. The radiological appearance was not typical and did not allow the diagnosis. A 42-year-old man had a cavernoma in the third ventricle, which was responsible for his short-term memory loss. This cavernoma had been revealed by computed tomography that was performed after intracerebral hemorrhage related to another cavernoma in the right parietal lobe occurred.
Stereotactic biopsies allowed the diagnosis of intraventricular cavernoma in the first case. Surgical removal via a right transcortical transventricular approach and a transcallosal approach in the first and second cases, respectively, was complete, resulting in good outcomes. Surgical removal via a right transcortical transventricular approach in the third case was partial.
Intraventricular cavernomas are so uncommon that only 42 well-documented cases have been previously reported in the literature. It seems that their radiological diagnosis may be difficult because of their uncommon location in the ventricular system and their voluminous size. A wrong preoperative diagnosis has sometimes been the cause of inefficient therapy, such as radiotherapy, for these surgically curable benign lesions 40).
A case of a cavernous haemangioma that appeared as an intraventricular mass at the foramen of Monro. Despite the unusual location the diagnosis was established by MRI because of the typical appearance. The differential diagnosis included primary and secondary neoplasms at the foramen of Monro 41).
Four cases of cavernous malformations of the third ventricle. Patients presented with symptoms of hydrocephalus, memory loss, and signs of hypothalamic dysfunction. Magnetic resonance imaging and computed tomography provided characteristic images of the three lesions preoperatively. All patients underwent direct surgical excision of the malformations. Two patients had a transcallosal, transventricular approach, the third underwent a transcortical, transventricular approach, and the fourth had an infratentorial supracerebellar approach. Postoperatively, the patient with hypothalamic dysfunction has not improved and underwent ventriculoperitoneal shunting. The second patient did well initially; however, 8 days postoperatively, she became comatose and later died. The presumed cause of her deterioration was a hypothalamic venous infarction. The third and fourth patients have returned to their normal neurological baseline. The presenting signs and symptoms, magnetic resonance imaging and computed tomography findings, and treatment options for this rare lesion are discussed and illustrated 42).
A 54-year-old female presented with a cryptic arteriovenous malformation (AVM) of the choroid plexus of the fourth ventricle causing intraventricular hemorrhage. Computed tomography and magnetic resonance imaging disclosed the lesion near the fourth ventricle, but bilateral vertebral angiograms showed no abnormalities. The preoperative diagnosis was cavernous angioma. The mass was removed completely, and histological examination demonstrated an AVM of the choroid plexus. Vascular malformations of the choroid plexus of the fourth ventricle are extremely rare. The possibility of this lesion being the cause of primary intraventricular hemorrhage of unknown origin should always be considered 43).
A case located in the trigone of the lateral ventricle in the dominant hemisphere and drained into the longitudinal caudate vein of Schlesinger via deep medullary veins. By a transsylvian transventricular approach, the CM was totally removed with successful preservation of the medullary venous malformation. This approach is available for trigonal lesions, especially in cases with enlarged inferior horn. They stress that CM removal can be conducted with preservation of the adjacent medullary venous malformation 44).
Since the availability of CT diagnosis 23 cases of intraventricular cavernous angioma (IVCA) have been published in the literature till 1991. Three additional cases have been operated upon in the Neurochirurgische Klinik, Krankenhaus Nordstadt, Hannover, Federal Republic of Germany. Based on these 26 cases the clinical data, radiological findings, treatment and outcome of IVCAs are reviewed 45).
A rare case of cavernous angioma located in the fourth ventricular floor occurred in a 44-year-old female complaining of occipital headache, vomiting, diplopia, and dysarthria. Computed tomographic scans demonstrated a high-density area in the fourth ventricle and slight hydrocephalus. Magnetic resonance (MR) imaging showed a mixed intensity mass on T2-weighted images and high- or isointensity regions on T1-weighted images. The tumor was totally removed and histologically diagnosed as cavernous angioma. Postoperatively, ataxic gait, nausea, and vomiting disappeared gradually. MR imaging was useful to accurately evaluate the anatomic relationship between the lesion and the brainstem 46).
Cavernous angioma arising in the third ventricle is an extremely rare disease. Ogawa et al., reviewed five cases previously reported, as well as they own two cases, and discuss the clinical characteristics of and surgical approach to cavernous angioma at this site. The bifrontal craniotomy and interhemispheric translamina terminalis approach minimize the damage to the brain and allow for an approach to the third ventricle in a wide operative field with minimal compression of the brain itself. They have found this approach to be suitable for surgery on angiomas of the anterior half of the third ventricle 47).