Cerebellar hemorrhage associated with capillary telangiectasia and venous angioma: A case report

We report a case of recurrent cerebellar hemorrhage from a capillary telangiectasia associated with a venous angioma. Based on the findings in this case and a review of the literature, we conclude that the hemorrhage was from the telangiectasia and not from the associated venous angioma. The association between capillary telangiectasias and cavernous malformations and the established association of the latter with venous angiomas are reviewed. This case illustrates the complex interrelationship between these histologically distinct malformations and its influence on their potential to hemorrhage.     

Venous angiomas: an underestimated cause of intracranial hemorrhage

Cerebral venous angiomas are considered by many investigators as infrequent and incidental. Our experience, however, has been different. Since 1975, we have compiled a group of 21 patients with 23 venous angiomas. Nine patients (43%) presented with intracranial hemorrhage, and two in this group had recurrent hemorrhage. Surgical extirpation of the venous angioma was performed in all nine cases. An additional resection of a frontal venous angioma was performed in a woman with intractable seizures and headache. We experienced limited morbidity and no mortality. Although venous angiomas may be uncommon clinically, they have a significant potential for hemorrhage, and particularly, rehemorrhage if left untreated.     

Sylvian subarachnoid and extra-pial cavernous angioma

Cavernous angiomas are one of the four types of vascular malformations of the central nervous system. Lesions situated subarachnoidally are rare although some cases with an infratentorial localization have been reported. Our case represents an unusual localization of a subarachnoid cavernous angioma. A 35- year-old patient was admitted to our department with signs of subarachnoid hemorrhage. A 1 cm hyperdense lesion placed at the right Sylvian fissure was distinguished by CT-scan examination and no arterial supply was revealed on cerebral angiography. Surgical intervention showed a lesion placed extra-pial and totally encased in the subarachnoid space in the superficial part of the Sylvian cistern. This case represents a radiologically visible supratentorial extra-pial subarachnoidal cavernous angioma. This case provides confirmation of one of the supposed causes of subarachnoid hemorrahage 'sine materia'.     

Cavernous angiomas of the central nervous system

The histopathological, epidemiological, clinical and neuroradiological findings of the cavernous angiomas of the central nervous system have been reviewed, based on an analysis of the literature. C.A. was considered a very rare lesion in the pre-CT era (5% of the vascular malformations). In the past mainly the cavernous angiomas operated on were responsible for a hemorrhagic syndrome or a space-occupying lesion syndrome and less frequently did they present with seizures. Since the introduction of the CT and moreover of MRI the reported cases of cavernous angiomas in the C.N.S. have increased considerably (25.1% of the vascular malformations). Familial cases, multiple localizations, association with tumors and other vascular malformations and extracerebral dural localizations have been documented. Angiographic examination can be negative, but in some cases a capillary blush in the late venous phase has been demonstrated. CT scan and MRI are the best procedures for the diagnosis of cavernous angiomas. MRI is able to demonstrate small and no recent traces of bleeding, but CT is more sensitive in distinguishing calcifications. Surgical results depending on the site of the angioma have been reported. Radical removal and good recovery have been documented in supratentorial cases, while partial removal and poor results have been reported in deeply sited cases (including spinal cord).     

An analysis of the natural history of cavernous angiomas

The advent of magnetic resonance (MR) imaging has permitted the recognition of many angiographically occult vascular malformations before the development of complications and subsequent surgical removal. This study reviews all patients at one institution who had radiographically identifiable vascular malformations believed to represent cavernous angiomas in order to obtain information on the natural history of this particular lesion. All 8131 craniospinal MR images performed at our medical center from January 1, 1986, to November 30, 1989, were reviewed, and 32 patients were identified with 76 lesions meeting the MR imaging criteria for cavernous angioma. Medical histories, physical examination records, and other data from these patients were then reviewed to determine the frequency of complications. Their mean age at latest follow-up examination (or at surgical removal of the lesion) was 37.6 years (range 16 to 72 years). Sixteen patients (50%) had a history of seizures, seven (22%) had focal neurological deficits, and three (9%) had clinically significant hemorrhage attributable to the cavernous angioma; six patients (19%) were asymptomatic. The estimated risk of hemorrhage for this population is 0.25%/person-year of exposure; the estimated risk of seizure development is 1.51%/person-year. Eight patients underwent surgical procedures, resulting in improved seizure control and/or lessened neurological deficit. Although these lesions are often excised with relative ease and minimal morbidity, the potential risks and benefits of surgery must be weighed carefully before removal of these relatively benign malformations.     

Natural history of intracranial vascular malformations: a review

It is difficult to assess the natural history of intracranial vascular malformations because they are varied in nature, they are frequently silent clinically, they are often treated when they are discovered, and untreated lesions are not often followed in an organized way. Capillary telangiectasias are usually occult lesions of no clinical significance. Cavernous hemangiomas may cause seizures and may bleed, but the approximate yearly risks of bleeding and of death have not been determined. Venous angiomas seldom cause symptoms, with the exception that those in the cerebellum seem to have a propensity to bleed. Intracranial dural arteriovenous malformations (AVMs) may bleed and may cause brain injury if there is insufficient outflow into a dural venous sinus. The dural AVMs that drain into the cavernous sinus have a more benign course than those that drain into the transverse or sigmoid sinus. The aneurysm of the vein of Galen presents a different clinical picture and threat to health according to whether the patient is a neonate, an infant, or an older child. The AVM of the brain encountered in the adult usually presents with hemorrhage or seizures. An unruptured AVM has approximately a 2 to 3% risk of bleeding per year, with about a 1% risk of death per year. The mortality rate of the first hemorrhage is about 10%. Among the survivors, there is about a 6% chance of rebleeding during the 1st year and then approximately a 2 to 3% risk of bleeding per year subsequently. The mortality rate associated with a second hemorrhage is about 13%, and for subsequent hemorrhages the mortality is roughly 20%.     

Intradural extramedullary cavernous angioma: case report.

Cavernous angiomas represent 5 to 12% of spinal vascular malformations and usually are located at the vertebral body level with possible extension into the extradural space. The intradural intramedullary cavernoma occurs in about 3% of cases, whereas extramedullary localization is extremely rare. A new case of an intradural extramedullary cavernous angioma is reported, and the clinical, diagnostic, and therapeutic aspects of this rare malformation are analyzed.     

Diagnosis and treatment of spinal angiomas

Summary An account is given of 93 spinal angiomas found in 74 patients. 55 were solitary malformations and 38 were complex. In 5 patients angioma and angioblastoma were both present, and in 1 case an angioma was found in association with an aneurysm.The main problems of morphology, diagnostic investigation, improvement of early diagnosis, complications as a result of bleeding and circulatory disturbances with arachnoiditis and myelo-malacia and the microsurgical treatment with radical removal and the palliative procedures are discussed.Results depend on the situation of the angioma. After total extirpation of intradural angiomas in 34 cases, 14 patients recovered completely, 13 recovered partially, 5 were unchanged, and 2 deteriorated.     

A case of familial cerebral cavernous angioma and review of Japanese cases

We present one pedigree of familial cerebral cavernous angioma (FCCA). Case 1 was a 52-year-old male with right hemiplegia. When he was 37 years old, a left occipital lesion was excised and histologically diagnosed as cavernous angioma. MR image showed many cavernous angiomas in the right temporal lobe, the right paraventriclar white matter, the right frontal lobe, the left basal ganglia, and the left parietal lobe. Stereotactic radiosurgery was undertaken for all the lesions. Although the size of each lesion was unchanged, neither hemorrhage nor neurological deterioration were recognized after radiosurgery. Case 2 was a 24-year-old male, a son of the patient in case 1. He has manifested tonic-clonic type epilepsy since the age of 2. MR image showed cavernous angiomas in the pons, the right frontal, and the left intra-Sylvian regions, and many paraventricular cysts with rims indication of previous hemorrhages. Two de novo lesions were observed on subsequent annual MR screening. Surgical excision for the left intra-Sylvian lesion and stereotactic radiosurgery for all lesions were undertaken. Histological diagnosis was cavernous angioma. In the literature, there were 17 pedigrees and 37 cases of FCCA in Japan. The incidence of both multiple lesions and hemorrhage were less than in found in Spanish or French cases. Stereotactic radiosurgery is considered an useful treatment for FCCA, because lesions are multiple and de novo lesions occur.     

Intracranial cavernous angioma: presentation and management

Thirteen cases of cerebral cavernous angiomas are reported, and their radiological and pathological features are reviewed. The clinical presentation was variable: 53.8% of patients had seizures, 30.8% intracerebral hemorrhage, and 15.4% focal neurological signs, including one unusual case that presented as a cerebellopontine angle tumor. The computerized tomography (CT) scans were abnormal in all cases. Angiography was positive only when there was mass effect due to associated hematoma. Surgery was performed in 12 cases with good results. The prognosis of the 13 cases was favorable, with no mortality in this series. These data suggest that the combination of a recent seizure disorder with a hyperdense intracerebral nodule on the CT scanning should alert the neurosurgeon to the possibility of a cavernous angioma.     

Toward an anatomoclinical classification for arteriovenous malformations?

This brief study tends to point out that each clinical symptom (hemorrhage, seizures or epilepsy, headaches and progressive neurological deficits) related to cerebral arteriovenous malformations cerebral arteriovenous malformations can be correlated with the anatomy and the angioarchitecture of the cerebral arteriovenous malformations. The most dramatic data were the bipolar-like correlations between hemorrhage and seizures: each parameter correlated positively with hemorrhage was correlated negatively with seizures and vice versa. These data seem to show that cerebral arteriovenous malformations causing seizures have a low risk of hemorrhage, and cerebral arteriovenous malformations with a high hemorrhage risk have a low risk of seizures (excepting with an hematoma). For headaches and progressive neurological deficits, anatomoclinical correlations can improve knowledge of their pathophysiology.     

A case of cavernous angioma at the septum pellucidum

A 35-year-old female presented with a cavernous angioma of the septum pellucidum manifesting as headache and nausea. Computed tomography and magnetic resonance imaging revealed a mass at the septum pellucidum with intraventricular hemorrhage and mild hydrocephalus. Digital subtraction angiography showed no abnormal finding and thallium-201 single photon emission computed tomography showed no uptake in the lesion. The preoperative diagnosis was intraventricular tumor, such as subependymoma. The lesion was completely resected through the transcallosal approach. The histological diagnosis was cavernous angioma. Cavernous angiomas are usually located in the cerebral parenchyma and rarely occur in the cerebral ventricles. In particular, cavernous angioma at the septum pellucidum is very rare. If magnetic resonance imaging does not clearly show the typical peripheral hypointense rim, neuroimaging diagnosis will be difficult. Surgical resection should be considered to confirm the histopathology, in particular if bleeding and hydrocephalus are present.     

Vascular pressures and cortical blood flow in cavernous angioma of the brain

This study was designed to investigate the hemodynamic characteristics of cavernous angiomas of the brain. Five adult patients with a cavernous angioma underwent local cortical blood flow studies and vascular pressure measurements during surgery for the excision of the cavernous angioma. Clinical presentation included headache in four patients, seizures in four patients, and recurring diplopia in one patient. Magnetic resonance imaging demonstrated the cavernous angiomas in all patients and revealed an associated small hematoma in two. Four patients with a cerebral cavernous angioma were operated on in the supine position and the remaining patient, whose lesion involved the brain stem, was operated on in the sitting position. Mean local cortical blood flow (+/- standard error of the mean) in the cerebral cortex adjacent to the lesion was 60.5 +/- 8.3 ml/100 gm/min at a mean PaCO2 of 35.0 +/- 0.6 torr. Mean CO2 reactivity was 1.1 +/- 0.2 ml/100 gm/min/torr. The local cortical blood flow results were similar to established normal control findings. Mean pressure within the lesion in the patients undergoing surgery while supine was 38.2 +/- 0.5 mm Hg; a slight decline in cavernous angioma pressure occurred with a drop in mean systemic arterial blood pressure and PaCO2. Mean pressure in the cavernous angioma in the patient operated on in the sitting position was 7 mm Hg. Jugular compression resulted in a 9-mm Hg rise in cavernous angioma pressure in one supine patient but no change in the patient in the sitting position. Direct microscopic observation revealed slow circulation within the lesions. The hemodynamic features demonstrated in this study indicate that cavernous angiomas are relatively passive vascular anomalies that are unlikely to produce ischemia in adjacent brain. Frank hemorrhage would be expected to be self-limiting because of relatively low driving pressures.     

Cavernous angioma of the cauda equina: Report of two cases and review of the literature

Two cases of intradural cavernous angioma of the cauda equina are reported, one of which presented with symptoms of subarachnoid hemorrhage. The clinical and therapeutic aspects of intradural angiomas, in particular those of the cauda equina, are discussed.     

Cerebral vascular reactivity response to anaesthetic induction with propofol in patients with intracranial space-occupying lesions and vascular malformations

BACKGROUND AND OBJECTIVE: In clinical trials, autoregulation and carbon dioxide reactivity are preserved during propofol anaesthesia. Paradoxical increases of blood flow velocity during induction of anaesthesia could be demonstrated in patients with brain tumours. This study evaluates the effects of propofol on cerebral blood flow velocity in patients undergoing surgery for brain tumours and vascular malformations. METHODS: Changes in cerebral blood flow velocity after the administration of propofol were assessed using bilateral 2 MHz transcranial Doppler probes in 47 patients undergoing surgery for brain tumours and in 22 patients undergoing surgery for aneurysms and angiomas. RESULTS: Flow reduction after propofol was slightly less pronounced on the side of the tumour; in patients with cerebrovascular lesions, no difference between the two sides was detectable. After the administration of propofol a flow increase was present on the side of the tumour in 2 patients. In 3 patients with angiomas, the flow decrease after the administration of propofol was less pronounced on the side of the angioma. Neither observation gave statistical proof of abnormality. CONCLUSIONS: The flow changes after propofol may give a hint of cerebrovascular reactivity. Further investigations should focus on combined measurements of cerebral autoregulation and carbon dioxide reactivity and should focus on patients with impaired consciousness to test for reliability.     

Vascular malformations of the central nervous system: A morphological overview

Summary Vascular malformations of the central nervous system (C.N.S.) are classified by size, location, and morphologic type, distinguishing a) capillary telangiectasias, b) cavernous malformations, c) venous angiomas, d) arteriovenous malformations (AVMs) including varix of the great vein of Galen, and other vascular malformations (e.g. Sturge-Weber syndrome). The morphology and predominant location pattern of the different types of vascular malformations in the brain and spinal cord, and their embryology are reviewed. In the brain and its coverings, all types mainly AVMs and venous angiomas do occur, representing 5–9% of all intracranial space-occupying lesions and 20–40% of the sources of surgically treated intracranial hemorrhages. 50–80% of the angiomas are located in the cerebral hemispheres, 10–18% in central brain areas (basal ganglia, internal capsule, choroid plexus), and 10–30% in the posterior fossa. The major types of cerebral vascular malformations are described with reference to their anatomical features, location, chief arterial and venous supply, and prominent complications. Spinal vascular malformations, accounting for 3 to 12% of spinal space-occupying lesions, include vertebral, extradural, dural, subpial and intramedullary angiomas which occur as isolated or complex vascular anomalies and may involve various covering layers at the same level. The preferential occurrence of angiomas on the dorsal surface of the cord and in the caudal regions is related to the embryologic development of spinal vasculature. Frequent association of spinal angiomas (20–25%) with other vascular anomalies and dysplasias emphasizes their hamartomatous nature and developmental origin. Spinal angiomas include a) capillary telangiectasias with extra- or intradural and, rarely, intramedullary location, b) cavernomas, mainly arising in vertebral bodies, c) venous angiomas, mainly located in vertebral bodies and in the extradural space, and c) AVMs constituting the commonest type, that may affect both the pial and radicular vessels and can penetrate into the cord. They present as simple AV fistulas, cirsoid angiomas with localized vascular plexuses and large complex convolutions (juvenile type). The complications of spinal angiomas include subarachnoid hemorrhage, rare epidural hematoma, hematomyelia, compression lesions of the cord and roots, and ischemic changes causing chronic progressive radiculomyelopathy, previously referred to as Foix-Alajouanine syndrome. Chronic damage to the cord and spinal roots results from pressure effects, thrombosis of the abnormal vessels, disorders of venous drainage, and steal phenomena related to the vascular anomalies.     

Intraventricular hemorrhage in a term neonate secondary to a third ventricular AVM. Case report

An initially healthy infant born of an uncomplicated full-term gestation was brought for evaluation of the acute onset of vomiting, irritability, lethargy, and opisthotonus at 14 days of age. Computerized tomography demonstrated an intraventricular hemorrhage. Arteriography defined an angioma on the roof of the third ventricle which was successfully removed via the transcallosal interfornicial approach on the 34th day of life. Other than an easily controlled seizure disorder, the postoperative course was uneventful. At 8 months of age the child is developing normally. Arteriovenous malformations should be considered in the differential diagnosis of intraventricular hemorrhage in full-term neonates without predisposing trauma or bleeding diathesis. High-speed digital subtraction arteriography may be used to screen for this diagnosis. The transcallosal interfornicial exposure offers a satisfactory approach for excising third ventricular angiomas in young infants.     

Indications for surgery and prognosis in patients with cerebral cavernous angiomas.

Seventy-three cerebral cavernous angiomas were removed microsurgically from a series of 71 patients between August, 1983 and December, 1989. This retrospective investigation assessed the current indications for surgery and determined the prognosis for patients with cerebral cavernous angioma. There were 38 males and 33 females with a mean age of 37 years. Analysis included clinical presentation and history, neuroradiological findings, indications for surgery, and postoperative course. After an average follow-up period of 15 months, 35 patients were symptom-free, 16 had improved preoperative complaints, six were unchanged, and eight had deteriorated. Microsurgical extirpation of the malformation is indicated in all symptomatic patients where neuroimaging demonstrates the presence of a readily accessible cerebral cavernoma. Surgery is recommended in cases with deep-seated lesions causing massive hemorrhage, repetitive minor bleeding, or significant long-standing and progressive neurological disabilities. Clinically silent cavernomas located in eloquent regions of the brain contraindicate surgery, but should be closely monitored. Patients presenting with convulsions or neurological deficits caused by easily accessible cavernomas of the hemispheres have the best prognosis and a negligible risk for surgical complications. Those with deep-seated lesions of eloquent regions of the brain that have bled or caused sustained neurological disorders face the highest risk for morbidity owing to the surgical intervention, requiring careful preoperative evaluation.     

Cavernous angiomas of the central nervous system in children.

A surgical series of 19 patients under the age of 18 years with pathologically verified cavernous angioma is presented. Most lesions were located in the cerebral hemispheres, but four were in the pons or midbrain, two in the diencephalon, and one in the spinal cord. Fourteen patients presented with an acute or progressing neurological deficit, three with seizures, one infant with irritability, and one with headache alone. Five patients had family histories of vascular malformations of the central nervous system, and five had multiple lesions. Surgery for small or deep lesions was aided considerably by intraoperative ultrasonographic or stereotactic localization techniques. Pathological examination of the resected malformations revealed a complex histology containing not only typical closely approximated cavernous vessels, but also areas of marked proliferation of granulation tissue and partially re-endothelialized hemorrhage, suggesting a mechanism for the apparent growth of certain cavernous angiomas. The postoperative results were good, with only one patient suffering a permanent worsening of neurological status after surgery. Incomplete resection was initially carried out in five patients, two of whom rebled within 1 year after operation. Long-term follow-up findings in these patients have emphasized the unusual history of certain of these malformations.     

Cerebral venous angiomas: Surgery as a mode of treatment for selected cases

Summary Eleven patients with venous angiomas, 6 males and 5 females ranging in age from 4 to 58, are presented. Four patients presented with intracerebral haematoma and 3 patients had associated cavernous angioma, respectively. Patients with intracerebral haematoma had signs and symptoms due to the localication of the haematoma. The other patients presented with headache, seizures, vertigo, ataxia and mental disturbances. Pre-operative diagnosis was based on computerized tomography, magnetic resonance imaging and cerebral angiography. In 9 surgical cases it was confirmed by histopathological examination of operative specimens.After establishing the type, size and location of the lesion decision for operative treatment was made in nine cases, in four of them because of the presence of an intracerebral haematoma and in 5 of them due to severe disability. Eight of these 9 patients recovered completely and one improved. No severe cerebral oedema was encountered after converging medullary veins were excised and main draining veins partially coagulated.In this small series we encountered an unexpectedly large percentage of venous angiomas causing intracerebral haemorrhage which are commonly considered more benign than other vascular malformations. After reviewing previously reported cases of venous angiomas causing intracerebral haemorrhage and severe neurological deficit we think that the term benign is worth reconsidering. We propose a thorough examination of each case of venous angioma and the operative treatment when appropriate taking into account patients state and location of angioma.