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.     

功能磁共振联合神经导航辅助切除功能区海绵状血管瘤

目的探讨功能磁共振联合神经导航辅助的显微手术在治疗功能区海绵状血管瘤中的应用价值。方法用功能磁共振联合神经导航技术,对功能区的5例海绵状血管瘤行显微外科手术切除。结果病灶全切除率为100％,无重要神经功能受损表现,无手术并发症及死亡。结论功能磁共振联合神经导航辅助的显微手术治疗功能区内海绵状血管瘤,定位精确,微侵袭,可提高海绵状血管瘤切除率,降低手术并发症。对手术切除脑功能区海绵状血管瘤并保留运动功能有重要的指导作用。     

Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management.

Cavernous angiomas of the spinal cord are rare lesions that can cause severe neurological symptoms. We add a series of 6 patients with intramedullary cavernous angiomas of the spinal cord to 30 patients with 31 histologically verified lesions already described in the literature. Four types of clinical presentation were seen in the 36 patients: 1) discrete episodes of neurological deterioration with varying degrees of recovery between episodes (13 patients); 2) slow progression of neurological decline (12 patients); 3) acute onset of symptoms with rapid decline (8 patients); and 4) acute onset of mild symptoms with subsequent gradual decline lasting weeks to months (3 patients). Of the 36 patients, 25 (69%) were women and 11 (31%) were men. The peak age of presentation was in the fourth decade. The thoracic spinal cord was affected in more than half the patients, with the cervical cord being the next most common location. Histological examination demonstrated the closely apposed vascular channels characteristic of cavernous angiomas. While most vascular channels were thickened and hyalinized, three angiomas had foci of small vessels resembling a capillary angioma. The available data suggest that surgical management of intramedullary angiomas should attempt complete extirpation. The lesions are often well circumscribed with a glial plane between the lesion and normal cord. However, spinal cord angiomas, unlike cranial lesions, have little room for enlargement before devastating symptoms occur. We achieved complete excision in all of our patients, who are all ambulatory and have improved symptoms. Little, if any, increase in neurological deficit was caused by myelotomy and lesion excision. These spinal lesions should be considered for surgery early, before repeated hemorrhage or enlargement can occur.     

Surgically treated cavernous angiomas of the brain stem: report of two cases and review of the literature

Two cases of surgically treated brain stem cavernous angiomas are presented. The previously reported cases are reviewed and the clinical features, radiographic appearance, and treatment of this condition are discussed. Symptomatic brain stem cavernous angiomas should be treated surgically and can be removed with good outcomes.     

Microsurgery of deep-seated cavernous angiomas: Report of 26 cases

Summary The authors review 26 patients with deep-seated cavernous angiomas which were removed by microsurgery. Ten of the angiomas were located in the insula and basal ganglia, 2 in the thalamus, 5 in the midbrain, 8 in the pons, and 1 in the brachium pontis. The patients were among 73 consecutive cases operated on between August 1983 and December 1989 for symptomatic cavernous angiomas in various locations.In 11 cases total excision of the cavernoma was achieved without producing additional neurological deficits. Postoperative neurological recovery was delayed in 7 patients. In the remaining 8, the complicated postoperative course was caused by bleeding from residual parts of the malformation or damage to long-tract pathways in two cases, respectively, vascular injury during dissection in three cases, and paradoxical air embolism in one case. In order to achieve a satisfactory surgical result, it is stressed that particular attention has to be paid to the operative approach, to careful dissection and complete removal of the malformation, to perforating arteries, and to anomalous venous drainage.     

Cavernous angioma of the pineal region.

The pineal region is one of the most rare localizations of intracranial cavernous angiomas, with only 8 cases reported up today. The Authors report a case of cavernous angioma of such localization and review the pertinent literature. Magnetic resonance allows the correct diagnosis of cavernous malformations on the basis of their typical aspect, even in the absence of histological verification. We suggest that this imaging technique will allow to identify more frequently pineal cavernomas preoperatively, thus avoiding useless irradiation.     

Cavernous angiomas of the spinal district: surgical treatment of 11 patients

Cavernous angiomas, also called cavernous malformations or cavernomas, are vascular hamartomas accounting for 3–16% of all angiomatous lesions of the spinal district. Although histologically identical, these vascular anomalies may exhibit different clinical behavior and radiological features, depending on their location, hinting at different managements and therapeutic approaches. The authors report 11 cases of symptomatic spinal cavernous angiomas diagnosed and surgically treated over the past 18 years. Age of patients ranged from 15–75 years; males outnumbered females. Three patients had vertebral cavernous malformations, secondarily invading the epidural space; two had pure epidural lesions; two patients had intradural extramedullary lesions, and four intramedullary lesions. Surgical removal was completely achieved in four patients with intramedullary lesions, in two with subdural extramedullary lesions, and in one with a pure epidural lesion. Subtotal excision of another one epidural and three vertebral cavernous angiomas was followed by radiotherapy. There was no morbidity related to surgery; the mean follow-up was 2 years. The outcome was excellent in two cases, good in six, and unchanged in the other three. The authors discuss the different modalities of treatment of these vascular lesions variously placed along the spine.     

Cavernous angiomas within the internal auditory canal

OBJECT: The aim of this study was to describe the symptomatology, radiological features, and surgical treatment of patients with cavernous angiomas within the internal auditory canal (IAC). METHODS: The authors reviewed the cases of seven patients with cavernous angiomas in the IAC that had been surgically treated in the 22-year period between 1983 and 2005. All the patients had presented with sensorineural hearing loss, and four suffered from tinnitus. Four patients also reported facial symptoms such as hemispasm or progressive palsy; one of these patients had presented with sudden facial paresis due to intrameatal tumor hemorrhage. According to computed tomography (CT) results, the lesions caused enlargement of the IAC. Interestingly, these same angiomas showed variable features on magnetic resonance (MR) imaging, making their differentiation from intrameatal vestibular schwannomas (VSs) sometimes impossible. In all patients the lesions were totally removed via the suboccipital retrosigmoid approach. They could be dissected away from the facial nerve in five cases, whereas in two cases, because of the location of the lesion, the seventh cranial nerve had to be sectioned and repaired with a sural nerve graft. Transient worsening of seventh cranial nerve symptoms occurred in two patients, with postoperative improvement in each of them. The cochlear nerve could not be functionally preserved because of its extreme adherence to the tumor, although its continuity was preserved in four patients. Complete deafness was the only postoperative complication. CONCLUSIONS: Cavernous angiomas of the IAC are very uncommon lesions that can imitate the symptoms of VSs. Although it is the most sensitive study available, MR imaging does not show sufficiently specific findings to differentiate the two lesion types. Thus, the preoperative diagnosis must be based on patient symptoms plus the CT and MR imaging features.     

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 hemangiomas of the dura mater at the convexity. Report of a case and therapeutical considerations

A rare case of cavernous angioma of the dura mater at the convexity is presented. In comparison with the more common cavernous hemangiomas that arise from dural sinuses, the ones on the convexity have a much better prognosis. They do not seem to be associated with spontaneous hemorrhage, their removal is simpler and, transoperatory bleeding, if it occurs, is scanty. Computerized tomography detects the presence of cavernous angiomas of the convexity in only 70% of the cases, magnetic resonance imaging in 100%.     

Cavernous angioma of the geniculate ganglion

Intracranial extraaxial cavernous angiomas are rare vascular malformations. Their occurrence at the geniculate ganglion of the facial nerve within the temporal bone is exceptional. The authors describe a 35-year-old man who developed a slowly progressing facial palsy. Initial cranial MR imaging showed no pathological findings, but 2 years later another MR examination detected a small tumor located at the geniculate ganglion of the facial nerve. The tumor was removed via a subtemporal approach. Histological examination revealed a cavernous angioma. Even small cavernomas located at the geniculate ganglion of the facial nerve may result in facial palsy. Isolated facial palsy in a young person should be monitored closely using imaging studies even if the initial imaging study is negative. Early decompression of the facial nerve may help to preserve its function.     

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 and orbital cavernous angiomas. A review of 30 cases

The authors review 30 documented cases of intracranial and orbital cavernous angiomas treated at their institution between 1965 and 1984. The diagnosis was based on computerized tomography (CT) or surgery; three patients were treated in the pre-CT era (1965 to 1976) and 27 since the advent of CT. The number of cases diagnosed preoperatively markedly increased after the introduction of CT, and 22 cases were verified histopathologically at surgery. Six cases were in children (aged 2 months to 17 years) and 24 in adults (aged 19 to 73 years). There was no significant sex difference (male:female ratio was 14:16). Nineteen lesions were intraparenchymal, five were intraventricular, three were in the middle fossa, two were intraorbital, and one originated from the tentorium. Symptoms varied according to the site of the lesion; hemorrhage occurred in 11 cases. Calcifications were seen on CT scans in all cases, but on plain skull films in only two. Angiography revealed hypovascular masses in all cases excluding those with lesions in the middle fossa; in two cases, tumor stain could be detected only with prolonged-injection angiography. Radionuclide brain scanning showed a dense hot area in eight of 19 patients. Recent experience has shown that magnetic resonance imaging clarified anatomic relationships that were obscure on CT. The overall outcome was favorable except for one patient who died in the postoperative period. The clinical results in this series are summarized and some diagnostic and therapeutic problems are discussed.     

Intraventricular haemorrhage caused by aneurysms and angiomas

Summary More than 200 intraventricular haematomas (IVH) have been treated in the Homburg Neurosurgical University Clinic since computed tomography was available and facilitated the diagnosis.Among 200 consecutive cases, which are analysed and presented in this publication, there were 71 patients with subarachnoid haemorrhage (SAH)-58 of whom with angiographically and/or pathologically verified aneurysms-, and 21 cases with intraventricular angiomas. IVH without concomitant intracerebral haematoma (ICH) and without evidence of SAH is highly suggestive of intraventricular angioma.In our experience panangiography [if available digital subtraction angiography (DAS)] should be done as soon as possible in all cases of IVH. It is a precondition for early diagnosis and operative elimination of the source of bleeding, because the retrospective analysis of our material shows that rebleeding is by far the highest single risk factor in cases with IVH caused by aneurysms or angiomas.We therefore recommend early microsurgical occlusion of the aneurysms and exstirpation or intravascular embolisation of the angioma.The best survival rate (76%) was achieved in IVH cases caused by angiomas. In aneurysms with IVH the survival rate was 35%, in IVH caused by other diseases 37%. The worst prognosis occurs in SAH with IVH without proven aneurysm or angioma. The survival rate of this group was only 8%.     

Radiologic and surgical aspects of pure spinal epidural cavernous angiomas. Report on 5 cases and review of the literature.

BACKGROUND: Cavernous angiomas (CAs) that are localized completely in the spinal epidural space are uncommon vascular malformations. Although they have increasingly been reported in the literature in recent years, diagnostic and surgical features are not clearly defined. METHODS: We report five patients with pure spinal epidural cavernous angiomas (PSECAs) and review the literature, focusing on their radiologic and surgical characteristics. We also compare these tumors with other extra-axial CAs as well as with their intra-axial counterparts. RESULTS: PSECAs, like all other extra-axial CAs, differ from intra-axial ones on MRI: the hemorrhagic variant is less frequent, hemosiderin rim is rare, the signal is different, and contrast enhancement is the rule. They are very similar to spinal meningiomas but they differ in their growth pattern and morphology, since they infiltrate intervertebral foramina and have an oval shape. In PSECA, intraoperative bleeding is rarely profuse, in contrast to other extra-axial CAs, especially those of the cavernous sinus. CONCLUSIONS: On MRI, PSECAs and other extra-axial CAs constitute a homogeneous group since they enhance significantly. At operation, since there is rarely enough bleeding to limit removal, radical excision of PSECAs can be achieved with good results.     

Cavernous-venous malformation of brain stem--report of a case and review of literature.

BACKGROUND: Because of improved imaging techniques, the association of cavernous and venous malformations is now being seen more often in neurosurgical practice. However, only a few cases have been subjected to surgery. Surgical excision of the cavernous malformation, sparing the venous component, has been the method of choice advocated by most authors. This, however, is not possible when the components are intimately associated with each other. It thus becomes important to predict the surgical outcome in such lesions, especially when they are located in an eloquent area. A surgically excised case of cavernous-venous malformation in the dorsal pontomedullary region is presented here; the histology confirmed the intimate association of both components. The relevant literature is also discussed. CASE DESCRIPTION: This 20-year-old male presented with episodic vomiting, giddiness, and persistent cerebellar signs over a six-year period. Neuroimaging studies-computed tomography scan/magnetic resonance imaging (CT/MRI) were suggestive of a cavernous malformation. MRI also showed a draining vein close to the lesion. Following total excision, histopathology confirmed the intimate association of both cavernous and venous malformations. The patient made an uneventful recovery. MRI at follow up confirmed total excision. CONCLUSIONS: The presence of venous channels draining into a lesion that otherwise appears to be a cavernous malformation may indicate an intimately associated venous component. Selective surgical excision of the cavernous component may be difficult in such cases. Surgical outcome, though favorable in this case, will be difficult to comment upon unless more such cases are reported. This may be of greater significance in lesions located in eloquent areas.     

Management of patients with cavernous angiomas presenting epileptic seizures

BACKGROUND: The aim of this study was to establish a treatment strategy for patients with cavernous angiomas and have seizures. METHODS: We analyzed retrospectively 31 patients with cavernous angiomas presenting with seizures. Of the 31 patients, 15 (11 men, 4 women) had been treated medically (the medical group) and 16 (11 men, 5 women) had been treated surgically (the surgical group). The percentage of patients who became seizure-free and the number of anticonvulsants used were compared between these 2 groups. The mean follow-up period was 5.3 years (range, 7 months-10 years). There was no statistically significant difference between the 2 groups with regard to the number of patients, sex, age, and follow-up period. RESULTS: The percentage of patients who became seizure-free was higher in the surgical group than in the medical group (80% vs 19%, P < .01). The number of anticonvulsants required was reduced in the surgical group after surgery (from 1.5 +/- 0.7 to 1.1 +/- 0.8, P < .01) and was not reduced after in the medical group. CONCLUSIONS: Surgical intervention may have greater benefits than medical intervention for patients with cavernous angiomas presenting with epileptic seizures.     

Cavernous angiomas of the spinal cord

Five cases of histologically verified cavernous angiomas of the spinal cord are reported. Acute lower-extremity sensory disturbance was the initial symptom in four patients, and one presented with weakness of the hand. Progressive neurological deficit occurred in all patients, but the clinical course and outcome were extremely variable. Myelography revealed an intramedullary lesion in two cases but was completely normal in three; magnetic resonance imaging was diagnostic in these patients. Subtotal removal was accomplished in two cases, and myelotomy and biopsy were carried out in three. Four of the cavernous angiomas were located in the cervicothoracic region, whereas one was found in the thoracolumbar cord. All of the patients exhibited characteristic gross and microscopic features as well as hemosiderin-laden macrophages indicating remote hemorrhage. The diagnostic, therapeutic, and prognostic implications of this rare condition are discussed.     

Cavernous angiomas of the internal auditory canal A case report and review of literature

Summary Cavernous angiomas of the internal auditory canal (IAC) are rare. They are angiographically occult; and because the clinical symptoms are similar both in intracanalicular cavernous angiomas and acoustic tumors it had been difficult to differentiate pre-operatively both of these pathologies until the advent of magnetic resonance imaging (MRI). Even nowadays the correct diagnosis may be missed if the patient is imaged only with gadolinium enhanced MRI without prior obtaining a non-contrast MRI. These diagnostic difficulties are illustrated by the report of a related case. The importance of thorough neuroradiological investigations stressed and MRI features, surgical management and relevant literature concerning the cavernous angiomas of the internal auditory canal are discussed.     

Proximal nerve root capillary hemangioma presenting as a lung mass with bandlike chest pain: case report and review of literature

BACKGROUND: Capillary hemangiomas occasionally occur in the peripheral nervous system (PNS), presenting as mass lesions causing proximal nerve root symptoms, thus prompting neurosurgical intervention. In contrast to cavernous angiomas, which may also occasionally involve the PNS, capillary hemangiomas have little or no tendency for acute or chronic bleeding and, hence, lack the apoplectic symptomatic onset and neuroimaging features of hemosiderin deposition as seen in cavernous angiomas. CASE DESCRIPTION: A 56-year-old man presented with chronic, radiating, bandlike, chest wall pain. Chest radiograph revealed an apical lung mass suspicious for malignancy. Subsequent magnetic resonance imaging revealed an enhancing dumbbell-shaped mass extending into the spinal canal and chest cavity at T3, causing a slight degree of lung compression. Differential diagnostic considerations included schwannoma, neurofibroma, and meningioma. At operation, the spongy, reddish purple vascular tumor was easily separated from dura but had to be excised en bloc, with the T3 nerve root at the neural foramen. The large intrathoracic portion measuring 37 x 41 mm was left behind. Pathology was consistent with a capillary hemangioma. CONCLUSIONS: We present the second case in the English language literature of a dumbbell-shaped, epidural capillary hemangioma with intrathoracic extension. We review the literature on proximal nerve capillary hemangiomas and contrast these with cavernous angiomas and capillary hemangioblastomas, both of which occasionally involve proximal nerves. Unlike capillary hemangioblastomas, which may be multiple and associated with von Hippel-Lindau (VHL) syndrome, capillary hemangiomas are solitary lesions and have not been associated with an inherited disorder.