Gradual deterioration of brainstem cavernous angioma associated with hemophilia--case report

A 49-year-old man presented with a brainstem cavernous angioma associated with hemophilia manifesting as gradual progression of neurological deficits over a period of 2 weeks. Computed tomography revealed a hematoma and perifocal edema on the left side of the pons, and T(2)-weighted magnetic resonance imaging revealed a hemosiderin rim around the lesion with venous malformation. The neurological deficits deteriorated despite conservative treatment, so surgery was performed 2 weeks after admission, after supplementary therapy of factor IX. The hematoma and anomalous vascular component were entirely removed without intractable bleeding. The postoperative course was uneventful and his neurological symptoms improved. The histological diagnosis was cavernous angioma. Six months after onset, he was doing well. Surgery can be effective for the treatment of hemorrhagic cavernous angioma associated with hemophilia after initiation of supplementary therapy with coagulation factor.     

Surgical treatment of cavernous angioma involving the brainstem and review of the literature

Summary Five cases of symptomatic cavernous angioma involving the brainstem are reported. Magnetic resonance (MR) imaging is of greatest value in the diagnosis and for surgical indication. All cases were treated by radical extirpation. All of them improved postoperatively. The surgical indications for this lesion of the brainstem are briefly discussed with a review of the literature, including 28 previous cases, operated on directly.     

Hemifacial spasm caused by intra-axial brainstem cavernous angioma with venous angiomas

Hemifacial spasms (HFS) are usually caused by vascular compression on the extra-axial facial nerve. In this case, we concluded that an intra-axial brainstem cavernous angioma with a venous angioma diagnosed by MRI must have been responsible for HFS, because no other possible causes were found during intraoperative observations.     

Controversies in the management of brainstem cavernous angioma: report of two cases.

Two cases of cavernous angioma involving the medulla oblongata are presented. Both cases underwent surgical excision with excellent outcome. The use of surgery via craniectomy is contrasted with stereotactic radiosurgery in light of the known natural history of the lesions. As a result, it is suggested that surgical excision provides immediate protection from the risks of recurrent haemorrhage, establishes a tissue diagnosis, allows complete removal at the primary intervention, avoids complications of radiation-induced damage and is performed more easily in these vascular anomalies due to the presence of a capsule with surrounding gliotic tissue. Additionally, it is implied that the natural history of lesions in this region is still unclear. For these reasons, it is suggested that surgical excision should be the primary therapeutic intervention for cavernous angiomata that involve the brainstem.     

Intracranial cavernous angioma

We report 14 cases of intracranial cavernous angioma, analyzing the clinical features, with special reference to the risk of bleeding, radiological images and treatment in these and in 153 published cases, 167 in all. Cerebral hemorrhage occurred in 44%: typical (intraparenchymal or subarachnoid) in 24.6%, and masked by epilepsy, headache or neurological deficits in 19.2%. In patients with the typical hemorrhagic pattern posthemorrhagic mortality was 12.2%. Of the patients who had a hemorrhage 42.5% were left with more or less disabling neurological deficits, and 16.4% had a rebleed. In discussing treatment we consider four groups of intracranial cavernous angioma: A) symptomatic in a zone of low surgical risk; B) asymptomatic with low surgical risk; C) symptomatic with high surgical risk; D) asymptomatic with high surgical risk. The treatment is surgical, except in the high risk asymptomatic variety, best followed initially with sequential CT scan and MRI and then considered for surgery if the lesion becomes symptomatic, increases in size or presents neuroradiological signs of bleeding.     

Diffusion tensor-based fiber tracking and intraoperative neuronavigation for the resection of a brainstem cavernous angioma

BACKGROUND: Surgical treatment of brainstem cavernous angioma involving eloquent fiber tracts is one of the most challenging areas of contemporary neurosurgery. Diffusion tensor imaging-based fiber tracking adds essential information for preoperative planning in neurosurgical practice. Integration of the tractography into neuronavigation system makes it possible for the intraoperative visualization of the major fiber tracts. It may help increase the likelihood of the total resection of tumors adjacent to the eloquent fiber tracts and avoid new neurologic deficits after surgery. CASE DESCRIPTION: We report our valuable experience of such integration for the resection of a deeply located brainstem cavernous angioma. CONCLUSION: Inclusion of tractography in standard imaging protocols for neuronavigation systems can provide important information regarding neural tracts for the planning of brainstem surgery. It can also increase the safety of neurosurgical intervention near white matter tracts in the brainstem.     

Intracranial cavernous angioma in the dura

Cavernous malformations located in the dura are rare. We present a case of a large cavernous malformation located in the anterior fossa and implanted in the dura of a pregnant woman. It showed great vascularization on an angiogram, and resembled a meningioma on computed tomographic scan. The operation was difficult because of the tumor's great vascularity and profuse bleeding.     

Dumbbell-shaped spinal epidural cavernous angioma

A case of a spinal epidural cavernous angioma extending in the intra- and extravertebral spaces through the intervertebral foramen is presented. It was dumbbell shaped, but did not show enlargement of the intervertebral foramen, a common sign that is thought to be helpful in the diagnosis of spinal neurinoma.     

Familial occurrence of intracerebral cavernous angioma

This article reported a familial occurrence of intracerebral cavernous angioma in four members of one generation diagnosed by X-ray CT, MRI or operative specimen. Case 1, a 34-year-old female, was examined just after an episode of sudden convulsive seizure. On examination, she had a cutaneous angioma without any neurological deficit. X-ray CT revealed a high density mass lesion in the left frontal lobe, and MRI demonstrated a mass lesion in the chronic stage with an old hematoma circumscribed by hypointensity ring indicating peripheral hemosiderosis. Complete excision was carried out and a diagnosis of cavernous angioma was made after histological examination. Case 2, the 37-year-old brother of Case 1, suddenly developed left hemiparesis and hypesthesia with severe headache. X-ray CT revealed a high density mass in the right parietal lobe and two other calcifications. The right parietal lesion was excised and a histopathological diagnosis of cavernous angioma with intracerebral hematoma was made. Case 3, the 49-year-old sister of Case 1, suddenly fell into a coma and was admitted immediately. X-ray CT revealed a large pontine hemorrhage. She died on the 4th day of hospitalization without operative treatment. Necropsy was not carried out. Case 4, the 39-year-old sister of Case 1, was asymptomatic, however, she was examined on the supposition of a familial occurrence of intracerebral cavernous angioma. On examination, it was found she had multiple cavernous angioma without any neurological deficit. X-ray CT revealed parietal intracerebral calcification. MRI demonstrated a mass lesion with peripheral hypointensity ring in the right parietal lobe, and another small lesion in the pons.(ABSTRACT TRUNCATED AT 250 WORDS)     

Familial occurrence of intracerebral cavernous angioma

A case of familial occurrence of intracerebral cavernous angioma is reported. The patients were a mother and her eldest son, a 48-year-old woman and a 28-year-old man, respectively. The mother, a hypovascular mass in the right frontal lobe was excised surgically, and in the son a well demarcated mass in the left temporal lobe was extirpated. Pathological examination in each case revealed cavernous angioma. Familial occurrence of intracerebral cavernous angioma is extremely rare. In the literature, 8 cases of familial occurrence have been reported, including our case, and in three out of the 8 cases the lesions were histologically confirmed. To our knowledge, this is the first case in Japan in which the diagnosis was established by surgical specimen. Of 17 cases in 8 family lines, multiple occurrence was observed in 7 cases. The clinical manifestations of the disease appear mostly in a form of adult epilepsy or intracerebral hematoma, and rarely sudden death associated with intracerebral hematoma. At present, CT scanning is widely used for diagnosis of the disease, and the lesions are surgically curable if they are located at the accessible sites. Although there has been no evidence of genetic basis, it is interesting that there is familial occurrence. From this point of view, we should carefully check the family tree of such patients.     

A case of tentorial cavernous angioma

A case of tentorial cavernous angioma is reported. A 61-year-old woman was admitted because of left auditory disturbance and left hemifacial spasm. CT scan and angiography, MRI revealed a dumb-bell typed tumor extended above and below the left tentorium cerebelli. Under the preoperative diagnosis of tentorial meningioma, a craniotomy was performed and the tumor was removed totally with minimal bleeding by left subtemporal approach. The tumor was well capsulated and spongy, attached to the tentorium cerebelli. Histologically, the tumor had many various vascular cavities with the wall lined with a single layer of endothelial cell, had neither glial cell nor meningeal cells. Some of cavities were thrombosed. Pathological diagnosis was a cavernous angioma. We discussed the characters of this rare tumor with a review of 4 cases.     

Intracranial cavernous angioma angiographically mimicking venous angioma in an infant

We present a case of a 15-month-old infant with intracranial cavernous angioma in the right frontal lobe in which the cerebral angiogram revealed transmedullary collecting veins characteristic of venous angioma around the lesion. This vascular malformation was histologically confirmed to be a cavernous angioma.     

Hemoperitoneum caused by cavernous angioma of the omentum

A case history is reported of a spontaneous, nontraumatic, hemoperitoneum in an eleven year old girl. The onset, the accompanying symptoms and the clinical course, mimicked those of acute appendicitis. However the etiology was found to be the spontaneous rupture of a cavernous hemangioma of the omentum. Only one other similar case has been reported in the literature.     

Report on successful surgery of cavernous angioma in the dorsal part of the pons--usefulness of MRI in diagnosis

A case with cavernous angioma which was located in the dorsal of the pons was cured surgically. A 16-year-old male was admitted with complaints of right motor and sensory disturbance. On neurological examination, the patient had right hemiparesis and hemiparesthesia, and right cerebellar signs. X-ray CT scan demonstrated a high density mass in the pons. Carotid and vertebral angiograms showed no abnormality. T1 and T2 weighted MRI clarified the pontine mass not as a tumor but a hematoma. Then, from the age of the patient, his clinical course, follow up X-ray CT and MRI findings, it was considered that a pontine lesion must have been an angioma with hemorrhage. Therefore, the operation was performed in an attempt to removal the hematoma and the mass of abnormal vessels in the dorsal part of the pons. Histological examination of the surgical specimen revealed abnormal vessels as a cavernous angioma. The postoperative course was uneventful.     

Intraventricular cavernous angioma. A survey

Summary Since the availability of CT diagnostis 23 cases of intraventricular cavernous angioma (IVCA) have been published in the literature. Three additional cases have been operated upon in our Department. Based on these 26 cases the clinical data, radiological findings, treatment and outcome of IVCAs are reviewed.     

Radiation-induced cavernous angioma mimicking metastatic disease

Patients with carcinoma of the lung typically have a limited life expectancy especially after developing metastatic disease in the brain. New enhancing lesions in the brain are usually felt to represent new areas of metastasis. Recently, there have been several case reports of cavernous angiomas appearing years after radiation to the brain, typically in children. We present a case of a 41-year-old gentleman with carcinoma of the lung with metastasis to the brain who received postoperative radiation. Five-and-a-half years later he presented with a new enhancing lesion of the brain with surrounding vasogenic oedema, thought to represent a metastatic tumour. It proved is the a radiation-induced cavernous angioma.     

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'.