Brainstem Cavernoma Research Articles

A brainstem cavernoma demonstrating a dramatic, spontaneous decrease in size during follow-up: case report and review of the literature

Background Many reports have demonstrated a worse prognosis for patients whose cavernomas were subtotally removed than for those whose cavernomas were not surgically treated. Therefore, it is better not to touch the cavernoma if a surgeon is not prepared to totally remove it. This report describes a large brainstem cavernoma showing a spontaneous, dramatic reduction in size after removal of only the biopsy specimen of the lesion. Case Description A 42-year-old woman experienced facial numbness, diplopia, and ataxia. A magnetic resonance (MR) study revealed a pontine cavernoma with hemorrhage. Two weeks later, recurrence of the patient's symptoms and consciousness disturbance were noted. An MR study revealed massive hemorrhage from the cavernoma with a ventricle dilatation. An operation on the lesion was conducted 3 months after the initial hemorrhage. However, the operation was aborted when an exploration of the lesion showed a tight adhesion between the lesion and the pons. Removal of only a biopsy specimen and evacuation of the suckable hematoma were done. No neurologic recovery and no decrease in the size of the cavernoma were detected postoperatively. Her family did not wish for further treatments. She was in a bedridden state with severe brainstem dysfunction when she was transferred to a local hospital. Sixteen months after the surgery, her consciousness was clear, and MR imaging confirmed a marked reduction in the size of the cavernoma. Conclusion A dramatic, spontaneous decrease in size does occur even in the case of a large brainstem cavernoma showing hemorrhages. Conservative therapy may be one of the treatment options for the symptomatic brainstem cavernoma.

Skeletal muscle involvement in congenital long QT syndrome

Skeletal muscle involvement in a genetically confirmed congenital long QT syndrome (LQTS) has not been reported. In a 36-year-old male, intracerebral bleeding from a brainstem cavernoma suddenly occurred. His history was noteworthy for congenital LQTS due to a point mutation in the KVLQT1-gene on chromosome 11. The mutation was also found in his two daughters, his sister, her daughter and his mother. His ECG showed sinusrhythm and a QTc-interval of 0.46 ms. Needle-EMG from the right abductor pollicis, left brachial biceps, left extensor digitorum, and right anterior tibial muscle(s), carried out because muscle involvement had been previously reported in clinically diagnosed congenital LQTS, revealed abnormal spontaneous activity, but otherwise normal motor unit architecture. LQTS, due to a KVLQT1 mutation, may manifest not only in the heart, but subclinically also in the skeletal muscle.

Microsurgical treatment of brainstem cavernous angiomas

The authors report the successful surgical resection of brainstem cavernous angiomas in a series of 42 consecutive patients. There were 22 females and 20 males whose ages ranged from 16 to 62 years with a mean of 39.5 years. Twelve lesions were located within the midbrain, 25 in the pons and 5 in the medulla. Immediately following the procedure, 3 patients showed dramatic clinical improvement, 30 patients had no additional neurological deterioration, 9 individuals developed new deficits or an accentuation of preoperative symptoms. There were no major complications and no mortality in this series. At follow-up ranging from 6 months to 7 years (mean 3 years), one patient remained with a permanent oculomotor paralysis and two others with a slightly accentuated gait ataxia. The vast majority of the remaining 39 individuals have experienced a clinical improvement of preoperative symptoms. All 42 patients in this series lead an independent life and none has suffered any further brainstem hemorrhage after surgery. A careful selection of the surgical approach and optimal entry zone into the brainstem play a major role for the success of the procedure. With a meticulous microsurgical technique, even deeply located intra-axial brainstem cavernomas can be removed without creating additional morbidity.

Deep and brainstem cavernomas: a consecutive 8-year series.

The goal of this study was to provide epidemiological and clinical data on the management of cavernomas of the basal ganglia and brainstem from a long-term series at one institution. All 68 patients who were referred to the authors' department between 1992 and 2000 for deep cavernomas were evaluated by clinic examinations, review of neuroimaging examinations, and review of charts and operative notes. Twenty-nine patients underwent microsurgical procedures, which carried a 69% risk of transitory neurological deterioration. Radical excision was achieved in 25 of these patients, as determined by a review of neuroimages; the remaining four patients all experienced new hemorrhages that led to increased morbidity or even to mortality. Surgical results were better if surgery was performed early, within 1 month posthemorrhage, than if operations were postponed. In selected patients, deep lesions not reaching a pial surface could be safely removed from the thalamus, basal ganglia, or medulla oblongata. Of five patients who underwent gamma knife surgery, two experienced hemorrhages, one at 2 and the other at 5 years following treatment. Patients who did not undergo surgery had a yearly incidence of hemorrhage that was 2% in cases of incidental cavernomas and 7% in symptomatic ones. Over the long term, outcomes were worse following conservative treatment or shunt insertion surgery than after microsurgery of symptomatic cavernomas. Incidental cavernomas carried a low risk of neurological deterioration. Surgery should follow generally accepted indications, but only with the confidence that total removal can be safely achieved. Surgery that is performed within 10 to 30 days following ictus may be preferable to delayed surgery.

Surgical management of brain-stem cavernous malformations: report of 137 cases

Background With the improvement in neuroimaging and microsurgical techniques, brain stem cavernous malformations are no longer considered inoperable. Surgical indications for brainstem cavernoma are evolving, with better understanding of its natural history and decreasing surgical complications. Methods During 1986 through 1998, a series of 137 patients (4 patients each with two brain stem lesions, total number of lesions, 141) with brain stem cavernous malformations were treated microsurgically at Beijing Neurosurgery Institute. The age distribution, lesion location, and clinical presentations were analyzed. The bleeding rate, surgical indications and microsurgical techniques were also discussed. Results In our series, 92 of 137 cases (67.2%) suffered more than one hemorrhage. Female patients had a higher risk of recurrent hemorrhage than that of male patients. Unlike cavernomas malformations from other locations, repeated hemorrhages from brain stem malformations are much more common and usually lead to new neurologic deficits. Among all 137 surgically treated patients, there was no operative mortality. Ninety-nine patients (72.3%) either improved or remained clinically stable postoperatively. The size of the cavernoma/hematoma does not necessarily correlate with the surgical result. While the acute hematoma can facilitate the surgical dissection, longer clinical history with multiple hemorrhages often makes total surgical resection difficult, partially because of the firmer capsule that may not shrink or collapse after hematoma is released. Pathologically those capsules were associated with more hyaline degeneration, fibrous proliferation and even calcifications. During the follow-up period between 0.5 to 11 years in 129 cases, 115 patients (89.2%) have been working, studying, or doing house work. Three patients (2.3%) suffered recurrent hemorrhages. Conclusion Surgical indications of brain stem cavernoma include (1) progressive neurologic deficits; (2) overt acute or subacute hemorrhage on MRI either inside or outside cavernous malformations with mass effect; (3) cavernoma/hematoma reaching brainstem surface (<2 mm brain tissue between cavernoma /hematoma and pial surface). Grave clinical presentations like coma, respiratory, or cardiac instability are not surgical contraindications. Emergent surgical evacuation may lead to satisfactory outcome. Repeated hemorrhages will worsen the pre-existing neurologic deficits and possibly make the surgical dissections more difficult. Patients with minimum, stable neurologic deficits and lesion/hematoma that has not reached the brain stem surface should be followed conservatively.

Surgical removal of brain stem cavernous malformations: surgical indications, technical considerations, and results

Objectives: This study was undertaken to review the indications for surgical treatment of brain stem cavernomas and to develop strategies to minimise the complications of surgery. Patients and results: Twelve...

Cerebral cavernomas in the adult. Review of the literature and analysis of 72 surgically treated patients

The authors review the pertinent literature dealing with all aspects of cerebral cavernous malformations in the adult. Clinical, neuroradiological, pathological, and epidemiological aspects are presented. The clinical significance of bleeding from cavernous malformations and various hemorrhage patterns are discussed in relation to the factors that influence hemorrhage rates. Recent reports describing the genetic mechanisms of inheritance, de novo formation, and angiogenesis of cavernomas are reviewed as well. Brainstem cavernomas have received special attention, since their clinical management is controversial in the literature. Presently, microsurgical removal is favored by the majority of authors and stereotactic radiosurgery appears to be inappropriate for prevention of bleeding from a cavernoma. Our own case material consists of data of 72 patients operated upon during the past 5 years. Twenty-four patients harbored the lesion within the brainstem, 18 within the deep white matter of the hemispheres, 12 in the basal ganglia or thalamus, 11 in superficial areas of the hemisphere, and seven within the cerebellum. The perioperative morbidity rate was 29.2% (21/72) while the rate of long-term morbidity was 5.5% (4/72), with no mortality in this series. It is concluded that cerebral cavernous malformations, including lesions in critical regions of the brain, can be treated microsurgically with excellent results and an acceptable morbidity.

Brain stem cavernoma excised by subtemporal-infratemporal approach

Brain stem cavernomas are most safely removed through the pial surface at which the cavernoma is surfacing. When a lower pontine or an upper medullary cavernoma comes to the surface of the anterior portion of the brain stem, it is difficult to reach by traditional approaches. We describe a case of mid- and lower pontine cavernoma, surfacing anteriorly, which was completely excised by the subtemporal-infratemporal approach. After making a small temporal craniotomy and a zygomatic osteotomy, the petrous carotid artery was mobilized anteriorly and the petroclival bone was drilled away to reach the anterior surface of the pons using the subtemporal-infratemporal approach. A small incision was made on the anterior surface of the pons, between the CN V and CN VI and the cavernoma was completely excised with the aid of the surgical microscope and the neuro-endoscope. Immediately after the operation, the patient had a complete abducens palsy and a mild increase of left hemiparesis, both of which resolved completely within 3 months. The patient returned to the full time work without any neurological deficit. A follow-up MRI 1 year later showed the complete excision of the cavernoma. The subtemporal-infratemporal approach is useful for anteriorly located mid to lower pontine and upper medullary cavernomas.

Surgical management of cavernous malformations of the brainstem

Cavernomas are well circumscribed lesions formed by sinusoidal vascular channels. They tend to slowly expand in size and carry a 0.7% to 1.1% annual risk of hemorrhage. Only 10% to 30% of intracranial cavernomas are located in the posterior fossa. When located in the brainstem they can cause recurrent hemorrhages and devastating neurological deficits. The authors report a series of cavernomas located in the brainstem and present a review on their epidemiology, pathogenesis, natural history, and methods of diagnosis and treatment. Although the surgical treatment of brainstem cavernomas can be associated with a significant risk, surgical resection is recommended of the lesions that have hemorrhaged or grown producing progressive deficits. The authors' experience on the surgical treatment of cavernous hemangiomas of the brainstem, indicating important aspects of intra-operative surgical techniques, is presented, including a clinical and anatomical correlation of different surgical approaches to brainstem cavernomas. [Neurol Res 2002; 24: 61-72]

Differential diagnosis of posterior fossa multiple sclerosis lesions--neuroradiological aspects.

Various infratentorial pathological conditions can mimic multiple sclerosis (MS) both clinically and radiologically. We review the inflammatory, vascular, neoplastic and metabolic conditions which show features similar to those of MS on magnetic resonance imaging (MRI). Behcet's disease, Lyme disease, progressive multifocal leukoencephalopathy, neurosarcoidosis, Whipple's disease, listeria rhombencephalitis, Bickerstaff's brainstem encephalitis, vasculitis due to systemic lupus erythematosus, and acute disseminated encephalomyelitis produce inflammatory lesions similar to those of MS in the brainstem and cerebellum. Neoplastic diseases, in particular pontine gliomas and lymphomas, can mimic MS. Vascular ischaemic lesions, either due to infarction produced by occlusion of a major posterior circulation artery or due to small vessel vasculopathy, can lead to posterior fossa lesions. The MRI changes of central pontine myelinolysis can also mimic MS. Diffuse axonal injury, radiation and chemotherapy induce lesions that resemble MS, however the clinical history will exclude these possibilities. Finally, we discuss a few conditions which are similar to MS in clinical presentation but have different MRI appearances, such as brainstem cavernomas, posterior fossa tumoural lesions, aneurysms and vascular loops producing neurovascular conflicts. Analysis of the MRI findings with clinical history and laboratory data helps to narrow down the diagnosis of the infratentorial pathology.

Surgical management of brainstem cavernomas.

A careful retrospective analysis of 36 cases was performed to evaluate the pre- and postoperative rates of morbidity that occur in patients with brainstem cavernous angiomas. The authors evaluated immediate postoperative and follow-up outcomes with regard to clinical findings, the incidence of preoperative hemorrhage(s), location and size of the lesions, and the timing of the surgical procedure after the last hemorrhagic event. Specifically. the following parameters were analyzed: 1) number of hemorrhages; 2) the precise brainstem location (pontomesencephalic, pons, and medulla oblongata); 3) pre- and postoperative cranial nerve status; 4) pre- and postoperative motor and sensory deficits; 5) size (volume) of the lesions; and 6) pre- and postoperative Karnofsky Performance Scale (KPS) scores. Multiple hemorrhages were observed in 16 patients, particularly in those with pontomesencephalic cavernous angiomas (75%). The mean preoperative KPS score was 70.3 +/- 16.3 (+/- standard deviation). Twenty-six patients (72.2%) presented with cranial nerve impairment, 13 (36.1%) with motor deficits, and 17 (47.2%) with sensory disturbance. Volume of the lesions ranged from 0.18 to 18.18 cm3 (mean 4.75 cm3). Postoperative complications included new cranial nerve deficits in 17 patients, motor deficits in three, and new sensory disturbances in 12 patients. In a mean follow-up period of 21.5 months, KPS scores were 80 to 100 in 22 patients. Timing of surgery (posthemorrhage) and multiple hemorrhages did not influence the long-term results. Higher preoperative KPS scores and smaller-volume lesions, however, were factors associated with a better final outcome (p < 0.05). Major morbidity was related mainly to preoperative status and less to surgical treatment. The incidence of new postoperative cranial nerve deficits was clearly lower than that demonstrated preoperatively because of the brainstem hemorrhages. Based on these findings, resection of brainstem cavernomas is the treatment of choice in the majority of these cases because of the high incidence of morbidity related to one or often several brainstem hemorrhages.

Transoral transclival removal of anteriorly placed cavernous malformations of the brainstem

BACKGROUND The natural history of brain stem cavernous malformations is unfavorable because of their high hemorrhage rate and resulting neurological deterioration among patients. However, direct surgery of intrinsic and anteriorly situated cavernomas is hazardous and leads to a bad postoperative outcome because of trauma to lateral and dorsally situated eloquent areas of the brain stem. METHODS We review the cases of two patients with symptomatic cavernous malformations of the anterior brain stem and describe the usefulness of a transoral-transclival approach. A 23-year-old man developed progressive hemihypaesthesia and paraesthesia, hemiparesis with gait ataxia, dysarthria, dysphonia, and dysphagia. A 38-year-old woman suffered from an acute onset of vertigo with nausea and vomiting, diplopia, and paraesthesia of the left hand and foot. In both patients, computed tomography demonstrated the presence of brain stem hemorrhage, because of cavernous malformation. Magnetic resonance imaging showed a close proximity of the lesions to the pia mater only on the ventral surface of the brain stem. RESULTS In both patients, the cavernomas could be safely approached and completely resected via a transoral transclival route. Three months after surgery, neurological examination revealed marked neurological improvement. The 23-year-old patient showed slight gait ataxia, no hemiparesis, no cranial nerve palsies; the 38-year-old woman demonstrated no neurological symptoms except for minimal motor dysfunction of the left hand. In both cases, under perioperative prophylactic antibiotics, no meningitis was observed. The patients could subsequently return to their previous employment. CONCLUSION The transoral transclival approach for ventrally situated brain stem cavernomas allows a largely atraumatic resection of the lesion.

Natural history of brainstem cavernous malformations.

To review the natural history and determine the rates of intra- and extralesional hemorrhaging of brainstem cavernous malformations (cavernomas) monitored by one neuro-ophthalmology service. A record review of all patients with brainstem cavernomas who were evaluated by a neuroophthalmology service between 1987 and 1999 was performed. We recorded the clinical symptoms and Rankin disability grade at presentation, during the worst clinical episode, and at the last follow-up examination. Magnetic resonance imaging scans were reviewed for evidence of intralesional hemorrhage (a bleeding episode), edema, or venous anomalies, and the cavernoma size was assessed. Thirty-seven patients (age range, 6-73 yr; mean age at presentation, 37.5 yr) underwent a mean of 4.9 years of follow-up monitoring. At presentation, there were 27 bleeding events and 8 nonhemorrhagic events; 2 patients did not exhibit symptoms. Patients who were at least 35 years of age exhibited a lower risk of bleeding episodes (odds ratio, 0.15; 95% confidence interval, 0.1-0.4). Cavernomas of at least 10 mm were associated with a higher risk of bleeding episodes (odds ratio, 3.48; 95% confidence interval, 1.3-9.4). Thirty-nine bleeding episodes occurred in 31 patients, yielding a bleeding rate of 2.46%/yr. There were eight rebleeding episodes, yielding a rebleeding rate of 5.1%/yr. Three patients experienced extralesional bleeding episodes; all of these patients experienced rebleeding. Of the 39 follow-up magnetic resonance imaging scans, the cavernoma size was unchanged in 66.7%, smaller in 18%, and larger in 15%. At the last follow-up examination, the mean Rankin grade was 1.0 for all patients, 0.6 for the 25 nonsurgically treated patients, and 1.4 for the 12 surgically treated patients. Rebleeding is not more common among patients who first present with bleeding, and it often has little effect on the neurological status of patients. Significant morbidity attributable to a brainstem cavernoma occurred in 8% of patients during follow-up monitoring of medium duration.

Gamma Knife Radiosurgery of the Brain Stem Cavernomas

Over 6 years (1992-1998) 26 patients with brain stem cavernomas were treated using the Leksell gamma knife in Prague. 25 patients had a follow up of 6-66, median 24 months. Annual risk of bleeding before radiosurgery was 4%. After gamma knife treatment sudden impairment of neurodeficit reported as rebleeding was observed in 4 patients at 6-51 months, median 16.5 months, after radiosurgery. This represented a 6.8% risk of rebleeding after radiosurgery, which is not significantly different from the risk before radiosurgery. MRI or CT was performed in 24 patients 6-48, median 24, months after radiosurgery. There were no signs of rebleeding in any of the patients, nor any increase of the cavernoma. A decrease of cavernoma size was observed in 8 (33%) of patients. Temporary collateral edema after radiosurgery was detected in 5 (21%) of patients 3-12, median 11, months after radiosurgery. Neurodeficit was observed in 21 of 26 patients before radiosurgery. Improvement of the neurodeficit was detected in 9 (43%) of them 6-36, median 8, months after radiosurgery. Temporary morbidity caused by collateral edema or rebleeding occurred in 7 patients (28%) and permanent morbidity remained in 2 patients (8%). 2 patients died because of rebleeding 6 and 51 months after radiosurgery and the third patient for unrelated reason. Radiosurgery of the brain stem cavernomas was indicated when there was bleeding in the history or progressive neurodeficit and microsurgery was considered too risky. Leksell gamma knife radiosurgery of cavernomas has proved its low morbidity and zero mortality. In case of an insufficient effect of radiosurgery, or if the protective effect from rebleeding comes too late, morbidity and mortality can correspond to the natural course of the disease, as it was left without any treatment.

Intracranial cavernous malformations - natural history and management

Thirteen papers on different aspects of cerebral cavernomas are reviewed; interest in this condition has increased since magnetic resonance imaging (MRI) became widely available. The prevalence of cavernomas is uncertain, but they are the most common of the angiographically occult vascular malformations. Some are isolated occurrences, and some are familial, with a dominant inheritance. Screening with MRI of first-degree relatives is justified. The reported annual risk of haemorrhage varies widely and is probably between 1 and 3%, with a possible increase in risk after a first haemorrhage; the risk may also be higher in deep or brain stem cavernomas. Opinions on when surgery should be offered vary, with prophylactic surgery not usually recommended. After symptomatic haemorrhages, surgery may be more justified for easily accessible lesions. For those that are more difficult to approach safely, especially in the brain stem, operation is not usually recommended unless there has been at least one clinically significant haemorrhage. Epilepsy owing to hemisphere cavernomas can often be successfully managed medically, with surgery reserved for intractable seizures. In children with epilepsy, there is a stronger argument for surgery. Radiosurgery has been used for symptomatic cavernomas that are surgically inaccessible. Strong arguments have been advanced both for and against this treatment, and the risks probably outweigh the benefits. Suggestions for a randomised trial have been made.

Surgical management of brain-stem cavernomas.

We present a series of seven patients who were operated on for symptomatic brain-stem cavernomas. The following approaches were used: medial suboccipital (N = 4), lateral suboccipital (N = 1), subtemporal-transtentorial (N = 1), and frontal transcortical-transventricular-subchorioidal-trans velum interpositum (N = 1). Intraoperative motor (N = 4) and somatosensory (N = 1) evoked potential monitoring revealed temporary changes in 3 patients. Immediately postoperatively, the following additional deficits were observed in 6 patients: oculomotor nerve paresis (N = 2), abducens nerve paresis (N = 3), facial nerve paresis (N = 2), deafness (N = 1), and increased ataxia (N = 3). One patient died due to septic complications not related to surgery. After a mean observation time of 2 years, 2 patients had improved, 3 were unchanged, and 1 patient deteriorated as compared to his preoperative status. In conclusion, surgical treatment of brain-stem cavernomas, although carrying a significant risk of temporary neurological deterioration is recommended in symptomatic patients in whom the cavernoma seems to reach the surface of the brain-stem. Intraoperative functional topographic mapping and monitoring have proven useful tools lowering the surgical risks in these patients.

Three-dimensional time-of-flight MR-angiography and the surgical indication in brainstem cavernomas

This study was designed to assess the diagnostic value of 3D time-of-flight MR-angiography in cerebral cavernomas. In seven patients, nine out of ten cavernomas were removed by microsurgery. While MR-angiography demonstrated well branches of brain arteries adjacent to the lesions, no flow signal in the vascular malformations was observed. On the other hand, there was a high intensity signal induced by methaemoglobin in those three patients with brainstem cavernomas who had experienced a significant bleeding attack seven months prior to admission. It had a spotted appearance in MR-angiography with volumes of the largest spots around 1.8 cm3. It is suggested that this spot signal could be used as a path marker for the surgical approach in brainstem cavernomas.