Natural history and spontaneous prognosis of cerebral arteriovenous malformations

Eight large series of patients presenting with untreated cerebral arterio-venous malformations (A.V.M.) were reviewed. Data were gathered on 1134 patients who were followed for 20 years or more. The risk of rupture and the rate of other complications were estimated, in relation with other factors like age of patient, size of A.V.M., or hemodynamic stresses. The prevalence of A.V.M. at autopsy is 14 per 10,000 population. Fifty percent of A.V.M.s remain asymptomatic during the whole patient's life. The risk of hemorrhage from rupture of an A.V.M. is 2% per year and per patient. Hemorrhage is more frequent from small-sized A.V.M.s, mostly under certain hemodynamic factors. The risk of hemorrhage is also higher in children between 5 and 10 years of age, and in women to the end of pregnancy. The incidence of seizures and neurological impairment is approximately 1% per year and per patient, and these symptoms mostly occur in elder patients with large A.V.M.s. If the A.V.M. has not been revealed by hemorrhage, the risk of rupture is 1% per year and per patient. The incidence of complications from fortuitously diagnosed A.V.M.s seems very low, and even nil in some series. The risks of therapeutic procedures could not be determined, but in some A.V.M.s, the association of different therapeutic means seems mor advisable than isolated therapy. The analysis of natural history of A.V.M.s is desirable to provide a better information to the patient, and to define the limits of therapeutic indications.     

Sacral agenesis and caudal spinal cord malformations

Thirty-three children and one adult with sacral agenesis (SA) were studied by computed tomographic myelography and/or magnetic resonance imaging and were monitored for a mean period of 4.7 years. Four children had the OEIS (concurrent omphalocele, cloacal exstrophy, imperforate anus, and spinal deformities) complex, and three others had VATER (vertebral abnormality, anal imperforation, tracheoesophageal fistula, and renal-radial anomalies) syndrome. All patients shared some of the characteristic features of SA, namely, a short, intergluteal cleft, flattened buttocks, narrow hips, distal leg atrophy, and talipes deformities. Neurologically, lumbosacral sensation was much better preserved than the motor functions, and urinary and bowel symptoms were universal. The level of the vertebral aplasia was correlated with the motor but not with the sensory level. The important neuroimaging findings of SA were as follows: 1) 12 patients (35%) had nonstenotic, tapered narrowing of the caudal bony canal, and 2 patients had hyperostosis indenting the caudal thecal sac; 2) 16 patients (47%) had nonstenotic, tapered narrowing and shortening of the dural sac, but 3 patients (9%) had true, symptomatic dural stenosis, in which the cauda equina was severely constricted by a pencil-sized caudal dural sac; 3) the coni could be divided into those ending above the L1 vertebral body (Group 1, 14 patients) and those ending below L1 (Group 2, 20 patients). Thirteen of 14 Group 1 coni were club or wedge-shaped, terminating abruptly at T11 or T12, as if the normal tip was missing. All 20 Group 2 coni were tethered: 13 were tethered by a thick filum; 2 were extremely elongated and had a terminal hydromyelia; 3 were terminal myelocystoceles; and 2 were tethered by a transitional lipoma. High blunt coni were highly correlated with high (severe) sacral malformations (sacrum ending at S1), but low-lying tethered coni were highly correlated with low sacral malformations (S2 or lower pieces present).(ABSTRACT TRUNCATED AT 400 WORDS)     

Intramedullary cavernous malformations of the spinal cord

Cavernous malformations of the spinal cord are extremely rare lesions. The 58 reported cases in the English literature and 9 of the authors' own cases are reviewed. The clinical presentation, patient characteristics, radiographic appearance, and histopathologic features are reviewed. The optimal surgical management and outcomes of treatment for intramedullary spinal cord cavernous malformations are discussed in detail.     

Chemotherapy for spinal cord astrocytoma: can natural history be modified?

Standard treatment of spinal cord astrocytomas is based upon surgery, followed by radiotherapy when resection is incomplete or when histology is of high grade. Owing to the major consequences of radiotherapy on the spine in childhood, alternative therapies must be explored. The potential role of chemotherapy in the management of spinal cord astrocytoma remains to be defined. Two patients are described. The first was a 19-month-old child with an anaplastic astrocytoma of the cervical spinal cord that progressed rapidly after initial partial resection. Chemotherapy was begun according to the UKCCSG Baby Brain Protocol, with marked clinical improvement. Reassessment by MRI at 4 months showed improvement, and at the end of treatment no evaluable disease remained. The second was a 4-year-old child with a recurrent low-grade astrocytoma. Chemotherapy according to the SIOP Protocol for Low Grade Gliomas was administered for 3 months, after which marked tumour regression was seen, with neurological recovery. These patients demonstrate the potential value and low morbidity of chemotherapy in spinal cord astrocytoma. The management of this rare tumour is discussed.     

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.     

Vascular malformations of the spinal cord. Diagnosis and therapy

A review is given on the current diagnosis and treatment of spinal vascular malformations with special emphasis on the use of selective spinal angiography. Spinal vascular malformations are mostly encountered within the thoraco-lumbar segment of the spinal cord. Typical clinical symptoms are due to either progressive myelopathy or hemorrhage (subarachnoidal hemorrhage). The prognosis of untreated spinal angiomas is poor. Therefore, therapeutic measures are aiming to prevent bleeding of angiomas resulting in subarachnoidal hemorrhages or hematomyelia or to prevent spinal cord ischemia due to compression. Using selective spinal angiography, spinal angiomas can now be treated successfully by complete surgical extirpation or embolization procedures. The evaluation of radicular myelopathies and subarachnoidal hemorrhages of unknown origin has also considerably benefited from this technique. It is concluded that early diagnosis of spinal vascular malformations is of crucial importance for good therapeutical results.     

The natural history of multiple sclerosis and its diagnosis

The treatment of multiple sclerosis (MS) is a dynamic field that is rapidly evolving. It encompasses tow main areas. The first is the direct treatment of MS itself, having evolved from using primarily steroids to the newest immunotherapies that come from the extensive research into the immune system and the role it plays in so many diseases. The second area for treatment is in the management of the effects or symptoms resulting from MS.     

An analysis of the natural history of cavernous malformations

BACKGROUND: The treatment of cavernous malformations has been controversial. Some reports suggest that surgical resection of the lesion for the prevention of recurrent hemorrhage should not be considered because of low hemorrhagic risk. However, the role of surgery in management of cavernous malformations is undergoing reevaluation. The decision for surgical resection should be based on a careful analysis of the natural history of this lesion, which is not well understood. METHODS: We investigated, retrospectively, the natural history of 108 cavernous malformations in 62 patients. Individual cavernous malformations were divided into four categories on the basis of magnetic resonance (MR) findings. The pattern of clinical and radiologic presentation and outcomes of management were analyzed. RESULTS: The age of the patients ranged from 4-63 years (mean: 32.2 years). Multiple lesions were found in 13 of 62 patients (21%) and two of these patients were siblings. Twenty-five out of 62 patients had suffered recurrent symptoms. The bleeding rate was 2.3%/person/year (1.4%/lesion/year) during 2509.6 patient years. There were no significant differences between the bleeding rates of each type of lesion. During the follow-up period of 12-48 months (mean: 22.4 months), two of 28 patients conservatively treated had recurrent hemorrhages (rebleeding rate: 3.8%/person/year). During the follow-up period of 12-66 months (mean: 21.7 months), recurrent hemorrhages were observed in two of 17 patients with radiosurgery (rebleeding rate: 7.8%/person/year). CONCLUSION: Our study has provided a profile of the natural history of these lesions. Based on our results, we recommend surgical excision of cavernous malformations in those patients with recurrent symptoms or acute progressive symptoms.     

Arteriovenous malformations of the head and neck: natural history and management

This is a retrospective review of 81 patients with extracranial arteriovenous malformation of the head and neck who presented to the Vascular Anomalies Program in Boston over the last 20 years. This study focused on the natural history and effectiveness of treatment. The male to female ratio was 1:1.5. Arteriovenous malformations occur in anatomic patterns. Sixty-nine percent occurred in the midface, 14 percent in the upper third of the face, and 17 percent in the lower third. The most common sites were cheek (31 percent), ear (16 percent), nose (11 percent), and forehead (10 percent). A vascular anomaly was apparent at birth in 59 percent of patients (82 percent in men, 44 percent in women). Ten percent of patients noted onset in childhood, 10 percent in adolescence, and 21 percent in adulthood. Eight patients first noted the malformation at puberty, and six others experienced exacerbation during puberty. Fifteen women noted appearance or expansion of the malformation during pregnancy. Bony involvement occurred in 22 patients, most commonly in the maxilla and mandible. In seven patients, the bone was the primary site; in 15 other patients, the bone was involved secondarily. Arteriovenous malformations were categorized according to Schobinger clinical staging: 27 percent in stage I (quiescence), 38 percent in stage II (expansion), and 38 percent in stage III (destruction). There was a single patient with stage IV malformation (decompensation). Stage I lesions remained stable for long periods. Expansion (stage II) was usually followed by pain, bleeding, and ulceration (stage III). Once present, these symptoms and signs inevitably progressed until the malformation was resected. Resection margins were best determined intraoperatively by the bleeding pattern of the incised tissue and by Doppler. Subtotal excision or proximal ligation frequently resulted in rapid progression of the arteriovenous malformation. The overall cure rate was 60 percent, defined as radiographic absence of arteriovenous malformation. Cure rate for small malformations was 69 percent with excision only and 62 percent for extensive malformations with combined embolization-resection. The cure rate was 75 percent for stage I, 67 percent for stage II, and 48 percent for stage III malformations. Outcome was not affected significantly by age at treatment, sex, Schobinger stage, or treatment method. Mean follow-up was 4.6 years.     

The endovascular treatment of cerebral arteriovenous aneurysms and malformations

Despite the progress made in cerebral aneurysm microneurosurgery, some morphologic and anatomic characteristics, or also clinical reasons, make surgical clipping of the aneurysmatic column difficult or unfeasible, justifying an endovascular therapeutic alternative. Despite the great progress made, the risk of endovascular intervention with microballoons is significant, particularly in the acute post-haemorrhagic phase: 17.9% mortality and 10.7% morbidity in endosaccular embolisation therapy with the detachable balloon maintaining the arterial lumen permeable. The use of the GDC system (Guglielmi Detachable Coil) has permitted the treatment of proximal and distal aneurysms in the carotid and vertebrobasilar arterial regions. Microcatheterisation also allows intravascular treatment of the vasospasm, by mechanical means--angioplasty, or by pharmacological vasodilatation. With the GDC system one can obtain a complete occlusion of small and medium aneurysms in over 85% of cases, definitive morbidity of 5% to 7% and mortality of 1% to 3%. The objective of AVM endarterial occlusions is to obliterate the nidus through the arterial pedicles that can be microcatheterised by means of a certain embolic agent (Cyanocrilate, PVA or other embolic products). Thus, it is possible to reduce the dimension of the nidus as well as diminish the severity of the arteriovenous shunt, later facilitating the operation or radiosurgery, with the possibility of complete surgical removal in 96% of patients after embolisation. The mortality directly related to this endovascular therapy is approximately 0.9% with severe morbidity below 2%. Complete obliteration of a cerebral AVM can be achieved with endovascular techniques in 15% to 20% of cases, particularly in small lesions, sustain AVMs require careful multidisciplinary discussion aimed at finding the best treatment for each case.     

Alcohol abuse history and adjustment following spinal cord injury.

Objective: Examined the relation of alcohol abuse history to emotional adjustment and pressure sore occurrence during the 1st 3 years following Spinal Cord Injury (SCI). Study Design: Comparisons were made between varying levels of prior abuse and depression and disability acceptance. A model predicting pressure sore occurrence over 3 years was computed. Participants: One hundred seventy-five persons completed the self-report measures; 105 were available for pressure sore evaluations. Main Outcome Measures: The Inventory to Diagnose Depression, the Acceptance of Disability Scales, and pressure sore occurrence. Results: Alcohol abuse was not associated with depression or disability acceptance. Severe alcohol abuse history was associated with pressure sores over the 3 years. Conclusions: Persons with prior history of severe alcohol abuse may be at increased risk for pressure sore occurrence during the 1st years of SCI. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

The clinico-neuropsychological manifestations of arteriovenous malformations of the thalamus

A comprehensive clinical and neuropsychological study was made of 34 patients with thalamic arteriovenous malformations. In the thalamus. Memory disorders were a common abnormality (in 31 of 34 patients). They were modally nonspecific and depended on the site of arteriovenous malformations. With the prevalent ventricular pattern of hemorrhages, memory disorders were accompanied by disorientation and emotional and volitional impairments. Arteriovenous malformation was removed in 12 patients (on the left side in 7 patients and on the right in 5). After surgery there was virtually progression of memory defects in all patients. Speech disorders combining the specific features of lesions in various cortical regions were detected in 5 of 7 patients with left thalamic arteriovenous malformations.     

Embolization of congenital pulmonary arteriovenous malformations

Patients with arteriovenous pulmonary malformations are at risk of developing secondary brain disease such as transient ischemic attacks, strokes or abscesses. Lethal haemothorax and haemoptysis also occur. 12 of 14 malformations in five patients were treated using a total of eight procedures. One patient experienced a transient unilateral hemiparesis, otherwise no complications occurred. None of the 43 deployed occlusion coils was lost through the fistulas. Complete occlusion was achieved in all lesions where coils could be placed in a stable position. One patient suffered a minor recurrence. The use of interlocking detachable coils (IDC) which can be retracted or repositioned prior to full deployment is recommended.     

Surgical and embolic treatment of cerebral arteriovenous malformations

Twenty-eight cases of cerebral arteriovenous malformations in which the treatment consisted of embolization and/or surgical excision are reviewed. Embolization was considered an adjuvant procedure; carried out to reduce the size of the malformation or eliminate the deep arterial supply to it prior to excision. In sixteen of the cases the combined treatment was carried out with one death and one significant postoperative deficit. In the other twelve cases surgical operation or embolization alone was carried out. The anatomy of the lesion determined the applicability of embolization. Although embolization has a high degree of safety, certain pitfalls must be avoided. Changing patterns in the cerebral circulation following embolization and operation as well as the role of collateral circulation to cerebral arteriovenous malformations are discussed.     

Spontaneous angiographic obliteration of cerebral arteriovenous malformations

OBJECTIVE: The factors associated with spontaneous angiographic obliteration of cerebral arteriovenous malformations (AVMs) are not well understood. We present a review of the literature and a report of our experience with six cases (four with no previous treatment intervention and two postoperative residual malformations) that were identified as having occurred during a 20-year period and describe the clinical and lesion features associated with this rare phenomenon. We present the first detailed histological study of a spontaneously thrombosed AVM specimen, including immunohistochemical analysis of angiogenesis factor expression. METHODS: A combined experience in the management of approximately 700 AVMs during 20 years identified six cases of spontaneous angiographic obliteration of cerebral AVMs. A literature review revealed another 24 cases with angiographic documentation of the initial AVMs and follow-up data showing nonfilling of the lesions. Histological analysis of a recently excised lesion included immunostaining with monoclonal antibodies to the antigens of Factor VIII, Tie, vascular endothelial growth factor, and its receptors, Flt-1 and Flk. RESULTS: A single draining vein was a feature in each of our 6 cases and in 12 of 14 (86%) cases from the literature. Hemorrhage as the presenting symptom was identified in 5 of our 6 (83%) cases and in 17 of 24 (71%) of the literature cases. The size of the AVM was less than 6 cm in each of our 6 cases and in 22 of 24 (92%) of the literature cases. A histological examination of a thrombosed AVM surgical specimen revealed persistent patent vascular channels within the lesion. Immunohistochemical analysis with angiogenesis and endothelia-specific factors showed expression of these factors within the lumen of the thrombosed nidus vessels. CONCLUSION: We propose that the occlusion of a single draining vein may lead to total venous outflow obstruction and lesion thrombosis. Hemorrhagic presentation and small nidus may also predispose to this phenomenon. Immunohistochemical analysis of a thrombosed AVM revealed possible ongoing angiogenic changes within the AVM vessels 1 month after angiographically documented thrombosis. It is possible that neovascularization within a thrombosed AVM may lead to lesion recanalization; however, this phenomenon seems to be clinically exceedingly rare.     

Reflections on the management of cerebral arteriovenous malformations

BACKGROUND: The authors report their personal experience in the management of cerebral arteriovenous malformations (AVMs), using the three techniques now available: surgical resection, endovascular embolization, and radiosurgery. They review the recent literature on this topic and present their current management algorithm based on this experience. METHODS: A series of 90 patients treated for cerebral AVMs is reported (68% Grade I-III and 32% Grade IV-V, Spetzler scale). The three methods of treatment were used, either individually or in combination, based on the size and the location of the malformation. The first intervention was surgical resection in 26% of cases, endovascular embolization in 57%, and radiosurgery in 17%. Surgery and embolization were followed by another technique in some cases and eventually single modality treatment was used in 58% of cases (surgical resection 21%, endovascular embolization 20%, radiosurgery 17%) and multimodality treatment in 42% (embolization + resection, 21%; embolization + radiosurgery, 17%; resection + radiosurgery, 4%). Embolization was used as reductive therapy in 38% of the overall series (65% of all embolized patients), and was followed by surgery in 56% of cases or by radiosurgery in 44%. Angiography was used to assess the cure rates. RESULTS: The following cure rates were obtained, when each technique was used as a first treatment: surgical resection, 82%; embolization, 6%; and radiosurgery, 83% (2-year angiographic follow-up). After combined treatment, embolization and resection resulted in a 100% cure rate, embolization and radiosurgery produced a 90% cure rate. The clinical outcome was evaluated in terms of deterioration attributable to treatment. Seventy-one percent of patients had no complication, minor complications were observed in 18%, and severe complications in 11%. Treatment mortality was 3%. All deaths were attributable to hemorrhage during the embolization procedure. CONCLUSIONS: In this management algorithm, AVMs submitted directly to surgery or to radiosurgery were considered "good risk" malformations, and the outcome for these cases was good in terms of clinical result and cure rate. AVMs submitted first to endovascular embolization were considered "poor risk" malformations, including a majority of Spetzler Grade IV-V lesions. Not surprisingly, the majority of severe complications occured in this group during embolization. Thus, the major risk of the treatment of AVMs has now shifted from surgery to endovascular techniques. Endovascular embolization as sole treatment gave a low rate of complete occlusion, but proved to be very useful as a reductive therapy, in preparation for further surgery or radiosurgery. Partial embolization permitted high rates of complete cure in difficult AVMs. Embolization should be used to the maximum extent possible as a reductive technique, despite the risks of the procedure. Because of its risks however, this technique of reductive embolization should be used only if absolutely necessary to allow the complete cure of the malformation. Thus, the use of embolization should be considered very cautiously in small malformations as well as in very large and complex AVMs in which partial embolization will not be sufficient to allow complete cure with either endovascular or surgical techniques.     

Interactive image-guided surgical resection of intracranial arteriovenous malformations

Febrile seizures occur in 2 to 5% of children ages three months to five years. Evaluation of children with febrile seizures begins with a thorough history and physical examination. Therapy and long-term management should focus on treatment of the febrile illness and counseling.