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Introduction

Arteriovenous malformation grading system

Robert F. Spetzler

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Robert F. Spetzler

✓ A new fixation technique for bone flaps is described. This technique avoids the use of hardware external to the skull in hairless areas where it may prove unsightly in patients with a thin scalp. The insertion of pins into the middle table of the skull firmly fixes bone flaps at one edge, eliminating the need for external plates at that site.

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Robert F. Spetzler and Hartmut Spetzler

✓ A laboratory technique that allows strain measurements of the skull is described. Holographic interferometry allows the entire surface displacement of the skull to be mapped within 1/10 of the wavelength of light. Holographic interferometric pictures are presented following various stress applications to the skull. The method, besides being exquisitely sensitive, allows strain measurements simultaneously in any desired direction over the entire skull. No physical contact with the skull is required, and the experimental set-up is simple.

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Robert F. Spetzler and Francisco A. Ponce

Object

The authors propose a 3-tier classification for cerebral arteriovenous malformations (AVMs). The classification is based on the original 5-tier Spetzler-Martin grading system, and reflects the treatment paradigm for these lesions. The implications of this modification in the literature are explored.

Methods

Class A combines Grades I and II AVMs, Class B are Grade III AVMs, and Class C combines Grades IV and V AVMs. Recommended management is surgery for Class A AVMs, multimodality treatment for Class B, and observation for Class C, with exceptions to the latter including recurrent hemorrhages and progressive neurological deficits. To evaluate whether combining grades is warranted from the perspective of surgical outcomes, the 3-tier system was applied to 1476 patients from 7 surgical series in which results were stratified according to Spetzler-Martin grades.

Results

Pairwise comparisons of individual Spetzler-Martin grades in the series analyzed showed the fewest significant differences (p < 0.05) in outcomes between Grades I and II AVMs and between Grades IV and V AVMs. In the pooled data analysis, significant differences in outcomes were found between all grades except IV and V (p = 0.38), and the lowest relative risks were found between Grades I and II (1.066) and between Grades IV and V (1.095). Using the pooled data, the predictive accuracies for surgical outcomes of the 5-tier and 3-tier systems were equivalent (receiver operating characteristic curve area 0.711 and 0.713, respectively).

Conclusions

Combining Grades I and II AVMs and combining Grades IV and V AVMs is justified in part because the differences in surgical results between these respective pairs are small. The proposed 3-tier classification of AVMs offers simplification of the Spetzler-Martin system, provides a guide to treatment, and is predictive of outcome. The revised classification not only simplifies treatment recommendations; by placing patients into 3 as opposed to 5 groups, statistical power is markedly increased for series comparisons.

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Leonardo Rangel-Castilla and Robert F. Spetzler

OBJECT

The ideal surgical approach to thalamic cavernous malformations (CMs) varies according to their location within the thalamus. To standardize surgical approaches, the authors have divided the thalamus into 6 different regions and matched them with the corresponding surgical approach.

METHODS

The regions were defined as Region 1 (anteroinferior), Region 2 (medial), Region 3 (lateral), Region 4 (posterosuperior), Region 5 (lateral posteroinferior), and Region 6 (medial posteroinferior). The senior author’s surgical experience with 46 thalamic CMs was reviewed according to this classification. An orbitozygomatic approach was used for Region 1; anterior ipsilateral transcallosal for Region 2; anterior contralateral transcallosal for Region 3; posterior transcallosal for Region 4; parietooccipital transventricularfor Region 5; and supracerebellar-infratentorial for Region 6.

RESULTS

Region 3 was the most common location (17 [37%]). There were 5 CMs in Region 1 (11%), 9 in Region 2 (20%), 17 in Region 3 (37%), 3 in Region 4 (6%), 4 in Region 5 (9%), and 8 in Region 6 (17%). Complete resection was achieved in all patients except for 2, who required a second-stage operation. The mean follow-up period was 1.7 years (range 6 months-9 years). At the last clinical follow-up, 40 patients (87%) had an excellent or good outcome (modified Rankin Scale [mRS] scores 0–2) and 6 (13%) had poor outcome (mRS scores 3–4). Relative to their preoperative condition, 42 patients (91%) were unchanged or improved, and 4 (9%) were worse.

CONCLUSIONS

The authors have presented the largest series reported to date of surgically treated thalamic CMs, achieving excellent results using this methodology. In the authors’ experience, conceptually dividing the thalamus into 6 different regions aids in the selection of the ideal surgical approach fora specific region.

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Robert F. Spetzler and Neil A. Martin

✓ An important factor in making a recommendation for treatment of a patient with arteriovenous malformation (AVM) is to estimate the risk of surgery for that patient. A simple, broadly applicable grading system that is designed to predict the risk of morbidity and mortality attending the operative treatment of specific AVM's is proposed. The lesion is graded on the basis of size, pattern of venous drainage, and neurological eloquence of adjacent brain. All AVM's fall into one of six grades. Grade I malformations are small, superficial, and located in non-eloquent cortex; Grade V lesions are large, deep, and situated in neurologically critical areas; and Grade VI lesions are essentially inoperable AVM's.

Retrospective application of this grading scheme to a series of surgically excised AVM's has demonstrated its correlation with the incidence of postoperative neurological complications. The application of a standardized grading scheme will enable a comparison of results between various clinical series and between different treatment techniques, and will assist in the process of management decision-making.

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Wouter I. Schievink and Robert F. Spetzler

Object. Isolated polycystic liver disease, that is, polycystic liver disease without kidney cysts, is an entity distinct from polycystic kidney disease. It is not known whether patients with isolated polycystic liver disease are at an increased risk for developing intracranial aneurysms, similar to patients with polycystic kidney disease. The authors screened individuals for intracranial aneurysms in a family in which isolated polycystic liver disease occurred to study the relationship between these two disorders.

Methods. Six siblings requested screening for intracranial aneurysms. Their father had died of a middle cerebral artery aneurysm. Isolated polycystic liver disease was found at autopsy. Their paternal aunt had died of a basilar artery aneurysm, but no autopsy had been performed in that case. Screening with magnetic resonance (MR) angiography and subsequent conventional angiography showed a 5-mm posterior communicating artery aneurysm in one sibling in whom abdominal ultrasound examination yielded normal findings and a posterior communicating artery infundibulum in another sibling in whom an ultrasound examination detected isolated polycystic liver disease. Screening did not detect aneurysms or polycystic liver disease in the other siblings. Thus, of the two patients with isolated polycystic liver disease in this family, one had a ruptured aneurysm and the other had an infundibulum.

Conclusions. Findings in this family suggest an association between isolated polycystic liver disease and intracranial aneurysms. However, because of the delay in onset of the appearance of liver cysts in individuals who carry the disease gene, abdominal ultrasonography is not a useful method to exclude those family members at risk for aneurysm development.