PubMed · 6972604
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R A Clark, D P Colley. 1981. Pharmacoangiography.. https://doi.org/10.1016/0037-198x(81)90018-3
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OBJECTIVE: To set up a more detailed classification system for spinal vascular malformations and to discuss the relationship between the classification and therapeutic alternatives. METHODS: Case series from the tertiary care center were studied. Spinal vascular malformations in 80 patients were reviewed and classified into intramedullary arteriovenous malformations (AVMs) (juvenile and glomus AVMs), intradural arteriovenous fistulas (AVFs) (Type I-III), dural AVFs, paravertebral AVMs, and Cobb's syndrome. Therapeutic alternatives to each type of spinal vascular malformations, including surgery, endovascular embolization, and a combination of both, as well as the short-term outcomes, were analyzed. RESULTS: Of the 80 patients, 38 (47.5%) had intramedullary AVMs (23 juvenile and 15 glomus AVMs), 27 (33.8%) had intradural AVFs (6 Type I, 14 Type II, and 7 Type III), 8 (10%) had dural AVFs, and 7 (8.8%) had other malformations. Most of the juvenile AVMs and the Types II and III AVFs were treated by endovascular embolization. Other lesions were treated by surgery or preoperative embolization plus surgery. Among the 76 patients whom we treated, early clinical improvement was achieved in 66 (86.8%). Six (7.9%) remained stable, and four (5.3%) became worse. CONCLUSION: Our classification system offers only limited guidance for the treatment of spinal vascular malformations. Successful treatment in each individual spinal vascular malformation requires correct understanding of the lesion's anatomic location and its angioarchitecture, as well as the limitations of both surgery and endovascular embolization.
We described four offspring of a consanguineous couple with arterial tortuosity "syndrome" (ATS). The affected children had extensive arterial involvement although the clinical presentations were quite variable. Clinical manifestations included cutis laxa or soft/thin skin, joint laxity or contractures, and arachnodactyly. Aortic tortuosity and pulmonary artery aneurysms with or without peripheral stenoses were demonstrated in all four sibs. All three males had inguinal hernias. Inconsistent facial anomalies were downslanting palpebral tissues, beaked nose, micrognathia, and high-arched palate. Results of collagen type I and type III biosynthesis studies were normal on skin fibroblasts. Histologic findings on autopsy of one affected child showed arterial changes with disruption of elastic fibers of the media and fragmentation of the internal elastic membrane as well as mucosal and transmural necrosis of the stomach, small bowel, colon, and extensive necrosis of the liver. Coronary artery involvement was also seen in this child as well as biventricular hypertrophy. We conclude that ATS is an autosomal recessive connective tissue condition associated with diffuse arterial changes and involvement of the skin, joints, and other organs.
OBJECTIVE: To determine whether radiologists' interpretations of images are biased by their context and by prevalence of disease in other recently observed cases. METHODS: A test set of 24 right pulmonary arteriograms with a 33% prevalence of pulmonary emboli (PE) was assembled and embedded in 2 larger groups of films. Group A contained 16 additional arteriograms, all showing PE involving the right lung, so that total prevalence was 60%. Group B contained 16 additional arteriograms without PE so that total prevalence was 20%. Six radiologists were randomly assigned to see either group first and then "cross over" to review the other group after a hiatus of at least 8 weeks. The direction of changes in a 5-point rating scale for the 2 readings of each film in the test set was compared with the sign test; mean sensitivity, specificity, and areas under receiver operating characteristic (ROC) curves were compared with the paired t test. RESULTS: In the context of group A's higher disease prevalence, radiologists shifted more of their diagnoses toward higher suspicion than expected by chance (P=.03, sign test). In group A, mean sensitivity for diagnosing PE was significantly higher (75% vs 60%; P=.04), and area under the ROC curve was significantly larger (0.88 vs 0.82; P=.02). CONCLUSIONS: Radiologists' diagnoses are significantly influenced by the context of interpretation, even when spectrum and verification bias are avoided. This "context bias" effect is unique to the evaluation of subjectively interpreted tests, and illustrates the difficulty of obtaining unbiased estimates of diagnostic accuracy for both new and existing technologies.