THE NATURAL-HISTORY OF KLIPPEL-FEIL SYNDROME - CLINICAL, ROENTGENOGRAPHIC, AND MAGNETIC-RESONANCE-IMAGING FINDINGS AT ADULTHOOD

被引:72
作者
GUILLE, JT [1 ]
MILLER, A [1 ]
BOWEN, JR [1 ]
FORLIN, E [1 ]
CARO, PA [1 ]
机构
[1] ALFRED I DUPONT INST,EDITORIAL SERV,WILMINGTON,DE 19899
关键词
CERVICAL SPINE; CONGENITAL DEFORMITY; KLIPPEL-FEIL SYNDROME; MAGNETIC RESONANCE IMAGING;
D O I
10.1097/01241398-199509000-00014
中图分类号
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学(修复外科学)];
学科分类号
摘要
Twenty-two patients (15 women and seven men) with Klippel-Feil syndrome were reviewed at an average age of 35 years (range, 26-57 years). The average age at diagnosis was 9 years (range, birth-32 years). All patients had a clinical neurologic and orthopaedic examination. Ten patients (45%) had abnormal findings on clinical examination, and 15 patients (68%) had at least one complaint that could be related to the syndrome. The roentgenograms were unremarkable in all patients, except for the typical findings of congenital fusion; no vertebral subluxation or stenosis was noted in any patient. The magnetic resonance images revealed degenerative changes in the disks of 100% of the patients, evidenced by a low-intensity signal on the T2-weighted images. Nineteen patients (86%) had abnormal findings on the magnetic resonance imaging scans, including disk protrusion (16 patients), osteophytes (four patients), syringomyelia (four patients), and narrowing at the level of the craniovertebral junction (six patients). Our results confirm the well-held belief that the Klippel-Feil syndrome can cause problems in adult life.
引用
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页码:617 / 626
页数:10
相关论文
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