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

来源：

JOURNAL OF PEDIATRIC ORTHOPAEDICS | 1995年 / 15卷 / 05期

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

页码：617 / 626

页数：10

共 30 条

[21]

Michie I., Clark M., Neurological syndromes associated with cervical and craniocervical anomalies, Arch Neurol, 18, pp. 241-247, (1968)

[22]

Mosberg W.H., The Klippel-Feil syndrome. Etiology and treatment of neurologic signs, J Nerv Ment Dis, 117, pp. 479-491, (1953)

[23]

Nagib M.G., Maxwell R.E., Chou S.N., Identification and management of high-risk patients with Klippel-Feil syndrome, J Neurosurg, 61, pp. 523-530, (1984)

[24]

Pizzutillo P.D., Klippel-Feil syndrome, The Cervical Spine, pp. 258-271, (1989)

[25]

Prusick V.R., Samberg L.C., Wesolowski D.P., Klippel-Feil syndrome associated with spinal stenosis, J Bone Joint Surg [Am], 67, pp. 161-171, (1985)

[26]

Ritterbusch J.F., McGinty L.D., Spar J., Orrison W.W., Magnetic resonance imaging for stenosis and subluxation in Klippel-Feil syndrome, Spine, 16, pp. 539-541, (1991)

[27]

Sherk H.H., Uppal G.S., Congenital bony anomalies of the cervical spine, The Adult Spine: Principles and Practice, pp. 1015-1038, (1991)

[28]

Shoul M.I., Ritvo M., Clinical and roentgenological manifestations of the Klippel-Feil syndrome (Congenital fusion of the cervical vertebrae, brevicollis), AJR am J Roentgenol, 68, pp. 369-385, (1952)

[29]

Southwell R.B., Reynolds A.F., Badger V.M., Sherman F.C., Klippel-Feil syndrome with cervical cord compression resulting from cervical subluxation in association with an omo-vertebral bone, Spine, 5, pp. 480-482, (1990)

[30]

Torg J.S., Pavlov H., Genuario S.E., Et al., Neurapraxia of the cervical spinal cord with transient quadriplegia, J Bone Joint Surg [Am], 68, pp. 1354-1370, (1986)

← 1 2 3 →