Cervical Spinal Cord Dimensions and Clinical Outcomes in Adults with Klippel-Feil Syndrome: A Comparison with Matched Controls
24 December 2013
20 May 2014
23 July 2014 (eFirst)
Study Design Retrospective case–control study.
Objectives To confirm the fact that spinal cord dimensions are smaller in adults with Klippel-Feil syndrome (KFS) than in pediatric patients with KFS and to compare the clinical characteristics and outcomes of neurologic complications in patients with KFS with matched controls.
Methods We performed an independent 1:2 case–control retrospective radiographic and chart review of a consecutive series of adults with KFS who underwent surgical intervention. The control group consisted of consecutive non-KFS surgical patients. Patients were matched in 1:2 case–control manner. Their charts were reviewed and the clinical characteristics were compared. Axial T2-weighted magnetic resonance imaging (MRI) was used to measure the anteroposterior and mediolateral axial spinal cord and spinal canal at the operative levels and measurements were compared.
Results A total of 22 patients with KFS and 44 controls were identified. The KFS group had a tendency of more myeloradiculopathy, and the control group had a tendency toward more radiculopathy. Both tendencies, however, were not significantly different. MRIs of 10 patients from the KFS group and 22 controls were available. There was no difference in the area of both spinal cord and canal at the operative levels.
Conclusion Contrary to the finding in previous reports on pediatric patients, there were no differences between KFS and well-matched control groups in terms of age of onset, presentation, revision rate, complication rate, surgical outcome, and cross-sectional spinal cord and canal dimensions at the operative level.
- 1 Pizzutillo PD. Klippel-Feil syndrome. In: Clark C, ed. The Cervical Spine. 4th ed. Philadelphia, PA: Lippincott Williams, & Wilkins; 2005: 448-458
- 2 Klippel M, Feil A. Un cas d'absence des vertebres cervicales. Avec cage thoracique remontant jusqu'a la base du crane (cage thoracique cervicale). Nouv Iconog Salpetriere 1912; 25: 223-250
- 3 Das A, Das D, Das S , et al. Klippel-Feil syndrome: a case report and current understanding of molecular genetic background. J Indian Med Assoc 2007; 105 (4) 213-214 , 222
- 4 Duncan PA. Embryologic pathogenesis of renal agenesis associated with cervical vertebral anomalies (Klippel-Feil phenotype). Birth Defects Orig Artic Ser 1977; 13 (3D) 91-101
- 5 Falk RH, Mackinnon J. Klippel-Feil syndrome associated with aortic coarctation. Br Heart J 1976; 38 (11) 1220-1221
- 6 Haktanir A, Değirmenci B, Albayrak R, Acar M, Yucel A. Klippel-Feil syndrome associated with pneumatocyst of the right cervical rib. South Med J 2005; 98 (11) 1132-1134
- 7 Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome; a constellation of associated anomalies. J Bone Joint Surg Am 1974; 56 (6) 1246-1253
- 8 Ulmer JL, Elster AD, Ginsberg LE, Williams III DW. Klippel-Feil syndrome: CT and MR of acquired and congenital abnormalities of cervical spine and cord. J Comput Assist Tomogr 1993; 17 (2) 215-224
- 9 Rouvreau P, Glorion C, Langlais J, Noury H, Pouliquen JC. Assessment and neurologic involvement of patients with cervical spine congenital synostosis as in Klippel-Feil syndrome: study of 19 cases. J Pediatr Orthop B 1998; 7 (3) 179-185
- 10 Pizzutillo PD, Woods M, Nicholson L, MacEwen GD. Risk factors in Klippel-Feil syndrome. Spine (Phila Pa 1976) 1994; 19 (18) 2110-2116
- 11 Baba H, Maezawa Y, Furusawa N, Chen Q, Imura S, Tomita K. The cervical spine in the Klippel-Feil syndrome. A report of 57 cases. Int Orthop 1995; 19 (4) 204-208
- 12 Auerbach JD, Hosalkar HS, Kusuma SK, Wills BP, Dormans JP, Drummond DS. Spinal cord dimensions in children with Klippel-Feil syndrome: a controlled, blinded radiographic analysis with implications for neurologic outcomes. Spine (Phila Pa 1976) 2008; 33 (12) 1366-1371
- 13 Nagib MG, Maxwell RE, Chou SN. Identification and management of high-risk patients with Klippel-Feil syndrome. J Neurosurg 1984; 61 (3) 523-530
- 14 Theiss SM, Smith MD, Winter RB. The long-term follow-up of patients with Klippel-Feil syndrome and congenital scoliosis. Spine (Phila Pa 1976) 1997; 22 (11) 1219-1222
- 15 Prasad SS, O'Malley M, Caplan M, Shackleford IM, Pydisetty RK. MRI measurements of the cervical spine and their correlation to Pavlov's ratio. Spine (Phila Pa 1976) 2003; 28 (12) 1263-1268
- 16 Brown MW, Templeton AW, Hodges III FJ. The incidence of acquired and congenital fusions in the cervical spine. Am J Roentgenol Radium Ther Nucl Med 1964; 92: 1255-1259
- 17 Agrawal A, Badve AM, Swarnkar N, Sarda K. Disc prolapse and cord contusion in a case of Klippel-Feil syndrome following minor trauma. Indian J Orthop 2009; 43 (2) 210-212
- 18 Elster AD. Quadriplegia after minor trauma in the Klippel-Feil syndrome. A case report and review of the literature. J Bone Joint Surg Am 1984; 66 (9) 1473-1474
- 19 Louw JA, Albertse H. Traumatic quadriplegia after minor trauma in the Klippel-Feil syndrome. S Afr Med J 1987; 72 (12) 889-890
- 20 Gupta SN, Piatt Jr JH, Belay B. Cervical spinal cord neurapraxia in the setting of Klippel-Feil anomaly: a diagnostic and therapeutic challenge. Spinal Cord 2007; 45 (9) 637-640
- 21 Matsumoto K, Wakahara K, Sumi H, Shimizu K. Central cord syndrome in patients with Klippel-Feil syndrome resulting from winter sports: report of 3 cases. Am J Sports Med 2006; 34 (10) 1685-1689
- 22 Kidd D, Thorpe JW, Thompson AJ , et al. Spinal cord MRI using multi-array coils and fast spin echo. II. Findings in multiple sclerosis. Neurology 1993; 43 (12) 2632-2637
- 23 Kidd D, Thorpe JW, Kendall BE , et al. MRI dynamics of brain and spinal cord in progressive multiple sclerosis. J Neurol Neurosurg Psychiatry 1996; 60 (1) 15-19
- 24 Stevenson VL, Leary SM, Losseff NA , et al. Spinal cord atrophy and disability in MS: a longitudinal study. Neurology 1998; 51 (1) 234-238
- 25 Kanchiku T, Taguchi T, Kaneko K, Fuchigami Y, Yonemura H, Kawai S. A correlation between magnetic resonance imaging and electrophysiological findings in cervical spondylotic myelopathy. Spine (Phila Pa 1976) 2001; 26 (13) E294-E299
- 26 Okada Y, Ikata T, Yamada H, Sakamoto R, Katoh S. Magnetic resonance imaging study on the results of surgery for cervical compression myelopathy. Spine (Phila Pa 1976) 1993; 18 (14) 2024-2029
- 27 Uchida K, Nakajima H, Sato R , et al. Multivariate analysis of the neurological outcome of surgery for cervical compressive myelopathy. J Orthop Sci 2005; 10 (6) 564-573
- 28 Samartzis D, Kalluri P, Herman J, Lubicky JP, Shen FH. 2008 Young Investigator Award: The role of congenitally fused cervical segments upon the space available for the cord and associated symptoms in Klippel-Feil patients. Spine (Phila Pa 1976) 2008; 33 (13) 1442-1450