Prevalence of Klippel-Feil Syndrome in a Surgical Series of Patients with Cervical Spondylotic Myelopathy: Analysis of the Prospective, Multicenter AOSpine North America Study

Abstract

Study Design Prospective study. Objective To evaluate the prevalence of Klippel-Feil syndrome (KFS) in a prospective data set of patients undergoing surgical treatment for cervical spondylotic myelopathy (CSM) and to evaluate if magnetic resonance imaging (MRI) features in patients with KFS are more pronounced than those of non-KFS patients with CSM. Methods A retrospective analysis of baseline MRI data from the AOSpine prospective and multicenter CSM-North American study was conducted. All the patients presented with at least one clinical sign of myelopathy and underwent decompression surgery. The MRIs and radiographs were reviewed by three investigators. The clinical and imaging findings were compared with patients without KFS but with CSM. Results Imaging analysis discovered 5 of 131 patients with CSM (∼3.82%) had single-level congenital fusion of the cervical spine. The site of fusion differed for all the patients. One patient underwent posterior surgery and four patients received anterior surgery. Postoperative follow-up was available for four of the five patients with KFS and indicated stable or improved functional status. All five patients demonstrated pathologic changes of adjacent segments and hyperintensity signal changes in the spinal cord on T2-weighted MRI. Multiple MRI features, most notably maximum canal compromise (p = 0.05) and T2 signal hyperintensity area (p = 0.05), were worse in patients with CSM and KFS. Conclusions The high prevalence of KFS in our surgical series of patients with CSM may serve as an indication that these patients are prone to increased biomechanical use of segments adjacent to fused vertebra. This supposition is supported by a tendency of patients with KFS to present with more extensive MRI evidence of degeneration than non-KFS patients with CSM.

Change in Function, Pain and Quality of Life following Structured Nonoperative Treatment in Patients with Degenerative Cervical Myelopathy: A Systematic Review

“…[8][9][10][11][12] In contrast, the role of nonoperative treatment in these patients has not been well defined. It is therefore the objective of this review to evaluate change in function, pain, and quality of life following structured nonoperative treatment in patients with DCM.…”

“…[8][9][10][11][12] In contrast, the effectiveness of structured nonoperative treatment in stabilizing or improving symptoms is not well defined. This knowledge gap makes it challenging to determine the appropriate role of nonoperative treatment in the management of DCM, particularly in individuals with mild symptoms.…”

Change in Function, Pain, and Quality of Life Following Structured Nonoperative Treatment in Patients With Degenerative Cervical Myelopathy: A Systematic Review

“…Surgery is increasingly recommended as the preferred treatment strategy for patients with DCM as decompression not only effectively halts disease progression, but also results in significant gains in function and quality of life. 8 – 12 In contrast, the effectiveness of structured nonoperative treatment in stabilizing or improving symptoms is not well defined. This knowledge gap makes it challenging to determine the appropriate role of nonoperative treatment in the management of DCM, particularly in individuals with mild symptoms.…”

Klippel–Feil Syndrome with Multiple Cervical Anomalies Discovered Following Trauma

“…11,12 Our case presented the classic triad: A short neck, decreased range of motion, and low-posterior hairline, which is seen in up to 50% of cases with KFS. 8,10,13,14 Patients with KFS also present concomitantly with other congenital anomalies such as spina bifida, Arnold-Chiari, scoliosis, concurrent neuromuscular abnormality, Sprengel deformity, congenital torticollis, cardiovascular abnormalities, abnormalities of the urinary tract, and nervous system anomalies. 3 Regarding vertebral artery anomalies, Patil et al reported an incidence between 3 and 8% of vertebral artery anomalies.…”