Omovertebral bone causing traumatic compression of the cervical spinal cord and acute neurological deficits in a patient with Sprengel’s deformity and Klippel-Feil syndrome: case report

Abstract

The presence of an omovertebral bone with Sprengel’s deformity and Klippel-Feil syndrome is a complex congenital anomaly that is not well understood. It most commonly manifests as cosmetic deformity, limited range of motion, and functional disability, although there are reports of the insidious development of cervical myelopathy. In this paper, the authors present the case of a 49-year-old man with acute neurological deficits after a low-energy mechanism of traumatic spinal cord compression, resulting from an impinging omovertebral bone through a traumatic laminar defect. The patient underwent resection of the omovertebral bone, laminectomy decompression of the spinal canal, and anterior stabilization. This case highlights a rarely discussed complication of undiagnosed Sprengel’s deformity and its associated conditions following even low-energy traumatic mechanisms.

Transmandibular Cervical Corpectomy for Persistent Spinal Cord Compression in a Patient With Klippel-Feil Syndrome: A Technical Note and Systematic Review

“…4,12 In fact, these patients may also concomitantly manifest with other skeletal deformities, such as scoliosis, spina bifida, and Sprengel deformity, and other nonskeletal congenital deformities, including renal disease, loss of hearing, facial asymmetry, and heart disease. 2,7,9,[13][14][15][16][17][18][19][20][21][22][23][24][25] The timing of clinical symptoms for patients with KFS patients, with the literature reporting 20% of patients having symptoms younger than 5 years, while 65% developing symptoms younger than 30 years. 2,7,9,[13][14][15][16][17][18][19][20][21][22][23][24][25] Owing to the anomalous fusion of the cervical spine and altered biomechanics, patients with KFS are especially susceptible to cervical spinal hypermobility of nonfused segments, spondylolisthesis, stenosis, and degenerative changes, which predisposes these patients to radiculopathy and myelopathy.…”

Report of two cases with omovertebral bone and Klippel–Feil syndrome with craniovertebral junction instability

“…[ 3 11 ] The indication of surgery in patients with Sprengel's deformity and omovertebral bone is usually for cosmesis and improvement of range of motion. [ 2 3 ] Rarely, the omovertebral bone has been implicated to cause cervical myelopathy and has been treated with laminectomy and excision of the omovertebral bone. [ 1 3 ]…”

“…[ 2 3 ] Rarely, the omovertebral bone has been implicated to cause cervical myelopathy and has been treated with laminectomy and excision of the omovertebral bone. [ 1 3 ]…”

“…Approximately 20%–25% of patients with Sprengel's shoulder deformity have an omovertebral connection that is either fibrous or osseous between the superomedial border of the scapula and the spinous process, transverse process, or the lamina of the C4–7 cervical vertebrae. [ 1 2 3 ] We present a hitherto unreported association of Klippel–Feil abnormality, Sprengel's deformity, and omovertebral bone connection with basilar invagination and atlantoaxial instability. The significance of musculoskeletal alterations in cases with chronic or long-standing atlantoaxial instability is discussed on the basis of our earlier published work.…”

Klippel-Feil Syndrome

“…There have been documented complications in patients with SD associated with KFS. Several case reports have described findings of spinal cord compression from aberrant growth from an omovertebral bone or acute impingement from low-energy trauma 43-45 . All neurologic deficits improved after resection of the omovertebral bone and decompression of the spine.…”