Congenital Spondylolytic Spondylolisthesis of C2 Vertebra Associated With Atlanto-Axial Dislocation, Chiari Type I Malformation, and Anomalous Vertebral Artery: Case Report With Review Literature.

BACKGROUND: Congenital spondylolytic spondylolisthesis of C2 vertebra resulting from deficient posterior element of the axis is rarely described in the literature. CASE DESCRIPTION: We describe a unique case of agenesis of posterior elements of C2 with craniovertebral junction anomalies consisting of osseous, vascular, and soft tissue anomalies. A 26-year-old man presented with symptoms of upper cervical myelopathy of 12 months' duration. A computed tomography scan of the cervical spine including the craniovertebral junction revealed spondylolisthesis of C2 over C3, atlantoaxial dislocation, occipitalization of the atlas, hypoplasia of the odontoid, and cleft posterior C1 arch. Additionally, the axis vertebra was found devoid of its posterior elements except bilaterally rudimentary pedicles. Magnetic resonance imaging revealed tonsilar herniation, suggesting associated Chiari type I malformation. CT angiogram of the vertebral arteries displayed persistent bilateral first intersegmental arteries crossing the posterior aspect of the C1/2 facet joint. This patient underwent foramen magnum decompression, C3 laminectomy with occipito-C3/C4 posterior fusion using screw and rod to maintain the cervical alignment and stability. CONCLUSION: We report this rare constellation of congenital craniovertebral junction anomaly and review the relevant literature.

Cervical congenital spondylolytic spondylolisthesis associated with duplication of the vertebral artery: case report.

BACKGROUND CONTEXT: Cervical bilateral congenital spondylolysis with spondylolisthesis is an abnormality both of congenital and mechanical origin, characterized by its primary feature, cervical bilateral spondylolysis. We are unaware of any reports describing cervical congenital spondylolytic spondylolisthesis associated with duplication of the vertebral artery. PURPOSE: To report the case of a patient affected with cervical bilateral congenital spondylolysis with spondylolisthesis associated with duplication of the vertebral artery. STUDY DESIGN: A unique case report from a university hospital and a literature review. PATIENT SAMPLE: An 18-year-old man who arrived at the emergency department complaining of neck pain starting from a car accident 5 days ago. METHODS: Neurologic examination and images taken by ordinary radiographs, magnetic resonance imaging (MRI) scans, ordinary computed tomography (CT) scans, and CT angiograms with three-dimensional (3D) reconstruction. RESULTS: Neurologic examination did not find evidence of strength deficit in upper extremities. Ordinary radiographs of the cervical spine showed spondylolisthesis of C6 and C7 and a cortical cleft between the superior and inferior articular facets of the C6 vertebra and spina bifida of the C6 and C2 vertebrae and an abnormal appearance of the remnant spinous processes of the cervical vertebrae. Magnetic resonance imaging confirmed the abnormalities that had been noted on the radiographs. Computed tomography scans of the cervical spine showed congenital spondylolytic spondylolisthesis and spina bifida of the C6 vertebra and duplication of the vertebral artery. They also showed double origins of the vertebral artery depicted by 3D angiographic reconstruction. Conservative treatment of wearing a cervical collar and receiving muscle relaxants and anti-inflammatory drugs was effective. With the pain completely subsided, the patient was discharged 5 days after arriving at the emergency department. CONCLUSIONS: Vascular abnormalities should be suspected and investigated in cases of congenital spondylolysis or spondylolytic spondylolisthesis. We strongly suggest performing angio-CT or angio-MRI and 3D reconstruction in these cases. Awareness of the presence of a duplicated vertebral artery and the course of its limbs could significantly help planning in cases proceeding to surgery.

Embryology of the spine and associated congenital abnormalities.

BACKGROUND CONTEXT: The spine is a complex and vital structure. Its function includes not only structural support of the body as a whole, but it also serves as a conduit for safe passage of the neural elements while allowing proper interaction with the brain. Anatomically, a variety of tissue types are represented in the spine. Embryologically, a detailed cascade of events must occur to result in the proper formation of both the musculoskeletal and neural elements of the spine. Alterations in these embryologic steps can result in one or more congenital abnormalities of the spine. Other body systems forming at the same time embryologically can be affected as well, resulting in associated defects in the cardiopulmonary system and the gastrointestinal and genitourinary tracts. PURPOSE: This article is to serve as a review of the basic embryonic development of the spine. We will discuss the common congenital anomalies of the spine, including their clinical presentation, as examples of errors of this basic embryologic process. STUDY DESIGN/SETTING: Review of the current literature on the embryology of the spine and associated congenital abnormalities. METHODS: A literature search was performed on the embryology of the spine and associated congenital abnormalities. RESULTS: Development of the spine is a complex event involving genes, signaling pathways and numerous metabolic processes. Various abnormalities are associated with errors in this process. CONCLUSION: Physicians treating patients with congenital spinal deformities should have an understanding of normal embryologic development as well as common associated abnormalities.

High-grade dysplastic spondylolisthesis and spondyloptosis: report of three cases with surgical treatment and review of the literature.

High-grade dysplastic spondylolisthesis is extremely rare and always involves the L5-S1 level. It is attributed to congenital dysplasia of the superior articular process of the sacrum. It can remain asymptomatic for a long time and can progress to a more severe grade of olisthesis and spondyloptosis. Surgical treatment has varied from posterior-only in situ fusion to anterior and posterior fusion with complete reduction. Three cases of symptomatic high-grade (4th and 5th grade) dysplastic spondylolisthesis treated surgically with reduction and fusion are presented. Interbody fusion at the level of olisthesis is crucial.

Spondylolisthesis in pre-school children.

Although the natural history of spondylolisthesis is poorly described, we know that it is rare to see the condition in children before they are 5 or 6 years old. Treatment in these patients may be confusing because there is very little literature on the subject, and most papers are case reports. We present a series of eight pre-school patients (mean age 3.5 years; range 9 months to 5 years) with spondylolisthesis with an average follow up of 11.5 years (range 9-14 years). In our experience, general guidelines for treatment as given by Wiltse and Jackson are useful in patients younger than 5 years. Nowadays we have to keep strict control of these patients due to their important capacity for growth that increases the possibility of further slippage.

Treatment of congenital spondyloptosis in an 18-month-old patient with a 10-year follow-up.

STUDY DESIGN: Case report. OBJECTIVES: To present the case of a patient with congenital spondyloptosis treated and followed over 10 years. SUMMARY OF BACKGROUND DATA: The surgical management of spondyloptosis in children is variably reported in the literature. Some authors propose that posterior fusion in situ is a safe and reliable procedure, whereas others suggest that reduction of the slipped vertebra may prevent some of the adverse sequelae of in situ fusion, which include nonunion, bending of the fusion mass, and persistent lumbosacral deformity. Many investigators advocate a combined anterior and posterior fusion using instrumentation. METHODS: At the time of the first symptoms an 18-month-old boy with congenital spondyloptosis of L5-S1 was referred to the authors' institution. Because of the progression of pain, neurologic disturbance, mild foot deformity, muscle contractures, and lumbosacral kyphosis, surgical intervention was undertaken. Operative intervention began with a resection of the L5 lamina and wide bilateral L5 nerve root decompression. This was followed by anterior subtotal resection of L5 and interbody bone graft of the morcelized vertebral body for fusion from L5 to S1. The next step was reduction of the spondyloptosis and stabilization by posterior instrumentation L2-S1 with a sacral Cotrel-agraffe device. RESULTS: The procedure achieved almost complete reduction of the spondyloptosis with near-normal restoration of lumbar lordosis allowing more physiologic lumbar spinal biomechanics. There were no neurologic complications. After surgery there was no suggestion of back pain or gait disturbance and no progression of any deformity. CONCLUSION: In the treatment of severe congenital spondylolisthesis a staged procedure of decompression, reduction, and instrumented fusion is recommended for those cases in which intervention is indicated.

Successful staged surgical correction of congenital segmental spinal dysgenesis and complete rotary subluxation of the thoracolumbar spine in an infant.

Congenital segmental spinal dysgenesis is a rare lesion that usually is associated with neurological deficits. Affected infants who are neurologically intact may suffer progressive kyphosis and subsequent neurological deterioration. Results of external casting, laminectomy with spinal fixation, or bone grafting of the defect have been disappointing. The authors present a successful staged surgical management scheme consisting of complete resection of the dysraphic vertebral bodies, maintenance of normal vertebral distance with rib strut grafting, and fusion with autogenous bone.

Case report 868. Congenital bilateral spondylolysis and spondylolisthesis of the fourth cervical vertebra.

A case of congenital bilateral spondylolysis of fourth cervical vertebra was reported and the characteristic radiological features shown. Although the diagnosis is often suggested by the plain films, demonstration of the typical CT findings is often necessary to reach a final diagnosis. Awareness of this entity and its specific radiological features will help to differentiate this relatively benign cervical anomaly from other, more ominous, unstable causes of cervical spondylolisthesis such as those related to acute cervical injury. It may also prevent any inappropriate treatment from being undertaken.

Congenital spondylolisthesis in the upper spinal column. Management of two cases.

Two cases of congenital spondylolisthesis involving the cervical and thoracic spinal column are presented. Both patients presented with significant neurologic deficits and responded favorably to stabilization and fusion.

Computed tomography of congenital spondylolisthesis of the sixth cervical vertebra.

Congenital anomalies of the cervical spine occur relatively commonly. An interesting anomaly is congenital spondylolisthesis of the sixth cervical vertebra. We are presenting a case of this anomaly as imaged with plain radiography and computed tomography. We have also reviewed the previous literature and attempted to clarify distinctions between bilateral cervical spondylolysis with spondylolisthesis and other cervical spine lesions.

Congenital spondylolisthesis of the 6th cervical vertebra: CT findings.

The CT demonstration of a case of congenital spondylolisthesis of the C6 vertebral body is reported. This entity includes bilateral bony defects in the pars interarticularis regions, deranged facet joints, as well as a midline bony cleft in the spinous process. This congenital deformity should be recognized and not mistaken for traumatic spondylolisthesis.

Factors affecting fusion rate in adult spondylolisthesis.

The authors examined factors affecting fusion rate in the surgical treatment of 89 consecutive adult patients with spondylolisthesis. Two factors significantly improved fusion rate: combined anterior and posterior fusion and rigid postoperative immobilization in the cast. In 65 patients with isthmic spondylolisthesis, the fusion rate was raised from 70% when posterior fusion alone was used to 88% when combined anterior and posterior fusion was used. In 20 patients with degenerative spondylolisthesis, frequent use of combined anterior and posterior fusion contributed to a high overall fusion rate of 95%. Among patients with isthmic spondylolisthesis, postoperative cast immobilization resulted in a higher fusion rate of 90% compared with a fusion rate of 63% obtained after brace immobilization.