Limits...
Syncope caused by congenital anomaly at the craniovertebral junction: a case report.

Miyakoshi N, Hongo M, Kasukawa Y, Shimada Y - J Med Case Rep (2014)

Bottom Line: A physical examination showed generalized hyperreflexia, but motor and sensory examinations were normal.In this case, the arteries were nearly pinched between the C1 posterior arch and the pars interarticularis of the C2.C1 laminectomy and occiput-cervical fusion (O-C2) was performed using an instrumentation system.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Orthopedic Surgery, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan. miyakosh@doc.med.akita-u.ac.jp.

ABSTRACT

Introduction: Anomalies in the craniovertebral junction may be a rare cause of syncope. The mechanisms of syncope related to craniovertebral junction anomaly remain unknown.We present an extremely rare case with anomaly in the craniovertebral junction and syncope, and discuss the mechanism of the syncope.

Case presentation: A 10-year-old Japanese boy with a congenital anomaly in the craniovertebral junction presented with recurrent syncope. A physical examination showed generalized hyperreflexia, but motor and sensory examinations were normal. Computed tomography and magnetic resonance imaging showed basilar invagination and spinal cord compression at his craniovertebral junction. Three-dimensional computed tomography angiography revealed an anomalous course of his bilateral vertebral arteries, both of which showed a persistent first intersegmental artery that entered the spinal canal at the caudal portion of the C1 posterior arch. In this case, the arteries were nearly pinched between the C1 posterior arch and the pars interarticularis of the C2. C1 laminectomy and occiput-cervical fusion (O-C2) was performed using an instrumentation system. After surgery, the syncope was not observed.

Conclusions: Syncope can be related to compression of extracranial arteries within the neck. In this case, transient brain ischemia caused by the anomalous course of vertebral arteries that were pinched between the C1 posterior arch and the pars interarticularis of C2 in cervical motion was the suspected cause of the syncope.

Show MeSH

Related in: MedlinePlus

Preoperative imaging of the craniovertebral junction. (A) Lateral radiography shows malalignment of the craniovertebral junction. (B) Midsagittal reconstruction from computed tomography shows an anteriorly tilted odontoid process of C2 and migration of a hypoplastic C1 posterior arch into the foramen magnum. (C) Posterolateral view of three-dimensional computed tomography shows migration of the C1 posterior arch into the foramen magnum and rachischisis (spina bifida) of the C1 posterior arch. (D) Midsagittal T2-weighted magnetic resonance imaging shows compression of the spinal cord by the basilar invagination and an anteriorly shifted C1 posterior arch. (E) Posteroinferior view of three-dimensional computed tomography angiography shows anomalous courses of bilateral vertebral arteries (persistent first intersegmental artery), which were located between the C1 posterior arch and the pars interarticularis of C2. Asterisks, odontoid process; arrowheads, C1 anterior arch.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
getmorefigures.php?uid=PMC4196201&req=5

Figure 1: Preoperative imaging of the craniovertebral junction. (A) Lateral radiography shows malalignment of the craniovertebral junction. (B) Midsagittal reconstruction from computed tomography shows an anteriorly tilted odontoid process of C2 and migration of a hypoplastic C1 posterior arch into the foramen magnum. (C) Posterolateral view of three-dimensional computed tomography shows migration of the C1 posterior arch into the foramen magnum and rachischisis (spina bifida) of the C1 posterior arch. (D) Midsagittal T2-weighted magnetic resonance imaging shows compression of the spinal cord by the basilar invagination and an anteriorly shifted C1 posterior arch. (E) Posteroinferior view of three-dimensional computed tomography angiography shows anomalous courses of bilateral vertebral arteries (persistent first intersegmental artery), which were located between the C1 posterior arch and the pars interarticularis of C2. Asterisks, odontoid process; arrowheads, C1 anterior arch.

Mentions: Plain lateral radiography of his CVJ showed abnormal alignment with a clivo-axial angle (the angle formed by lines drawn along the clivus and the posterior aspect of the odontoid process) of 112° (normal, 150 to 180°; Figure 1A)[3]. Lateral radiographies in flexion and extension positions showed significant instability between C1 and C2. Computed tomography (CT) and three-dimensional (3D)-CT of his CVJ showed an anteriorly tilted odontoid process of C2 and a hypoplastic C1 posterior arch with rachischisis (spina bifida) that was migrating into his foramen magnum (Figures 1B and1C). His bilateral lateral atlantoaxial joints were dislocated anteriorly and his C1 anterior arch was hypertrophied. Magnetic resonance imaging showed basilar invagination and compression of his spinal cord at the C1 level (Figure 1D). Subsequent 3D-CT angiography showed abnormal courses of bilateral vertebral arteries (VAs; Figure 1E). Both VAs showed a “first persistent intersegmental artery” that entered his spinal canal at the caudal portion of the C1 posterior arch after emerging from the C2 transverse foramen, without passing through the C1 transverse foramen. These arteries appeared to be nearly pinched between the C1 posterior arch and the pars interarticularis of C2. The VAs at the subaxial spine were not hypoplastic. Based on these findings, we suspected that the cause of syncope was this arterial anomaly at his CVJ, and transient brain ischemia could be caused by these anomalous courses of the VAs being pinched between the C1 posterior arch and the pars interarticularis of C2 during cervical motions, especially in flexion. Posterior decompression and occiput-cervical fusion was therefore planned for decompression of the spinal cord and to prevent compression of the first persistent intersegmental arteries during motion.


Syncope caused by congenital anomaly at the craniovertebral junction: a case report.

Miyakoshi N, Hongo M, Kasukawa Y, Shimada Y - J Med Case Rep (2014)

Preoperative imaging of the craniovertebral junction. (A) Lateral radiography shows malalignment of the craniovertebral junction. (B) Midsagittal reconstruction from computed tomography shows an anteriorly tilted odontoid process of C2 and migration of a hypoplastic C1 posterior arch into the foramen magnum. (C) Posterolateral view of three-dimensional computed tomography shows migration of the C1 posterior arch into the foramen magnum and rachischisis (spina bifida) of the C1 posterior arch. (D) Midsagittal T2-weighted magnetic resonance imaging shows compression of the spinal cord by the basilar invagination and an anteriorly shifted C1 posterior arch. (E) Posteroinferior view of three-dimensional computed tomography angiography shows anomalous courses of bilateral vertebral arteries (persistent first intersegmental artery), which were located between the C1 posterior arch and the pars interarticularis of C2. Asterisks, odontoid process; arrowheads, C1 anterior arch.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4196201&req=5

Figure 1: Preoperative imaging of the craniovertebral junction. (A) Lateral radiography shows malalignment of the craniovertebral junction. (B) Midsagittal reconstruction from computed tomography shows an anteriorly tilted odontoid process of C2 and migration of a hypoplastic C1 posterior arch into the foramen magnum. (C) Posterolateral view of three-dimensional computed tomography shows migration of the C1 posterior arch into the foramen magnum and rachischisis (spina bifida) of the C1 posterior arch. (D) Midsagittal T2-weighted magnetic resonance imaging shows compression of the spinal cord by the basilar invagination and an anteriorly shifted C1 posterior arch. (E) Posteroinferior view of three-dimensional computed tomography angiography shows anomalous courses of bilateral vertebral arteries (persistent first intersegmental artery), which were located between the C1 posterior arch and the pars interarticularis of C2. Asterisks, odontoid process; arrowheads, C1 anterior arch.
Mentions: Plain lateral radiography of his CVJ showed abnormal alignment with a clivo-axial angle (the angle formed by lines drawn along the clivus and the posterior aspect of the odontoid process) of 112° (normal, 150 to 180°; Figure 1A)[3]. Lateral radiographies in flexion and extension positions showed significant instability between C1 and C2. Computed tomography (CT) and three-dimensional (3D)-CT of his CVJ showed an anteriorly tilted odontoid process of C2 and a hypoplastic C1 posterior arch with rachischisis (spina bifida) that was migrating into his foramen magnum (Figures 1B and1C). His bilateral lateral atlantoaxial joints were dislocated anteriorly and his C1 anterior arch was hypertrophied. Magnetic resonance imaging showed basilar invagination and compression of his spinal cord at the C1 level (Figure 1D). Subsequent 3D-CT angiography showed abnormal courses of bilateral vertebral arteries (VAs; Figure 1E). Both VAs showed a “first persistent intersegmental artery” that entered his spinal canal at the caudal portion of the C1 posterior arch after emerging from the C2 transverse foramen, without passing through the C1 transverse foramen. These arteries appeared to be nearly pinched between the C1 posterior arch and the pars interarticularis of C2. The VAs at the subaxial spine were not hypoplastic. Based on these findings, we suspected that the cause of syncope was this arterial anomaly at his CVJ, and transient brain ischemia could be caused by these anomalous courses of the VAs being pinched between the C1 posterior arch and the pars interarticularis of C2 during cervical motions, especially in flexion. Posterior decompression and occiput-cervical fusion was therefore planned for decompression of the spinal cord and to prevent compression of the first persistent intersegmental arteries during motion.

Bottom Line: A physical examination showed generalized hyperreflexia, but motor and sensory examinations were normal.In this case, the arteries were nearly pinched between the C1 posterior arch and the pars interarticularis of the C2.C1 laminectomy and occiput-cervical fusion (O-C2) was performed using an instrumentation system.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Orthopedic Surgery, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan. miyakosh@doc.med.akita-u.ac.jp.

ABSTRACT

Introduction: Anomalies in the craniovertebral junction may be a rare cause of syncope. The mechanisms of syncope related to craniovertebral junction anomaly remain unknown.We present an extremely rare case with anomaly in the craniovertebral junction and syncope, and discuss the mechanism of the syncope.

Case presentation: A 10-year-old Japanese boy with a congenital anomaly in the craniovertebral junction presented with recurrent syncope. A physical examination showed generalized hyperreflexia, but motor and sensory examinations were normal. Computed tomography and magnetic resonance imaging showed basilar invagination and spinal cord compression at his craniovertebral junction. Three-dimensional computed tomography angiography revealed an anomalous course of his bilateral vertebral arteries, both of which showed a persistent first intersegmental artery that entered the spinal canal at the caudal portion of the C1 posterior arch. In this case, the arteries were nearly pinched between the C1 posterior arch and the pars interarticularis of the C2. C1 laminectomy and occiput-cervical fusion (O-C2) was performed using an instrumentation system. After surgery, the syncope was not observed.

Conclusions: Syncope can be related to compression of extracranial arteries within the neck. In this case, transient brain ischemia caused by the anomalous course of vertebral arteries that were pinched between the C1 posterior arch and the pars interarticularis of C2 in cervical motion was the suspected cause of the syncope.

Show MeSH
Related in: MedlinePlus