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

Postoperative imaging of the craniovertebral junction. (A) Lateral radiography shows improved alignment of the craniovertebral junction. (B) A midsagittal reconstruction of computed tomography obtained 4 months postoperatively shows complete bone union.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Postoperative imaging of the craniovertebral junction. (A) Lateral radiography shows improved alignment of the craniovertebral junction. (B) A midsagittal reconstruction of computed tomography obtained 4 months postoperatively shows complete bone union.

Mentions: C1 laminectomy and posterior occiput-cervical fusion (O-C2) were performed using an instrumentation system (VERTEX® MAX; Medtronic Sofamor Danek, Memphis, TN, USA). Intraoperatively, the migrated C1 posterior arch was repositioned after skull traction and we were able to reach the upper surface of C1. Decompression of his foramen magnum was therefore not required for C1 laminectomy. A portion of the C1 posterior arch rachischisis consisted of tendon-like tissue that adhered severely to the dura. During C1 laminectomy, the locations of VAs were detected using Doppler ultrasonography. C2 pedicle screws were inserted with the assistance of a navigation system (Stealth Station®; Medtronic Sofamor Danek). After C1 laminectomy and screwing, monocortical autologous bone was harvested from the right posterior iliac crest and grafted between the decorticated posterior surfaces of the occipital bone and C2.The postoperative course was uneventful. Occiput-cervical alignment appeared improved after surgery, with a clivo-axial angle of 130° (Figure 2A). Since the surgery, he has not experienced any episodes of syncope. Cervical orthosis was applied for 6 weeks. Bone union was confirmed on CT at 4 months postoperatively (Figure 2B). As of the latest follow-up at 3.5 years postoperatively, he still showed no syncope, and was able to walk and run without falls.


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)

Postoperative imaging of the craniovertebral junction. (A) Lateral radiography shows improved alignment of the craniovertebral junction. (B) A midsagittal reconstruction of computed tomography obtained 4 months postoperatively shows complete bone union.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Postoperative imaging of the craniovertebral junction. (A) Lateral radiography shows improved alignment of the craniovertebral junction. (B) A midsagittal reconstruction of computed tomography obtained 4 months postoperatively shows complete bone union.
Mentions: C1 laminectomy and posterior occiput-cervical fusion (O-C2) were performed using an instrumentation system (VERTEX® MAX; Medtronic Sofamor Danek, Memphis, TN, USA). Intraoperatively, the migrated C1 posterior arch was repositioned after skull traction and we were able to reach the upper surface of C1. Decompression of his foramen magnum was therefore not required for C1 laminectomy. A portion of the C1 posterior arch rachischisis consisted of tendon-like tissue that adhered severely to the dura. During C1 laminectomy, the locations of VAs were detected using Doppler ultrasonography. C2 pedicle screws were inserted with the assistance of a navigation system (Stealth Station®; Medtronic Sofamor Danek). After C1 laminectomy and screwing, monocortical autologous bone was harvested from the right posterior iliac crest and grafted between the decorticated posterior surfaces of the occipital bone and C2.The postoperative course was uneventful. Occiput-cervical alignment appeared improved after surgery, with a clivo-axial angle of 130° (Figure 2A). Since the surgery, he has not experienced any episodes of syncope. Cervical orthosis was applied for 6 weeks. Bone union was confirmed on CT at 4 months postoperatively (Figure 2B). As of the latest follow-up at 3.5 years postoperatively, he still showed no syncope, and was able to walk and run without falls.

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