The Long and Winding Road: Thoracic Myelopathy Associated With Occipitocervical Dural Arteriovenous Fistula

Angelique Sao-Mai Do, Joseph Kapurch, Ravi Kumar, John D Port, Jeffrey W. Miller, Jamie Van Gompel

Research output: Contribution to journalArticle

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Abstract

Objectives: Spinal dural arteriovenous fistulas (DAVFs) have diverse presentations. Magnetic resonance imaging (MRI) reveals spinal cord swelling in only 45% to 74% of cases. We present an unusual case of a 57-year old man with a craniocervical junction DAVF in which the edema appeared in the thoracolumbar region, skipping the cervical spinal cord. Methods: A case report and literature review from a tertiary referral center are provided. Results: In our patient, symptoms progressed over 3 months from low back and radicular pain to weakness, saddle anesthesia, and urinary retention. MRI showed T2 hyperintensity from T3-4 to the conus medullaris. The results from initial brain and spine angiograms were negative. Repeated angiography with the patient under anesthesia revealed a DAVF in the craniocervical junction composed of a cervical branch arising from the right vertebral artery and entering the nidus at the foramen magnum. A prominent intradural draining vein extended inferiorly along the posterior aspect of the cervical spinal cord. The patient was taken to the operating room, where the extradural vertebral artery was dissected and a suboccipital craniectomy was performed. After the dura was opened, the arterialized vein was visualized and clipped. Somatosensory evoked potentials and motor evoked potentials remained at baseline throughout the occlusion. The dura and fistula were removed en bloc, and the draining vein was confirmed to lose flow on indocyanine green. Postoperatively, the patient's symptoms completely resolved, and he experienced full strength in the lower extremities and perineal sensation. A review of 19 cases of DAVF at the craniocervical junction with cord edema shows that >90% involve the brainstem or cervical cord. Only 1 other patient had a similar "skip lesion," in which the cord edema first appeared in the thoracic cord. Conclusions: This rare presentation highlights the importance of diagnostic persistence when unexplained cord findings are seen on MRI. Repeated angiography and the inclusion of proximal vascular lesions may be considered in the evaluation of isolated thoracic edema.

Original languageEnglish (US)
JournalWorld Neurosurgery
DOIs
StateAccepted/In press - 2017

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Central Nervous System Vascular Malformations
Spinal Cord Diseases
Thorax
Edema
Veins
Spinal Cord
Angiography
Vertebral Artery
Magnetic Resonance Imaging
Anesthesia
Foramen Magnum
Motor Evoked Potentials
Indocyanine Green
Urinary Retention
Somatosensory Evoked Potentials
Operating Rooms
Low Back Pain
Tertiary Care Centers
Brain Stem
Fistula

Keywords

  • Craniocervical dural arteriovenous fistula
  • Foix-Alajouanine syndrome
  • Myelopathy
  • Spinal cord edema

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

The Long and Winding Road : Thoracic Myelopathy Associated With Occipitocervical Dural Arteriovenous Fistula. / Sao-Mai Do, Angelique; Kapurch, Joseph; Kumar, Ravi; Port, John D; Miller, Jeffrey W.; Van Gompel, Jamie.

In: World Neurosurgery, 2017.

Research output: Contribution to journalArticle

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title = "The Long and Winding Road: Thoracic Myelopathy Associated With Occipitocervical Dural Arteriovenous Fistula",
abstract = "Objectives: Spinal dural arteriovenous fistulas (DAVFs) have diverse presentations. Magnetic resonance imaging (MRI) reveals spinal cord swelling in only 45{\%} to 74{\%} of cases. We present an unusual case of a 57-year old man with a craniocervical junction DAVF in which the edema appeared in the thoracolumbar region, skipping the cervical spinal cord. Methods: A case report and literature review from a tertiary referral center are provided. Results: In our patient, symptoms progressed over 3 months from low back and radicular pain to weakness, saddle anesthesia, and urinary retention. MRI showed T2 hyperintensity from T3-4 to the conus medullaris. The results from initial brain and spine angiograms were negative. Repeated angiography with the patient under anesthesia revealed a DAVF in the craniocervical junction composed of a cervical branch arising from the right vertebral artery and entering the nidus at the foramen magnum. A prominent intradural draining vein extended inferiorly along the posterior aspect of the cervical spinal cord. The patient was taken to the operating room, where the extradural vertebral artery was dissected and a suboccipital craniectomy was performed. After the dura was opened, the arterialized vein was visualized and clipped. Somatosensory evoked potentials and motor evoked potentials remained at baseline throughout the occlusion. The dura and fistula were removed en bloc, and the draining vein was confirmed to lose flow on indocyanine green. Postoperatively, the patient's symptoms completely resolved, and he experienced full strength in the lower extremities and perineal sensation. A review of 19 cases of DAVF at the craniocervical junction with cord edema shows that >90{\%} involve the brainstem or cervical cord. Only 1 other patient had a similar {"}skip lesion,{"} in which the cord edema first appeared in the thoracic cord. Conclusions: This rare presentation highlights the importance of diagnostic persistence when unexplained cord findings are seen on MRI. Repeated angiography and the inclusion of proximal vascular lesions may be considered in the evaluation of isolated thoracic edema.",
keywords = "Craniocervical dural arteriovenous fistula, Foix-Alajouanine syndrome, Myelopathy, Spinal cord edema",
author = "{Sao-Mai Do}, Angelique and Joseph Kapurch and Ravi Kumar and Port, {John D} and Miller, {Jeffrey W.} and {Van Gompel}, Jamie",
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T1 - The Long and Winding Road

T2 - Thoracic Myelopathy Associated With Occipitocervical Dural Arteriovenous Fistula

AU - Sao-Mai Do, Angelique

AU - Kapurch, Joseph

AU - Kumar, Ravi

AU - Port, John D

AU - Miller, Jeffrey W.

AU - Van Gompel, Jamie

PY - 2017

Y1 - 2017

N2 - Objectives: Spinal dural arteriovenous fistulas (DAVFs) have diverse presentations. Magnetic resonance imaging (MRI) reveals spinal cord swelling in only 45% to 74% of cases. We present an unusual case of a 57-year old man with a craniocervical junction DAVF in which the edema appeared in the thoracolumbar region, skipping the cervical spinal cord. Methods: A case report and literature review from a tertiary referral center are provided. Results: In our patient, symptoms progressed over 3 months from low back and radicular pain to weakness, saddle anesthesia, and urinary retention. MRI showed T2 hyperintensity from T3-4 to the conus medullaris. The results from initial brain and spine angiograms were negative. Repeated angiography with the patient under anesthesia revealed a DAVF in the craniocervical junction composed of a cervical branch arising from the right vertebral artery and entering the nidus at the foramen magnum. A prominent intradural draining vein extended inferiorly along the posterior aspect of the cervical spinal cord. The patient was taken to the operating room, where the extradural vertebral artery was dissected and a suboccipital craniectomy was performed. After the dura was opened, the arterialized vein was visualized and clipped. Somatosensory evoked potentials and motor evoked potentials remained at baseline throughout the occlusion. The dura and fistula were removed en bloc, and the draining vein was confirmed to lose flow on indocyanine green. Postoperatively, the patient's symptoms completely resolved, and he experienced full strength in the lower extremities and perineal sensation. A review of 19 cases of DAVF at the craniocervical junction with cord edema shows that >90% involve the brainstem or cervical cord. Only 1 other patient had a similar "skip lesion," in which the cord edema first appeared in the thoracic cord. Conclusions: This rare presentation highlights the importance of diagnostic persistence when unexplained cord findings are seen on MRI. Repeated angiography and the inclusion of proximal vascular lesions may be considered in the evaluation of isolated thoracic edema.

AB - Objectives: Spinal dural arteriovenous fistulas (DAVFs) have diverse presentations. Magnetic resonance imaging (MRI) reveals spinal cord swelling in only 45% to 74% of cases. We present an unusual case of a 57-year old man with a craniocervical junction DAVF in which the edema appeared in the thoracolumbar region, skipping the cervical spinal cord. Methods: A case report and literature review from a tertiary referral center are provided. Results: In our patient, symptoms progressed over 3 months from low back and radicular pain to weakness, saddle anesthesia, and urinary retention. MRI showed T2 hyperintensity from T3-4 to the conus medullaris. The results from initial brain and spine angiograms were negative. Repeated angiography with the patient under anesthesia revealed a DAVF in the craniocervical junction composed of a cervical branch arising from the right vertebral artery and entering the nidus at the foramen magnum. A prominent intradural draining vein extended inferiorly along the posterior aspect of the cervical spinal cord. The patient was taken to the operating room, where the extradural vertebral artery was dissected and a suboccipital craniectomy was performed. After the dura was opened, the arterialized vein was visualized and clipped. Somatosensory evoked potentials and motor evoked potentials remained at baseline throughout the occlusion. The dura and fistula were removed en bloc, and the draining vein was confirmed to lose flow on indocyanine green. Postoperatively, the patient's symptoms completely resolved, and he experienced full strength in the lower extremities and perineal sensation. A review of 19 cases of DAVF at the craniocervical junction with cord edema shows that >90% involve the brainstem or cervical cord. Only 1 other patient had a similar "skip lesion," in which the cord edema first appeared in the thoracic cord. Conclusions: This rare presentation highlights the importance of diagnostic persistence when unexplained cord findings are seen on MRI. Repeated angiography and the inclusion of proximal vascular lesions may be considered in the evaluation of isolated thoracic edema.

KW - Craniocervical dural arteriovenous fistula

KW - Foix-Alajouanine syndrome

KW - Myelopathy

KW - Spinal cord edema

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