Spinal arteriovenous malformation with a calcified nodule: illustrative case

Ping-Chuan Liu Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan

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Chung-Chia Huang Medical Education Department, Far Eastern Memorial Hospital, Taipei, Taiwan

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Ching-Lin Chen Department of Trauma, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan; and
Neurosurgical Department of Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan

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BACKGROUND

This article describes a rare case of cervical spinal arteriovenous malformation (AVM) mimicking a neurogenic spinal tumor.

OBSERVATIONS

A 22-year-old female presenting with a C6–7 AVM with a calcification nodule experienced new-onset acute right upper radiculopathy associated with extradural compression of the spinal cord. Note that spinal AVMs with a calcified nodule are rare. Endovascular embolization is generally used to relieve the symptoms of AVM; however, this procedure cannot relieve cord compression, particularly in cases complicated by calcified nodules. This article discusses treatment options.

LESSONS

Decompression surgery is preferable to endovascular embolization because it alleviates symptoms while preventing cord compression and minimizing the risk of recurrence.

ABBREVIATIONS

AVF = arteriovenous fistula; AVM = arteriovenous malformation; CT = computed tomography; MRI = magnetic resonance imaging

BACKGROUND

This article describes a rare case of cervical spinal arteriovenous malformation (AVM) mimicking a neurogenic spinal tumor.

OBSERVATIONS

A 22-year-old female presenting with a C6–7 AVM with a calcification nodule experienced new-onset acute right upper radiculopathy associated with extradural compression of the spinal cord. Note that spinal AVMs with a calcified nodule are rare. Endovascular embolization is generally used to relieve the symptoms of AVM; however, this procedure cannot relieve cord compression, particularly in cases complicated by calcified nodules. This article discusses treatment options.

LESSONS

Decompression surgery is preferable to endovascular embolization because it alleviates symptoms while preventing cord compression and minimizing the risk of recurrence.

ABBREVIATIONS

AVF = arteriovenous fistula; AVM = arteriovenous malformation; CT = computed tomography; MRI = magnetic resonance imaging

Spinal arteriovenous malformations (AVMs) are uncommon, and their pathogenesis is largely unknown. When the condition is left untreated, the prognosis for patients with associated neurological symptoms is poor. Calcified nodules in spinal lesions (e.g., meningioma and AVMs) are particularly rare. This article presents the case of a cervical spinal AVM with a calcified nodule in accordance with Consensus Surgical Case Report (SCARE) guidelines.1 This article also includes a brief review of the relevant literature and a discussion of treatment options.

Illustrative Case

A 22-year-old female presenting with progressive right shoulder pain for 2 days was admitted to the emergency department of our hospital for an examination. The patient reported radiating pain in the right arm and numbness over the sensory dermatome of C5–7. Her symptoms progressively deteriorated as numbness spread to the left forearm and left lower leg. A neurological examination revealed grade 4 muscle power in the right upper limb. The patient also reported abnormal touch-vibration and pain-temperature sensations in the same area. A cervical spine lesion was suspected; however, radiographs revealed good alignment without instability (Fig. 1). Computed tomography (CT) scans revealed a calcified nodule measuring 0.47 × 0.51 × 0.58 cm over the right C6 vertebrae (Fig. 2). Magnetic resonance imaging (MRI) revealed an epidural lesion over the C5 to C6–7 right-posterior epidural space causing cord compression and C6–7 neural foramen encroachment. An epidural hematoma and a neurogenic tumor could not be ruled out (Fig. 3). Note that nerve conduction velocity and electromyography results were typical for an individual of her age.

FIG. 1
FIG. 1

Radiographs showing a small, calcified nodule on the right side of the C6 body (left) and good alignment without instability (right).

FIG. 2
FIG. 2

A: Axial CT scan showing a well-defined calcified lesion on the right side at the C6 level surrounded by an ill-defined hyperintense lesion. B: Sagittal CT scan showing a calcified lesion at the inferior endplate of the C6 vertebral body. C: Coronal CT scan showing a hyperdense lesion extending from the inferior to the superior endplate of C6. Arrows indicate the calcified lesion.

FIG. 3
FIG. 3

MRI showing a lesion in the epidural space at the right-posterior aspect of the central canal between C5 and C7 vertebrae and extending into the neuroforamen of C6–7, causing compression on the spinal cord at C6–7 and narrowing of the C6–7 neuroforamen. Axial (A), sagittal (B), and coronal (C) views. Note that the non-contrast–enhanced part of the lesion indicates a calcified lesion.

Under the assumption that this was a neurogenic tumor, we adopted a posterior approach to a C5 to C6–7 laminectomy and tumor excision. An initial examination of the epidural space revealed a bundle of abnormal vessels (Fig. 4). The bleeding tendency of the lesion was high, and a whitish round tumor was observed adjacent to the blood vessels. A histological examination revealed a number of dilated arterial and venous vessels, indicating an AVM and a calcified nodule in the dermis. After the operation, the clinical symptoms completely subsided.

FIG. 4
FIG. 4

Excised tissue samples (0.5 × 0.3 × 0.2 cm) presenting irregular blood vessels and blood clots. The tissue appeared black and friable. Microscope images of the specimen revealed blood clots and fat tissue with irregular dilated arterial and venous vessels. Hematoxylin and eosin, original magnification ×40 (left and right).

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

Spinal lesions can be classified according to their location as extradural, intradural extramedullary, and intramedullary. The spinal lesion examined in this study was located in the extradural region. In the neuroradiological differential diagnosis of epidural lesions, it is important for clinicians to consider metastasis, bone tumors, nerve sheath tumors, and vascular lesions.2 Calcification is common in intracranial meningioma; however, it is rarely encountered in extradural tumors along the spinal cord.3

Spinal AVMs represent only 3% to 4% of all space-occupying lesions affecting the spinal cord,4 and most of these appear in the third or fourth decade of life. No predominant sex has been noted in adult populations.5 Multiple systems have been established for the classification of spinal AVMs.6–10 The most widely used system is based on the appearance of fistulas on radiographic images.11 Extradural arteriovenous fistulas (AVFs) do not fall within this classification; therefore, they were added as a new type, resulting in a classification system involving five types.10 The patient in the current study presented with a type Vb AVF lesion.

The clinical manifestations of spinal AVMs include hypoesthesia (sudden or gradual) as well as paresthesia, progressive paraparesis, back pain, and sphincter disturbances.12 Similarities between spinal AVMs and spinal tumors in terms of clinical manifestation make it difficult to differentiate between the two based solely on clinical symptoms.

The appearance of calcified nodules in spinal AVMs is unexpected, and even in spinal meningioma, this manifestation is rare (1%–5%).13,14 Radiological features of spinal meningioma on CT and MRI are often broad-based dural attachments located posterolaterally. In the current case, the calcified lesion was round and well differentiated, without dural attachment. Calcified nodules are considered benign, and the pathology is unknown. Bertoni posited that they are caused by inflammatory reactions,15 whereas Liccardo et al.16 and Song et al.17 described them as spinal epidural calcified pseudoneoplasms.

The treatment options for spinal AVM include surgery and endovascular intervention, and selecting between the two relies on suitable medical imaging.11,18 Endovascular treatment is the preferred course when the vessels feeding the AVM can be obliterated and/or the risk of surgery is significant.19 Surgical interventions are indicated when the lesion is resectable.20 In the current study, surgery was considered the most effective approach to preventing the growth of the calcified lesion and corresponding spinal cord compression.

Lessons

AVMs can generally be treated via endovascular embolization as long as the feeding artery can be identified. The rare appearance of calcified nodules within a malformation should be considered a slow-growing spinal calcified extradural lesion, appearing as a well-defined, hyperdense lesion on CT scans. Nonetheless, identifying epidural lesions can be challenging, and it may be necessary to arrange for an angiogram if slow flow is suspected after MRI. If left untreated, these lesions can cause epidural compression-induced myelopathy or radiculopathy. Surgical interventions aimed at lesion removal generally provide favorable outcomes in terms of symptom regression and cord compression, and the incidence of recurrence is low.

Acknowledgments

The study was supported by Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan, and the manuscript was edited by acceptediting, Taiwan.

Author Contributions

Conception and design: Chen, Liu. Acquisition of data: Chen, Liu. Analysis and interpretation of data: all authors. Drafting the article: all authors. Critically revising the article: Chen. Reviewed submitted version of manuscript: Chen, Liu. Approved the final version of the manuscript on behalf of all authors: Chen. Statistical analysis: Chen. Administrative/technical/material support: Chen. Study supervision: Chen.

References

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    Agha RA, Franchi T, Sohrabi C, Mathew G, Kerwan A, Scare Group. The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines. Int J Surg. 2020;84:226230.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Duong LM, McCarthy BJ, McLendon RE, et al. Descriptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004-2007. Cancer. 2012;118(17):42204227.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Alafaci C, Grasso G, Granata F, Salpietro FM, Tomasello F. Ossified spinal meningiomas: clinical and surgical features. Clin Neurol Neurosurg. 2016;142:9397.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Bao YH, Ling F. Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery. 1997;40(1):7581.

  • 5

    Rodesch G, Hurth M, Alvarez H, Ducot B, Tadie M, Lasjaunias P. Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981-1999. Acta Neurochir (Wien). 2004;146(3):217227.

    • PubMed
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  • 6

    Mourier KL, Gobin YP, George B, Lot G, Merland JJ. Intradural perimedullary arteriovenous fistulae: results of surgical and endovascular treatment in a series of 35 cases. Neurosurgery. 1993;32(6):885891.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Rodesch G, Hurth M, Alvarez H, Tadié M, Lasjaunias P. Classification of spinal cord arteriovenous shunts: proposal for a reappraisal--the Bicêtre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery. 2002;51(2):374380.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Spetzler RF, Detwiler PW, Riina HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg. 2002;96(2 suppl):145156.

  • 9

    Geibprasert S, Pereira V, Krings T, et al. Dural arteriovenous shunts: a new classification of craniospinal epidural venous anatomical bases and clinical correlations. Stroke. 2008;39(10):27832794.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Takai K. Spinal arteriovenous shunts: angioarchitecture and historical changes in classification. Neurol Med Chir (Tokyo). 2017;57(7):356365.

  • 11

    Kim LJ, Spetzler RF. Classification and surgical management of spinal arteriovenous lesions: arteriovenous fistulae and arteriovenous malformations. Neurosurgery. 2006;59(5 suppl 3):S195S201.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Rubin MN, Rabinstein AA. Vascular diseases of the spinal cord. Neurol Clin. 2013;31(1):153181.

  • 13

    Koeller KK, Shih RY. Intradural extramedullary spinal neoplasms: radiologic-pathologic correlation. Radiographics. 2019;39(2):468490.

  • 14

    Lee JW, Lee IS, Choi KU, et al. CT and MRI findings of calcified spinal meningiomas: correlation with pathological findings. Skeletal Radiol. 2010;39(4):345352.

  • 15

    Bertoni F, Unni KK, Dahlin DC, Beabout JW, Onofrio BM. Calcifying pseudoneoplasms of the neural axis. J Neurosurg. 1990;72(1):4248.

  • 16

    Liccardo G, Lunardi P, Menniti A, Floris R, Pastore FS, Fraioli B. Calcifying pseudo-tumor of the spine: description of a case and review of the literature. Eur Spine J. 2003;12(5):548551.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Song SY, Ahn SY, Rhee JJ, Lee JW, Hur JW, Lee HK. Three cases of calcifying pseudoneoplasm which involve the epidural space of the spine. Korean J Spine. 2015;12(3):235238.

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    • Search Google Scholar
    • Export Citation
  • 18

    Endo T, Endo H, Sato K, Matsumoto Y, Tominaga T. Surgical and endovascular treatment for spinal arteriovenous malformations. Neurol Med Chir (Tokyo). 2016;56(8):457464.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Varshneya K, Pendharkar AV, Azad TD, Ratliff JK, Veeravagu A. A descriptive analysis of spinal cord arteriovenous malformations: clinical features, outcomes, and trends in management. World Neurosurg. 2019;131:e579e585.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Baldassarre B, Balestrino A, D’Andrea A, et al. Management of spinal aneurysms associated with arteriovenous malformations: systematic literature review and illustrative case. Eur Spine J. 2021;30(10):27672774.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • Collapse
  • Expand
  • FIG. 1

    Radiographs showing a small, calcified nodule on the right side of the C6 body (left) and good alignment without instability (right).

  • FIG. 2

    A: Axial CT scan showing a well-defined calcified lesion on the right side at the C6 level surrounded by an ill-defined hyperintense lesion. B: Sagittal CT scan showing a calcified lesion at the inferior endplate of the C6 vertebral body. C: Coronal CT scan showing a hyperdense lesion extending from the inferior to the superior endplate of C6. Arrows indicate the calcified lesion.

  • FIG. 3

    MRI showing a lesion in the epidural space at the right-posterior aspect of the central canal between C5 and C7 vertebrae and extending into the neuroforamen of C6–7, causing compression on the spinal cord at C6–7 and narrowing of the C6–7 neuroforamen. Axial (A), sagittal (B), and coronal (C) views. Note that the non-contrast–enhanced part of the lesion indicates a calcified lesion.

  • FIG. 4

    Excised tissue samples (0.5 × 0.3 × 0.2 cm) presenting irregular blood vessels and blood clots. The tissue appeared black and friable. Microscope images of the specimen revealed blood clots and fat tissue with irregular dilated arterial and venous vessels. Hematoxylin and eosin, original magnification ×40 (left and right).

  • 1

    Agha RA, Franchi T, Sohrabi C, Mathew G, Kerwan A, Scare Group. The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines. Int J Surg. 2020;84:226230.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Duong LM, McCarthy BJ, McLendon RE, et al. Descriptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004-2007. Cancer. 2012;118(17):42204227.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Alafaci C, Grasso G, Granata F, Salpietro FM, Tomasello F. Ossified spinal meningiomas: clinical and surgical features. Clin Neurol Neurosurg. 2016;142:9397.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Bao YH, Ling F. Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery. 1997;40(1):7581.

  • 5

    Rodesch G, Hurth M, Alvarez H, Ducot B, Tadie M, Lasjaunias P. Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981-1999. Acta Neurochir (Wien). 2004;146(3):217227.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Mourier KL, Gobin YP, George B, Lot G, Merland JJ. Intradural perimedullary arteriovenous fistulae: results of surgical and endovascular treatment in a series of 35 cases. Neurosurgery. 1993;32(6):885891.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Rodesch G, Hurth M, Alvarez H, Tadié M, Lasjaunias P. Classification of spinal cord arteriovenous shunts: proposal for a reappraisal--the Bicêtre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery. 2002;51(2):374380.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Spetzler RF, Detwiler PW, Riina HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg. 2002;96(2 suppl):145156.

  • 9

    Geibprasert S, Pereira V, Krings T, et al. Dural arteriovenous shunts: a new classification of craniospinal epidural venous anatomical bases and clinical correlations. Stroke. 2008;39(10):27832794.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Takai K. Spinal arteriovenous shunts: angioarchitecture and historical changes in classification. Neurol Med Chir (Tokyo). 2017;57(7):356365.

  • 11

    Kim LJ, Spetzler RF. Classification and surgical management of spinal arteriovenous lesions: arteriovenous fistulae and arteriovenous malformations. Neurosurgery. 2006;59(5 suppl 3):S195S201.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Rubin MN, Rabinstein AA. Vascular diseases of the spinal cord. Neurol Clin. 2013;31(1):153181.

  • 13

    Koeller KK, Shih RY. Intradural extramedullary spinal neoplasms: radiologic-pathologic correlation. Radiographics. 2019;39(2):468490.

  • 14

    Lee JW, Lee IS, Choi KU, et al. CT and MRI findings of calcified spinal meningiomas: correlation with pathological findings. Skeletal Radiol. 2010;39(4):345352.

  • 15

    Bertoni F, Unni KK, Dahlin DC, Beabout JW, Onofrio BM. Calcifying pseudoneoplasms of the neural axis. J Neurosurg. 1990;72(1):4248.

  • 16

    Liccardo G, Lunardi P, Menniti A, Floris R, Pastore FS, Fraioli B. Calcifying pseudo-tumor of the spine: description of a case and review of the literature. Eur Spine J. 2003;12(5):548551.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Song SY, Ahn SY, Rhee JJ, Lee JW, Hur JW, Lee HK. Three cases of calcifying pseudoneoplasm which involve the epidural space of the spine. Korean J Spine. 2015;12(3):235238.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Endo T, Endo H, Sato K, Matsumoto Y, Tominaga T. Surgical and endovascular treatment for spinal arteriovenous malformations. Neurol Med Chir (Tokyo). 2016;56(8):457464.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    Varshneya K, Pendharkar AV, Azad TD, Ratliff JK, Veeravagu A. A descriptive analysis of spinal cord arteriovenous malformations: clinical features, outcomes, and trends in management. World Neurosurg. 2019;131:e579e585.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    Baldassarre B, Balestrino A, D’Andrea A, et al. Management of spinal aneurysms associated with arteriovenous malformations: systematic literature review and illustrative case. Eur Spine J. 2021;30(10):27672774.

    • PubMed
    • Search Google Scholar
    • Export Citation

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