Arteriovenous malformation presenting as complex regional pain syndrome: illustrative case

Dayna C Sloane Department of Neurological Surgery, Loyola University Stritch School of Medicine, Maywood, Illinois;

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Diego D Luy Department of Neurological Surgery, Loyola University Stritch School of Medicine, Maywood, Illinois;

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Atul K Mallik Department of Neurological Surgery, Loyola University Stritch School of Medicine, Maywood, Illinois;
Department of Neuroradiology, Loyola University Medical Center, Maywood, Illinois; and

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Joseph C Serrone Department of Neurological Surgery, Loyola University Stritch School of Medicine, Maywood, Illinois;
Department of Neuroradiology, Loyola University Medical Center, Maywood, Illinois; and
Department of Neurological Surgery, Edward Hines Jr. VA Hospital, Maywood, Illinois

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Douglas E Anderson Department of Neurological Surgery, Loyola University Stritch School of Medicine, Maywood, Illinois;

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BACKGROUND

Complex regional pain syndrome (CRPS) is typically described as a peripheral nerve disorder in which exaggerated allodynia and hyperalgesia follow a minor injury. Some researchers propose a central mechanism, although current evidence is lacking.

OBSERVATIONS

A 14-year-old female presented with classic CRPS symptoms of left upper-extremity weakness and hyperalgesia after a bout of sharp pain in her thumb while shoveling snow. A possible seizure prompted magnetic resonance imaging, revealing a right frontal Spetzler-Martin grade II arteriovenous malformation (AVM) adjacent to the primary motor cortex. Brodmann areas 1, 3a, and 3b, which are responsible for localizing and processing burning and painful sensations, were also involved. The patient underwent transarterial Onyx embolization in two sessions and microsurgical resection, after which her CRPS symptoms completely resolved.

LESSONS

To our knowledge, this is the first reported case of a cerebral AVM presenting as CRPS, which supports a central mechanism. The authors propose that rapid growth of the AVM led to a vascular steal phenomenon of surrounding parenchyma, which disrupted the patient’s normal motor function and nociceptive processing. Further validation in other series is needed.

ABBREVIATIONS

AVM = arteriovenous malformation; CRPS = complex regional pain syndrome; CT = computed tomography; fMRI = functional magnetic resonance imaging; LUE = left upper-extremity; MRI = magnetic resonance therapy; PT = physical therapy; TCD = transcranial Doppler

BACKGROUND

Complex regional pain syndrome (CRPS) is typically described as a peripheral nerve disorder in which exaggerated allodynia and hyperalgesia follow a minor injury. Some researchers propose a central mechanism, although current evidence is lacking.

OBSERVATIONS

A 14-year-old female presented with classic CRPS symptoms of left upper-extremity weakness and hyperalgesia after a bout of sharp pain in her thumb while shoveling snow. A possible seizure prompted magnetic resonance imaging, revealing a right frontal Spetzler-Martin grade II arteriovenous malformation (AVM) adjacent to the primary motor cortex. Brodmann areas 1, 3a, and 3b, which are responsible for localizing and processing burning and painful sensations, were also involved. The patient underwent transarterial Onyx embolization in two sessions and microsurgical resection, after which her CRPS symptoms completely resolved.

LESSONS

To our knowledge, this is the first reported case of a cerebral AVM presenting as CRPS, which supports a central mechanism. The authors propose that rapid growth of the AVM led to a vascular steal phenomenon of surrounding parenchyma, which disrupted the patient’s normal motor function and nociceptive processing. Further validation in other series is needed.

ABBREVIATIONS

AVM = arteriovenous malformation; CRPS = complex regional pain syndrome; CT = computed tomography; fMRI = functional magnetic resonance imaging; LUE = left upper-extremity; MRI = magnetic resonance therapy; PT = physical therapy; TCD = transcranial Doppler

Complex regional pain syndrome (CRPS) is characterized by hyperalgesia and allodynia after an injury.1 Another prevalent symptom in up to 97% of patients with CRPS is motor dysfunction and dystonia, often in all muscles of an affected limb.1,2 Mottled coloration of the affected limb can also be seen.1 The currently widely accepted diagnostic criteria, called the “Budapest criteria,” includes symptoms in three of the four following categories, with symptoms in two of the four following categories at the time of evaluation: sensory, vasomotor, sudomotor, and motor.3 Additionally, there must be no other diagnostic explanation for the symptoms.3 Although the exact mechanism of CRPS remains unknown, evidence has been proposed to support both peripheral and central mechanisms. Proponents of the peripheral mechanism suggest that an inflammatory event in deep tissue due to fracturing, crushing, or spraining injuries can lead to edema within the interstitial space and further damage. This results in a compartment syndrome leading to microvasculature damage and chronic inflammation affecting the nerve.4

Those in support of a central mechanism posit that the primary motor (area 4) and somatosensory (areas 1, 2, and 3) cortices can be potential mediators of CRPS; however, there are conflicting results in the literature. Limited evidence supports bilateral disinhibition in M1, and only a few studies examining spatial representation of the hand region of S1 have found that the affected side had a smaller cortical area.5,6

In this study, we report on a 14-year-old patient presenting with CRPS secondary to an arteriovenous malformation (AVM) with evidence to support a central mechanism for the development of CRPS through a vascular steal mechanism in which the high-flow AVM causes hypoperfusion of surrounding eloquent parenchyma. Additionally, we present a concise review of previous related studies. To our knowledge, this is the first report of CRPS presenting from a structural central lesion.

Illustrative Case

History and Examination

A 14-year-old female presented to our neurological surgery department 9 months after initially experiencing 8/10 persistent sharp pain to her left thumb while shoveling snow. Shortly after the injury, the patient experienced persistent hyperesthesia with sensations of burning, tingling, and stabbing pains exacerbated by light touch. Radiography showed no fracture or dislocation, with trace swelling in the left wrist. She was diagnosed with CRPS by an orthopedic surgeon 2 months postinjury. Physical therapy (PT) within these first 2 months of symptom onset failed to resolve symptoms. Approximately 7 months postinjury, she was found to have complete loss of movement of all left upper-extremity (LUE) muscle groups associated with a mottled discoloration, swelling, cold sensation, and severe pain throughout her entire LUE (Fig. 1A). Although her father reported that the patient had intermittent spontaneous LUE movement while she was asleep, the patient was unable to exhibit volitional movement. Additional complaints of intermittent dizziness and episodes of shaking with apparent postictal state, which also occurred 7 months postinjury, led her to seek out magnetic resonance imaging (MRI) of her brain. A right frontal Spetzler-Martin grade II AVM adjacent to the primary motor cortex was discovered (Fig. 2A–C and E). After one of the patient’s preoperative dizzy spells, she fell and was found unresponsive, leading to hospitalization. An electroencephalogram was obtained and revealed no seizure or epileptiform activity; thus, no anticonvulsants were prescribed. Although her presumed spell and postictal state were concerning for seizures, these spells did not correlate with electrographic evidence of epileptiform/seizure discharges on assessment with electroencephalography. Other imaging was performed and reviewed. Head computed tomography (CT) with contrast performed 8 years earlier because of severe headaches was negative for an AVM (Fig. 3). On cerebral angiography, the AVM was 1.2 × 2.4 × 2.7 cm and fed by the middle cerebral artery with three superficial draining veins (Fig. 4A and B). MRI of the cervical spine and left brachial plexus were negative. Functional magnetic resonance imaging (fMRI) while attempting to move the left arm demonstrated a small focus of activation in the left arm primary motor cortex within 1 cm of the AVM (Fig. 5).

FIG. 1
FIG. 1

A: The right arm shows normal coloration and movement, and the left arm shows mottled coloration and weakness. B: Postoperative image shows normal coloration and movement of both arms.

FIG. 2
FIG. 2

Sagittal (A) and coronal (B) T1-weighted MRI revealing a vascular lesion in the precentral gyrus of the frontal lobe. Red arrows indicate the central sulcus. Red outlines indicate area 3a. Blue outline indicates the expected location of area 3b, which is obscured by the AVM. Green outline indicates area 1. Pre- (C) and postoperative (D) coronal and pre- (E) and postoperative (F) axial T2-weighted MRI demonstrating complete resection of the AVM.

FIG. 3
FIG. 3

Axial CT 8 years prior to presenting with neurological deficits, demonstrating no evidence of an AVM.

FIG. 4
FIG. 4

Preoperative coronal (A) and sagittal (B) diagnostic cerebral angiograms reveal a Spetzler-Martin grade II AVM with a maximum nidal diameter of 2.7 cm. Postoperative coronal (C) and sagittal (D) cerebral angiograms are negative for AVM.

FIG. 5
FIG. 5

Axial fMRI (A) during bilateral finger tapping activation demonstrates expected activation near the left hand-knob but no activation near the right hand-knob or other somatic motor regions. A functional task of attempting to move the left arm (B) demonstrates a small but statistically significant focus of functional activation at the precentral gyrus slightly lateral to the right hand-knob, which may correspond with the somatotopic location of left arm motor function. This focus of activation is within 1 cm of the right frontal AVM. Red arrows indicate the central sulcus.

Treatment and Postoperative Results

The patient underwent transarterial Onyx embolization in two sessions and microsurgical resection without complication (Fig. 2D and F). Unlike with preoperative PT, the patient regained full function of her LUE after postoperative rehabilitation. By 4 months postoperatively, she reported diminished pain and had gained the ability to participate in physical activity previously disabled by her debilitating pain and loss of function. At her most recent follow-up 14 months postoperatively, she was noted to exhibit a remarkable recovery of movement and normal sensation of the LUE. Cerebral angiography at this time demonstrated no recurrence of the treated AVM (Fig. 4C and D). Postoperative photographs of her LUE received from the patient’s mother at 16 months postoperatively demonstrated normal coloration; the mother reported that the patient remained asymptomatic at the time (Fig. 1B).

Literature Review

A literature review was based on searches within the PubMed database using the key words (“arteriovenous malformation” AND “complex regional pain syndrome”) or (“arteriovenous malformation” AND “reflex sympathetic dystrophy”). Reports detailing intracranial AVMs with associated complex regional pain syndrome met our inclusion criteria and were included in the literature review. Excluded reports were those that did not describe patient presentation and outcome, were originally printed in a language other than English, or were nonhuman studies.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

In this report, we describe a patient who was initially diagnosed with classic CRPS until symptoms of seizure prompted brain imaging, revealing a right frontal AVM. Resection of the AVM completely resolved symptoms of allodynia and hyperalgesia and restored movement and normal coloration of the patient’s LUE.

A literature review yielded six unique articles, none of which were included because of their lack of an intracranial AVM. Therefore, our case, to our knowledge, is the first reported instance of CRPS due to any structural central lesion, including an intracranial AVM. Notably, there was substantial evidence in the literature demonstrating regional pain syndromes due to a steal phenomenon from upper-extremity arteriovenous fistulas for dialysis patients, but this has yet to be reported in relation to intracranial arteriovenous aberrations.7–9 However, there have been other neurovascular pathologies associated with CRPS. It has been reported that approximately 1.5% to 70% of stroke patients develop CRPS, also known as “hand-shoulder syndrome” for this etiology.10 Interestingly, there has been one report of a fortuitous traumatic injury resolving a patient’s right-sided CRPS: a patient accidentally fell and suffered a left frontal cerebral contusion.11 Upon regaining consciousness, the patient reported resolution of his 10-year history of CRPS in the contralateral extremity, which had been refractory to other treatment.

Steal syndrome, a well-known phenomenon that has been extensively described in the literature, may have contributed to the symptoms in our patient.12–14 When an arteriovenous shunt with low vascular resistance is present, hypoperfusion in adjacent tissue (with higher vascular resistance) ensues, leading to ischemic symptoms such as weakness, seizures, cognitive deficit, and other neurological deficiencies.12,13,15–17 Rapid enlargement of AVMs has also been shown to lead to ischemic symptoms in patients.18,19 Based on our patient’s negative brain imaging 8 years prior, we suspect the AVM formed de novo and conceivably enlarged rapidly, resulting in a steal syndrome. This central disturbance may have contributed to the patient’s autonomic symptoms, which are thought to be related to centrally mediated thermoregulation dysfunction and irregularity of impulses in a motor neuron pool.2 The precise location of the patient’s AVM may explain the motor deficit and pain syndrome observed. The AVM was noted to involve the central sulcus, extending as far down as the fundus, and therefore was close to Brodmann areas 3a, 3b, 1, and 4 (the motor cortex).20,21 Area 3a, which is located at the fundus of the central sulcus and borders the motor cortex anteriorly, mediates the processing of burning and painful sensations from injury that originate in deep tissue.20,21 Area 3b and 1 are both situated in the anterior bank of the postcentral gyrus and are involved in localizing tactile stimuli.20,21 The clear spatial association of these four Brodmann areas with the AVM may have caused disruption in motor and nociceptive processing.

There are numerous reports of a steal syndrome leading to motor deficits similar to those seen in our patient.13,22–26 However, directly conflicting with our patient’s presentation, other reports have documented decreased sensation or even numbness to the body.16 But this is not the first instance in which neurological pathologies result in opposing pain responses. Marshall et al.27 presented a case series of 18 patients with cortical wounds, 2 of whom experienced hyperpathia of their extremities on the side contralateral to the injury. The other 16 patients experienced either a preserved or diminished pain response. Previously, Moftakhar et al.15 presented evidence in favor of the vascular steal syndrome. Within the referenced evidence was a study utilizing CT perfusion with an acetazolamide challenge to evaluate hemodynamic reserves surrounding cerebral AVMs. A 27% decrease in vascular reserve was noted immediately adjacent to AVMs, and a 17% decrease in vascular reserve was noted in regions remote from the AVMs. This evidence suggests that AVM-associated hemodynamic alterations can cause a widespread decline in cerebral vascular reserves.16,17 Decreased vascular reserves may induce CRPS with expected resolution posttreatment, after the cerebral hemodynamics are normalized.

Despite this evidence, the vascular steal phenomenon has had controversy, with researchers arguing that there is evidence against the theory. Notably, reports including a transcranial Doppler (TCD) ultrasound study measuring flow velocities around medium and large AVMs have demonstrated no relationship between feeding artery pressures or flow velocities and focal neurological deficits.28,29 Inherently, TCD is a limited technology that is highly operator-dependent, requiring detailed three-dimensional knowledge of cerebrovascular anatomy and its variations.30 Up to 10% to 15% of acoustic windows are deemed inadequate, particularly in Blacks, Asians, and elderly women, possibly due to the varying thickness and porosity of bone around acoustic windows and the attenuation of ultrasound energy transmission.30 Additionally, TCD ultrasound measurements are limited to large basal arteries and only provide a global index of blood flow velocities rather than an assessment of local cerebral blood flow.30

The limited studies available to support or refute this phenomenon and subsequent syndromes, including CRPS, present a limitation to this case report. Additionally, many reports of CRPS resolve without any specific form of treatment.1,31 Therefore, the possibility of coexisting CRPS and AVM accompanied by the spontaneous resolution of CRPS symptoms cannot be entirely discounted in our patient. However, the temporal profile of symptoms, the conceivable de novo formation and rapid growth of the AVM, the association of the motor cortex and nociceptive processing areas with the AVM, and the success of postoperative PT versus preoperative PT suggest an association between the two disease processes. Ultimately, however, additional research is needed to elucidate the underlying mechanisms for CRPS and intracranial steal syndromes.

Lessons

This is the first report of a central primary structural lesion correlating with symptoms of CRPS. We propose that rapid growth of the AVM adjacent to the arm region of Brodmann area 4 (the motor cortex) and areas 3a, 3b, and 1 (sensory cortex) led to dysfunction in movement and nociceptive processing through a vascular steal phenomenon. Further validation in additional series is needed; however, this case provides evidence for central mechanisms potentially inducing CRPS, as previously suggested by other investigators for non-AVM CRPS patients. Advanced cerebral perfusion imaging in patients with AVMs may aid in explaining atypical presentations for seemingly unrelated syndromes, including CRPS.

Acknowledgments

We would like to thank the Christopher Family Foundation for their ongoing support.

Author Contributions

Conception and design: Anderson, Sloane, Serrone. Acquisition of data: Sloane, Luy, Mallik. Analysis and interpretation of data: Sloane, Luy, Mallik. Drafting the article: Anderson, Sloane, Luy, Serrone. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Anderson. Administrative/technical/material support: Mallik. Study supervision: Anderson, Luy, Serrone.

References

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  • Collapse
  • Expand
  • FIG. 1

    A: The right arm shows normal coloration and movement, and the left arm shows mottled coloration and weakness. B: Postoperative image shows normal coloration and movement of both arms.

  • FIG. 2

    Sagittal (A) and coronal (B) T1-weighted MRI revealing a vascular lesion in the precentral gyrus of the frontal lobe. Red arrows indicate the central sulcus. Red outlines indicate area 3a. Blue outline indicates the expected location of area 3b, which is obscured by the AVM. Green outline indicates area 1. Pre- (C) and postoperative (D) coronal and pre- (E) and postoperative (F) axial T2-weighted MRI demonstrating complete resection of the AVM.

  • FIG. 3

    Axial CT 8 years prior to presenting with neurological deficits, demonstrating no evidence of an AVM.

  • FIG. 4

    Preoperative coronal (A) and sagittal (B) diagnostic cerebral angiograms reveal a Spetzler-Martin grade II AVM with a maximum nidal diameter of 2.7 cm. Postoperative coronal (C) and sagittal (D) cerebral angiograms are negative for AVM.

  • FIG. 5

    Axial fMRI (A) during bilateral finger tapping activation demonstrates expected activation near the left hand-knob but no activation near the right hand-knob or other somatic motor regions. A functional task of attempting to move the left arm (B) demonstrates a small but statistically significant focus of functional activation at the precentral gyrus slightly lateral to the right hand-knob, which may correspond with the somatotopic location of left arm motor function. This focus of activation is within 1 cm of the right frontal AVM. Red arrows indicate the central sulcus.

  • 1

    Birklein F, Riedl B, Sieweke N, Weber M, Neundörfer B Neurological findings in complex regional pain syndromes—analysis of 145 cases. Acta Neurol Scand. 2000;101(4):262269.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Wasner G, Schattschneider J, Binder A, Baron R Complex regional pain syndrome—diagnostic, mechanisms, CNS involvement and therapy. Spinal Cord. 2003;41(2):6175.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Sebastin SJ Complex regional pain syndrome. Indian J Plast Surg. 2011;44(2):298307.

  • 4

    Coderre TJ, Bennett GJ A hypothesis for the cause of complex regional pain syndrome-type I (reflex sympathetic dystrophy): pain due to deep-tissue microvascular pathology. Pain Med. 2010;11(8):12241238.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Di Pietro F, McAuley JH, Parkitny L, et al. Primary motor cortex function in complex regional pain syndrome: a systematic review and meta-analysis. J Pain. 2013;14(11):12701288.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Di Pietro F, McAuley JH, Parkitny L, et al. Primary somatosensory cortex function in complex regional pain syndrome: a systematic review and meta-analysis. J Pain. 2013;14(10):10011018.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Fominykh NM, Zulkarnaev AB, Gegenava BB, Kardanakhishvili ZB Endovascular embolization of arteriovenous fistula in a patient with hand ischemia. Article in Russian. Khirurgiia (Mosk). 2019;6:111116.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Sathiavageesan S, Annamalai I, Karki A, Kamalanathan M, Annadurai A, Chandrasekaran A Complex regional pain syndrome following vascular access creation for hemodialysis. J Vasc Access. 2023;24(6):15351537.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Unek IT, Birlik M, Cavdar C, et al. Reflex sympathetic dystrophy syndrome due to arteriovenous fistula. Hemodial Int. 2005;9(4):344348.

  • 10

    Pertoldi S, Di Benedetto P Shoulder-hand syndrome after stroke. A complex regional pain syndrome. Eura Medicophys. 2005;41(4):283292.

  • 11

    Shibata M, Nakao K, Galer BS, Shimizu T, Taniguchi H, Uchida T A case of reflex sympathetic dystrophy (complex regional pain syndrome, type I) resolved by cerebral contusion. Pain. 1999;79(2-3):313315.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Feindel W, Yamamoto YL, Hodge CP Red cerebral veins and the cerebral steal syndrome. Evidence from fluorescein angiography and microregional blood flow by radioisotopes during excision of an angioma. J Neurosurg. 1971;35(2):167179.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Ellis MJ, Armstrong D, Dirks PB Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis. J Neurosurg Pediatr. 2011;7(1):1521.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Marks MP, Lane B, Steinberg G, Chang P Vascular characteristics of intracerebral arteriovenous malformations in patients with clinical steal. AJNR Am J Neuroradiol. 1991;12(3):489496.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Moftakhar P, Hauptman JS, Malkasian D, Martin NA Cerebral arteriovenous malformations. Part 2: physiology. Neurosurg Focus. 2009;26(5):E11.

  • 16

    Homan RW, Devous MD Sr, Stokely EM, Bonte FJ Quantification of intracerebral steal in patients with arteriovenous malformation. Arch Neurol. 1986;43(8):779785.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Okabe T, Meyer JS, Okayasu H, et al. Xenon-enhanced CT CBF measurements in cerebral AVM’s before and after excision. Contribution to pathogenesis and treatment. J Neurosurg. 1983;59(1):2131.

    • PubMed
    • Search Google Scholar
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