Basal ganglia cavernous malformations: case series and systematic review of surgical management and long-term outcomes

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  • 1 Department of Neurosurgery and Stanford Stroke Center, Stanford University School of Medicine and Stanford Health Center, Stanford;
  • 2 University of California, Irvine, California;
  • 3 University of Wisconsin School of Medicine, Madison, Wisconsin; and
  • 4 Department of Medicine, Stanford Center for Biomedical Informatics Research, Stanford University School of Medicine, Stanford, California
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OBJECTIVE

Reports on basal ganglia cavernous malformations (BGCMs) are rare. Here, the authors report on their experience in resecting these malformations to offer insight into this infrequent disease subtype.

METHODS

The authors retrospectively reviewed a prospectively managed departmental database of all deep-seated cerebral cavernous malformations (CCMs) treated at Stanford between 1987 and 2019 and included for further analysis those with a radiographic diagnosis of BGCM. Moreover, a systematic literature review was undertaken using the PubMed and Web of Science databases.

RESULTS

The departmental database search yielded 331 patients with deep-seated CCMs, 44 of whom had a BGCM (13.3%). Headache was the most common presenting sign (53.5%), followed by seizure (32.6%) and hemiparesis (27.9%). Lesion location involved the caudate nucleus in 21.4% of cases compared to 78.6% of cases within the lentiform nucleus. Caudate BGCMs were larger on presentation and were more likely to present to the ependymal surface (p < 0.001) with intraventricular hemorrhage and hydrocephalus (p = 0.005 and 0.007, respectively). Dizziness and diplopia were also more common with lesions involving the caudate. Because of their anatomical location, caudate BGCMs were preferentially treated via an interhemispheric approach and were less likely to be associated with worsening perioperative deficits than lentiform BGCMs (p = 0.006 and 0.045, respectively). Ten patients (25.6%) were clinically worse in the immediate postoperative period, 4 (10.2%) of whom continued to suffer permanent morbidity at the last follow-up. A long-term good outcome (modified Rankin Scale [mRS] score 0–1) was attained in 74.4% of cases compared to the 69.2% of patients who had presented with an mRS score 0–1. Relative to their presenting mRS score, 89.8% of patients had an improved or unchanged status at the last follow-up. The median postoperative follow-up was 11 months (range 1–252 months). Patient outcomes after resection did not differ among surgical approaches; however, patients presenting with hemiparesis and lesions involving the globus pallidus or posterior limb of the internal capsule were more likely to suffer neurological deficits during the immediate perioperative period. Patients who had undergone awake surgeries were more likely to suffer neurological decline at the early as well as the late follow-up. When adjusting for awake craniotomy as a potential confounder of lesion location, a BGCM involving the posterior limb was predictive of developing early postoperative deficits, but this finding did not persist at the long-term follow-up.

CONCLUSIONS

Surgery is a safe and effective treatment modality for managing BGCMs, with an estimated long-term permanent morbidity rate of around 10%.

ABBREVIATIONS BGCM = basal ganglia CM; CM = cavernous malformation; MEP/SSEP = motor evoked potential/somatosensory evoked potential; mRS = modified Rankin Scale; ND = neurological deficit.

Supplementary Materials

    • Supplementary Tables 1–4 (PDF 530 KB)

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Contributor Notes

Correspondence Gary K. Steinberg: Stanford University School of Medicine, Stanford, CA. gsteinberg@stanford.edu.

INCLUDE WHEN CITING Published online January 1, 2021; DOI: 10.3171/2020.7.JNS2098.

Disclosures Dr. Steinberg is a consultant for Qool Therapeutics, Peter Lazic US, NeuroSave, SanBio, Audaxion Therapeutics, Zeiss, and Surgical Theater.

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