Recurrence of cavernous malformations after surgery in childhood

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  • 1 Departments of Neurosurgery and
  • 2 Pathology, Stanford University School of Medicine, Stanford, California; and
  • 3 Department of Pediatric Neurosurgery, Global Neuroscience Institute/St. Christopher’s Hospital for Children, Philadelphia, Pennsylvania
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OBJECTIVE

Cavernous malformations (CMs) are commonly treated cerebrovascular anomalies in the pediatric population; however, the data on radiographic recurrence of pediatric CMs after surgery are limited. The authors aimed to study the clinical presentation, outcomes, and recurrence rate following surgery for a large cohort of CMs in children.

METHODS

Pediatric patients (≤ 18 years old) who had a CM resected at a single institution were identified and retrospectively reviewed. Fisher’s exact test of independence was used to assess differences in categorical variables. Survival curves were evaluated using the Mantel-Cox method.

RESULTS

Fifty-three patients aged 3 months to 18 years underwent resection of 74 symptomatic CMs between 1996 and 2018 at a single institution. The median length of follow-up was 5.65 years. Patients most commonly presented with seizures (45.3%, n = 24) and the majority of CMs were cortical (58.0%, n = 43). Acute radiographic hemorrhage was common at presentation (64.2%, n = 34). Forty-two percent (n = 22) of patients presented with multiple CMs, and they were more likely to develop de novo lesions (71%) compared to patients presenting with a single CM (3.4%). Both radiographic hemorrhage and multiple CMs were independently prognostic for a higher risk of the patient requiring subsequent surgery. Fifty percent (n = 6) of the 12 patients with both risk factors required additional surgery within 2.5 years of initial surgery compared to none of the patients with neither risk factor (n = 9).

CONCLUSIONS

Patients with either acute radiographic hemorrhage or multiple CMs are at higher risk for subsequent surgery and require long-term MRI surveillance. In contrast, patients with a single CM are unlikely to require additional surgery and may require less frequent routine imaging.

ABBREVIATIONS aHR = adjusted HR; CM = cavernous malformation; DVA = developmental venous anomaly; GTR = gross-total resection; HR = hazard ratio; STR = subtotal resection.

Supplementary Materials

    • Supplemental Table 1 (PDF 428 KB)

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Contributor Notes

Correspondence Gerald A. Grant: Stanford University School of Medicine, Stanford, CA. ggrant2@stanford.edu.

INCLUDE WHEN CITING Published online May 1, 2020; DOI: 10.3171/2020.2.PEDS19543.

Disclosures Dr. Steinberg is a consultant for Qool Therapeutics, NeuroSave, SanBio, and Zeiss. He is a patent holder with Peter Lazic, US.

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