Clinical outcomes of pediatric cerebral cavernous malformation: an analysis of 124 consecutive cases

Youngbo ShimDivision of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea;

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Ji Hoon PhiDivision of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea;

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Kyu-Chang WangNeuro-oncology Clinic, Center for Rare Cancers, National Cancer Center, Goyang, Republic of Korea;

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Byung-Kyu ChoDepartment of Neurosurgery, Armed Forces Capital Hospital, Seongnam, Republic of Korea;

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Ji Yeoun LeeDivision of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea;
Department of Anatomy and Cell Biology, Seoul National University College of Medicine, Seoul, Republic of Korea;

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Eun Jung KohDivision of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea;

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Kyung Hyun KimDivision of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea;

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Eun Jung LeeDepartment of Neurosurgery, Seoul National University Hospital, Seoul, Republic of Korea;

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Ki Joong KimDivision of Pediatric Neurology, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea; and

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Seung-Ki KimDivision of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea;
Neuroscience Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea

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OBJECTIVE

One-fourth of cerebral cavernous malformation (CCM) patients are children, but studies on these patients are scarce. This study aimed to identify the clinical presentation of pediatric CCM patients and to investigate clinical outcomes according to the treatment modalities applied on the basis of our institution’s treatment strategy.

METHODS

The authors performed a retrospective analysis of 124 pediatric CCM patients with a follow-up of more than 1 year from 2000 to 2019. They performed resection (n = 62) on lesions causing intractable seizure, rebleeding, or mass effect and observed the clinical courses of patients with lesions in deep or eloquent areas without persistent symptoms (n = 52). Radiosurgery (n = 10) was performed when the patient refused resection or strongly desired radiosurgery. The authors investigated the clinical characteristics, performance status (modified Rankin Scale [mRS] score), and rebleeding rate at the 1-year and last follow-up examinations and compared these among 3 groups classified on the basis of treatment applied. The authors evaluated seizure outcomes at the 1-year and last follow-up examinations for the surgery (n = 32) and observation (n = 17) groups. Finally, the authors drew cumulative incidence curves for the discontinuation of antiepileptic drugs (AEDs) for patients in the surgery (n = 30) and observation (n = 9) groups.

RESULTS

The 3 groups showed slight differences in initial symptoms, lesion locations, and rates of recent hemorrhage. The proportion of patients with improved mRS score at the 1-year follow-up was significantly greater in the surgery group than in the other groups (67% of the surgery group, 52% observation group, and 40% radiosurgery group; p = 0.078), as well as at the last follow-up (73% surgery group, 54% observation group, and 60% radiosurgery group; p = 0.097). The surgery group also had the lowest rebleeding rate during the follow-up period (2% surgery group, 11% observation group, and 20% radiosurgery group; p = 0.021). At the 1-year follow-up, the proportion of seizure-free patients without AEDs was significantly higher in the surgery group than the observation group (88% surgery group vs 53% observation group, p < 0.001), and similar results were obtained at the last follow-up (91% surgery group vs 56% observation group, p = 0.05). The 5-year AED-free rates for the surgery and observation groups were 94% and 50%, respectively, on the cumulative incidence curve (p = 0.049).

CONCLUSIONS

The clinical presentation of pediatric CCM patients was not significantly different from that of adult patients. Lesionectomy may be acceptable for pediatric CCM patients with indications of persistent seizures despite AED medications, rebleeding, and mass effects.

ABBREVIATIONS

AED = antiepileptic drug; CCM = cerebral CM; CM = cavernous malformation; EEG = electroencephalography; mRS = modified Rankin Scale.

Supplementary Materials

    • Supplemental Tables S1–4 (PDF 1,216 KB)
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Illustration from Wang et al. (pp 538–546). © Ju-lei Wang, published with permission.

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