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Clinical Features and Treatment of Pediatric Cerebral Cavernous Malformations

  • Ji Hoon Phi (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Seung-Ki Kim (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
  • Received : 2024.02.20
  • Accepted : 2024.03.27
  • Published : 2024.05.01

Abstract

Cerebral cavernous malformation (CCM) is a vascular anomaly commonly found in children and young adults. Common clinical presentations of pediatric patients with CCMs include headache, focal neurological deficits, and seizures. Approximately 40% of pediatric patients are asymptomatic. Understanding the natural history of CCM is crucial and hemorrhagic rates are higher in patients with an initial hemorrhagic presentation, whereas it is low in asymptomatic patients. There is a phenomenon known as temporal clustering in which a higher frequency of symptomatic hemorrhages occurs within a few years following the initial hemorrhagic event. Surgical resection remains the mainstay of treatment for pediatric CCMs. Excision of a hemosiderin-laden rim is controversial regarding its impact on epilepsy outcomes. Stereotactic radiosurgery is an alternative treatment, especially for deep-seated CCMs, but its true efficacy needs to be verified in a clinical trial.

Keywords

Acknowledgement

This study was supported by a grant from SNUH Kun-hee Lee Child Cancer & Rare Disease Project, Republic of Korea (No. 22A-003-0100; to Phi JH).

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