Cavernous Malformations in Children Homepage

 

Author

Xian-Lun Zhu, M.D.

Section Editors

Takayuki Inagaki, M.D.

Edward Smith, M.D.

Gianpiero Tamburrini, M.D.

Editor in Chief

Rick Abbott, M.D.

Introduction

Cavernous malformations are low-flow vascular malformations that have a multilobulated, mulberry-like appearance. They are known also as cavernous hemangiomas, cavernous angiomas, or cavernomas. They are angiographically occult. Cavernous malformations occur mainly in the brain and less often in the spinal cord. They are found in 0.4–0.5% of the general population. 25% are observed in children under 18 years of age, with a mean age of approximately 10 years and no sex predominance. Cavernous malformations occur in sporadic or autosomal dominant inherited form characterized by multiple lesions in children and a strong family history of seizures. They are dynamic lesions with de novo formation, growth, and regression.

About one-third of patients with cavernous malformations are asymptomatic. For symptomatic patients, the common presentations are headache, seizure, and focal neurological deficit. MRI is the imaging method of choice for these lesions. The main indication for therapeutic intervention is the presence of symptoms, such as severe or recurrent symptomatic hematoma or refractory epilepsy. Surgical excision is the primary method of intervention. Radiosurgery may be considered for deep-sited lesions, but its use is controversial.

Key Points

  • Low-flow lesions: Cavernous malformations are low-flow and angiographically occult vascular malformations of the brain and spine.
  • Sporadic and inherited forms: Cavernous malformations occur in sporadic or autosomal dominant inherited form characterized by multiple lesions in children and a strong family history of seizures. De novo formation, growth, or regression may occur.
  • Limited indications for treatment: Therapeutic intervention is indicated mainly for lesion(s) with symptomatic hematoma or refractory epilepsy.
  • Treatment surgical: Surgical excision is the main treatment method. For deep-sited lesions such as those in the brainstem, the surgical risk must be carefully evaluated. The effect of radiosurgery is controversial.