Key insights
A review of 630 pediatric CCM cases from 94 publications surfaced age-related patterns in presentation and management:
Infants more often presented with signs of elevated intracranial pressure, while older children were more likely to have headaches and multiple lesions.
Decisions about surgery versus observation depend on each child’s symptoms, hemorrhage history, lesion characteristics and surgical risk.
The review findings can inform treatment discussions with families but can’t establish that surgery caused better outcomes nor predict results over a child’s lifetime.
When should pediatric CCMs be treated surgically?
For a child with a cerebral cavernous malformation (CCM), deciding whether to operate means weighing the immediate risks of surgery against the possibility of hemorrhage over decades of life. A prior hemorrhage increases the risk of another bleed, which can cause seizures or neurological deficits.
Brett Whittemore, M.D. and Bruno Braga, M.D., both Pediatric Neurosurgeons at Children’s Health℠ and Associate Professors at UT Southwestern, worked with colleagues throughout the U.S. and abroad to analyze 630 cases from 94 publications. Their review, published in the July 2025 Journal of Clinical Neuroscience, identified age-related patterns in presentation and management of CCMs. Although these patterns can help physicians counsel families about surgery and observation, treatment decisions for individual children still depend on their unique symptoms, hemorrhage history and lesion characteristics.
What did you hope to learn from reviewing published pediatric CCM cases?
Dr. Whittemore: We hoped to identify patterns in how children with CCMs present, which patients undergo surgery and what outcomes are reported. Pooling published cases gives us a broader picture than our own experience, although it can’t tell us which treatment choice is best for a particular child.
How did presentation differ among infants, children and adolescents?
Dr. Braga: The study found that infants more often presented with signs of elevated intracranial pressure, while headaches became more common with age. That difference partly reflects how children communicate: infants can’t describe headaches or numbness.
Dr. Whittemore: Older children in the review were more likely to have multiple lesions. That finding can prompt a closer look at family history and whether genetic evaluation is appropriate.
What weighs most heavily when deciding whether to operate?
Dr. Whittemore: A seizure associated with a solitary, resectable lesion can be a strong reason to consider surgery. Removing the lesion may offer a chance to control seizures and eventually stop antiseizure medication. In adolescents in the review, seizures and isolated lesions were associated with surgical management. However, a review can’t establish whether surgery improved long-term seizure control.
Dr. Braga: Other important considerations include whether a lesion has bled, its location and which functions surgery might put at risk. With multiple lesions, removing one doesn’t address the others. Surgical risk may outweigh the benefit for a deep lesion or one near an area controlling critical functions. At Children’s Health, we discuss those trade-offs with the family. If we choose observation, we continue to follow the child and can revisit the decision as their condition changes.
Dr. Whittemore: In our clinical practice, infants presenting with signs of elevated intracranial pressure require prompt evaluation. Depending on what that evaluation shows, we may consider surgery more urgently.
What do the outcome findings tell us about surgery versus observation?
Dr. Braga: A limitation of our study is that reviewing patient cases doesn't provide the type of comparison needed to compare treatment paths. For example, improvement and symptom-free follow-up were reported more often after surgery among children ages 1 through 12. However, we recognize that most of the published cases involved surgery and that the clinicians may have selected patients they expected to do well. Thus, we can’t conclude, based on this study, that surgery caused better results.
Dr. Whittemore: The review found no significant difference in outcomes across age groups at the last reported follow-up. That finding doesn’t tell us what happens over a child’s lifetime or which treatment will help a particular patient.
Did the review change how you care for children with CCMs?
Dr. Whittemore: It strengthened the approach we already use by showing how our clinical judgments fit with patterns reported elsewhere. For example, headaches are common in adolescents, and a CCM found during a headache workup may be incidental. We shouldn’t assume that surgery will relieve the headache.
What questions should future research answer?
Dr. Whittemore: If a child with seizures has a lesion that we can remove safely, would operating earlier improve long-term seizure control? We also need better ways to identify which lesions are likely to grow, bleed or cause seizures.
Dr. Braga: Another interesting question is whether removing an accessible lesion before it causes symptoms benefits a child over their lifetime. Prospective studies across institutions could help answer questions that individual centers can’t resolve alone.
Why Children’s Health: Expertise and collaboration in pediatric CCM care
Our nationally ranked, high-volume pediatric neurosurgery program has the expertise to treat children with complex vascular malformations. The team meets monthly to discuss these cases, and collaborations with researchers at other top-tier institutions – like this review – help advance care for children with CCMs.
