Clinical Decisions in Cavernous Angiomas Issam A. Awad, MD, MSc, FACS, MA (hon) Professor of Neurosurgery Northwestern University Feinberg School of Medicine Evanston Northwestern Healthcare Evanston, Illinois Chairman, Scientific Advisory Board Angioma Alliance (

Confirming the Diagnosis of CM and Assessing Prognosis Is it a CM? Is it solitary or multiple/familial? Is there an associated VM? Risks and Consequences of hemorrhage?

Projecting Natural Risk of CM Likelihood of hemorrhage? (consider recent clinical behavior) Consequences of hemorrhage ( consider lesion location) Impact of hemorrhage(s) over lifetime (consider host’s life expectancy)

Assessing Management Options Expectant-- medical therapy, regular surveillance CM microsurgical excision-- define threshold for intervention (preventive, after one bleed, near disability, etc…) Other options-- ? radiosurgery

Evaluate Options of Medical Therapy Seizure control, medicines and side effects Bleeding risk, consequences Impact of living with the lesion or with epilepsy-- life decisions, careers, parenting, etc… Do not “wrap yourself in bubble”-- few real restrictions

Microsurgical Excision of CM Hemorrhage or other symptoms Opportunity for “cure” (solitary, no large VM) Accessibility, approach Eloquence Risk-- defines the threshold for choosing surgery

What About Radiosurgery ? Does not eliminate the lesion May not alter natural risk Complications high unless dosimetry is very low (? effective) Radiation and CM genesis Truly inaccessible lesions with repeated bleeds (any such CMs?)

Special Considerations for Epilepsy and CM Single versus multiple lesions and epilepsy Lesion and seizure concordance--clinical, diagnostic, role of mapping Lesionectomy, versus lesionectomy “plus” Control versus cure (lesion-free, seizure- free, medication-free)

Special Considerations for Brainstem CMs Expensive real estate-- natural risk and treatment risks Higher stakes, higher threshold-- not surgery at any cost, but do not wait too long Approach and exposure-- experience Access, size, hematoma, associated VM-- what is operable?

Special Considerations for Brainstem CMs

Special Consideration for CM with associated VM Leave alone if large VM and many CMs Leave alone if extensive VM and minimal CM Excise CM if large, growing or symptomatic-- preserve the VM, unless very tiny

Special Considerations for Spinal CMs Not very different from brainstem CMs-- pathoanatomy and clinical sequels Keep high index of suspicion Excise if solitary, accessible, growing, symptomatic Do not wait till advanced symptoms

What About Pregnancy? CCMs may bleed during pregnancy (more likely ?) Great majority of patients and lesions have unremarkable pregnancies Epilepsy and pregnancy-- anticonvulsant medications ESSENTIAL Expectant management during pregnancy-- be aware and watch CLOSELY

Balance of Clinical Decisions Factors Favoring Surgery Solitary lesion Accessible lesion Symptomatic lesion Growing lesion Long life expectancy Bad consequences of lesion misbehavior Factors Favoring Expectant Management Multiple lesions Associated VMs (large) Deep lesions Longstanding quiescence Shorter life expectancy Risks of surgery

Surgical Adjuncts Microsurgery, stereotactic guidance Brain mapping-- preoperative, intraoperative Skull base approaches Team experience-- critical care, surgery, rehabilitation

Threshold for Intervention: Wisdom and Experience Preventive ? Curative ? In response to CM misbehavior ? How long? Threshold of acceptable morbidity of treatment

Threshold for Intervention: Wisdom and Experience Preventive ? Curative ? In response to CM misbehavior ? How long? Threshold of acceptable morbidity of treatment

Research Directions Epidemiology, natural history, outcome studies, QOL Genotyping, genotype- phenotype correlations Molecular architecture and function in CCM lesions Molecular mechanisms of lesion genesis and clinical behavior Translational Research: Bedside to bench to patients and loved ones..