Presentation of Cavernous Malformations in Children

Symptoms

Incidental

• Common incidental finding: In the MRI era, many cavernous malformations are diagnosed as an incidental finding. The percentage of cavernous malformation patients with incidental presentation has been reported to be from 2–32% (66).

Symptomatic

A patient may present with symptoms that include headache and seizure. Imaging finding can include intracranial hemorrhage. Neurological deficits can be present with or without hemorrhage.

• Headache: 6–65% of patients present with chronic headache.
• Seizures: Seizures are the most common presentation, accounting for 40–50% of symptomatic cases, especially for supratentorial cavernous malformations. The risk for new seizures following a cavernous malformation diagnosis ranges from 1.5–4.3%/patient/year, and a history of a previous seizure increases this rate to 5.5%/ patient/year (66).

Patterns of evolution

• Repeated hemorrhages with seizures: A common pattern of evolution for a cavernous malformation is recurrent seizures after a presenting hemorrhage. It is exemplified in the case history in the following child page.

Time for evolution

• 0.1–2.7% risk of hemorrhage/year: Cavernous malformations have symptomatic hemorrhage rates ranging from 0.1–2.7% /lesion/year and 0.7–16.5% /patient/year (66).
• Risk of future hemorrhage increases to 4.5–8.9%/year after first hemorrhage: Patients presenting with symptomatic hemorrhage have an increased risk of further hemorrhage, ranging from 4.5–8.9% per patient-year (66). The period of increased hemorrhage risk is time limited, approximately within 2 years of the initial hemorrhage. This phenomenon is also known as “temporal clustering” (2).

Intervention at Presentation

Stabilization

• Routine measures for stroke and/or seizures: Principles of management for acute stroke or seizures are applied.

Preparation for definitive intervention, nonemergent

• Proceed to routine evaluation

Preparation for definitive intervention, emergent

• CT angiography or angiography: For massive intracranial hematomas, emergency evacuation may be needed. To prepare for it, apart from a diagnostic CT to determine the size and location of the hematoma, CT angiography or DSA may be considered if AVM is suspected. Possible coagulopathy should be checked.
• Prepare for surgery: Preoperative preparation is standard.

Admission Orders

• Routine orders for patient with hemorrhagic stroke: The patient should be kept NPO, with IV fluids to maintain physiological requirements. Vital signs, intake-output and neurological status should be observed closely for signs of deterioration. Tracheal intubation and ventilation may be needed for comatose patients or patients with refractory status epilepticus. Clotting profile, complete blood analysis, and renal and liver function studies as well as type and screen for blood