Brief history Chief Complaint : Progressive malaise and sleepy for more than 3 months. Present illness : Progressive sleepy, drowsy, impaired recent memory since 3 months ago. Right limbs hemiparesis and clumsy since 2 days prior to admission-> 署立豐原醫院 Brain MRI: multiple brain lesion. Chest, Abdomen CT from other hospital: negative finding.
Hospital course Admission for second opinion Tumor markers: all within normal range CT guide navigation assisted biopsy was performed Frozen section report: old hemorrhage Permanent biopsy.
Cavernous hemangioma AKA: cavernoma, cavernous malformation, angioma Benign vascular harmatoma consisting of irregular thick and thin walled sinusoidal vascular channels located within brain without interventing neural parenchyma.
Cavernoma--Epidemiology 1-5 cm in size. Multiple in 50 % cases. In White matter. About 0.4-0.9% in population.Comprise 5-13% CNS vascular malformation Mostly supratentorial, but 10-23% locates in posterior fossa with a predilection for the pons. Sporadic or hereditary type. The hereditary type (familial type) may be inherited in AD pattern. It is more common in Hispanics. J Neurosurg 95:825–832, 2001
Presentation Seizures (60%), Neurological deficit(50%), hemorrhage (20%), hydrocephalus. Hemorrhage tend to occur in young group in familial than sporatic ones. Through repeated small hemorrhage in these lesions, they are rarely devastating. In familial ones, CCM1(KRIT 1 gene) and CCM2 gene on Chromosome 7 had been demonstrated. Risk of bleeding: 0.5%-1% (May be more frequent in basal ganglia, thalamus, spinal cord). Rebleeding: 4-10 % per year.
Evaluation Best detected in MRI best sequence: regular T2 : popcorn appearance. Gradient echo (GRE): Indian ink, blotch appearance. CT: heterogenous hyperdense (hemorrhage or calcification)without enhancement). Non detectable on angiography( i.e. AOVM : angiographic occult venous malformation)
Treatment Accessible lesion with focal neurological deficit: surgically excision. Do not response well to radiation therapy or radiosurgery. (But some reported it to reduce re- bleeding rate) Conservative treatment: conservative management is recommended for patients harboring asymptomatic lesions without bleeding, especially if deeply located, in eloquent areas, or in patients with multiple lesions(1)
Surgical indications include (1) progressive neurologic deficits; (2) grave neurologic deficits like coma, cardiopulmonary instability; (3) overt acute or subacute hemorrhage on MRI either inside or outside cavernous malformations with mass effect;(4) cavernoma or hematoma 2 mm from brain stem surface. Timing of surgery should be decided at about 1-2 week, the time after edema subsided and before hematoma resorption and gliosis development. (2)