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Combine conference
R3 陳斯逸 /VS 孫銘希Jan.8.2007
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General data ID: 1878644F Male 67 y/o Farmer
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Brief history Chief Complaint: Progressive malaise and sleepy for more than 3 months. Present illness: Progressive sleepy, drowsy, impaired recent memory si
nce 3 months ago.Right limbs hemiparesis and clumsy since 2 days prior t
o admission-> 署立豐原醫院Brain MRI: multiple brain lesion.Chest, Abdomen CT from other hospital: negative findi
ng.
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Neurological examination
Arousable, but stupor. Incoherent speech. GCS level: E4V4M5-6.
Intact cranial nerve function Muscle power: RUL/LUL: grade 3/5 Pathological reflex: bilateral Babinski’s sign (+).
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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.
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Cavernous hemangioma AKA: cavernoma, cavernous malformation, angio
ma Benign vascular harmatoma consisting of irregula
r thick and thin walled sinusoidal vascular channels located within brain without interventing neural parenchyma.
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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
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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.
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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)
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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)
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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)
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J. Neurosurg. / Volume 95 / November, 2001
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Reference
1. Neurosurg Focus 21 (1):E11, 2006
2. J. Neurosurg. / Volume 95 / November, 2001
3. Surgical Neurology 2003(59) 444-454
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Thank you for attention.