Cerebrovascular malformations
Cerebrovascular malformations are congenital, non-neoplastic developmental abnormalities of the cerebral blood vessels. It is an abnormality in the number and structure of local blood vessels in the brain caused by the developmental disorder of cerebral blood vessels, and affects the normal cerebral blood flow. Its rupture and bleeding mainly manifests as intracerebral hemorrhage or hematoma. It is mostly seen in young people, with an average age of onset of 20 to 40 years.
Etiology
1.Arteriovenous malformation
There are two types of arteriovenous malformations, typical and Galen’s large vein malformation: clinical symptoms are important because of blood theft, in addition to occupancy and compression. Intracranial vascular murmur is obvious. Hydrocephalus may appear in infancy. The morbidity and mortality rate is high, with approximately 50% death. Those with small blood fractions have mild heart failure and may have recurrent transient hemiparesis. Treatment is difficult and staged surgery may be performed.
2. Congenital intracranial cystic aneurysm
It is less common in pediatric patients. It mainly occurs at the base of the skull in the internal carotid artery, anterior and posterior communicating arteries, or vertebrobasilar artery. The local elastic and muscular layers of the artery are weakened and protrude as aneurysms, usually below 1 cm.
3.Venous hemangioma
They are more common, occurring in the cerebral hemispheres, and are mostly seen in older children. Vascular malformations of l mm to several cm in diameter can be seen in neuroimaging, and about 15% have calcification.
4.Cavernous hemangioma
It is a dense, thin-walled blood vessel mostly found in the cerebral hemisphere. They are often asymptomatic in childhood and are discovered incidentally. Symptoms usually do not appear until older children or adults, mainly epilepsy, headache and intracerebral hemorrhage. Familial cases are common and are dominantly inherited. Similar cavernous hemangiomas of the retina, liver, kidneys, and skin are also seen in this disease.
Clinical manifestations
1.Pulsatile headache
Located on the diseased side and may be accompanied by intracranial vascular murmurs.
2.Hemorrhage
It is often the first symptom, manifested as subarachnoid hemorrhage or intracerebral hematoma.
3.Epilepsy
It can be the first symptom or seen after hemorrhage, mostly generalized seizures or limited seizures, and limited seizures have localization significance.
4.Concomitant symptoms
Those with supratentorial lesions may have mental abnormalities, hemiparesis, aphasia, dyslexia, and dyscalculia. In subscapular lesions, vertigo, diplopia, ophthalmoplegia and gait instability are often seen.
Examination
1.Cephalometric plain film
It shows the tortuous widening of middle meningeal artery, suggesting the possibility of malformed vessels.
2.CT of the head
It can detect hematoma and provide the possibility of malformed blood vessels.
3.Head magnetic resonance imaging
Superior to CT, it can not only show the malformed blood vessels and their relationship with the surrounding brain tissue, but also distinguish between hemorrhage and calcification.
4.Cerebral angiography
It is the most reliable and main diagnostic method for this disease, and can perform endovascular intervention.
Diagnosis
The diagnosis can be confirmed based on clinical manifestations combined with laboratory tests.
Treatment
1.Surgical treatment
2.Interventional endovascular treatment
3.Stereotactic radiotherapy (γ-knife, X-knife)
4.Treatment of spontaneous bleeding
5.Symptomatic treatment
6.Medication principles
(1) In case of spontaneous bleeding, immediately use hemostatic drugs such as 6-aminocaproic acid, lower cranial pressure with 20% mannitol or tachyphylaxis, and use human albumin if necessary.
(2) Long-term systematic treatment of epileptic patients, according to the type of epilepsy, respectively: phenytoin sodium, sodium valproate, carbamazepine, etc., and barbiturates if necessary.
(3) Other symptoms, symptomatic and supportive treatment.