head-facial hemangioma syndrome
Introduction
Introduction to head-face hemangioma syndrome Head-to-face hemangioma syndrome is a congenital disease involving the blood vessels of the face and brain. It is characterized by facial vasospasm, seizures, intracranial calcification, brain vascular malformations and mental retardation. Mainly involving the nervous system, skin and eyes. Nervous system symptoms are mainly seizures, and the skin lesions are mainly red wine-colored flat vasospasm (born at birth) along the third branch of the trigeminal nerve, which can spread to the II or III branch, and the severe spread to the side and neck. The Ministry, the trunk, and a few are found in the oral mucosa. 30% of patients with glaucoma and sudden, contralateral isotropic hemianopia (occipital lobe damage), congenital anomalies (such as iris defect and lens opacity), vision loss. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious Complications: glaucoma optic atrophy cryptorchidism spina bifida syringomyelia epilepsy
Cause
Cause of head-face hemangioma syndrome
Capillary dilatation or cavernous hemangioma in the facial trigeminal nerve distribution area and hemangioma of the ipsilateral occipital, apical or frontal lobe (mainly venous), more common in the occipital lobe, cerebral cortical atrophy, sclerosis and Calcification, loss of neurons under the microscope, gliosis and calcium deposition.
Prevention
Head-to-face hemangioma syndrome prevention
The disease is a kind of cerebral vascular malformation, no effective preventive measures, early detection and early treatment is the key to prevention and treatment.
Complication
Head-to-face hemangioma syndrome complications Complications glaucoma optic atrophy cryptorchidism spina bifida sinus epilepsy
There may be a loss of intelligence, contralateral hemianopia and contralateral limb hemiparesis, atrophy and limb growth behind the healthy side, may have the same side of the convex eye, glaucoma, bull's eye or optic atrophy, other parts of the body may also have a wine color Skin sputum, with retinal, kidney, liver and other hemangioma, may also be associated with cryptorchidism, spina bifida, syringomyelia, etc., may be life-threatening due to intracranial hemorrhage or status epilepticus.
Symptom
Symptoms of head-to-face hemangioma syndrome Common symptoms Epilepsy of absence of seizures, facial hypertrophy, iris defect, intelligent loss, lens opacity, occipital lobe, impaired vasospasm, hemian vascular malformation, partial atrophy
Mainly involving the nervous system, skin and eyes.
(1) symptoms of the nervous system: common seizures, may be associated with Todd paralysis, occurring in the age of 1 year, anti-epileptic drugs are difficult to control; with the increase in age often have intelligent decline; facial hemangioma can occur on the opposite side of the hemiplegia and partial body atrophy.
(2) Skin changes: red wine-colored flat vasospasm (born at birth) along the third branch of the trigeminal nerve, can spread to the second or III branch, severe cases spread to the side, neck, trunk, a few found in the oral mucosa The edge of the vasospasm is clear, slightly higher than the skin, the pressure does not fade, involving the forehead, the upper eyelid may be associated with glaucoma and neurological complications, and only the third and third nerves of the trigeminal nerve are rare.
(3) Ocular symptoms: 30% of patients with glaucoma and exophthalmos (protuberance due to prenatal intraocular pressure), contralateral isotropic hemianopia (occipital lobe impaired), congenital anomalies (such as iris Defect and lens opacity), vision loss, etc.
Examine
Examination of head-to-face hemangioma syndrome
The disease can be done the following checks:
After 12 years old, the X-ray film of the skull found the pathological calcification of the ipsilateral side of the facial hemangioma. It was a double-layered wavy shape with a brain-shaped or dendritic shape, which was confirmed.
2CT showed calcification and unilateral brain atrophy, MRI, PET, SPECT showed pial hemangioma, CT showed calcification and unilateral brain atrophy, MRI, PET, SPECT showed pial hemangioma.
3 digital subtraction angiography (DSA) capillary and venous anomalies, capillary surface hyperplasia of the affected hemisphere, significant reduction of veins, dysplasia of the superior sagittal sinus.
4 Electroencephalogram (EEG) indicates that the amplitude of the affected hemisphere is low, and the decrease of wave is consistent with the degree of intracranial calcification, and epileptic waves can be seen.
Diagnosis
Diagnosis and differentiation of head-face hemangioma syndrome
It is not difficult to distinguish from hemangioma and seizure by examination and symptom.
1. Hemangioma (haemangioma) is a congenital benign tumor or vascular malformation. It is more common in infants born or shortly after birth. It originates from residual embryonic hemangioblasts. Active endothelium-like germs invade adjacent tissues to form endothelial splines. Suspension, after the tube is connected with the remaining blood vessels to form a hemangioma, the intratumoral blood vessels are self-contained and are not connected to the surrounding blood vessels. The hemangioma that occurs in the oral and maxillofacial region accounts for 60% of systemic hemangioma, most of which occurs in facial skin, subcutaneous tissue and oral mucosa, such as the tongue, lips, and fundus.
2. Epilepsy is commonly known as "sheep epilepsy", "shofar wind", "lamb mad" or "sexual epilepsy", which is caused by a variety of causes of paroxysmal abnormal discharge of brain neuron group, feeling, A disease of consciousness, spirit, and autonomic dysfunction. As early as 2,200 years ago, the "Yellow Emperor's Internal Classic" was recorded. I believe that people will not be familiar with this disease, and they are often referred to as exhaustion by people.
