Craniocerebral congenital malformations

Introduction to congenital malformations of the brain The cranial fissure occurs in the midline of the skull, and both the cranial and cranial bases can occur. More common in the occipital region, also seen in the top of the forehead; the bottom of the skull can bulge from the base of the nose, nasal cavity, nasopharyngeal cavity or eyelids. Individual cases can bulge to the side. Recessive cranial fissure, no mass on the outside. Cystic cleft lip can be divided into meningocele or meningococcal bulging, which contains tissue, or partially dilated ventricles. In this case, the skull is often a small head, and the bulk is particularly large. The base of the mass is large or small. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: hydrocephalus, mental retardation

The cause of congenital malformation of the brain

The causes of brain penetrating malformations are diverse and can be roughly divided into two categories: congenital and acquired. Naef (1958) proposed the etiology and variability of this disease.

(1 ) Congenital brain penetrating malformation

It is generally believed that congenital sputum perforation malformation is related to dysplasia of embryonic stage or maternal dystrophic disorder, and may also be related to genetic factors. Familial cerebral perforation malformation has been reported. In 1983, Berg reported a group of 5 cases of familial cerebral perforation malformation. In 1986, Zonana reported that 6 of the 2 families had hemiparesis in infants, and 5 of them had congenital cerebral perforation malformations.

(2) Acquired brain penetrating malformation

Acquired brain penetrating malformation is caused by brain tissue destruction caused by various causes, including birth injury, craniocerebral trauma (especially craniocerebral firearm injury), intracranial hematoma, intracranial inflammation, asphyxia, brain surgery, cerebral infarction, etc. Various diseases that cause cerebral vascular dysfunction. In addition, cerebrospinal fluid circulatory disorders, ventricular puncture, hydrocephalus, intracranial benign cysts spontaneously break into the ventricles and brain degenerative diseases may also be the cause.

Cranial congenital malformation prevention

The disease is congenital malformation, and prenatal diagnosis should be done.

Cranial congenital malformation complications Complications, hydrocephalus, mental retardation

1. Brain fissure hole brain: It is a true defect in brain development. It is characterized by ectopic gray matter ectopic, multiple cerebellar gyrus and fibrosis, usually bilateral. The symmetry is connected to the ventricle, and can also communicate with the subarachnoid space, and the wall of the capsule is the ependymal membrane. The wall of the brain penetrating malformation is connective tissue, sometimes accompanied by lymphocyte infiltration. This will distinguish the two.

2, benign brain cyst: the so-called pseudo brain penetrating malformation. About 0.4 to 1% of intracranial space-occupying lesions are a type of single or multiple brain cavity that does not communicate with the ventricles. A clear, sleek film envelops the colorless, clear liquid. The cause may be caused by cerebral vascular injury during production, and occurs mostly along the middle cerebral artery distribution area. The two can be identified by means of CT.

Symptoms of Congenital Malformations of the Brain Common Symptoms Craniofacial Malformation Periodic Sleepiness Strong Eating Disease Brain Occupational Lesion Intracranial High Pressure Oblique Head Malformation Cerebrovascular Arteriovenous Malformation Cysts Perforation Malformation Tension

Brain penetrating malformations are different because of their different causes and symptoms. Its clinical manifestations depend mainly on the size, location and tension of the cyst. Due to its diversified performance and low incidence, it is difficult to understand clinically. Ramery (1977) pointed out that this disease can cause chronic brain compression, ventricular system enlargement and secondary intracranial hypertension. Barret (1965) believes that the disease is characterized by congenital hemiplegia, partial hemiplegia of the contralateral sacral hemiplegia, unilaterally visible light transmission on the skull, and a low voltage on one side of the EEG. Brain tissue defects, brain atrophy, thrombosis, and brain tissue necrosis can cause significant hemiplegia. Positive signs of light transmission and low voltage of unilateral EEG are secondary to cortical atrophy and cerebrospinal fluid accumulation. The thinning of the inner plate of the local skull bulge may be caused by the choroid plexus pulsation spreading to the sac.

Examination of congenital malformations of the brain

According to the appearance characteristics of the bulging mass and the combined neurological examination, it is not difficult to make a correct diagnosis. At the base of the mass, sometimes the skull defect can be touched, including the light transmission test, to know whether the brain tissue is included. A flat slice of the skull can determine the location and extent of the bone defect.

Diagnosis and diagnosis of congenital malformation of the brain

Differential diagnosis

(A) congenital hydrocephalus hydrocephalus is mainly caused by a large increase in hydrocephalus in the brain. The clinical manifestations are mainly the rapid increase of the skull, the separation of the cranial suture, the enlargement of the cardia, and the positive test of the head light. There are different degrees of mental retardation and other signs of the nervous system depending on the length of the disease.

(B) cranial stenosis due to premature closure of the skull suture, can be divided into hereditary diseases, can also spread the disease. Clinical manifestations of small cranial circumference, the formation of sharp cranial malformations, often increased intracranial pressure and mental retardation.

(3) Brain penetrating malformation lesions have one or more leaky head-like cavities in the cerebral hemisphere, which can communicate with the ventricle or the subarachnoid space. Symptoms are obvious mental disorders and other symptoms of the nervous system.

(4) The gross deformity is a rare condition, with a large head and a large brain (partially caused by the proliferation of glial cells). Intelligence can be low, normal or abnormal.

(5) The primary sex of the microcephaly is caused by autosomal recessive inheritance, and the secondary is caused by viral infection of the pregnant woman or other reasons. The former is often accompanied by moderate and severe mental retardation, and the latter's intelligence level depends on the cause and the size of the head.

(6) Cerebral gyrus deformities include no brain return, large or small cerebral gyrus, and obvious mental and emotional disorders.

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