What Is Congenital Hydrocephalus?

Congenital hydrocephalus is also called infantile hydrocephalus. Under normal circumstances, intracranial cerebrospinal fluid is continuously produced and absorbed, maintaining a dynamic balance; if excessive production or (and) absorption and reflux disorders occur, the ventricular system or (and) subarachnoid space will accumulate a large amount of cerebrospinal fluid and expand To form hydrocephalus.

Basic Information

English name: congenital hydrocephalus
Visiting department: neurosurgery
Multiple groups: baby
Common locations: Intracranial

Common causes: The causes are various, with congenital malformations such as midbrain aqueduct stenosis and occlusion, cerebellar subtonsillar hernia, and atresia of the fourth or middle ventricle.
Common symptoms: Symptoms of increased intracranial pressure, often with intellectual changes and developmental disorders
Contagious: no

Causes of congenital hydrocephalus

The causes are many, with congenital malformations such as midbrain aqueduct stenosis and occlusion, cerebellar subtonsillar hernia, and atresia of the fourth or right ventricle. According to the nature of cerebrospinal fluid system dysfunction, it can be divided into obstructive (non-traffic) hydrocephalus and non-obstructive (traffic) hydrocephalus. The former occurs due to the obstruction of the interventricular foramen, the third ventricle, the midbrain aqueduct, the fourth ventricle, its mesopores and lateral foramen, and the cerebellar medulla cistern; the latter is mostly caused by excess cerebrospinal fluid secretion or malabsorption. Due to long-term increased intraventricular pressure, the brain tissue is compressed and degenerative changes occur, which can become extremely thin.

Clinical manifestations of congenital hydrocephalus

Most infants with congenital hydrocephalus begin to increase their skulls in the first few weeks after birth. Generally, they are gradually discovered after 3 to 5 months, and some of them have an enlarged skull at birth. The clinical feature is the progressive abnormal increase of the skull due to the increase of intracranial pressure, which is disproportionate to the development of the whole body. The forehead protrudes forward, the orbital top is compressed downward, the eyes are down, and the eyeballs are turned downward, causing the upper part of the sclera to appear white, the anterior sacral enlargement and increased tension, other cardiac valves can also be enlarged, the skull bone suture separated, and the scalp veins dilated. Skull percussion showed a "broken pot sound". Infants and young children are not closed, and when intracranial pressure is increased, the head may have a compensatory enlargement, so the symptoms of early intracranial pressure increase may not be obvious. However, hydrocephalus is severe and can occur when the disease progresses rapidly. The symptoms are repeated vomiting. Brain degenerative changes, brain development disorders, central paralysis of the limbs, especially the lower limbs, often have intellectual changes and developmental disorders. Optic nerve compression and atrophy can cause blindness. Nystagmus and convulsions are also common. Frequent deformities in other parts of the body are also common. In a few cases, hydrocephalus can stop on its own after a certain period of time, the skull no longer continues to increase, and the intracranial pressure is not high, becoming "quiescent hydrocephalus."

Congenital hydrocephalus examination

Plain skull

The cranial cavity is enlarged, the skull is thinned, the barrier structure is scarce or completely disappeared, the vascular groove is shallow or not visible, the cerebral backpressure trace may be deepened, the cranial suture is separated, the anterior condyle is widened, and the skull-to-face ratio is significantly increased.

2.CT

As the most direct diagnostic method for hydrocephalus, ventriculography has been replaced. CT can not only judge the degree of hydrocephalus, speculate the location of the obstruction, the etiology, or whether it is associated with malformations, and has great guiding significance for treatment.

3. Magnetic resonance imaging

It is also an ideal diagnostic method at present, which can perform three-dimensional imaging. Intracranial structures can be displayed more clearly, especially those at the base of the skull, and the cause and obstructed area can be detected.

Diagnosis of congenital hydrocephalus

Diagnosis can be made based on clinical manifestations and auxiliary examinations.

Congenital hydrocephalus treatment

Divided into non-surgical treatment and surgical treatment.

In general, mild hydrocephalus should be tried first with non-surgical treatment, mainly dehydration therapy and systemic support therapy.

Surgical treatment is suitable for cases with high intraventricular pressure (over 250mmHg) or failure of non-surgical treatment. Severe hydrocephalus, such as a head circumference of more than 44.2cm, and atrophic thickness of the cerebral cortex below 0.88cm, have been combined with severe dysfunction and deformity, the risk of surgery is greater, it is recommended to use an adjustable pressure shunt tube. Cerebrospinal fluid shunt surgery is often used for surgical methods. Such as ventricle-atrial shunt, ventricle-sagittal sinus shunt, ventricle-abdominal shunt. The latter procedure is safe, with fewer complications and more use.