What Is Encephalocele?

The tissue bulging outward from the skull defect mouth is like a mushroom, so it is also called brain mushroom. Brain bulging in the front of the skull is common in males, and brain bulging in the back of the skull is more common in women. Those with mild neurological symptoms have no obvious neurological symptoms, and those with severe neurological symptoms are related to the site of the occurrence and the degree of damage. They can show mental retardation, convulsions and different degrees of paralysis, hypertenoid reflexes, and unstable pathological reflexes.

Causes of Encephalocele

Encephalocele cases are mostly sporadic, and only a few have family history. Other factors such as nutrition, folic acid deficiency, and elevated body temperature in pregnant women.

Clinical manifestations of encephalocele

Local symptoms

Generally, it is a round or oval cystic bulge. If it is located at the root of the nose, it is a flat bulge. The size is different. The large one is similar to a child's head. The small one can be a few centimeters in diameter. Larger, and some gradually grow up. The thickness of the covered soft tissues varies widely. Individuals can be thin, transparent, or even ruptured and leak cerebrospinal fluid, causing repeated infections, resulting in purulent meningitis. Thicker soft tissue is plump, soft and elastic to the touch, and some surfaces appear scarlike and hard. Its base can be a thin pedicle or a broad base. Some can touch the edges of bone defects. Cystic masses are generally soft and elastic, and they can have a fluctuating feeling and increased intracranial pressure when pressure is applied. When the child is crying, the mass increases and the tension increases. The light transmission test is positive, and it is possible to see the shadow of the bulging brain tissue during meningocele.

2. Nervous system

Those with mild symptoms have no obvious neurological symptoms, while those with severe symptoms are related to the location of the occurrence and the degree of damage. They can show mental retardation, convulsions and different degrees of paralysis, hypertenoid reflexes, and unstable pathological reflexes. If it occurs at the root of the nose, one or both sides of the olfactory sense may be lost. If the bulge protrudes into the orbit, there may be involvement of the first, second, and third cerebral nerves of the , , , and brain nerves. If meningocele occurs in the occipital region, there may be manifestations of cortical visual impairment and cerebellar damage.

3. Compression manifestations of adjacent organs

Those who bulge at the root of the nose often cause facial deformity. The nose is flat and wide, the eye distance is enlarged, the orbital cavity becomes smaller, sometimes the eyes are triangular, and the eyes are squeezed to the outside, which can involve the lacrimal gland and cause dacryocystitis. Penetration into the nasal cavity can affect breathing or patency when lying on its side. When the bulge protrudes into the orbit, it can cause the eyeball to protrude and shift, and the orbital cavity increases. Swelling occurs in different parts, and there may be different changes in the shape of the head. For example, the bulge of the occipital part is huge, and the anteroposterior diameter of the head is significantly enlarged due to the prolonged lateral lying position. Sometimes there may be abnormal hair locally.

Encephalocele

CT examination

Not only can it show the morphology of skull defects, but also the presence of cerebrospinal fluid or brain tissue in the bulging soft tissue. If the meningocele is combined, the same density as the brain can be seen, and the size, displacement and deformation of the ventricle can be seen. For patients with cerebral bulge in the front half of the skull, CT examination, especially the application of 3D reconstruction technology, is very helpful in determining whether a craniofacial reconstruction is needed and choosing a reconstruction method. Coronary CT scans are better for those with skull base brain bulge.

2. Magnetic resonance (MRI)

Skull defects and bulging signals from the cerebrospinal fluid, brain tissue, cerebrovascular and dura mater can be seen. The resolution of skull defects is not as clear as CT, but the resolution of bulging contents is higher.

Magnetic resonance angiography

If you need to know the blood supply, you can choose magnetic resonance angiography.

4. Plain skull

The size and extent of the crack can be found.

5. Cranial brain ultrasound

After repairing posterior craniocele, craniocerebral ultrasound is an effective method to track the size of the ventricle and the formation of hydrocephalus. Once hydrocephalus is formed, a cerebrospinal fluid shunt operation is required.

Encephalocele diagnosis

According to the history and clinical manifestations, the location, nature, and appearance of the mass, and a positive light transmission test, a correct diagnosis is generally made.

Differential diagnosis of encephalocele

The occipital meningocele is mainly distinguished from benign scalp masses and pericranial sinuses. Skull base meningocele should be distinguished from nasal polyps and nasopharyngeal tumors, especially in children before nasopharyngeal puncture biopsy, the possibility of meningoencephalocele should be considered to avoid cerebrospinal fluid leakage and concurrent skull Infection.

Encephalocele complications

If surgical treatment is performed, the following complications may occur:

Local effusion

If the cerebrospinal fluid is not absorbed, the fluid will accumulate at the repair site and affect the healing of the incision. Can be controlled by intermittent pumping and pressure bandaging.

Hydrocephalus

It is manifested that the head circumference of the child is enlarged, and the anterior condyle is full. Transcranial ultrasound examination shows progressive expansion of the ventricle. At this time, the ventricle shunt can be placed for treatment. If an infection occurs in the cerebrospinal fluid pathway, the child must be drained outside the brain. After the wound is healed and the infection is cured, hydrocephalus surgery is performed. Although the incidence of hydrocephalus in meningocele is much higher than that of meningocele, the treatment of hydrocephalus is the same.

3. Epilepsy

The occurrence of epilepsy has a greater relationship with central nervous system hypoplasia than with cerebral bulge repair.

Encephalocele treatment

General principle

(1) Meningocele of the newborn should be repaired, and the surgical effect is often ideal.

(2 For those patients with meningoencephalocele, in addition to the size of the lesion, the amount of nerve tissue and the degree of microcephaly, the severity of other deformities should also be considered. The tissue exceeds the intracranial, and the possibility of future intellectual development of the child is zero, and surgical repair may not be considered.

(3) The purpose of the operation is to remove the swollen sac, recapture and protect functional nerve tissue. Removal of dysplastic tissue during repair surgery has no effect on nerve function. In addition to the general dangers of surgery such as anesthesia reactions, bleeding, and infection, the special danger of repairing encephalocele is the contents of the capsule and their relationship with important neurovascular structures, which can be estimated before surgery by MRI and CT scans.

2. Notes

(1) The body temperature of children during surgery should be maintained at 36 ° C to 37 ° C. It is difficult for children with low body temperature to tolerate surgery.

(2) Comprehensive monitoring of the child during the operation. Usually includes: blood pressure, pulse, ECG, oxygen saturation and other vital signs; a Doppler ultrasound probe is placed on the patient's chest to monitor the presence of gas in the circulatory system when the operation is performed to the sinus site.

(3) Correct estimation of blood loss: Children have poor tolerance, so blood loss must be closely monitored to ensure fluid balance. Hemorrhage must be carefully stopped during surgery to minimize blood loss.

(4) Broad lesion antibiotics should be given during the perioperative period for large posterior brain bulging with cerebrospinal fluid leakage or craniofacial reconstruction surgery; for small, completely epithelialized lesions, this is not necessary . When repairing the meningocele of the occipital part and the top part, you can choose a straight or fusiform incision. The resection range should be moderate to prevent excessive tension after suture and poor healing.

Encephalocele prevention

With the routine application of fetal ultrasound and maternal blood alpha-fetoprotein detection, cerebral bulge can be diagnosed in the uterus, which has an important role in determining whether to terminate the pregnancy.

Prognosis of encephalocele

Simple meningocele is generally effective after surgery. It can reduce mortality, reduce the incidence of hydrocephalus, and reduce or alleviate symptoms of neurological damage. Meningocele and ventricular meningocele are generally All were combined with neurological dysfunction, mental retardation and deformity in other parts, and the prognosis was poor. Surgery cannot resolve other deformities and improve intelligence.