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Encephalocele - Seattle Children's

What is an encephalocele?

An encephalocele (pronounced in-SEF-a-lo-seal) is a birth defect that affects the brain. It occurs early in a woman’s pregnancy when part of the baby’s skull does not close all the way. Part of the baby’s brain may come through the hole in the skull. Sometimes, part of the membrane that covers the brain and spinal cord (meninges) and cerebrospinal fluid (CSF) also come through the hole in the skull.

Normally, a baby’s brain and spinal cord (central nervous system) develop inside a structure called the neural tube. When the neural tube does not close the normal way, part of the brain may stick outside the neural tube. Either skin or a thin membrane covers the part of the brain that is outside the skull. Doctors call this covering a sac.

An encephalocele can be in any of these places:

• In the base of the skull
• In the area of the nose, sinuses and forehead
• From the top of the skull around to the back of the skull at the midline

Encephalocele in Children

The Centers for Disease Control and Prevention estimates about 375 babies are born each year in the United States with encephalocele. In some parts of the world, encephalocele is more common. It is one of the least common neural tube defects in the United States.

These are some common traits of encephaloceles:

• Girls are more likely to have an encephalocele in the back of their skull.
• Boys are more likely to have an encephalocele in the front of their skull.
• In North America, encephalocele happens more often in the back of the skull.
• In Southeast Asia, encephalocele happens more often in the front of the skull.

Doctors think that the genes babies get from their parents might play a role in causing an encephalocele. The condition happens more often in families that have a history of neural tube defects called spina bifida and anencephaly.

Encephalocele at Seattle Children’s

Seattle Children’s has the largest team in the region to treat encephalocele. After we carefully evaluate your baby, we tailor a treatment plan to their needs.

Based on where the encephalocele is, babies may have other conditions, like these:

• Hydrocephalus
• Vision changes
• Pituitary problems
• Differences with the bones in their skull and face

Seattle Children’s brings together experts from many medical specialties, including Neurosurgery, Craniofacial, Plastic Surgery, Ophthalmology and Endocrinology, to take care of your child.

If your doctor suspects or sees from a prenatal ultrasound that your baby has an encephalocele, our experts can use fetal MRI (magnetic resonance imaging) to get more details about your baby’s condition before birth. We have one of the leading fetal MRI researchers in the country, Dr. Dan Doherty. Pregnant women can come to Seattle Children’s Fetal Care and Treatment Center for an evaluation. Our doctors can consult with you about what to expect and what treatment your baby might need after birth.

Symptoms of Encephalocele

Some babies with encephaloceles have other problems with their skulls and brains. These are some of the symptoms and conditions that might happen with encephalocele:

• Too much cerebrospinal fluid (CSF) in parts of the brain (hydrocephalus)
• Very small head (microcephaly)
• Seizures
• Problems with vision
• Problems with breathing if there is a large encephalocele around the nose
• Swallowing problems
• Pain around the encephalocele
• Delayed growth and development
• Spasticity (high muscle tone) or other movement disorders

Diagnosing Encephalocele

Usually doctors can see an encephalocele when a baby is born because there is a bulge on the head and a divide (cleft) in the skull or facial bones. But sometimes encephalocele is diagnosed later, even when a child is a few years old. Sometimes, doctors may not see a small encephalocele right away. These small encephaloceles are usually around the baby’s nose, sinuses and forehead.

Sometimes doctors can see an encephalocele on a prenatal ultrasound. If this happens, fetal MRI (magnetic resonance imaging), available at Seattle Children’s, can provide more details about your baby’s condition. Pregnant women can come to Seattle Children’s Fetal Care and Treatment Center or the Prenatal Diagnosis Clinic at University of Washington Medical Center for an evaluation. Our doctors can consult with you about what to expect and what treatment your baby might need after birth.

Treating Encephalocele

In most cases, treatment for encephalocele is surgery to put the part of the brain that is outside the skull back into place and close the opening. Our neurosurgeons can often repair even large encephaloceles without causing your baby to lose more function.

Your baby’s treatment will be tailored to their needs. Often, babies with encephaloceles need to be evaluated and treated by experts from Craniofacial, Plastic Surgery, Ophthalmology and Endocrinology along with Neurosurgery. Sometimes a shunt is needed to drain cerebrospinal fluid (CSF) from around the brain. Seattle Children’s brings together a complete team to care for your child in one place.

Surgery for Encephalocele

Usually, neurosurgeons repair encephaloceles within the first few months of life. If skin covers your baby’s encephalocele, giving it some protection, the neurosurgeon may recommend waiting for a few months. If there is no skin protecting the encephalocele, your baby’s neurosurgeon may recommend surgery soon after birth. In more complex cases, babies may have several surgeries done in stages. They may need surgery delayed until they are bigger, which makes surgery safer and easier on your child.

To treat an encephalocele, the neurosurgeon cuts and removes a piece of bone from your child’s skull. Next, the neurosurgeon cuts the membrane that protects the brain (dura mater). This part of the surgery (craniotomy) allows them to get to your child’s brain.

The neurosurgeon then replaces the brain tissue and any membranes or fluids that have come out of the hole in the skull. They remove the sac that surrounded it. Then the neurosurgeon closes the dura mater. They close the skull using the same piece of bone they removed, if possible. If there is a large hole in the skull, the neurosurgeon may use an artificial plate to close it.

Craniostenosis in children

Craniostenosis in children can be either congenital or acquired after birth. This defect is an early fusion or lack of sutures in the bones of the skull, which should normally remain plastic for the natural growth and development of the child's brain. As a result, intracranial pressure rises, and the shape of the skull changes. If we are talking about a developmental defect, craniostenosis in newborns may be accompanied by other defects that affect the brain and other organs of the body. In the case of an acquired disease, fusion of the sutures occurs due to trauma or surgery.

Craniostenosis occurs in one in 1,000 newborns.

What causes disease

Doctors are unanimous in their opinion: heredity does not affect the premature fusion of the bones of the skull. And if the situation with an acquired disease is more or less clear, then the appearance of a congenital disease can be influenced with varying degrees of probability by:

• congenital malformations of the fetus;
• difficulties in the formation of the skull of the embryo in the first 12 weeks of pregnancy;
• infections that affect the fetus in utero (herpes, rubella, toxoplasmosis, and others).

Why craniostenosis is dangerous

The lack of movable sutures in the skull severely limits the brain. Therefore, the sooner help comes for craniostenosis, the more likely the child will be completely cured in the future. Compensation mechanisms in infants under 2 years of age are very high, while rehabilitation after this age is more complex and lengthy. If not treated in a timely manner, the disease can cause the following conditions:

If not treated in a timely manner, the disease can cause the following conditions:

• delayed physical, mental, intellectual development;
• disruption of normal brain functions;
• skeletal underdevelopment;
• compression and atrophy of the optic nerve up to complete loss of vision;
• headaches;
• ophthalmic diseases;
• death.

What is craniostenosis in children

First of all, it depends on whether the child has other malformations. Sometimes pathology accompanies various syndromes - then it is called syndromic. For example, it can occur simultaneously with fusion of fingers or toes, cleft palate or lip, cerebral hernia.

If the fusion of the sutures in the bones of the skull occurs without other developmental defects, it is considered non-syndromic, that is, independent.

Doctors classify the disease into types based on which cranial sutures are fused:

• metopic,
• lambdoidal,
• coronary,
• sagittal.

Synostosis - or fusion of the sutures - can involve from one to several sutures. There is the concept of pansynostosis - complete overgrowth of all sutures. This type of pathology can be considered the most severe, it is less common than others.

How is craniostenosis treated in children

There is only one way to eliminate the pathology - surgery. It is carried out to restore the shape of the bones of the skull. After the bones take their natural shape, they are fastened with mini-screws or mini-plates, surgical wire, which are removed after a year. They are removed after a year. During the operation, doctors use a modern technological biodegradable material that does not need to be removed surgically - it gradually dissolves itself, and its own bone tissue grows in its place. This greatly simplifies patient recovery.

The best results can be expected if the operation is performed between the ages of 3–4 months and 2 years. Severe deformities of the skull can sometimes be noticed only after the bones have completed their formation, that is, at the age of 5–6 years. In such cases, the pathology manifests itself as severe headaches, blurred vision and increased intraocular pressure, increased fatigue and irritability.

If the disease occurs as a response to a craniotomy or after an injury, the deformed area of ​​the bone is removed, and an implant made of a modern material — polymer, metal or ceramic — is placed in its place.

Medical organizations

Help for children with this pathology is provided in the following Russian clinics:

• Children's Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan (Kazan),
• FGBU NMITs DGOI them. Dmitry Rogachev of the Ministry of Health of Russia.
  

The help of the Liniya Zhizni Charitable Foundation in the treatment of children with craniostenosis consists in the purchase of dressing materials and instruments that are required to correct the defect.

A child has an injury: what to do?

April 24, 2021 read 7-10 minutes

Children do not know how to calculate the consequences of their actions, they are curious and do not understand when to stop in exploring the world. We tell you how to reduce the likelihood of injury and what to do if this happens.

How it happens

Every year, 830,000 children die worldwide due to accidental injury. Half of the cases in our country, according to Rosstat, are domestic injuries, 34% are injuries on the street. In boys, injuries occur more often and the consequences are more serious. This is usually associated with a higher level of activity and social approval, parents are less likely to limit their exploratory activities. Most childhood injuries are superficial, about 15% are dislocations and fractures of the arms, and a very small part are fractures of the legs and head.

In the first place for reasons - parental oversight. When it seems that nothing will happen to the child or adults have underestimated the danger of the situation. This is also confirmed by our experts.

“Children’s traumatism and its prevention is a serious problem,” says Ilya Zelenkin, a pediatric orthopedic traumatologist, surgeon at the Fantasy clinic, “especially during school holidays, when children have more free time, are more often on the street and are left without adult supervision . Despite the wide variety of injuries in children, the causes that cause them are typical. First of all, it is the inconvenience of the external environment, the neglect of adults, the careless, incorrect behavior of the child at home, on the street, during games, sports. The psychological characteristics of children also contribute to the occurrence of injuries: curiosity, great mobility, emotionality, lack of life experience, and hence the lack of a sense of danger.

In infancy, falls from the bed or changing table most often occur, in second place is the “shaken baby” syndrome, when a parent who has lost his temper shakes the child with strong crying. In both cases, the main danger is damage to the brain and neck.

When a child starts to crawl, he often injures his fingers. To avoid this, you need stoppers in the doors and protection on the drawers. As soon as a child starts learning to stand up, head bruises from unsuccessful attempts and minor injuries from meeting with the outside world are added.

As soon as a child starts walking, he can get to dangerous objects, so after a year, children can swallow adult pills, household chemicals, or climb onto a closet. Burns are also common.

An older child is more likely to collide with other children on the slide or fall off the carousel on the playground. Also, many people have scooters and balance bikes, so the child should immediately be taught to ride in a helmet and protection on his knees.

At school age, injuries decrease, children develop a sense of danger and the necessary knowledge about the world that allows the child to avoid injury. But at the same time, the child often gets faster transport: hoverboards, mopeds and rollers, which without protection can severely injure a teenager.

How to help with an injury

For superficial abrasions:

• Rinse the wound with tap water or a bottle if it happened outside.
Treat
• with an antiseptic, hydrogen peroxide, chlorhexidine or miramistin are suitable for this.
• if the child is walking on the street, then after treatment it is better to seal the wound with a band-aid to reduce the risk of suppuration due to dirt. At home, it is better not to glue it, so the abrasion will dry out in the air and heal faster.
• if earth could get into the wound, and the child is not vaccinated against tetanus, then you need to urgently contact a pediatrician or an emergency room for emergency prevention.
• wound requires observation for several days. If redness has appeared around, the abrasion site has increased or pus is oozing, then most likely an infection has got inside and you need to contact a surgeon.

“For any bruise, a cold compress will not be superfluous,” explains Ilya Zelenkin, “it will partially relieve soreness and swelling. Consult a doctor if the child does not have pain or swelling after a bruise for a long time. If blood is bleeding from the wound, apply a bandage/gauze pressure bandage to the bleeding area and apply additional pressure to it for 1-2 minutes. If bleeding still continues, add more gauze and continue to apply pressure to the wound for about 5 minutes. You can also make a bandage out of an elastic bandage, putting it on gauze, and additionally compress the wound.”

With a severe bruise and a suspected fracture, you need to seek medical help to take an x-ray and make sure that the bones are intact.

“If the injury site is very painful, swollen or deformed, wrap it in a towel or soft cloth and make a splint out of cardboard, plywood or other suitable material to fix the limb,” advises Ilya Zelenkin. - The arm or leg should not move in the joints. Do not try to straighten the injured limb. Ice wrapped in polyethylene and a thin cloth can be applied to the injury site, or a cold compress can be made, but not more than 20 minutes. If the skin near the injury site is damaged or you see a protruding bone, then cover the wound with a clean bandage, make a splint and seek help immediately. If there are no visible injuries, but the limb in the area of ​​injury is cold or discolored (blue or pale), call an ambulance without delay.”

In what cases you need to go to the hospital, says GMS Сlinic pediatrician Daria Zakharova:

• with a head injury (the child fell and hit his head / the child was hit on the head or he himself hit some object) - it is important to exclude the presence of a traumatic brain injury. It is urgent to consult a doctor if there was a short-term loss of consciousness after the injury, and also if headache, nausea/vomiting, weakness and drowsiness appeared within three days after the injury. In this case, the child should be examined by a neurologist and an ophthalmologist, and an x-ray or computed tomography of the head may also be needed. If the child feels well after the injury, he still needs to be provided with a calm regime, without physical activity in the first 3 days after the injury.
• in case of injuries of the limbs, it is worth contacting a traumatologist if the child complains of severe pain, swelling has appeared at the site of the injury, the color of the skin has changed, there is a sharp pain when moving.
• with bruises of the anterior abdominal wall (this may be, for example, a kick of a soccer ball), it is important to pay attention to whether the child has weakness, pallor of the skin. In this case, contacting a doctor and performing an ultrasound scan of the abdominal organs should be immediate.

In young children, it is more difficult to notice the problem, so any change in the child's behavior after a possible injury will be the reason for contacting a doctor. Poor or vice versa, too much sleep, strong crying for no apparent reason, refusal to eat or vomiting.

“Call the emergency service right away,” recommends Ilya Zelenkin, “if the child has lost consciousness, has convulsions, can hardly raise an arm / leg or smile, blood or a watery liquid comes out of the ears or nose, talks and behaves inappropriately.”

To make the child less injured

First of all, you need to protect it. At a young age, make sure that he does not fall from a height, put stoppers on doors and protection on boxes where dangerous items are stored. Leaving a child alone is only possible after making sure that the space around him is safe.

But at the same time, it is worth teaching the baby the consequences of actions. Gently show that the stove is hot, and the drawer can pinch your fingers. This is how cause-and-effect relationships and concepts of danger are formed. After several repetitions, the child will become more careful.

Therefore, it is harmful to constantly keep a child in a playpen or other closed space, as well as to put pith helmets on a healthy baby to protect the back of the head from falls. In the future, this can lead to careless behavior and an increase in injuries.

As Ilya Zelenkin says, adults are obliged to prevent possible risks and protect children from them. But at the same time, it is important not to develop feelings of timidity and fear in the child, but, on the contrary, to inspire him that danger can be avoided if you behave correctly. The older the child, the more important it is to explain to him the safety rules:

• stay next to the child on the territory of the playground and in transport.
• while waiting for transport, stand in a well-lit place next to people and always hold the child's hand.
• at bus stops, do not turn your back on the road and do not stand in the front row of an impatient crowd with a child.
• when entering the transport, children under the age of three must be picked up and exited in the same way.
• A personal example of the behavior of parents on the street, in transport is very important.
• , it is imperative to use protective equipment: a helmet, elbow pads, knee pads when rollerblading, cycling, on a skating rink.

It is better to direct irrepressible children's energy in the right direction, go in for sports or dance. In this way, the child will learn to control his body and to group himself when he falls - this reduces the risks, despite possible sports injuries.

It is also important to observe the sleep and rest regime, sleepy children coordinate their actions worse and are more likely to get injured.

“Active walks and sports help children whose energy is overflowing,” says Daria Zakharova. It is difficult for them to sit in one place, and when it is a closed space of an apartment with many objects that a child can be injured about, the risk increases. Sports and active walks help the child get rid of this "excess" of energy."

• The risk of injury to the infant is reduced by constant supervision and provision of a safe space.
• The kid needs to be taught to control his body, so excessive protection and limitation of movements only get in the way.
• When a baby gets his first transport, he definitely needs a helmet and protection on his knees.
• An older child needs sports, sleep and rest, and active walks to reduce the risk of injury.

Pediatrician

SpringPharmacy

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