Overview.
Excessive cerebrospinal fluid secretion or arachnoid resorption disorder, resulting in excessive accumulation of fluid in the brain Infants may have increased head circumference, fontanel bulging, children may have headache, vomiting, severe irritability, lethargy, etc. Causes of the disease include dysplasia, hemorrhage, infection, etc. Surgery is the main treatment, and medication plays a supplementary role.
Definition
Traffic hydrocephalus refers to hydrocephalus that occurs when the circulatory pathways of cerebrospinal fluid (a fluid that surrounds the brain and nerves and has protective, supportive, and nutritive functions for the brain and nerves) are open, but is caused by overproduction of cerebrospinal fluid or impaired arachnoidal resorption.
The ventricles and subarachnoid space are generally clear, or cerebrospinal fluid flows only to the subarachnoid space of the spinal cord and does not reach the subarachnoid space on the surface of the brain or the cerebral arachnoid granules.
Epidemiology.
There are no results of a national epidemiologic survey of traffic hydrocephalus.
Etiology
Causes
Congenital disorders
Congenital hypoplasia of the cerebral pools, bilateral transverse or sigmoid sinus occlusion, stenosis, and arachnoid granular dysplasia.
Infection and hemorrhage
Neonatal or infantile meningitis and subarachnoid hemorrhage.
Pathogenesis
Cerebrospinal fluid absorption dysfunction
Cerebrospinal fluid absorption is impaired due to occlusion of the arachnoid granules and their superficial vascular spaces caused by subarachnoid hemorrhage and meningitis before or after birth. Traffic hydrocephalus is mostly such a case.
Congenital cerebral pool hypoplasia, venous sinus occlusion or stenosis, arachnoid granule dysplasia, etc. affect the absorption of cerebrospinal fluid.
Hypersecretion of cerebrospinal fluid
Very few patients present with choroid plexus hyperplasia, or suffer from choroid plexus papilloma with excessive cerebrospinal fluid secretion.
Symptoms
Main Symptoms
Abnormal head shape in infants
Head circumference increases more than 2 centimeters per month during infancy.
Enlargement of fontanel, bulging fontanel, and splitting of cranial sutures.
The scalp thins and the veins of the scalp are markedly enraged (venous filling is evident).
Pot-breaking sounds may be present when the head is tapped.
There may be a special expression of downward glancing eyes with sclera (the white part of the eyeballs) exposed, i.e., the sunset eye sign.
Symptoms of elevated intracranial pressure
In children and adults with closed fontanels and cranial sutures, symptoms such as headache, vomiting, and loss of vision due to edema of the optic nerve papillae are more pronounced.
Infants may present with feeding difficulties, crying, and irritability.
Neuropsychiatric abnormalities
Continued elevation of intracranial pressure may cause the patient to become less conscious and fall into lethargy until coma.
Children may show growth stagnation, decreased intelligence, and decreased sense of smell and vision.
Adults with normal pressure hydrocephalus may show signs of brain dysfunction such as mental retardation, walking disorders and urinary incontinence (uncontrolled outflow of urine).
Medical attention
Conditions that require medical attention
Infants with feeding difficulties, irritability, and rapid head growth (head circumference increasing at a rate of more than 2 centimeters per month) require prompt medical attention.
If the child is depressed, lethargic, crying, convulsions, etc., immediate medical attention is required.
Children or adults need immediate medical attention if they have headaches, vomiting, or vision loss.
Adults need prompt medical attention if they have mental retardation, walking disorders, and urinary incontinence.
Suggested departments
Pediatrics, neurosurgery in general hospitals or pediatric surgery, pediatric neurosurgery or neurology in specialty hospitals.
Preparation for consultation
If there are no special circumstances, you need to register through the hospital’s official website, official app and other official channels, prepare your social security card (medical insurance card) and other medical documents, and bring your previous medical documents with you, as well as filing procedures for those who seek medical treatment in a different location.
What questions the doctor may ask
When did you notice that your child was not in good spirits?
Did the baby vomit?
Was the baby delivered by normal labor or cesarean section?
Did the child have a difficult labor, birth injuries, oxygen deprivation, or intracranial hemorrhage?
Did the child have any head trauma?
Did the child have any infections?
How was the mother’s health when she was pregnant?
Did the mother take any medication during pregnancy?
How many children are there in the family? （Has the older child (if any) had a similar condition?
Has anyone in the family had this condition?
(Child or adult visits) Any headaches, nausea, vision loss? Do you feel irritable?
(For adults) How is memory? Is there any gait abnormality? Is urination normal, is there incontinence?
Have you been to the hospital?
Have you had a checkup? What is the result of the test?
Have you been treated in the hospital? What is the result?
Questions you can ask your doctor
Is it serious?
What kind of tests do I need to do?
Do I need to be hospitalized?
Will there be any after-effects?
Does this disease have any effect on intelligence?
Do I need surgery? If surgery is needed, what kind of surgery is suitable?
Diagnosis
Disease Diagnosis
Medical History
Mother’s poor health during pregnancy and possible history of taking teratogenic drugs.
Birth may have been difficult, with the use of forceps or fetal head attractors.
There may be a history of head trauma, infection, or intracranial hemorrhage.
Symptoms.
The head is large and growing rapidly in infancy, with enlarged and bulging fontanels and cracked cranial sutures. Pot-breaking sounds may be present when the head is tapped. There may be a special expression of downward glancing eyes with the sclera (the white part of the eyeball) exposed, known as the sunset eye sign.
Headache, nausea, vomiting, loss of vision, and irritability are more pronounced in children and adults because the fontanel and cranial suture have closed. Infants may present with feeding difficulties, crying, and irritability.
Continued elevation of intracranial pressure may cause the child to become less conscious and fall into a drowsy state until coma.
Children may have growth retardation, decreased intelligence, and decreased sense of smell and vision.
Adults may have mental retardation, walking problems, and urinary incontinence.
Head circumference measurement and light transmission test
Measurement of head circumference: The length of the head from the center of the eyebrows to the middle of the back of the head, around the head, is the maximum head circumference. Normal newborn head circumference is 33-35 centimeters, increasing by 1.2-1.3 centimeters per month during the first 6 months of life. Infant hydrocephalus head circumference increased significantly, sometimes head circumference increased up to 2 to 3 times the normal increase value.
Transillumination test: hydrocephalus with brain parenchyma thickness less than 1 cm shows whole head transillumination.
Imaging examination
Head X-ray: in infants, there is an enlargement of the skull, thinning of the skull bones, separation of the cranial sutures, enlargement of the fontanel, and disproportion of the craniofacial bones. In children, the bone sutures have been closed and the enlargement of the skull is not obvious. In severe cases, the skull sutures may be separated again.
Cranial CT: Infants have generalized enlargement of the ventricular system with normal or absent sulci. In the early stage, only the lateral ventricles are enlarged, with the aggravation of hydrocephalus, the third ventricle may be enlarged, and the fourth ventricle enlarges later and to the smallest extent, which is conducive to the diagnosis once it appears. The changes of the cerebral sulcus are shallower and flatter, with more typical features; it may also manifest as enlargement of the cerebral sulcus and cerebral pool.
Magnetic resonance imaging (MRI): changes in the infant’s ventricles, sulci, and cerebral pools are similar to those seen on CT, whereas interstitial edema in the paraventricular white matter tends to be more pronounced than on CT.
Differential diagnosis
Megalencephaly
Similarities: Children with macrocephaly have a larger head than normal infants and children, with rapid growth in head circumference.
Differences: Children with megalencephaly have a normal lumbar puncture cerebrospinal fluid (CSF) pressure test and no signs of increased intracranial pressure on head radiographs. There is no ventricular enlargement on cranial CT and MRI.
Rickets
Similarities: The head of the child is enlarged in circumference compared to normal infants.
Differences: Children with rickets have an irregular or square skull, generalized skeletal abnormalities such as “chicken breast” deformity, and a hypotonic fontanel. Laboratory tests show decreased calcium or increased phosphorus.
Subdural hematoma
Similarities: Progressive increase in head circumference, bulging fontanel, vomiting, irritability or lethargy.
Differences: Children with subdural hematoma have less head enlargement than children with hydrocephalus, and the chronic subdural hematoma area is often blunt and solid on percussion. Imaging can be used to identify the subdural hematoma, and fontanel puncture can be used to identify the subdural hematoma, but it is less commonly used in clinical practice.
Obstructive hydrocephalus
Similarity: children with progressive increase in head circumference, sunset eye sign, bulging fontanel, vomiting, irritability or lethargy; children or adults may have headache, nausea, vomiting, vision loss.
Differences: Obstructive hydrocephalus usually has obstruction or occupancy of the ventricular system, with enlarged proximal ventricles of the obstruction and normal or reduced distal ventricles.CT and magnetic resonance imaging (MRI) can be used to differentiate.
Benign external hydrocephalus
Similarity: the head of the child is enlarged in circumference compared to normal infants.
Difference: Children with benign external hydrocephalus have normal or mildly full fontanels. The disease is self-limiting and usually disappears at one and a half years of age without signs of increased intracranial pressure, so no special treatment is needed.
Treatment
Surgery is the main treatment for traffic cerebral edema.
Drugs generally play a supplementary role, mainly used to temporarily reduce the secretion of cerebrospinal fluid or increase the excretion of water from the body.
Surgery
Indications
Patients with clinical manifestations of elevated intracranial pressure and cerebral impairment, such as headache, vomiting, developmental delay, and optic disc edema.
CT or magnetic resonance imaging (MRI) shows ventricular dilatation.
Contraindications to ventriculo-peritoneal shunting
Acute stage of intracranial hemorrhage.
Intracranial infection with cerebrospinal fluid infection or infected lesions.
Infection in the skin of the scalp, neck, chest, and abdomen.
Infection in the abdominal cavity.
Surgical approach
A cerebrospinal fluid shunt can be effective in reducing intracranial pressure, especially in acute rapidly progressive hydrocephalus. A cerebrospinal fluid shunt is used to shunt cerebrospinal fluid from the ventricles of the brain to the cerebral pools, or from the ventricles or lumbar spinal canal cavities to other body cavities.
The most widely used treatment for traffic hydrocephalus is still the ventriculo-peritoneal shunt. Lumbar pool-peritoneal shunt can also be used, and there have been some clinical reports in recent years.
With the development of medical technology, neuroendoscopy has become one of the important treatments for hydrocephalus. Infants (especially those less than 1 year old) and patients with severe ventricular enlargement have a low success rate, and caution should be exercised in choosing this type of surgery.
Postoperative complications
Shunt surgery has the highest rate of complications, mainly shunt infection, shunt obstruction, excessive drainage, and shunt displacement.
Common postoperative infections include intracranial infection, incision infection, intra-abdominal infection, and shunt subcutaneous channel infection.
With shunt obstruction, the patient’s clinical manifestations may show no significant improvement, and the shunt needs to be replaced.
With excessive drainage, clinical signs of intracranial hypotension are present, and in severe cases, this may lead to subdural effusion/hematochezia, intraventricular hemorrhage, or epidural hematoma.
Displacement of the shunt at the ventral end of the abdomen is also frequent, most often to the subcutaneous chest and abdominal wall, but also to the subcutaneous neck or subcapsular tendon membrane of the scalp.
Drug treatment
Symptomatic treatment
Commonly used diuretic drugs such as hydrochlorothiazide, acetazolamide, aminopterin, chlortalidone, etc., can be used for a short period of time in children with slowly progressive hydrocephalus whose condition is unstable and inoperable, and acetazolamide is preferred. Hormone therapy can be tried in children with arachnoid adhesions.
Anti-infection treatment
According to the etiological examination and drug sensitivity test, choose the appropriate anti-infective drug treatment.
Prognosis
Cure
The perinatal period (28 weeks of gestation to 1 week after birth is the perinatal period; fetuses and newborns during the perinatal period are called perinatal infants) has a high morbidity and mortality rate and a poor prognosis.
Neurologic deficits in patients with traffic hydrocephalus are proportional to the severity of the hydrocephalus.
Hazards
Hydrocephalus that develops too quickly or lasts too long can cause brain damage or even death.
Daily life
Daily life
Ensure energy and nutrient supply
Breastfed children should continue to breastfeed and increase the frequency of breastfeeding.
Artificially fed children should have their diet adjusted to ensure adequate nutrition and calories.
Children and adults should eat a balanced diet that is light and avoids oily and irritating foods.
Avoiding infections
Avoid contact with people with infectious and acute infectious diseases.
Pay attention to keep warm, keep the room temperature at 18~21℃ and the humidity at 55%.
Ventilate regularly and arrange outdoor activities as appropriate.
Observe closely and seek medical advice if there is any abnormality.
Caregivers pay attention to monitor the patient’s mental state, body temperature, pulse, respiration, blood pressure and so on. If the patient has depression, drowsiness, irritability, etc., consult a doctor promptly.
Regular review
For children with hydrocephalus that has been controlled by surgical treatment or static hydrocephalus, it is important to review the condition regularly so that the shunt is not clear or the hydrocephalus worsens before the brain tissue is seriously damaged, and to ask the doctor to treat the condition in a timely manner.
Prevention
Mothers should take good care of themselves during pregnancy to prevent infection.
Prenatal ultrasound examination has high sensitivity and specificity for hydrocephalus malformations, and mothers should have regular prenatal checkups.
Selective termination of pregnancy can reduce the incidence of the disease if there is a genetic disease or high-risk condition in the family and the prenatal diagnosis suggests that the fetus has hydrocephalus.