It is no secret that during the formation of the embryo in the maternal womb, various pathologies may occur, including an Arnold Chiari type 1 anomaly. What is this disease? Doctors call this phenomenon a deviation that develops in the fetus due to a mismatch in the size of the part of the cranium and the brain located in it. We are talking about the area where the cerebellum is located. As a result, a shift of the tonsils to the upper spine occurs, with their further infringement.
Causes of occurrence
Can a person normally exist if he has an Arnold Chiari type 1 anomaly? Life expectancy, doctors say, depends on several factors: the severity and form of the disease. Before making a final diagnosis, they determine the causes that led to the development of deviations:
- Congenital Even in the womb of the fetus, the bones of the skull are deformed: the fossa in which the cerebellum is located is too small for the growing and developing gray matter. Among other congenital causes, a strong increase in the occipital foramen during the formation of the skeleton is called.
- Acquired. First of all, these are head injuries during childbirth. Also, spinal injuries are attributed to this category: the negative effect of cerebrospinal fluid - a special fluid, the occurrence of dropsy and other pathologies of the spinal cord.
To be extremely frank, scientists cannot yet name the exact reasons. The only thing they are sure of: the syndrome is not associated with chromosomal developmental abnormalities.
Risk factors
Everyone knows that during the first trimester of pregnancy, the fetus undergoes the laying of virtually all organs. Therefore, the correct lifestyle of the mother is very important for the normal development of the child. If a woman does not comply with elementary rules, this will cause many complications in the future. As for Chiari syndrome, the following factors increase the risk of its occurrence:
- Mom's bad habits: smoking, alcohol and drug abuse.
- The use of potent drugs, self-medication.
- Past viral infections, especially rubella.
With especially mild forms of the disease, the born baby does not feel special symptoms until the end of his life, especially if he has an Arnold Chiari type 1 anomaly. How many children live who have a third or even fourth stage of the disease? Doctors give disappointing predictions. Unfortunately, such babies in most cases are doomed to death. Even if the baby has the first or second type of ailment, he needs treatment: the effectiveness of the surgical intervention is 85%.
Types of Syndrome
Regardless of their severity, they are all associated with a very low location of the cerebellum. The deeper and more the tonsils descend into the spinal canal, the more dangerous and unpredictable the disease: other deviations are diagnosed with it. Based on this, 4 forms of pathology are distinguished:
- Arnold Chiari type 1 anomaly - what kind of disease is it? Usually it does not cause any other problems in the development of the baby. Structural changes in the brain do not occur, tonsils are located in the cervical region.
- The syndrome of the second type. The cerebellum is partially displaced into the occipital foramen, with various anomalies of the spine, brain and spinal cord observed in the embryo.
- The disease is of the third type. The structures of the hindbrain are completely displaced into the occipital foramen. A hernia forms in this area.
- Arnold Chiari's disease of the fourth type. Cerebellar hypoplasia is characteristic of it - its underdevelopment. However, it does not move and is often combined with hydrocephalus and congenital cysts of the posterior cranial fossa.
Doctors say that type 2, type 3 and type 4 abnormalities are often diagnosed in combination with other serious abnormalities in the baby’s development: cortical heterotopy, hypoplasia of the subcortical structures, corpus callosum anomalies , and so on.
Main symptoms
As already mentioned, if the stage of the disease is very mild, and the brain is slightly affected, then the disease can occur virtually all of life without disturbing a person. But this is a rather rare case. Usually permanent and regular headaches are the main symptom of such an ailment as Arnold Chiari type 1 anomaly. Symptoms for this form other than migraine are: pain in the cervical region, tinnitus, nausea and vomiting, weakness of the hands, loss of sensation of the extremities, flies and double vision, insecure gait, blurred speech and shortness of breath.
As for the syndrome of the second type, which is more dangerous and complex, its characteristic signs appear immediately after birth or at a very young age (often in preschool). Usually this is wheezing noisy breathing in infants, a weak cry, respiratory failure and swallowing. Such newborns are urgently resuscitated and trying to save by surgical intervention. In these cases, the main thing is not to miss the moment when you can still save the life of the child.
Other symptoms
They are observed with a more severe degree of the syndrome. These signs of an Arnold Chiari anomaly are often the primary sources of a heart attack of the brain or spinal cord: type 1 is not as scary as its other subspecies. They are characterized not only by double vision, but also complete blindness, loss of consciousness, impaired coordination, tremor of the extremities, problems with urination, muscle weakness, loss of sensation in a large part of the body (sometimes the whole half of the body).
The development of various health effects can trigger an Arnold Chiari type 1 anomaly. Life expectancy and its normal course depend on a quick and accurate diagnosis. The only method to detect pathology is an MRI (magnetic resonance imaging) of the brain. It requires the patient to be completely still, therefore active babies are euthanized with special medicines. Using this apparatus, the spine, spinal cord, cervical and thoracic parts of the skeleton are also examined. MRI can detect not only the syndrome, but also all the pathologies that accompany the disease.
Complications
The development of various pathologies provokes an anomaly of Arnold Chiari type 1. The consequences can be very different: starting from chronic arachnoiditis and ending with a parenchymal lesion of the axon. These complications are especially pronounced against the background of circulatory disorders in nerve tissues. Neuropsychiatric disorders in this case appear late and pass in the form of developmental delay, paraplegia. If the anomaly develops very progressively, it leads to hydrocephalus - excessive fluid accumulation in the ventricular system of the brain.
In addition,
Arnold Chiari syndrome can cause such severe compression of the spinal cord that a person is completely paralyzed. Under its negative influence, cysts and cavities in the spine often begin to form: they receive fluid, which disrupts the spinal cord. Complications with breathing up to its complete stop are also possible. Sometimes doctors register congestive pneumonia in patients - it becomes a consequence of the fact that a person loses the ability to move freely.
Conservative treatment
The syndrome is treated in two ways. Doctors choose a conservative or surgical route, depending on the symptoms and signs that appear in the patient, the severity of the person's condition, complications and consequences of the disease. Sometimes it is exclusively with the help of medications that you can fight a disease such as Arnold Chiari type 1 anomaly. What kind of therapy is this? Firstly, the physician constantly conducts preventive examinations of the patient, sends him to consultations with various specialists who, after appropriate consultation, take the necessary measures.
If the patient complains of pain in the occipital region of the head or in the cervical spine, he is prescribed painkillers and anti-inflammatory drugs, as well as drugs that help relax the muscles. A special physiotherapy exercise is also very useful - a set of exercises aimed at eliminating tremors in the limbs and normalizing coordination. For these purposes, active occupational therapy is also prescribed. Also, lessons with a speech therapist who will be able to eliminate speech problems will be useful. All of these methods can delay or completely prevent a more aggressive treatment - surgery.
Surgical intervention
It happens that the above measures do not help - Arnold Chiari type 1 anomaly continues to actively develop and cause a lot of trouble. The treatment then changes dramatically: the doctor begins to prepare the person for surgery. Neurosurgery is performed for three purposes. Firstly, to eliminate such serious signs of the disease as loss of consciousness, visual disturbances, muscle weakness. Secondly, the intervention helps to remove the primary source of symptoms - to eliminate the compression of the brain. Thirdly, through the operation, the movement of the cerebrospinal fluid is normalized.
Intervention stops the process of changes in the structure of the spine and gray matter: as a result, the symptoms weaken, the patient begins to feel much better. Usually, surgeons remove a fragment of a bone from the back of the cranial fossa: the space for the brain increases, the gray matter stops moving and squeezing. Intracranial pressure also drops . In addition, doctors are actively using methods such as shunting and closure of the spinal canal.
Prevention
So that the child does not have the above problems, the expectant mother should take her position seriously. Firstly, she must familiarize herself with all the possible pathologies that may occur in the fetus: neural tube development disorders, Down syndrome, Arnold Chiari type 1 anomaly. What are these diseases, why they arise and what measures can be taken to reduce the risk of developing these and other deviations, every expectant mother should know. A competent and knowledgeable pregnant woman will maximally avoid all those factors that negatively affect the formation of the embryo.
Secondly, a woman must fully eat, eat a variety of healthy dishes: fish, meat, fruits, vegetables, cereals and dairy products. Strongly not allowed alcohol. In addition, you need to quit smoking, not to mention drugs and potent drugs. The expectant mother takes any medicine, even homeopathic, only after the appointment of a doctor. Thirdly, a woman should also walk a lot, breathe fresh air, move a lot. It is worth forgetting about nerves, feelings and negative emotions, enjoying your position and getting maximum joy from life.