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Chiari malformation- A prominent form of brain malformation

  • Posted on- Sep 11, 2015
  • 383 Views

Chiari malformation is the malformation of a part of the brain. It is a medical condition in which brain tissues (cerebral tonsils) project out into the spinal canal (foramen magnum). Most of the time, this condition occurs due to some irregularities and conditions of the skull, like an abnormally smaller-sized or a misshapen skull, which press down these tissues into the spinal canal. Also called Arnold-Chiari malformation, this disorder is very uncommon and not often diagnosed without any special tests.

This condition leads to accumulation of cerebrospinal fluid into brain cavities, medically termed as 'hydrocephalus'. There are also chances of blockage of this fluid as a result of this displacement of such important parts of the brain. It is divided into two main types - type I (in adults) and type II (in children). It is not always that a person encounters the symptoms, as they could also be concealed until any specific tests are performed.

Types of Chiari malformation

The symptoms vary as per the severity and the type of the malformation. Signs of type I appear in late childhood or early adulthood. While paediatric (type II) symptoms can be detected by simple ultrasounds performed during pregnancy, let's see them divided by each type. Along with the ones mentioned below, there are also some signs in toddlers and infants that can further be distributed into type III (a condition in which cerebellum extends to back of the skull) and type IV (a condition in which the brain is not developed properly, in the first place). These two types are not so common, and they could also be detected by a simple intrauterine ultrasound test, performed on a pregnant woman.

Type I Chiari malformation
This includes the signs that are usually observed in adults, and also children that are in their late childhood.

  • Severe neck pain that runs in downward direction, along with chest pain
  • Balancing problems or unsteadiness while walking
  • Difficulty in fine motor skills or lack of hand-eye coordination
  • Stuttered speech, dizziness, and blurred or doubled vision
  • Tingling in hands and feet along with numbness
  • Difficulty in swallowing that sometimes results into vomiting and chocking
  • Tinnitus or ringing in one or both the ears
  • Loss and lack of bladder control
  • Abnormal bending of the spine and abnormal breathing

Type II Chiari malformation
This is one of the types of malformation, which is congenital and develops along with the body system. This disorder is deeply related to 'myelomeningocele' or 'spina bifida cystica', which is a neural tube defect, characterized by underdevelopment of the spinal cord. In this condition, the affected person suffers from headaches, which is most often confused with migraines. Tremendous pressure in the back of the head is also one of the signs. If undetected before the childbirth, they could also be somewhat similar to that of multiple sclerosis.

Treatment of Chiari malformation

As most of the cases are asymptomatic, meaning, that there are hardly any noticeable symptoms, it gets very difficult to determine the disorder in the first place. The only solution is to go and visit a doctor if you find the child suffering from any of the mentioned problems that are frequent and unmanageable after taking all the curative measures. As we can see, each of the symptoms is similar to signs of some other disorders, which makes it all the more confusing.

There are a few tests, like the X-ray, MRI scan, and CT scan of the head and neck that can be used for determining the severity of the disorder and need for surgery. In case of excessive spinal fluid, there is always the need of a surgery that facilitates safe removal of the fluid. As far as research is concerned, surgery is the only available option to treat this malformation. There are a few complications involved when it comes to surgery, and hence, it is a test of skill as far as the surgeon is concerned.