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Chiari Malformation (CMs)


Chiari malformations (CMs) is congenital malformation of brain. It is a structural defects in the cerebellum, the part of the brain that controls balance. It occurs when a part of your skull is abnormally tiny or twisted, pressing on your brain and forcing it downward.

It is more common in women than men. The age of diagnosis is near about 24. It also occurs in many children. Chiari malformation is generally present at birth but it can develop in some children after the child birth.

Types of Chiari Malformation

There are four different types of Chiari malformations these are e types I, II, III and IV . The type III and IV is very rare.

  • Type I - This type of malformation occurs during fetal development and is characterized by downward displacement of the cerebellar tonsils near the foramen magnum into the cervical spinal canal. It includes bony union of the first level of the spine , compression of the brainstem and shows the symptoms likes severe head and neck pain, spasticity, dizziness, balance problems and vision problem.
  • Type II - This type of malformation is characterized by downward displacement of the medulla, fourth ventricle, and cerebellum into the cervical spinal canal and elongation of the pons and fourth ventricle. This type occurs almost exclusively in patients with myelomeningocele. It shows the symptoms like loss of arm strength, alteration in the pattern of breathing and trouble swallowing.
  • Type III - In this type, the back of the brain protrudes out of an opening in the back of the skull area.
  • Type IV - In this type, the back of the brain fails to develop normally.

Causes of Chiari Malformation

The exact cause of Chiari malformation is unknown but it is causes during fetal development and also cause the abnormal brain formation

  • Chiari malformation can be caused by structural defects in the brain and spinal cord which occurs during fetal development.
  • Chiari malformation caused due to genetic mutations or lack of proper vitamins or nutrients in the maternal diet which is also called primary or congenital CM.
  • Chiari malformation may caused by exposure to harmful substances during fetal development.
  • Prescription or illegal drug and alcohol consumption causes the Chiari malformation.

Symptoms of Chiari Malformation

The symptoms of the Chiari malformation are as follows

  • Headaches
  • Neck pain
  • Vomiting
  • Dizziness
  • Vision problems
  • Breathing problems
  • Balance problems
  • Muscle weakness
  • Difficulty swallowing
  • Frequent gagging and choking
  • Deafness
  • Paralysis of the arms and legs

Diagnosis of Chiari Malformation

The diagnosis of Chiari Malformation take place with help of some tests like MRI and Ct scan test.

  • MRI Scan - It includes which images of the brain using a magnetic field and radio waves instead of X-rays.
  • CT scan - In this, imaging procedure and a combination of x ray and computer technology produces cross-sectional images of the body.

Treatment of Chiari Malformation

The treatment of Chiari Malformation is depends on the type of malformation and symptoms.

  • Chiari I malformations  are asymptomatic. If the malformation is definite as symptomatic then a  syrinx, treatment is generally optional.
  • Chiari II malformations are treated if the patient is symptomatic. In this there are no complications from hydrocephalus. In many infants having a Chiari II malformation then the symptom progressed  rapidly  and  requires an emergency treatment.
  • Surgery - Surgical treatment of these malformations depends on the type of malformation. Chiari I malformations is treated surgically with only local decompression of the overlying bones, decompression of the bones and release of the dura.
  • Chiari II decompression is treated similarly with surgical therapy but is usually restricted to decompressing the tissues in the spinal canal and leaving the back of the skull alone.
  • Infants and children with myelomeningocele require surgery to reposition the spinal cord and close the opening in the back.

Prevention of Chiari Malformation

Chiari Malformation there is no method of prevention known.

What is Prognosis of Chiari Malformation?

The prognosis for Chiari malformation different depending on which type of malformation is present. Many people with Type I CMs are asymptomatic and don’t know about  the condition. Many patients with the more severe types of CMs  require  surgery  to  a reduce the  symptoms and expanded periods of relative constancy. Children with Type I malformation has corrective surgery which is highly effective to reduce symptoms related to compression of the brain, such as vision problems; headaches; difficulties with balance, coordination, and swallowing.