What is Chiari Malformation Type II, How Does it Affect Your Body and What is its Treatment?

What is Chiari Malformation Type II ?

Chiari malformation is an issue in the structure of the cerebellum of the brain. There exist four types of Chiari malformations namely type l, type II, type III and type IV respectively. These types can be characterized differently according to certain signs and symptoms and how they affect the body. Chiari malformations are named for Hans Chiari, an Austrian pathologist, who initially found out type I-III in 1891.

The Chiari malformation type ll is also known as Arnold-Chiari malformation in honor of the German pathologist Julius Arnold. Diagnosing a patient with Chiari malformation includes a lot of processes like examining the patient’s neurological condition, their medical history in general and also their medical imaging with the help of an MRI or CT scan. Other methods of diagnosis include myelogram, sleep study and swallowing study. Chiari malformation II first diagnosed in the year 1883 by Cleland but the first surgery was performed in the year 1932 although all the patients who had undergone the surgery eventually died because of postoperative complications. The year 1985 was a revolutionary year for Chiari malformation because of Aboulezz who used MRI for extension of scope for the defect. Today less than one in thousand people are said to have Chiari but it still poses as a great threat to people who are detected.

What is Chiari Malformation Type II ?

How Does Chiari Malformation Type II Affect Your Body?

There are various symptoms that are found in patients with Chiari malformation II that include the difference in breathing patterns, weak arm muscles, and involuntary eye movements. Usually, these can be identified with symptoms like headache close to the skull base, neck pain, and shoulder pain. The pain that occurs is not subtle but sharp and pulsating. Chiari malformation II is caused by the displacement of the medulla, fourth ventricle, and cerebellum all parts of the brain into the cervical spinal canal. Chiari malformation II is mostly found in patients who suffer from myelomeningocele, a defect that occurs when the backbone and spinal canal do not close before the birth of a baby resulting in paralysis in the area below the spinal opening. It can cause complications but the type of complications depends on the severity. Chiari malformation II can lead to abnormal bony compressions of the spinal cord, increased damage to the vital structures of the body and reduced flexibility[1]. It also causes a fluid buildup in the spinal cord which can be a huge hindrance to the functioning of the body in the long run. Although it isn’t deadly, it can cause an obstruction to the body functions.

Who Does Chiari Malformation Type ll Affect and Why?

This can be quite hard to find out considering the symptoms that are common to several other disorders and sometimes the patient may not have any symptom at all. Chiari malformation II is usually found in babies or a fetus and they should be treated just then to avoid future complications. It usually affects children because of genetics and hence there is nothing that can be done to prevent it except surgery.

The treatment of Chiari malformation II includes putting a flexible tube in the spinal cord which drains fluid from the syrinx of the body. Other methods also include placing a flexible tube in the brain which drains the extra fluid that is present in the brain cavity, letting the spinal fluid flow by easing the pressure on the brain and because Chiari malformation II is accompanied by myelomeningocele we can close a myelomeningocele after the birth of the child. Chiari malformation II affects less than one in a thousand people that are around 0.02% of children at birth and according to statistics, it affects girls twice as much as it affects boys.[2]

Surgery and Treatment for Chiari Malformation Type ll

Surgery for Chiari malformation II is basically done to create more space for the brain and spinal cord. Mostly a decompression surgery is performed on the child where the bone that is compressing the cerebellar tonsils. This surgery reduces the pressure on the brain which in turn restores the cerebrospinal fluid flow in the brain. The patient is given general anesthesia before the surgery is performed. If space is okay, the surgery is over and the incision is closed. Even then if during the surgery, the surgeon feels like the space created isn’t enough. Then the surgeon makes a cut in the covering of the brain, called the Dura mater and then the surgeon may need to sew a patch over the opening to keep cerebrospinal fluid from leaking. The surgical technique may vary. If your child has a syrinx or hydrocephalus, he or she may need a tube (shunt) to drain the excess fluid.

Decompression surgery can take two to three hours. Recovery typically includes one night in the intensive care unit and three to four days in the hospital. After the surgery it may take about 10-14 days for a child to recover. Medicines and pain killers prescribed by the doctor should be given to the child for subsiding the pain after the surgery is performed.

Role of Nurses for Patients With Chiari Malformation Type ll

The role of a nurse is very critical during the process of the treatment of Chiari malformation II. The nurse takes care of the initial part of the treatment alone. A nurse makes sure the initial part goes smoothly and is responsible for taking care of the child. The nurse also plays a very crucial part during the surgery and helps the doctor. After the surgery is done the nurse has to make sure the patient is in optimal condition and doesn’t experience any pain and if there is some pain the nurse has to help the patient during the process. Hence the treatment process cannot be possible without nurses.

Types of Pediatric Neuro-Ophthalmology

Brain and eyes get affected by various types of eye conditions. Some of the condition that is related to the ophthalmology is listed below:

  • Diplopia, which is also known as having double vision.
  • Crossed eye syndrome or impaired vision is technically known as esotropia.
  • Droopy eyelid and mouth condition is technically known as Myasthenia gravis.
  • Damages to the optic nerve that causes vision problems also known as optic nerve atrophy.
  • Lifelong vision problems that are caused due to incorrect optic nerve also known as abnormalities in the development of optic nerve.

Conclusion

Although Chiari malformation II is detected during childhood and is found in kids it can pose a threat to adults too because the reason behind people getting affected is still unknown. The consequences of Chiari malformation II are not that dire but they still can alter a person’s life and cause them a lot of problems during the course of their lives. The awareness in people about Chiari malformation is increasing every year but it still isn’t enough. A lot of famous celebrities like J.B Holmes, Rosanne Cash, Joanna David, and Allysa Seely are doing their part in spreading awareness about Chiari malformation. Also, various TV shows like The Choice, CSI: Crime Scene Investigation and House M.D have spoken about Chiari malformation type ll during their shows.

Women are at a higher risk of suffering from Chiari malformation type ll than men. Although the reason for this is still unknown we can make sure we do our daily check-ups and visit our doctors frequently. The results may not be something that will be pleasant, but it is important to know that treating Chiari malformation type ll is of utmost importance and instead of panicking we should believe in our doctors and follow their instructions. Also as Chiari malformation II usually affects kids the diagnosis can be very shocking for the parents but it is very important for the parents to keep a calm mind and follow all the plans and procedures that are prescribed to them. Always follow and believe in the words of your medical examiner for a smooth diagnosis, treatment, and recovery.

References:  

  1. Geerdink, N., van der Vliet, T., Rotteveel, J., Feuth, T., Roeleveld, N., & Mullaart, R. Essential features of Chiari II malformation in MR imaging: an interobserver reliability study—part 1. Child’s Nervous System, (2012). 977-985. doi: 10.1007/s00381-012-1761-5
  2. Otera, Y., Morokuma, S., Fukushima, K., Anami, A., Yumoto, Y., & Ito, Y. et al. Neurological outcomes in Chiari type II malformations and their correlation to morphological findings and fetal heart rate patterns: a retrospective study. BMC Research Notes, (2015). 57. doi: 10.1186/s13104-015-1014-2

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