About chiari's disease
What is chiari's disease?
Budd-Chiari syndrome is a rare disorder characterized by narrowing and obstruction (occlusion) of the veins of the liver (hepatic veins). Symptoms associated with Budd Chiari syndrome include pain in the upper right part of the abdomen, an abnormally large liver (hepatomegaly), and/or accumulation of fluid in the space (peritoneal cavity) between the two layers of the membrane that lines the stomach (ascites). Additional findings that may be associated with the disorder include nausea, vomiting, and/or an abnormally large spleen (splenomegaly). The severity of the disorder varies from case to case, depending upon the site and number of affected veins. In some cases, if the major hepatic veins are involved, high blood pressure in the veins carrying blood from the gastrointestinal (GI) tract back to the heart through the liver (portal hypertension) may be present. In most cases, the exact cause of Budd-Chiari syndrome is unknown.
What are the symptoms for chiari's disease?
The more common types of Chiari malformation are:
- Type 1
- Type 2
Although these types are less serious than the rarer pediatric form, type 3, signs and symptoms still can be life disrupting.
Chiari malformation type 1
In Chiari malformation type 1, signs and symptoms usually appear during late childhood or adulthood.
Headaches, often severe, are the classic symptom of Chiari malformation. They generally occur after sudden coughing, sneezing or straining. People with Chiari malformation type 1 can also experience:
- Neck pain
- Unsteady gait (problems with balance)
- Poor hand coordination (fine motor skills)
- Numbness and tingling of the hands and feet
- Difficulty swallowing, sometimes accompanied by gagging, choking and vomiting
- Speech problems, such as hoarseness
Less often, people with Chiari malformation may experience:
- Ringing or buzzing in the ears (tinnitus)
- Slow heart rhythm
- Curvature of the spine (scoliosis) related to spinal cord impairment
- Breathing problems, such as central sleep apnea, which is when a person stops breathing during sleep
Chiari malformation type 2
In Chiari malformation type 2, a greater amount of tissue extends into the spinal canal compared with that in Chiari malformation type 1.
The signs and symptoms can include those related to a form of spina bifida called myelomeningocele that nearly always accompanies Chiari malformation type 2. In myelomeningocele, the backbone and the spinal canal don't close properly before birth.
Signs and symptoms may include:
- Changes in breathing pattern
- Swallowing problems, such as gagging
- Quick downward eye movements
- Weakness in the arms
Chiari malformation type 2 is usually noted with ultrasound during pregnancy. It may also be diagnosed after birth or in early infancy.
Chiari malformation type 3
In the most severe type of the condition, Chiari malformation type 3, a portion of the lower back part of the brain (cerebellum) or the brainstem extends through an opening in the back of the skull. This form of Chiari malformation is diagnosed at birth or with an ultrasound during pregnancy.
This type of Chiari malformation has a higher mortality rate and may also cause neurological problems.
What are the causes for chiari's disease?
Chiari malformation type 1 occurs when the section of the skull containing a part of the brain (cerebellum) is too small or is deformed, thus putting pressure on and crowding the brain. The lower part of the cerebellum (tonsils) is displaced into the upper spinal canal.
Chiari malformation type 2 is nearly always associated with a form of spina bifida called myelomeningocele.
When the cerebellum is pushed into the upper spinal canal, it can interfere with the normal flow of cerebrospinal fluid that protects the brain and spinal cord.
This impaired circulation of cerebrospinal fluid can lead to the blockage of signals transmitted from the brain to the body, or to a buildup of spinal fluid in the brain or spinal cord.
Alternatively, the pressure from the cerebellum on the spinal cord or lower brainstem can cause neurological signs or symptoms.
What are the treatments for chiari's disease?
Treatment for Chiari malformation depends on the severity and the characteristics of your condition.
If you have no symptoms, your doctor likely will recommend no treatment other than monitoring with regular examinations and MRIs.
When headaches or other types of pain are the primary symptom, your doctor may recommend pain medication.
Reducing pressure with surgery
Doctors usually treat symptomatic Chiari malformation with surgery. The goal is to stop the progression of changes in the anatomy of the brain and spinal canal, as well as ease or stabilize symptoms.
When successful, surgery can reduce pressure on the cerebellum and spinal cord and restore the normal flow of spinal fluid.
In the most common surgery for Chiari malformation, called posterior fossa decompression, the surgeon removes a small section of bone in the back of the skull, relieving pressure by giving the brain more room.
In many cases, the covering of the brain, called the dura mater, may be opened. Also, a patch may be sewn in place to enlarge the covering and provide more room for the brain. This patch may be an artificial material, or it could be tissue harvested from another part of the body.
Your doctor may also remove a small portion of the spinal column to relieve pressure on the spinal cord and allow more space for the spinal cord.
The surgical technique may vary, depending on whether a fluid-filled cavity (syrinx) is present, or if you have fluid in your brain (hydrocephalus). If you have a syrinx or hydrocephalus, you may need a tube (shunt) to drain the excess fluid.
Surgical risks and follow-up
Surgery involves risks, including the possibility of infection, fluid in the brain, cerebrospinal fluid leakage or problems with wound healing. Discuss the pros and cons with your doctor when deciding whether surgery is the most appropriate treatment for you.
The surgery reduces symptoms in most people, but if nerve injury in the spinal canal has already occurred, this procedure won't reverse the damage.
After the surgery, you'll need regular follow-up examinations with your doctor, including periodic imaging tests to assess the outcome of surgery and the flow of cerebrospinal fluid.
What are the risk factors for chiari's disease?
There's evidence that Chiari malformation runs in some families. However, research into a possible hereditary component is still in its early phase.
Is there a cure/medications for chiari's disease?
The degree and characteristics of your problem will determine how to treat your Chiari malformation. If you don't have any symptoms, your doctor won't likely prescribe anything other than monitoring through routine checkups and MRIs. Your doctor could prescribe painkillers if your main symptom is a headache or another kind of pain. Surgery is typically used by doctors to treat symptomatic Chiari malformation. In addition to easing or stabilizing symptoms, the aim is to halt the course of alterations in the structure of the brain and spinal canal. If surgery is successful, pressure on the cerebellum and spinal cord can be relieved, and the regular flow of spinal fluid can be reinstated. The procedure known as posterior fossa decompression, which is the most popular treatment for Chiari malformation, involves the surgeon removing a small piece of bone from the back of the skull to relieve pressure by giving the brain more space. The dura mater, the brain's protective covering, may frequently be opened. Additionally, a patch might be sewn in to make the covering bigger and provide the brain more space. This patch could be made of synthetic material or from tissue removed from another area of the body. Symptoms: neck pain, dizziness Drugs: indomethacin, naproxen Conditions: poor hand coordination, unsteady gait