About chiari i syndrome
What is chiari i syndrome?
Chiari-Frommel Syndrome is a rare endocrine disorder that affects women who have recently given birth (postpartum) and is characterized by the over-production of breast milk (galactorrhea), lack of ovulation (anovulation), and the absence of regular menstrual periods (amenorrhea). In Chiari-Frommel Syndrome, these symptoms persist long (for more than six months) after childbirth. The absence of normal hormonal cycles may result in reduced size of the uterus (atrophy). Some cases of Chiari-Frommel Syndrome resolve completely without treatment (spontaneously); hormone levels and reproductive function return to normal.
What are the symptoms for chiari i syndrome?
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 i syndrome?
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 i syndrome?
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 i syndrome?
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 i syndrome?
The particular type of malformation and the evolution of anatomical changes or symptoms strongly influence how Chiari malformations and syringomyelia are treated.
1. Asymptomatic Chiari I syndrome should be ignored (this involves most Chiari malformations). Of these, there is no need for "prophylactic" surgery. Treatment is typically advised if the abnormality is deemed symptomatic or creates a syrinx.
2. If the patient is symptomatic and doctors have verified that there are no hydrocephalus-related problems, Chiari II abnormalities are treated. Consideration of a tethered cord is also investigated in some patients. The onset and progression of symptoms in many newborns with a Chiari II malformation are severe and quick, necessitating an urgent or emergency response.
3. Depending on the type of deformity, several surgical procedures are used to treat it. Surgery aims to alleviate symptoms or halt the progression of an illness or symptoms.
4. Based on the degree of compression or other irregularities, surgery can take many different forms or span a wide range of procedures. Surgical decompression of the overlaying bones alone, decompression of the bones and release of the dura (a thick membrane encasing the brain and spinal cord), or decompression of the bone and dura with some degree of cerebellar tissue removal may be used to treat Chiari I malformations. Sometimes individuals will additionally need a fusion of their cervical spines.
Loss of bowel and bladder control,Motor impairment