Chiari malformation type 2 treatment
The treatment of Chiari malformation type 2 (CM-2) often involves surgical intervention, particularly when there are associated complications such as hydrocephalus or myelomeningocele. The primary goal of surgery is to relieve pressure on the brain and spinal cord and to restore normal cerebrospinal fluid flow. The specific surgical procedures may vary depending on the individual's condition, age, and the severity of symptoms.
Posterior fossa decompression for Chiari malformation
Posterior fossa decompression for Chiari malformation
Treatment of associated conditions: If there is a myelomeningocele present, surgical repair of the spina bifida defect may be necessary. Hydrocephalus may be managed with the placement of a shunt, a device that helps drain excess fluid from the brain to another part of the body where it can be absorbed.
It's important to note that not all individuals with Chiari malformation type 2 may require surgery, especially if they are asymptomatic or have mild symptoms. In such cases, careful monitoring and non-surgical management may be appropriate.
The decision to pursue surgery and the specific approach used depend on the individual's overall health, the severity of symptoms, and the presence of associated complications. The surgical team, typically including neurosurgeons and other specialists, will carefully evaluate the risks and benefits of surgery for each patient.
After surgery, individuals may need ongoing follow-up care to monitor their condition and address any residual symptoms. Rehabilitation and physical therapy may also be recommended to help with recovery and improve quality of life. As with any medical condition, treatment plans are individualized, and discussions with healthcare providers are crucial for making informed decisions about the management of Chiari malformation type 2.
Surgical intervention to repair myelomeningocele is commonly required. Some evidence has demonstrated benefits in performing the procedure on the in utero fetus 1)
Chiari malformation type 2 patients are now operated on with the first detectable symptom or evidence of a syrinx, and yet medullary dysfunction from the Chiari II malformation remains the leading cause of death in myelomeningocele treatment today. Our knowledge of the natural history of the untreated conditions and the increased safety of the operation has made surgical intervention a much more viable option for this group of patients 2).
In a large series of 148 patients with CM II, only 14% of patients required surgical decompression 3). Treating the obstructive hydrocephalus by shunting is a more common method that is associated with fewer risks. In a series of 71 cases of CM II, 64 (90%) patients had hydrocephalus, 89% of which required VP shunting 4).
Treatment of patients with Chiari II malformations is complex due to the variety and variable severity of malformations:
myelomeningocoele repair and management of neurogenic bladder
ventricular shunting (usually ventriculoperitoneal)
hydrocephalus usually requires shunting and can help ameliorate cranial nerve and brainstem dysfunction
craniovertebral decompression
may also be required in neonates which brainstem dysfunction if hydrocephalus is not present or symptoms and signs do not improve with shunting
older patients with hind brain herniation or syringohydromyelia may also benefit.