Isolated Dandy-Walker malformation with hydrocephalus is a rare congenital brain malformation that affects the cerebellum, the part of the brain responsible for coordination and movement. It is characterized by an enlarged fourth ventricle, a cyst in the back of the brain, and a partial or complete absence of the cerebellar vermis, the part of the cerebellum that connects the two hemispheres. This condition can cause hydrocephalus, a buildup of cerebrospinal fluid in the brain, which can lead to increased pressure in the skull and cause neurological problems. Treatment typically involves surgery to place a shunt to drain the excess fluid and relieve the pressure.

The symptoms of Isolated Dandy-Walker malformation with hydrocephalus can vary depending on the severity of the condition. Common symptoms include:

-Headache
-Vomiting
-Lethargy
-Developmental delays
-Seizures
-Vision problems
-Hearing loss
-Balance problems
-Difficulty walking
-Abnormal eye movements
-Abnormal head shape
-Increased head circumference

The exact cause of Isolated Dandy-Walker malformation with hydrocephalus is unknown. However, it is believed to be caused by a combination of genetic and environmental factors. Genetic factors may include mutations in certain genes, such as the L1CAM gene, which is involved in the development of the cerebellum. Environmental factors may include exposure to certain toxins or infections during pregnancy.

1. Shunt Surgery: A shunt is a small tube that is surgically placed in the brain to drain excess fluid and reduce pressure.

2. Endoscopic Third Ventriculostomy (ETV): This procedure involves making a small hole in the floor of the third ventricle to allow the fluid to drain.

3. Ventriculoperitoneal Shunt (VP Shunt): This procedure involves placing a tube in the brain to drain the excess fluid into the abdomen.

4. Cranial Vault Expansion: This procedure involves surgically opening the skull to create more space for the brain and reduce pressure.

5. Stereotactic Radiosurgery: This procedure uses radiation to reduce the size of the cyst and reduce pressure.

6. Medication: Medications such as diuretics

1. Genetic mutations: Mutations in the L1CAM gene, which is responsible for the development of the cerebellum, are the most common cause of Isolated Dandy-Walker malformation with hydrocephalus.

2. Family history: A family history of Isolated Dandy-Walker malformation with hydrocephalus increases the risk of the condition.

3. Maternal age: Women over the age of 35 are more likely to have a baby with Isolated Dandy-Walker malformation with hydrocephalus.

4. Maternal infections: Maternal infections during pregnancy, such as rubella, cytomegalovirus, and toxoplasmosis, can increase the risk of Isolated Dandy-Walker malformation with hydrocephalus.

5. Maternal drug use: Maternal drug use,

There is no cure for Isolated Dandy-Walker malformation with hydrocephalus. Treatment typically involves medications to reduce the pressure in the brain, such as diuretics, and surgery to place a shunt to drain the excess fluid. In some cases, physical therapy may be recommended to help with any motor delays.