What is hydranencephaly?
Hydranencephaly is a central nervous system disorder characterized by an enlarged head and neurological deficits. The exact cause of Hydranencephaly is not known. This extremely rare form of Hydrocephalus involves the absence of portions of the brain. Results of neurologic examination in newborns may be normal or abnormal. The head usually appears enlarged at birth. Vision impairment, lack of growth and intellectual deficits are symptomatic of this disorder.
What are the symptoms for hydranencephaly?
Babies born with hydranencephaly may have obvious symptoms at birth. In some cases, it may take weeks or months for obvious signs to appear. One of the most noticeable symptoms of hydranencephaly is an enlarged head size. Infants with the disease may have increased muscle tone, but limited movement.
Other common hydranencephaly symptoms include:
- Seizures and uncontrolled movements
- lack of growth
- breathing and digestion problems
- difficulty regulating body temperature
- vision problems
- intellectual disability
What are the causes for hydranencephaly?
Hydranencephaly is the result of injury to a baby’s nervous system or an abnormal formation of it. It occurs during the earliest stages of development in the uterus. But it’s not entirely clear what causes these problems. Hydranencephaly may be an inherited condition.
One theory about the abnormal brain development is that there’s blockage in the carotid artery. That’s the blood vessel that carries blood to the brain. However, researchers don’t have an explanation for why the carotid artery would become blocked.
Other possible causes include:
- an infection of the uterus early in the pregnancy
- a mother’s exposure to environmental toxins
- other circulation problems with the baby
What are the treatments for hydranencephaly?
Because hydranencephaly currently has no cure, treatment focuses on managing symptoms.
To help remove excess cerebrospinal fluid from the brain, a surgeon can place a shunt, or one-way valve, inside the skull. The shunt is attached to a catheter, a flexible tube. The catheter will run into the abdominal cavity, where the fluid is transferred from the brain. This procedure can help relieve painful pressure inside your child’s head. It may also prolong life.
Seizures can be treated with medications, including anticonvulsants, as well as other therapies. Diet and calming techniques can sometimes reduce seizures, too.
Calming and relaxation strategies may also help reduce irritability. Medications, such as stool softeners, may also be helpful in treating constipation.
It’s important that treatment for all symptoms and complications are coordinated by your child’s doctor. This will help to avoid overmedication or unwanted side effects from multiple medications and procedures.
What are the risk factors for hydranencephaly?
An infection of the uterus early in the pregnancy a mother’s exposure to environmental toxins other circulation problems with the baby
Is there a cure/medications for hydranencephaly?
Each case of hydranencephaly is unique. The condition may be so severe the baby doesn’t survive pregnancy.
But there are more cases of children living for a few years with hydranencephaly. Still, the odds of reaching adulthood are slim. The emotional impact of having a child with hydranencephaly is profound. Don’t hesitate to seek counseling and support to help you get through this challenging chapter in your life.
Research into the causes and prevention of fetal neurological diseases continues.