Hydranencephaly is a rare neurological condition which indicates that a child is missing much or most of their cerebral hemispheres, that is, the two masses of follded brain tissue (cortex) that surround the brain stem.
With hydranencephaly the baby’s brain develops normally until “something” happens to cut off the flow of blood to the baby’s brain. The affected part of the brain then starts to die and the tissue is reabsorbed by the body and replaced with cerebral spinal fluid (CSF). The “something” that cuts off the flow of blood to the baby’s brain can be quite brief. Some of the most common causes are a stroke in the baby, prenatal drug exposure, and the death of a twin in utero. In many of the children the cause is unknown. The damage to the brain usually occurs in the 2nd or 3rd trimester of pregnancy.
Facts about Hydranencephaly taken from the Rays of Sunshine Website:
Treatment:
There is no definitive treatment for Hydranencephaly. Treatment is symptomatic and supportive.
Hydrocephalus
Hydrocephalus is often one of the first difficulties faced by the child. In our experience 60% of the children have needed a shunt. Some children have mild hydrocephalus but live their entire life without a shunt and some never do develop an increase in intracranial pressure from fluid.
Hydrocephalus is often one of the first difficulties faced by the child. In our experience 60% of the children have needed a shunt. Some children have mild hydrocephalus but live their entire life without a shunt and some never do develop an increase in intracranial pressure from fluid.
Irritability
A child with Hydranencephaly is often very irritable in the first year. A build up of fluid is one of the first things to check if your child is irritable. As 65% of the children have gastro esophageal reflux, which can be very painful this is something that should also be checked. As the children grow older, 57% are said to be happy with 18% being seen as quiet. Only 19% were still described as being fussy or irritable past the one year mark.
A child with Hydranencephaly is often very irritable in the first year. A build up of fluid is one of the first things to check if your child is irritable. As 65% of the children have gastro esophageal reflux, which can be very painful this is something that should also be checked. As the children grow older, 57% are said to be happy with 18% being seen as quiet. Only 19% were still described as being fussy or irritable past the one year mark.
Increased Tone
Children with Hydranencephaly may have increased tone in their bodies (spasticity). In our study, 41% of the children were described as having increased tone, and 6% were described as being "floppy". Another 47% were described as having mixed tone. This means that they are sometimes very floppy and sometimes very tight. Physical and Occupational therapy are very helpful in preventing problems from increased tone. However, children do frequently need various orthopedic surgeries as they get older. The therapists will likely suggest a variety of equipment that will also help in preventing a child from developing problems. The children are sometimes referred to as having Cerebral Palsy or Spastic Paralysis.
Children with Hydranencephaly may have increased tone in their bodies (spasticity). In our study, 41% of the children were described as having increased tone, and 6% were described as being "floppy". Another 47% were described as having mixed tone. This means that they are sometimes very floppy and sometimes very tight. Physical and Occupational therapy are very helpful in preventing problems from increased tone. However, children do frequently need various orthopedic surgeries as they get older. The therapists will likely suggest a variety of equipment that will also help in preventing a child from developing problems. The children are sometimes referred to as having Cerebral Palsy or Spastic Paralysis.
Seizures
Seizures are fairly common in children with Hydranencephaly (75%). The type and severity of the seizures vary from child to child. Most of the seizures are fairly short and mild but some children do have longer more severe seizures. There are many different seizure medications children take. Often a child needs more than one medication at a time and they frequently need to have their medications changed. A problem both in diagnosing a child who has Hydranencephaly with seizures and in treating them is that EEGs, and medications are geared towards those who are having cortical seizures. Most children with Hydranencephaly have no cortex and have brain stem seizures. So, you and your Dr may agree that your child is having seizures, but nothing shows up on the EEG. And, medications geared at controlling cortical seizures may not work well on a child with Hydranencephaly. That is why your child may need a variety of medications.
Seizures are fairly common in children with Hydranencephaly (75%). The type and severity of the seizures vary from child to child. Most of the seizures are fairly short and mild but some children do have longer more severe seizures. There are many different seizure medications children take. Often a child needs more than one medication at a time and they frequently need to have their medications changed. A problem both in diagnosing a child who has Hydranencephaly with seizures and in treating them is that EEGs, and medications are geared towards those who are having cortical seizures. Most children with Hydranencephaly have no cortex and have brain stem seizures. So, you and your Dr may agree that your child is having seizures, but nothing shows up on the EEG. And, medications geared at controlling cortical seizures may not work well on a child with Hydranencephaly. That is why your child may need a variety of medications.
Feeding
Children with Hydranencephaly may have trouble sucking and swallowing. Many families are told that their child will lose these abilities within a few weeks of birth when the "higher" cortical areas of the brain normally kick in. We haven't found this to be true. In our study 39% of the children eat orally, 39% eat only by tube and 16% eat both orally and by tube. As the child gets older he may have more trouble eating and eventually need a feeding tube.
Children with Hydranencephaly may have trouble sucking and swallowing. Many families are told that their child will lose these abilities within a few weeks of birth when the "higher" cortical areas of the brain normally kick in. We haven't found this to be true. In our study 39% of the children eat orally, 39% eat only by tube and 16% eat both orally and by tube. As the child gets older he may have more trouble eating and eventually need a feeding tube.
Vision/Hearing
In our study, 92% of the children can hear. Some children may not understand what they hear but do respond to sounds and familiar words. However, parents feel that 46% of the children understand at least some of what is said to them. 60% of the children are thought to see at least some of the time. Most of the children have what is called Cortical Visual Impairment. This means that there is nothing wrong with their eyes, but that their brains aren’t able to tell them what they are seeing. This sort of vision can come and go so that children see some things and not others or will see one day and not the next.
In our study, 92% of the children can hear. Some children may not understand what they hear but do respond to sounds and familiar words. However, parents feel that 46% of the children understand at least some of what is said to them. 60% of the children are thought to see at least some of the time. Most of the children have what is called Cortical Visual Impairment. This means that there is nothing wrong with their eyes, but that their brains aren’t able to tell them what they are seeing. This sort of vision can come and go so that children see some things and not others or will see one day and not the next.
Other Conditions Children with Hydranencephaly may have:
Asthma/Reactive Airways disease (RAD): 20%.
Diabetes Insipidus (a high sodium level and excessive urine output): 27%
Gastro esophageal Reflux (food comes up which causes vomiting or excessive acid production): 65%
Constipation: 66%
Asthma/Reactive Airways disease (RAD): 20%.
Diabetes Insipidus (a high sodium level and excessive urine output): 27%
Gastro esophageal Reflux (food comes up which causes vomiting or excessive acid production): 65%
Constipation: 66%
Some children also develop obstructed or difficult breathing and need to have a tracheostomy. At this time 7% of the children have a trach. Several of the children have also needed help from a Bipap or a Ventilator as they get older.
Prognosis:
Of the 146 children we have information on, 93 are living and 53 have died. Of the children who have died 40%** died before their first birthday. However of the children who are alive, only 10% are under the age of 1. Our oldest member is 24. It appears that the first year is the most difficult for our children. If they survive that year then many will live a good number of years further.
