What is Hydranencephaly: Causes, Symptoms, Treatment, Prognosis

What is Hydranencephaly?

Hydranencephaly is an extremely rare congenital medical condition which affects the central nervous system of the individual.  Hydranencephaly is characterized by the absence of the cerebral hemispheres and the corpus striatum in the baby and this area is replaced by cerebrospinal fluid[1, 2, 3].

Hydranencephaly does not have a gender bias and both baby boys and girls can be equally affected.  However, some babies are at a higher risk simply because their parents may be the carriers of the genes responsible for Hydranencephaly[1, 2, 3].

The root cause of Hydranencephaly is not yet known but experts believe genetic makeup is a major contributing factor.  As of now, there is no cure for Hydranencephaly and in most cases the baby with this condition survives only for a few months after delivery or may even succumb in the womb itself[1, 2, 3].

What Causes Hydranencephaly?

As stated, the root cause of Hydranencephaly is not yet known.  It is an inherited condition but how the disease gets transmitted is not entirely clear.  Some experts believe that a blockage of the carotid artery at the area where it enters the cranium may be a potential cause.  However, why this blockage occurs is something which still remains unknown[2].

Hydranencephaly is believed to be autosomal recessive disorder.  This means that one copy of the defective gene should be inherited by each parent for the baby to develop Hydranencephaly.  In cases where a baby receives one defective gene from either parent then he or she will be a carrier of the disease and will not have any symptoms[2].

The chances of Hydranencephaly being transferred to the offspring are around 25% for couple who are both carriers of the defective gene and 50% will be the chances of their offspring also becoming a carrier of the defective gene.  The risk of transferring the disease to the offspring remains the same for each pregnancy[2].

What are the Symptoms of Hydranencephaly?

When a baby is born with Hydranencephaly, there may not be any telltale symptoms aside from may be an enlarged head. If an enlarged head is not present then it may take weeks if not months for a physician to diagnose Hydranencephaly. During this time, the baby will have jerking movements[3].

Other symptoms that can be seen in babies with Hydranencephaly include failure to thrive, seizures, problems with seeing things clearly, and hypertonia. Babies with Hydranencephaly will also have intellectual disability. They will have problems with digestion and breathing[3].

How is Hydranencephaly Treated?

As stated, Hydranencephaly being a congenital condition does not have a cure.  Thus treatment is mainly supportive and symptomatic. It is imperative for the parents to adhere to the recommendations of the physician. The treatment plan should be discussed thoroughly with the physician reviewing all the pros and cons[3].

Symptomatically, for babies experiencing seizures due to Hydranencephaly he or she may be given antiepileptics to cut down the episodes. For digestive problems, stool softeners will be given for issues with constipation[3].

Some physicians recommend surgery to drain out the fluid in the cranial hemisphere.  This tends to increase the survival rate of the child. The procedure involves placing a valve in the head. A catheter is then attached to this valve and the fluid is gently drained from the head into the upper body. This helps in relieving the pressure from the head caused due to excess fluid[3].

What Is The Prognosis Of Hydranencephaly?

The prognosis of a baby with Hydranencephaly is quite poor.  In some instances, the baby may not even be able to survive till the time of delivery.  In other cases, the baby may at maximum survive for a few days to a month.  However, there have been some cases where children with Hydranencephaly have survived for years but are not able to survive into adulthood due to the missing portion of the brain[3].

It is important for parents of children with Hydranencephaly to seek emotional support as diagnosis and treatment for their baby with this condition and the prognosis associated with this condition can be emotionally draining for parents.  Additional help may be sought from support groups and mental health services[3].

Research is ongoing as to identify the causes and come up with some ways to not only treat and increase survival rates but also prevent children from developing a condition like Hydranencephaly[3].

References:

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