Description
Hydranencephaly is a rare condition in which the brain's cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid. An infant with hydranencephaly may appear normal at birth. The infant's head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal. However, after a few weeks the infant usually becomes irritable and has increased muscle tone. After a few months of life, seizures and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain) may develop. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis (paralysis), and intellectual deficits. Hydranencephaly is considered to be an extreme form of porencephaly (a rare disorder characterized by a cyst or cavity in the cerebral hemispheres) and may be caused by vascular infections or traumatic disorders after the 12th week of pregnancy. Diagnosis may be delayed for several months because early behavior appears to be relatively normal. Some infants may have additional abnormalities at birth including seizures, myoclonus (spasm or twitching of a muscle or group of muscles), and respiratory problems.
Treatment
There is no definitive treatment for hydranencephaly. Treatment is symptomatic and supportive. Hydrocephalus may be treated with a shunt (a surgically implanted tube that diverts fluid from one pathway to another).
Prognosis
The outlook for children with hydranencephaly is poor. Death generally occurs before age 1.
Research
The National Institute of Neurological Disorders and Stroke (NINDS) conducts and supports a wide range of studies that explore the complex mechanisms of normal brain development. The knowledge gained from these fundamental studies provides the foundation for understanding how this process can go awry and, thus, offers hope for new means to treat and prevent developmental brain disorders, including hydranencephaly.

Additional information is available from the following organizations:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
914-428-7100
National Organization for Rare Disorders
P.O. Box 8923
New Fairfield, CT 06812-8923
203-746-6518
1-800-999-6673
National Information Center for Children & Youth with Disabilities
P.O. Box 1492
Washington, DC 20013-1492
202-884-8200
1-800-695-0285
Information provided by the National Institute of Neurological Disorders and Stroke's factsheet titled, "Hydranencephaly." Prepared for Healthtouch, December 1997.



I had not been on the Internet long when I met a wonderful lady named Barb. She has a daughter, *Krista, who has Hydranencephaly. I had never heard of this condition before, so I just had to read what I could on it. I felt so sorry for Barb, just knowing that Krista would not live long. Then I found out something amazing…. Krista had just turned 9 at the time and she will be 10 YEARS OLD in December (1998)!!!  Barb now has her own mailing list that has many parents of Hydran kids on it. On CPSK we also have another mom, Erica, who has a son, Robbie, and he is almost 3 years old. You can read more about Krista, Robbie, and some other beautiful children with Hydranencephaly on Barb's site. It's very informative and will really encourage any new moms with special needs kids.
Here's a link for her site and if you have a child with
Hydranencephaly please consider joining her mailing list :)


*Name has been changed for privacy.


Since the making of this website Krista has went to be with the Lord. Look for more about her on our Memorial Page.