Hydrocephalus = Hydro (water) cephalus (brain), meaning there is too much “water” or fluid on the brain. Cerebrospinal fluid, or CSF, is is a type of fluid continuously produced within the brain at a rate of about 2 cups per day. If this fluid isn’t cleared or drained properly, it builds up and puts pressure on the brain.

Some of the common reasons for too much fluid on the brain are:

*Structural: A problem that is blocking the fluid such as a malformation of the brain structures like a Chiari malformation or brain tumor
*Brain bleed, can be seen more in premature babies
*Meningitis: This creates a situation where the CSF fluid can’t circulate properly or be absorbed appropriately
*Unknown reason (ie idiopathic)

Most children with clinically significant hydrocephalus will need a shunt which is a small, flexible plastic tubing that drains the fluid from the brain, down through the neck and into the abdominal area (peritoneum) or occasionally into the heart (atria). Another treatment called endoscopic third ventriculostomy (ETV) can be used to treat hydrocephalus that is caused by a blockage within the ventricles. One of the problems we see in international adoption is that shunting or ETV should have been done at a much earlier time and the fluid can build up significantly if treatment is delayed, causing pressure on the brain and subsequent damage. Another problem is that these procedures must be performed by experienced neurosurgeons (brain surgeon) and there are many children in developing countries that do not have access to the surgical services needed. Once the brain is damaged, some recovery can usually take place but typically there is damage that may be permanent. The earlier the hydrocephalus is caught, the better the potential outcome as kids who have early intervention with shunting can lead very full lives.

Children with hydrocephalus need careful monitoring and maintenance. The failure rate of shunts in the first year after being placed in the brain is as high as 40%2 and most shunts will have some failures in childhood due to accidental disconnection, kinking of the tubing or infection.3 Malfunction of the shunts or ETV can be serious but subtle and many common pediatric complaints like nausea/vomiting, fatigue, fussiness, or headaches will require a full hydrocephalus evaluation before being able to assume that the problem is caused by a common virus like most kids have. Other neurological problems like eye movements or increase in head size can also indicate malfunction.1

Age at the time of referral is important when considering the history of hydrocephalus. Because a brain infection or structural/brain malformation can be the cause of hydrocephalus, this can have potential cognitive or learning impacts as well. The older the child and the more they are able to demonstrate cognitive achievement and flexibility, the more we will know about their future outcome and ability to be independent adults. Under 2-3 years of age, milestones are primarily motor (sittting, standing, running) and so we don’t know a lot about their future brain functioning yet at this point. History of hydrocephalus therefore requires significant consideration about the potential future learning and brain implications as well as the medical and surgical needs that the child and family will have to maintain a shunt as their child grows.

Summary:

*Hydrocephalus: Increased CSF fluid inside the brain that causes pressure

*The longer it is untreated (not shunted), the more serious the outcome

*Shunted kids can do very well, but often identification and treatment is delayed in children who are in the adoption system. Age at the time of referral, neurological and developmental milestones, reason for hydrocephalus, and severity all factor into long-term outcomes.

References

1. Lee TT, Uribe J, Ragheb J, Morrison G, Jagid JR. Unique clinical presentation of pediatric shunt malfunction. Pediatr Neurosurg. 1999;30(3):122-126.
2. Drake JM, Sainte-Rose C, Drake J. The shunt book. Blackwell Science Cambridge, MA; 1995.
3. Sainte-Rose C, Piatt J, Renier D, et al. Mechanical complications in shunts. Pediatr Neurosurg. 1991;17(1):2-9.

This article was generously shared by Dr. Judith Eckerle of the University of Minnesota,  Adoption Medicine Clinic. Child referrals are becoming increasingly complex, and adoption medicine professionals are identifying many more children with special needs from all countries that participate in intercountry adoption, as well as from the United States. To help families prepare for these changes, Dr. Eckerle and other medical specialists are writing a book: Health Topics For Preadoptive Families.