Kristin Crow got a big surprise when her little bundle of joy arrived prematurely while she was visiting family in Texas in November 2011. She gave birth to her son, Skyler Crow, who was born at 28 weeks, weighing just two pounds, seven ounces.
When in utero, Skyler had been diagnosed with ventriculomegaly, or enlarged ventricles in the brain, and he developed hydrocephalus soon after he was born while in the neonatal intensive care unit (NICU) in Texas.
Hydrocephalus is one of the most common congenital conditions in children, affecting 1 in 500 to 1,000 births. It is a condition where cerebrospinal fluid (CSF) doesn’t reabsorb properly in the brain, causing a harmful build-up. This can cause problems with the development and function of a child’s brain, and without treatment, hydrocephalus can be deadly.
The traditional treatment for hydrocephalus is surgery to implant a shunt, or a small tube, that drains spinal fluid from the brain ventricles down to the abdomen where it is absorbed. Doctors told Kristin and her husband that a shunt was their only treatment option.
“I was terrified as shunts come with a lot of risks,” said Kristin. “There’s a high rate of infections, complications and we would be in the NICU for a very long time, possibly more than five months.”
The Burden of a Shunt
Dr. Samuel Browd, pediatric neurosurgeon and Director of Hydrocephalus at Seattle Children’s Hospital, understands that these risks can be very difficult for families.
Dr. Browd explains that families live on pins and needles because the shunt technology has changed very little since the 1950s and their failure rates are as high as 30 percent in the first year, and almost 100 percent within 10 years. Shunt failure is a life-threatening emergency requiring surgical replacement, and each year about 38,000 surgeries are performed to replace failed shunts in the U.S.
“Shunts have worked well in terms of getting the job done and moving fluid from place to another but they come with a lot of baggage,” said Dr. Browd. “There is a high infection rate within the first few months and, once you get passed that, they eventually fail. Many families don’t go on vacations anymore because they don’t know when the shunt may fail and they’re worried there’s not going to be a neurosurgeon close by if it does. It’s a very difficult way to live life.”
Hydrocephalus and shunt failure creates major social, medical and economic problems. It accounts for half of all pediatric neurosurgery cases, more than $3 billion a year in health care expenses and about 4 percent of all pediatric hospital charges in the U.S.
New Hope Found in Seattle: Choroid Plexus Cauterization (CPC)
Luckily, Skyler’s condition improved enough for him to be released, and after 70 days in the NICU, they returned home to Seattle. They had been referred to Children’s and this is where they found hope and learned that a shunt wasn’t their only option.
Children’s neurosurgery team recently began offering a new treatment for babies within their first year of life called Choroid Plexus Cauterization (CPC). It is performed during a surgery called Endoscopic Third Ventriculostomy (ETV) where neurosurgeons create a pathway to allow the fluid to reach the areas around the brain where it is absorbed. In the CPC portion of the surgery, they then use a flexible endoscope to apply heat to the tissue in the brain that makes the fluid, reducing the amount of fluid that is produced.
“The beauty of ETV/CPC is that if it’s successful, then you’ve essentially treated the hydrocephalus and the child can become shunt independent,” said Dr. Browd. “The pressure is reduced, the brain can re-expand and children can hopefully go about a normal life and growth phase.”
Children’s is the only hospital in the region that offers this treatment for babies with hydrocephalus.
“I was so excited when I learned there was another option for Skyler,” said Kristin. “In Texas, all we heard was shunt, shunt, shunt and we didn’t know there was another way.”
Skyler is now 10 months old and in less than a month after surgery, Kristin is thrilled with the results.
“Skyler’s progress has blown me out of the water!” said Kristin. “He is like a whole new baby where his development has improved significantly. He went from acting like a 6- to 7-month-old to acting like a regular 10-month-old within a month’s time. There is such a difference in his personality where he used to cry all the time and now he is laughing non-stop. He can now also sit unassisted, crawl and say his first words!”
Kristin has a message for other parents who may find themselves in a similar situation.
“Take a deep breath and research all your options,” she said. “We didn’t know there was another way and it is important for parents to know that there are other options out there,” said Kristin.
Implications for the Future
Dr. Browd is very excited about this promising new procedure. The neurosurgery team has had great results with it and he thinks there is a large portion of patients that will benefit from it.
While this currently is a possible treatment for some babies with hydrocephalus, Dr. Browd also thinks that over time they may be able to offer it to older children and adolescents.
“It opens up the possibility that a large portion of patients may be become shunt independent and that is an exciting new phase in the treatment of hydrocephalus,” said Dr. Browd. “We are thrilled about the technology and we think it will be great for patients and families.”
If you have medical questions regarding this procedure, please contact our neurosurgery team directly at 206-987-2544.
- For patients who require a shunt, Dr. Browd is working with Dr. Barry Lutz at the University of Washington to revolutionize the shunt and develop a new device that is failure-resistant and can be monitored remotely. Watch the video.
- Hydrocephalus research to improve patient care and cure the condition
- Hydrocephalus Research Guild
- Children’s Hydrocephalus Support Group
- Hydrocephalus Association