Pioneering neurosurgery helps babies with hydrocephalus

Calgary only centre west of Toronto offering minimally invasive procedure

CALGARY – Alberta Children's Hospital in Calgary is the only centre in Western Canada that offers an innovative surgical procedure to treat babies with hydrocephalus, which is a buildup of fluid in the brain.

Performed by neurosurgeon Dr. Jay Riva-Cambrin, the minimally invasive procedure involves creating new channels for the cerebrospinal fluid to drain and circulate normally, and then eliminating some of the tissue that creates the excess fluid.

Standard practice is to place an internal shunt, or tube, to drain the fluid into the abdominal cavity, where it is reabsorbed by the body. However, infection is a common risk with shunts. People with shunts sometimes face multiple neurosurgeries over their lifetimes to repair the problems that can arise.

“Right now, this is one of the hottest topics in pediatric neurosurgery,” says Dr. Riva-Cambrin, who brought the technique to Calgary last year after receiving training from surgeons in Mbale, Uganda, and Boston Children’s Hospital. “The quality of life for these children is substantially improved when we can eliminate the risks of infection and the need for implanted hardware. It will be less likely that they’ll have to have more brain surgeries as they grow up.”

The full name of the procedure is endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC). During the operation, the surgeon creates a small opening in the skull, then uses a tiny camera to guide miniature surgical tools to create a bypass for the blocked cerebrospinal fluid. The second part of the procedure involves cauterizing and thereby disabling some of the choroid plexus, a type of tissue that creates much of the cerebrospinal fluid.

About two-thirds of infants with hydrocephalus are eligible for the procedure, according to
Dr. Riva-Cambrin. Generally they must be under 24 months of age and have an MRI that confirms there is minimal scarring in the exit passageway.

About 50 to 60 babies are treated for hydrocephalus in Alberta every year; about half of those are from the Calgary area. Without treatment, pressure from accumulating fluid inside the brain and skull is eventually fatal.

Dr. Riva-Cambrin can perform the ETV/CPC procedure on eligible patients from Alberta, eastern B.C. and western Saskatchewan. He is currently working with colleagues in Edmonton to introduce the procedure there.

Lauren and Jamie Plessis’s daughter, Baya, developed an infection at birth that required three weeks of treatment in hospital before she could go home. Five months later, a routine checkup revealed her skull was growing too rapidly.

“We saw a pediatrician, who immediately told us there was likely pressure building up because of hydrocephalus,” Lauren says. “Baya went in for an MRI right away and then,
a week later, she was in surgery. She’s been stronger than all of us throughout this.”

Because she was still under 24 months of age, Baya was a good candidate for the ETV/CPC procedure. Both parents are relieved she won’t have a permanent shunt.

“Dr. Riva-Cambrin is a hero to us,” says Baya’s dad Jamie. “He gave us our baby back.”

In Baya’s case, she may have developed bleeding in her brain, then a blood clot, and then scar tissue that could have impeded the flow of the cerebrospinal fluid.

A child with a shunt will need to be monitored yearly. Although they can otherwise lead normal lives, it’s not unusual that they will require anywhere from two to 30 surgeries to replace the shunt, Dr. Riva-Cambrin says. By comparison, the ETV/CPC procedure eliminates the risk of infection and hardware failure, but can still require additional surgery in about one-third of cases due to the body closing over the hole made by the surgeon.

Causes of hydrocephalus include meningitis and spontaneous bleeding. It’s also associated with pre-term birth.

Only two neurosurgeons in Canada have been trained in the procedure, according to
Dr. Riva-Cambrin. It was developed by Dr. Benjamin Warf as a solution for children in Africa, where medical supports aren’t ideal for the lifelong maintenance of shunts. Dr. Warf then brought the procedure to Boston Children’s Hospital.

Dr. Riva-Cambrin performed about 70 of the procedures in Salt Lake City before moving to Calgary, where he has performed seven since arriving last September.

The University of Calgary and the Alberta Children’s Hospital are currently taking part in many studies examining the effects of the ETV/CPC procedure, and further evaluating who make the best candidates. Dr. Riva-Cambrin is also actively involved in developing endoscopy programs and research projects in both Uganda and Mali in Africa.

Alberta Health Services is the provincial health authority responsible for planning and delivering health supports and services for more than four million adults and children living in Alberta. Its mission is to provide a patient-focused, quality health system that is accessible and sustainable for all Albertans.

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