Doctors performed a unique surgical procedure to repair a potentially fatal brain abnormality in a fetus to prevent injury and heart failure after birth.
The procedure described recently in the journal Stroke, repaired a potentially fatal developmental disorder called Vein of Galen Malformation (VOGM) in the fetus’ brain.
Aggressive blood vessel malformation is a rare condition in which arteries that bring high-pressure blood from the heart to the brain connect directly to veins deep at the base of the brain, rather than to the capillaries that slow blood flow and deliver oxygen to surrounding brain tissue .
It’s the most common congenital vascular brain malformation, occurring in up to 1 in 60,000 births, and seriously affecting the heart and brain of newborns after birth, researchers including those from Boston Children’s Hospital in the US said.
The condition, which is first seen on an ultrasound, takes a tremendous toll on the baby’s life and can lead to heart failure or other potentially life-threatening conditions in the newborn.
A current treatment method involves a catheter-based postpartum procedure called embolization. It occludes the direct artery-to-vein connections in the malformation to block excessive blood flow to the brain and heart.
However, scientists said this procedure carries a high risk and may not always be successful in reversing heart failure.
They said severe brain damage could have occurred before the baby was born, which could result in lifelong cognitive impairment and life-threatening conditions for the child, or even death.
In the ongoing clinical trial, researchers used ultrasound to target VOGM before birth.
“We were thrilled to see that the aggressive decline normally seen after birth just didn’t occur. We are pleased to report that at six weeks the infant is doing remarkably well, is off medication, eating normally, gaining weight and is home. There is no evidence of adverse effects on the brain,” said study author Darren B. Orbach.
The infant was delivered by induction of vaginal labor two days later, when scans showed progressive normalization of cardiac output.
Researchers said the newborn was observed in the NICU for several weeks before being sent home.
During this time, the infant had a normal neurological examination and showed no seizures, fluid accumulation, or bleeding on brain MRI scans.
“Although this is only our first patient treated and it is vital that we continue the study to evaluate safety and efficacy in other patients, this approach has the potential to usher in a paradigm shift in the treatment of vein of galen malformation where we repair the deformity before birth and prevent the heart failure before it occurs,” said Dr. Orbach.
“This can significantly reduce the risk of long-term brain damage, disability, or death in these infants,” he said.
