May 5, 2023 — In a first-of-its-kind operation, doctors have successfully repaired a brain malformation in a fetus still in the womb.
team from Boston Children’s Hospital and Brigham and Women’s Hospital Using ultrasound guidance, it causes excessively high blood flow, known as Galen malformation, which can lead to neurological and cardiac complications such as heart failure, severe brain damage, and death shortly after birth. I repaired the veins of things.
The surgery was performed on a 34-week fetus with amazing results. Since birth, the baby girl, identified as being at high risk of suffering serious complications of malformation while in utero, required no medications or further surgery to treat heart failure.
A repeat echocardiogram after the girl’s birth showed marked improvement in cardiac function, and an MRI of the brain showed no damage and a normal neurological examination.
“This is incredibly exciting. The hope is that this baby and others with this condition who will have this intrauterine surgery in the future will lead normal lives,” said lead researcher Darren B. Orbach. Doctor of Medicine said.
“I was thrilled that I didn’t see any of the rapid decline that is usually seen after birth,” he said. he came home There are no signs of adverse effects on the brain. ”
Galen malformation vein
Orbach said the Veins of Galen The malformation, which occurs in about 1 in every 60,000 births, is an abnormality in which arteries connect directly to veins, rather than the capillaries needed to slow blood flow and supply oxygen to the surrounding brain tissue.
“The arterial and venous systems are fundamentally very different. The arterial system has high pressure and high flow, whereas the venous system has low pressure and low flow. They should not be directly connected,” he said.
The galenic vein is the most extreme version of such an anomaly. It is known to significantly increase blood flow through the brain, which develops early in a baby’s formation and grows over time, and may double the work of the heart.
The placenta is thought to prevent this, as most babies do not have physiological problems in the womb, but they are in crisis after birth and the extremely high blood flow causes great stress on the heart.
Babies usually develop heart failure as the first major symptom soon after birth, Orbach said. “The anatomical problem is in the brain, but the clinical manifestation is high-output heart failure. It doesn’t work either, it’s a convenience feature.
“These newborns can be very sick. They need multiple medications to support their cardiovascular system, and they need to have procedures done to reduce blood flow,” he said. .
Brain injury is also a common problem. “The cerebral circulation is very abnormal. Blood is shunted through the malformation instead of circulating through the brain tissue,” Orbach said.
“Sick babies have a very high mortality rate (up to 90%) without specialist care. %, and mortality among surviving infants is at high risk for neurological and cognitive impairment.”
Current treatment for babies born with this condition includes a catheter inserted into the artery to close the deformity.
But Orbach said some babies were born too sick to have surgery. “Heart failure and brain damage are very serious and cannot be reversed by anything and usually these babies do not survive. It’s trying to help a baby who can’t. It’s the current postpartum approach,” he said.
Although other fetal surgeries are now routinely performed, this is believed to be the first intrauterine surgery targeting the cerebral vasculature.
“There were a lot of uncertainties,” says Orbach. “We didn’t even know if our instruments could be seen by ultrasound.” To model the procedure, the researchers had a phantom fetal skull and brain constructed with the veins of Galen’s malformation. rice field. This was key to gaining his FDA approval of the study.
If the study shows success in other patients, the technology could be expanded to other centers. We definitely need a capable fetal surgery and neurointervention team, but we are not the only center with these capabilities, yes other teams in specialty pediatric hospitals around the world can do this too. I hope,” he said.