Doctors saved an unborn baby from certain death after performing the first brain surgery of its kind while the child was still in the womb.
A 10-person team at Boston Children’s Hospital and Massachusetts General Hospital performed the painstaking procedure, cutting into the baby’s uterus and skull and then operating on the developing brain.
The baby was diagnosed with Galen’s vein malformation at 30 weeks. Children born with the condition have a 30 percent chance of dying before age 11.
The condition — which affects one in 60,000 babies — occurs when arteries in the brain drain blood directly into veins instead of capillaries. This floods the heart with blood and can lead to dangerously high blood pressure.
For in-utero surgery, doctors cut into the mother’s uterus and operate on the unborn child. In the procedure performed in Boston, scientists used an ultrasound to find nerves that would be affected by a rare 60,000 birth defect called Galen’s vein malformation. Children born with this condition have a 30 percent chance of dying by age 11. In this case, the surgery was successful and the child was born days later with no heart or brain problems.
The surgery, performed by doctors in Boston, was performed by cutting into the pregnant woman’s abdomen and using an ultrasound to identify the artery and guide the operation.
The woman gave birth to a healthy child two days later and he was born with no birth defects. Researchers are working with the Food and Drug Administration (FDA) to conduct trials on the safety and effectiveness of this surgery, in hopes of expanding its use.
“The fetal intervention team at Boston Children’s Hospital and Brigham and Women’s Hospital have successfully devised another in utero procedure that has the potential to be very impactful,” said Dr. said.
The surgery was documented in a case study published Wednesday in the journal Stroke of the American Heart Association.
Doctors identified the malformation in a woman who was 34 weeks pregnant.
Using an MRI, they identified an overly wide falcine sinus – a curved vein in the brain that drains the artery. This tells surgeons there was a 99 percent chance the deformity would occur.
Doctors cut into the woman’s abdomen and exposed her uterus.
Then, using ultrasound to pinpoint the right spot, they cut into her uterine wall.
Surgeons then proceeded to cut into the unborn child’s brain and performed surgery to implant a piece of fat near the artery that would restrict blood flow.
“This approach has the potential to mark a paradigm shift in the management of Galen’s vein malformation,” said Dr. Darren Orbach, a surgeon at Boston Children’s Hospital.
‘We [repaired] preventing the pre-birth deformity and heart failure before it occurs, rather than trying to reverse it after birth.
“This could significantly reduce the risk of long-term brain damage, disability or death in these babies.”
After a successful operation, they initiated labor in the woman two days later. This is because the surgeries have ruptured the tissue around the baby in the womb that retains fluid – otherwise known as ‘rupturing the membranes’.
The child was born with limited complications at 4.2 pounds — light for a newborn.
Three weeks after birth, the infant did not require cardiovascular assistance. MRI scans also found no sign of abnormal blood flow in the brain.
“We were thrilled to see that the aggressive decline usually seen after birth just didn’t show up,” said Dr. Orbach.
“We are pleased to report that at six weeks the baby is progressing remarkably well, taking no medication, eating normally, gaining weight and is back home.
“There are no signs of negative effects on the brain.”
A 10-person team at Boston Children’s Hospital and Massachusetts General Hospital performed the painstaking procedure, cutting into the baby’s uterus and skull and then operating on the developing brain. Pictured: Ultrasound of a healthy baby in the womb
Dr. Darren Orbach (pictured), a surgeon at Boston Children’s Hospital, said: “This approach has the potential to mark a paradigm shift in treating Galen’s vein malformation.”
The condition occurs when the circulatory system does not form properly in the first trimester of pregnancy.
The anterior choroidal artery in the center of the brain drains blood to capillaries – tiny blood vessels that connect blocks to the veins.
They serve almost as speed bumps, controlling how fast blood flows from the arteries to the body’s venous network.
However, this system can be disrupted if the veins and capillaries do not develop properly in the uterus.
In children suffering from a deformity of the vein of Galen, the blood goes directly into the veins, bypassing the capillary.
This leads to the artery draining the blood too quickly into the circulation. In turn, a person’s blood pressure will rise sharply, as the increase in flow puts more pressure on the rest of the circulatory system.
Over time, this can restrict blood flow to the heart, lungs, and other vital organs, potentially leading to failure.
Elevated high blood pressure also dramatically increases the risk of heart disease.
A 2019 study by French researchers found that 36 percent of children with the condition had died by age 11.
Current treatment for the deformity involves a brain embolism, which uses fat to restrict blood flow and work as capillaries do.
However, surgeons believe they can act before the child is born to prevent complications while alive.
This surgery is the first in a clinical trial being conducted to gauge whether this surgery is an appropriate treatment for the condition. Doctors have received FDA approval for their work.
‘As always, a number of these fetal cases will need to be performed and monitored to establish a clear pattern of improvement in both neurological and cardiovascular outcomes,’ said Dr Satou.
“So the national clinical trial will be crucial to obtain sufficient data and hopefully successful results.”