A case of vein of Galen malformation has been successfully treated through brain surgery in the uterus, reducing the risk of life-long adverse outcomes.
The first successful in-utero brain surgery has been performed through the treatment of an aggressive vascular malformation known as vein of Galen malformation (VOGM), repairing a possibly fatal developmental condition.
Arteries in neonates carrying high-flow, high-pressure blood from the heart to the brain are meant to connect to capillaries which slow blood flow and deliver oxygen to surrounding brain tissue. In VOGM, these arteries instead connect to a main collecting vein at the brain’s base, which leads to significant pressure on an infant’s heart and lungs during and after birth.
Outcomes of VOGM include heart failure, pulmonary hypertension, and other life-threatening conditions. A prenatal ultrasound can be used to detect VOGM, with diagnosis by magnetic resonance imaging (MRI) occurring during the late second or third trimester.
In a recent study at Boston Children’s Hospital and Brigham and Women’s Hospital, investigators performed in-utero embolization on a fetus aged 34 weeks 2 days’ gestation. This process was accomplished through ultrasound-guided transuterine embolization, after which signs of aggressive decline seen in patients with VOGM after birth did not appear.
“We are pleased to report that at 6 weeks, the infant is progressing remarkably well, on no medications, eating normally, gaining weight, and is back home. There are no signs of any negative effects on the brain,” said lead study author Darren B. Orbach, MD, PhD, co-director of the Cerebrovascular Surgery & Interventions Center at Boston Children’s Hospital.
Delivery by induction of vaginal birth occurred 2 days following surgery because of premature rupture of membranes from in-utero embolization. Cardiac output showed progressive normalization during echocardiography after birth, and no cardiovascular support or surgery was needed following in-utero surgery.
During neonatal intensive care unit admission, strokes, fluid buildup,and hemorrhage were not found on the brain during MRI.
Orbach stated this approach could change management of VOGM, lowering the risk of long-term brain damage. VOGM is estimated to occur in up to 1 in 60,000 births and is currently treated through embolization, where direct artery-to-vein connections are closed to prevent excess blood flow.
Embolization is a high-risk procedure and does not reverse heart failure in every case. Severe brain damage has also already occurred in many cases of embolization, causing long-term cognitive disabilities and increased mortality risk. An in-utero approach may reduce these adverse outcomes.
“As always, a number of these fetal cases will need to be performed and followed in order to establish a clear pattern of improvement in both neurologic and cardiovascular outcomes,” said Gary M. Satou, MD, FAHA, the director of pediatric echocardiography at UCLA Mattel Children's Hospital. “Thus, the national clinical trial will be crucial in order to achieve adequate data and, hopefully, successful outcomes.”
In first in-utero brain surgery, doctors eliminated symptoms of dangerous condition. Newsroom. May 4, 2023. Accesed May 8, 2023. https://newsroom.heart.org/news/in-first-in-utero-brain-surgery-doctors-eliminated-symptoms-of-dangerous-condition