Hospital in Barcelona avoids liver transplants in four children with innovative operation

The Abernethy deformity affects one out of 30,000 children and causes severe complications

Edu playing with his toys (by Laura Busquets)
Edu playing with his toys (by Laura Busquets) / ACN

ACN | Barcelona

June 15, 2017 05:34 PM

Barcelona’s Vall d’Hebron Hospital successfully avoided liver transplants for four children, who suffer from the Abernethy deformity—a congenital malformation that affect one out 30,000 children—by using a brand new technology for the first time in Spain. The children’s only alternative before was to wait for the disease to cause them brain, lung, or tumor complications, and then to opt for a liver transplant with all of its associated difficulties. Instead, the center successfully tested a surgical intervention that “cures the disease without replacing the organ” according to Javier Bueno, the head of the Hepatic Surgery unit in the Pediatric Surgery Department.

The Abernethy deformity is a congenital anatomic abnormality which keeps the blood in the portal vein—which transports the blood to the liver for purification—from getting to the liver. Instead that blood goes to the vena cava, where it is not purified and thus, the toxins get spread around the body. This can produce severe complications. Bueno explained that these can affect the brain, and therefore, it can mean mental delays in the children, lung problems, or hemodynamic changes, which end up provoking tumors that can become malignant.

To date, the children with this malformation were not operated on until the complications were obvious. Interventional radiology was used, which is a technique in which the Vall d’Hebron Hospital is also a pioneer. It consisted of introducing devices in order to close the communication between the two veins, but this process could not be carried out if the connection was too big, as the device could migrate to the heart. The second option was a liver transplant, a risky operation, which, in addition, forced the patients to take medication for the rest of their lives.