The liver has several important jobs, including cleaning the blood and getting rid of toxins. So when a child’s liver becomes badly diseased or damaged, it may need to be replaced with a healthy liver (or a portion of one), so the body can function normally. Fortunately, pediatric liver transplants can be extremely safe and successful, and they can cure many cases of childhood liver failure. 

The most common reason for a pediatric liver transplant is biliary atresia, which is a rare disease affecting 1 in 8,000 to 1 in 18,000 live births worldwide. Inflammation forms around a child’s bile ducts — which usually allow digestive liquid to drain into the intestines — and the ducts become blocked. The liver becomes scarred and damaged when it cannot get rid of this fluid.

Biliary atresia is usually diagnosed when a child is about 5 to 6 weeks old, says Udeme Ekong, MD, MPH, medical director of the Pediatric Hepatology Program at Yale Medicine. Doctors aren’t sure what causes biliary atresia or whether it begins before or after birth. “What we do know is that the earlier a diagnosis is made, the better the outcome after surgery,” Ekong says.

Other types of liver disease in children include inherited genetic, metabolic, and autoimmune disorders like autoimmune hepatitis.

Children of any age can also experience sudden liver failure from several different causes. Despite numerous tests, however, doctors are often unable to determine why children experience liver failure.

Many babies are born with jaundice, a slight yellowing of the skin and eyes that indicates that their livers aren’t mature enough to remove bilirubin, a yellow-colored pigment, from their blood. But most of these babies get better on their own in a few weeks.

When babies have liver disease, however, their jaundice does not go away. Other signs of liver disease in infants can include dark urine, pale or clay-colored stools, a swollen abdomen, or weight loss. Young children may be irritable and cry more than usual, while older children may seem angry, drowsy, confused, or extremely tired much of the time.

Liver disease can also cause increased pressure within the blood circulation of the liver, called portal hypertension. “In this situation, the liver has a lot of scar tissue, which makes it very stiff and makes it difficult for blood to flow through it back to the heart,” says Dr. Ekong. This can lead to children vomiting blood or passing black-colored stools – signs of gastrointestinal bleeding. It can also lead to accumulation of fluid in the belly.

Liver disease is usually diagnosed through a series of blood tests, imaging tests, and a biopsy of the liver. Once doctors determine that a liver is failing and needs to be replaced, the child is referred to a transplant team.

At Yale Medicine, our transplant team consists of hepatologists, transplant surgeons, nurses, transplant pharmacists, dieticians, social workers, and more. “We also have other specialists such as cardiologists and infectious disease doctors assess the patient, as we want to be sure the patient’s heart can handle the transplant and that there aren't any infections or other health issues that would complicate the surgery,” says Dr. Ekong.  

The child’s family also meets with a social worker, who makes sure that they understand the care that will be needed after surgery. “Then, after every one of the specialists signs off and approves the patient for a transplant, we sit and discuss that patient’s individual case,” Dr. Ekong says.

The child is then put on a waiting list for a liver from a deceased donor. The waiting time for a liver varies, and a child’s place on the list is determined largely by how sick the child is and how urgently a new liver is needed.

Yale Medicine also offers living donor transplantation, and family members or others close to the child can be evaluated as potential donors. This can significantly shorten the patient’s wait time for a new liver.

If a living donor has been confirmed, a date is then set for the surgery. Otherwise, the family waits for a phone call that a deceased donor organ is available. During this waiting period, they will be given medicine and nutritional support to keep their child’s liver as healthy as possible until a transplant is available.

When a donor liver becomes available, the patient and the transplant team meet at the hospital for final tests. Once the patient is cleared for surgery, the transplant is performed as soon as possible.

A pediatric liver transplant takes about eight hours, says Dr. Ekong. The patient is put to sleep using general anesthesia, and an incision is made across the abdomen. The damaged liver is disconnected from the blood supply and gastrointestinal tract, removed from the body, and replaced with the new one. Blood vessels and bile ducts are reattached, so that blood and bile flow are restored through the new liver. The incision is then closed and stapled or stitched up.

Because children are smaller than adults, they usually only need a small portion of a donor liver, which quickly grows into a fully working organ. If a deceased donor liver is available, it may be used for one pediatric and one adult patient.

Because children need smaller portions of donated livers, adult-to-child living donations can be very successful, and very safe for the donor.

After surgery, transplant patients stay in the hospital for about 10 days. They spend the first few days in the pediatric intensive care unit, having their liver function and vital signs tested frequently. Then they are moved to the general pediatric ward for the remainder of their stay.

For the first few days, patients receive nutrients intravenously but can then begin drinking and eating solid food. The incision typically heals quickly, although a tube may be attached to the wound for a few days to drain away fluid.

Doctors will prescribe drugs to help manage pain after surgery, and medicines that help to prevent infection and facilitate the body’s acceptance of its new organ.

Liver transplant recipients usually need weekly check-ups for the first few months after surgery. The appointments then become more spread out – once a month and eventually just once or twice a year. “Lab tests, however, are done frequently, as this is the only way to know Dr. Ekong.  

Doctors also use these check-ups to make sure that patients are taking their medications. People who receive a liver transplant have to take several drugs every day for the rest of their lives to prevent their bodies from rejecting the organ.

Parents need to make sure that their children stick to this regimen, especially during big life changes, such as going away to college as a teenager, or switching doctors or insurance plans. Patients or their parents should call their doctor if their medication ever gets off schedule because a single missed dose can put transplant recipients at risk for a rejection episode, which may lead to organ failure and the need for another transplant.

“We make sure that families understand that this is a lifelong commitment,” Dr. Ekong says. “We are going to see these patients for pretty much all of their pediatric lives, and then transition them to an adult transplant hepatologist after that.”

The Yale New Haven Transplant Center is the only transplant center providing pediatric liver transplantation in Connecticut, Rhode Island, Vermont, and southern Massachusetts. Patients are also referred to Yale Medicine from across the country and the world.

Liver surgery is complex, says Dr Ekong. “You’re dealing with small blood vessels and smaller bile ducts,” she says. “It’s the type of procedure for which you want experienced surgeons who have done a large number of these types of surgeries, and we have that at Yale.”

The pediatric liver specialists at Yale Medicine also have the expertise to make diagnoses early and accurately, and to address diseases that respond well to treatment, says Dr. Ekong. Patients who need liver transplants are worked up for this as soon as possible, “so we start talking to families right off the bat about the option of living donors,” she says, “which we know can lead to the best possible outcomes.”