After an abdominal mass was found on prenatal scans, a female newborn was referred to Duke Children’s Hospital, undergoing surgery at just three days of age. The surgery identified and resected a benign infantile myofibroma. Despite the operation’s success, the infant soon developed jaundice and cholestasis of unclear etiology.
Although jaundice is common for newborns, pediatric transplant hepatologist Gillian O. Noel, MD, MSc, was concerned. “If there’s ongoing jaundice after two weeks of age, even if the infant is exclusively breastfed, you have to look into other causes,” Noel says. “The initial testing is to fractionate the bilirubin, and if the conjugated bilirubin is abnormal, infants should be seen by a liver specialist.”
After an extensive work-up, imaging and an endoscopy indicated that the child had an anatomical anomaly causing a significant stricture or blind end to her biliary system, preventing it from draining into the intestine.
With both of the country’s only infant-sized endoscopic retrograde cholangiopancreatograms (ECRP) in use on the West Coast, the Duke team opted for surgery, which verified the biliary abnormality as well as significant scarring in the liver. Postsurgery, the child improved initially but then began experiencing a succession of symptoms. Her liver scarring caused abdominal swelling, severe itching, and stool changes, with no signs of improvement.
How did Noel and the Duke team decide to treat the patient?
Ultimately, the infant’s poor growth and weight loss was the deciding factor. Noel determined the patient’s malnutrition and failure to thrive likely represented worsening liver disease. “Poor growth in a pediatric patient with advanced liver disease is an indication for transplant,” Noel says. “Many people think it’s only about liver dysfunction — not being able to create factors or albumin — but a liver that’s having to work too hard to maintain normal functions, not allowing the baby to grow, is also an indication for transplant.”
The patient was evaluated and listed for liver transplant at Duke. Infant-sized organs are rare, and the wait could have been much longer, but pediatric liver transplant surgeon Deepak Vikraman, MD, was able to use a segmental graft, taking a left lateral liver segment from a deceased donor and implanting it in the infant at the age of 16 months. “Not all centers will do segmental grafts on infants,” Noel says. In the process, the team also created a roux, using part of her intestine to act as a bile duct and provide adequate drainage.
The surgery was successful, and the infant recovered well. Seven months after surgery, the toddler continues to develop well, with resolution of itching and excellent growth.
Specialized, multidisciplinary care from birth helped to ensure timely treatment. Along with Mansi Amin, MD, Noel is one of two pediatric liver specialists who are always on call at Duke, with the team slated to grow soon. “We round on every patient with our surgeons, infectious disease doctors, dieticians, nurses, nurse practitioners, and social workers who all specialize in transplant,” says Noel. “We consider not only the medical aspects but also surgical and social aspects to best support the patient. Because the percent of patients under four that we transplant at Duke is higher than at the majority of centers, we have the expertise to offer advanced treatments for young children.”