Hackensack Meridian Children’s Health Surgeon Performing Complex Kasai Procedure to Treat Rare Bile Duct Disorder at K. Hovnanian Children’s Hospital

The procedure was performed to treat a newborn with biliary atresia, a rare complex congenital condition that requires surgical treatment within the first weeks of life

Hackensack Meridian Children’s Health pediatric general surgeon Victoriya S. Staab, M.D.  at K. Hovnanian Children’s Hospital at Jersey Shore University Medical Center is performing operations to reconstruct the drainage to the liver called the Kasai procedure. This operation is used to treat a rare congenital condition called biliary atresia, a condition that if left untreated, will cause liver failure in newborns. A complex procedure such as this could only be done at a children’s hospital staffed with skilled pediatric surgeons and subspecialists.

Infant patients diagnosed with biliary atresia are often born with or develop an obstruction in their bile ducts. During the surgery, Dr. Staab carefully identifies the area of the blockage and then removes the area by performing a thorough dissection into the liver, so she could reconstruct the path of the liver’s drainage into the intestines.

“When a baby presents with signs of liver failure, the pediatric gastroenterology team and the pediatric surgery team work very closely together to do the work up in an expedited and thorough manner,” said Dr. Staab. “We then collaborate with our pediatric radiologist, pediatric anesthesiologists, and our neonatal and pediatric intensivists to have a thorough workup and diagnosis, a safe surgery, and a smooth recovery.”

This congenital condition occurs when the bile ducts inside or outside the liver do not develop normally. The bile duct is a tube that allows the passage of bile from the liver into the gallbladder and, eventually, the small intestine. It is not known why this blockage occurs in infants, but there are some known genetic and infectious causes. It is relatively rare, occurring in 1 out of every 10,000 live births. It occurs slightly more often in females than in males (1.4:1), and affects children of all races. In the United States, approximately 300 new cases of biliary atresia in infants are diagnosed each year.

Symptoms typically appear within a few weeks after birth due to an abnormal accumulation of bile in the liver and include:

• Yellowing of the skin and whites of the eyes, called jaundice, that lasts more than a few weeks
• Dark yellow or brown urine
• Pale or clay-colored stools
• Enlarged liver and spleen
• Poor weight gain

“Bile flow can be re-established in approximately 80 percent of infants who undergo the Kasai procedure,” said Dr. Staab. “We are able to achieve the best results when the condition is diagnosed early and we perform the procedure during the first eight weeks of life.”

“Our pediatric surgeons are equipped to deliver advanced surgical care for newborns with biliary atresia, and through our collaborative care network, these children also have access to hepatologists and gastroenterologists who can help them manage their condition as they grow,” said Harpreet Pall, M.D., chair of Pediatrics at K. Hovnanian Children’s Hospital, and academic chair of Pediatrics at Hackensack Meridian School of Medicine.