Surgical Case Study: Complex Cholodochal Cyst in 15-Year-Old

Physicians:

• Brad Warner, MD, surgeon-in-chief, St. Louis Children’s Hospital (SLCH)
• William Hawkins, MD, hepatobiliary pancreatic and gastroenterology surgeon, Barnes-Jewish Hospital (BJH)
• Shawn Larson, MD, pediatric surgery fellow, SLCH

Background: The patient, 15-year-old Amanda, had been experiencing severe abdominal pain, to the point of twice visiting a hospital emergency room in her hometown of Springfield, Ill. According to her mother, Lisa, Amanda had frequent stomach problems throughout her life; even as an infant, Lisa described her daughter as being a “puker.”

“Amanda’s stomach pain began getting out of control. We didn’t know that she’d already been diagnosed as having a cholodochal cyst,” says Lisa. “It was identified on CT scans done when Amanda had her appendix removed at age 14. Unfortunately, we weren’t told about that discovery.”

Amanda was seen by a local surgeon, who performed an endoscopic retrograde cholangiopancreatography (ERCP). The study showed Amanda had a large cyst located within the head of her pancreas. In an attempt to alleviate Amanda’s pain, the surgeon placed a stent within the pancreas as a drain. When another study showed the cyst hadn’t changed, Lisa contacted Dr. Warner at St. Louis Children’s Hospital.

The surgical challenge: “Usually cholodochal cysts are diagnosed in infants and young children when they present with jaundice,” says Dr. Warner. “However, most of these cysts involve the bile duct above the pancreas. In Amanda’s case, her cyst was actually within the pancreas, which is probably the reason her symptom was stomach pain rather than jaundice. No matter the position of these cysts, however, we remove them because these patients are at risk for developing pancreatitis and, over time, cancer in the bile duct.”

The location of Amanda’s cyst meant she needed a more complex surgery than usual to remove it. Dr. Warner first considered a Whipple procedure, which most often is the treatment for pancreatic cancer. It involves removing the head of the pancreas, the duodenum and part of the bile duct.

“The Whipple is associated with a significant complication rate and long-term consequences in young teenagers,” says Dr. Warner. “Early on there’s a risk of leakage from the point where the intestine and what remains of the pancreas are sewn together. Long term, there’s the risk of malnutrition because patients are unable to gain weight and grow well. In addition, the blood supply to the stomach and pylorus is sometimes disturbed, resulting in problems with the stomach emptying.”

He adds, “It was obvious a Whipple procedure was not the ideal surgery for Amanda, which led me to consult with my surgical colleagues at Barnes-Jewish Hospital. I found they had experience with these more-complex cholodochal cysts in the adult population. Fortunately, our mutual affiliation with Washington University School of Medicine makes this type of collaboration possible.”

The surgical approach: Drs. Warner and Hawkins’ alternative surgical approach was to identify the bile duct above the pancreas, cut across it, and then, with traction on the lower bile duct, dissect close to the bile duct wall and ultimately identify the cyst. They removed the cyst within the pancreas gland without cutting across any pancreas tissue but rather with traction on the cyst and gentle dissection.

“We left open the upper part of the bile duct coming from the liver and removed the lower part all the way down to where it joined the pancreas duct. We oversewed it there,” explains Dr. Warner. “To replace the missing segment of bile duct within the pancreas, we brought up a loop of intestine and sewed it to the bile duct coming out of the liver. Now instead of the bile going through the bile duct and pancreas, into the cyst and then into the duodenum, the bile goes out of the liver into the upper bile duct and then drains directly into a piece of small intestine.”

The outcome: Approximately six months after the surgery, Amanda is doing well, with testing showing normal pancreas and liver enzymes. Other than periodic check-ups, she does not require medications or any other ongoing treatment.

“Amanda was a great patient—really upbeat, and she worked hard at getting better after the surgery,” says Dr. Warner. “The outcome was significantly better than may have been possible following a Whipple procedure. It was gratifying to offer her and her family a more effective, less invasive surgical solution thanks to the combined expertise of pediatric and adult surgeons.”