In neonatal patients with congenital cholestasis and liver disease, liver biopsies alone often cannot rule out biliary atresia, thus requiring a second invasive intraoperative cholangiogram and additional round of anesthesia to exclude the condition. According to the authors of Featured Abstract 194, “Percutaneous Cholecystocholangiography for Early Exclusion of Biliary Atresia: A Single-Center 5-Year Experience” (which will be presented Tuesday, March 7, 3:27 p.m., Phoenix Convention Center, Room 224AB), percutaneous cholangiogram is a safe and effective minimally invasive method for excluding biliary atresia at the time of biopsy.
“Many times, despite adequate biopsies, a pathologist cannot rule out biliary atresia,” said presenter Rachel Todd, a medical student at the Icahn School of Medicine at Mount Sinai. “After collaborating with pediatric hepatology and liver surgery, we realized that we could potentially help streamline the process of evaluating for biliary atresia by cutting out the need for an intraoperative cholangiogram in patients whose liver disease is clearly not caused by biliary atresia based on what we could see from our procedure.”
Early diagnosis is crucial in patients with biliary atresia. If left untreated, the condition quickly leads to fibrosis and ultimately portal hypertension and end-stage liver disease.1
In this single-center, retrospective study of 22 patients from December 2017 to August 2022, 20 infants under 100 days old with suspected biliary atresia underwent percutaneous cholangiogram (PCC). Ultrasound or fluoroscopic-guided liver biopsies were performed at the same time, and biliary atresia was ruled out when contrast material was visualized in the gallbladder, biliary tree and duodenum. Two patients did not undergo PCC due to a lack of visualization of the gallbladder.
In the 20 patients who underwent PCC, technical success was achieved in 16, and biliary atresia was ruled out in 14. Patients with excluded biliary atresia were diagnosed with neonatal hepatitis (6), bile duct paucity (5), A1AT deficiency (1), total parenteral nutrition-associated cholestasis (1) and CMV hepatitis (1). The four patients who underwent failed PCC were diagnosed with biliary atresia (1), vanishing bile duct (1), neonatal hepatitis (1) and Alagille syndrome (1).
PCC gives physicians a minimally invasive method for excluding biliary atresia so that additional surgery can be avoided if possible.
“From the surgeon’s perspective, I think it’s reassuring to know that the operation and stress that comes along with it are all the more warranted for the patients for whom percutaneous cholecystocholangiography didn’t rule out biliary atresia,” Ms. Todd said.
Having a streamlined approach also lessens the time to exclusion of biliary atresia.
“For families of these children, the process of either being diagnosed with biliary atresia or having it ruled out is a very stressful time with a lot of waiting and worrying,” Ms. Todd said. “I think that using percutaneous cholecystocholangiography to quickly rule out biliary atresia helps cut down on that stressful time because in the case of rule-outs, we can give them the news without waiting for the diagnosis from pathology results or a second open cholangiogram. In some places, that can mean knowing the same day versus waiting for multiple days.”
The researchers aimed to demonstrate that percutaneous cholangiograms can be performed even at institutions without pediatric IRs.
“In speaking with our team, we realized that even in dedicated pediatric hospitals, PCs were not being performed routinely in these patients,” Ms. Todd said. “We want to show that even in institutions such as ours, without dedicated pediatric IRs, we can still perform PCs safely in most patients.”
Looking ahead, the researchers will continue to monitor the patients who underwent PCC.
“At this time, we are continuing to follow these patients and track how they are doing in the months and years following our intervention,” Ms. Todd said. “Overall, I think we know as a field that many of our procedures have a multitude of potential applications that can improve the patient experience, and we wanted to explore one of those possibilities.”
References
- Vij M, Rela M. Biliary atresia: pathology, etiology and pathogenesis. Future Sci OA. 2020 Mar 17;6(5):FSO466. doi: 10.2144/fsoa-2019-0153.
