Patient Presentation
A 17-year-old female came to clinic for her health maintenance visit. She had a history of multiple gastrointestinal complaints throughout her lifetime including slow-transit constipation and gastroesophageal reflux disease. For the past 2 years she had increased right upper quadrant and epigastric pain that would occur at random intervals and was severe enough to disrupt her activities and which precipitated emergency room evaluation one time. Her gastroenterologist also evaluated her for gallbladder disease which had shown no gallstones, but had a slow gallbladder ejection fraction. She had been referred to a surgeon who agreed with the diagnosis of biliary dyskinesia and had performed a cholecystectomy 5 months previously. She reported that most of her symptoms had abated but that she still had intermittent similar pain but much less frequently and with less intensity.

The pertinent physical exam revealed normal vital signs and she had a BMI of 31.2. Her abdomen examination had well-healed laparoscopic incisions and was otherwise normal. The diagnosis of a healthy female with obesity who was status-post laparoscopic cholecystectomy was made. She was encouraged to followup with her gastroenterologist as planned.

Discussion
Biliary disease includes gallstones, cholecystitis (inflammation of the gallbladder), cholangiopathy and cholangitis (pathology of the bile ducts), biliary dyskinesia, gangrene, and cancer. Some are often overlapping as gallstones often contributing to cholecystitis and cholangitis.

Biliary dyskinesia (BD) is well-recognized in adults. Adult criteria include: “abdominal pain located in the epigastrium and/or right upper quadrant along with; buildup of pain to a steady level and lasting 30 minutes or longer, pain occurring at different intervals and not daily, severe enough to interrupt activities or lead to an emergency department visit, the pain is not significantly (<20%) related to bowel movements, and not significantly (<20%) relieved by postural change or acid suppression.” There is not a similar BD definition for the pediatric population, making research to improve outcomes and treatment decisions more difficult. According to the US National Library of Medicine BD is “[a] motility disorder characterized by biliary [colic], absence of [gallstones], and an abnormal [gallbladder] ejection fraction. It is caused by gallbladder dyskinesia and/or [sphincter of Oddi dysfunction].” Criteria often used in the pediatric population includes “chronic or recurrent epigastric or right upper quadrant pain or other discomfort, absent gallstones and abnormal [gallbladder ejection fraction]…” on cholecystokinin-cholescintigraphy.

Learning Point
Most cholecystectomies (~95%) are performed laparoscopically as opposed to an open procedure. Overall there has been an increase in cholecystectomies in the US for all indications and also for BD. Approximately 10% of all pediatric cholecystectomies were for BD and ~73% were for calculous cholecystitis between 2002-2011. Cholecystectomy increases are related at least partly to increasing gallstone disease in children mainly due to the pediatric obesity epidemic. Cholecystectomies overall and for BD occur more often in teenagers, females and those that are obese.

In a pediatric systematic review, outcomes after cholecystectomy for BD showed 34-100% (average ~66%) symptomatic symptom success in the short term, but longer term (1-2 years) many symptoms recurred. There are probably many reasons for this including the actual definition of BD used, individual patient symptoms, placebo effect of surgery and alternative diagnoses. One example is delayed emptying of the gallbladder (as noted above is used for BD diagnosis) can be caused by other problems including obesity, constipation, gastroesophageal reflux, allergies and parasitic infection in children. Alternative diagnoses are possible as some patients (adults and pediatric) have other functional gastrointestinal disease or other disease diagnosed often within 1-2 years after cholecystectomy. Functional dyspepsia is one example with many symptoms that overlap with BD including “post-prandial fullness, early satiety, [and] epigastric pain or burning not associated with defecation.” Other alternatives include Crohn’s syndrome, cyclic vomiting syndrome, hiatal hernia and irritable bowel syndrome. One author concluded, “…almost 34% of patients will have persistent symptoms, whereas 50% will be diagnosed with another disorder soon after cholecystectomy, making the BD diagnosis doubtful.” There is other data supporting improved long term cholecystectomy outcomes for patients with pre-operative post-prandial pain and those with lower gallbladder ejection fractions (i.e. <15%).

Questions for Further Discussion
1. What are the common gallstones made of? A review can be found here
2. What are the functions of the liver? A review can be found here
3. How is a cholecystokinin-cholescintigraphy test performed?

Related Cases

Disease: Biliary Dyskinesia | Gallbladder Diseases | Bile Duct Diseases

Symptom/Presentation: Abdominal Pain

Specialty: Gastroenterology | Surgery

Age: Teenager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Gallbladder Diseases and Bile Duct Diseases.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Biliary Dyskinesia. MeSH Browser. Accessed November 8, 2022. https://meshb-prev.nlm.nih.gov/record/ui?ui=D001657

Santucci NR, Hyman PE, Harmon CM, Schiavo JH, Hussain SZ. Biliary Dyskinesia in Children: A Systematic Review. J Pediatr Gastroenterol Nutr. 2017;64(2):186-193. doi:10.1097/MPG.0000000000001357

Matta SR, Kovacic K, Yan K, Simpson P, Sood MR. Trends of Cholecystectomies for Presumed Biliary Dyskinesia in Children in the United States. J Pediatr Gastroenterol Nutr. 2018;66(5):808-810. doi:10.1097/MPG.0000000000001777

Liebe HL, Phillips R, Handley M, Gastanaduy M, Burton JH, Roybal J. A pediatric surgeon’s dilemma: does cholecystectomy improve symptoms of biliary dyskinesia? Pediatr Surg Int. 2021;37(9):1251-1257. doi:10.1007/s00383-021-04922-1

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa