The diagnosis and management of biliary dyskinesia in children and adolescents remains variable and controversial. The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee (APSA OEBP) performed a systematic review of the literature to develop evidence-based recommendations.

Through an iterative process, the membership of the APSA OEBP developed five a priori questions focused on diagnostic criteria, indications for cholecystectomy, short and long-term outcomes, predictors of success/benefit, and outcomes of medical management. A systematic review was conducted, and articles were selected for review following Preferred Reporting Items for Systematic Review and Meta-analyses (PRISMA) guidelines. Risk of bias was assessed using Methodologic Index for Non-Randomized Studies (MINORS) criteria. The Oxford Levels of Evidence and Grades of Recommendation were utilized.

The diagnostic criteria for biliary dyskinesia in children and adolescents are not clearly defined. Cholecystectomy may provide long-term partial or complete relief in some patients; however, there are no reliable predictors of symptom relief. Some patients may experience resolution of symptoms with non-operative management.

Pediatric biliary dyskinesia remains an ill-defined clinical entity. Pediatric-specific guidelines are necessary to better characterize the condition, guide work-up, and provide management recommendations. Prospective studies are necessary to more reliably identify patients who may benefit from cholecystectomy.

Level 3-4.

Systematic Review of Level 3-4 Studies.

Biliary dyskinesia is a disorder characterized by biliary pain, a sonographically normal gallbladder, and a reduced gallbladder ejection fraction on cholecystokinin-cholescintigraphy (CCK-HIDA) scan. Laparoscopic cholecystectomy remains a common treatment for biliary dyskinesia despite a lack of high-quality evidence supporting the practice. The following review summarizes the current biliary dyskinesia outcomes data, the diagnostic strategies and their limitations, biliary dyskinesia in the pediatric population, the emerging phenomenon of the hyperkinetic gallbladder, and suggestions for addressing identified knowledge gaps.

The majority of studies on the topic are retrospective, with wide variations in inclusion criteria and definition of biliary pain. Most report a very short follow-up interval, often a single office visit, with variable and nonstandardized definitions of a satisfactory outcome. Despite a published Society of Nuclear Medicine guideline for its performance, CCK-HIDA scan protocols vary among institutions, which has led to considerable variability in the consistency and reproducibility of CCK-HIDA results. The few prospective studies available, although small and heterogeneous, support a role for cholecystectomy in the treatment of adult biliary dyskinesia. Despite these knowledge gaps, biliary dyskinesia is now the number 1 indication for cholecystectomy in children. Cholecystectomy for the hyperkinetic gallbladder appears to be an emerging phenomenon, despite, as in biliary dyskinesia, a lack of quality data supporting this practice. Randomized trials addressing these gaps are needed but have been difficult to conduct owing to strong clinician and patient bias toward surgery and the lack of a criterion-standard nonsurgical treatment for the control arm.

The use of cholecystectomy for adult biliary dyskinesia is reasonable based on the available data. Insufficient data exist regarding laparoscopic cholecystectomy for pediatric dyskinesia and the hyperkinetic gallbladder population. Large-scale prospective studies, either randomized trials or large prospectively followed cohort studies, are needed to address the knowledge gaps surrounding this controversial diagnosis.

In patients with biliary colic, high-quality prospective data supporting the precise timing of cholecystectomy are lacking. The purpose of this study was to determine the effectiveness of early laparoscopic cholecystectomy in children with biliary colic.

A multicenter, parallel-group, randomized study was conducted in patients with biliary colic at 5 hospitals in China. Pediatric patients with biliary colic were prospectively randomized to either the early cholecystectomy or conservative management strategy. The clinical outcomes within 6 months, including the number of biliary colic-free patients and gallstone-related complications, were compared (register number ChiCTR1900021830).

During the first 2 months of follow-up, 71 patients (59.2%, 71/120) receiving conservative management and 124 patients (97.6%, 124/127) in the early cholecystectomy group (p < 0.001) reported being entirely colic-free. The GIQLI measures were higher in the early cholecystectomy group than in the conservative management group (p = 0.032). Acute readmissions occurred in 7 (5.5%) of 127 patients in the early cholecystectomy group, compared with 23 (19.2%) of 120 patients in the conservative management group (risk ratio [RR] 0.25; 95% CI [0.10-0.60], p = 0.001) in the 6-month period.

Early cholecystectomy is effective in providing beneficial outcomes in terms of both short-term and long-term improvement of symptoms.

Introduction: While functional gallbladder disorder is a well-recognized and defined condition in adults, its pediatric analog, biliary dyskinesia, lacks uniformity in diagnosis. Yet, biliary dyskinesia is among the most common conditions resulting in cholecystectomy in youth and its frequency continues to rise. The primary aims of the current review were assess the efficacy of cholecystectomy in children diagnosed with biliary dyskinesia and the utility of cholescintigraphy in predicting outcomes. Results: All previous studies assessing outcomes in youth with biliary dyskinesia have been retrospective and therefore of low quality. There is a lack of uniformity in patient selection. Short term follow-up reveals partial response in 63.4-100% with complete resolution in 44.2-100%. Only 4 studies have reported long-term outcomes with complete symptom resolution in 44-60.7%. The published research generally indicates that the gallbladder ejection fraction (GBEF) as determined by cholescintigraphy lacks utility in predicting cholecystectomy outcome utilizing the commonly used cut-off values. There are data suggesting that more extreme cut-off values may improve the predictive value of GBEF. Conclusion: There is a lack of consensus on the symptom profile defining biliary dyskinesia in youth and current literature does not support the use of cholescintigraphy to select patients for cholecystectomy. There is a substantial portion of pediatric patients diagnosed with biliary dyskinesia who do not experience long-term benefit from cholecystectomy. Well-designed prospective studies of surgical outcomes are lacking. Increasing the uniformity in patient selection, including both symptom profiles and cholescintigraphy results, will be key in understanding the utility of cholecystectomy for this condition.

Biliary dyskinesia (BD) is a common indication for pediatric cholecystectomy. While diagnosis is primarily based on diminished gallbladder ejection fraction (GB-EF), work-up and management in pediatrics is controversial.

We conducted a multi-institutional retrospective review of children undergoing cholecystectomy for BD to compare perioperative work-up and outcomes.

Six hundred seventy-eight patients across 16 institutions were included. There was no significant difference in gender, age, or BMI between institutions. Most patients were white (86.3%), non-Hispanic (79.9%), and had private insurance (55.2%). Gallbladder ejection fraction (EF) was reported in 84.5% of patients, and 44.8% had an EF <15%. 30.7% of patients were initially seen by pediatric surgeons, 31.3% by pediatric gastroenterologists, and 23.4% by the emergency department with significant variability between institutions (p < 0.001). Symptoms persisted in 35.3% of patients post-operatively with a median follow-up of 21 days (IQR 13, 34). On multivariate analysis, only non-white race and the presence of psychiatric comorbidities were associated with increased risk of post-operative symptoms.

There is significant variability in evaluation and follow-up both before and after cholecystectomy for BD. Prospective research with standardized data collection and follow-up is needed to develop and validate optimal care pathways for pediatric patients with suspected BD.

Case Series, Retrospective Review.

Level IV.

Chronic acalculous cholecystitis (CAC) increasingly is being diagnosed as a cause of recurring biliary symptoms in children, but its clinical diagnosis remains challenging. The primary objective was to evaluate the utility of hepatocholescintigraphy in pediatric patients with suspected CAC. A secondary objective was to describe their clinical follow-up after diagnosis.

Medical records of patients (aged 9-20 years) who underwent hepatocholescintigraphy from February 2008 to January 2012 were reviewed. Patients with gallstones, and with ≤1 year of clinical follow-up, and studies without gallbladder (GB) stimulation were excluded. GB ejection fraction (GBEF) of <35% after sincalide or fatty meal (Lipomul) stimulation were considered abnormal. Diagnosis of CAC was based on histopathology after cholecystectomy. Patients with negative GB pathology, or complete resolution of symptoms without surgery, or alternative diagnoses for persistent symptoms were considered to not have CAC.

Eighty-three patients formed the study group (median age 14.9 years), of which 81.9% were girls. Median duration of symptoms and clinical follow-up were 6 months and 2.9 years, respectively. Fifty-two patients had at least 1 study with sincalide and 36 patients had at least 1 study with Lipomul. Initial cholescintigraphy was 95.0% sensitive and 73.0% specific in diagnosing CAC, with a negative predictive value of 97.9%. Of the 31 patients with abnormal GBEF, 22 underwent cholecystectomy with improvement in pain in 72.7%, whereas all of the 9 without surgery improved.

Hepatocholescintigraphy is useful for excluding CAC, although the clinical implications of an abnormal GBEF need to be further defined.

The purpose of this study is to describe a cohort of pediatric patients undergoing cholecystectomy for biliary dyskinesia (BD) and characterize postoperative resource utilization.

Single-institution, retrospective chart review of pediatric patients after cholecystectomy for BD was done. Patient demographics and clinical characteristics as well as operative details and postoperative interventions were abstracted. Telephone follow-up was performed to identify persistent symptoms, characterize the patient experience, and quantify postoperative resource utilization.

Forty-nine patients were included. Twenty-two patients (45%) were seen postoperatively by a gastroenterologist, of which, only 32% were known to the gastroenterologist before surgery. Postoperative studies included 13 abdominal ultrasounds for persistent pain, 13 esophagogastroduodenoscopies, five endoscopic retrograde cholangiopancreatographies (ERCPs), one endoscopic ultrasound, one magnetic resonance cholangiopancreaticogram, and five colonoscopies. Of the patients with additional diagnostic testing postoperatively, one had mild esophagitis, three had sphincter of Oddi dysfunction, and one was suspected to have inflammatory bowel disease. Telephone survey response rate was 47%. Among respondents, 65.2% reported ongoing abdominal pain, nausea, or vomiting at an average of 26 mo after operation. Of note, all patients who underwent postoperative ERCP with sphincterotomy reported symptom relief following this procedure.

Relief of symptoms postoperatively in pediatric patients with BD is inconsistent. Postoperative studies, though numerous, are of low diagnostic yield and generate high costs. These findings suggest that the initial diagnostic criteria and treatment algorithm may require revision to better predict symptom improvement after surgery. Improvement seen after ERCP/sphincterotomy is anecdotal but appears to merit further investigation.

Cholecystectomy rates for biliary dyskinesia in children are rising in the United States, but not in other countries. Biliary dyskinesia is a validated functional gallbladder disorder in adults, requiring biliary colic in the diagnosis. In contrast, most studies in children require upper abdominal pain, absent gallstones on ultrasound, and an abnormal gallbladder ejection fraction (GBEF) on cholecystokinin-stimulated cholescintigraphy for diagnosis. We aimed to systematically review existing literature in biliary dyskinesia in children, determine the validity and reliability of diagnostic criteria, GBEF, and to assess outcomes following cholecystectomy. We performed a systematic review following the PRISMA checklist and searched 7 databases including PubMed, Scopus, Embase, Ovid, MEDLINE, ProQuest, Web of Science, and the Cochrane library. Bibliographies of articles were screened for additional studies. Our search terms yielded 916 articles of which 28 were included. Three articles were manually added from searched references. We reviewed 31 peer-reviewed publications, all retrospective chart reviews. There was heterogeneity in diagnostic criteria and GBEF values. Outcomes after laparoscopic cholecystectomy varied from 34% to 100% success, and there was no consensus concerning factors influencing outcomes. The observational, retrospective study designs that comprised our review limited interpretation of safety and efficacy of the investigations and treatment in biliary dyskinesia in children. Symptoms of biliary dyskinesia overlapped with functional dyspepsia. There is a need for consensus on symptoms defining biliary dyskinesia, validation of testing required for diagnosis of biliary dyskinesia, and randomized controlled trials comparing medical versus surgical management in children with upper abdominal pain.

We hypothesize that chronic cholecystitis accounts for the majority of inflammatory diseases in the pediatric population and it is difficult to predict with preoperative ultrasound.

Despite the increase in gallbladder disease, there is a paucity of data on pediatric cholecystitis. Most pediatric studies focus on cholelithiasis and biliary dyskinesia rather than inflammatory gallbladder disease.

We performed a single center retrospective review of all patients who underwent cholecystectomy from 1/1/10 - 1/1/15. Relevant data was extracted, including age, sex, acute vs. chronic presentation, duration of symptoms, preoperative imaging findings, and surgical pathology results.

Out of the 170 patients identified, there were 129 (75.9%) females and 41 (24.1%) males. The average age was 14 years (range 4-23 years). Seventy-six patients presented with acute symptoms with an average duration of pain of 2 days. Ninety-four patients presented with chronic symptoms and had an average duration of pain of 7.4 months. Eight patients (4.7%) had preoperative ultrasound that suggested inflammation, while the remaining showed only cholelithiasis. Pathology revealed chronic cholecystitis in 148 (87.1%). Among those who had pathologic evidence of chronic cholecystitis, preoperative inflammation was seen in only 5 patients (3.3%).

Chronic cholecystitis accounts for the majority of pediatric inflammatory diseases. These data suggest that most pediatric patients experience episodes of inflammation prior to cholecystectomy. Underappreciated gallbladder inflammation may delay surgical referral, increase emergency department and primary doctor visits, and lead to more difficult operations. Surgeons should consider early cholecystectomy when cholelithiasis and symptoms are present.

Barely 130 years after its first description, cholecystectomies are among the most commonly performed surgeries in the USA. The success of this operation with subsequent technical improvements, such as laparoscopic approaches, caused a paradigm shift in the management of gallstone disease. However, symptoms persist in 10-40 % of successfully operated patients. Reviewing monographs, textbooks, and articles published during the last 300 years, several important factors emerge as likely contributors to limited or poor treatment responses. Early on, clinicians recognized that cholelithiasis is quite common and thus often an incidental finding, especially if patients present with vague or atypical symptoms. Consistent with these observations, patients with such atypical symptoms are less likely to benefit from cholecystectomy. Similarly, lasting improvements are more reliably seen in patients with symptoms of presumed biliary origin and documented gallstones compared to individuals without stones, an important point in view of increasing rates of surgery for biliary dyskinesia. While cholelithiasis can cause serious complications, the overall incidence of clinically relevant problems is so low that prophylactic cholecystectomy cannot be justified. This conclusion corresponds to epidemiologic data showing that the rise in elective cholecystectomies decreased hospitalizations due to gallstone disease, but was associated with a higher volume of postoperative complications, ultimately resulting in stable combined mortality due to gallstone disease and its treatment. These trends highlight the tremendous gains in managing gallstone disease, while at the same time reminding us that the tightening rather than expanding indications for cholecystectomy may improve outcomes.