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Frontiers in Pediatrics 2020While functional gallbladder disorder is a well-recognized and defined condition in adults, its pediatric analog, biliary dyskinesia, lacks uniformity in diagnosis.... (Review)
Review
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. 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. 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.
PubMed: 32266192
DOI: 10.3389/fped.2020.00122 -
Orphanet Journal of Rare Diseases Sep 2022Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a... (Review)
Review
Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left-right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.
Topics: Anti-Bacterial Agents; Bronchiectasis; Child; Humans; Intestinal Volvulus
PubMed: 36085154
DOI: 10.1186/s13023-022-02515-2 -
Translational Gastroenterology and... 2021Biliary dyskinesia generally refers to a hypofunctioning gallbladder with an ejection fraction (EF) of <35% on hepatobiliary iminodiacetic acid scan with cholecystokinin...
BACKGROUND
Biliary dyskinesia generally refers to a hypofunctioning gallbladder with an ejection fraction (EF) of <35% on hepatobiliary iminodiacetic acid scan with cholecystokinin stimulation (CCK-HIDA testing). In adults, biliary hyperkinesia has a defined association with biliary colic symptoms and can be relieved with surgical intervention. This clinical entity has not been well described in children or adolescents. In fact, only recently have we seen biliary hyperkinesia on HIDA at our centers. To that end, we reviewed our recent experience with adolescents who have presented and been treated for this unusual clinical entity.
METHODS
With IRB approval, we retrospectively reviewed the records of all patients with abnormally high HIDA EFs (>80%) cared for by the pediatric surgery services at two tertiary care centers over the span of a three-year period. Age, sex, BMI, CCK-HIDA results, and preoperative testing and post-operative pathology were noted. Resolution of symptoms was determined by subjective patient self-reporting at postoperative visit.
RESULTS
Eighteen patients met inclusion criteria. Average age 15.7 (range, 10-17 years), median BMI 27.3 (±8.2). Fifteen patients were female and 3 were male. Average CCK-HIDA EF was 91.6% (±5.2), 82.4% of the patients had evidence of chronic cholecystitis and/or cholesterolosis on pathology. Postoperatively, 82.4% of the patients available for follow up (n=17) reported complete or near complete resolution of symptoms.
CONCLUSIONS
Biliary hyperkinesia is an emerging clinical entity in children and adolescents and has a similar presentation to biliary hypokinesia. While the pathophysiologic mechanism of pain is not fully elucidated, laparoscopic cholecystectomy appears to provide a surgical cure for these patients and should be considered in the differential for the patient with an unremarkable workup and history suggestive of biliary colic.
PubMed: 34423157
DOI: 10.21037/tgh-20-258 -
Cureus Dec 2023Objective Our goal is to describe and report the outcomes of patients undergoing robotic single-site cholecystectomy at a single institution. Background Cholecystectomy...
Objective Our goal is to describe and report the outcomes of patients undergoing robotic single-site cholecystectomy at a single institution. Background Cholecystectomy is a common procedure performed to remove the gallbladder. Robotic single-site cholecystectomy (RSSC) is rapidly emerging as a safe and feasible alternative to conventional 4-port laparoscopic techniques. Patients who undergo RSSC procedures may also have a decreased need for postoperative analgesics and a lower postoperative pain score. Methods From September 2020 to August 2023, there were 33 cases of RSSC performed by a single surgeon at a single institution. We recorded demographic data including sex, age, and BMI as well as postoperative outcome data including wound dehiscence, postoperative infection, biliary leakage, wound herniation, blood loss, and conversion to open procedure. Results The patient group included 24 females (72.7%) and nine males (27.3%) with a median age of 32 (Range: 9-70) and a median BMI of 24.2 kg/m (Range: 18.1-30.7). The majority of these patients were receiving cholecystectomies for benign conditions such as symptomatic cholelithiasis (n = 18, 54.5%), biliary dyskinesia (n = 13, 39.4%), acute cholecystitis (n = 1, 0.03%), and biliary colic (n = 1, 0.03%). The average estimated blood loss was 5.91 mL. Thirty-two patients (96.9%) were discharged home the same day of surgery. One patient was admitted overnight for observation due to severe biliary dyskinesia diagnosed preoperatively. The patient had no complications and was discharged the following day. One patient presented with acute abdominal wall cellulitis and omphalitis with no underlying abscess four weeks after the operation. They were treated with therapeutic antibiotics. No patients underwent conversion to an open procedure. There were no incidences of postoperative wound dehiscence or biliary leakages. One patient was admitted overnight for observation of biliary dyskinesia and another experienced abdominal wall cellulitis four weeks post-operation. Conclusions Although conventional multi-incision laparoscopic cholecystectomy remains the gold standard treatment for benign gallbladder disease, our study demonstrates the practicality and safety of Robotic Single-Site Cholecystectomy procedures.
PubMed: 38196423
DOI: 10.7759/cureus.50271 -
Case Reports in Gastroenterology 2021Sphincter of Oddi dysfunction (SOD) is a syndrome caused by either dyskinesia or stenosis of the sphincter of Oddi. It has been categorized into biliary and pancreatic...
Sphincter of Oddi dysfunction (SOD) is a syndrome caused by either dyskinesia or stenosis of the sphincter of Oddi. It has been categorized into biliary and pancreatic SOD based on clinical features and laboratory findings. We present a case of a 51-year-old female (post-cholecystectomy) who presented with intermittent chronic right upper quadrant pain. Laboratory investigations showed persistently elevated liver function tests and a dilated common bile duct without the presence of any stones. Endoscopic retrograde cholangiopancreatography with manometry showed an elevated sphincter of Oddi pressure, thus confirming the diagnosis of SOD. She underwent endoscopic sphincterotomy and papillotomy with normalization of liver function tests and resolution of her chronic symptoms.
PubMed: 34054398
DOI: 10.1159/000514542 -
World Journal of Gastroenterology Jan 2024Biliary dyskinesia is a relatively common gastrointestinal disease that is increasing in incidence as living standards improve. However, its underlying pathogenesis...
Biliary dyskinesia is a relatively common gastrointestinal disease that is increasing in incidence as living standards improve. However, its underlying pathogenesis remains unclear, hindering the development of therapeutic drugs. Recently, "Expression and functional study of cholecystokinin-A receptors on the interstitial Cajal-like cells of the guinea pig common bile duct" demonstrated that cholecystokinin (CCK) regulates the contractile function of the common bile duct through interaction with the CCK-A receptor in interstitial Cajal-like cells, contributing to improving the academic understanding of biliary tract dynamics and providing emerging directions for the pathogenesis and clinical management of biliary dyskinesia. This letter provides a brief overview of the role of CCK and CCK-A receptors in biliary dyskinesia from the perspective of animal experiments and clinical studies, and discusses prospects and challenges for the clinical application of CCK and CCK-A receptors as potential therapeutic targets.
Topics: Animals; Guinea Pigs; Cholecystokinin; Receptor, Cholecystokinin A; Biliary Dyskinesia; Common Bile Duct; Receptors, Cholecystokinin
PubMed: 38314130
DOI: 10.3748/wjg.v30.i3.283 -
Journal of Pediatric Gastroenterology... Jun 2019We reviewed medical records and conducted a nationwide survey to characterize the clinical features and determine the prevalence of biliary tract disease in girls and...
OBJECTIVE
We reviewed medical records and conducted a nationwide survey to characterize the clinical features and determine the prevalence of biliary tract disease in girls and women with Rett syndrome (RTT).
METHODS
Sixty-two individuals with RTT and biliary tract disease were identified from the membership of Rett Syndrome Organization and patient files of the principal investigator. Medical records of 46 individuals were reviewed for presenting features, diagnostic tests, and treatment outcomes of biliary tract disease. We designed a questionnaire that probed the frequency of risk factors and treatment outcomes of biliary tract disease in RTT. The questionnaire was completed by 271 parents whose daughters met the clinical criteria for RTT and/or had MECP2 mutations and participated in the Natural History of Rett Syndrome Study.
RESULTS
Presenting symptoms identified by record review included abdominal pain (94%), irritability (88%), weight loss (64%), and vomiting (52%). Biliary dyskinesia, cholecystitis, and cholelithiasis were identified in 90%, 77%, and 70%, respectively, by cholescintigraphy, surgical pathology, and abdominal ultrasound. The prevalence of biliary tract disease was 4.4% (n = 12) in the RTT cohort. Risk factors included older age (P < 0.001) and a positive family history (P < 0.01). Diagnoses included cholecystitis (n = 5), biliary dyskinesia (n = 6), and cholelithiasis (n = 7). Ten individuals underwent surgery; 7 had resolution of symptoms after surgical intervention.
CONCLUSIONS
Biliary tract disease is not unique to RTT, but may be under-recognized because of the cognitive impairment of affected individuals. Early diagnostic evaluation and intervention may improve the health and quality of life of individuals affected with RTT and biliary tract disease.
Topics: Adolescent; Biliary Tract Diseases; Child; Female; Humans; Methyl-CpG-Binding Protein 2; Mutation; Prevalence; Prospective Studies; Retrospective Studies; Rett Syndrome; Risk Factors; Surveys and Questionnaires; Young Adult
PubMed: 30664568
DOI: 10.1097/MPG.0000000000002273 -
Surgery Open Science Apr 2021The accuracy of hepatobiliary scintigraphy to assess gallbladder function remains controversial. National supply shortages of pharmaceutical-grade cholecystokinin led to...
BACKGROUND
The accuracy of hepatobiliary scintigraphy to assess gallbladder function remains controversial. National supply shortages of pharmaceutical-grade cholecystokinin led to the use of an oral fatty meal to stimulate gallbladder contraction during hepatobiliary scintigraphy. The goal of this study was to compare the predictive indices of cholecystokinin and fatty meal ingestion for stimulation of gallbladder contraction.
METHODS
Patients evaluated with hepatobiliary iminodiacetic acid scan from 2014 to 2017 were reviewed and grouped based on testing stimulant (fatty meal versus cholecystokinin). Patients who later underwent cholecystectomy were selected for analysis. Hepatobiliary iminodiacetic acid results were correlated with surgical pathology and postoperative resolution of symptoms. Two-way statistical analysis was performed.
RESULTS
A total of 359 patients underwent hepatobiliary iminodiacetic acid scan followed by cholecystectomy for biliary dyskinesia. Patients who received fatty meal stimulant (n = 86) were compared to those that received cholecystokinin (n = 273). Mean gallbladder ejection fraction during hepatobiliary iminodiacetic acid was 38% and 44% for the cholecystokinin and fatty meal groups, respectively, P = .073. Predictive metrics were not statistically different between groups with regard to pathology, symptomatic improvement, or accuracy. Symptomatic resolution (cholecystokinin-hepatobiliary iminodiacetic acid 78%, fatty meal-hepatobiliary iminodiacetic acid 68%; P = 0.058) and specificity (cholecystokinin-hepatobiliary iminodiacetic acid 26%, fatty meal-hepatobiliary iminodiacetic acid 44%, P = 0.417) were comparable in both testing groups.
CONCLUSION
Stimulation of gallbladder contraction with a fatty meal during hepatobiliary iminodiacetic acid testing is a more affordable and reliable alternative to cholecystokinin for patients undergoing evaluation for gallbladder dysmotility.
PubMed: 33569543
DOI: 10.1016/j.sopen.2020.10.003