Overview
Definition:
Biliary dyskinesia is a functional gastrointestinal disorder characterized by symptoms of biliary colic without evidence of gallstones, biliary sludge, or other structural abnormalities of the gallbladder or biliary tree
It is thought to result from abnormal gallbladder motility and/or sphincter of Oddi dysfunction, leading to delayed or incomplete emptying of bile
In adolescents, it represents a significant cause of chronic or recurrent abdominal pain.
Epidemiology:
The exact incidence and prevalence of biliary dyskinesia in adolescents are not well-established due to diagnostic challenges and underdiagnosis
It is more commonly diagnosed in females and typically presents in young adulthood, but has been reported in children and adolescents
Estimates suggest it accounts for a notable percentage of children undergoing cholecystectomy for biliary-type pain.
Clinical Significance:
Biliary dyskinesia is clinically significant as it is a common, albeit often missed, cause of recurrent, debilitating right upper quadrant or epigastric pain in adolescents
This pain can significantly impact quality of life, leading to missed school days, anxiety, and unnecessary diagnostic workups
Accurate diagnosis and appropriate management are crucial to alleviate symptoms and prevent unnecessary interventions or long-term sequelae.
Clinical Presentation
Symptoms:
Recurrent episodes of moderate to severe right upper quadrant or epigastric pain
Pain may radiate to the right shoulder or back
Associated symptoms can include nausea, vomiting, and bloating after fatty meals
Symptoms often mimic those of symptomatic cholelithiasis, leading to diagnostic confusion
Pain episodes typically last for several hours.
Signs:
Physical examination is often unremarkable between episodes
During an acute episode, there may be mild right upper quadrant tenderness
Murphy's sign is usually absent
Vital signs are typically normal, unless complications arise
Prolonged episodes may be associated with dehydration or electrolyte imbalances.
Diagnostic Criteria:
The Rome IV criteria for functional gallbladder disorder are often adapted for diagnosing biliary dyskinesia in adolescents
These include: Episodic upper abdominal pain
Pain characteristics consistent with biliary pain (e.g., steady, moderate-to-severe intensity, lasting >30 minutes, located in the right upper quadrant or epigastrium, often occurring after meals)
No gallstones or sludge on ultrasound
Normal liver function tests and bilirubin
Gallbladder ejection fraction (EF) of <35-40% on HIDA scan
Exclusion of other disorders that could explain the pain.
Diagnostic Approach
History Taking:
Detailed history of abdominal pain is paramount, focusing on: location, character, duration, frequency, aggravating factors (especially fatty meals), relieving factors, associated symptoms (nausea, vomiting, fever), and impact on daily activities
History of previous abdominal surgeries or gastrointestinal issues is important
Inquiry about anxiety or stress can also be relevant, though not diagnostic.
Physical Examination:
A thorough abdominal examination should be performed, focusing on identifying tenderness in the right upper quadrant or epigastrium
Palpation for masses or organomegaly
Careful assessment for signs of peritoneal irritation, which would suggest a more acute surgical condition
Examination of the back for referred pain
Examination should also include a general assessment of the child's well-being and nutritional status.
Investigations:
Abdominal ultrasound: Essential to rule out gallstones, sludge, biliary dilation, and other structural anomalies
Liver function tests (LFTs) and complete blood count (CBC): Typically normal in biliary dyskinesia, but important to rule out other causes of pain and assess for infection
Cholescintigraphy (HIDA scan): The cornerstone investigation for assessing gallbladder motility
A gallbladder ejection fraction (EF) below a specific threshold (typically <35-40%) in response to a stimulus (e.g., cholecystokinin or a fatty meal) is considered diagnostic
Oral cholecystography: An older modality but can also assess gallbladder emptying
Magnetic resonance cholangiopancreatography (MRCP): May be used to further evaluate the biliary tree if there is suspicion of sphincter of Oddi dysfunction or other complex biliary issues.
Differential Diagnosis:
Peptic ulcer disease: Pain is often related to meals, but location and radiation might differ
Gastroesophageal reflux disease (GERD): Burning epigastric pain, often positional or related to specific foods
Irritable bowel syndrome (IBS): Diffuse abdominal pain, often relieved by defecation, associated with changes in bowel habits
Chronic pancreatitis: Less common in adolescents but can cause epigastric pain
Functional abdominal pain: A diagnosis of exclusion
Intestinal parasites: Can cause abdominal pain
Mesenteric adenitis: Often associated with viral illness
Gynecological causes: In adolescent females, ovarian cysts or other pelvic pathology should be considered.
Management
Initial Management:
Pain relief with analgesics (e.g., NSAIDs or acetaminophen) is the first step
Dietary modifications, particularly avoiding high-fat meals, can provide symptomatic relief
Education of the patient and family about the nature of the disorder and prognosis is crucial.
Medical Management:
If symptoms persist despite conservative measures, pharmacological interventions may be considered
Low-dose prokinetic agents (e.g., cisapride, though availability and safety profile are concerns in pediatrics, or other agents with evidence of improving GI motility) have been explored
However, evidence for efficacy in biliary dyskinesia is limited
Antispasmodics might offer some relief for pain symptoms
Long-term medical management is generally not the primary approach due to limited efficacy and potential side effects.
Surgical Management:
Cholecystectomy is the definitive treatment for symptomatic biliary dyskinesia when conservative management fails and HIDA EF is unequivocally low
It is indicated for patients with recurrent, debilitating symptoms consistent with biliary colic, a confirmed low gallbladder ejection fraction (<35-40%), and exclusion of other pathologies
Laparoscopic cholecystectomy is the preferred surgical approach due to its minimally invasive nature and faster recovery.
Supportive Care:
Nutritional support is important, especially if the child has poor oral intake due to pain
Psychological support may be beneficial as chronic pain can lead to anxiety and depression
Regular follow-up appointments to monitor symptoms, adherence to dietary recommendations, and evaluate treatment effectiveness
Education on stress management techniques can also be helpful.
Complications
Early Complications:
Potential early complications following cholecystectomy include surgical site infection, bleeding, bile leak, and injury to surrounding structures (e.g., common bile duct injury)
Post-cholecystectomy syndrome, characterized by persistent abdominal pain, can occur in a small percentage of patients
Delayed gastric emptying is also a possibility.
Late Complications:
Long-term complications are rare but can include bile duct strictures, retained stones in the common bile duct (though unlikely in true biliary dyskinesia without stones), and post-cholecystectomy diarrhea
Some patients may experience a recurrence of pain if the diagnosis was not accurate or if other functional disorders coexist
The impact of lifelong altered bile flow on digestion is generally considered minimal.
Prevention Strategies:
Meticulous surgical technique during cholecystectomy is crucial for preventing intraoperative complications
Adequate preoperative assessment to confirm the diagnosis of biliary dyskinesia and rule out other pathologies can prevent unnecessary surgeries and subsequent complications
Postoperative care should focus on early mobilization, pain control, and monitoring for signs of complications
Patient education regarding potential post-operative symptoms and when to seek medical attention is vital.
Prognosis
Factors Affecting Prognosis:
Factors influencing prognosis include the accuracy of diagnosis, the completeness of gallbladder emptying (HIDA EF value), the presence of comorbid functional gastrointestinal disorders, and the patient's adherence to treatment recommendations
Younger age at diagnosis and severity of symptoms may also play a role.
Outcomes:
The prognosis for biliary dyskinesia in adolescents is generally good, especially with appropriate management
Cholecystectomy in carefully selected patients with confirmed low HIDA EF has a high success rate in alleviating symptoms and improving quality of life
A significant percentage of patients experience complete resolution of pain
However, a small proportion may have persistent or recurrent symptoms.
Follow Up:
Post-operative follow-up is essential to monitor for complications, assess symptom resolution, and address any new concerns
For patients managed medically, regular follow-up is needed to evaluate treatment efficacy and adjust management as required
Long-term follow-up is typically guided by symptom persistence and the absence of new findings
Emphasis should be placed on lifestyle modifications and psychological well-being.
Key Points
Exam Focus:
DNB/NEET SS examine the diagnostic criteria for biliary dyskinesia, the interpretation of HIDA scan EF values, and the indications for cholecystectomy versus conservative management
Understanding the controversies surrounding the HIDA EF threshold and management strategies is key
Remember that biliary dyskinesia is a diagnosis of exclusion in adolescents with functional abdominal pain.
Clinical Pearls:
Always consider biliary dyskinesia in adolescents with recurrent right upper quadrant pain despite a normal ultrasound
The HIDA scan EF threshold of <35-40% is critical
Remember that a normal HIDA scan does not rule out all functional gallbladder disorders, but is the current gold standard for assessing motility-related issues
Do not overlook the psychosocial impact of chronic pain in this age group.
Common Mistakes:
Misdiagnosing biliary dyskinesia as other common GI disorders without adequate investigation
Proceeding with cholecystectomy without a confirmed low HIDA EF or clear exclusion of other diagnoses
Inadequate dietary advice or lifestyle modification support
Overreliance on medications without addressing underlying functional components of the disorder.