Gall bladder and Biliary Tree.pdf

Gallbladder and Biliary Tree Management of management. To thoughtfully approach this predicament, a general unders...

Gallbladder and Biliary Tree Management of management. To thoughtfully approach this predicament, a general understanding of the relative risks of expectant management versus Asymptomatic (Silent) cholecystectomy is crucial. Gallstones RISK OF PROGRESSION TO SYMPTOMATIC CHOLELITHIASIS Theodore N. Pappas, MD, and Christopher R. Reed, MD Gallstone disease is generally thought of as a sequence from asymp- tomatic to symptomatic cholelithiasis (i.e., stone formation initially with subsequent development of pain, infection, pancreatitis, or NATURAL HISTORY OF CHOLELITHIASIS cancer), although the time between development of stones and symptoms is not currently estimable. Through a combination of The first description of incidental gallstones probably dates back to cross-sectional and longitudinal studies, it has been demonstrated the 14th century, a relatively recent observation in the grand scheme that only a minority of patients with asymptomatic gallstones will of pathologic observations. This has been attributed to the relative ultimately develop symptoms. In their large, longitudinal investiga- rarity of gallstone formation before modern diets. These postmortem tion of 673 European subjects with asymptomatic cholelithiasis and findings preceded the first descriptions of symptomatic gallstones by a median follow-up of nearly 20 years, Shabanzadeh and colleagues about 200 years, perhaps a testament to their frequently asymptom- found that only 20% of gallstones were ultimately found to cause any atic nature. Without the benefits of modern ultrasonography, open disease, which has been reiterated by smaller studies. The majority of cholecystectomy became the preferred treatment modality for symp- disease was uncomplicated (i.e., biliary pain without acute cholecys- tomatic gallstone disease throughout the early 1900s, and it was not titis, choledocholithiasis, or pancreatitis). until the 1980s that incidental, asymptomatic cholelithiasis became a There are some demographic and ultrasonographic characteris- very common clinical problem. tics that are associated with increased risk of symptoms, especially The decreased morbidity of laparoscopic cholecystectomy intro- with complicated presentation. Female sex, immobility of stones, duced considerable interest in surgery for the treatment of asymp- numerous stones, and large (>10 mm) or small (3 cm) gallstones have demonstrated a small Gastrectomy but significant increase in the risk for malignancy. Given that these Cholecystectomy in patients with asymptomatic gallstones had stones have a higher risk of lifetime symptom development as well, been historically performed at the time of open gastrectomy (i.e., special consideration should be given to cholecystectomy in other- for cancer or peptic ulcer disease). However, with the emergence wise appropriate patients with very large gallstones. of laparoscopic techniques for gastric resection that effect minimal scarring and increasing availability of ERCP, most surgeons feel that concomitant cholecystectomy adds unnecessary morbidity without Weight Loss Surgery adequate benefit. As in the weight loss surgical population, the deci- There is no question that obesity is an independent risk factor for sion should ultimately be individualized to each patient based on gallstone formation. Moreover, rapid weight loss appears to change their risk for complications from cholelithiasis. the chemical composition of bile and promote stone formation, lead- ing to a further increased risk of gallstone formation beyond obesity alone. This connection has been studied and is quite profound. In a Diabetes prospective study involving 51 obese adults dieting for weight loss, After early uncontrolled studies demonstrated an increased risk of 25% of dieting subjects had developed new gallbladder sludge or complications following surgical treatment of acute cholecystitis stones after 8 weeks of weight loss, whereas 0% of nondieting con- among diabetic patients, there was some enthusiasm for prophylactic trols had new gallbladder abnormalities. cholecystectomy in diabetics with gallstones. However, data eventu- Cholecystectomy has historically been performed during open ally emerged demonstrating acceptable outcomes for these patients Roux-en-Y gastric bypass for weight loss for patients with preexist- from cholecystectomy. Most surgeons currently recommend chole- ing gallstones. This was justified given (1) the association between cystectomy only for those patients with symptomatic cholelithiasis weight loss and gallstone formation, (2) historical difficulty in and diabetes, similar to those guidelines for the general population. endoscopic management of complications from gallstones (i.e., cho- One caveat is that mild symptoms may herald the development of ledocholithiasis) with Roux-en-Y anatomy, and (3) local adhesion both more severe symptoms as well as complications (i.e., acute cho- formation causing a “hostile” and scarred environment for future lecystitis or pancreatitis), and diabetics with early symptoms should cholecystectomy. With the increasing performance of sleeve gas- be medically optimized and offered surgery if otherwise appropriate trectomy and improved availability of push enteroscopy and other to prevent complications and the need for urgent cholecystectomy. Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 460 Management of Biliary Dyskinesia Hemolytic Anemias uncommon but important clinical dilemma to surgeons. Due to the Rapid bilirubin catabolism can lead to formation of pigmented gall- high rate of complications associated with common bile duct stones, stones, with a characteristic black appearance on gross examination. cholecystectomy is typically recommended in good candidates to The most commonly encountered congenital hemolytic anemias are prevent recurrence and complications. Cholecystectomy should sickle cell disease (and trait) and thalassemia, both of which cause be performed after endoscopic stone retrieval, sphincterotomy, or enhanced formation of gallstones. Acquired hemolytic anemias, such surgical common duct clearance procedures in appropriate surgical as immune-mediated hemolysis, may also predispose patients to candidates. gallstone formation. The management of asymptomatic gallstones in patients with hemoglobinopathy is somewhat controversial. The fre- quency of asymptomatic cholelithiasis in these patients (roughly 25% Spinal Cord Injury in a cross-sectional study), abdominal pain of other etiologies, and Perhaps through autonomic dysfunction and biliary stasis, patients likelihood of symptom development make the treatment of asymp- with spinal cord injuries are at very high risk for the development tomatic gallstones among these patients distinct from the general of gallstones. Compared with age- and gender-matched controls, population. In particular, sickle cell disease deserves special mention. patients with spinal cord injuries are about three times as likely to Many surgeons perform cholecystectomy for asymptomatic gallstones develop gallstones in their lifetime. One of the most feared com- at the time of splenectomy, such that cholecystitis may be excluded as a plications of apparently asymptomatic gallstones in this unique potential etiology for abdominal pain during vaso-occlusive crises. The patient population is the late, complicated presentation of acute on pre- and perioperative management of patients with sickle cell disease chronic cholecystitis due to sensory neuropathy. In select patients (hemoglobin SS) in particular involves careful management of volume with asymptomatic gallstones, prophylactic cholecystectomy may status, oxygenation, and analgesia. Ideally, surgery should be per- be reasonable to prevent a late and complicated presentation after formed in a center with experience in the management of these patients development of cholecystitis. with cooperation among surgery, anesthesia, and hematology teams. SUMMARY Intestinal Insufficiency (Short Gut Syndrome) Asymptomatic cholelithiasis remains a common clinical dilemma, Patients with chronic intestinal insufficiency are a highly specialized and the lifetime risk of complicated disease from initially silent gall- population but one with which all surgeons must be familiar none- stones is low but not negligible (estimated at 8%). Although the rela- theless. Long-term parenteral nutrition dependence leads to biliary tive morbidity of laparoscopic cholecystectomy continues to decline stasis and clearly predisposes to development of both gallstones since its advent in the 1980s, there is still occasional major morbidity and attendant complications. In one small but illustrative series, 5/5 associated with the procedure (roughly 5%, with the majority being (100%) patients with severe intestinal insufficiency and parenteral infectious complications). Therefore, a thorough understanding of nutrition dependence developed symptomatic cholelithiasis or com- the relative risks of the procedure versus conservative management plications. It is also worthwhile to note that decreasing remaining are foundational to individualizing surgical treatment recommenda- viable bowel as well as parenteral nutritional dependence are both tions for each patient. independent risk factors for development of symptomatic cholelithi- asis. Given the relative rarity of this chronic problem as well as the likelihood of complicating factors (i.e., prior abdominal surgery, Suggested Readings inflammatory bowel disease), the decision regarding prophylactic Festi D, Reggiani ML, Attili AF, et al. Natural history of gallstone disease: cholecystectomy should be individualized to the patient based on Expectant management or active treatment? Results from a popula- their individual risk factors for symptomatic cholelithiasis and tion-based cohort study. J Gastro and Hep. 2010;25(4):719–724. potential morbidity from the procedure. Friedman GD. Natural History of Asymptomatic and Symptomatic Gallstones. Am J Surg. 1993;65(4):399–404. Shabanzadeh DM, Sørensen LT, Jørgensen T, et al. A prediction rule for risk Asymptomatic Choledocholithiasis stratification of incidentally discovered gallstones: Results from a large cohort study. Gastroenterology. 2016;150(1):156–167. e1. Asymptomatic choledocholithiasis, typically an incidental finding on Williams E, Beckingham I, El Sayed G, et al. Updated guideline on the man- ultrasonography or CT ordered for a separate indication, presents an agement of common bile duct stones. Gut. 2017;66(5):765–782. Management of Biliary the decade between 1991 to 2001. This is in stark contrast to the 25 cases/million noted in countries outside of the United States. This Dyskinesia increase has been most prominent in the pediatric patient popula- tion with a sevenfold increase between 1997 and 2010. It is unclear if the discrepancy noted in incidence between the United States J. Bart Rose, MD and other countries represents a difference in patient population or overdiagnosis. EPIDEMIOLOGY CLINICAL PRESENTATION, EVALUATION, Biliary dyskinesia is a rare diagnosis. However, up to 20% of the AND DIAGNOSIS cholecystectomies performed annually in the United States are for a diagnosis of functional gallbladder disorder. The incidence in the Patients presenting with biliary dyskinesia often have vague abdom- United States has risen from 43 cases/million to 89 cases/million in inal complaints centered around recurrent episodes of abdominal Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved.  G a l l b l a d d e r a n d B i l i a ry T r e e   461 pain. Biliary dyskinesia is a diagnosis of exclusion and more common The definition of an abnormally low GbEF can also be a moving causes of episodic abdominal pain should be excluded before inter- target as reported cutoffs have ranged from 4 and/or ASA class ≥ III) (see Fig. 2). the Charlson Comorbidity Index (CCI) and American Society of Anesthesiologists’ (ASA) Physical Status Classification System. In ROLE OF CHOLECYSTOSTOMY TUBE addition, the TG18 guidelines established predictive factors that have PLACEMENT IN COVID-19 PATIENTS been associated with increased surgical mortality risk on multivariate analyses, including jaundice (total bilirubin ≥2 mg/dL) with coexist- The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ing neurologic dysfunction or respiratory dysfunction. and COVID-19 pandemic has had a major impact on emergent Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 470 Proper Use of Cholecystotomy Tubes Supportive Care Grade I Early Laparoscopic with (MILD) Cholecystectomy Antibiotics Emergent Cholecystectomy for symptoms 72 hours Marked local inflammation (i.e., gangrenous cholecystitis, pericholecystic abscess, hepatic abscess, biliary peritonitis, emphysematous cholecystitis) III (Severe) Cholecystitis with associated organ dysfunction to include any of the following: Circulatory failure (hypotension requiring treatment with dopamine >5 µg/kg/min, or any dose of norepinephrine Neurologic disturbance (decreased level of consciousness) Respiratory failure (partial pressure of oxygen in arterial blood/fraction of inspired oxygen ratio 2.0 mg/dL) Hepatic failure (international normalized ratio >1.5) Thrombocytopenia (platelet count 5 days Khasawneh (2015) 245 Retrospective Calculous cholecystitis Acalculous cholecystitis Sepsis likely from a biliary source Cha (2014) 82 Retrospective Patient comorbidities Chang (2014) 60 Retrospective Failure to respond to initial medical treatment in patients with high perioperative risk Impending rupture of a severely distended gallbladder that may cause clinical deterioration Suspected gallbladder necrosis or perforation in patients with severe comorbidities and no other treatments available Hsieh (2012) 166 Retrospective Septic shock/severe sepsis Gallbladder rupture Failed conservative treatment after 48 hours Joseph (2012) 106 Retrospective Poor surgical candidates McKay (2012) 68 Retrospective Surgeon discretion Kortram (2011) 27 Retrospective A component of one or more of the following: Age ASA APACHE Comorbidity Nasim (2011) 62 Retrospective ASA grade II/IV Significant sepsis resulting in hemodynamic instability Patients deemed moderate or high risk for general anesthesia Saeed (2010) 41 Observational Calculous cholecystitis case series Acalculous cholecystitis Gallbladder perforation and/or empyema Paran (2006) 54 Prospective Poor surgical candidate secondary to comorbidities and/or symptoms >72 hours Basaran (2005) 18 Retrospective Medical comorbidity including terminal cancer, uncontrolled hypertension and diabetes, CAD, HTN, CHF, ARF Byrne (2003) 45 Retrospective Medical comorbidities including cardiovascular disease and malignancy Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved.  G a l l b l a d d e r a n d B i l i a ry T r e e   473 TABLE 4 Studies Reporting Various Indications for Cholecystostomy Tube Placement—cont’d Study Sample Size Study Design Reported Indications Hatzidakis (2002) 63 Prospective Randomized to cholecystostomy tube group, but patients were referred to surgical team for possible tube placement Spira (2002) 55 Retrospective Biliary sepsis Septic shock Severe comorbidities Berber (2000) 15 Retrospective High risk for general anesthesia secondary to comorbidities and/or chronic illness Inflammation too severe during attempted laparoscopic cholecystectomy APACHE, Acute Physiology and Chronic Health Evaluation II; ARF, acute renal failure; ASA, American Society of Anesthesiologists; CAD, coronary artery disease; CHF, congestive heart failure; HTN, hypertension. A B C D E FIG. 3 Ultrasound placement of a cholecystostomy tube. (A) Gallbladder is visualized. (B, C) Gallbladder is accessed with a Yeah needle. (D, E) Pigtail catheter is placed into the gallbladder lumen. this fluid will be clear secondary to resultant hydrops. Once the gall- technique. If placed directly into the gallbladder, the drain can be bladder is accessed, a wire (short 75-cm Amplatz or Rosen curved secured to the gallbladder wall to prevent bile leakage around the tip wire) is placed through the needle and into the gallbladder lumen tube. If the gallbladder wall is necrotic, a fenestrating partial cholecys- (Fig. 3C,D). A dilator is placed over the wire, and an 8-Fr pigtail tectomy with removal of stones along with wide drainage may be an catheter is then advanced and coiled in the gallbladder lumen using alternative option if cholecystectomy is deemed unsafe or unfeasible. the Seldinger technique (Fig. 3E and Fig. 4D–F). At the end of the procedure, CT and/or ultrasound should be utilized to confirm the COMPLICATIONS OF proper position of the drainage tube (Fig. 4D–F). The catheter is then CHOLECYSTOSTOMY TUBE PLACEMENT secured to the skin by a suture or proprietary adhesive device. Although cholecystectomy tube placement is often not difficult and allows for poor surgical candidates to avoid an operation, both Operative Approach immediate and long-term complications can occur. Data on com- A cholecystostomy tube can also be placed surgically via laparoscopic plications are largely derived from single-institution retrospective or open approach. These options should be kept in mind when the studies. surgeon begins the operation with the intent of performing chole- cystectomy but finds inflammation so severe that the dissection is deemed unsafe or the patient becomes unstable during the operation. Immediate Complications In either situation, the gallbladder is visualized by the surgeon and the Immediate complications from cholecystostomy placement include tube can be inserted under direct vision through a small cholecystot- continuation/progression of acute cholecystitis, biliary peritoni- omy made in the gallbladder or transhepatically using the Seldinger tis, sepsis, bleeding, biliary leak, and tube dislodgement. Overall, Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 474 Proper Use of Cholecystotomy Tubes A B C D E F FIG. 4 Computed tomography placement of a cholecystostomy tube. (A, B) Gallbladder is visualized. (C) Computed tomography markers are placed on the abdominal skin on the right upper quadrant to help with guidance. (D–F) Verification of pigtail catheter placement in the gallbladder lumen. complications specifically related to cholecystostomy tube placement quadrant pain. Cha and colleagues reported a technical success rate range from 5% to 33% across studies, but most are related to the of 100% in 82 patients undergoing cholecystostomy tube placement, latter three issues (i.e., bleeding, biliary leak, or tube dislodgement). and a clinical success rate of 98% with one patient dying of chole- To prevent bleeding, especially with the transhepatic approach, the cystitis-related complications. But, in a study by Joseph et al., 32% of interventional radiologist and/or surgeon should ensure that any critically ill patients who had a cholecystostomy tube placed did not coagulopathy is corrected before tube placement. Bile leakage can improve or declined clinically after tube placement. occur if the tube is dislodged or the gallbladder wall is necrotic and the bile leaks around the tube itself. This can lead to sepsis, LONG-TERM MANAGEMENT OF diffuse biliary peritonitis, and/or a biloma/abscess in the right upper CHOLECYSTOSTOMY TUBES quadrant if the leakage is contained and localized. This should be suspected when a patient’s leukocytosis worsens or does not improve The management of cholecystostomy tubes after initial treatment after tube placement as well as when symptoms initially improve but remains somewhat controversial. There are no broadly accepted the patient subsequently develops sepsis, hemodynamic instability, guidelines regarding definitive tube management. Across studies, 5% fever, worsening leukocytosis, or worsening abdominal pain. to 63% of patients eventually undergo definitive cholecystectomy, but this is not well defined. However, the TG18 recommends delayed cholecystectomy after tube placement, regardless of initial grade of Later Complications the cholecystitis. In the literature, several authors have proposed In the long-term, both readmission to the hospital and recurrence different algorithms for the management of cholecystostomy tubes, of acute cholecystitis are common after cholecystostomy tube place- many of which involve cholangiography to assess the patency of ment when delayed definitive therapy with cholecystectomy is not the cystic duct and biliary tree. In a study by Cha and colleagues, performed. There is significant cost associated with frequent hospital patients underwent a cholangiogram through the cholecystostomy visits, radiologic interventions, and overall increased hospital days, tube to evaluate for patency of the cystic duct and biliary tract once stressing the importance of definitive cholecystectomy when at all the patient’s symptoms and clinical status improved. This was done possible. Recurrence of acute cholecystitis after cholecystostomy during the index hospitalization. If patency was demonstrated via tube placement ranges from 9% to 41% in small series, and readmis- contrast emptying into the duodenum, the catheter was clamped. If sions rates are as high as 40%. Readmissions are mostly related to patients developed recurrent cholecystitis after clamping, worsening inadvertent tube dislodgement or removal, tube occlusion, recurrent laboratory values (e.g., leukocytosis, hyperbilirubinemia, transami- cholecystitis, or catheter-site related pain. nitis), or worsening symptoms, the catheter was placed back to exter- nal drainage for 7 days, after which patients were reassessed. If the patient tolerated clamping and had continued clinical improvement, Ineffective Placement the cholecystostomy tube was removed during the initial admission. Although placement of cholecystostomy tubes is 90% to 100% effec- If the cystic duct was not patent, patients were discharged with the tive in most studies, some patients will not resolve their cholecystitis cholecystostomy tube placed to an external drainage bag. Zarour as measured by ongoing sepsis, leukocytosis, and/or right upper and colleagues reviewed outcomes of 119 patients who underwent Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved.  G a l l b l a d d e r a n d B i l i a ry T r e e   475 cholecystostomy tube placement for acute cholecystitis. In their placement across many retrospective series. Ideally, cholecystectomy study, all patients who underwent tube placement were discharged should be performed in the elective setting after the patient’s clinical with the tube in place and draining externally. Follow-up cholangio- status has improved. Cholecystectomy may be needed more urgently if gram was performed 2 to 3 weeks later; if the duct was patent and the cholecystostomy tube placement fails to control local inflammation and patient was deemed an appropriate surgical candidate, the tube was systemic sepsis or if inflammation recurs acutely. However, data from clamped and left in place, and the patient subsequently underwent Medicare beneficiaries undergoing cholecystostomy tube placement for interval cholecystectomy. In patients who were not deemed fit but grade III cholecystitis demonstrate that only one-third of these patients had biliary tract patency, the tube was removed. undergo definitive treatment with a delayed cholecystectomy. In the absence of prospective data, (1) the duration of recom- The timing for delayed cholecystectomy is also a topic of deliber- mended tube drainage, (2) the need for definitive cholecystectomy, ation. In a study by Hung et al., there is some evidence that optimal (3) the timing of tube removal, and (4) the timing of cholecystectomy timing for laparoscopic cholecystectomy is 9 to 10 weeks after place- remain topics of debate. The reported median length of time that the ment of a cholecystostomy tube. In another retrospective study by tube remained varies widely in the literature and depends on whether Woodward et al., increased complications occurred when interval definitive cholecystectomy was performed. Times ranged from 10 cholecystectomy was performed less than 1 month after cholecys- days in those who eventually underwent cholecystectomy to 70 days tostomy tube placement. However, patients with tubes in place for in patients who did not undergo definitive treatment with cholecys- greater than 8 weeks demonstrated higher rates of tube-related tectomy. Most studies recommend drainage for 3 to 6 weeks because complications, leading the authors to suggest that the most favorable this allows a tract to develop. Others recommend earlier removal and timing for interval cholecystectomy is between 4 and 8 weeks after early definitive cholecystectomy. In contrast, separate studies recom- cholecystostomy tube placement. A retrospective analysis of the New mend a longer period of tube drainage in patients with uncontrolled York SPARCS database involving 9728 patients suggested that early diabetes, persistent infection, malnutrition, and/or those on steroids cholecystectomy (≤8 weeks after cholecystostomy tube placement) because these conditions may hinder the healing process. was associated with a higher risk of complications and longer hospi- Ultimately, tube removal is dependent on resolution of the tal length of stay. Ultimately, the timing of delayed cholecystectomy patient’s symptoms, the presence or absence of cystic duct obstruc- should be delayed at least 4 to 6 weeks after tube insertion, but there tion, and whether the patient is a candidate for cholecystectomy. are no prospective analyses to determine this timing. This is determined on a case-by-case basis through evaluation of laboratory values, abdominal examination, and the patient’s report PROPOSED ALGORITHM FOR of resolved abdominal pain. CHOLECYSTOSTOMY TUBE PLACEMENT In our opinion, if the patient’s acute cholecystitis has resolved AND MANAGEMENT and he or she is a candidate for cholecystectomy, tube evaluation is not necessary. Cholecystectomy should be scheduled, and the tube The proper use of cholecystostomy tubes includes temporary treat- should be left in situ and draining externally until the operation is ment for patients with calculous or acalculous cholecystitis who performed. This can be done laparoscopically or open, though con- cannot tolerate surgery according to the TG18 guidelines. The sever- version rates in this setting are higher than normal and should not ity of cholecystitis and the duration of symptoms are not absolute be unexpected. If the patient’s clinical status improves and he or she contraindications for cholecystectomy, and they do not mandate is not a candidate for cholecystectomy, the patient should undergo cholecystostomy tube placement in isolation. Potential reasons for a a tube cholangiogram, and tube removal should only be considered patient’s inability to tolerate surgery include severe systemic disease, if there is patency of the patient’s cystic duct. This is because the including cardiovascular disease, underlying terminal malignancy, likelihood of recurrent episodes of cholecystitis is extremely high and/or any condition that precludes general anesthesia. if the cystic duct remains obstructed. The timeline in which a chol- The TG18 algorithm for management of patients with acute chole- angiogram is done in these patients varies in the literature, but we cystitis (see Fig. 2) notes that initial evaluation should include assess- recommend 3 to 6 weeks to allow for a track to form before tube ment of the patient’s clinical status and the severity of the gallbladder removal. If a cholangiogram demonstrates a patent cystic duct, the disease. If patients are hemodynamically stable and able to tolerate a next step is clamping of the cholecystostomy tube, with removal in general anesthetic, cholecystectomy should be performed as soon as the absence of recurring symptoms after clamping. It is important possible during the index admission, regardless of the Tokyo grade. to note that the criterion of cystic duct patency does not necessarily Cholecystostomy tubes should be reserved for patients who are not apply to patients with acalculous cholecystitis as the pathophysiol- candidates for cholecystectomy because of underlying comorbidity ogy is different from calculous cholecystitis. But patients should at and/or physiologic decompensation resulting from acute illness and for least undergo clamping trials before removal to reduce the risk of those who do not rapidly respond to antibiotics and supportive care. having recurrent symptoms after tube removal that may lead to an In cases of grade III disease, if a patient improves with antibiotics and additional procedure. organ support, reevaluation should be undertaken during the index hospitalization for possible cholecystectomy. If not performed on the DEFINITIVE TREATMENT WITH index admission, then delayed or elective cholecystectomy should be CHOLECYSTECTOMY performed in patients who have a life expectancy of greater than 1 year as cholecystitis recurrence rates are high. As with all interventions, Cholecystectomy remains the only definitive treatment for patients surgeons must consider the risks and benefits of this procedure and its with acute cholecystitis. In individuals who undergo cholecys- long-term consequences with each individual patient in mind. tostomy tube placement, the TG18 recommend delayed-interval cholecystectomy after tube placement (see Figs. 1 and 2), except for patients with initial grade III disease, poor performance status, and Suggested Readings limited predicted life expectancy. These recommendations apply to calculous cholecystitis, but other reports on patients with acalculous Altieri MS, Yang J, Yin D, Brunt LM, Talamini MA, Pryor AD. Early cho- cholecystitis agree that cholecystectomy may not be necessary. The lecystectomy (1.3 mg/dL 84 (64–94) 91 (86–94) Gurusami, 2015 (PMID: 25719223) Total bilirubin >twice the normal limit 42 (22–63) 97 (95–99) Gurusami, 2015 (PMID: 25719223) Alkaline phosphatase (ALP) >125 IU/L 92 (74–99) 79 (74–84) Gurusami, 2015 (PMID: 25719223) Alkaline phosphatase >twice the normal limit 38 (19–59) 97 (95–99) Gurusami, 2015 (PMID: 25719223) Gamma-glutamyltransferase (GGT) >95.5 IU/L 90.8 83.6 Mei, 2019 (PMID: 30705891) GGT >95.5 IU/L and ALP >151.5 IU/L 93.5 85.1 Mei, 2019 (PMID: 30705891) Transabdominal ultrasound (TAUS) 73 (44–90) 91 (84–95) Gurusami, 2015 (PMID: 25719223) Magnetic resonance cholangiopancreatography 93 (87–96) 96 (90–98) Giljaca, 2015 (PMID: 25719224) (MRCP) Endoscopic ultrasound (EUS) 95 (91–97) 97 (94–99) Giljaca, 2015 (PMID: 25719224) Intraoperative cholangiography (IOC) 99 (83–100) 99 (95–100) Gurusami, 2015 (PMID: 25719223) Laparoscopic ultrasound (LUS) 90 (87–92) 99 (99–99) Jamal, 2016 (PMID: 26985813) Endoscopic retrograde cholangiopancreatography 83 (72–90) 99 (94–100) Gurusami, 2015 (PMID: 25719223) (ERCP) Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 478 Management of Common Bile Duct Stones increases 0.2 mm to 1 mm per decade of life before CCY; following duct stents and rectal indomethacin. In some instances, retrograde CCY, it is normal to have a dilated CBD.) Computed tomography passage of a guidewire through a ductotomy in the cystic duct (CD) (CT) and MRCP are also commonly used to evaluate patients with in the rendezvous approach may allow for smooth CBD cannulation suspected biliary pathology. CT may overestimate CBD diameter and significantly reduce the risk of pancreatitis. compared with TAUS or MRCP. EUS and MRCP both have high accuracy for revealing CBDS. In patients with an intermediate ERCP in Patients with Altered Anatomy likelihood of CBDS (see Fig. 1) and a tentative plan for CCY, both Patients with surgically altered anatomy (e.g., Billroth II gastrectomy, IOUS and IOC are acceptable options with high accuracy in skilled Roux-en-Y gastric bypass [RYGB]) preclude standard access to the hands. duodenum. ERCP in this population requires advanced expertise and use of special endoscopic equipment such as single- or dou- ble-balloon enteroscopy or spiral enteroscopy. Alternatively, lapa- MANAGEMENT roscopically assisted, transgastric ERCP (LATG-ERCP is a useful option. Choledocholithiasis following RYGP is one of the most General Considerations common such scenarios. All CBDS that have not passed into the duodenum spontaneously LATG-ERCP may be performed with four ports: three 5-mm need to be evacuated. Timing is dictated by severity of symptoms. ports across the midabdomen (a camera port and two working In general, more symptomatic cholangitis warrants more urgent ports) and a 12- to 15-mm port in the left upper quadrant for the clearance of the obstructing CBDS, and patients with grade II or III duodenoscope. Retraction of the liver segments 2/3 with a Nathan- cholangitis according to the 2018 Tokyo Guidelines need emergent son retractor or sutures may be needed. After the excluded gastric drainage. Selection of management strategy is largely dependent on remnant is identified and dissected from adhesions, two to three availability of local surgical and endoscopic instruments, expertise, stay sutures are placed in the anterior wall and pulled through the and accessible referral centers. ERCP is most commonly used and abdominal wall with a laparoscopic suture-passer. A purse-string provides CBD clearance in more than 90% of patients. suture may be placed around the area of future gastrotomy, which is Data from recent systematic reviews and meta-analyses com- made with an energy device. Pneumoperitoneum is then decreased, paring a two-stage approach (pre- or postoperative ERCP with and a 12- to 15-mm port (one with a balloon tip for better anchoring CCY) versus single-stage procedure (CCY with CBDE) vary. Both and seal is useful) is placed through the gastrotomy while pulling on approaches share comparable morbidity (13%–16%), mortality the stay sutures for good apposition of the excluded stomach and (0.7%–1%), and failure rates. The single-stage approach may have a abdominal wall. If a CCY is also being performed, a guidewire may shorter length of hospitalization, fewer reinterventions, and reduced be passed through a cystic ductotomy into the duodenum to facilitate costs but a higher rate of postoperative bile leak. smooth CBD cannulation in a laparoendoscopic rendezvous (LERV) Percutaneous transhepatic cholangiogram (PTC) and biliary approach (see earlier and later discussions). After the ERCP, the gas- drainage (PTBD) serve as a last resort when the biliary tree cannot trotomy is closed with sutures or a stapler. be drained endoscopically or surgically to stabilize the patient with cholangitis, alleviate biliary obstruction, and temporize for transfer Laparoendoscopic Rendezvous Approach to a referral center with available expertise and resources. An alter- LERV is a relatively novel, single-stage approach to CBD clearance, native to PTBD is endoscopic ultrasound-guided choledochoduo- facilitating endoscopic cannulation of the CBD by a guidewire passed denostomy, which, however, is rarely used in nonmalignant CBD through the CD into the duodenum during CCY. According to a obstruction. recent meta-analysis, the LERV approach is associated with signifi- cantly decreased morbidity than a two-stage approach with ERCP then CCY (7.5% vs. 14.5%), decreased incidence of acute pancreatitis Endoscopic CBD Clearance (1.5% vs. 6.4%), and shorter hospital stay (mean difference 3.5 days). Conventional ERCP is performed with a side-viewing duodenoscope The LERV and the two-stage approaches were equivalent in CBD and, in expert hands, successfully clears the CBD in 90% of cases. clearance (90%–95%), conversion to open (5%–8.4%), postopera- After papilla identification, the CBD is cannulated and cholangiog- tive cholangitis (1.6%–2.6%), bleeding (1.2%–1.9%), and bile leak raphy detects CBDS or sludge, which may be cleared with a sphinc- (1.9%–2.1%). terotomy and sweeping of the CBD with a balloon catheter. In cases of distal CBD strictures or hematologic contraindications to sphinc- terotomy, balloon dilation may be useful. Larger stones may require Surgical CBD Clearance retrieval by endoscopic basket or fragmentation by lithotripsy. Surgical clearance of CBDS and CCY may also be done with a single- Factors associated with failure of endoscopic CBD clearance or two-stage approach. Single-stage CBD clearance (CCY + CDBE) include impacted, large (>15 mm) or multiple (>4) stones, periamp- offers shorter length of hospitalization and is likely cost-effective, ullary diverticula, distal CBD strictures, altered gastric or duodenal while sharing similar morbidity and mortality with the two-stage anatomy, and advanced age. approach (isolated CCY with pre- or post-CCY ERCP). The choice Recurrence of CBDS, as well as calculous cholecystitis, cholangi- is up to the surgeon and based on available resources, expertise, tis, and biliary colic, are all expected consequences of failure to per- and logistics. A minimally invasive surgery CBDE (MIS-CBDE) form CCY after endoscopic clearance of secondary CBDS, and thus shares the same contraindications as any other MIS. CDBE should CCY is recommended within 2 to 4 weeks post-ERCP, ideally during be avoided in patients with a small (5 mm) nontortuous CD, a lateral insertion into the CBD, FIG. 3 Balloon dilation of the cystic duct in preparation for transcystic common bile duct exploration and stone extraction. See text for details. FIG. 2 Intraoperative cholangiogram showing filling of the common bile FIG. 4 Transcystic stone extraction with basket. (From Qandeel H, et al. duct and the left and right hepatic ducts, but absent filling of the duodenum Basket-in-catheter access for transcystic laparoscopic bile duct exploration: tech- due to a distal stone obstructing the duct, with a meniscus sign (arrow). nique and results. Surg Endosc. 2016;30:1958–64.) Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 480 Management of Common Bile Duct Stones available, a flexible choledochoscope (see earlier) may be used for direct visualization of CBD and controlled retrieval of stones. The choledochoscope should ideally have video with an integrated light source and picture-in-picture capabilities. With available expertise and equipment, MIS-CDBE may be coupled with mechanical, elec- trohydraulic, or laser lithotripsy. An endoscopic retrieval bag and/ or gauze sponge close to the ductotomy may facilitate collection of debris. After a satisfactory completion IOC, the CD is transected and closed, preferably with an endoscopic loop. If a transcystic approach fails to achieve CBD clearance or is otherwise inappropriate, the sur- geon may proceed with a transcholedochal MIS-CBDE, convert into open CBDE (OCBDE), or plan for postoperative ERCP or PTBD. Transcholedochal Approach Unlike a transcystic CBDE, transcholedochal CBDE provides access to both the CBD and the hepatic ducts. However, transcholedochal CDBE is associated with a higher risk of biliary strictures and post- operative bile leaks, and is contraindicated when CBD diameter is 5 mm, and it is contraindicated if the CBD have been used for distally impacted CBDS but are rarely performed diameter is ≤3 mm. currently. The abdomen is entered through an upper midline or right sub- costal incision, and the liver is retracted superiorly. An IOC should be performed before CBDE. After IOC and characterization of the Suggested Readings CBD and contents, the duodenum is Kocherized and all portions of Hope WW, Fanelli R, Walsh DS, et al. SAGES clinical spotlight review: intra- CBD are gently palpated. IOUS may be useful to identify CBDS. Stay operative cholangiography. Surgical Endoscopy. 2017;31(5):2007–2016. Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved.  G a l l b l a d d e r a n d B i l i a ry T r e e   481 Narula VK, Fung EC, Overby DW, et al. Clinical spotlight review for the man- Shmelev A, Axentiev A, Hossain MB, Cunningham SC. Predictors of agement of choledocholithiasis. Surgical Endoscopy. 2020;34(4):1482–1491. same-admission cholecystectomy in mild, acute, biliary pancreatitis. HPB Parthasarathy M, Maqsood H, Sill AM, et al. Abandoning Hasty Conclusions: (Oxford). 2021 S1365-182X(21)00102-7. The Use of Magnetic Resonance Cholangiopancreatography in Clinical Zerey M, Haggerty S, Richardson W, et al. Laparoscopic common bile duct Practice. J Am Coll Surg. 2016;222(3):326–328. exploration. Surgical Endoscopy. 2018;32(6):2603–2612. Management of Acute of Oddi acts as a barrier against ascending infection from duodenal reflux. The causes of acute cholangitis can therefore be conceptual- Cholangitis ized as related to stasis from biliary obstruction or to direct seeding of the biliary tree (Table 1). In the setting of acute inflammation, increased biliary pressure Rebecca Tang, MD, Erica Barnett, BA, and David Berger, MD causes increased permeability of biliary ductules and translocation of bacteria from the portal venous system into the biliary tract. As INTRODUCTION a result, infection in acute cholangitis is typically polymicrobial and most commonly includes gram-negative rods of colonic origin. The Acute cholangitis is a clinical syndrome first described by Jean-Mar- most common bacteria identified in biliary cultures are Escherichia tin Charcot in 1877 characterized by abdominal pain, fever, and coli (25%–50%), Klebsiella (10%–20%), Enterococcus (10%–30%), jaundice from stasis and infection in the biliary tract. In 2006, the and Enterobacter (5%–10%). Anaerobes such as Bacteroides and Tokyo International Consensus Meeting consolidated literature Clostridia are more commonly seen in patients with recurrent infec- about acute cholangitis into evidence-based guidelines, which were tions or biliary instrumentation. most recently updated in 2018. The underlying permeability of the biliary system facilitates bac- terial translocation from the biliary tract to the portal and systemic EPIDEMIOLOGY venous systems, leading to sepsis in severe cases. Acute cholangitis most commonly occurs in the fifth through sev- enth decades of life with equal prevalence in men and women. The DIAGNOSTIC WORKUP most common causes of acute cholangitis include choledocholi- thiasis (40%–70%), malignancy (10%–60%), or benign stricturing History and Physical Examination (5%–30%). Cholangitis can also occur after instrumentation from Less than 60% of patients present with the complete Charcot’s either endoscopic retrograde cholangiopancreatography (ERCP) triad of fever, right upper quadrant pain, and jaundice. The most (1%–7%) or placement of biliary stents and indwelling biliary drains. common presenting symptoms are fever and right upper quad- rant pain (80%) and jaundice (60%). Patients may rarely (5%) PATHOPHYSIOLOGY present with additional altered mental status and hemodynamic instability to complete Reynold’s pentad. The differential diag- The primary issue in acute cholangitis is obstruction of the common nosis for patients presenting with symptoms of acute cholangitis bile duct. Under physiologic conditions, bile remains sterile due to includes acute cholecystitis, Mirizzi’s syndrome, biliary leak, continuous flow, secretion of immunoglobulin A in the biliary tree, acute pancreatitis, liver abscess, right lower lobe pneumonia, and and the presence of bacteriostatic bile salts. In addition, the sphincter empyema. TABLE 1 Causes of Acute Cholangitis Biliary Stasis Seeding of Biliary Tree Intrinsic obstruction Disruption of sphincter of Oddi Choledocholithiasis Endoscopic retrograde cholangiopancreatography Stricture (benign or malignant) Sphincterotomy Tumor (cholangiocarcinoma) Biliary stent insertion Stent occlusion Biliary reconstruction after choledochal surgery Polyp Biliary drain placement Blood clot Infectious parasite Food impaction Extrinsic Obstruction Mirizzi’s syndrome Tumor (pancreatic, ampullary, gallbladder, or duodenal cancer) Duodenal periampullary diverticulum (Lemmel syndrome) Chronic pancreatitis Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 482 Management of Acute Cholangitis Laboratory Workup Magnetic resonance cholangiopancreatography (MRCP) is the The laboratory workup for suspected acute cholangitis should best noninvasive imaging modality for identifying causes of biliary include CBC, BMP, electrolytes, LFT, PT-INR, β-hCG, blood cul- obstruction, characterizing biliary strictures, and detecting choled- tures, and biliary cultures. Leukocytosis is most commonly seen. ocholithiasis. MRCP is 90% sensitive and 95% specific in detection Liver function tests most often demonstrate a cholestatic pattern of of choledocholithiasis, although its sensitivity decreases significantly abnormalities, although a mixed hepatocellular pattern may be seen with small stones 1.5 Hematologic: Plt 12,000/mm3 or 2 cm < 2 cm given that only approximately one-third of general surgeons in the United States routinely use intraoperative cholangiography. The cur- rent literature is mixed, with one large retrospective study of nearly E1 E2 E3 100,000 patients showing no statistically significant association between intraoperative cholangiography and bile duct injury, with several smaller studies demonstrating decreased rates of bile duct injury when intraoperative cholangiography is performed. Recent prospective data suggest that intraoperative cholangiography can be performed routinely without a significant increase in case duration or morbidity. However, the question of whether intraoperative chol- angiography actually prevents bile duct injury remains controversial. Finally, other intraoperative techniques for imaging biliary anatomy, including laparoscopic ultrasonography or near-infrared biliary imaging using indocyanine green or white light technology, have been reported but are not routinely used due to lack of familiarity and high-level evidence. E4 E5 FIG. 3 Classification scheme for benign biliary strictures. Type A, Cystic Classification of Biliary Injuries duct leaks or leaks from small ducts in the liver bed. Type B, Occlusion of part of the biliary tree, typically clipped and divided right hepatic ducts. There are several anatomic classification systems for describing Type C, Transection (but not ligation) of the aberrant right hepatic ducts. biliary injuries. The most widely used system in the prelaparoscopic Type D, Lateral injuries to major bile ducts. Type E1, Common hepatic era was described by Bismuth based on the location of the injury duct division, more than 2 cm from bifurcation. Type E2, Common hepatic in the biliary tract with respect to the hepatic bifurcation. After duct division, less than 2 cm from bifurcation. Type E3, Common bile duct the popularization of laparoscopic cholecystectomy, when injuries division at bifurcation. Type E4, Hilar stricture, involvement of confluence became more common and more complex, Strasberg and colleagues and loss of communication between right and left hepatic duct. Type E5, proposed a modified classification system (Fig. 3). Involvement of aberrant right hepatic duct alone or with concomitant Although not specifically described within a classification stricture of the common hepatic duct. schema, it is important to discuss the scenario of concurrent vas- cular injury at the time of biliary injury, which has been reported in up to 15% of cases. Although biliary injuries are typically caused the common hepatic duct. Arterial injury can lead to biliary ischemia by direct operative trauma, they can also be ischemic in origin or a and may propagate the injury to a higher level than the mechanical combination of both. The right hepatic artery is the most common injury on the bile duct. Given the impact this can have on successful structure involved in a vasculobiliary injury given its proximity to bile duct repair, it is imperative to assess the hepatic arteries when Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 488 Management of Benign Biliary Strictures investigating any major biliary injury. In the postoperative period, cholangiography and biliary drainage is necessary. Although this can arterial phase contrast computed tomography (CT) can usually be difficult in a nondilated biliary system, this step is crucial. Once provide adequate delineation of the arterial anatomy. Excessive external catheter drainage is obtained, there is no urgency to inter- dissection around the bile duct during cholecystectomy or bile duct vene operatively. In most cases, 6 to 8 weeks is an appropriate amount anastomosis can disrupt the arterial supply of the bile duct that of time to allow the inflammation in the area of the transected/ travels in the 3 o’clock and 9 o’clock positions. This can lead to the leaking duct(s) to resolve. Percutaneous biliary catheters that control development of late strictures and should be avoided. Finally, there the bile leak and associated sepsis are also extremely helpful for the has been an unfortunate increase in “catastrophic biliary-vascular intraoperative identification of the hepatic ducts in a proximal biliary injuries” seen over the last decade. Such cases involving injury to the injury. For injuries above the hepatic duct bifurcation, both right and bile duct and the arterial and portal venous system result in rapid left main hepatic ducts should be stented individually. and progressive clinical deterioration of the patient. Urgent liver In patients with cholangitis, usually a later presentation, biliary transplant often offers the only chance of patient survival. drainage to control sepsis is the priority, followed by percutaneous or endoscopic retrograde cholangiopancreatography (ERCP) to define the biliary anatomy. In patients with biliary obstruction without infec- Recognition of Bile Duct Injuries tion, magnetic resonance cholangiopancreatography (MRCP) can Immediate recognition of bile duct injuries can avoid the develop- be used to define the anatomy noninvasively. In this scenario, repair ment of complications in many cases. However, only approximately without placement of a biliary catheter is considered appropriate. one-third of biliary injuries are recognized at the time of laparo- scopic cholecystectomy. Most bile duct injuries present in a delayed fashion with signs and symptoms of biliary leak or obstruction, and Operative Repair of Bile Duct Injuries and their management depends on the nature of the injury and the mode Strictures and timing of the presentation. The primary goal of operative management for bile duct injuries is to establish biliary flow into the proximal gastrointestinal tract, which is Intraoperative Recognition best accomplished with a tension-free anastomosis between healthy When a biliary injury is noted intraoperatively during a laparoscopic tissues. The technique for operative repair depends on the extent and cholecystectomy, immediate repair should be performed if the sur- location of the injury. Options for reconstruction include end-to-end geon is experienced with these procedures. If not, then consultation repair, Roux-en-Y hepaticojejunostomy, or hepatico- (choledocho-) with a hepatobiliary surgeon is warranted, even if this requires trans- jejunostomy. ferring the patient to another institution. If an experienced team is available, prompt cholangiography should be performed to further Segmental or Accessory Duct Injuries characterize the bile duct injury, followed by operative repair as For segmental or small accessory duct injuries where cholangiog- described in the subsequent section. It is appropriate to postpone the raphy demonstrates segmental or subsegmental drainage of the repair in some situations even if recognized during cholecystectomy. injured ductal system, simple ligation of the injured duct is adequate. Examples include a very proximal injury, diminutive bile ducts, sig- A larger duct (greater or equal to 4 mm) is likely to drain multiple nificant inflammation, or technical limitations of the surgeon requir- hepatic segments, thus requires reconstruction. ing transfer of the patient to another surgical team. In this scenario, the key technical principles are to provide appropriate and effective Partial Injury to the Bile Duct drainage and avoid propagation of the injury. A catheter (such as In some cases, the injury is limited to a lateral injury. These can best a pediatric feeding tube) should be placed into the proximal tran- be repaired over a T-tube with absorbable suture (typically 5-0) and sected duct and loosely secured if possible without further damage drained externally. Another scenario involves a patient who has had to the duct. This catheter will assist in controlling the bile leak and a clip placed without an injury to the duct or a small hole in the duct allow future access for cholangiography. The surgeon should avoid from performance of an intraoperative cholangiogram. Such cases, ligation of the bile duct to prevent necrosis and a more challenging in the experience of the authors, can be managed with removal of definitive reconstruction. Closed-suction drains should be placed in the clip, repair of the ductotomy, and external drainage. If stricture the subhepatic space to control the bile leak and prevent peritonitis develops either early or late postoperatively, endoscopic dilation and or biloma formation. stenting will usually manage the condition successfully. Most bile duct injuries during laparoscopic cholecystectomy occur during procedures by surgeons who are not experienced with Transection of the Common Bile Duct complex biliary reconstruction. Data suggest the best outcomes The most common ductal injury involves complete transection of the after iatrogenic biliary injuries occur from centers with experienced common bile duct or common hepatic duct. For such injuries recog- hepatobiliary surgeons and interventionalists. Thus, the decision to nized at the time of laparoscopic cholecystectomy with appropriate transfer care to a more experienced surgeon is entirely appropriate, expertise available, immediate repair should be performed if ductal and full disclosure to patients and their families, including the ratio- length can be maintained without tension. All repairs performed at nale behind a decision for transfer, is paramount. the time of initial operation should be drained externally. If the injured bile duct segment is short (less than 1 cm) and the Delayed Presentation two ends can be opposed without tension, an end-to-end anastomo- The majority of patients with an iatrogenic biliary injury present in sis can be performed. A generous Kocher maneuver, mobilizing the the early postoperative period with symptoms such as fever, jaun- duodenum out of the retroperitoneum, will help approximate the dice, abdominal pain, or peritonitis. The management of the injury ends of the bile duct. A T-tube can also be placed through a separate depends on the timing and character of the presentation; however, in choledochotomy either above or below the anastomosis (Fig. 4). A almost all scenarios, immediate return to the operating room is not disadvantage of this approach is that it is associated with a high risk indicated. In most situations, a CT scan upon arrival is necessary to of stricture formation. However, an end-to-end anastomosis is less identify fluid collections, bile ascites, or a vascular injury. Patients complex and allows future endoscopic transampullary intervention with an intraperitoneal bile leak typically present within 72 hours including balloon dilation and stenting, which have excellent long- of the initial operation. The bile leak should be controlled with per- term outcomes. cutaneous drainage of any collections, and then the biliary anatomy For proximal injuries or those greater than 1 cm in length, the should be defined with cholangiography, either endoscopically or distal bile duct should be oversewn, the proximal bile duct debrided, percutaneously. In patients with complete transection, percutaneous and an end-to-side Roux-en-Y hepaticojejunostomy performed Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved.  G a l l b l a d d e r a n d B i l i a ry T r e e   489 (Fig. 5). A Roux-en-Y jejunal limb is preferred over an anastomosis General Approach to Repair of Bile Duct Injuries to the duodenum because, in the latter case, an anastomotic leak Recognized in the Postoperative Period results in a duodenal fistula. In most cases, a transanastomotic stent The definitive repair of a bile duct transection recognized in the should be placed across the anastomosis and certainly in cases when postoperative period and controlled by external biliary drainage two ducts have been transected. Such catheters can be easily placed should not be planned until the patient has fully recovered from retrograde through the duct, exited through the surface of the liver, any sepsis or significant inflammation associated with a bile leak. In and then placed through the abdominal wall for external drainage. many cases, a period of 6 to 8 weeks is allowed before repair. These The anastomosis is typically constructed with interrupted 4-0 or patients will have complete bile diversion during this period, so fluid, 5-0 absorbable sutures. Closed suction drains are placed near the electrolytes, and hydration must be monitored. If there has been no anastomosis. bile leak, repair can occur at any time postoperatively. The abdomen is explored through a midline incision. Usually there are significant adhesions in the right upper quadrant. If biliary stents are placed preoperatively, they can be used as a guide to help the surgeon identify the transected duct, which can be difficult in a reoperative field. Catheters should be advanced by the interventional radiologist across the transected segment into the subhepatic space Liver or up to the transected end if the duct was completely clipped. After identification of the injured duct, the surgeon then dissects the bile duct proximal to the injury circumferentially in a cephalad direction for approximately 5 mm. Excessive proximal dissection should be avoided to prevent ischemia to the future anastomosis. A guidewire is placed in the catheter, and appropriately sized soft T-tube in common Silastic stents can be placed over the guidewires after dilation of the bile duct system with a series of Coudé catheters. Silastic stents, sized 12 to 22 French, which have side holes through a portion of their length, can be placed with the perforated aspect of the catheter in the Roux- en-Y jejunal limb and intrahepatic biliary tree and the nonperforated aspect passed through the liver parenchyma and brought out exter- Duodenum nally through the anterior abdominal wall. Biliary decompression in the early postoperative period can minimize the consequences of an anastomotic leak and provide access for postoperative cholangiogra- phy and future therapeutic intervention if indicated. After the stent has been placed, the anastomosis is performed in an end-to-side fashion, typically using interrupted 4-0 or 5-0 absorbable suture. FIG. 4 If the injured segment of the bile duct is short (70% of all (ERCP), percutaneous transhepatic cholangiography (PTC), mag- CCs and consisting of dilations of the extrahepatic biliary tree. Type netic resonance imaging (MRI), and magnetic resonance cholan- II cysts comprise about 1% to 3% of all CCs and consist of a saccular giopancreatography (MRCP) are each used to varying degrees with diverticulum off of the extrahepatic biliary tree. Type III cysts (cho- variable sensitivity and specificity to diagnose cystic disorders of the ledochoceles) consist of biliary ductal dilation within the duodenal bile ducts. A CBD diameter of >10 mm in an adult on any imaging wall and make up 1% to 3% of all CCs. Recent evidence suggests that modality should alert practitioners to the possibility of CC; however, choledochoceles represent a separate entity from the remainder of this guideline should be used with caution in older patients, as bile CCs. Due to the unique demographics, presentation, pancreatico- duct diameter increases with age. biliary ductal anatomy, and management of choledochoceles, many US is the most frequently used imaging modality to diagnosis authors have suggested they should not be included in the general CC and is the first imaging study recommended in the pediatric classification system for CCs (Fig. 3). Type IV cysts are the second population given its high sensitivity in this population, low cost, and most common type of cyst and make up 24% of all CCs. These cysts avoidance of radiation exposure. Although both US and CT have a consist of cystic dilation of the intrahepatic and extrahepatic biliary sensitivity of >90% for diagnosing CC, these studies are limited in tract. Type IV cysts are further subdivided into type IVa, consisting their ability to delineate the exact pathologic anatomy, including of multiple intrahepatic cysts and a single extrahepatic cyst, while identification of APBDJ. IVb consists of multiple extrahepatic cysts. Type V cysts, also known Cholangiography, including ERCP and PTC, represents the most as Caroli’s disease, comprise 1% to 3% of CCs and consist of multiple sensitive technique to delineate biliary anatomy. ERCP permits intrahepatic cysts. direct visualization of the pancreaticobiliary junction, decompres- sion of jaundice, and sampling of biliary epithelium. PTC is also CLINICAL PRESENTATION sensitive in delineating the anatomy of the intrahepatic bile ducts but is more invasive than ERCP and requires extended percutane- CCs present most classically in female infants and young children, ous drainage. Both ERCP and PTC are invasive procedures that are though presentation in adulthood is relatively common. The classic operator dependent and may be difficult to perform in the pediatric triad of presenting symptoms includes right upper quadrant pain, population. jaundice, and abdominal pain. However, this triad is rarely docu- MRCP is becoming the preferred imaging modality due not only mented in modern series, and perhaps more importantly, presen- to its noninvasiveness, high sensitivity (70%–100%), and specificity tation differs between children and adults. Children are more likely (90%–100%), but also to its superiority in identifying the precise to present with jaundice and/or abdominal mass, while adults are pathologic anatomy, including APBDJ. more likely to present with pain, cholangitis, and malignancy. Acute pancreatitis can also be a presenting symptom, most commonly in WHEN IS A DILATED COMMON BILE the case of choledochoceles. DUCT A CHOLEDOCHAL CYST? DIAGNOSIS A common clinical conundrum is the presentation of a patient with dilated CBD. The natural dilation of the CBD with age and after Diagnosis of CCs relies heavily on imaging findings, as clinical symp- cholecystectomy may make differentiating true CC pathology from toms overlap with many other pancreaticobiliary and gastrointestinal benign biliary dilation challenging. In these settings, MRI/MRCP Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved.  G a l l b l a d d e r a n d B i l i a ry T r e e   493 FIG. 2 Todani Classification of choledochal cysts. A-E, Types I-V. (Courtesy IUSM Office of Visual Media; copyright The Trustees of Indiana University.) is extremely helpful. In the absence of long common channel, the bypass. Figure 4 illustrates a case of biliary dilation raising initial diagnosis of CC is extremely unlikely. concern for CC (Fig. 4A). Subsequent imaging showed no APBDJ, The differential diagnosis of biliary dilation also includes sphinc- and the bile duct decompressed completely after endoscopic sphinc- ter of Oddi spasm, most commonly seen in females after gastric terotomy (Fig. 4B). Downloaded for Nikita Machado ([email protected]) at ClinicalKey Global Guest Users from ClinicalKey.com by Elsevier on May 31, 2024. For personal use only. No other uses without permission. Copyright ©2024. Elsevier Inc. All rights reserved. 494 Management of Cystic Disorders of the Bile Ducts anatomic relationship is particularly important should the surgeon operate laparoscopically. All operations for type I CC should involve preoperative counseling regarding the potential need for pancreatic head resection. Following transection of the distal CBD, the superior portion of the cyst should be mobilized to the level of the common hepatic duct bifurcation. Transection of the duct at the level of the confluence is generally recommended (Fig. 6B). However, any abnormality of the hepatic duct above the level of the bifurcation should prompt further dissection until the surgeon is able to identify a normal duct, and transection should occur proximal to this point. The proximal and distal bile duct margins should be sent for frozen section testing to exclude cholangiocarcinoma at the margin. Bili- ary-enteric anastomosis is accomplished via end-to-side Roux-en-Y hepaticojejunostomy (HJ) (Fig. 6C). Preoperative percutaneous cholangiography and biliary drainage may be considered in cases of type I cysts. Advantages include pre- operative delineation of biliary anatomy and easier intraoperative FIG. 3 Endoscopic view of type III choledochal cyst (choledochocele). identification of the hepatic duct confluence. Transhepatic biliary drainage may be continued postoperatively to decompress the HJ during the early healing period. MANAGEMENT The presence of a CC is in itself an indication for surgery. CCs are Type II: Extrahepatic Biliary Diverticulum associated with increased risk of cholangitis, pancreatitis, and malig- Type II CCs are not typically associated with APBDJ and thus nant degeneration into cholangiocarcinoma. The risk of malignant carry a relatively lower risk of malignant transformation. For this degeneration is felt to be related to APBDJ; reflux of pancreatic reason, resection of the CBD during surgical management is not secretions into the biliary tree results in inflammation, dysplasia, required. Rather, diverticulectomy with CBD closure at cyst neck and ultimately carcinoma of the biliary epithelium. Stasis of bile provides adequate surgical management. Closure of the bile duct within CC also likely contributes to ongoing inflammation and over a T-tube may be useful in managing a small (normal-sized) malignant transformation. The incidence of cholangiocarcinoma bile duct. has been reported to be as high as 75% in certain series from Japan, with higher risk in patients with intrahepatic involvement (Fig. 5). Surgical management is tailored to specific type of CCs but generally Type III: Choledochocele includes cholecystectomy, complete resection of the CC, and biliary Contemporary understanding of this rare condition suggests that enteric anastomosis. choledochocele is a discrete entity from CC. Endoscopic sphincter- otomy for choledochoceles was first performed in 1981 and in the decades since has been shown to a be a safe, effective, and durable Type I: Extrahepatic Bile Duct Cyst means of managing choledochoceles. Although no clearly established The surgical treatment for Todani type I bile duct cysts begins link between choledochocele and malignancy exists, two case reports with cholecystectomy, after which a Kocher maneuver should be have identified ampullary (1) and pancreatic (2) malignancy in asso- performed to allow for visualization of the CBD and CC (Fig. 6A). ciation with choledochoceles. Dissection follows the CBD distally to the caudal portion of the cyst. The question of how far distally to resect the CBD is an important clinical challenge. The ideal situation includes complete resection Type IV: Intrahepatic and Extrahepatic Bile Duct of the cyst to its point of insertion into the main pancreatic duct. Cyst Intraoperative US may be helpful in identifying the distal most Like type I cysts, type IV cysts carry a significant risk of malig- aspect of the cyst and bile duct. Understanding and identifying this nant transformation, and surgical management is the primary FIG. 4 Magnetic resonance images demonstrating a case of biliary dilation concerning for choledochal cyst (A) and subsequent decompression after endo- A B scopic sphincterotomy (B). D o w n l o a d e d f o r N i k i t a M a c M a y 3 1 , 2 0 2 4. F o r p e r s o n a  G a l l b l a d d e r a n d B i l i a ry T r e e   495 treatment. The diffuse nature of type IV CCs, with both intra- and with biliary-enteric anastomosis. If the disease is unilobar, partial extrahepatic components, presents a somewhat more demanding hepatectomy is the appropriate treatment. Bilobar disease should clinical challenge. The extrahepatic portion can be treated as out- be managed with aggressive surveillance with serial imaging and lined before when discussing type I CCs: complete cyst resection CA 19-9 levels, understanding that, like Caroli’s disease, definitive management of bilobar involvement requires orthotopic liver trans- plantation (OLT). Type V: Caroli’s Disease Type V CCs also represent a significant clinical challenge. Some authors have suggested managing the asymptomatic patient with aggressive surveillance including serial imaging and CA 19-9 levels. Most patients diagnosed with Caroli’s disease will eventually also experience portal hypertension, cholangitis, and cholangiocarci- noma. Cholangitis related to intrahepatic cyst disease can be man- aged with antibiotics, biliary drainage via large bore stents, and stone extraction. However, these approaches should be considered tempo- rary measures because they do not mitigate the risk of malignancy. OLT is the definitive surgical management for patients with type V CCs. Timing of OLT remains a primary question for the multidisci- plinary clinical care team and should be individualized to the specific patient condition. Morbidity of Surgical Therapy Short-term morbidities following surgical therapy for CCs include wound infection, intraabdominal infection, postoperative panc