Endoscopic retrograde cholangiopancreatography (ERCP) is a combined endoscopic and radiologic procedure used for both diagnostic, and more commonly, therapeutic interventions within the pancreatic and biliary tree. The procedure utilizes the simultaneous acquisition of endoscopic and fluoroscopic images, and radiographic images are obtained and stored for documentation and clinical use.
Initially developed as a diagnostic procedure, the current practice of ERCP has seen a shift to an almost exclusively therapeutic role, with modalities such as computed tomography (CT), magnetic resonance imaging (MRI), and endoscopic ultrasound (EUS) being used to noninvasively image the pancreas and bile ducts first. Nonetheless, ERCP can still provide valuable diagnostic information in selected cases.
Increased experience over the last 10 years with the use of ERCP by dedicated endoscopists in tertiary care centers has resulted in an extremely high success rate, coupled with a low complication rate, in the performance of diagnostic and therapeutic procedures in both adults and children. Optimal performance of ERCP requires skilled endoscopists who are familiar with the equipment and techniques combined with significant experience. With the infrequent use of ERCP in children, pediatric gastroenterologists have rightly relied on experienced colleagues in adult and pediatric gastroenterology for this procedure; at most centers, pediatric ERCP procedures are thus performed by adult gastroenterologists.
Pancreaticobiliary Imaging Methods
Several imaging modalities are available for the diagnosis of pancreaticobiliary disease. Appropriate selection of imaging technique depends on the underlying pathologic process.
Transabdominal ultrasonography (US) is often the first test used to evaluate the biliary tree despite some key shortcomings. US has the advantages of being inexpensive, portable, well tolerated in patients of all ages, and readily available. The procedure also requires no sedation or even intravenous access. US is the method of choice for diagnosing cholelithiasis in patients of any age. Although US is helpful in detecting and estimating the degree of biliary and pancreatic ductal dilation or in detecting some pseudocysts, it may fail to provide accurate information about the distal common bile duct and head of the pancreas due to overlying bowel gas. As such, US is a poor study if choledocholithiasis is a clinical concern as the common bile duct (CBD) may be inadequately imaged.
Abdominal CT is useful for evaluating the degree and level of biliary obstruction. It provides superior imaging of the pancreatic parenchyma and presence of pancreatic ductal obstruction as compared to US. However, CT often fails to detect gallstones because of their similar density to the surrounding bile and should not be utilized if the presence or absence of choledocholithiasis is the primary question.
Magnetic resonance cholangiopancreatography (MRCP) has become extremely useful and, more importantly, widely available, for imaging the bile and pancreatic ducts. MRI and MRCP are noninvasive and can provide detailed information about the patient’s anatomy and the presence of obstruction, stricture, neoplasm, or injury following trauma. MRCP can be a valuable tool in diagnosing primary sclerosing cholangitis (PSC) and can demonstrate pathologic findings in the intrahepatic and extrahepatic bile ducts. MRCP is an excellent noninvasive tool for the identification of choledocholithiasis. Disadvantages of MRCP include the need for sedation in younger or uncooperative patients and contraindication in patients with metallic implants.
Percutaneous transhepatic cholangiography (PTC) is an invasive procedure that also has excellent diagnostic accuracy, but because of its invasiveness, it is usually reserved for use in patients who require therapeutic intervention that cannot be achieved with ERCP. Access to the biliary tree is achieved by passing a needle through the liver and into an intrahepatic duct, and from there into the main biliary tree. Dilation of the biliary tree facilitates biliary access in patients undergoing PTC, while PTC can be difficult to achieve in patients with nondilated ducts. Although imaging of the pancreatic duct is generally not achieved unless an abnormally long pancreaticobiliary junction is present, occasionally a percutaneous cholangiogram results in pancreatic duct opacification in the absence of distal bile duct obstruction. Because almost all therapeutic interventions following PTC require an external drain (which is poorly tolerated by most patients, especially children), PTC is generally reserved for those cases when access to the duodenum is not possible or when ERCP or traversal of a guidewire across an obstruction fails. Complications of PTC include bleeding, bile leakage around the percutaneous catheter, entry site into the abdomen with resultant bile peritonitis, and dehydration and electrolyte abnormalities from excessive fluid loss due to external biliary drainage.
EUS is a valuable and increasingly available tool for the evaluation of gastrointestinal diseases in adults and is being used more commonly in children. EUS provides high-fidelity images of the esophagus, mediastinum, pancreas, bile ducts, ampulla, the rectum, and the perirectal space. EUS has also been used to evaluate the spleen, the adrenal glands, and the kidneys to a limited extent. EUS, like ERCP, requires additional advanced endoscopic training and is typically performed by specially trained adult gastroenterologists. Tissue can be obtained during EUS by passing a needle under direct ultrasound guidance into areas of interest, such as lymph nodes and masses (fine needle aspiration, or FNA).
The role of EUS in pediatric patients with pancreaticobiliary disorders has been studied both retrospectively and prospectively. EUS and EUS-guided FNA were found to be feasible and safe and to significantly alter subsequent management in the majority of children in the pediatric series reported to date.
EUS is also a useful modality that offers the precise evaluation of portal hypertension in orthotopic liver transplantation (OLT) candidates. EUS displays abnormal vessels that develop in the circulation called “deep varices,” which have a high risk of bleeding in patients with cirrhosis and are not detected with routine ultrasonography.
Indications for ERCP
The primary indication for ERCP in neonates and young infants is the evaluation of cholestasis in cases where the diagnosis is not established by other modalities. Although liver biopsy has a very high sensitivity with a lower specificity for the diagnosis of neonatal cholestasis, it is complemented by ERCP in cases where bile duct abnormalities are noted on imaging studies. Use of MRI with MRCP has supplanted diagnostic ERCP in many patients, but ERCP plays a role when imaging studies are not feasible or are suboptimal.
Diagnostic and/or therapeutic biliary indications for ERCP in children older than 1 year of age and in adolescents include, but are not limited to, obstructive jaundice, high clinical suspicion of or known choledocholithiasis, suspected primary sclerosing cholangitis in the setting of inflammatory bowel disease when MRCP is nondiagnostic, treatment of bile ductal leaks after blunt abdominal trauma or cholecystectomy, treatment of anastomotic biliary strictures and leaks following liver transplantation, and for the evaluation and treatment of abnormal imaging studies (US, CT, or MRCP).
Pancreatic indications for ERCP in children include, but are not limited to, pancreatic duct strictures and/or stones that can be seen in patients with acute and/or chronic pancreatitis; evaluation of persistently increased levels of pancreatic enzymes; evaluation of abnormal imaging studies; treatment of pancreatic fluid collections; and the evaluation and treatment of pancreatic ductal leaks from blunt abdominal trauma, surgical injury, or other causes. Box 62-1 outlines the indications for ERCP. It should be stressed that EUS and MRI with MRCP provide excellent pancreatic imaging and should be utilized when available if the indications for ERCP are not clear. Diagnostic ERCP in children with acute recurrent pancreatitis can be performed but should be done only in select patients in whom there is a high suspicion of pancreatic ductal disease that would benefit from endoscopic therapy. ERCP should not be used for the investigation of chronic pain not suspected to be of pancreaticobiliary origin.
CONGENITAL ANOMALIES *
* Initial presentation may not be at birth or in infancy.
Biliary atresia vs. neonatal hepatitis
Alagille syndrome and paucity syndrome
Congenital hepatic fibrosis
Caroli’s disease and Caroli’s syndrome
Biliary strictures
Choledochal cyst
Treatment of pancreas divisum
Diagnosis of annular pancreas
Cystic dilation of the pancreatic duct (pancreatocele)
Acquired Diseases
Treatment of benign biliary strictures
Removal of bile duct stones
Treatment of biliary complications after liver transplantation
Diagnosis and treatment of primary sclerosing cholangitis
Biliary obstruction due to parasitic infestation
Parasitic infestation: Ascaris
Diagnosis and treatment of sphincter of Oddi dysfunction
Diagnosis and treatment of pancreatic trauma
Treatment of acquired immune deficiency syndrome cholangiopathy
Treatment of chronic pancreatitis
Drainage of pancreatic pseudocysts
Contraindications to ERCP
Relative contraindications to ERCP are coagulation disorders that are severe or uncorrectable. Absolute contraindications to ERCP include inability to obtain informed consent; cardiovascular, respiratory and neurologic instability; luminal perforation (although in some cases luminal perforation can be closed during ERCP); and esophageal, gastric, or duodenal obstruction precluding passage of the duodenoscope to the level of the papilla. Surgically altered anatomy of the gastroduodenal and/or pancreaticobiliary tract (e.g., Roux-en-Y choledochojejunostomy with orthotropic liver transplantation) may make ERCP difficult or impossible to perform, although newer endoscopes (double balloon enteroscopes) and increasing experience are making these procedures achievable in more patients.
Contraindications to ERCP
Relative contraindications to ERCP are coagulation disorders that are severe or uncorrectable. Absolute contraindications to ERCP include inability to obtain informed consent; cardiovascular, respiratory and neurologic instability; luminal perforation (although in some cases luminal perforation can be closed during ERCP); and esophageal, gastric, or duodenal obstruction precluding passage of the duodenoscope to the level of the papilla. Surgically altered anatomy of the gastroduodenal and/or pancreaticobiliary tract (e.g., Roux-en-Y choledochojejunostomy with orthotropic liver transplantation) may make ERCP difficult or impossible to perform, although newer endoscopes (double balloon enteroscopes) and increasing experience are making these procedures achievable in more patients.
Preparation for ERCP
As it is often difficult to predict which tools and accessories will be needed for a given ERCP; an endoscopy suite with fluoroscopy and a well-stocked complement of necessary endoscopic accessories is required. Equipment for monitoring vital signs, oxygen saturation, and medications as well as equipment for resuscitation is essential. Occasionally ERCP is done in an operating room, and the same equipment is needed. Pediatric endoscopy assistants and specially trained nurses are required for assistance in handling accessories such as guidewires and catheters. One team member, often an RN, should be present whose sole purpose is monitoring the patient and administration of medication. More often, a pediatric anesthesiologist is available to ensure proper, adequate sedation and airway control to optimize the smooth performance of the procedure. The primary goals of most sedation regimens for pediatric endoscopic procedures are to ensure a patient’s safety, comfort, and cooperation continuously throughout the procedure.
Preparation for ERCP is the same as for upper gastrointestinal endoscopy except for the administration of antibiotic prophylaxis in selected cases and the potential need for preprocedural laboratory work. Antibiotic prophylaxis for cardiac conditions is given according to the current guidelines of the American Heart Association for children with congenital and rheumatic heart disease. Antibiotics are also administered to prevent infectious complications of cholangitis or pancreatic abscess formation from injection of contrast material into potentially inadequately drained spaces, such as in those with biliary obstruction in whom inadequate drainage is anticipated (primary sclerosing cholangitis, hilar obstruction) and those with pancreatic pseudocysts. Antibiotics should be administered if there is any additional concern about infection on the part of the treating physicians.
Current guidelines should be followed for children with a valvular prosthesis, vascular graft material, and indwelling catheters, or status following organ transplantation, and in immunosuppressed patients. A period of fasting precedes sedation and intubation with the endoscope as per published guidelines.
Secondary and often desirable goals of sedation are to affect periprocedural amnesia, maximize procedural efficiency, minimize recovery times, and maintain cost-effectiveness. Premedication can be administered to sedate the child, minimize discomfort, and induce amnesia. Anesthesia support is most often used in children undergoing ERCP because it is a complex procedure and requires the patient to remain relatively motionless.
There are two main types of sedation for pediatric endoscopy: general endotracheal anesthesia and conscious sedation. Conscious sedation can be administered by the endoscopist (moderate sedation) or an anesthesia team member (monitored anesthesia care, or MAC).
Conscious sedation regimens include the use of intravenous meperidine, fentanyl, or versed, or a combination thereof. Nurse administered propofol is also becoming widely used as well in some states, and is at least as safe as the use of narcotics and benzodiazepines in adults, although this has not yet been studied in pediatric patients. For sedation best practices, children undergoing gastrointestinal endoscopy should also be classified in accordance with guidelines defined by the American Society of Anesthesiologists (ASA) to identify patients who will be best served by general anesthesia. Patients can be divided into age groups as follows: younger than 6 months, 6 months to 3 years, school-aged (4 to 11 years), and adolescents. Infants 6 months of age and younger may have little anxiety and tend to sedate easily. Infants older than 6 months who have developed “stranger anxiety” may be more easily sedated if parents remain next to them during induction. Sedation for endoscopic procedures in infants and young children should be performed only by individuals with significant pediatric sedation and airway management experience and appropriate credentialing, as children may progress rapidly from conscious sedation to a level of sedation consistent with general anesthesia, and airway issues are especially challenging in this age group. School-aged children manifest “concrete thinking” and may be surprisingly difficult to sedate because of higher anxiety levels than may be appreciated. Older children and teenagers can usually understand the nature of the procedure and are often calm. Modes of sedation include conscious sedation, MAC, and general anesthesia. No one modality is ideal, and sedation choices should be made based on institutional practices, guidelines, and preferences. Sedation for endoscopy is discussed in greater detail in Chapter 61 .
Technique of ERCP
Standard adult side-viewing endoscopes (duodenoscopes) can be used in many children older than 1 year of age, and in most children older than 3 to 4 years of age. Adolescents easily accommodate a full-sized adult instrument. Specialized smaller diameter video pediatric side-viewing instruments are available for use in infants younger than 1 year of age, but these may have to be special ordered from endoscope manufacturers. The disadvantages of such small-caliber endoscopes are the decrement in optics and a small working channel diameter. Furthermore, these devices have only a limited number of endoscopic accessories.
The patient is placed in the prone or supine position (based on endoscopist and anesthesia preference) to allow ideal fluoroscopic views of the pancreaticobiliary tree. After adequate sedation, a plastic bite-block is inserted between the teeth. After the endoscope is guided into the descending duodenum, the papilla is brought into view so that the desired target duct may be cannulated from an en face position. Specialized catheters and/or guidewires are manipulated into the papilla, and contrast material is injected to outline the duct(s) of interest (biliary and/or pancreatic duct). Care must be taken to remove any air in the contrast syringe, because introduction of air into the ducts will appear as rounded filling defects and mimic stones.
Special tapered-tip cannulas should be available for pediatric patients and for cannulation of the accessory (minor) papilla if access to the duct of Santorini is necessary. Most endoscopists use a sphincterotome instead of a cannula as a primary bile duct cannulation device because this results in faster cannulation of the selected duct and can be used to perform a sphincterotomy if indicated as well. Selected fluoroscopic images are saved, and therapeutic maneuvers are performed as required.
After removal of the endoscope, placing the patient in the supine position or in the right lateral decubitus position facilitates filling of the upper hepatic ducts with the use of gravity. Spot images may be obtained without the endoscope in the field to ensure full visualization. Cholangioscopy can be performed using very small-caliber endoscopes passed through the working channel of the duodenoscope, and may be of value in patients with large common bile duct or pancreatic duct stones that require lithotripsy. Cholangioscopy may also facilitate tissue acquisition in patients with PSC or bile duct cancer, the latter of which is extremely rare in pediatric patients.
Biliary Disorders
Diagnosis of Biliary Atresia and Neonatal Hepatitis
Neonatal cholestasis is defined as prolonged conjugated hyperbilirubinemia that occurs in the newborn period. It results from diminished bile flow and/or excretion. The neonatal liver is susceptible to a wide variety of injuries, and the histologic reaction is characteristic but nonspecific. Liver biopsy may demonstrate cholestasis, giant cell transformation, inflammation, hepatocellular necrosis, extramedullary hematopoiesis, fibrosis, and bile duct proliferation. Proliferation of bile ducts on liver biopsy is typical for biliary atresia, but percutaneous liver biopsy may result in insufficient tissue for diagnosis, and early biopsy may not be diagnostic due to lack of specificity.
The findings on biopsy of several cholestatic liver diseases may overlap in early infancy. The differential diagnosis of idiopathic neonatal obstructive cholangiopathy is critical in the first weeks of life because early hepatoportoenterostomy in patients with biliary atresia is known to reduce long-term morbidity and mortality.
Exclusion of infectious, metabolic, and other causes of prolonged cholestasis in the newborn will leave 70% to 80% of patients with conjugated hyperbilirubinemia without an identified etiology. The key differentiation in these patients is between extrahepatic biliary atresia and neonatal hepatitis, which account for most of the cases. These conditions are discussed in detail in Chapter 17 , Chapter 67 , Chapter 68 .
Extrahepatic biliary atresia (BA) is rare, occurs in one in 10,000 to 20,000 births, and is characterized by inflammation of bile ducts leading to progressive obliteration of the extrahepatic biliary tract leading to cirrhosis and hepatic failure. BA represents the most common indication for pediatric liver transplantation, representing more than 50% of cases in most series. This condition has a very poor prognosis and may be associated with other congenital defects; in that case it is called embryonal BA. Without treatment, patients with BA will ultimately develop cirrhosis, and rapid identification of this condition is necessary because the success rate of establishing bile flow declines with surgical procedures performed after 2 months of age. Increased age at surgery has a progressive and deleterious effect on the results of the Kasai operation until adolescence. These findings provide a basis for early recognition of BA to reduce the need for liver transplantation in infancy and childhood.
None of the currently available tests or combinations thereof is 100% reliable in diagnosing BA. Laparotomy with intraoperative cholangiography and wedge hepatic biopsy has been considered the best available method to establish the presence or absence of BA. If BA is identified, a Kasai procedure can be performed. The chief disadvantage of this approach is that some infants with neonatal hepatitis will undergo surgery to exclude BA, whereas others with diseases such as arteriohepatic dysplasia (Alagille syndrome) will undergo hepatoportoenterostomy but remain cholestatic. More recently, cholangiography has been performed laparoscopically with or without liver biopsy in the diagnosis of BA and is another way to obtain cholangiography. Finally, PTC can also provide a diagnosis of BA.
ERCP is a direct method of establishing a diagnosis of biliary continuity. ERCP in neonates can be technically challenging. However, in expert hands, ERCP can be safely performed, even in the smallest infants. Cholangiographic features of BA are illustrated in Figures 62-1 through 62-4 .
One large series was recently published in which ERCP was performed in 140 consecutive patients who were younger than 6 months of age (mean age 60 days, weight 4 kg) with suspected extrahepatic cholestasis over a 7-year period. ERCP diagnosis was correlated with intraoperative findings. ERCP excluded BA in 34 (25%) but failed in 18 newborns (13%) for technical reasons. No severe complications occurred. Exploratory laparotomy was performed in 106 patients (75%) and revealed BA in 80 (75% of the operated patients). Thus, the sensitivity of ERCP for diagnosing biliary atresia was 92%, with a specificity of 73%, suggesting that although ERCP is not a substitute for noninvasive imaging, it has the potential to help avoid unnecessary surgery in almost 25% of cases. Hence, some recommend ERCP, if available, before exploratory laparotomy in all patients with suspected BA. The approach to BA and use of ERCP differs from center to center, but ERCP is feasible and safe in the evaluation of neonatal cholestasis when other imaging modalities are inconclusive. In addition, despite the expanding role of MRCP, there may still be a role for ERCP in the multidisciplinary workup of these patients, especially in those with an atypical presentation and in those with a nondiagnostic evaluation before surgery. One major drawback in patients with BA is that an inability to visualize the biliary tree may be due to technical reasons as well as complete obliteration of the bile duct, thus rendering a negative study inconclusive.
Choledocholithiasis
Common bile duct stones rarely occur in infants and children. Symptomatic choledocholithiasis, usually associated with cholelithiasis, is the predominant indication for ERCP in children ( Figure 62-5 ). Laparoscopic cholecystectomy is now commonly performed in children for treatment of symptomatic cholelithiasis. ERCP is performed preoperatively when patients present with cholangitis and pancreatitis, and when there are confirmed stones or there is a clinical suspicion of choledocholithiasis based on symptoms and imaging studies. EUS and MRI/MRCP can help identify patients with choledocholithiasis in whom labs and transabdominal ultrasound are indeterminate. ERCP is performed in the early postoperative period when bile duct stones are identified by intraoperative cholangiography and have not been removed intraoperatively by laparoscopic methods, which in practice is an extremely common situation. Finally, ERCP is frequently performed in patients undergoing postcholecystectomy who present with choledocholithiasis.
Black pigment stones are usually found in children with hematologic conditions that favor biliary pigment cholelithiasis, whereas light-colored cholesterol stones are more typical in adults.
Conditions associated with the presence of stones include biliary tract malformations such as choledochal cyst, chronic liver disease, hemolysis, and progressive familial intrahepatic cholestasis type 3 (PFIC3). Bile duct stones may occur without any known predisposing conditions and may be identified incidentally when imaging of other organ systems is performed. Asymptomatic neonatal cholelithiasis may resolve spontaneously, and even asymptomatic choledocholithiasis can resolve in some circumstances without the need for aggressive intervention. Choledocholithiasis, even in an asymptomatic patient, often warrants stone removal because bile duct stones may become impacted in the distal common bile duct, resulting in clinical and biochemical obstructive jaundice and/or pancreatitis, even in infants. In the setting of ascending cholangitis, urgent ERCP should be undertaken.
In patients with suspected choledocholithiasis, EUS and/or MRCP are useful for diagnosis, but ERCP allows for stone extraction. Bile duct stones are most commonly removed by performing endoscopic biliary sphincterotomy using electrosurgical generators followed by the use of endoscopic accessories to remove the stones immediately thereafter. Passage of retrieval balloons or baskets into the bile duct allows withdrawal of the stones into the duodenum ( Figure 62-6 ). Endoscopic sphincterotomy with stone removal using balloons and/or baskets is effective in more than 90% of patients. Balloon dilation of the biliary sphincter (sphincteroplasty) rather than sphincterotomy might seem to be an appealing alternative in young children because the long-term effects of sphincterotomy performed in childhood are unknown, although the risk of pancreatitis is higher. In North America, biliary sphincter dilation without an antecedent biliary sphincterotomy is uncommonly performed.
Choledochal Cyst
A choledochal cyst is a malformation of the biliary tract characterized by abnormal dilation (fusiform, saccular types) of the biliary tree. Often these cysts are accompanied by an anomalous pancreaticobiliary junction (APBJ). However, APBJ may also be found incidentally without cystic changes in the bile duct or in association with acute recurrent pancreatitis.
The etiology of choledochal cysts remains obscure, and it is speculated that an anomalous connection of the pancreatic and bile ducts may be a factor in the development of these cysts. Choledochal cysts are often an incidental finding. The classic triad of intermittent abdominal pain, jaundice, and a right upper quadrant abdominal mass is found in the minority of patients. More commonly, these lesions are sometimes identified in patients being evaluated for abdominal complaints that cannot always be explained by the cyst itself.
Choledochal cysts are classified according to the method proposed by Todani et al. ( Figure 62-7 ). Type 1 choledochal cyst is the most common and accounts for 90% of all choledochal cysts. Congenital cystic dilation of the common bile duct is seen, and the terminal common bile duct is frequently narrowed as it enters the duodenum. Type I cysts vary in size and shape. Type II cysts are rare congenital diverticula of the common bile duct and do not typically produce jaundice.
The term choledochocele is applied to a type III cyst that is limited to the small intraduodenal segment of the common bile duct that herniates into the duodenal lumen. The clinical presentation is intermittent cholangitis and/or pancreatitis, although some patients may be asymptomatic. Type III cysts are generally treated by endoscopic biliary sphincterotomy to promote drainage and reduce stasis of bile in the cyst itself.
Caroli’s disease is classified as type IVA biliary cystic dilation with multiple intrahepatic and extrahepatic cysts; type IVB is cyst in the extrahepatic duct system only. Solitary liver cysts make up a type V lesion.
Choledochal cysts have the potential for development of primary bile duct cancer (cholangiocarcinoma). Therefore, complete surgical excision of the cyst with the formation of a Roux-en-Y biliary enteric anastomosis is often recommended and should be performed as early as possible to prevent complications. Patients with type III cysts often undergo biliary sphincterotomy alone and rarely undergo surgery. Long-term follow-up is required for surveillance for late complications and for cancer, particularly in type IV and V choledochal cysts where complete excision is not possible.
ERCP and MRCP are of value in outlining these cysts and their relationship to both ductal systems, and in detecting anomalous union of the duct. These studies allow the surgical approach to be planned. EUS and CT do not provide the same quality of anatomic details, but can provide information about size, contour, position, and the presence of stones.
Biliary Strictures
Primary Sclerosing Cholangitis (PSC)
Primary sclerosing cholangitis (PSC) is a chronic, insidious cholestatic liver disease of uncertain etiology, characterized by inflammation and progressive obliterative intrahepatic and/or extrahepatic bile duct fibrosis. It may lead to cirrhosis, end-stage liver disease, cholangiocarcinoma, and the need for liver transplantation. In children, the incidence of PSC is reported to be 0.23 cases per 100,000 person-years compared with 1.11 cases per 100,000 person-years in adults, but this may be an underestimation as children with PSC may not undergo evaluation and early disease may be clinically silent. PSC in children is commonly associated with inflammatory bowel disease (ulcerative colitis and Crohn’s disease) and is the most common hepatic complication of primary immunodeficiency disorders. PSC is associated primarily with colitis; patients with Crohn’s disease that is isolated to the small bowel are unlikely to develop PSC. Patients with PSC may present with pruritus, right upper quadrant pain, fevers, or clinical features that are indistinguishable from autoimmune hepatitis. The diagnosis of PSC is most commonly made via cholangiography and/or liver biopsy.
Liver histopathologic evaluation can suggest the presence of large duct obstruction in PSC but frequently cannot provide a specific diagnosis, as can ERCP. The accuracy of ERCP to diagnose PSC in children is the same as in adult patients. Characteristic radiographic findings include diffuse strictures of the intrahepatic or extrahepatic biliary tree, or both ( Figure 62-8 ).
