Spontaneous Perforation of the Bile Duct in a Toddler: The Role of Endoscopic Retrograde Cholangiopancreatography in Diagnosis and Therapy

INTRODUCTION Spontaneous perforation of the bile duct (SPBD) or idiopathic bile duct perforation is a rare but potentially fatal disorder that requires urgent diagnosis and management. Most of the cases present insidiously in infancy, although as in our case, SPBD can occur in an older child. The more common case presentation is a healthy infant developing progressive symptoms. Less commonly, the presentation is that of an acute surgical abdomen. The typical age of patients with SPBD is between 2 and 20 weeks, although the age range varies from birth to 7 years (2,3,5–7,9–18). Although the etiology is often unknown, distal biliary obstruction from either a stone or stricture is a postulated mechanism. In cases of suspected distal biliary obstruction, biliary intestinal anastomosis is recommended (2,6,8,10–15). We present a case of a 34-month-old male who presented with an acute abdomen. Radiographic studies suggested a bile leak, and the patient was taken emergently to endoscopic retrograde cholangiopancreatography (ERCP) which provided the diagnosis of a bile duct leak at the junction of the common and cystic duct and allowed decompression and endoscopic treatment. CASE REPORT A 3-year-old, former 26-week preterm male infant presented to the emergency department after a 2-day history of abdominal pain, nausea and vomiting. The patient had been well until the acute onset 2 days before admission of postprandial pain, nausea and multiple episodes of nonbloody, nonbilious emesis. On the day before admission, he was diagnosed with acute gastroenteritis with instructions for home oral hydration. Continued pain and vomiting resulted in dehydration and hospital admission. There was no history of fever, respiratory symptoms, diarrhea, rash, ill contacts, possible ingestions or abdominal trauma. The patient had been receiving levothyroxine for hypothyroidism of prematurity since the neonatal period. The patient was twin B of a 26-week gestation and received approximately 12 weeks of intravenous nutrition in the neonatal intensive care unit. Twin A died during the neonatal period awaiting liver and small-bowel transplantation for cirrhosis and short-bowel syndrome after necrotizing enterocolitis. During physical examination, the patient was febrile, tachycardic, tachypneic and irritable. The patient weighed 15 kg. The abdomen was distended with voluntary guarding and severe discomfort with palpation and percussion. There was no hepatosplenomegaly, mass or palpable ascites. The rest of the examination findings was normal. Laboratory evaluation showed leukocytosis (18 000/μL), a normal platelet count and hemoglobin. Serum bicarbonate was 12 mmol/L; potassium, 3.0 mmol/L; glucose, 130 mg/dL; blood urea nitrogen, 18 mg/dL; aspartate aminotransferase, 132 IU/L; alanine aminotransferase, 178 IU/L; alkaline phosphatase, 366 IU/L; total bilirubin, 2.2 mg/dL and conjugated bilirubin, 0.5 mg/dL. The remainder of the serum chemistries, albumin, amylase and lipase were normal. Prothrombin time was elevated at 16.4 seconds. A computed tomographic scan of the abdomen with intravenous contrast (Fig. 1) showed a distended gallbladder with a significant amount of fluid surrounding the portahepatis, mesenteric edema and a dilated common bile duct of approximately 5 mm with a stone at the duodenal papilla. Intravenous fluid resuscitation and broad-spectrum antibiotics were started.FIG. 1: Computed tomographic scan image at initial presentation. Black arrow demonstrates common bile duct stone at duodenal papilla and adjacent fluid in peritoneal space.Because of a suspected perforation of the bile duct, the patient underwent emergent ERCP performed under general anesthesia. An Olympus TJF-160 duodenoscope was used to cannulate the major papilla with a sphincterome, and contrast injection demonstrated 2 small stones in the distal common bile duct. A sphincterotomy was performed over a guide wire, and the stones were extracted with a balloon extraction catheter. A balloon occlusion cholangiogram demonstrated a bile leak at the region of junction of the common hepatic duct and cystic duct (Fig. 2). A 5-cm 7F internal biliary stent was deployed across the leak.FIG. 2: Endoscopic retrograde cholangiopancreatography image after cholangiographic injection. White arrow demonstrates bile leak as white blush. Black arrow demonstrates 2 stones at distal common bile duct.The patient tolerated the procedure well and was treated with intravenous antibiotics, fluids and internal biliary drainage. The ileus and abdominal pain resolved by postoperative day 4, and the patient was discharged on day 7. Of note, the patient had no previous imaging studies, laboratory evidence or symptoms suggestive of choledocholithiasis despite such risks as prematurity and total parenteral nutrition. One month after discharge, the stent was removed at endoscopy, and a repeat cholangiogram was concerning for a possible stricture at the level of the common hepatic duct. The patient remained asymptomatic without further therapy. Six weeks later, the result of abdominal ultrasound was normal without evidence of ductal dilatation. The patient's clinical course and liver function test results, including γ-glutamyl transpeptidase, have remained normal for 11 months. DISCUSSION Although uncommon, SPBD is frequently cited as the second most common indication for jaundice requiring surgical intervention in infancy (2,3,5–15). Since the initial description by Dijkstra (1) in 1932, there have been approximately 150 cases described in the literature. Most of the patients have a normal newborn and perinatal course and present with insidious symptoms including abdominal distension, ascites, fluctuating acholic stools and jaundice, poor weight gain, emesis, intermittent fever, irritability and abdominal or inguinal hernias that can become progressively bile stained (2,3,6–9,11,13–18). Laboratory studies are variable but may be helpful. Serum aminotransferase levels and bilirubin can be normal or elevated, although alkaline phosphatase and γ-glutamyl transpeptidase are often elevated. There may be leukocytosis, anemia, thrombocytosis and elevations of inflammatory markers (2–9,11–17). Ultrasonography or abdominal computed tomography can identify ascites and biliary ductal dilatation, stones or sludge (2,4,6,8,15–17). If the patient is clinically stable, a radionucleotide hepatobiliary scan (ie, cholescintigraphy) is highly sensitive and specific for a bile duct leak (2–4,6–10,14–17). Spontaneous perforation of the bile duct is somewhat of a misnomer as the “spontaneity” of the perforation may be secondary to one or multiple etiologies as in our patient. These include trauma, ischemia, infection, distal bile duct stenosis or obstruction secondary to stones or sludge, reflux of pancreatic secretions secondary to pancreaticobiliary malformations and congenital malformation or weakness of the bile duct wall (2–15,17,18). First proposed by Johnston (18) in 1961 and further expanded by Lilly et al. (5) in 1974, the most commonly accepted unifying theory is that there exists a localized embryogenic defect at the junction of the common bile duct and cystic duct that is susceptible to a variety of insults and therefore prone to rupture. Most of perforations are on the anterior wall of the common bile duct at the junction of the cystic duct (2,4,5,8,9,11–15,17,18). Previously, there was almost universal consensus in the literature supporting surgical intervention for SPBD (2–18). During the initial exploration, an operative cholangiogram is performed to ascertain the location of the perforation and to assess for distal obstruction (2,3,5–8,10,11,13–15,17). For nonobstructive perforation of the common or hepatic duct, drainage of the biliary leak is recommended by placing an external drain at or near the site of the perforation without intervention to the bile duct itself (2,4–17). A cholecystostomy tube may be placed for postoperative management until the perforation has sealed (2,5–8,12,15,17). With simple conservative drainage, most of the perforations seal with decompression and drainage (2,4–15,17). In cases of SPBD with distal biliary obstruction unrelieved by the cholangiogram, most authors recommend a biliary-intestinal anastomosis (2,6–8,10–15,17). Surgical repair of the bile duct is controversial because of possible biliary stricture formation postoperatively, and T-tube drainage is no longer recommended (2,5,13,16). Endoscopic retrograde cholangiopancreatography has been a useful diagnostic and therapeutic procedure in children since the 1980s (19). Endoscopic retrograde cholangiopancreatography has long been used to both diagnose and treat choledocholithiasis (20–24). To date, we are unaware of any reports in the literature describing the role of ERCP in the diagnosis and primary treatment of SPBD in children. In our case, ERCP was used as the initial cholangiographic technique and the primary therapy, sparing the need for exploratory surgery. Although there are relatively few centers with the resources and personnel available for pediatric ERCP, this experience suggests that when the proper equipment and endoscopic expertise are available, ERCP may be an alternative therapy of SPBD in infants and children.