Therapeutic endoscopic retrograde cholangiopancreatography for giant bile duct stones in a child: A case report

Abstract

BACKGROUND

Therapeutic endoscopic retrograde cholangiopancreatography for giant common bile duct stones with pancreaticobiliary maljunction (PBM) in children remains challenging. As a congenital anomaly, PBM is characterized by anatomical variants that may predispose affected children to recurrent pancreatitis and adversely affect growth and development.

CASE SUMMARY

A 6-year-old boy was admitted with nonprojectile vomiting and intermittent abdominal pain that had begun one week before admission. Yellowing of the skin and sclerae developed four days before admission followed by fever with a peak temperature of 38.8 ℃ and lethargy two days before admission. No pruritus, joint swelling or pain, diarrhea, bloody stools, abdominal distension, rash, or petechiae was reported. After admission, abdominal color ultrasound and magnetic resonance cholangiopancreatography were performed. Laboratory testing revealed a white blood cell count of 16.3 × 10⁹/L, a neutrophil percentage of 91.5%, and a C-reactive protein level of 68.68 mg/L. The clinical diagnoses included acute suppurative cholangitis, biliary pancreatitis, common bile duct stones, PBM, and pancreas divisum. Symptoms were relieved after endoscopic retrograde cholangiopancreatography-based stone extraction and biliary decompression.

CONCLUSION

In this case, an individualized endoscopic stone-extraction strategy combined with endoscopic sphincterotomy was successfully used to remove the biliary stones and relieve the obstruction. The patient’s clinical symptoms resolved rapidly after the procedure, thereby providing favorable conditions for subsequent definitive surgery.

Key Words: Child; Endoscopic retrograde cholangiopancreatography; Common bile duct stones; Pancreaticobiliary maljunction; Case report

Core Tip: This case describes the successful therapeutic endoscopic retrograde cholangiopancreatography-based extraction of giant common bile duct stones in a child with concomitant pancreaticobiliary maljunction and pancreas divisum. A precisely tailored endoscopic approach can achieve effective biliary decompression and rapid symptom resolution while avoiding urgent surgery. This report supports the feasibility and safety of endoscopic retrograde cholangiopancreatography for themanagement of complex pediatric biliary anomalies.

INTRODUCTION

Common bile duct (CBD) stones are relatively uncommon in children, although their incidence has gradually increased in recent years. Conventional endoscopic techniques, including endoscopic sphincterotomy (EST) combined with basket stone extraction, have traditionally achieved favorable outcomes; however, stone extraction becomes considerably more difficult when the stone diameter exceeds 1 cm, often requiring advanced endoscopic techniques such as mechanical or laser lithotripsy. Congenital pancreatobiliary anomalies, including pancreaticobiliary maljunction (PBM), may cause pathologic reflux that promotes stone formation and increases the risk of biliary infection and pancreatitis, thereby increasing the complexity of clinical management. At present, no standardized therapeutic strategy has been established for complex giant CBD stones with PBM. With advances in endoscopic technology, endoscopic retrograde cholangiopancreatography (ERCP) has become the preferred approach for managing biliary obstruction in experienced medical centers because of its minimally invasive nature and high therapeutic efficacy. However, endoscopic experience in pediatric patients, particularly those with complex anomalies such as PBM and pancreas divisum, remains limited. This report describes a 6-year-old child with giant CBD stones, approximately 1.8 cm in diameter, with concomitant PBM and pancreas divisum. After preoperative imaging was performed to delineate the pancreatobiliary anatomy, ERCP combined with EST and endoscopic papillary balloon dilatation (EPBD) was performed, resulting in successful stone extraction and biliary drainage. This case provides clinically relevant evidence for endoscopic management and long-term follow-up in children with complex pancreatobiliary malformations.

CASE PRESENTATION

Chief complaints

A 6-year-old boy was admitted with a one-week history of vomiting and intermittent abdominal pain, yellowing of the skin and sclerae for four days, and fever for two days.

History of present illness

One week before admission, nonprojectile vomiting developed without an identifiable precipitating factor, occurring predominantly after meals and accompanied by intermittent abdominal pain that was mainly localized to the right upper quadrant and varied in intensity. Four days before admission, yellowing of the skin and sclerae was observed, accompanied by yellow discoloration of the urine and stool. Two days before admission, fever developed, with a peak temperature of 38.8 °C; the fever was unresponsive to antipyretic therapy, and lethargy was noted.

History of past illness

The child had previously been healthy, with no clinically significant medical history.

Personal and family history

No history was reported of exposure to epidemic areas, contaminated water, or infectious sources; blood product transfusion; or exposure to radiation, toxic substances, or specific medications.

Physical examination

Physical examination on admission showed the following. Temperature: 38.7 °C, pulse: 135 bpm, respiratory rate: 36 breaths/minute, blood pressure: 113/72 mmHg, body weight: 17 kg, body mass index: 13.8 kg/m².

The patient was conscious but poorly responsive; yellowing of the skin and sclerae was present; no rash or petechiae was observed; respiration was regular; the abdomen was soft with tenderness in the right upper quadrant; no muscle guarding or rebound tenderness was detected; Murphy’s sign was positive; the liver was palpable approximately 2 cm below the costal margin and was soft in consistency; the spleen was not palpable below the costal margin; bowel sounds were normal; and no lower-limb edema was observed.

Laboratory examinations

Complete blood count: White blood cells 16.3 × 10⁹/L, neutrophil percentage 91.5%, C-reactive protein: 68.68 mg/L.

Procalcitonin: 0.70 ng/mL, interleukin-6: 29.00 pg/mL, erythrocyte sedimentation rate: 21 mm/hour.

Biochemical indicators: Total bilirubin 141.4 μmol/L, Conjugated bilirubin 68.9 μmol/L, Unconjugated bilirubin 21.3 μmol/L, alanine aminotransferase: 650.0 U/L, alanine aminotransferase: 383.0 U/L, alkaline phosphatase: 475.0 U/L, gamma-glutamyl transpeptidase: 555.0 U/L, serum amylase: 170 U/L, serum lipase: 1228.0 U/L.

Imaging examinations

Abdominal ultrasound: Dilatation of the intrahepatic bile ducts was observed; the left hepatic duct measured approximately 15.4 mm in diameter, and the right hepatic duct measured approximately 11.3 mm. The CBD measured approximately 17.3 mm at its widest point and contained an isoechoic structure measuring approximately 15.3 mm × 8.7 mm. A hyperechoic structure measuring approximately 18.3 mm × 7.9 mm was identified distally, posterior to the pancreatic head.

Pancreas: Normal size and morphology were observed, with well-defined margins and homogeneous echotexture. Pancreatic duct dilatation was detected, with a maximum diameter of approximately 4.0 mm. No pancreatic space-occupying lesion was observed (Figure 1).

Figure 1 Abdominal ultrasound features of common bile duct stone with obstructive dilatation. A: Dilatation of the intrahepatic bile duct; B: Common bile duct dilatation with echogenicity visible within; C: Pancreatic duct dilatation.

Upper abdominal computed tomography: Dilatation of the common hepatic duct, left and right hepatic ducts, segments of the intrahepatic bile ducts, CBD, and pancreatic duct was observed. The CBD measured approximately 14.5 mm at its widest point. A nodular, slightly hyperdense shadow was identified at the distal CBD, with a computed tomography attenuation value of approximately 31 HU (Figure 2).

Figure 2 Preoperative abdominal computed tomography findings of common bile duct stones. A: Coronal computed tomography image demonstrating intrahepatic bile duct dilatation; B: Axial computed tomography image showing a calculus within the common bile duct.

Magnetic resonance cholangiopancreatography: Multifocal dilatation of the intrahepatic and extrahepatic bile ducts was observed; the left hepatic duct measured approximately 15.8 mm in width, the right hepatic duct approximately 12.5 mm, the common hepatic duct approximately 17.2 mm, and the CBD approximately 18 mm at its widest point. Signal heterogeneity was noted in the distal CBD, with a round filling defect measuring approximately 18 mm in diameter at the lower end. The pancreatic duct was dilated, approximately 7 mm, with proximal bifurcation; the accessory pancreatic duct drained into the minor duodenal papilla, whereas the main pancreatic duct joined the distal CBD (Figure 3).

Figure 3 Preoperative magnetic resonance cholangiopancreatography findings of biliary-pancreatic duct abnormalities. A: Dilated common bile duct with an intraluminal stone; B: Tortuously dilated main pancreatic duct; C: Pancreatobiliary maljunction; D: Bifurcation of the main pancreatic duct.

FINAL DIAGNOSIS

The patient was preliminary diagnosed with: (1) Acute suppurative cholangitis; (2) Obstructive jaundice; (3) CBD stones; (4) Biliary pancreatitis; (5) PBM; and (6) Pancreas divisum.

TREATMENT

MDT consultation

Gastroenterology: Intensive antimicrobial therapy, inhibition of pancreatic enzyme secretion, and fluid resuscitation/supportive care were recommended. After completion of the preoperative assessment, ERCP should be performed promptly to extract the stones, relieve the obstruction, and achieve biliary drainage. However, given the patient’s young age, the large size of the CBD stones, the technical complexity of the procedure, and the elevated risk of complications, the procedure must be performed with caution.

Ultrasound/radiology: Based on magnetic resonance imaging and ultrasound findings, pancreas divisum with PBM was suspected, and the current departmental treatment plan was recommended to be continued temporarily.

General surgery: Marked pancreatic duct dilatation suggested distal obstruction; ERCP was indicated for obstruction relief, followed by regular follow-up and surgical intervention if required.

The patient presented with severe abdominal pain and markedly elevated serum lipase levels; treatment included nothing by mouth, inhibition of pancreatic enzyme secretion, antimicrobial therapy, and fluid resuscitation. ERCP was scheduled after completion of the relevant preoperative examinations.

Under general anesthesia, the endoscope was advanced using the standard approach. The duodenal papilla was nipple-shaped, with a granular orifice. A sphincterotome was advanced into the CBD over a guidewire, followed by contrast injection. Marked dilatation of the intrahepatic and extrahepatic bile ducts was observed, with a maximum diameter of approximately 1.8 cm. Multiple large filling defects each measuring approximately 1.8 cm in diameter, were identified in the distal CBD. A triple-lumen sphincterotome was advanced over the guidewire, and EST was performed. A balloon dilation catheter was then introduced over the guidewire and inflated to 8 mm. After balloon deflation, a large volume of turbid bile and black, fragmented stones drained from the papillary orifice. A stone-extraction balloon was used to retrieve fragmented and sludge-like stones from the CBD. Repeated balloon sweeps of the CBD were performed, and follow-up cholangiography showed no residual filling defects. A 7f-5 cm pigtail stent was placed in the bile duct. Repeated attempts to cannulate the pancreatic duct via the minor papilla were unsuccessful; however, pancreatography through the minor papilla delineated the pancreatic duct and demonstrated contrast passage into the CBD, suggesting a confluence or communicating branch between the pancreatic and biliary ducts, although the guidewire could not be advanced into the pancreatic duct (Figure 4).

Figure 4 Endoscopic retrograde cholangiopancreatography combined with endoscopic sphincterotomy for common bile duct stone extraction. A: Cholangiography stones; B: Duodenal papillotomy; C: Balloon dilatation of the duodenal bulb; D: Common bile duct stones; E: Place a common bile duct stent; F: Paraphiliac ductography with simultaneous visualization of the pancreatic duct and bile duct.

OUTCOME AND FOLLOW-UP

The patient’s abdominal pain resolved rapidly after the procedure, body temperature normalized, and yellowing of the skin and sclerae improved substantially; murphy’s sign became negative. Follow-up testing showed that transaminase, bilirubin, and inflammatory marker levels had largely returned to the normal ranges, and abnormal imaging findings had markedly improved. Imaging performed 12 days after the procedure showed that the widest portion of the CBD measured approximately 6.6 mm, whereas pancreatic duct dilatation persisted, with a maximal internal diameter of approximately 1.8 mm (Figures 5 and 6).

Figure 5 Changes in inflammatory and biochemical markers before and after endoscopic retrograde cholangiopancreatography. WBC: White blood cell; CRP: C-reactive protein; ERCP: Endoscopic retrograde cholangiopancreatography; TBIL: Total bilirubin; ALT: Alanine aminotransferase; GGT: Gamma-glutamyl transpeptidase.

Figure 6 Follow-up abdominal ultrasound findings at 2 weeks post-procedure. A: Significantly reduced diameter of the common bile duct; B: Markedly decreased width of the pancreatic duct.

DISCUSSION

CBD stones in children are uncommon but clinically recognized, with an overall incidence of approximately 0.13%-0.22%[1]. However, giant stones with PBM are exceedingly rare and often manifest as obstructive jaundice or biliary pancreatitis, creating considerable diagnostic and therapeutic complexity. In this report, a pediatric patient with giant stones in the distal CBD complicated by obstructive jaundice and biliary pancreatitis is described, with PBM considered the underlying cause. Although surgical interventions, such as choledochotomy with stone extraction or choledochojejunostomy, have traditionally been favored for pediatric patients with giant CBD stones, the present case was successfully managed using ERCP. Complete stone extraction and stent placement enable effective drainage of bile and pancreatic juice while preserving the native structure and function of the pancreaticobiliary ducts, thereby supporting a feasible minimally invasive approach for giant CBD stones with rare congenital anomalies.

PBM is a congenital malformation characterized by the extra duodenal union of the pancreatic and bile ducts, resulting in an abnormally long common channel. This anomaly impairs sphincter of Oddi regulation at the junction, causing bidirectional reflux of pancreatic juice and bile and predisposing affected patients to cholelithiasis, cholangitis, and pancreatitis[2]. PBM is also associated with an increased risk of biliary malignancy. A multicenter study from Japan reported that, among patients with PBM and bile duct dilatation, the incidences of gallbladder cancer and bile duct cancer were 13.5% and 7%, respectively; among those without bile duct dilatation, the corresponding rates were 37.2% and 4%[3,4]. Pancreas divisum, another common congenital pancreatic anatomical variant, is also recognized as an important contributor to impaired pancreatic juice drainage. The coexistence of congenital pancreaticobiliary anomalies and giant CBD stones creates considerable diagnostic and therapeutic complexity.

Imaging evaluation is central to the diagnosis and management of complex pancreatobiliary disorders. Magnetic resonance cholangiopancreatography (MRCP) permits noninvasive delineation of pancreaticobiliary ductal anatomy and serves as a key modality for preoperative assessment[5,6]. In this case, MRCP not only identified the presence and size of the CBD stones but also clearly demonstrated the anatomical characteristics of PBM and pancreas divisum, providing clinically useful information for treatment planning. Subsequent ERCP confirmed the biliary anatomy and precisely localized the stones, therapy providing a reliable basis for safe endoscopic intervention. This case supports the clinical value of an imaging-guided endoscopic strategy in pediatric patients with complex pancreatobiliary anomalies. Relevant literature indicates that EST combined with EPBD, compared with either method alone, can shorten procedure time and improve both single-session and overall stone clearance rates. Balloon dilation after EST directs the dilation trajectory and improves procedural precision, thereby reducing procedure-related complications such as bleeding and perforation. Moreover, this combined technique causes less injury to the sphincter of Oddi, helps preserve its physiological function, and reduces the risk of postoperative biliary-enteric reflux and secondary infection[7,8]. Accordingly, adequate drainage of bile and pancreatic juice can be achieved while sphincter dysfunction caused by excessive sphincterotomy is avoided, thereby reducing the risk of cholangitis and pancreatitis.

In this case, the treatment strategy was directed primarily toward the biliary system. At admission, acute suppurative cholangitis and severe biliary obstruction were present, requiring urgent biliary decompression and infection control. Although pancreatic juice drainage may be impaired by pancreas divisum and PBM, these anomalies were not considered the direct cause of the patient’s acute symptoms. Therefore, the primary therapeutic objective was relief of biliary obstruction, whereas conservative management with close follow-up was adopted for pancreas divisum and PBM to avoid unnecessary intervention.

Although ERCP remains technically challenging in children, available studies indicate that, in well-equipped pediatric endoscopy centers staffed by experienced teams, ERCP is a safe and effective therapeutic modality for pediatric pancreatobiliary disorders. However, because of the technical complexity of the procedure, indications must be strictly followed and inappropriate use should be avoided[9]. In this case, after comprehensive preoperative imaging assessment and implementation of a standardized procedural protocol, post-ERCP abdominal pain and jaundice resolved rapidly, and serum lipase and amylase levels normalized within 48 hours. The initially abnormal imaging findings showed marked improvement, and the short-term outcome was favorable.

CONCLUSION

ERCP is a safe and effective therapeutic option for pediatric patients. In children with giant CBD stones accompanied by rare pancreatobiliary anatomical anomalies, such as PBM and pancreas divisum, comprehensive preoperative imaging assessment, particularly MRCP, is essential for individualized treatment planning. EST combined with EPBD represents a safe and effective minimally invasive approach, with a high technical success rate and a low risk of complications. However, because PBM and pancreas divisum may be associated with long-term pancreatobiliary complications, long-term follow-up is required.

ACKNOWLEDGEMENTS

The authors would like to convey their heartfelt appreciation to the researchers who generously shared the data we utilized.