Preoperative cholangioscopic identification of traumatic type IVa choledochal cyst perforation after motor vehicle collision: A case report

Abstract

BACKGROUND

Choledochal cyst (CC) perforation is a rare and potentially life-threatening complication of an uncommon congenital biliary abnormality. There are few case reports in adults, especially in settings of traumatic injury. In all reports of traumatic CC perforation, the diagnosis was made during emergent laparotomy.

CASE SUMMARY

A 63-year-old female with right upper quadrant and epigastric abdominal pain following a motor vehicle collision presented to the emergency room, with imaging suggestive of choledocholithiasis without hepatic injury. Endoscopic retrograde cholangiopancreatography (ERCP) and direct cholangioscopy revealed a contained hemorrhage secondary to a perforated bile duct, which raised suspicion for perforated CC. This prompted evaluation with cholangiogram, and a cholecystostomy tube bridge to surgical excision of CC and hepaticojejunostomy. Intraoperative anatomical classification of the cyst and surgical pathology confirmed a perforated Type IVa CC, which was treated with surgical excision and Roux-en-Y hepaticojejunostomy and cholecystectomy. Symptoms did not reoccur within a three month follow-up period.

CONCLUSION

This case highlights the diagnostic value of adding cholangioscopy to ERCP in identifying occult biliary injury in hemodynamically stable patients presenting with right upper quadrant abdominal pain in settings of trauma, which was supported by extravasation on cholangiogram. Utilization of these tools can lead to prompt perioperative recognition and management.

Key Words: Choledochal cyst; Endoscopic retrograde cholangiopancreatography; Cholangioscopy; Traumatic rupture; Case report

Core Tip: Traumatic rupture or perforation of a previously undiagnosed choledochal cyst in an adult is a rare cause of biliary injury and may mimic common conditions such as choledocholithiasis. Hemobilia or trauma-associated biliary obstruction should raise suspicion for occult biliary tree injury. Endoscopic retrograde cholangiopancreatography combined with cholangioscopy can directly visualize subtle duct injuries not apparent on initial imaging. Cholecystostomy tube placement may be a helpful bridge to surgery by improving source control in clinically stable patients. Early evaluation with cholangioscopy can facilitate preoperative delineation of biliary anatomy, guiding early diagnostic accuracy of biliary cysts, excision, and reconstruction.

INTRODUCTION

Choledochal cyst (CC) is a rare congenital anomaly characterized by cystic dilation of the biliary tree. It has an incidence of 1 in 100000-150000 live births in the western population[1]. CC is associated with significant morbidity, including recurrent cholangitis, pancreatitis, and cholangiocarcinoma. Spontaneous perforation has a reported frequency of 1.8%-2.8%, with traumatic breaks rarely reported[2]. Prior to this report, there are only four documented cases in the literature, thus there is a knowledge gap in the diagnostic challenges of traumatic CC perforation. The Todani classification categorizes CC into five types based on their anatomic location and morphology of the biliary dilation. Type IVa cysts, characterized by combined intrahepatic and extrahepatic ductal dilatation, are among the more complex subtypes and carry a higher risk of complications. Surgical excision and biliary reconstruction are the definitive standards of care, though there is no formally standardized strategy[3].

Computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) are central to the initial evaluation of biliary pathology; however, subtle bile duct injuries or intraductal abnormalities may remain occult. We present a case of traumatic perforation of a previously undiagnosed Type IVa CC following a motor vehicle collision. Endoscopic retrograde cholangiopancreatography (ERCP) with direct cholangioscopy and fluoroscopic contrast extravasation was pivotal in identifying bile duct perforation, which raised suspicion for CC. This guided ultimate treatment with surgical exploration and excision.

CASE PRESENTATION

Chief complaints

A 63-year-old female presented to a community hospital’s emergency department with right upper quadrant and epigastric abdominal pain one hour after a motor vehicle collision.

History of present illness

Her vehicle was struck from the side at 40 miles per hour. She was wearing a seatbelt at the time of injury. She did not require intubation on scene. Her abdominal pain started immediately after the collision and gradually worsened in severity. She denied nausea, vomiting, fever, chills, or jaundice. Primary and secondary trauma surveys did not identify solid-organ or hollow-viscus injury, hepatic laceration, or active bleeding.

History of past illness

She had a history of iron deficiency anemia and generalized anxiety disorder.

Personal and family history

There was no prior history of biliary colic, known abnormal liver tests or abdominal imaging prior to presentation. Preadmission medications were noncontributory. She had no surgical history. She had no alcohol, tobacco, or drug use. There was no notable family or psychosocial history.

Physical examination

Vital signs were significant for hypertension at 169/83 mmHg. Physical exam was significant for mild epigastric tenderness to palpation. There were no signs of peritonitis, abdominal distension, or ecchymosis.

Laboratory examinations

Initial labs revealed hemoglobin of 11.1 g/dL without leukocytosis, elevated alanine aminotransferase (ALT) at 119 U/L and aspartate aminotransferase (AST) at 245 U/L, elevated alkaline phosphatase at 175 U/L, and elevated total bilirubin at 2.0 mg/dL. Two days after presentation, her hemoglobin decreased to 6.4 g/dL, she was transfused with 1 unit of packed red blood cells. Her ALT and AST increased to 245 U/L and 408 U/L, respectively. Her alkaline phosphatase and total bilirubin remained stably elevated.

Imaging examinations

On the day of presentation, CT of the abdomen demonstrated a hyperdense filling defect within the common bile duct (CBD) with upstream biliary dilation without evidence of hepatic laceration or other intra-abdominal injury. These findings were initially interpreted as choledocholithiasis; no traumatic bile duct injury was recognized at this time (Figure 1).

Figure 1  Coronal computed tomography of the abdomen showing a hyperdense filling defect in the common bile duct (arrowhead).

Due to the 4.7 g/dL drop in hemoglobin two days after presentation, further evaluation with ERCP (ED-580XT; Fujifilm; Lexington, MA, United States) was prompted. This revealed a large filling defect in the lower CBD with extrahepatic CBD dilation (Figure 2), which was subsequently evaluated with direct visualization of the bile duct with cholangioscopy (SpyGlass; Boston Scientific, Inc, Marlborough, MA, United States) advanced to the middle third of the main bile duct. A large intraductal blood clot and biliary wall disruption with contrast extravasation on fluoroscopy raised concern for a contained bile duct perforation from a CC (Figure 3).

Figure 2 Direct visualization of biliary tract via endoscopic retrograde cholangiopancreatography with fluoroscopy. A: Fluoroscopic image showing a large filling defect in the lower and middle third of the main bile duct (orange circle); B: Fluoroscopic image showing contrast extravasation and the guide wire escaping the normal confines of the biliary architecture, concerning for bile duct perforation (orange circle).

Figure 3 Direct visualization of biliary tract via endoscopic retrograde cholangiopancreatography with cholangioscopy. A: Cholangioscopic image showing partial suction of a large blood clot in the middle third of the common bile duct (CBD). The proximal bile duct could not be visualized beyond this point due to the large clot; B: Cholangioscopic image showing mucosal erythema in the CBD; C: Cholangioscopic image showing an intraductal guide wire in the distal CBD with active bleeding, concerning for hemobilia.

Clots were swept from the duct. One temporary pancreatic stent was placed in the ventral pancreatic duct to prevent post-ERCP pancreatitis. A CBD stent could not be placed due to poor visualization of the proximal CBD. The patient remained hemodynamically stable. The procedure was terminated, and an urgent CT angiogram on the same day of ERCP and cholangioscopy did not reveal any active extravasation or vascular injury. The following day, Interventional Radiology placed a cholecystostomy tube with a cholangiogram which showed active extravasation of bile, confirming a perforation of the bile duct wall (Figure 4). The findings of an upstream biliary dilation on the CT scan, a disrupted blood clot on cholangioscopy, and extravasation on the cholangiogram were concerning for a contained CC perforation rather than choledocholithiasis as initially suspected, prompting surgical evaluation.

Figure 4 Cholecystostomy cholangiogram showing common bile duct extravasation. Fluoroscopic images obtained via injection of contrast through percutaneously placed cholecystostomy tube. A: Early-phase image showing opacification of the gallbladder and proximal biliary tree, confirming the appropriate position of the tube; B: Progressive-phase image obtained with continued contrast injection, demonstrating extravasation of the contrast into an irregular, poorly marginated collection consistent with perforation of the bile duct and active bile leak.

FINAL DIAGNOSIS

Due to extrahepatic and intrahepatic dilation found intraoperatively, the CC was diagnosed anatomically as a type IVa CC according to Todani’s classification. Final surgical histopathology showed a cyst with walls of collagen and smooth muscle, notably an absence of a complete mucosal epithelial lining. This confirmed a perforated CC with acute and chronic inflammation, without dysplasia or malignancy.

TREATMENT

Surgical management was delayed due to the patient’s request for a second opinion. Nine days after presentation, the patient was subsequently transferred to a university-affiliated academic medical center with hepatobiliary surgical services, where she underwent abdominal laparotomy, surgical excision of the CC, and reconstruction of the biliary tree. During surgical exploration and repair, a 15 cm hematoma was identified in the right retroperitoneum, which was completely suctioned. A large cystic mass with a 5 cm perforation on the lateral wall distal to the cystic duct was discovered along the head of the pancreas extending to the hepatic bifurcation. Complete lymphadenectomy of the medial and lateral portal vein lymph nodes was required to identify the anatomy and excise the CC. The CC was approached from the medial aspect of the bile duct and tedious dissection was performed to completely excise the cyst with Roux-en-Y hepaticojejunostomy and cholecystectomy.

OUTCOME AND FOLLOW-UP

Following surgical exploration and repair, the patient remained hemodynamically stable without postsurgical complications. During her four-day postoperative stay, her liver enzymes and hemoglobin returned to normal range. The patient was hospitalized for a total of thirteen days. Three months after discharge, symptoms concerning for biliary leak, stricture, or cholangitis did not recur at follow-up appointments. Longer term monitoring is necessary to evaluate for residual intrahepatic disease, anastomotic complications, cholangitis, and malignancy risk. She continues to feel well and remains asymptomatic at the time of this report. The overall case timeline is shown in Figure 5.

Figure 5 Timeline of overall diagnosis and treatment course. CT: Computed tomography; ERCP: Endoscopic retrograde cholangiopancreatography; CBD: Common bile duct.

DISCUSSION

The proposed mechanism of CC rupture or perforation suggests that pooling secretions refluxing from the biliary tree increases intraductal pressure, causing bile duct weakness and eventual burst[2]. In our case, the patient did not have imaging prior to the collision, thus a direct causal link between the CC rupture and trauma cannot be definitively established. However given the clinical timeline, the collision is a more probable precipitant and spontaneous rupture independent of the trauma is unlikely. Type IVa CCs, characterized by both intrahepatic and extrahepatic biliary dilatation, carry one of the highest lifetime risks of complications such as cholangiocarcinoma. As reports of traumatic rupture involving type IVa CCs are particularly scarce in the literature, the optimal diagnostic and management approach is incompletely defined.

Ultrasound is the initial choice of imaging modality, with CT and/or MRCP being required when the distal CBD cannot be visualized. However, these modalities may be insufficient in visualizing subtle bile duct injuries or contained perforations. ERCP is a sensitive diagnostic modality defining biliary anatomy and providing therapeutic decompression in CC. However, sensitivity decreases in settings of recurrent inflammation and scarring where the procedure becomes difficult. ERCP is invasive, which may cause cholangitis, pancreatitis, and increased exposure to radiation as CCs need large amounts of fluoroscopic dye to fill the cyst[4]. In our case, CT demonstrated the filling defect but could not characterize the wall injury itself. Cholangioscopy and cholangiogram confirmed blood clots and extravasation of the CBD, allowing us to reinterpret the initial CT findings as a contained perforation after the motor vehicle collision.

There are four cases of traumatic adult CC rupture or perforation in the literature. In all of these cases, the diagnosis was established intraoperatively following emergent exploratory laparotomy. In the first documented report of traumatic CC rupture, a 21-year-old male underwent emergent laparotomy after a motor vehicle accident, which demonstrated a large CC that had split from the porta hepatitis to duodenum. This was stented with a foley catheter in the common hepatic duct; the patient underwent Roux-en-Y cystjejunostomy five days later without complications[5]. In Duan et al’s report[6], a 26-year-old male was admitted for peritonitis and abdominal hemorrhage following a fall, urgent laparotomy revealed a large cystic mass near the porta hepatis with active arterial bleeding indicative of CC rupture. This was managed with surgical excision of the CC and hepaticojejunostomy. In Raj and Walsh’s report[7], a 31-year-old male was admitted for abdominal pain following a motorcycle collision, CT revealed a hematoma extending from the porta hepatis to the inferior vena cava with subsequent laparotomy identifying the source of bleeding from a dilated bile duct. Afterwards, HIDA scan and ERCP confirmed the presence of a ruptured CC, prompting subsequent surgical excision of the CC with primary reconstruction of the biliary tree. There was no classification of the cyst described in the report. In Chen et al’s report[8], a 41-year-old woman was admitted for abdominal pain following physical assault to the upper abdomen. Urgent laparotomy found a large lacerated cystic mass in the CBD connected to the hepatic bifurcation and gallbladder confirmed to be Type IVa CC, which was managed with T-tube cystotomy and peritoneal lavage.

In contrast to these cases, our patient was hemodynamically stable on presentation and did not require emergent surgery early in admission. She underwent preoperative ERCP, cholangioscopy, and cholangiogram which revealed a large hematoma within the CBD and extravasation, which raised suspicion for a contained perforated CC on initial presentation. Similarly, Ku et al[9] demonstrated the effectiveness of ERCP with sphincterotomy and cholangioscopy in differentiating between a type II and type VI cyst to guide surgical management. However in this case, the patient did not present following a trauma. Although cholangioscopy may be limited in patients with a narrow-caliber bile duct or lesions located in the far distal CBD[10], its use in identifying CC remains rarely reported. In our case specifically, direct cholangioscopy provided an additional, complementary visual confirmation of the ductal injury seen on ERCP. This was helpful in informing preoperative management, including placement of a cholecystostomy tube which demonstrated a perforated bile duct, and surgical excision of the CC and reconstruction of the biliary tree.

Treatment of Type IVa cysts is not standardized due to various involvements of the intrahepatic and extrahepatic components. Type IVa cysts are managed by excision of primarily the extrahepatic component with hepaticojejunostomy. In select cases of extensive intrahepatic dilation concerning for cholangiocarcinoma, hepatic resection with extrahepatic duct reconstruction may be considered[11]. The literature suggests that endoscopic techniques are emerging to be useful in cases of isolated bile duct perforation without liver laceration, such as our patient. Miller et al[12] reported a nonoperative repair of a transected CBD utilizing an endoscopic rendezvous technique to place stents in a retrograde fashion, achieving biliary drainage. Endoscopy offers the advantage of locating the distal end of the CBD behind the pancreas, which may be difficult in surgical exploration.

CONCLUSION

To our knowledge, this case is among a small number of reported adult cases of traumatic CC perforation and is the first of these cases to be identified preoperatively with complementary direct cholangioscopy. This case also emphasizes its presentation as a mimic of other biliary diseases, such as choledocholithiasis. In hemodynamically stable patients with findings suggestive of occult biliary injury, ERCP with direct cholangioscopy may warrant further consideration as a diagnostic adjunct. A larger case series or comparative studies are needed to define its role in early recognition of CC and multidisciplinary surgical planning.