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Extrahepatic bile duct duplication with choledocholithiasis in an accessory left bile duct draining into the antrum of the stomach: A case report
European Journal of Radiology Extra 75 (2010) e21–e25
Contents lists available at ScienceDirect
European Journal of Radiology Extra journal homepage: intl.elsevierhealth.com/journals/ejrex
Extrahepatic bile duct duplication with choledocholithiasis in an accessory left bile duct draining into the antrum of the stomach: A case report Tom Turmezei ∗ , Greg Ramjas 1 , Maruti Kumaran 1 Department of Radiology, Queen’s Medical Centre, Derby Road, Nottingham NG7 2UH, UK
a r t i c l e
i n f o
Article history: Received 29 December 2009 Received in revised form 27 April 2010 Accepted 28 April 2010 Keywords: Accessory Bile duct Choledocholithiasis Stomach Imaging Radiology
a b s t r a c t We present the case of a 60-year-old woman admitted to hospital with acute abdominal pain on a background history of vague dyspeptic symptoms managed as an outpatient. Serial imaging investigation with a barium contrast meal, oesophagogastroduodenoscopy, ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography (MRCP) established a very rare diagnosis. Our patient had extrahepatic bile duct duplication with an accessory bile duct draining the left lobe of the liver into the antrum of the stomach without intrahepatic communication of the left and right bile duct systems, complicated by choledocholithiasis of the accessory duct. Diagnosis of this variant was finally made with MRCP at 2 months follow-up to the initial admission, which also demonstrated spontaneous passage of the accessory duct stone. A differential diagnosis list for cystic structures in the lesser omentum is considered. We believe that this is the first such case to be demonstrated with all 6 forms of imaging described, which were performed, undoubtedly, because of its rarity. Retaining awareness of such rare anomalies in our clinical practice should lead to appropriate imaging being initiated early and avoid delay in diagnosis, especially because of associations with potentially serious complications. © 2010 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
2. Case report
Accessory hepatic bile ducts have been demonstrated in up to 4% of necropsies and most commonly involve the right lobe of the liver [1]. Yet duplication of the extrahepatic bile duct is very rare, with an accessory bile duct that drains into the gastrointestinal tract even rarer [2]. Here we present a case of extrahepatic bile duct duplication with an accessory duct passing from the left lobe of the liver to the antrum of the stomach without intrahepatic communication complicated by choledocholithiasis, being demonstrated uniquely with barium meal examination, ultrasonography (US), computed tomography (CT), oesophagogastroduodenoscopy (OGD), endoscopic retrograde cholangiopancreatography (ERCP), and magnetic resonance cholangiopancreatography (MRCP). We also describe the current classification of extrahepatic biliary duct duplication and consider a differential diagnosis of cystic structures in the lesser omentum.
A 60-year-old woman was admitted to our hospital in July 2009 with central abdominal pain radiating to the right upper quadrant that was progressively worsening. On examination she had a positive Murphy’s sign, but there were no clinical signs of peritonism or obstructive jaundice. She was apyrexial with stable observations. Blood investigations showed a white blood cell count of 11,7000 mm−3 . Liver function tests showed a raised alanine transferase, ␥-glutamyl transferase and alkaline phosphatase. Total bilirubin was normal. Her prior medical history included ongoing symptoms of a ‘lump in the throat’ and heartburn, having been seen in the Ear Nose and Throat clinic over the last 2 years with a provisional diagnosis of laryngopharyngeal reflux that had not responded to proton-pump inhibitor and vocal hygiene therapy. She underwent a barium swallow and meal examination for these symptoms in February 2009, which showed a small, contained leak of contrast from the superior aspect of the antrum of the stomach tracking towards the left lobe of the liver that was thought to represent a long-standing perforation, or possibly an abscess fistulating to the stomach (Fig. 1). The patient had subsequently been referred for a gastroenterology review and in early July 2009 she had an OGD for her ongoing dyspeptic symptoms with the abnormal barium meal findings. OGD demonstrated moderate gastritis and identified a small
∗ Corresponding author. Tel.: +44 01159 249924. E-mail addresses: [email protected] (T. Turmezei), [email protected] (G. Ramjas), [email protected] (M. Kumaran). 1 Tel.: +44 01159 249924. 1571-4675/$ – see front matter © 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2010.04.010
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Fig. 3. Axial CT scan through the liver with oral and intravenous contrast medium showing dilated left intrahepatic bile ducts (black arrow) with a dilated, fluid-filled structure passing from the left lobe of the liver to the antrum of the stomach (white arrow).
Fig. 1. Barium meal showing contrast medium in a ‘diverticulum’ in the antrum of the stomach (white arrow) passing along a tract towards the left lobe of the liver (black arrow) that was thought to represent long-standing perforation or the fistulating track from an abscess.
diverticulum in an erythematous antrum that correlated with the abnormality on the barium meal examination. On the morning of admission in July 2009, she had urgent US of the abdomen, which identified an echogenic filling defect with posterior acoustic shadowing in what was thought to be a dilated common bile duct (CBD) with dilated intrahepatic ducts noted in the left lobe of the liver (Fig. 2(a) and (b)). Four days after admission the patient developed an episode of biliary sepsis, subsequently confirmed with culture of the biliary pathogen Streptococcus pneumoniae. On account of the suspicion of an abscess fistulating from the gallbladder to the stomach raised by findings from the barium examination of February 2009, a CT scan of the abdomen was performed with oral and intravenous contrast to investigate for an upper abdominal collection. No collection or
perforation was found, however dilated left intrahepatic bile ducts with a fluid-filled tubular structure passing from the left lobe of the liver to the antrum of the stomach were noted (Fig. 3). No other significant abnormalities were identified. ERCP was then performed 1 week after admission, with initial OGD examination again showing the diverticulum in the antrum of the stomach as previously identified on OGD in July 2009 (Fig. 4). Proceeding to ERCP, the CBD was cannulated to reveal a normal cholangiogram of the CBD and right main hepatic duct. The antral ‘pit’ was then successfully cannulated with the guidewire. Contrast injection revealed an ovoid filling defect in a tubular track running towards the liver (Fig. 5(a) and (b)). In line with the interpretation of the barium swallow examination, impressions from the ERCP were that of an impacted gallstone that had perforated and formed an abscess with fistulation to the antrum of the stomach. As a result no intervention was performed during the procedure. Her symptoms subsequently settled and she was discharged the day after ERCP with plans for further surgical outpatient review.
Fig. 2. (a) Longitudinal oblique US of the liver showing an echogenic filling defect with posterior acoustic shadowing in a tubular structure thought to represent the CBD (white arrow). (b) On transverse scanning dilatation of the left intrahepatic bile ducts was also noted (white arrowheads).
T. Turmezei et al. / European Journal of Radiology Extra 75 (2010) e21–e25
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keen to avoid surgery if possible. She remains well under follow-up with no further imaging or intervention required to date. 3. Discussion
Fig. 4. Picture of the antrum of the stomach taken on OGD as part of the ERCP examination showing puckering of an erythematous mucosal surface around a central ‘pit’ (white arrow).
Two months after discharge with ongoing outpatient follow-up and clinical multi-disciplinary team (MDT) discussion, MRCP was performed on a 1.5T scanner (Signa Excite, GE Medical Systems, Milwaukee, WI, USA) to further investigate the anatomy of the biliary tree. MRCP revealed a tubular fluid-filled structure running from dilated left lobe intrahepatic bile ducts to the antrum of the stomach with focal antral mucosal thickening but no evidence of a stricture, calculus, pneumobilia or filling defect (Fig. 6(a) and (b)). The diagnosis of bile duct duplication with an accessory duct draining the left intrahepatic bile duct system to the antrum of the stomach was made. This imaging also demonstrated presumed spontaneous passage of the accessory duct stone. At 5 months follow-up after admission the patient reported to be suffering less from pain and dyspeptic symptoms. After discussion of the MRCP findings at the clinical MDT meeting, the decision was made to follow up with clinical observation and to consider surgical intervention should her symptoms recur because the patient was
The anomaly identified with imaging in our patient was an accessory bile duct draining the left intrahepatic bile ducts to the antrum of the stomach, with a normal CBD and right intrahepatic duct system. US, CT and ERCP demonstrated dilatation of this accessory duct and the left intrahepatic ducts secondary to an accessory duct calculus with no evidence of communication between the left and right bile duct systems. The fact that the accessory duct was larger than the CBD was presumed to be secondary to the presence of a calculus causing long-standing obstruction. By the time that our patient had MRCP no calculus or pneumobilia was identified in the accessory duct that, along with the left intrahepatic bile ducts, was still dilated—a feature that could be expected in a chronically obstructed duct system even after relief of the cause of obstruction. The accessory duct stone was therefore assumed to have passed asymptomatically in the intervening 2-month period, with the absence of pneumobilia on MRCP suggesting that calculus was passed soon after ERCP. Cystic structures in the region of the lesser omentum between the liver and the lesser curvature of the stomach are an uncommon finding. However this case demonstrates the benefit of having a differential diagnosis in mind. The differential list should include: cholegastric or choleduodenal fistula, healed gastrointestinal perforation, gastric diverticulum [3], and more rarely foregut duplication cyst of the stomach [4], omental cystic lymphangioma [5], and a dilated accessory bile duct. Accessory hepatic bile ducts have been identified in up to 4% of necropsies and are most commonly found arising from the right lobe of the liver draining into to the right hepatic duct, cystic duct, common bile duct or, rarely, the gallbladder [1]. Duplication of the extrahepatic bile ducts is a rarer congenital anomaly with 55 cases having been reported in the medical literature up to 1992 [2], being recognised as more frequent in people of Oriental origin [6]. Accessory or duplicated ducts that drain into the gastrointestinal tract are even rarer, with distal drainage sites described into the duodenum, stomach and less commonly the pancreatic duct (46%, 52% and 2% respectively) [2]. In 2001, only 23 case reports describing an accessory duct draining into the stomach were reported
Fig. 5. (a) ERCP image after cannulation and cholangiogram of the CBD showing the CBD, right main hepatic and intrahepatic ducts, the gallbladder, and a smaller left main hepatic duct which appeared to be connected to the posterior aspect of the right lobe rather than the left lobe (white arrows). (b) A filling defect (black arrow) was noted within the contrast filled track cannulated from the ‘pit’ in the antrum of the stomach. The CBD and right main hepatic bile ducts are also shown from the cholangiogram performed moments earlier. No connection between the right and left intrahepatic duct systems was demonstrated.
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Fig. 6. (a) Coronal T2 single-shot fast spin echo image from the MRCP showing dilated left intrahepatic bile ducts (black arrow) and a tubular fluid-filled structure passing from the left lobe of the liver to the antrum of the stomach (white arrow) representing the accessory duct. No calculus or pneumobilia was identified and the duct itself was less prominent than shown on ERCP 2 months earlier (Fig. 5). These features suggested spontaneous passage of the calculus soon after ERCP. (b) Maximal intensity projection image from the MRCP series showing the accessory duct passing from the left lobe of the liver to the antrum of the stomach (white arrow), but with no calculus or pneumobilia demonstrated. The gallbladder and CBD were normal in appearance.
in the medical literature by Bernard, with 5 more cases having been described since [1,2,7–9]. Vesalius is credited with the first recorded observation of an accessory bile duct draining into the stomach in 1543, describing a patient with two ducts, one draining into the antrum of the stomach and the other draining into the duodenum [10]. Embryologically the presence of extrahepatic bile duct duplication (a normal anatomical feature in reptiles, birds and fish) is thought to be from failure of regression of normal duplication found in early fetal life [11]. Early subdivision of the developing diverticulum hepaticum is thought to lead to a cranially displaced pars hepatica developing into an accessory duct that drains into the stomach [12]. Saito et al. classified extrahepatic bile duct duplication into 4 main configurations [13], with a further modification to 5 proposed by Choi et al. [11]: type 1 representing a septum within the lumen of the CBD; type 2 representing bifurcation of the CBD to produce two separate drainage tracks; type 3 representing double biliary drainage without extrahepatic communication (3a being without intrahepatic communication, 3b being with intrahepatic communication); type 4 representing double biliary drainage with one or more communicating channels; type 5 representing single drainage of two extrahepatic bile ducts (5a being without communication, 5b with communication). Accordingly the anomaly in our patient could be classified as type 3a. The first case of an accessory left bile duct with drainage into the stomach demonstrated by ERCP was reported in 1966, with the first case demonstrated by MRCP in 1991 [12]. It has subsequently been reported in the literature using barium swallow, ultrasound, OGD, ERCP, CT, MRCP and nuclear scintigraphy [1]. In the western literature, the presence of choledocholithiasis within a duplicated extrahepatic bile duct system has been previously reported by Kim et al. [14] in a type 5b duplication and Bernard et al. [12] in a type 3b duplication. Yamashita et al. reported choledocholithiasis in 28% of the 47 cases of double common bile duct found in the Japanese literature prior to 2002 [6]. Other complications of duplicated extrahepatic bile ducts described include choledochal cysts, cholangitis, bile gastritis, and malignancy, specifically cholangiocarcinoma of the aberrant duct and adenocarcinoma of the stomach near to point of opening [2]. A definite link between accessory duct drainage into the stomach and
adenocarcinoma has not been clearly established [1], and given that the patient was keen to avoid surgical intervention a decision was made to follow up her case clinically, with the role of surgery being reviewed should her symptoms recur. Despite the fact that the patient had a culture confirmed episode of biliary sepsis, the background provisional diagnosis of laryngopharyngeal reflux and continuing vague dyspeptic symptoms still leave some uncertainty over the precise cause of her symptoms over the last 2 years. Had the possibility of this anomaly also been considered at the time of the barium meal examination (Fig. 1) then there may have been less of a delay in correct diagnosis. Both these factors contributed significantly to the large number of imaging investigations being performed. 4. Conclusion In conclusion, we present an extremely rare anomaly of type 3a duplication of the biliary tree with an accessory left extrahepatic bile duct draining to the antrum of the stomach complicated with choledocholithiasis. This anomaly has been demonstrated on imaging with barium contrast meal, ultrasound, CT, OGD, ERCP, and MRCP, partly because of the unexpected nature of such a rarity. Retaining awareness of such a rare anomaly and its differentials should lead to appropriate imaging being initiated early and avoid delay in diagnosis, which is especially important because of potential association with serious complications. Conflicts of interest The authors hereby declare that they have no conflicts of interest. References [1] Joo YE, Kim HS, Choi SK, Rew JS, Cho CK, Kim SJ. Congenital anomalous connection between the left intrahepatic bile duct and the stomach. J Gastroenterol 2002;37(11):961–5. [2] Kanematsu M, Imaeda T, Seki M, Goto H, Doi H, Shimokawa K. Accessory bile duct draining into the stomach: case report and review. Gastrointest Radiol 1992;17(1):27–30. [3] Kim SH, Lee SW, Choi WJ, Choi IS, Kim SJ, Koo BH. Laparoscopic resection of gastric diverticulum. J Laparoendosc Adv Surg Tech A 1999;9(February (1)):87–91.
T. Turmezei et al. / European Journal of Radiology Extra 75 (2010) e21–e25 [4] Murakami S, Isozaki H, Shou T, Sakai K, Toyota H. Foregut duplication cyst of the stomach with pseudostratified columnar ciliated epithelium. Pathol Int 2008;58(March (3)):187–90. [5] Sakurai Y, Taniguchi K, Uyama I, et al. Laparoscopic excision of the cystic lymphangioma occurred in the lesser omentum: report of a case and review of literature. Surg Laparosc Endosc Percutan Tech 2009;19(February (1)):e11–4. [6] Yamashita K, Oka Y, Urakami A, Iwamoto S, Tsunoda T, Eto T. Double common bile duct: a case report and a review of the Japanese literature. Surgery 2002;131(June (6)):676–81. [7] Lee JH, Yu JS, Park MS, Yoon DS, Yang SW. MR cholangiography of accessory bile duct connected to the stomach. AJR Am J Roentgenol 2007;189(December (6)):W344–7. [8] Mascarenhas R, Varadarajan R, Mathias J, Traynor O, Geoghegan J. Accessory left biliary duct draining into the lesser curve of the stomach. Gut 2002;51(December (6)):884. [9] Amano Y, Takahashi M, Oishi T, Kumazaki T. MR cholangiopancreatography of double common bile duct with ectopic drainage into stomach. J Comput Assist Tomogr 2002;26(1):141–2.
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[10] Quintana EV, Labat R. Ectopic drainage of the common bile duct. Ann Surg 1974;180(July (1)):119–23. [11] Choi E, Byun JH, Park BJ, Lee MG. Duplication of the extrahepatic bile duct with anomalous union of the pancreaticobiliary ductal system revealed by MR cholangiopancreatography. Br J Radiol 2007;80(July (955)): e150–4. [12] Bernard P, Le Borgne J, Dupas B, Kohnen-Shari N, Raoult S, Hamel A. Double common bile duct with ectopic drainage into the stomach. Case report and review of the literature. Surg Radiol Anat 2001;23(4): 269–72. [13] Saito N, Nakano A, Arase M, Hiraoka T. A case of duplication of the common bile duct with anomaly of the intrahepatic bile duct. Nippon Geka Gakkai Zasshi 1988;89(August (8)):1296–301. [14] Kim SW, Park do H, Shin HC, et al. Duplication of the extrahepatic bile duct in association with choledocholithiasis as depicted by MDCT. Korean J Radiol 2008;9(6):550–4.
Contents lists available at ScienceDirect
European Journal of Radiology Extra journal homepage: intl.elsevierhealth.com/journals/ejrex
Extrahepatic bile duct duplication with choledocholithiasis in an accessory left bile duct draining into the antrum of the stomach: A case report Tom Turmezei ∗ , Greg Ramjas 1 , Maruti Kumaran 1 Department of Radiology, Queen’s Medical Centre, Derby Road, Nottingham NG7 2UH, UK
a r t i c l e
i n f o
Article history: Received 29 December 2009 Received in revised form 27 April 2010 Accepted 28 April 2010 Keywords: Accessory Bile duct Choledocholithiasis Stomach Imaging Radiology
a b s t r a c t We present the case of a 60-year-old woman admitted to hospital with acute abdominal pain on a background history of vague dyspeptic symptoms managed as an outpatient. Serial imaging investigation with a barium contrast meal, oesophagogastroduodenoscopy, ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography (MRCP) established a very rare diagnosis. Our patient had extrahepatic bile duct duplication with an accessory bile duct draining the left lobe of the liver into the antrum of the stomach without intrahepatic communication of the left and right bile duct systems, complicated by choledocholithiasis of the accessory duct. Diagnosis of this variant was finally made with MRCP at 2 months follow-up to the initial admission, which also demonstrated spontaneous passage of the accessory duct stone. A differential diagnosis list for cystic structures in the lesser omentum is considered. We believe that this is the first such case to be demonstrated with all 6 forms of imaging described, which were performed, undoubtedly, because of its rarity. Retaining awareness of such rare anomalies in our clinical practice should lead to appropriate imaging being initiated early and avoid delay in diagnosis, especially because of associations with potentially serious complications. © 2010 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
2. Case report
Accessory hepatic bile ducts have been demonstrated in up to 4% of necropsies and most commonly involve the right lobe of the liver [1]. Yet duplication of the extrahepatic bile duct is very rare, with an accessory bile duct that drains into the gastrointestinal tract even rarer [2]. Here we present a case of extrahepatic bile duct duplication with an accessory duct passing from the left lobe of the liver to the antrum of the stomach without intrahepatic communication complicated by choledocholithiasis, being demonstrated uniquely with barium meal examination, ultrasonography (US), computed tomography (CT), oesophagogastroduodenoscopy (OGD), endoscopic retrograde cholangiopancreatography (ERCP), and magnetic resonance cholangiopancreatography (MRCP). We also describe the current classification of extrahepatic biliary duct duplication and consider a differential diagnosis of cystic structures in the lesser omentum.
A 60-year-old woman was admitted to our hospital in July 2009 with central abdominal pain radiating to the right upper quadrant that was progressively worsening. On examination she had a positive Murphy’s sign, but there were no clinical signs of peritonism or obstructive jaundice. She was apyrexial with stable observations. Blood investigations showed a white blood cell count of 11,7000 mm−3 . Liver function tests showed a raised alanine transferase, ␥-glutamyl transferase and alkaline phosphatase. Total bilirubin was normal. Her prior medical history included ongoing symptoms of a ‘lump in the throat’ and heartburn, having been seen in the Ear Nose and Throat clinic over the last 2 years with a provisional diagnosis of laryngopharyngeal reflux that had not responded to proton-pump inhibitor and vocal hygiene therapy. She underwent a barium swallow and meal examination for these symptoms in February 2009, which showed a small, contained leak of contrast from the superior aspect of the antrum of the stomach tracking towards the left lobe of the liver that was thought to represent a long-standing perforation, or possibly an abscess fistulating to the stomach (Fig. 1). The patient had subsequently been referred for a gastroenterology review and in early July 2009 she had an OGD for her ongoing dyspeptic symptoms with the abnormal barium meal findings. OGD demonstrated moderate gastritis and identified a small
∗ Corresponding author. Tel.: +44 01159 249924. E-mail addresses: [email protected] (T. Turmezei), [email protected] (G. Ramjas), [email protected] (M. Kumaran). 1 Tel.: +44 01159 249924. 1571-4675/$ – see front matter © 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2010.04.010
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Fig. 3. Axial CT scan through the liver with oral and intravenous contrast medium showing dilated left intrahepatic bile ducts (black arrow) with a dilated, fluid-filled structure passing from the left lobe of the liver to the antrum of the stomach (white arrow).
Fig. 1. Barium meal showing contrast medium in a ‘diverticulum’ in the antrum of the stomach (white arrow) passing along a tract towards the left lobe of the liver (black arrow) that was thought to represent long-standing perforation or the fistulating track from an abscess.
diverticulum in an erythematous antrum that correlated with the abnormality on the barium meal examination. On the morning of admission in July 2009, she had urgent US of the abdomen, which identified an echogenic filling defect with posterior acoustic shadowing in what was thought to be a dilated common bile duct (CBD) with dilated intrahepatic ducts noted in the left lobe of the liver (Fig. 2(a) and (b)). Four days after admission the patient developed an episode of biliary sepsis, subsequently confirmed with culture of the biliary pathogen Streptococcus pneumoniae. On account of the suspicion of an abscess fistulating from the gallbladder to the stomach raised by findings from the barium examination of February 2009, a CT scan of the abdomen was performed with oral and intravenous contrast to investigate for an upper abdominal collection. No collection or
perforation was found, however dilated left intrahepatic bile ducts with a fluid-filled tubular structure passing from the left lobe of the liver to the antrum of the stomach were noted (Fig. 3). No other significant abnormalities were identified. ERCP was then performed 1 week after admission, with initial OGD examination again showing the diverticulum in the antrum of the stomach as previously identified on OGD in July 2009 (Fig. 4). Proceeding to ERCP, the CBD was cannulated to reveal a normal cholangiogram of the CBD and right main hepatic duct. The antral ‘pit’ was then successfully cannulated with the guidewire. Contrast injection revealed an ovoid filling defect in a tubular track running towards the liver (Fig. 5(a) and (b)). In line with the interpretation of the barium swallow examination, impressions from the ERCP were that of an impacted gallstone that had perforated and formed an abscess with fistulation to the antrum of the stomach. As a result no intervention was performed during the procedure. Her symptoms subsequently settled and she was discharged the day after ERCP with plans for further surgical outpatient review.
Fig. 2. (a) Longitudinal oblique US of the liver showing an echogenic filling defect with posterior acoustic shadowing in a tubular structure thought to represent the CBD (white arrow). (b) On transverse scanning dilatation of the left intrahepatic bile ducts was also noted (white arrowheads).
T. Turmezei et al. / European Journal of Radiology Extra 75 (2010) e21–e25
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keen to avoid surgery if possible. She remains well under follow-up with no further imaging or intervention required to date. 3. Discussion
Fig. 4. Picture of the antrum of the stomach taken on OGD as part of the ERCP examination showing puckering of an erythematous mucosal surface around a central ‘pit’ (white arrow).
Two months after discharge with ongoing outpatient follow-up and clinical multi-disciplinary team (MDT) discussion, MRCP was performed on a 1.5T scanner (Signa Excite, GE Medical Systems, Milwaukee, WI, USA) to further investigate the anatomy of the biliary tree. MRCP revealed a tubular fluid-filled structure running from dilated left lobe intrahepatic bile ducts to the antrum of the stomach with focal antral mucosal thickening but no evidence of a stricture, calculus, pneumobilia or filling defect (Fig. 6(a) and (b)). The diagnosis of bile duct duplication with an accessory duct draining the left intrahepatic bile duct system to the antrum of the stomach was made. This imaging also demonstrated presumed spontaneous passage of the accessory duct stone. At 5 months follow-up after admission the patient reported to be suffering less from pain and dyspeptic symptoms. After discussion of the MRCP findings at the clinical MDT meeting, the decision was made to follow up with clinical observation and to consider surgical intervention should her symptoms recur because the patient was
The anomaly identified with imaging in our patient was an accessory bile duct draining the left intrahepatic bile ducts to the antrum of the stomach, with a normal CBD and right intrahepatic duct system. US, CT and ERCP demonstrated dilatation of this accessory duct and the left intrahepatic ducts secondary to an accessory duct calculus with no evidence of communication between the left and right bile duct systems. The fact that the accessory duct was larger than the CBD was presumed to be secondary to the presence of a calculus causing long-standing obstruction. By the time that our patient had MRCP no calculus or pneumobilia was identified in the accessory duct that, along with the left intrahepatic bile ducts, was still dilated—a feature that could be expected in a chronically obstructed duct system even after relief of the cause of obstruction. The accessory duct stone was therefore assumed to have passed asymptomatically in the intervening 2-month period, with the absence of pneumobilia on MRCP suggesting that calculus was passed soon after ERCP. Cystic structures in the region of the lesser omentum between the liver and the lesser curvature of the stomach are an uncommon finding. However this case demonstrates the benefit of having a differential diagnosis in mind. The differential list should include: cholegastric or choleduodenal fistula, healed gastrointestinal perforation, gastric diverticulum [3], and more rarely foregut duplication cyst of the stomach [4], omental cystic lymphangioma [5], and a dilated accessory bile duct. Accessory hepatic bile ducts have been identified in up to 4% of necropsies and are most commonly found arising from the right lobe of the liver draining into to the right hepatic duct, cystic duct, common bile duct or, rarely, the gallbladder [1]. Duplication of the extrahepatic bile ducts is a rarer congenital anomaly with 55 cases having been reported in the medical literature up to 1992 [2], being recognised as more frequent in people of Oriental origin [6]. Accessory or duplicated ducts that drain into the gastrointestinal tract are even rarer, with distal drainage sites described into the duodenum, stomach and less commonly the pancreatic duct (46%, 52% and 2% respectively) [2]. In 2001, only 23 case reports describing an accessory duct draining into the stomach were reported
Fig. 5. (a) ERCP image after cannulation and cholangiogram of the CBD showing the CBD, right main hepatic and intrahepatic ducts, the gallbladder, and a smaller left main hepatic duct which appeared to be connected to the posterior aspect of the right lobe rather than the left lobe (white arrows). (b) A filling defect (black arrow) was noted within the contrast filled track cannulated from the ‘pit’ in the antrum of the stomach. The CBD and right main hepatic bile ducts are also shown from the cholangiogram performed moments earlier. No connection between the right and left intrahepatic duct systems was demonstrated.
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Fig. 6. (a) Coronal T2 single-shot fast spin echo image from the MRCP showing dilated left intrahepatic bile ducts (black arrow) and a tubular fluid-filled structure passing from the left lobe of the liver to the antrum of the stomach (white arrow) representing the accessory duct. No calculus or pneumobilia was identified and the duct itself was less prominent than shown on ERCP 2 months earlier (Fig. 5). These features suggested spontaneous passage of the calculus soon after ERCP. (b) Maximal intensity projection image from the MRCP series showing the accessory duct passing from the left lobe of the liver to the antrum of the stomach (white arrow), but with no calculus or pneumobilia demonstrated. The gallbladder and CBD were normal in appearance.
in the medical literature by Bernard, with 5 more cases having been described since [1,2,7–9]. Vesalius is credited with the first recorded observation of an accessory bile duct draining into the stomach in 1543, describing a patient with two ducts, one draining into the antrum of the stomach and the other draining into the duodenum [10]. Embryologically the presence of extrahepatic bile duct duplication (a normal anatomical feature in reptiles, birds and fish) is thought to be from failure of regression of normal duplication found in early fetal life [11]. Early subdivision of the developing diverticulum hepaticum is thought to lead to a cranially displaced pars hepatica developing into an accessory duct that drains into the stomach [12]. Saito et al. classified extrahepatic bile duct duplication into 4 main configurations [13], with a further modification to 5 proposed by Choi et al. [11]: type 1 representing a septum within the lumen of the CBD; type 2 representing bifurcation of the CBD to produce two separate drainage tracks; type 3 representing double biliary drainage without extrahepatic communication (3a being without intrahepatic communication, 3b being with intrahepatic communication); type 4 representing double biliary drainage with one or more communicating channels; type 5 representing single drainage of two extrahepatic bile ducts (5a being without communication, 5b with communication). Accordingly the anomaly in our patient could be classified as type 3a. The first case of an accessory left bile duct with drainage into the stomach demonstrated by ERCP was reported in 1966, with the first case demonstrated by MRCP in 1991 [12]. It has subsequently been reported in the literature using barium swallow, ultrasound, OGD, ERCP, CT, MRCP and nuclear scintigraphy [1]. In the western literature, the presence of choledocholithiasis within a duplicated extrahepatic bile duct system has been previously reported by Kim et al. [14] in a type 5b duplication and Bernard et al. [12] in a type 3b duplication. Yamashita et al. reported choledocholithiasis in 28% of the 47 cases of double common bile duct found in the Japanese literature prior to 2002 [6]. Other complications of duplicated extrahepatic bile ducts described include choledochal cysts, cholangitis, bile gastritis, and malignancy, specifically cholangiocarcinoma of the aberrant duct and adenocarcinoma of the stomach near to point of opening [2]. A definite link between accessory duct drainage into the stomach and
adenocarcinoma has not been clearly established [1], and given that the patient was keen to avoid surgical intervention a decision was made to follow up her case clinically, with the role of surgery being reviewed should her symptoms recur. Despite the fact that the patient had a culture confirmed episode of biliary sepsis, the background provisional diagnosis of laryngopharyngeal reflux and continuing vague dyspeptic symptoms still leave some uncertainty over the precise cause of her symptoms over the last 2 years. Had the possibility of this anomaly also been considered at the time of the barium meal examination (Fig. 1) then there may have been less of a delay in correct diagnosis. Both these factors contributed significantly to the large number of imaging investigations being performed. 4. Conclusion In conclusion, we present an extremely rare anomaly of type 3a duplication of the biliary tree with an accessory left extrahepatic bile duct draining to the antrum of the stomach complicated with choledocholithiasis. This anomaly has been demonstrated on imaging with barium contrast meal, ultrasound, CT, OGD, ERCP, and MRCP, partly because of the unexpected nature of such a rarity. Retaining awareness of such a rare anomaly and its differentials should lead to appropriate imaging being initiated early and avoid delay in diagnosis, which is especially important because of potential association with serious complications. Conflicts of interest The authors hereby declare that they have no conflicts of interest. References [1] Joo YE, Kim HS, Choi SK, Rew JS, Cho CK, Kim SJ. Congenital anomalous connection between the left intrahepatic bile duct and the stomach. J Gastroenterol 2002;37(11):961–5. [2] Kanematsu M, Imaeda T, Seki M, Goto H, Doi H, Shimokawa K. Accessory bile duct draining into the stomach: case report and review. Gastrointest Radiol 1992;17(1):27–30. [3] Kim SH, Lee SW, Choi WJ, Choi IS, Kim SJ, Koo BH. Laparoscopic resection of gastric diverticulum. J Laparoendosc Adv Surg Tech A 1999;9(February (1)):87–91.
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