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A dilated common bile duct with “atypical” gall bladder
Journal Pre-proof A dilated common bile duct with “atypical” gall bladder Achintya D. Singh, C.Roberto Simons-Linares, Prabhleen Chahal
PII: DOI: Reference:
S0016-5085(20)30133-5 https://doi.org/10.1053/j.gastro.2020.01.039 YGAST 63181
To appear in: Gastroenterology Accepted Date: 24 January 2020 Please cite this article as: Singh AD, Simons-Linares CR, Chahal P, A dilated common bile duct with “atypical” gall bladder, Gastroenterology (2020), doi: https://doi.org/10.1053/j.gastro.2020.01.039. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 by the AGA Institute
A dilated common bile duct with “atypical” gall bladder
Achintya D Singha, C.Roberto Simons-Linaresb, Prabhleen Chahal*b
Departments of a- Internal Medicine, b- Gastroenterology, Cleveland Clinic, Cleveland, OH
* Address for Correspondence,
Prabhleen Chahal MD, Program Director, Advanced Endoscopy Fellowship Program Digestive Disease and Surgery Institute Cleveland Clinic | 9500 Euclid Avenue | Cleveland, OH 44195 Phone: 216-444-9261 | Fax: 216-444-6284 | [email protected]
Word Count: 486
Figures: 3 Conflicts of interest: We declare no conflicts of interest Keywords: Multiseptate gallbladder, Congenital anomalies, choledochal cysts, Endoscopic ultrasound
Question:
A 49-year-female presented to the office with the complaint of intermittent right upper quadrant abdominal pain for the last few months. The abdominal pain was mild, non-radiating with no associated nausea, vomiting, skin discoloration without clear exacerbating or relieving factors. She had a developmental delay but no other co-morbidities. On examination, the abdomen was soft, non-tender. Her BMI was 26.4 kg/m2. Her comprehensive metabolic panel was unremarkable apart from a mild elevation of alkaline phosphatase 273 U/L (34-123 U/L). Her ultrasound abdomen revealed multiple echo densities with band like septations in the gallbladder with a dilated common bile duct. The suggested differentials were chronic cholecystitis, polyps or malignancy. An MR cholangiogram reportedly showed a 4.3 cm irregular multilocular cystic structure in the gallbladder region that had an atypical appearance for a gallbladder. The extrahepatic bile duct was dilated, measuring 12 mm. There was no choledocholithiasis or enhancing mass within the common bile duct. As the diagnosis was still ambiguous, she underwent an upper endoscopic ultrasound evaluation (EUS). Her EUS findings are shown in the figure. What is the likely diagnosis of this clinical condition?
Answer: The imaging studies of the patient are consistent with multi-septate gallbladder (figure A and B) with type 1 choledochal cyst (figure C). Multi-septate gallbladder is a rare congenital condition considered to be due to incomplete vacuolization of the gallbladder bud or persistence of the developmental gallbladder wall invaginations and wrinkling.(1) Choledochal cysts (CC) are rare congenital dilatations of the biliary tree also likely due to an anomalous pancreaticoduodenal anastomosis. Type 1 CC is characterized by diffuse dilation of the extra-hepatic bile duct and carries a higher risk of cholangiocarcinoma(2) The co-occurrence of type 1 CC and multi-septate gallbladder is a rare phenomenon that led to ambiguity in diagnosing these anomalies from imaging alone.
The current knowledge of multi-septate gallbladder is derived from anecdotal cases and there are very few reported cases globally. Most cases present with chronic abdominal pain, nausea and vomiting, though multi-septate gallbladder can be an incidental finding. The exact etiology behind the symptoms is not known, however, impaired biliary flow is the most likely postulated mechanism as observed on biliary manometry and scintigraphy studies.(3) In the present case, the abdominal pain could also be due to the long-standing gastric reflux and gastritis.
Various imaging modalities have been used to diagnose multi-septate gallbladder. Typical abdominal sonographic appearance is a 'honeycombing' pattern due to the presence of multiple septa. The various differentials of such findings include multi-septate gallbladder, hyperplastic cholecystosis and chronic cholecystitis. MR cholangiography and hepatobiliary scintigraphy reportedly show high diagnostic accuracy. In our case, the diagnosis could not be ascertained based on abdominal ultrasound and MRI. This is the first reported case of diagnosing multi-septate gallbladder with an EUS. It clearly showed multiple septations in the gallbladder and the cystic duct. Our patient was referred to a surgeon for cholecystectomy and resection of the bile duct . The surgery has not been performed yet.
References: 1. Rivera-Troche EY, Hartwig MG, Vaslef SN. Multiseptate Gallbladder. J Gastrointest Surg. 2009 Sep 1;13(9):1741–3. 2. Soares KC, Goldstein SD, Ghaseb MA, Kamel I, Hackam DJ, Pawlik TM. Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int. 2017 Jun;33(6):637–50. 3. Saimura M, Ichimiya H, Naritomi G, Ogawa Y, Chijiiwa K, Yamaguchi K, et al. Multiseptate gallbladder: biliary manometry and scintigraphy. J Gastroenterol. 1996 Feb;31(1):133–6.
PII: DOI: Reference:
S0016-5085(20)30133-5 https://doi.org/10.1053/j.gastro.2020.01.039 YGAST 63181
To appear in: Gastroenterology Accepted Date: 24 January 2020 Please cite this article as: Singh AD, Simons-Linares CR, Chahal P, A dilated common bile duct with “atypical” gall bladder, Gastroenterology (2020), doi: https://doi.org/10.1053/j.gastro.2020.01.039. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 by the AGA Institute
A dilated common bile duct with “atypical” gall bladder
Achintya D Singha, C.Roberto Simons-Linaresb, Prabhleen Chahal*b
Departments of a- Internal Medicine, b- Gastroenterology, Cleveland Clinic, Cleveland, OH
* Address for Correspondence,
Prabhleen Chahal MD, Program Director, Advanced Endoscopy Fellowship Program Digestive Disease and Surgery Institute Cleveland Clinic | 9500 Euclid Avenue | Cleveland, OH 44195 Phone: 216-444-9261 | Fax: 216-444-6284 | [email protected]
Word Count: 486
Figures: 3 Conflicts of interest: We declare no conflicts of interest Keywords: Multiseptate gallbladder, Congenital anomalies, choledochal cysts, Endoscopic ultrasound
Question:
A 49-year-female presented to the office with the complaint of intermittent right upper quadrant abdominal pain for the last few months. The abdominal pain was mild, non-radiating with no associated nausea, vomiting, skin discoloration without clear exacerbating or relieving factors. She had a developmental delay but no other co-morbidities. On examination, the abdomen was soft, non-tender. Her BMI was 26.4 kg/m2. Her comprehensive metabolic panel was unremarkable apart from a mild elevation of alkaline phosphatase 273 U/L (34-123 U/L). Her ultrasound abdomen revealed multiple echo densities with band like septations in the gallbladder with a dilated common bile duct. The suggested differentials were chronic cholecystitis, polyps or malignancy. An MR cholangiogram reportedly showed a 4.3 cm irregular multilocular cystic structure in the gallbladder region that had an atypical appearance for a gallbladder. The extrahepatic bile duct was dilated, measuring 12 mm. There was no choledocholithiasis or enhancing mass within the common bile duct. As the diagnosis was still ambiguous, she underwent an upper endoscopic ultrasound evaluation (EUS). Her EUS findings are shown in the figure. What is the likely diagnosis of this clinical condition?
Answer: The imaging studies of the patient are consistent with multi-septate gallbladder (figure A and B) with type 1 choledochal cyst (figure C). Multi-septate gallbladder is a rare congenital condition considered to be due to incomplete vacuolization of the gallbladder bud or persistence of the developmental gallbladder wall invaginations and wrinkling.(1) Choledochal cysts (CC) are rare congenital dilatations of the biliary tree also likely due to an anomalous pancreaticoduodenal anastomosis. Type 1 CC is characterized by diffuse dilation of the extra-hepatic bile duct and carries a higher risk of cholangiocarcinoma(2) The co-occurrence of type 1 CC and multi-septate gallbladder is a rare phenomenon that led to ambiguity in diagnosing these anomalies from imaging alone.
The current knowledge of multi-septate gallbladder is derived from anecdotal cases and there are very few reported cases globally. Most cases present with chronic abdominal pain, nausea and vomiting, though multi-septate gallbladder can be an incidental finding. The exact etiology behind the symptoms is not known, however, impaired biliary flow is the most likely postulated mechanism as observed on biliary manometry and scintigraphy studies.(3) In the present case, the abdominal pain could also be due to the long-standing gastric reflux and gastritis.
Various imaging modalities have been used to diagnose multi-septate gallbladder. Typical abdominal sonographic appearance is a 'honeycombing' pattern due to the presence of multiple septa. The various differentials of such findings include multi-septate gallbladder, hyperplastic cholecystosis and chronic cholecystitis. MR cholangiography and hepatobiliary scintigraphy reportedly show high diagnostic accuracy. In our case, the diagnosis could not be ascertained based on abdominal ultrasound and MRI. This is the first reported case of diagnosing multi-septate gallbladder with an EUS. It clearly showed multiple septations in the gallbladder and the cystic duct. Our patient was referred to a surgeon for cholecystectomy and resection of the bile duct . The surgery has not been performed yet.
References: 1. Rivera-Troche EY, Hartwig MG, Vaslef SN. Multiseptate Gallbladder. J Gastrointest Surg. 2009 Sep 1;13(9):1741–3. 2. Soares KC, Goldstein SD, Ghaseb MA, Kamel I, Hackam DJ, Pawlik TM. Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int. 2017 Jun;33(6):637–50. 3. Saimura M, Ichimiya H, Naritomi G, Ogawa Y, Chijiiwa K, Yamaguchi K, et al. Multiseptate gallbladder: biliary manometry and scintigraphy. J Gastroenterol. 1996 Feb;31(1):133–6.