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Congenital Abnormalities of the Gallbladder
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Top Differential Diagnoses

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Gallbladder malformation
        • Definitions

          • Spectrum of congenital malformations of gallbladder (GB) shape, morphology, number, or position
            • Agenesis of GB: Congenital absence of GB
            • Hypogenesis of GB: Rudimentary or atretic GB
              • Differentiate from acquired microgallbladder in cystic fibrosis due to viscous bile or chronic cholecystitis
            • Bilobed GB: 2 completely divided GB cavities with a single common cystic duct
              • Differentiate from cholecystomegaly (abnormally large GB) in sickle cell disease, pregnancy, or obesity
            • Duplicated GB: Duplicated GBs with separate cystic ducts for each moiety
              • Cystic ducts may separately insert into extrahepatic bile duct (H-type) or have common insertion (Y-type)
              • Each gallbladder has a separate cystic artery
            • Triplicate GB (vesica fellea triplex): 3 separated GB
            • Multiseptate GB: Single GB with "honeycomb" appearance due to innumerable internal septations, likely due to incomplete vacuolization of developing GB bud
            • Hourglass GB: Hourglass shape of GB may be due to abnormal vacuolization
              • In adults, may be acquired abnormality (due to chronic inflammation and scarring)
            • Congenital diverticulum: Usually a true diverticulum (containing all layers of GB wall) that can be located anywhere in GB
              • Differentiate from acquired diverticula due to prior cholecystitis or traction from prior surgery or duodenitis
            • Phrygian cap: Folding of GB fundus that is considered normal variant given its high prevalence
              • Most common variant of GB shape
            • Ectopic GB: Ectopically positioned GB has been reported in nearly every possible position in abdomen and pelvis
              • Most common positions are intrahepatic, under left hepatic lobe, transverse, retrohepatic, and retroperitoneal
            • Left-sided GB: Ectopic GB positioned in left side of abdomen
              • Isolated or associated with situs inversus
              • Cystic duct usually inserts into left hepatic duct
              • May be associated with left portal vein anomalies
            • Intrahepatic GB: Subcapsular GB partially or completely embedded in liver
            • Horizontal GB: Ectopic GB within porta hepatis; usually deeply embedded in liver
            • Retrodisplaced GB: Retrohepatic or retroperitoneal ectopic GB
            • Floating or wandering GB: Mobile GB due to long mesentery, completely covered by peritoneum
              • May be positioned throughout abdomen
              • High risk of GB torsion and may cause pain by herniating through foramen of Winslow into lesser sac

        IMAGING

        • General Features

          • CT Findings

            • MR Findings

              • Ultrasonographic Findings

                • Nuclear Medicine Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Reporting Tips

                                        Selected References

                                        1. Revzin MV et al: The gallbladder: uncommon gallbladder conditions and unusual presentations of the common gallbladder pathological processes. Abdom Imaging. Epub ahead of print, 2014
                                        2. Khadim MT et al: Triple gallbladder: a rare entity. Am J Gastroenterol. 106(10):1861-2, 2011
                                        3. Walbolt TD et al: Laparoscopic management of a duplicated gallbladder: a case study and anatomic history. Surg Laparosc Endosc Percutan Tech. 21(3):e156-8, 2011
                                        4. Causey MW et al: Gallbladder duplication: evaluation, treatment, and classification. J Pediatr Surg. 45(2):443-6, 2010
                                        5. Rivera-Troche EY et al: Multiseptate gallbladder. J Gastrointest Surg. 13(9):1741-3, 2009
                                        6. Faure JP et al: Abnormalities of the gallbladder, clinical effects. Surg Radiol Anat. 30(4):285-90, 2008
                                        7. Hsu SL et al: Left-sided gallbladder: its clinical significance and imaging presentations. World J Gastroenterol. 13(47):6404-9, 2007
                                        8. Kabiri H et al: Agenesis of the gallbladder. Curr Surg. 63(2):104-6, 2006
                                        9. Bani-Hani KE: Agenesis of the gallbladder: difficulties in management. J Gastroenterol Hepatol. 20(5):671-5, 2005
                                        10. Grand D et al: CT of the gallbladder: spectrum of disease. AJR Am J Roentgenol. 2004 Jul;183(1):163-70. Erratum in: AJR Am J Roentgenol. 183(20:543, 2004
                                        11. Nakazawa T et al: Multiseptate gallbladder: diagnostic value of MR cholangiography and ultrasonography. Abdom Imaging. 29(6):691-3, 2004
                                        12. Ozgen A et al: Gallbladder duplication: imaging findings and differential considerations. Abdom Imaging. 24(3):285-8, 1999
                                        13. Richards RJ et al: Agenesis of the gallbladder in symptomatic adults. A case and review of the literature. J Clin Gastroenterol. 16(3):231-3, 1993
                                        14. Meilstrup JW et al: Imaging of gallbladder variants. AJR Am J Roentgenol. 157(6):1205-8, 1991
                                        15. Gross RE: Congenital abnormalities of the gallbladder: a review of 148 cases with report of double gallbladder. Arch Surg. 32(1):131-62, 1936
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Top Differential Diagnoses

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Synonyms

                                                • Gallbladder malformation
                                              • Definitions

                                                • Spectrum of congenital malformations of gallbladder (GB) shape, morphology, number, or position
                                                  • Agenesis of GB: Congenital absence of GB
                                                  • Hypogenesis of GB: Rudimentary or atretic GB
                                                    • Differentiate from acquired microgallbladder in cystic fibrosis due to viscous bile or chronic cholecystitis
                                                  • Bilobed GB: 2 completely divided GB cavities with a single common cystic duct
                                                    • Differentiate from cholecystomegaly (abnormally large GB) in sickle cell disease, pregnancy, or obesity
                                                  • Duplicated GB: Duplicated GBs with separate cystic ducts for each moiety
                                                    • Cystic ducts may separately insert into extrahepatic bile duct (H-type) or have common insertion (Y-type)
                                                    • Each gallbladder has a separate cystic artery
                                                  • Triplicate GB (vesica fellea triplex): 3 separated GB
                                                  • Multiseptate GB: Single GB with "honeycomb" appearance due to innumerable internal septations, likely due to incomplete vacuolization of developing GB bud
                                                  • Hourglass GB: Hourglass shape of GB may be due to abnormal vacuolization
                                                    • In adults, may be acquired abnormality (due to chronic inflammation and scarring)
                                                  • Congenital diverticulum: Usually a true diverticulum (containing all layers of GB wall) that can be located anywhere in GB
                                                    • Differentiate from acquired diverticula due to prior cholecystitis or traction from prior surgery or duodenitis
                                                  • Phrygian cap: Folding of GB fundus that is considered normal variant given its high prevalence
                                                    • Most common variant of GB shape
                                                  • Ectopic GB: Ectopically positioned GB has been reported in nearly every possible position in abdomen and pelvis
                                                    • Most common positions are intrahepatic, under left hepatic lobe, transverse, retrohepatic, and retroperitoneal
                                                  • Left-sided GB: Ectopic GB positioned in left side of abdomen
                                                    • Isolated or associated with situs inversus
                                                    • Cystic duct usually inserts into left hepatic duct
                                                    • May be associated with left portal vein anomalies
                                                  • Intrahepatic GB: Subcapsular GB partially or completely embedded in liver
                                                  • Horizontal GB: Ectopic GB within porta hepatis; usually deeply embedded in liver
                                                  • Retrodisplaced GB: Retrohepatic or retroperitoneal ectopic GB
                                                  • Floating or wandering GB: Mobile GB due to long mesentery, completely covered by peritoneum
                                                    • May be positioned throughout abdomen
                                                    • High risk of GB torsion and may cause pain by herniating through foramen of Winslow into lesser sac

                                              IMAGING

                                              • General Features

                                                • CT Findings

                                                  • MR Findings

                                                    • Ultrasonographic Findings

                                                      • Nuclear Medicine Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Staging, Grading, & Classification

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Revzin MV et al: The gallbladder: uncommon gallbladder conditions and unusual presentations of the common gallbladder pathological processes. Abdom Imaging. Epub ahead of print, 2014
                                                                              2. Khadim MT et al: Triple gallbladder: a rare entity. Am J Gastroenterol. 106(10):1861-2, 2011
                                                                              3. Walbolt TD et al: Laparoscopic management of a duplicated gallbladder: a case study and anatomic history. Surg Laparosc Endosc Percutan Tech. 21(3):e156-8, 2011
                                                                              4. Causey MW et al: Gallbladder duplication: evaluation, treatment, and classification. J Pediatr Surg. 45(2):443-6, 2010
                                                                              5. Rivera-Troche EY et al: Multiseptate gallbladder. J Gastrointest Surg. 13(9):1741-3, 2009
                                                                              6. Faure JP et al: Abnormalities of the gallbladder, clinical effects. Surg Radiol Anat. 30(4):285-90, 2008
                                                                              7. Hsu SL et al: Left-sided gallbladder: its clinical significance and imaging presentations. World J Gastroenterol. 13(47):6404-9, 2007
                                                                              8. Kabiri H et al: Agenesis of the gallbladder. Curr Surg. 63(2):104-6, 2006
                                                                              9. Bani-Hani KE: Agenesis of the gallbladder: difficulties in management. J Gastroenterol Hepatol. 20(5):671-5, 2005
                                                                              10. Grand D et al: CT of the gallbladder: spectrum of disease. AJR Am J Roentgenol. 2004 Jul;183(1):163-70. Erratum in: AJR Am J Roentgenol. 183(20:543, 2004
                                                                              11. Nakazawa T et al: Multiseptate gallbladder: diagnostic value of MR cholangiography and ultrasonography. Abdom Imaging. 29(6):691-3, 2004
                                                                              12. Ozgen A et al: Gallbladder duplication: imaging findings and differential considerations. Abdom Imaging. 24(3):285-8, 1999
                                                                              13. Richards RJ et al: Agenesis of the gallbladder in symptomatic adults. A case and review of the literature. J Clin Gastroenterol. 16(3):231-3, 1993
                                                                              14. Meilstrup JW et al: Imaging of gallbladder variants. AJR Am J Roentgenol. 157(6):1205-8, 1991
                                                                              15. Gross RE: Congenital abnormalities of the gallbladder: a review of 148 cases with report of double gallbladder. Arch Surg. 32(1):131-62, 1936