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1st Branchial Cleft Cyst
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Branchial cleft cyst (BCC)
            • Synonyms

              • Pharyngeal cleft anomaly = branchial cleft anomaly (BCA)
              • Pharyngeal apparatus = branchial apparatus (cleft, arch, or pouch)
            • Definitions

              • 1st branchial cleft anomaly: Most are cysts or sinuses; fistula from skin to external auditory canal (EAC) or middle ear is rare
              • 1st BCC: Benign, congenital cyst in or adjacent to parotid gland, EAC, or pinna
                • Remnant of 1st branchial apparatus: Most commonly used classification
                  • Work type I: Duplication of membranous EAC; ectodermal (cleft) origin
                  • Work type II: Duplication of membranous EAC & cartilaginous pinna
                    • Skin (ectodermal cleft) & cartilage (mesodermal arch) origin
                    • May also have contribution from 2nd arch
                • Less commonly used classification
                  • Arnot type I: Derived from buried rests of 1st branchial cleft (BC); intraparotid cyst or sinus
                  • Arnot type II: Secondary to incomplete closure of 1st BC
                    • Cyst or sinus in anterior triangle of neck ± communication with EAC
              • 1st BC sinus tract opens near parotid gland, EAC, parapharyngeal space, or anterior triangle of neck

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Schmidt R et al: Management of first branchial cleft anomalies via a cartilage-splitting technique. Otolaryngol Head Neck Surg. 152(6):1149-51, 2015
                                        2. Shinn JR et al: First branchial cleft anomalies: otologic manifestations and treatment outcomes. Otolaryngol Head Neck Surg. 152(3):506-12, 2015
                                        3. Gonzalez-Perez LM et al: Bilateral first branchial cleft anomaly with evidence of a genetic aetiology. Int J Oral Maxillofac Surg. 43(3):296-300, 2014
                                        4. Maithani T et al: First branchial cleft anomaly: clinical insight into its relevance in otolaryngology with pediatric considerations. Indian J Otolaryngol Head Neck Surg. 66(Suppl 1):271-6, 2014
                                        5. Bajaj Y et al: Branchial anomalies in children. Int J Pediatr Otorhinolaryngol. 75(8):1020-3, 2011
                                        6. Johnson JM et al: Syndromes of the first and second branchial arches, part 1: embryology and characteristic defects. AJNR Am J Neuroradiol. 32(1):14-9, 2011
                                        7. Johnson JM et al: Syndromes of the first and second branchial arches, part 2: syndromes. AJNR Am J Neuroradiol. 32(2):230-7, 2011
                                        8. Gupta AK et al: Bilateral first and second branchial cleft fistulas: a case report. Ear Nose Throat J. 87(5):291-3, 2008
                                        9. Martinez Del Pero M et al: Presentation of first branchial cleft anomalies: the Sheffield experience. J Laryngol Otol. 121(5):455-9, 2007
                                        10. Schroeder JW Jr et al: Branchial anomalies in the pediatric population. Otolaryngol Head Neck Surg. 137(2):289-95, 2007
                                        11. Koch BL: Cystic malformations of the neck in children. Pediatr Radiol. 35(5):463-77, 2005
                                        12. Nicollas R et al: Unusual association of congenital middle ear cholesteatoma and first branchial cleft anomaly: management and embryological concepts. Int J Pediatr Otorhinolaryngol. 69(2):279-82, 2005
                                        13. Nicollas R et al: Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol. 55(2):117-24, 2000
                                        14. Sichel JY et al: Clinical update on type II first branchial cleft cysts. Laryngoscope. 108(10):1524-7, 1998
                                        15. Triglia JM et al: First branchial cleft anomalies: a study of 39 cases and a review of the literature. Arch Otolaryngol Head Neck Surg. 124(3):291-5, 1998
                                        16. Nofsinger YC et al: Periauricular cysts and sinuses. Laryngoscope. 107(7):883-7, 1997
                                        17. Van der Goten A et al: First branchial complex anomalies: report of 3 cases. Eur Radiol. 7(1):102-5, 1997
                                        18. Arndal H et al: First branchial cleft anomaly. Clin Otolaryngol. 21(3):203-7, 1996
                                        19. Choi SS et al: Branchial anomalies: a review of 52 cases. Laryngoscope. 105(9 Pt 1):909-13, 1995
                                        20. Mukherji SK et al: Evaluation of first branchial anomalies by CT and MR. J Comput Assist Tomogr. 17(4):576-81, 1993
                                        21. Benson MT et al: Congenital anomalies of the branchial apparatus: embryology and pathologic anatomy. Radiographics. 12(5):943-60, 1992
                                        22. Doi O et al: Branchial remnants: a review of 58 cases. J Pediatr Surg. 23(9):789-92, 1988
                                        23. Finn DG et al: First branchial cleft cysts: clinical update. Laryngoscope. 97(2):136-40, 1987
                                        24. Graham MD et al: First branchial cleft cyst presenting as a mass within the external auditory canal. Am J Otol. 6(6):500-2, 1985
                                        25. Harnsberger HR et al: Branchial cleft anomalies and their mimics: computed tomographic evaluation. Radiology. 152(3):739-48, 1984
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Branchial cleft cyst (BCC)
                                                  • Synonyms

                                                    • Pharyngeal cleft anomaly = branchial cleft anomaly (BCA)
                                                    • Pharyngeal apparatus = branchial apparatus (cleft, arch, or pouch)
                                                  • Definitions

                                                    • 1st branchial cleft anomaly: Most are cysts or sinuses; fistula from skin to external auditory canal (EAC) or middle ear is rare
                                                    • 1st BCC: Benign, congenital cyst in or adjacent to parotid gland, EAC, or pinna
                                                      • Remnant of 1st branchial apparatus: Most commonly used classification
                                                        • Work type I: Duplication of membranous EAC; ectodermal (cleft) origin
                                                        • Work type II: Duplication of membranous EAC & cartilaginous pinna
                                                          • Skin (ectodermal cleft) & cartilage (mesodermal arch) origin
                                                          • May also have contribution from 2nd arch
                                                      • Less commonly used classification
                                                        • Arnot type I: Derived from buried rests of 1st branchial cleft (BC); intraparotid cyst or sinus
                                                        • Arnot type II: Secondary to incomplete closure of 1st BC
                                                          • Cyst or sinus in anterior triangle of neck ± communication with EAC
                                                    • 1st BC sinus tract opens near parotid gland, EAC, parapharyngeal space, or anterior triangle of neck

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Ultrasonographic Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Schmidt R et al: Management of first branchial cleft anomalies via a cartilage-splitting technique. Otolaryngol Head Neck Surg. 152(6):1149-51, 2015
                                                                              2. Shinn JR et al: First branchial cleft anomalies: otologic manifestations and treatment outcomes. Otolaryngol Head Neck Surg. 152(3):506-12, 2015
                                                                              3. Gonzalez-Perez LM et al: Bilateral first branchial cleft anomaly with evidence of a genetic aetiology. Int J Oral Maxillofac Surg. 43(3):296-300, 2014
                                                                              4. Maithani T et al: First branchial cleft anomaly: clinical insight into its relevance in otolaryngology with pediatric considerations. Indian J Otolaryngol Head Neck Surg. 66(Suppl 1):271-6, 2014
                                                                              5. Bajaj Y et al: Branchial anomalies in children. Int J Pediatr Otorhinolaryngol. 75(8):1020-3, 2011
                                                                              6. Johnson JM et al: Syndromes of the first and second branchial arches, part 1: embryology and characteristic defects. AJNR Am J Neuroradiol. 32(1):14-9, 2011
                                                                              7. Johnson JM et al: Syndromes of the first and second branchial arches, part 2: syndromes. AJNR Am J Neuroradiol. 32(2):230-7, 2011
                                                                              8. Gupta AK et al: Bilateral first and second branchial cleft fistulas: a case report. Ear Nose Throat J. 87(5):291-3, 2008
                                                                              9. Martinez Del Pero M et al: Presentation of first branchial cleft anomalies: the Sheffield experience. J Laryngol Otol. 121(5):455-9, 2007
                                                                              10. Schroeder JW Jr et al: Branchial anomalies in the pediatric population. Otolaryngol Head Neck Surg. 137(2):289-95, 2007
                                                                              11. Koch BL: Cystic malformations of the neck in children. Pediatr Radiol. 35(5):463-77, 2005
                                                                              12. Nicollas R et al: Unusual association of congenital middle ear cholesteatoma and first branchial cleft anomaly: management and embryological concepts. Int J Pediatr Otorhinolaryngol. 69(2):279-82, 2005
                                                                              13. Nicollas R et al: Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol. 55(2):117-24, 2000
                                                                              14. Sichel JY et al: Clinical update on type II first branchial cleft cysts. Laryngoscope. 108(10):1524-7, 1998
                                                                              15. Triglia JM et al: First branchial cleft anomalies: a study of 39 cases and a review of the literature. Arch Otolaryngol Head Neck Surg. 124(3):291-5, 1998
                                                                              16. Nofsinger YC et al: Periauricular cysts and sinuses. Laryngoscope. 107(7):883-7, 1997
                                                                              17. Van der Goten A et al: First branchial complex anomalies: report of 3 cases. Eur Radiol. 7(1):102-5, 1997
                                                                              18. Arndal H et al: First branchial cleft anomaly. Clin Otolaryngol. 21(3):203-7, 1996
                                                                              19. Choi SS et al: Branchial anomalies: a review of 52 cases. Laryngoscope. 105(9 Pt 1):909-13, 1995
                                                                              20. Mukherji SK et al: Evaluation of first branchial anomalies by CT and MR. J Comput Assist Tomogr. 17(4):576-81, 1993
                                                                              21. Benson MT et al: Congenital anomalies of the branchial apparatus: embryology and pathologic anatomy. Radiographics. 12(5):943-60, 1992
                                                                              22. Doi O et al: Branchial remnants: a review of 58 cases. J Pediatr Surg. 23(9):789-92, 1988
                                                                              23. Finn DG et al: First branchial cleft cysts: clinical update. Laryngoscope. 97(2):136-40, 1987
                                                                              24. Graham MD et al: First branchial cleft cyst presenting as a mass within the external auditory canal. Am J Otol. 6(6):500-2, 1985
                                                                              25. Harnsberger HR et al: Branchial cleft anomalies and their mimics: computed tomographic evaluation. Radiology. 152(3):739-48, 1984