Type your tag names separated by a space and hit enter

Branchial Cleft Fistula

Branchial Cleft Fistula is a topic covered in the 5-Minute Clinical Consult.

To view the entire topic, please or purchase a subscription.

Medicine Central™ is a quick-consult mobile and web resource that includes diagnosis, treatment, medications, and follow-up information on over 700 diseases and disorders, providing fast answers—anytime, anywhere. Explore these free sample topics:

Medicine Central

-- The first section of this topic is shown below --



  • A congenital, abnormal tract connecting the skin of the neck with an internal structure
  • Results from the incomplete closure or development of the branchial arches or associated clefts
  • Involves branchial clefts I to IV, which develop in the 4th week of gestation
  • System(s) affected: skin/exocrine

Pediatric Considerations
Most occur in the pediatric age group


  • Predominant age: By definition, all branchial cleft fistulae are present at birth; however, they may remain unnoticed for some time.
  • Branchial cleft cysts may not present until adulthood and are commonly diagnosed in the 3rd and 5th decades of life.
  • Predominant sex: male = female



Etiology and Pathophysiology

  • Branchial anomalies result from the incomplete obliteration of pharyngeal clefts and pouches during embryogenesis.
  • Both respiratory and squamous epithelium (alone or together) may line branchial anomalies.
  • Squamous epithelium is found more commonly in cysts.
  • Ciliated, columnar epithelium is found more commonly in sinuses and fistulae.
  • The first branchial cleft contributes to the external auditory canal, middle ear cavity, mastoid air cells, and eustachian tube. Related fistulae are very rare and tend to be infra- or retroauricular. Preauricular cysts and sinuses are not thought to be of branchial cleft origin:
    • First branchial cleft anomalies enter the external auditory canal and/or occasionally the middle ear.
    • They represent 1–4% of all branchial cleft malformations.
    • Type I anomalies contain ectodermal elements only and course lateral to the facial nerve.
    • Type II anomalies contain ectoderm and mesoderm, coursing medial to the facial nerve.
  • The second branchial cleft forms the hyoid bone and tonsillar fossa. Related fistulas (most common variant) course between the internal and external carotid arteries:
    • Second branchial anomalies represent 90–95% of all branchial cleft lesions.
    • They course close to the glossopharyngeal and hypoglossal nerves, entering the pharynx at the level of supratonsillar fossa.
    • The external opening runs along anterior border of sternocleidomastoid muscle.
    • Second branchial cleft anomalies are subdivided into four subtypes:
      • Type I lesions are anterior to the SCM and do not involve the carotid sheath.
      • Type II lesions are the most common 2nd arch anomalies, deep to the SCM, and anterior or posterior to the carotid artery.
      • Type III lesions pass between the internal and external carotid arteries and are adjacent to the pharynx.
      • Type IV lesions are medial to the sheath, adjacent to the tonsillar fossa.
  • The third and fourth branchial clefts form the parathyroid glands, thymus, and portions of thyroid gland (parafollicular cells):
    • Third branchial cleft anomalies represent 5% of all branchial anomalies.
    • Sinus tracts (also called pyriform sinuses) originate in the pyriform sinus and course adjacent to the thyroid cartilage.
    • Fistulas are rare, usually resulting from recurrent infections and/or repeated surgery.
    • Both third and fourth fistulas should have external ostia on the lower anterior neck. Left-sided lesions are more common than right-sided ones.
    • They are often called pyriform sinus “fistulae,” despite the frequent lack of an external opening to the skin.
    • Those from the third branchial cleft course posterior to carotid artery.
    • Differentiated from second branchial cleft anomalies by the location of their internal opening (external openings should be the same)
    • Presence of thymic tissue does not differentiate between third and fourth branchial cleft anomalies, as accessory thymic tissue has been described in the latter (1).

10% have family history.

Risk Factors

Positive family history

Commonly Associated Conditions

Microtia and aural atresia occur with failure of development of the first branchial cleft.

-- To view the remaining sections of this topic, please or purchase a subscription --


Stephens, Mark B., et al., editors. "Branchial Cleft Fistula." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816322/all/Branchial_Cleft_Fistula.
Branchial Cleft Fistula. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816322/all/Branchial_Cleft_Fistula. Accessed March 24, 2019.
Branchial Cleft Fistula. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816322/all/Branchial_Cleft_Fistula
Branchial Cleft Fistula [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 24]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816322/all/Branchial_Cleft_Fistula.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Branchial Cleft Fistula ID - 816322 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816322/all/Branchial_Cleft_Fistula PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -