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Branchial Cleft Malformations

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Description

  • The fetal branchial apparatus is a foregut derivative and develops in the 2nd fetal week.
  • 5 paired pharyngeal arches are separated by 4 endodermal pouches internally and 4 ectodermal clefts externally.
  • Overgrowth of the 2nd through 4th cleft creates the cervical sinus and occurs during weeks 4 and 5 of gestation.
  • Persistence of the cervical sinus produces a spectrum of cysts, sinus tracts, and fistulas.
  • Cyst: Epithelial lined structure without an external opening
  • Sinus: Blind tract with an opening either externally through the skin (representing persistence of a branchial groove) or internally into the foregut (representing persistence of branchial pouch)
  • Fistula tract: Communicates between the skin externally and the foregut internally (representing persistence of branchial groove with its corresponding pouch, with no branchial membrane between them).
  • Classification:
    • 1st branchial cleft anomalies:
      • Site: Anywhere from external auditory canal to angle of mandible, usually superior to or within parotid
      • Fistula tract: External auditory canal
    • 2nd branchial cleft anomalies:
      • Site: Ventral to anterior border of sternocleidomastoid muscle, lateral to carotid sheath, and dorsal to submandibular gland
      • Fistula tract: Palatine tonsil
    • 3rd branchial cleft anomalies:
      • Site: Posterior triangle in middle to lower left side of the neck, near level of upper thyroid lobe
      • Fistula: Upper lateral piriform sinus wall to lower lateral neck posterior to sternocleidomastoid muscle
    • 4th branchial cleft anomalies:
      • Site: Close association to thyroid gland and associated with clinical thyroiditis if cyst infected
      • Fistula: Apex of piriform sinus to base of neck anterior to sternocleidomastoid muscle

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