Branchial Cleft Malformations
Basics
DESCRIPTION
- Phylogenetically, the branchial apparatus represents the “gills” seen in fish and amphibians.
- The fetal branchial apparatus is a foregut derivative and develops in the 2nd fetal week.
- Five paired pharyngeal arches are separated by four endodermal pouches internally and four ectodermal clefts externally.
- Overgrowth of the second through fourth cleft creates the cervical sinus and occurs during weeks 4 and 5.
- Persistence of the cervical sinus produces a spectrum of cysts, sinus tracts, and fistulae.
- Classification
- First branchial cleft anomalies
- Site: anywhere from external auditory canal to angle of mandible, usually superior to or within parotid
- Fistula tract: external auditory canal
- Second branchial cleft anomalies
- Site: ventral to anterior border of sternocleidomastoid muscle, lateral to carotid sheath, and dorsal to submandibular gland
- Fistula tract: palatine tonsil
- Third 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
- Fourth branchial cleft anomalies
- Site: close association to thyroid gland associated with clinical thyroiditis if cyst infected
- Fistula: apex of piriform sinus to base of neck anterior to sternocleidomastoid muscle
- First branchial cleft anomalies
EPIDEMIOLOGY
- Overwhelming majority of cysts in newborns and infants are developmental, whereas in children and adults, they are inflammatory or neoplastic.
- Branchial cleft cysts are the most common congenital neck lesion. Although congenital, usually present in older children and adults.
- Branchial fistula and sinuses are common in children, but cysts are more commonly seen in adults.
- Midline malformations are most often thyroglossal duct cysts or dermoids.
- Cysts occurring in the laterocervical region are usually branchial cleft malformations; the most common of these are derivatives of the second cleft, followed by those of the first cleft, of the fourth pouch and thymic cysts.
- Third and fourth branchial cleft anomalies are rare, with most presenting as sinus tracts rather than cysts.
- Suspect congenital anomaly in the clinical setting of recurrent infection.
RISK-FACTORS
GENETICS
Familial history of branchial defects occasionally noted
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Citation
Cabana, Michael D., editor. "Branchial Cleft Malformations." Select 5-Minute Pediatrics Topics, 7th ed., Wolters Kluwer Health, 2015. Medicine Central, im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14102/all/Branchial_Cleft_Malformations.
Branchial Cleft Malformations. In: Cabana MDM, ed. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14102/all/Branchial_Cleft_Malformations. Accessed November 14, 2024.
Branchial Cleft Malformations. (2015). In Cabana, M. D. (Ed.), Select 5-Minute Pediatrics Topics (7th ed.). Wolters Kluwer Health. https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14102/all/Branchial_Cleft_Malformations
Branchial Cleft Malformations [Internet]. In: Cabana MDM, editors. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. [cited 2024 November 14]. Available from: https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14102/all/Branchial_Cleft_Malformations.
* Article titles in AMA citation format should be in sentence-case
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