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Branchial cleft cyst

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(Redirected fromBranchial cyst)
Medical condition
Branchial cyst
Other namesBranchial arch fistula
Benign cervical lymphoepithelial cyst
Pharyngeal arch cyst
Fistulogram (sinogram) of a right branchial cleft sinus.
SpecialtyMedical genetics Edit this on Wikidata
SymptomsPainless, firm mass lateral to midline, usually anterior to theSCM, which does not move with swallowing
CausesFamily history
Differential diagnosisVascular anomaly,dermoid cyst,thymic cyst[broken anchor],lymphadenopathy,lymphoma,HPV-related oropharyngeal cancer
TreatmentConservative,surgical excision

Abranchial cleft cyst or simplybranchial cyst is acyst as a swelling in the upper part of neck anterior tosternocleidomastoid. It can, but does not necessarily, have an opening to the skin surface, called afistula. The cause is usually adevelopmental abnormality arising in the early prenatal period, typically failure of obliteration of the second, third, and fourthbranchial cleft, i.e. failure of fusion of the secondbranchial arches and epicardial ridge in lower part of the neck. Branchial cleft cysts account for almost 20% of neck masses in children.[1] Less commonly, the cysts can develop from the first, third, or fourth clefts, and their location and the location of associated fistulas differs accordingly.

Symptoms and signs

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Most branchial cleft cysts present in late childhood or early adulthood as a solitary, painless mass, which went previously unnoticed, that has now becomeinfected (typically after anupper respiratory tract infection). Fistulas, if present, areasymptomatic until infection arises.[2]

Pathophysiology

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Thepharyngeal arches as seen duringembryonic development

Branchial cleft cysts are remnants ofembryonic development and result from a failure of obliteration of one of the branchial clefts, which arehomologous to the structures infish that develop intogills.[3][4]

Pathology

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The cyst wall is composed of squamousepithelium (90%), columnar cells with or withoutcilia, or a mixture of both, with lymphoid infiltrate, often with prominentgerminal centers and few subcapsular lymph sinuses. The cyst is typically surrounded by lymphoid tissue that has attenuated or absent overlying epithelium due to inflammatory changes.[5]

Diagnosis

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Thediagnosis of branchial cleft cysts is typically done clinically due to their relatively consistent location in the neck, typically anterior to thesternocleidomastoid muscle. For masses presenting inadulthood, the presumption should be amalignancy until proven otherwise, since carcinomas of the tonsil, tongue base and thyroid may all present as cystic masses of the neck.[6] Unlike a thyroglossal duct cyst, when swallowing, the mass should not move up or down.[7]

Types

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Bilateral branchial cleft sinuses during surgery.

Four branchial clefts (also called "grooves") form during the development of a human embryo. The first cleft normally develops into theexternal auditory canal,[8] but the remaining three arches are obliterated and have no persistent structures in normal development. Persistence or abnormal formation of these four clefts can all result in branchial cleft cysts which may or may not drain via sinus tracts.

  • First branchial cleft cysts - These are also known as periauricular because of their position near the ear. They are always in or adjacent to theparotid gland. These account for 8% of the sinuses and cysts of the neck. They are lateral to thefacial nerve and run parallel to the external auditory canal.[9]
  • Second branchial cleft cysts - These account for 90 to 95% of the neck cysts. Anterior to sternocleidomastoid muscle, posterior tosubmandibular gland, lateral to carotid sheath. They are medial to the facial nerve at the anterior neck and above the hyoid bone. Skin pit can be found in this location. However, if skin pits are found on both sides of the neck, then,branchio-oto-renal syndrome should be ruled out. Infection of the cysts in this region can compresstrachea, causing respiratory problems, or it can compress theoesophagus, causingdysphagia, and irritating the sternocleidomastoid muscle, causingtorticollis.[9]
  • Third branchial cleft cysts - These are rare and located in the posterior triangle of the upper neck or the anterior triangle in the lower neck.[10]
  • Fourth branchial cleft abnormalities are not technically cysts, and so are referred to as branchial arch anomalies. They consist of a sinus tract or fistula extending from apex of pyriform sinus to anterior lower neck, usually adjacent to left thyroid lobe. If infected, they can causeacute infectious thyroiditis in children and if enlarge rapidly, can cause tracheal compression in children.[9]

Treatment

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Conservative (i.e. no treatment), orsurgical excision. With surgical excision, recurrence is common, usually due to incomplete excision. Often, the tracts of the cyst will pass near important structures, such as theinternal jugular vein,carotid artery, orfacial nerve, making complete excision impractical due to the high risk of complications.[11]

An alternative and less invasive treatment is ultrasound-guidedsclerotherapy.[12]

See also

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References

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  1. ^Pincus RL (2001). "Congenital neck masses and cysts".Head & Neck Surgery - Otolaryngology (3 ed.). Lippincott Williams & Wilkins: 933.
  2. ^Colman R (2008).Toronto Notes. pp. OT33.
  3. ^Hong Ch."Branchial cleft cyst".eMedicine.com. Retrieved24 August 2008.
  4. ^Shubin N (2009).Your Inner Fish. Vintage.ISBN 978-0-307-27745-9.
  5. ^Nahata V (2016)."Branchial Cleft Cyst".Indian Journal of Dermatology.61 (6): 701.doi:10.4103/0019-5154.193718.PMC 5122306.PMID 27904209.
  6. ^"Differential diagnosis of a neck mass".www.uptodate.com. UpToDate. Retrieved2018-08-18.
  7. ^"Branchial Cleft Cyst".missinglink.ucsf.edu. Archived fromthe original on 2019-06-26. Retrieved2019-06-26.
  8. ^"Duke Embryology - Craniofacial Development".web.duke.edu. Retrieved2016-09-08.
  9. ^abcQuintanilla-Dieck, Lourdes; Penn, Edward B. (December 2018). "Congenital Neck Masses".Clinics in Perinatology.45 (4):769–785.doi:10.1016/j.clp.2018.07.012.PMID 30396417.S2CID 53224066.
  10. ^Koch, BL (May 2005). "Cystic malformations of the neck in children".Pediatric radiology.35 (5):463–77.doi:10.1007/s00247-004-1388-0.PMID 15785931.
  11. ^Waldhausen JH (May 2006). "Branchial cleft and arch anomalies in children".Seminars in Pediatric Surgery.15 (2):64–9.doi:10.1053/j.sempedsurg.2006.02.002.PMID 16616308.
  12. ^Kim J (April 2014)."Ultrasound-guided sclerotherapy for benign non-thyroid cystic mass in the neck".Ultrasonography.33 (2):83–90.doi:10.14366/usg.13026.PMC 4058977.PMID 24936500.

External links

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Classification
External resources
Congenital malformations and deformations offace andneck
Face
Neck
Ungrouped
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