Branchial cleft cyst

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Branchial arch fistula
Synonyms Benign cervical lymphoepithelial cyst
Pharyngeal arch cyst
Bilateral Branchial Cleft Sinus fistulography right.jpg
Fistulogram (sinogram) of a right branchial cleft sinus.
Specialty Medical genetics Edit this on Wikidata
Symptoms Solitary neck mass anterior to the SCM that is mobile, soft and painless
Causes Family history
Differential diagnosis Vascular anomaly, dermoid cyst, thymic cyst, lymphadenopathy, lymphoma, HPV-related oropharyngeal cancer
Treatment Conservative, surgical excision

A branchial cleft cyst is a cyst in the skin of the lateral part of the neck. It can, but does not necessarily, have an opening to the skin surface, called a fistula. The cause is usually a developmental abnormality arising in the early prenatal period, typically failure of obliteration of the second branchial cleft, i.e. failure of fusion of the second and third branchial arches. 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[edit]

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

Pathophysiology[edit]

Branchial cleft cysts are remnants of embryonic development and result from a failure of obliteration of one of the branchial clefts, which are homologous to the structures in fish that develop into gills.[3][4]

Pathology[edit]

The cyst wall is composed of squamous epithelium (90%), columnar cells with or without cilia, or a mixture of both, with lymphoid infiltrate, often with prominent germinal 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] The cyst may or may not contain granular and keratinaceous cellular debris. Cholesterol crystals may be found in the fluid extracted from a branchial cyst.

Diagnosis[edit]

The diagnosis of branchial cleft cysts are typically done clinically due to their relatively consistent location in the neck, typically anterior to the sternocleidomastoid muscle. For masses presenting in adulthood, the presumption should be a malignancy until proven otherwise. Since carcinomas of the tonsil, tongue base and thyroid may all present as cystic masses of the neck.[6]

Types[edit]

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 the external auditory canal,[7] 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.

Treatment[edit]

Conservative (i.e. no treatment), or surgical 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 the internal jugular vein, carotid artery, or facial nerve, making complete excision impractical due to the high risk of complications.[9]

See also[edit]

References[edit]

  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 C. "Branchial cleft cyst". eMedicine.com. Retrieved 24 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 5122306Freely accessible. PMID 27904209. 
  6. ^ a b "Differential diagnosis of a neck mass". www.uptodate.com. UpToDate. Retrieved 2018-08-18. 
  7. ^ "Duke Embryology - Craniofacial Development". web.duke.edu. Retrieved 2016-09-08. 
  8. ^ a b c "Differential diagnosis of a neck mass". www.uptodate.com. Retrieved 2016-09-08. 
  9. ^ 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. 

External links[edit]

Classification
External resources