Branchial cleft cyst

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Branchial cyst
Other namesBranchial arch fistula
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   OOjs UI icon edit-ltr-progressive.svg
Symptoms Painless, firm mass lateral to midline, usually anterior to the SCM, which does not move with swallowing
CausesFamily history
Differential diagnosis Vascular anomaly, dermoid cyst, thymic cyst, lymphadenopathy, lymphoma, HPV-related oropharyngeal cancer
TreatmentConservative, surgical excision

A branchial cleft cyst or simply branchial cyst is a cyst as a swelling in the upper part of neck anterior to sternocleidomastoid. 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, third, and fourth branchial cleft, i.e. failure of fusion of the second branchial 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.

Contents

Symptoms and signs

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

The pharyngeal arches as seen during embryonic development PharyngealArchHuman.jpg
The pharyngeal arches as seen during embryonic development

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

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.[ citation needed ]

Diagnosis

The diagnosis of branchial cleft cysts is 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] Unlike a thyroglossal duct cyst, when swallowing, the mass should not move up or down. [7]

Types

Bilateral branchial cleft sinuses during surgery. Bilateral Branchial Cleft Sinus intraoperative.jpg
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, [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.

Treatment

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. [10]

An alternative and less invasive treatment is ultrasound-guided sclerotherapy. [11]

See also

Related Research Articles

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<span class="mw-page-title-main">Congenital cartilaginous rest of the neck</span> Medical condition

Congenital cartilaginous rest of the neck (CCRN) is a minor and very rare congenital cutaneous condition characterized by branchial arch remnants that are considered to be the cervical variant of accessory tragus. It resembles a rudimentary pinna that in most cases is located in the lower anterior part of the neck.

Ectopic thymus is a condition where thymus tissue is found in an abnormal location. It usually does not cause symptoms, but may leads to a mass in the neck that may compress the trachea and the esophagus. It is thought to be the result of either a failure of descent or a failure of involution of normal thymus tissue. It may be diagnosed with radiology, such as an ultrasound or magnetic resonance imaging. If it causes illness, surgery can be used to remove it. Recurrence after surgery is very unlikely.

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The face and neck development of the human embryo refers to the development of the structures from the third to eighth week that give rise to the future head and neck. They consist of three layers, the ectoderm, mesoderm and endoderm, which form the mesenchyme, neural crest and neural placodes. The paraxial mesoderm forms structures named somites and somitomeres that contribute to the development of the floor of the brain and voluntary muscles of the craniofacial region. The lateral plate mesoderm consists of the laryngeal cartilages. The three tissue layers give rise to the pharyngeal apparatus, formed by six pairs of pharyngeal arches, a set of pharyngeal pouches and pharyngeal grooves, which are the most typical feature in development of the head and neck. The formation of each region of the face and neck is due to the migration of the neural crest cells which come from the ectoderm. These cells determine the future structure to develop in each pharyngeal arch. Eventually, they also form the neurectoderm, which forms the forebrain, midbrain and hindbrain, cartilage, bone, dentin, tendon, dermis, pia mater and arachnoid mater, sensory neurons, and glandular stroma.

Collaural fistula or cervico-aural fistula is a type of fistula whose openings are at external auditory canal and the neck, usually in the upper part of anterior border of sternocleidomastoid muscle. It occurs at birth because the defect is in the embryological branchial cleft. It is rare, and accounts for 8% of the branchial cleft anomalies, which is why it is sometimes misdiagnosed.

A cervical thymic cyst, also called thymopharyngeal duct cyst, is a fluid-filled mass that occurs when the thymopharyngeal duct, an embryonic structure connecting the nascent thymus with the embryonic pharynx, fails to close and disappear. A thymic cyst is typically a solitary mass on one side of the neck, and is usually found near the carotid sheath. Some cervical thymic cysts may extend into the mediastinum. It is usually asymptomatic. The diagnostic process includes differentiating between other causes of neck masses in infants and children, including branchial cleft cysts and cystic hygromas. The treatment is surgical excision. On histologic examination, the wall of the cyst includes thymic tissue, and may include parathyroid gland tissue because of the parathyroid gland's common embryonic origin with the thymus gland in the third pharyngeal pouch. Fewer than 100 cases of cervical thymic cysts have been reported in the medical literature.

References

  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   5122306 . PMID   27904209.
  6. "Differential diagnosis of a neck mass". www.uptodate.com. UpToDate. Retrieved 2018-08-18.
  7. "Branchial Cleft Cyst". missinglink.ucsf.edu. Archived from the original on 2019-06-26. Retrieved 2019-06-26.
  8. "Duke Embryology - Craniofacial Development". web.duke.edu. Retrieved 2016-09-08.
  9. 1 2 3 Quintanilla-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. 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.
  11. 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.