eMedicine Specialties > Dermatology > Pediatric Diseases

Branchial Cleft Cyst

Chih-Ho Hong, MD, FRCPC, Clinical Assistant Professor, Department of Dermatology and Skin Science, University of British Columbia, Canada
Richard Crawford, MD, FRCPC, Head of Dermatology, Clinical Professor, Department of Medicine, Divisions of Pathology and Dermatology, University of British Columbia

Updated: Jun 16, 2009

Introduction

Background

Branchial cleft cysts are congenital epithelial cysts, which arise on the lateral part of the neck from a failure of obliteration of the second branchial cleft in embryonic development.¹

Phylogenetically, the branchial apparatus is related to gill slits. In fish and amphibians, these structures are responsible for the development of the gills, hence the name branchial (branchia is Greek for gills).

Pathophysiology

At the fourth week of embryonic life, the development of 4 branchial (or pharyngeal) clefts results in 5 ridges known as the branchial (or pharyngeal) arches, which contribute to the formation of various structures of the head, the neck, and the thorax. The second arch grows caudally and, ultimately, covers the third and fourth arches. The buried clefts become ectoderm-lined cavities, which normally involute around week 7 of development. If a portion of the cleft fails to involute completely, the entrapped remnant forms an epithelium-lined cyst with or without a sinus tract to the overlying skin.^2,3,4,5

Frequency

United States

The exact incidence of branchial cleft cysts in the US population is unknown. Branchial cleft cysts are the most common congenital cause of a neck mass. An estimated 2-3% of cases are bilateral. A tendency exists for cases to cluster in families.⁶

Mortality/Morbidity

Many branchial cleft cysts are asymptomatic. They may become tender, enlarged, or inflamed, or they may develop abscesses, especially during periods of upper respiratory tract infection, due to the lymphoid tissue located beneath the epithelium. Spontaneous rupture of an abscessed branchial cleft cyst may result in a purulent draining sinus to the skin or the pharynx.
Depending on the size and the anatomical extension of the mass, local symptoms, such as dysphagia, dysphonia, dyspnea, and stridor, may occur.

Race

No ethnic predilection has been reported for branchial cleft cysts.

Sex

No sexual predilection is recognized for branchial cleft cysts.

Age

Branchial cleft cysts are congenital in nature, but they may not present clinically until later in life, usually by early adulthood.

Clinical

History

A branchial cyst commonly presents as a solitary, painless mass in the neck of a child or a young adult. A history of intermittent swelling and tenderness of the lesion during upper respiratory tract infection may exist. Discharge may be reported if the lesion is associated with a sinus tract.
In some instances, branchial cleft cyst patients may present with locally compressive symptoms.
A family history of branchial cleft cysts may be present.

Physical

Primary branchial cleft cyst lesion: Branchial cysts are smooth, nontender, fluctuant masses, which occur along the lower one third of the anteromedial border of the sternocleidomastoid muscle between the muscle and the overlying skin.
Secondary branchial cleft cyst lesion: The lesion may be tender if secondarily inflamed or infected. When associated with a sinus tract, mucoid or purulent discharge onto the skin or into the pharynx may be present.

Causes

The branchial cleft cyst is a congenital lesion formed by incomplete involution of branchial cleft structures during embryonic development.

Differential Diagnoses

Workup

Laboratory Studies

No specific laboratory investigations need to be obtained in the workup of a branchial cleft cyst.

Imaging Studies

A sinogram may be obtained. If a sinus tract exists, radiopaque dye can be injected to delineate the course and to examine the size of the cyst.
Ultrasonography helps to delineate the cystic nature of these lesions.⁷
A contrast-enhanced CT scan shows a cystic and enhancing mass in the neck. It may aid preoperative planning and identify compromise of local structures.
MRI allows for finer resolution during preoperative planning. The wall may be enhancing on gadolinium scans.⁷

Other Tests

No other investigations are needed beyond routine preoperative workup.

Procedures

Fine-needle aspiration may be helpful to distinguish branchial cleft cysts from malignant neck masses.⁸ Fine-needle aspiration and culture may help guide antibiotic therapy for infected cysts.

Histologic Findings

Most branchial cleft cysts are lined with stratified squamous epithelium with keratinous debris within the cyst. In a small number, the cyst is lined with respiratory (ciliated columnar) epithelium. Lymphoid tissue is often present outside the epithelial lining. Germinal center formation may be seen in the lymphoid component, but true lymph node architecture is not seen. In infected or ruptured lesions, inflammatory cells are seen within the cyst cavity or the surrounding stroma.

Treatment

Medical Care

Antibiotics are required to treat infections or abscesses related to branchial cleft cysts.

Surgical Care

Surgical excision is definitive treatment for branchial cleft cysts.⁹

A series of horizontal incisions, known as a stairstep or stepladder incision, is made to fully dissect out the occasionally tortuous path of the branchial cleft cysts.
Branchial cleft cyst surgery is best delayed until the patient is at least age 3 months.
Definitive branchial cleft cyst surgery should not be attempted during an episode of acute infection or if an abscess is present.
Surgical incision and drainage of abscesses is indicated if present, usually along with concurrent antimicrobial therapy.
Sclerotherapy with OK-432 (picibanil) has been reported to be an effective alternative to surgical excision of branchial cleft cysts by some groups.¹⁰

Consultations

Referral to an otolaryngologist for surgical excision is indicated.

Follow-up

Further Outpatient Care

Postoperatively, patients should be monitored for branchial cleft cyst recurrence.
Because some patients have bilateral branchial cleft cyst lesions, the contralateral side should be examined.

Complications

Untreated branchial cleft cyst lesions are prone to recurrent infection and abscess formation with resultant scar formation and possible compromise to local structures.
Complications of surgical excision of branchial cleft cysts result from damage to nearby vascular or neural structures, which include carotid vessels and the facial, hypoglossal, vagus, and lingual nerves.

Prognosis

Following surgical excision of branchial cleft cysts, recurrence is uncommon, with a risk estimated at 3%, unless previous surgery or recurrent infection has occurred, in which case, it may be as high as 20%.

Patient Education

Patients should be reassured that branchial cleft cysts are benign.

Miscellaneous

Medicolegal Pitfalls

Failure to refer patients to an experienced head and neck surgeon is a pitfall. Patients should be referred to a surgeon experienced in head and neck surgery for surgical removal under general anesthesia.

References

Wagner AM, Hansen RC. Neonatal skin and skin disorders. In: Schachner LA, Hansen RC, eds. Pediatric Dermatology. Vol 1. 2^nd ed. New York, NY: Churchill Livingston; 1995:291-3.
Doi O, Hutson JM, Myers NA, McKelvie PA. Branchial remnants: a review of 58 cases. J Pediatr Surg. Sep 1988;23(9):789-92. [Medline].
Little JW, Rickles NH. The histogenesis of the branchial cyst. Am J Pathol. Mar 1967;50(3):533-47. [Medline].
Rickles NH, Little JW. The histogenesis of the branchial cyst. II. A study of the lining epithelium. Am J Pathol. May 1967;50(5):765-77. [Medline].
Telander RL, Deane SA. Thyroglossal and branchial cleft cysts and sinuses. Surg Clin North Am. Aug 1977;57(4):779-91. [Medline].
Anand TS, Anand CS, Chaurasia BD. Seven cases of branchial cyst and sinuses in four generations. Hum Hered. 1979;29(4):213-6. [Medline].
Bloch R. Images in emergency medicine. Branchial cleft cyst. Ann Emerg Med. Mar 2006;47(3):291, 308. [Medline].
Rosa PA, Hirsch DL, Dierks EJ. Congenital neck masses. Oral Maxillofac Surg Clin North Am. Aug/2008;20:339-52. [Medline].
Donegan JO. Congenital neck masses. In: Cummings CW, Schuller DE, eds. Otolaryngology - Head and Neck Surgery. 2^nd ed. St. Louis, Mo: Mosby; 1993:1554-9.
Kim MG, Kim SG, Lee JH, Eun YG, Yeo SG. The therapeutic effect of OK-432 (picibanil) sclerotherapy for benign neck cysts. Laryngoscope. Dec 2008;118(12):2177-81. [Medline].
Thompson LD. Branchial cleft cyst. Ear Nose Throat J. Nov 2004;83(11):740. [Medline].

Keywords

branchial cleft cyst, branchial cyst, lateral cervical cyst, congenital epithelial cyst

Contributor Information and Disclosures

Author

Chih-Ho Hong, MD, FRCPC, Clinical Assistant Professor, Department of Dermatology and Skin Science, University of British Columbia, Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Richard Crawford, MD, FRCPC, Head of Dermatology, Clinical Professor, Department of Medicine, Divisions of Pathology and Dermatology, University of British Columbia
Richard Crawford, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Pacific Dermatologic Association, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Jean Paul Ortonne, MD, Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France
Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology and American Dermatological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

Further Reading