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Dermatology > PEDIATRIC DISEASES
Branchial Cleft Cyst
Article Last Updated: Oct 31, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Chih-ho Hong, MD, FRCPC, Staff Physician, Clinical Instructor, Division of Dermatology, University of British Columbia, Canada
Coauthor(s):
Richard Crawford, MD, FRCPC, Head of Dermatology, Clinical Professor, Department of Medicine, Divisions of Pathology and Dermatology, University of British Columbia
Editors: Jean Paul Ortonne, MD, Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; 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; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
branchial cyst, lateral cervical cyst, congenital epithelial cyst
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.
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.
Frequency
United States
The exact incidence 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.
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.
Sex
No sexual predilection is recognized.
Age
Branchial cleft cysts are congenital in nature, but they may not present clinically until later in life, usually by early adulthood.
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, patients may present with locally compressive symptoms.
- A family history may be present.
Physical
- Primary 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 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.
Other Problems to be Considered
Lymphadenopathy (reactive, neoplastic, lymphoma, metastasis)
Vascular neoplasms and malformations
Capillary hemangioma
Carotid body tumor
Lymphatic malformation (cystic hygroma)
Ectopic thyroid tissue
Ectopic salivary tissue
Lab 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.
- 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.
Other Tests
- No other investigations are needed beyond routine preoperative workup.
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.
Medical Care
Antibiotics are required to treat infections or abscesses.
Surgical Care
- Surgical excision is definitive treatment for this condition.
- A series of horizontal incisions, known as a stairstep or stepladder incision, is made to fully dissect out the occasionally tortuous path of the cyst.
- Surgery is best delayed until the patient is at least age 3 months.
- Definitive 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.
Consultations
Referral to an otolaryngologist for surgical excision is indicated.
Further Outpatient Care
- Postoperatively, patients should be monitored for recurrence.
- Because some patients have bilateral lesions, the contralateral side should be examined.
Complications
- Untreated lesions are prone to recurrent infection and abscess formation with resultant scar formation and possible compromise to local structures.
- Complications of surgical excision 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, 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 the condition is benign.
Medical/Legal 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.
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Branchial Cleft Cyst excerpt Article Last Updated: Oct 31, 2006
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