AUTHOR AND EDITOR INFORMATION

Section 1 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

INTRODUCTION

Section 2 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

Background

A true cyst is an enclosed space usually containing fluid and lined by epithelium. A pseudocyst is a cystlike structure without epithelial lining.

Cysts may be classified on the basis of their pathogenesis and histologic features. The primary categories are appendageal and adnexal cysts, developmental cysts, pseudocysts, and neoplasms with cystic changes. The most common lining is stratified squamous epithelium, which is observed in the most common appendageal cysts, such as the epidermoid (infundibular) and pilar (isthmic catagen, trichilemmal) cysts. Some developmental cysts have a lining of columnar epithelium and are classified as cutaneous columnar cysts; these cysts include branchial, thyroglossal, thymic, bronchogenic, cutaneous ciliated, and median raphe cysts. The term ciliated cyst has been used for several unrelated entities with a ciliated epithelial lining; these unrelated entities represent aberrant embryologic processes because no typical cutaneous structures contain cilia.

Pathophysiology

Columnar cysts are developmental cysts derived from embryologic vestiges, such as the branchial arch cleft, the thyroglossal duct, the tracheobronchial bud, the urogenital sinus, and müllerian structures. Some of these cysts arise along the lines of embryologic closure. Many midline cystic structures represent incomplete involution of the embryologic vestiges and are typically located in areas where these structures appear. Most of these lesions may appear anywhere in the course of these embryologic structures from the deep parts of the tissue up to the skin surface. They are clinically noticed if they cause recognizable subcutaneous masses, but they may remain unrecognized if they are deep and asymptomatic. All lesions may have cysts, fistulae, or sinuses in the same anatomical region.

Neck

Branchial cleft cysts (lateral neck cysts) are located in the anterior triangle of the neck, anterior of the sternocleidomastoid muscle. They originate from remnants of the second, third, and fourth cervical clefts.

Thyroglossal cysts are the most common midline structures in the neck, occasionally visible at the base of the tongue. When the thyroid anlage descends from the base of the tongue to the anterior part of the neck, it remains connected to the base of the tongue by a midline embryonic structure, the thyroglossal duct. This duct disappears by the ninth week of gestation. If the duct or a part of it persists, a thyroglossal sinus, a cyst, or ectopic thyroid tissue may develop anywhere along the duct.

The thymus develops from the inferior part of the third pharyngeal pouch and migrates to the mediastinum. Parts may remain in the neck and the thyroid gland and may form an ectopic thymus; thymic cysts may develop from remnants of the thymopharyngeal duct.

Bronchogenic cysts are midline lesions, usually located in the suprasternal notch. They arise from remnants of the lung bud (tracheobronchial) of the foregut.

Genitals

In male patients, median raphe cysts may be observed on the ventral aspect of the penis as subcutaneous cysts anywhere in the midline from the urethral meatus to the anus. Several hypotheses exist to explain median raphe cysts, including incomplete fusion of the urethral folds during embryonic development, ectopic periurethral glands of Littre, and anomalous outgrowth and sequestration of columnar epithelium from the urethra occurring after closure of the median raphe.

Legs

Cutaneous ciliated cysts may occur anywhere but are primarily observed on the legs of female patients. They may represent migratory müllerian duct structures related to the fallopian tubes, the uterus, and the upper part of the vagina. The occasional appearance of cutaneous ciliated cysts in males may be the result of vestigial müllerian structures, or the cysts may be the result of a different genesis (eg, derived from sweat glands). Female hormones may play a role in stimulating the ciliated epithelium.

Frequency

United States

All developmental cysts are uncommon. The least common cyst is probably the cutaneous ciliated cyst, with fewer than 30 cases reported. The most common cyst is the thyroglossal cyst.

Mortality/Morbidity

The cutaneous presentation of a cyst often represents only the superficial aspect of a deeper process. In areas of the head and the neck, deep extension may involve vital structures in the neck and the central nervous system.

• Lingual thyroglossal cysts may cause feeding problems and airway obstruction in neonates.
• Thyroglossal duct cysts may be inapparent until complications, such as inflammation, rupture, or infection, occur.
• Although rare, papillary thyroid carcinoma may develop in thyroglossal cysts during adulthood.
• Squamous cell carcinoma in branchial cysts is another rare complication.

Race

No racial predilection is known.

Sex

• Cutaneous ciliated cysts are primarily observed in females.
• Median raphe cysts are primarily observed in males.
• The other cysts have an equal sex ratio.

Age

All developmental cysts are present at birth and are enhanced by agents, such as hormones and trauma, to become clinically evident.

• Thyroglossal cysts present in the first decade of life.
• Thymic cysts present in the first or second decade of life.
• Bronchogenic cysts and median raphe cysts present early, some at birth, while others present within the first 3 decades of life.
• The cutaneous ciliated cyst usually presents in the second or third decade of life.

CLINICAL

Section 3 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

History

• Patients with dermal or subcutaneous nodules are usually asymptomatic and present with an enlarging swelling cyst.
• If cysts are secondarily infected, inflamed, or ruptured, they may be symptomatic, causing pain and tenderness. Carcinomas may also arise within cysts and cause pain or other complications of enlarging tumors.

Physical

Physical findings are limited to the skin.

• Thyroglossal cysts may occur anywhere along the thyroglossal duct from the base of the tongue to the anterior part of the neck. In the neck, they present as asymptomatic, gradually enlarging, round, midline or off-midline masses that move with swallowing because of their attachment to the hyoid bone. Sinuses or fistulae that drain clear or purulent fluid may be associated with the anterior part of the neck.
• Thymic cysts are rare lesions found in the mediastinum or the neck. In children, the cervical lesions present as painless swellings. These lesions are most often found posterior of the lateral lobe of the thyroid, more often on the left side. They are 1-15 cm in diameter.
• Bronchogenic cysts are present at or shortly after birth in the suprasternal notch. They have also been reported on the neck, the scapular area, and the chin. Cysts may increase in size, form sinuses, and drain mucoid fluid.
• Cutaneous ciliated cysts occur primarily on the legs of female patients, but they also have been seen in other locations. They present as subcutaneous swellings without a central pore.
• Median raphe cysts present as midline developmental cysts on the ventral aspect of the penis and the scrotum on the raphe connecting the external urethral meatus to the anus. They are most often observed close to the meatus on the glans.

Causes

• The causes of cutaneous columnar cysts are genetic. They are mostly caused by embryologic remnants and incomplete fusion.
• Abrupt onset of median raphe cysts is observed with local trauma or infection.

DIFFERENTIALS

Section 4 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

Other Problems to be Considered

Intradermal nevi
Adnexal tumors

WORKUP

Section 5 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

Imaging Studies

• Imaging should be performed on these cysts to exclude a deep connection; however, whether one imaging modality is superior to others is uncertain. Ultrasonography, CT scanning, or MRI may be helpful in locating thyroid tissue.
• Perform thyroid function tests with radionucleotide scanning on thyroglossal cysts to assess the location and to determine the function of the thyroid. Perform these tests preoperatively because sometimes the ectopic thyroid is the only functioning thyroid tissue and removal of the ectopic thyroid causes hypothyroidism.

Histologic Findings

In thyroglossal cysts, tubular glands are lined by respiratory epithelium with columnar cells.

The wall of thymic cysts contains Hassall corpuscles; lymphoid tissue; and, occasionally, parathyroid tissue and cholesterol granulomas. Although the lining is often columnar, it may also be squamous, cuboidal, or pseudostratified columnar.

Bronchogenic cysts are lined by ciliated pseudostratified columnar or cuboidal epithelium that produces mucin. Smooth muscle and mucous glands are found in the wall. Cartilage is seldom observed. Some inflammation and fibrosis in the surrounding tissues are common.

Cutaneous ciliated cysts are lined by ciliated cuboidal or columnar pseudostratified epithelium. Papillary projections into the lumen and cysts may be multilocular. Mucinous cells are rare. No smooth muscle or glands can be demonstrated.

Median raphe cysts are located in the dermis and are lined by pseudostratified columnar epithelium. Mucous glands and cilia are occasionally found. Near the meatus, the lining changes to stratified squamous epithelium.

TREATMENT

Section 6 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

Medical Care

In cases of secondary infection, systemic antibiotics are indicated.

Surgical Care

Surgical excision of the entire thyroglossal duct cyst is indicated.

• Duct remnants and part of the hyoid bone are usually excised to prevent recurrence.
• Removal should be complete, and removal of the entire embryologic remnant should be attempted.

Consultations

• Dermatologist - For evaluation of skin lesions
• Radiologist - For ultrasonography, CT scanning, MRI, and radionucleotide scanning
• Pathologist - For histologic evaluation
• Otolaryngologist, surgeon, or urologist - For surgical excision and repair

FOLLOW-UP

Section 7 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

Complications

• Infrequently, cysts may be associated with a malignant neoplasm, such as papillary thyroid carcinoma in the thyroglossal duct cyst. Branchial cleft cysts may also develop malignancies, such as carcinoma, in their wall. Infection, inflammation, rupture, fistulation or sinus formation, and drainage are further problems that may be encountered. These complications are the reasons why these cysts are surgically removed.

Prognosis

• In general, the prognosis of patients with these cysts is favorable.
• Recurrence is infrequent provided that the excision was complete and that embryologic remnants were also removed.
• If a cyst develops a malignancy, the type of neoplasm determines the prognosis.

REFERENCES

Section 8 of 8

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• References

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Article Last Updated: Jan 12, 2007