You are in: eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > HEAD AND NECK ONCOLOGY Neck, CystsArticle Last Updated: Nov 29, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Jonathan C Smith, MD, Voluntary Attending, Clinical Assistant Professor, Department of Otolaryngology, Albert Einstein College of Medicine, Montefiore Medical Center Jonathan C Smith is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Laryngological Rhinological and Otological Society Coauthor(s): Jonas T Johnson, MD, FACS, Professor and Chairman, Department of Otolaryngology, University of Pittsburgh School of Medicine; Professor, Department of Radiation Oncology, Professor, Department of Oral Maxillofacial Surgery, University of Pittsburgh School of Dental Medicine Editors: Benoit J Gosselin, MD, FRCSC, Associate Professor of Surgery, Dartmouth Medical School, Dartmouth College, Hanover, NH. Director, Comprehensive Head and Neck Oncology Program, Norris Cotton Cancer Center, Lebanon, NH. Staff Otolaryngologist, Division of Otolaryngology-Head and Neck Surgery, Dartmouth-Hitchcock Medical Center, Lebanon, NH; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Karen Hall Calhoun, MD, Chair, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Missouri; Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders; Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine Author and Editor Disclosure Synonyms and related keywords: neck cysts, branchial cleft cysts, branchial anomalies, thyroglossal duct cysts, TGDC, neck masses, congenital neck masses, metastatic squamous cell carcinoma, acquired laryngoceles, cystic schwannomas, lymphatic malformations, benign inflammatory lesions, thyroid gland anomalies, lymphangioma, cystic hygroma, macrocystic lymphatic malformations INTRODUCTIONSection 2 of 12
  The differential diagnosis for cysts in the neck includes congenital neck masses, metastatic squamous cell carcinoma, acquired laryngoceles, and cystic schwannomas. Congenital masses in the neck include branchial cleft cysts, thyroglossal duct cysts (TGDCs), ectopic thymus cysts, dermoid and teratoid cysts, cystic vascular abnormalities, and lymphatic malformations such as the cystic lymphangioma. This article mainly focuses on the common entities of branchial cleft cysts and TGDCs, and, to a lesser degree, it discusses lymphatic malformations. ProblemMost neck masses in children are benign inflammatory lesions, which can be successfully treated medically with antibiotics. Most neck masses in children that require surgery for diagnostic and therapeutic purposes are congenital in origin. TGDCs and branchial cleft cysts are the 2 most common congenital lesions. Baer first described the branchial apparatus in 1827. The branchial arches begin to develop during the second week of gestation. During the fourth week of fetal development, 5 ridges, known as branchial arches, form on the ventrolateral surface of the embryonic head. Each arch contains mesoderm from which cartilage, muscle, and bone develop. Each arch is separated from the other arches by an external cleft of ectodermal origin. Each arch also has an internal pouch of endodermal origin and an associated cranial nerve, artery, and cartilage. The anatomic location of a branchial cleft abnormality represents the presumed branchial cleft of origin. Branchial cleft anomalies can manifest in several different forms, including the following:
FrequencyMore than 90% of branchial cleft anomalies arise from the second branchial cleft system. Approximately 8% of branchial cleft anomalies arise from the first branchial cleft system. Cysts arising from the third and fourth branchial cleft system rarely occur. Branchial cleft anomalies and TGDCs occur with equal frequency in males and females. Branchial cysts are twice as common as either branchial sinuses or fistulas. TGDCs are the most common mass found in the midline of the neck in children. EtiologyBranchial cleft anomalies Several theories are proposed to explain the genesis of branchial cleft anomalies. Incomplete closure of branchial clefts and pouches with failure of obliteration of the cervical sinus of His is suggested as a possible mechanism. Incomplete closure of branchial clefts and pouches with rupture of the branchial plate is another possible mechanism. Thyroid gland anomalies The thyroid gland forms as a diverticulum from the floor of the pharynx (tuberculum impar) at a site that later becomes the foramen cecum of the base of the tongue. The thyroid gland then forms 2 lobes and descends along a hollow canal called the thyroglossal duct in the midline of the neck. During the descent, the thyroglossal duct passes in close proximity to the developing hyoid bone. The thyroglossal duct normally involutes; however, when involution does not occur, the potential for development of a TGDC increases. Arrest in the normal descent of the gland results in ectopic thyroid tissue. The recognition of a cyst in the neck may not occur until decades later, commonly in association with a minor upper respiratory infection. This suggests that the potential for a cyst had existed for years. Many patients with a TGDC have some thyroid tissue in the cyst; therefore, thyroglossal duct anomalies might be a better term for their condition. Lymphatic malformations Lymphatic malformations, which include lymphangioma and cystic hygroma, are areas of localized abnormal development of the lymphatic system. These malformations most commonly occur in the head and neck area. Although the exact embryonic origin of lymphatic malformations is unknown, several etiologies have been proposed. Possible causes include failure of the venous and lymphatic systems to connect, abnormal sequestration of the lymphatic tissue during embryogenesis, and abnormal budding of lymphatic structures from the cardinal vein. Other likely etiologies that focus on an acquired process include infection, trauma, and lymphatic obstruction. PathophysiologyBranchial system anomalies can manifest as a sinus, fistula, or cyst. Branchial cleft sinuses with external openings are usually associated with the first and second branchial cleft arches. Branchial cleft sinuses with internal openings are usually associated with the third and fourth arches. First branchial cleft anomalies are subdivided into 2 types based on anatomic location. In type 1 first branchial cleft anomaly, the cyst or sinus opening can be located medial, inferior, or posterior to the conchal cartilage and pinna. A sinus tract, when present, parallels the external auditory meatus. In a type 2 first branchial cleft anomaly, the cyst and sinus tract are located in the anterior neck, always superior to the hyoid bone. A sinus tract or fistula, when present, travels over the angle of the mandible and through the parotid gland to terminate around the bony-cartilaginous junction in the external auditory meatus. The relationship to the facial nerve and parotid gland is variable with first branchial cleft cysts. Second cleft anomalies are the most common of the branchial system. When an external opening is present in a second branchial cleft anomaly, the opening is located along the anterior border of the sternocleidomastoid muscle near the junction of the lower- and middle-third portions of the muscle, below the level of the hyoid bone. An internal opening, when present, is located in the tonsillar fossa. When present, a fistula ascends from the lower neck along the carotid sheath and crosses over the hypoglossal nerve and glossopharyngeal nerve. The fistula then passes between the internal and external carotid arteries to end in the tonsillar fossa. Second branchial cleft cysts are more common than sinuses or fistulas. The location of the cyst can be anywhere along the course of the fistula but most commonly is in the anterior triangle of the neck below the level of the hyoid bone. Third branchial cleft anomalies are rare. When present, the external opening is located in the same location as a second branchial cleft anomaly; that is, along the anterior border of the sternocleidomastoid muscle at the junction of the middle and lower third portions of the muscle. The internal opening is located in the pyriform sinus rather than in the tonsillar fossa. The fistula tract ascends along the carotid sheath posterior to the internal carotid artery, then between the hypoglossal and glossopharyngeal nerves. The fistula tract then pierces through the thyrohyoid membrane and opens into the pyriform sinus. Cysts can occur at any location along this course but are usually found in the anteroinferior cervical triangle on the left side. In 1972, Sanborn reported the first case of a fourth branchial arch anomaly.1 If a fourth branchial arch anomaly occurs, it may have (1) an internal opening located near the apex of the pyriform sinus, (2) a fistula or sinus tract that travels between the superior and inferior laryngeal nerves, or (3) an external opening along the anterior border of the sternocleidomastoid muscle in the lower neck. The literature contains more than 30 cases of reputed fourth branchial cleft anomalies. Most are on the left side, with only 5 occurring on the right side. ClinicalBranchial cleft anomalies Branchial cleft cysts manifest in a different manner than branchial sinuses and branchial fistulae. The typical branchial cleft cyst, in the absence of infection, manifests as a nontender, smooth, round mass located along the anterior border of, or just deep to, the sternocleidomastoid muscle. Depending on the arch of derivation, the location can be anywhere from the external auditory canal to the clavicle. Branchial cleft cysts usually enlarge gradually and often are not detectable until the second or third decade of life. Branchial cleft cysts that manifest in early childhood usually occur with an acute and painful enlargement of the cysts secondary to an upper respiratory infection. Branchial cleft cysts commonly increase in size in the presence of an upper respiratory tract infection and then decrease in size as the infection resolves. An infected branchial cleft cyst can progress into an abscess or rupture spontaneously to form a draining sinus tract. Neonatal patients and patients with larger cysts can present with aerodigestive tract compromise and associated symptoms of stridor, dyspnea, and dysphagia. Second and third branchial cleft cysts can cause stridor with life-threatening airway obstruction in neonates. In contrast to those patients with branchial cleft cysts, patients with branchial cleft sinuses and fistulas often present soon after birth because the external opening is visible on the skin. Branchial cleft fistulas and sinuses may also be palpable as a fibrous cord extending along the anterior border of the sternocleidomastoid muscle. Mucus drainage may occur from the external opening. Secondary infections from cutaneous organisms, most commonly Staphylococcus aureus and group A beta-hemolytic streptococci, cause purulent drainage and erythema and edema. An infected first branchial cleft sinus or fistula may cause aural drainage in the absence of otitis media or otitis externa. A child presenting with both a draining ear and a tender mass in the neck may rarely have a first branchial cleft cyst associated with a sinus or fistula tract. A child presenting with a mass in the neck and a draining tract along the anterior border of the sternocleidomastoid muscle may have a second or third branchial cleft anomaly. A child with paratracheal swelling associated with tenderness and fever may have acute suppurative thyroiditis. Multiple authors have documented the spread of bacteria from an internal opening in the pyriform sinus in third branchial cleft anomalies. Thyroglossal duct cysts A TGDC is the most common mass found in the midline of the neck. The mass is usually located at or below the level of the hyoid bone, although a TGDC can be located anywhere from the foramen cecum to the level of the thyroid gland. Most patients with a TGDC present with asymptomatic masses in the midline of the neck. The literature reports that most of these lesions occur in patients younger than 30 years. Interestingly, over the last decade, a number of older patients are presenting with a TGDC, some of whom are aged 80-90 years. Recurrent inflammation associated with infection of a TGDC is not uncommon. When infection is present, the cyst often enlarges and an abscess may form. Spontaneous rupture with secondary sinus tract formation can also occur. Lymphatic malformations Approximately half of all lymphatic malformations are diagnosed at birth, and 90% are diagnosed by age 2 years. With today's use of prenatal ultrasonography, diagnosing lymphatic malformations before birth is not uncommon. Most of these neck cysts present as slow-growing cystic cervical masses. When the mass is located in the suprahyoid location, the patient can present with respiratory compromise. Infection and hemorrhage of the malformation can cause sudden enlargement with respiratory compromise. INDICATIONSSection 3 of 12
Treat branchial cleft anomalies, TGDCs, and lymphatic malformations for several reasons, including the following:
RELEVANT ANATOMYSection 4 of 12
First branchial cleft fistulas and sinuses have a variable relationship to the facial nerve. No reliable methods are currently available to determine definitively the relationship between the sinus tract or fistula and the facial nerve prior to surgery. Surgeons, therefore, must be prepared for dissection of the facial nerve prior to embarking upon surgery for a first branchial cleft anomaly. A facial nerve monitor may be helpful. CONTRAINDICATIONSSection 5 of 12
Acute infection is a relative contraindication for surgical excision. Acute infection makes dissection more difficult and increases the risk of injury to surrounding structures. If possible, treat acute infection with appropriate IV antibiotics. If the infection is unresponsive secondary to abscess formation, incision and drainage or repeated aspiration in conjunction with antibiotic treatment may be required. WORKUPSection 6 of 12
Lab Studies
Imaging Studies
Other Tests
Diagnostic Procedures
TREATMENTSection 7 of 12
Medical therapyIf the cyst is infected, treatment with an antibiotic is indicated before planning surgical excision. Complete resolution of inflammation allows for an easier and safer dissection. Appropriate intravenous antibiotics that cover upper airway pathogens include ampicillin and sulbactam (Unasyn), clindamycin, and several other drugs. Oral antibiotics can be used after the infection has improved greatly on intravenous antibiotics and for minimally infected lesions. Appropriate oral antibiotics include amoxicillin (Augmentin), clindamycin, and several other agents. Base the final choice of antibiotics for both intravenous treatment and oral treatment on culture and sensitivity results; therefore, needle aspiration of an acutely infected cyst early in the course of treatment is often helpful. Macrocystic lymphatic malformations have been shown to be responsive to the sclerotherapy agent OK-432. OK-432 is a biologic preparation of lyophilized powder containing Streptococcus pyogens (group A streptococci) treated with benzylpenicillin potassium. Research with OK-432 is ongoing, but several authors have reported complete or substantial response with 4 injections of this agent given 6-8 weeks apart. Patients with microcystic disease did not respond to this treatment. Surgical therapyComplete surgical excision is the goal when treating branchial cleft anomalies and TGDCs. Unless dangerous compromise of the aerodigestive tract is present, avoid incision and drainage procedures. These procedures often obscure planes and make future complete surgical excision more difficult. Surgical excision is an excellent option for lymphatic malformations that can be removed without sacrificing important adjacent structures. However, lymphangiomas and cystic hygromas frequently involve adjacent structures, which can lead to high complication rates. Infrahyoid lymphatic malformations without mediastinal involvement have been shown to have the most favorable outcome with surgical excision (see Images 12-15). Preoperative detailsSee Medical therapy. Intraoperative detailsFirst branchial anomalies Excise branchial cleft anomalies under general anesthesia. Avoidance of paralytic agents by the anesthesia team is necessary in order to identify and dissect out the facial nerve. A parotidectomy incision is made with incorporation of an ellipse of skin around any prior surgical scars or sinus and fistula tracts. An s-shaped parotidectomy incision starting in the preauricular crease and extending under the mandible is appropriate. When operating on younger children, be aware that the typical cartilaginous pointer and styloid process landmarks often are not present or are poorly developed. In these cases, the facial nerve is just deep to the parotid fascia; therefore, locating the nerve first in the neck is safer. The facial nerve is found in the triangle formed by the anterior border of the sternocleidomastoid muscle, the posterior belly of the digastric muscle, and the cartilage of the external auditory meatus. Once the facial nerve is identified, the bifurcation and the upper and lower divisions are exposed completely with meticulous dissection. The sinus or fistula tract is dissected with a narrow cuff of normal tissue. Lacrimal probes and dilators can help identify and facilitate the dissection of a first branchial cleft fistula. The tract may be located either superficial or deep to the facial nerve. If required, the freely dissected branchial cleft anomaly is passed over or under the main branches of the facial nerve. For complete removal, a small portion of the external auditory cartilaginous canal may need to be excised along with the tract. The tract rarely involves the tympanic membrane, which requires a tympanoplasty for complete removal. Second and third branchial anomalies A horizontal skin incision is made in a natural skin crease, incorporating any prior incisions or sinus tracts in an ellipse. Flaps are raised, paying close attention to protect the marginal mandibular branch of the facial nerve. After the inferior aspect of the cyst is dissected free, the dissection is carried superiorly along the tract. As the tract crosses over the hypoglossal nerve, bleeding is often encountered from the lingual venous plexus. When controlling this bleeding, care must be taken not to injure the hypoglossal nerve. The tract is then followed between the internal and external carotid arteries with second arch anomalies or posterior to the internal carotid artery with third arch anomalies. If no internal opening is present, the tract can be ligated just as it passes deep to the posterior belly of the digastric muscle. A tonsillectomy may be required for complete excision of second branchial arch anomalies with an internal opening present in the tonsillar fossa. A thyroid lobectomy is required during the excision of a third branchial cleft anomaly. If the fistula tract is long, a useful technique involves making 2 separate horizontal skin incisions. This stepladder technique, with one low cervical incision and a higher incision at the level of the hyoid bone, allows for excellent exposure without excessive trauma from heavy traction on the skin flaps. Avoid vertical skin incisions because the resultant scar is cosmetically unappealing. Fourth branchial anomalies Although controversy remains regarding the identification of third and fourth branchial sinuses, most authors recommend complete excision of the cyst along with the sinus tract. Verret et al reported a series of 10 patients with fourth branchial cleft anomalies who were treated with endoscopic cauterization of the sinus tract.2 In this procedure, a direct laryngoscopy is performed and an intravenous balloon catheter, or angiocath, is passed into the sinus tract located at the apex of the pyriform sinus. The balloon is then inflated to dilate the opening of the sinus. An electrocautery ball coagulator is placed into the sinus tract opening and, with coagulation and manipulation, the tract is closed. Thyroglossal duct cysts The suggested surgical approach to the TGDC is the Sistrunk operation. The operation includes excision of the cyst in continuity with the mid portion of the body of the hyoid bone and a small block of muscle around the foramen cecum. The procedure is performed under general anesthesia with a shoulder roll in place. A skin incision is made in a natural skin crease near the cyst; any previous incisions or sinus tracts are included in the ellipse. A superior flap is raised to the level of the hyoid bone, and an inferior flap is raised until the inferior aspect of the cyst is identified. The strap muscles are separated, and the cyst is dissected from the surrounding structures until it is attached only to the hyoid bone superiorly. The body of the hyoid bone is skeletonized and then transected on each side of the pedicle. A curved retractor is placed transorally, which facilitates excision of the tract up to the base of tongue with the inclusion of a 5-10 mm core of muscle. Postoperative detailsA Penrose drain, or suction drain, is placed in the incision, and the neck is wrapped with a pressure dressing at the end of the operation. On postoperative day 1, the dressing can be removed and the patient is usually sent home later that day, after tolerating an oral diet. Some patients are discharged home on the same day as the surgery. Follow-upPatients are usually seen in the office 2-3 weeks after the procedure to make sure that the wound is well healed and that no problems are encountered. COMPLICATIONSSection 8 of 12
Complications from operating on a branchial cleft or thyroglossal duct anomaly include infection, hematoma, and recurrence. Branchial cleft anomalies Ideally, remove the cyst intact without entering the cystic cavity. Removing these lesions in a piecemeal manner increases the chance of leaving residual epithelial tissue and, therefore, increases the risk of recurrence. Take care also in obtaining meticulous intraoperative hemostasis. Transient, and even permanent, injury to the facial nerve can be a complication of operating on first branchial cleft anomalies. The internal and external carotid arteries, internal jugular vein, and cranial nerves IX, X, XI, and XII all are at risk when dissecting second and third branchial cleft anomalies. The recurrent laryngeal nerve especially is at risk when dissecting along the tract of a third branchial cleft anomaly. Thyroglossal duct cysts The greatest risk of recurrence of a TGDC is associated with failure to follow the surgical principles described by Sistrunk. Rates of recurrence are also increased when a TGDC is ruptured during dissection. Previous infection, prior incision and drainage procedures, and adherence of the cyst to the skin all are associated with an increased rate of rupture with dissection. Wound infections can also occur when the cyst is ruptured or when the pharynx is entered. OUTCOME AND PROGNOSISSection 9 of 12
With no history of infection or prior surgery, the recurrence rate after operating on a branchial cleft anomaly is approximately 3%. The recurrence rate increases to approximately 20% after prior unsuccessful attempts at surgical removal. The recurrence rate associated with simple excision of a TGDC is approximately 50%. The recurrence rate with a formal Sistrunk procedure is approximately 5%. FUTURE AND CONTROVERSIESSection 10 of 12
Branchial cleft anomalies The risk of malignancy arising from a branchial cleft cyst has been a topic of debate for years. Von Volkmann first described branchial cleft cyst carcinoma in 1882; however, the consensus today is that branchial cleft carcinoma rarely, if ever, occurs. In 1950, Martin et al reviewed the literature and rejected all of the previous reports of branchial cleft cyst carcinoma.3 They proposed that most of these patients had a metastatic squamous cell carcinoma cyst from an undiagnosed primary lesion. Before diagnosing a patient with a branchial cleft cystic carcinoma, the patient must meet the following criteria:
In 1979, Batsakis estimated that, even when including questionable cases of branchial cleft carcinoma, the incidence of branchial cleft carcinoma is approximately 0.3% of all malignant head and neck neoplasms.4 On the other hand, metastatic cystic lymph nodes are quite common; therefore, patients with cystic carcinoma in the neck are assumed to have carcinoma metastatic to cervical lymph nodes from an unknown primary site. Thyroglossal duct cysts Literature regarding carcinoma arising from a TGDC does not share the same controversy as reports of branchial cleft cyst carcinoma. In 1925, Ashurst and White first reported a case of carcinoma arising in a thyroglossal duct remnant.5 Since that time, more than 100 cases have been reported, with the overall incidence of TGDC cancer being less than 1%. For unclear reasons, cancer in a TGDC is more common in females than in males. The diagnosis of carcinoma arising in a TGDC is usually made histologically only after the tumor has been removed. MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
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