Operative Techniques in Otolaryngology (2005) 16, 213-222

Excision of branchial cysts

John Houck, MD, MS, FACS
From the Department of Otorhinolaryngology, University of Oklahoma, Health Science Center, Oklahoma City, Oklahoma. KEYWORDS
Congenital anomalies; Branchial apparatus Branchial cleft anomalies are uncommon ndings that are often confused with other causes of masses and infections in the neck. Although more common in children, they may also present in adults. Second cleft anomalies are 8-10 times more common than those of the rst. Third and fourth cleft anomalies are quite rare. All offending epithelium must be surgically removed. A thorough understanding of the embryologic origin, presentation, diagnostic evaluation, and surgical treatment of these challenging lesions will help the head and neck surgeon to a good outcome. 2005 Elsevier Inc. All rights reserved.

The branchial apparatus appears during the fourth week of gestation, consisting of 4 well-dened paired sets of mesodermal arches, divided by endothelial-lined pouches internally and ectodermal lined clefts (grooves) externally. They are sequentially numbered from cranial to caudal. The ectoderm of each cleft is initially in contact with the endoderm of its pouch, forming a double-layered branchial membrane (Figure 1). The mesenchyme of each arch initially comes from mesoderm, which gives rise to skeletal muscle and vascular endothelium. During the fourth week, neural crest cells from the mid and hindbrain migrate into the arches, contributing to the mesenchyme that produces most of the skeletal and connective tissue structures. Each branchial arch contains a cartilaginous structure (skeleton), muscle, a nerve that supplies the muscles and mucosa, and an artery (Figure 2). During the fth week, the ventral aspect of the second arch enlarges and grows caudally to overlap the second, third, and fourth arches. These arches coalesce and sink in to form an ectodermal depression, the cervical sinus (Figure 3). By the seventh week, the cervical sinus and the second to fourth grooves disappear, giving a smooth contour to the neck. The only remaining groove, the rst, forms the external auditory canal (EAC) (Figure 4). The rst branchial pouch elongates to form the eustachian tube, middle ear, and mastoid antrum. The second pouch develops into the tonsillar fossa. The third pouch develops into a dorsal portion, which forms the inferior thyroid gland, and a ventral portion that gives rise to the thymus. These primordial structures initially connect to the
Address reprint requests and correspondence: John Houck, Department of Otorhinolaryngology, University of Oklahoma, Health Science Center, PO Box 26901, WP 1360, Oklahoma City, OK 73190-3048. E-mail address: john-houck@ouhsc.edu. 1043-1810/$ -see front matter 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.otot.2005.09.007

pharynx by the thymopharyngeal duct, which then degenerates. The inferior parathyroid glands migrate caudally to the dorsal surface of the thyroid gland, and the thymus descends into the superior mediastinum. The fourth pouch develops similarly to the third, with a dorsal portion developing into superior thyroid glands and a ventral portion developing into the ultimobranchial body. This structure, which is formed by neural crest cells, fuses with the thyroid gland, giving rise to the calcitonin producing parafollicular cells (C cells) (Figure 5). The only branchial membrane that persists is the rst, which forms the tympanic membrane.

Anomalies
Pathogenesis
The abnormal persistence of branchial apparatus remnants gives rise to branchial anomalies (ie, cysts, stulae, and sinuses). A cyst is lined by epithelium but has no external opening. A sinus is a blind pocket that opens internally (persistence of a pouch) or externally (persistence of a cleft). A stula is a tract that has both internal and external openings. The origin of stulae and sinuses seems directly related to the branchial apparatus as a result of the anatomic similarities. However, the origin of lateral cervical cysts remains controversial. A likely explanation is that cysts form from entrapped remnants of either pouches, clefts, or persistent ectoderm of the cervical sinus. Histologically, squamous epithelium lines most cysts, although some are lined by respiratory (pseudostratied ciliated columnar) epithelium and some by both types of epithelium. The cysts are often lined by subepithelial lymphoid tissue, replete with follicles and germinal centers. However, sinuses and stulae

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Figure 2 arches.

Skeletal and ligamentous derivatives of the branchial

Figure 1 weeks.

Horizontal section through the cranial region at 3-4

are more likely to be lined by respiratory epithelium. Associated lymphoid tissue tends to lack well-formed follicles. The thymopharyngeal duct theory suggests that the cysts are remnants of the original connection between the thymus and third pouch. However, a persistent thymopharyngeal duct has not been found, and the cysts only rarely have parathyroid or thymic tissue in them. Another theory suggests that cysts result from deposition of salivary gland (parotid) inclusions within cervical lymph nodes, but salivary tissue is only rarely seen in the cysts. Another theory suggests that epithelial metaplasia occurs in cervical lymph nodes. Up to 96% of specimens contain well-differentiated lymphoid tissue that cannot easily be explained by a branchial origin. Also, many of these cysts rst become apparent during a respiratory tract infection.

both the membranous and cartilaginous EAC, containing both skin and cartilage from both ectodermal and mesodermal origins. Anatomically, type I lesions run parallel to the EAC, involving parotid tissue and usually passing superior to the facial nerve. They usually are parallel to the EAC and open in the preauricular region. Type II lesions are more commonly located within the substance of the parotid gland and more closely related to the facial nerve, running either lateral, medial, or even through the main trunk of the nerve (Figure 7). With time, exceptions to the Work classication have been reported, with cysts classied as type I, containing both cartilage and skin, and cysts classied as type II, containing only skin.2 Newer classication schemes stress only that a lesion be a cyst, stula, or sinus for adequate preoperative planning. Approximately 8% to 10% of all branchial anomalies are rst cleft origin. The lesions have been found in all age groups,

First branchial cleft

Several classication schemes for these anomalies have been proposed, the most widely accepted being that of Work in 1972.1,2 Type I lesions are duplication anomalies of the membranous EAC, ectodermal in origin and without cartilage (Figure 6). Type II lesions are duplication anomalies of

Figure 3 sinus.

Early formation of the branchial arches and cervical

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Figure 4

Horizontal section at 7 weeks.

more often on the left side. There is probably no gender predominance. Cysts occur twice as often as sinuses or stulae, with cystic lesions more common in adults, and stulae and sinuses more common in children. The location of cysts and external sinus openings varies, usually occurring around the ear (anterior, inferior, and posterior to the pinna) or in the upper neck, anterior to the sternocleidomastoid muscle (SCM). The internal tracts for both sinuses and stulae are most often found along any portion of the EAC, with the anteroinferior bonycartilaginous junction being most common. Diagnosis Diagnosis can be difcult, with an average delay of 4 years, often only after infection. Preoperative plain lms,

Figure 6

First branchial cleft cyst, type I.

computerized tomography, or magnetic resonance imaging are probably of little value. Treatment Treatment is complete surgical removal of all abnormally placed epithelium. Surgery should wait until acute infection has resolved. Incision and drainage may be appropriate for an abscess. Repeated infections and limited sur-

Figure 5

The fourth pouch.

Figure 7

First branchial cleft cyst, type II.

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Figure 8

Second branchial cleft cyst. CN, cranial nerve.

geries (incision and drainage [I D]) lead to more scarring and a more difcult denitive procedure. A standard cervicomastoid parotidectomy incision with formal dissection of the facial nerve and a lateral parotidectomy are recommended to give the best chance of preserving the facial nerve and removing all the offending epithelium. Injection of the tract with methylene blue or insertion of a wire probe may assist with identication of the tract. If the tract extends to the EAC, adjacent canal skin and cartilage should be removed. Rarely, the tract may extend to the middle ear space or even extend along the eustachian tube. Transient weakness of the facial nerve is common. Recurrence is unlikely but is more common with brosis from prior infections and surgery.

posterior belly of the digastric muscle, between the internal and external carotid arteries, over the glossopharyngeal nerve, deep to the stylohyoid ligament to an internal opening at the upper pole of the tonsillar fossa. Although cysts can occur anywhere along this tract, they are most common just lateral to the internal jugular vein, at the level of the carotid bifurcation (Figure 8). Cysts Cysts occur 3 times more often than stulae and tend to present in an older group, the second to fourth decades. There is probably no gender or side predominance. Presentation is typically a painless, smooth, round neck mass, usually along the upper third of the anterior border of the SCM, appearing often after an upper respiratory tract infection. Acute infection can lead to abscess. Diagnosis The correct diagnosis is made in approximately half the cases. Preoperative computerized tomography or ne-needle aspiration helps to distinguish cysts from carotid body tumors, cystic metastases of squamous cell carcinoma, adenopathy, lymphoma, cystic hygroma, neurobromas, tuberculosis, and parotid gland lesions.

Second cleft and pouch

These defects represent 67% to 93% of all branchial anomalies; approximately 10% are bilateral.3 Such lesions would be expected to pass deep to second arch structures and over third arch structures. Thus, a stula extends from an external opening along the anterior border of the SCM, pierces the platysma muscle, and then follows the carotid sheath upward to the level of the hyoid bone. It then turns medially to pass over the hypoglossal nerve, beneath the

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Figure 9

Incision for second branchial cleft cyst.

Treatment Complete surgical removal of all offending epithelium must be performed, preserving the surrounding blood vessels and nerves, using a cosmetically acceptable incision. Acute infections must rst be treated, including incision and drainage for abscesses. Similar to rst cleft cysts, repeated I Ds or insufcient surgery makes complete surgical excision more difcult, with damage to critical neurovascular structures and recurrence more likely. Because most of these patients are younger, cosmesis of surgical access is an important issue. Incisions should be made along Langer lines, generally directly over the midpoint of the cyst (Figure 9). Additional exposure may be facilitated through a McFee, or stair step, incision (Figure 10). Vertical incisions should be avoided because of unsightly scars. Partial aspiration of some cysts may help dissection. Cysts lying medial to the carotid sheath are more easily removed transorally. Surgical procedure Inferior and superior subplatysmal aps are raised. The cyst is separated from the supercial layer of the deep cervical fascia that envelopes the SCM. Supercial jugular veins and cutaneous nerves are transected (Figure 11). The cyst is then dissected off the SCM, preserving the spinal accessory nerve. Dissection is easier if the cyst is intact. Traction with forceps must be gentle to avoid rupture (Figure 12). The cyst is dissected medially, carefully avoiding the carotid sheath and ansa hypoglossi. The facial vein may require transaction (Figure 13). The cyst is dissected off the posterior belly of the digastric and stylohyoid muscles (Figure 14). Posteriorly, the cyst or its tract crosses the hypo-

glossal nerve (XII) and the veins overlying it. The veins must be dissected or transected carefully to avoid bleeding. Indiscriminate clamping because of bleeding can easily injure XII. The tract crosses the glossopharyngeal nerve near the pharyngeal attachment. The superior laryngeal nerve is much deeper, passing obliquely behind the external carotid (Figure 15). The duct of the cyst can be difcult to differentiate from fascial attachments. If present, it passes between the external and internal carotid arteries, deep to the posterior belly of the digastric and stylohyoid muscles (Figure 16). The wound is irrigated and closed in multiple layers, including the supercial layer of the deep cervical fascia. A careful cosmetic closure is performed, such as a subcuticular suture with Steri-Strips (3M, St. Paul, MN), for a good cosmetic result in a young person. Complications The most common complication is recurrence. One large series of 208 cases showed recurrence in 21% of those with a history of prior surgery, 14% with a history of infection, and 3% with a history of neither. Sinuses and stulae Most are noted shortly after birth, with 78% being diagnosed before age 5 years. The opening onto the neck is typically along the anterior border of the SCM, between the hyoid bone and suprasternal notch. It may drain mucoid material, which may become purulent during an acute infection. They occur slightly more often in females and on the right side. Fistulae are less common than sinuses. They may be lined with squamous or respiratory epithelium.

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Figure 10

A stair step incision.

Lymphoid tissue tends to be scanty and not have a follicular pattern like that in cysts. Diagnosis Preoperative imaging is not likely to be helpful. Injection of the tract with contrast material may show the extent of the lesion and assist with preoperative planning. Treatment Complete surgical excision is necessary and can be delayed until an infant is 2-3 years old. Acute infection should

be treated and resolved before surgery. A stepladder or McFee incision will gain adequate access and leave less scarring than a vertical incision. The tract opening is excised along with an elliptical piece of surrounding skin. Dissection proceeds through the subcutaneous tissues, platysma, and the supercial layer of the deep cervical fascia. The second incision is made about the level of the hyoid bone and the tract threaded through it. Dissection can easily proceed to remove the entire tract. Dissection may be assisted by injecting the tract with methylene blue or inserting a exible catheter, such as an em-

Figure 11

Initial exposure of cyst.

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Figure 12

Further dissection of cyst with careful traction.

bolectomy catheter. Enough supporting tissue should be excised with the tract to ensure complete removal of all epithelium. Complications Dysfunction of the hypoglossal nerve is usually transient. Recurrences may require functional neck dissection.

Third and fourth pouch

Anomalies are relatively uncommon, with third arch anomalies at 2% to 8% and fourth at 1% to 2% of all branchial anomalies.4,5 Distinguishing between the 2 can be difcult because both enter the pyriform sinus and have

similar presentations. Tracts originating in the pyriform sinus have been associated with recurrent acute thyroiditis. Theoretically, a third arch pouch and cleft anomaly should pass between third and fourth arch structures. Therefore, a complete stula would then start in the pyriform sinus, pierce the thyroid-hyoid membrane, pass above the superior laryngeal and hypoglossal nerves, go beneath the glossopharyngeal nerve and carotid artery, pierce the platysma and surface in the skin anterior to the SCM (Figure 17). Theoretically, a fourth pouch anomaly would pass between the fourth and sixth arch structures (the fth arch is rudimentary). A complete stula would start in the pyriform sinus, exit the larynx near the cricothyroid joint, pass be-

Figure 13

Medial dissection.

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Figure 14

Dissection superiorly.

tween the superior and recurrent laryngeal nerves, then track down the tracheoesophageal groove. On the left side, it would curve around the aorta from posterior to anterior. On the right side, it would curve around the subclavian artery. The tract would then lie posterior to the carotid, pass over the hypoglossal nerve, and exit near the anterior border of the SCM. A fourth arch stula has never been described; a possible fourth pouch sinus tract has been described but was not completely dissected inferior to the clavicle.6 Clinically, both types begin at the pyriform sinus and end blindly in the paratracheal or thyroid regions. The fourth pouch sinuses only rarely extend beneath the clavicles. Histologically, both third and fourth pouch tracts may be associated with ectopic thymus and aberrant parathyroid tissue (both third pouch).7

Most fourth pouch anomalies are diagnosed in childhood, and 97% occur on the left side. There are 2 types of clinical presentation. In the rst, lateral neck cysts or abscesses occur in neonates and may be a cause of neonatal stridor.7 In the second, recurrent lateral neck abscesses or recurrent episodes of acute suppurative thyroiditis present in children, adolescents, and occasionally adults.8 Diagnosis The diagnosis can be quite difcult, often being made after repeat admissions for incision and drainage, and intravenous antibiotics. A plain lm showing air in the cyst can help differentiate a branchial anomaly from other causes of pediatric neck masses. The pharyngeal opening may be visualized by exible beroptic nasopharyngoscopy. A bar-

Figure 15

The hypoglossal nerve.

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Figure 16

Dissection of the duct.

ium pharyngoesophagram will show the sinus tract in almost every case, if 6 weeks are allowed to pass after infection to let associated inammation and edema to subside.9,10 The initial episode of acute thyroiditis in a child should promote a search for a pharyngeal sinus. Direct laryngoscopy should be performed before any surgical excisions and may be most easily performed at an incision and drainage procedure. Treatment The tract must be completely excised, including the area of the pyriform sinus attachment. The procedure can be difcult and should be performed only in the absence of acute infection. Some have found injection of the tract with methylene blue or catheterization with an embolectomy catheter helpful. The tract must be removed surgically. The relationship of the tract to the recurrent and superior laryngeal nerves must be determined. If the tract goes inferior to the superior laryngeal nerve (fourth arch) and superior to the recurrent nerve (sixth arch), then the anomaly is derived from the fourth pouch. However, if the tract passes superior to the superior laryngeal nerve and inferior constrictor, then a third pouch origin is likely. To permit visualization and resection of the pyriform sinus origin, the thyroid ala should be retracted medially, and the inferior constrictor muscle should be divided. If necessary, a vertical strip of posterior thyroid cartilage can be excised after disarticulation of the ipsilateral cricothyroid joint. After removing the pharyngeal attachment, the defect is closed with a purse-string suture.7 Partial or total thyroid lobectomy may be needed for fourth pouch sinuses. Dissection of the recurrent laryngeal nerve may be needed to distinguish clearly this nerve from the tract. The superior parathyroid gland should be preserved. Fourth pouch remnants terminate in the prethyroid space or within the gland itself. Care must be taken to preserve the superior parathyroid gland.

Figure 17

Third arch anomaly. CN, cranial nerve.

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4. Choi SS, Zalzal GH: Branchial anomalies: A review of 52 cases. Laryngoscope 105:909-913, 1995 5. Ford GR, Baladrishnan A, Evans JNG, et al: Branchial cleft and pouch anomalies. J Laryngol Otol 106:137-143, 1992 6. Shugar MA, Healy GB: The fourth branchial cleft anomaly. Head Neck Surg 3:72-75, 1980 7. Rosenfeld RM, Biller HF: Fourth branchial pouch sinus: Diagnosis and treatment. Otolaryngol Head Neck Surg 105:44-50, 1991 8. Narcy P, Aumont-Grosskopf C, Bobin S, et al: Fistulae of the fourth endobranchial pouch. Int J Pediatr Otorhinolaryngol 16:157-165, 1988 9. Lin JN, Wang KL: Persistent third branchial apparatus. J Pediatr Surg 26:663-665, 1991 10. Godin MS, Kearns DB, Pransky SM, et al: Fourth branchial pouch sinus: Principles of diagnosis and management. Laryngoscope 100: 174-178, 1990

Complications Complications include recurrent nerve vocal cord paralysis or pharyngocutaneous stula.

References
1. Work WP, Proctor CA: The otologist and rst branchial cleft anomalies. Ann Otol Rhinol Laryngol 72:548-562, 1963 2. Work WP: Newer concepts of rst branchial cleft defects. Laryngoscope 82:1581-1593, 1972 3. Gatti WM, Zimm J: Bilateral branchial cleft stulas: Diagnosis and management of two cases. Ear Nose Throat J 67:256, 258, 261, 1988