Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

A branchial anomaly, also known as a lateral cervical anomaly, is a congenital developmental defect that arises from the primitive branchial apparatus. Branchial anomalies have been recognized since at least 1932, when they were described by Ascherson.

The branchial apparatus undergoes its development and differentiation between the third and seventh weeks in the human embryo. Initially, five ridges appear on the ventrolateral surface of the embryonic head. These ridges represent branchial arches which consist of a core of mesenchyme, covered externally with ectoderm and internally with endoderm. Therefore adjacent arches are separated by ectodermal clefts externally and endodermal pouches internally. The clefts and pouches approach each other to form a closing membrane. In man, the cleft and pouch is gradually obliterated by invasion of the surrounding mesenchyme. In fish the closing membranes rupture to form gill clefts.

As development proceeds, the second arch increases in thickness and proliferates caudally to meet the enlarging epipericardial ridge of the fifth arch. As a result, the second, third, and fourth branchial clefts become enclosed in an ectodermally-lined cavity, the cervical sinus of His. Eventually, the sinus becomes obliterated by the apposition and fusion of its walls, yielding a smooth uniform contour to the external surface of neck. The endoderm derivatives of the pharyngeal pouches, including the thymus, parathyroid glands, and ultimobranchial body, then migrate to their final position.

Each arch is supplied by its own artery, which connects the primitive paired dorsal and ventral aortas. The first two arch arteries usually become vestigial; however, the artery of the second arch may persist as the stapedial artery. The artery of the third arch persists as the proximal portion of the internal carotid artery. The right fourth artery becomes the proximal portion of the right subclavian artery, while the left fourth becomes the arch of the aorta. The right fifth arch artery involutes while the left one remains as the ductus arteriosus. The sixth pair forms the pulmonary arteries.

A number of theories exist as to how branchial cysts and sinuses are formed. Most widely accepted is the branchial hypothesis which attributes the formation of the branchial anomalies to the incomplete closure of branchial clefts and pouches or failure of obliteration of the cervical sinus of His. The sinuses are vestigial branchial pouches or clefts. Fistulas result from remnants of both the pouch and cleft with rupture of their interposed branchial plate, and cysts are formed from entrapped remnants of either branchial clefts or pouches without complete sinus tracts.

Branchial cysts present in a different morphologic pattern from that of sinuses and fistulas. The branchial cysts are more common than the sinus or fistula.

The branchial cleft cyst is usually a smooth, round, nontender mass located along the anterior border of the sternocleidomastoid muscle at any position between the tragus and the clavicle. It may be first noticed at any age, but it is most commonly noticed in the second through fourth decades of life. Males and females are equally affected. It may enlarge with or without tenderness during periods of upper respiratory tract infection. An inflamed cyst may progress to abscess formation with the possibility that rupture will lead to permanent sinus formation.

A branchial sinus or fistula is almost always seen shortly after birth. It may occur bilaterally in as many as one-third of those affected. Its external opening usually presents in the lower third of the neck as a small skin pit. The sinus often secretes a mucous material, which may become infected and purulent from time to time.

Whereas others are lined by respiratory epithelium, branchial cleft cysts and fistula are generally lined with stratified squamous epithelium. They often contain keratin, hair follicles, sweat glands and sebaceous glands, and they may also contain hyaline cartilage. An organized lymphocyte infiltrate with germinal centers is often present in the wall. This lymphoid tissue enlarges during inflammation. The lumen of the cyst is usually filled with a thick yellow fluid characteristically containing a large amount of cholesterol crystals.

One can predict the anatomic location of each branchial anomaly by assuming that it would lie approximately between the structure formed from the corresponding branchial arches found immediately superiorly and inferiorly.

First branchial anomalies result from incomplete obliteration of the cleft between the mandibular process of the first arch and the second arch. The cyst and its tract may lie adjacent or within the parotid gland in close association with the facial nerve. Work (1972) classified first branchial anomalies into two groups. Type I is considered as a duplication of the membranous external auditory canal. A cystic mass in the postauricular area extends medially and anteriorly along the external auditory canal. It usually passes lateral to the facial nerve and ends at the bony meatus. The cyst is lined with stratified squamous epithelium, indicating ectodermal origin. Type II anomalies are considered to be a duplication the membranous external auditory canal and pinna. A sinus passes from an external opening high in the neck along the anterior border of the sternocleidomastoid muscle, superficial or deep to the facial nerve and closely related to the parotid gland. It usually ends blindly near the floor of the membranous external auditory canal, or opens into the canal in the same area as a complete fistula. It is lined with skin and cartilage, indicating ectodermal and mesodermal origin.

The lateral opening of the first branchial fistula will be anterior to the external auditory canal, posterior to the lobule or at the angle of the mandible. A cyst can be seen as a parotid or preauricular mass. If a patient presents with a history of recurrent otorrhea in the absence of a demonstrable middle ear disease, a diagnosis of first branchial anomaly should be suspected.

Second branchial anomalies are by far the most common. They present as a swelling or sinus in the mid to lower neck, anterior to the sternocleidomastoid and usually in the anterior triangle. It courses deep to the platysma muscle between the second and third arch structures by ascending along the carotid sheath and passing medially between the internal and external carotid arteries, above the glossopharyngeal nerve and below the stylohyoid ligament. A cyst may exist anywhere along the this pathway but most commonly presents lateral to the internal jugular vein at the level of the carotid bification. Its tract may exit near the tonsillar fossa.

Third branchial anomalies are very rare. The external opening and initial course are similar to those described for the second. It passes posterior to the glossopharyngeal nerve and the internal carotid artery. It then crosses over the hypoglossal and superior laryngeal nerve back to the region of the thyrohyoid membrane and pyriform sinus. The cysts are most frequently located just proximal to its external opening.

Fourth branchial cleft anomalies have never been demonstrated in their entirety. Like a second or third branchial anomaly, its external opening may be located along the anterior border of the sternocleidomastoid muscle. It crosses over the hypoglossal nerve and then descend in the neck posterior to the common carotid arteries. On the left, the tract would hook around the aortic arch and then reascend the neck lateral to the trachea and esophagus to enter the pharynx. The entry point must be inferior to the superior laryngeal nerve and caudal to the cricothyroid muscle. On the right, the tract would hook around the subclavian artery.

Third and fourth branchial anomalies have been recognized as a potential cause of acute thyroiditis in children. In 1979, Takai reported five cases of acute suppurative thyroiditis in the presence of the pyriform sinus fistula. The infection always involved the left side, and the fistula tract from the pyriform sinus was demonstrated by either barium swallow or endoscopy.

The treatment of branchial anomalies begins with a careful and complete history and physical examination searching for associated systemic anomalies such as craniofacial or branchio-oto-renal syndrome. CT scanning is very useful to determine the location, extent, and size of branchial anomalies.

The definite treatment for branchial anomalies is a complete surgical excision. Because of the high incidence of secondary infection of these anomalies, an early excision is recommended. However,if branchial anomalies are noted in the neonatal period, surgery can be postponed until three to six months of age. This allows the child to grow but hopefully precedes the first upper respiratory infection which may lead to infection of the anomaly. Patients who have undergone acute inflammatory episodes should await subsidence following antibiotic therapy, with incision and drainage of any abscess if indicated.

General anesthesia is usually recommended because of the complexity of the dissection. A maximal effort should be made to minimize the scar because it is a benign lesion, but adequate surgical exposure should never be compromised.

The stepladder incision operation has proved to be the most satisfactory procedure for the removal of such a benign lesion as a branchial anomaly. Traditionally, a fistula is first filled with methylene blue or liquid paraffin to facilitate its dissection. Recently some authors recommend a technique of using a Fogarty embolectomy catheter.

An elliptical incision is recommended around the external opening of the sinus or fistula tract. An adequate amount of supporting connective tissue around the tract is excised in a cephalad direction until the second parallel stepladder incision is made. Such techniques avoid large, disfiguring vertical scars. If one is suspected of having a recurrent neck abscess from an infected branchial pouch sinus, tonsillectomy for internal tract may be required. An operation for the first branchial anomaly may include a superficial parotidectomy and facial nerve dissection.

In children with a recurrent suppurative thyroiditis, a documentation of the pyriform sinus or fistula by barium swallow or endoscopy is important. Cannulation of the sinus opening with a catheter may facilitate the external dissection. A standard thyroidectomy collar incision is recommended.The identification and preservation of the recurrent laryngeal nerve are crucial. The sinus fistula and tract should identified and ligated at the pyriform sinus. A partial or total ipsilateral thyroid lobectomy is often necessary.

Complications of operations for branchial anomalies include secondary infection and damage to nearby anatomic structure including facial, recurrent laryngeal, hypoglossal, spinal accessory, and glossopharyngeal nerves.

Recurrence rates vary from 3% in cases without previous infections or prior surgery to about 20% in cases of prior excisonal attempts. Recurrences are about twice as likely in surgery of branchial sinuses as in surgery of branchial cysts.

A three-year-old black female child presented to the Otolaryngology Clinic with a history of intermittent postauricular swelling associated with otorrhea.

Physical examination revealed an erythematous mass behind her right auricle at the level of the ear lobe. A red bulging lesion in the floor of the external auditory canal was noted. The tympanic membrane was intact with normal landmarks. An infected first branchial cleft cyst was suspected and the patient was treated with oral antibiotics.

Once the acute infection resolved, the patient was taken to the operating room for excision of a right first brachial cleft cyst with identification and preservation of the right facial nerve. Intraoperative findings revealed a duplication of the membranous portion of the external auditory canal lying in close approximation to the facial nerve. Microscopic examination of the specimen revealed stratified squamous epithelium with hyalin cartilage consistent with a branchial cleft cyst.

Her postoperative care was uneventful. Further follow-up showed no evidence of recurrence.

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