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Various presentations of fourth branchial pouch anomalies.


Embryologic anomalies of the fourth branchial pouch are rarely seen. They usually present as recurring episodes of deep neck infections and/or abscesses or acute suppurative thyroiditis. Failure to recognize these unusual cases may result in misdiagnosis, inadequate treatment, and subsequent recurrence. We report 3 cases of patients with fourth branchial anomalies. Diagnosis starts with a preoperative evaluation consisting of a barium swallow or sonogram followed by direct hypopharyngoscopy at the time of surgery. Treatment of acutely infected sinuses is best done with appropriate antibiotics and, if necessary, with incision and drainage. Surgical excision should be planned after the inflammation has completely resolved.


The branchial pouches of the primitive pharynx are the endodermal companions to the four ectodermal clefts first noted in the human embryo by Von Baer in 1827. (1) After penetrating the mesoderm, the pharyngobranchial ducts of the third and fourth pouches typically lose their pharyngeal connections. (1) Incomplete obliteration of these ducts, however, results to a vestigial sinus of the piriform fossa. The origin and course of the sinus is determined by the mesodermal derivatives of adjacent arches.

It has been estimated that 95% of branchial apparatus congenital anomalies are derived from the second arch, pouch, and cleft. (2) The majority of the remaining 5% arise from the first and third arches. (3) Fourth branchial cleft remnants are rare; and until the early 1990s, their very existence was questioned. In 1973, Tucker and Skolnick first described a patient with a recurrent neck abscess, who was subsequently found to have a sinus tract from the apex of the piriform sinus. (2) Since then, others have reported similar processes that are believed to be anomalies originating from the fourth branchial pouch. (3)

We present 3 cases of fourth branchial pouch anomalies, describing their diagnosis, clinical course, and management, and review the literature on these anomalies.

Case Studies

Case 1. A 45-year-old white man with recurrent deep neck infections had been treated with multiple courses of antibiotics and had several recurrences at another facility. After an unsuccessful local incision and drainage, the patient was referred to our facility, where an operative procedure was performed. The patient underwent an external neck exploration. He was noted to have a lot of fibrotic tissue and a tract that appeared consistent with a fourth branchial anomaly. The tract and fibrotic tissue were excised, and it was noted that the tract did not appear to penetrate the thyrohyoid membrane. The pathology was consistent with the remnants of a branchial anomaly. The patient had an unremarkable postoperative course and has had no further recurrences during the past 10 years.

Case 2. A 24-year-old white woman with recurrent deep neck infections had been treated with repeated incision and drainage at another facility. The patient subsequently developed an iatrogenic, externally draining fistula (figure 1, A). A barium swallow was performed, revealing a tract consistent with a fourth branchial cleft anomaly (figure 1, B). She was then placed on oral antibiotics and referred to our facility.


Surgical exploration revealed a fistulous tract that opened into the apex of the piriform sinus. The cyst tract was excised, and the piriform aperture was closed using electrocautery. The patient has had no further complications.

Case 3. A 14-year-old white boy complained of a sore throat after swallowing a large pill. A barium swallow was obtained (figure 2), and the patient was sent to our facility with a diagnosis of esophageal perforation.


The patient was not in distress and was hemodynamically stable. On physical examination, he was noted to have no neck crepitus and minimal fluctuance on palpation of the neck. After review, the results of the swallow were more consistent with a right-sided fourth branchial anomaly than esophageal perforation. The patient was observed conservatively, remained stable, and was discharged on a short course of oral antibiotics. He has had no further sequelae.


The branchial apparatus consists of five pairs of mesodermal arches separated by four pairs of endodermal and ectodermal invaginations, called pouches and clefts, respectively, as demonstrated in figure 3. (3)

A fistula of branchial origin is composed of remnants of both pouch and cleft, with rupture of the interposed branchial plate. (3) A sinus is a tract that is open to either gut or skin, but not both; a cyst is open to neither. (3)

Figure 4 represents the path that a fistula of the fourth branchial pouch and cleft would be expected to take. Most authors accept the 1981 analysis of Liston, who described the route of a fourth branchial fistula. (4) The tract would be expected to begin at the apex of the piriform sinus and travel inferiorly to exit the pharynx caudal to the superior laryngeal nerve, cricothyroid muscle, and thyroid cartilage. After exiting caudal to the cricothyroid muscle, the tract would continue inferiorly, lateral to the trachea and recurrent laryngeal nerve. On the left side, the fistula would continue inferiorly, loop forward under the arch of the aorta, then course superiorly just posterior to the common carotid artery. The fistula would then descend, after looping over the hypoglossal nerve, to open in the skin of the neck at the anterior lower portion of the sternocleido-mastoid muscle. Aright-sided fistula, which is rare, would follow a similar course, but pass underneath the right subclavian artery before ascending in the neck.

A true fistula of the fourth arch, although theoretically possible, would follow an extremely tortuous course and has never been reported. A pseudofistula may develop after iatrogenic or spontaneous rupture of a cervical abscess caused by a branchial pouch sinus. (1) As noted in the table, fourth branchial pouch anomalies virtually always occur on the left side, which is thought to be due to diminished growth of the right ultimobranchial body. (5) In most cases, the sinus tract ends in the perithyroid space or in the thyroid gland. (6)

Fourth branchial pouch anomalies most frequently manifest as recurring episodes of deep neck infections and/or abscesses or acute suppurative thyroiditis. (3) Historically, patients are symptomatic by 10 years of age. Because of the paucity of cases, the diagnosis is often missed, resulting in inappropriate or prolonged treatment. Although up to 65 documented cases of fourth branchial anomalies have been documented (table), several of these cases do not satisfy the current criteria for the diagnosis.

Diagnosis starts with a preoperative evaluation consisting of a barium swallow or sonogram followed by direct hypopharyngoscopy at the time of surgery. Failure to demonstrate a sinus argues strongly against the diagnosis of a fourth branchial pouch anomaly. Treatment of acutely infected sinuses is best done with appropriate antibiotics and with incision and drainage, if necessary. (1) Surgical excision should be planned after the inflammation has completely resolved.

Most surgical texts recommend that the surgical approach begin with the exposure of the thyroid ala and carotid sheath, which allows the operation to begin in a region relatively free of postinflammatory fibrosis. (1) Ideal treatment is complete excision of the tract of the sinus, including the piriform attachment. Any pharyngeal defect is repaired with a purse-string closure. (1) Most texts do not recommend following the tract into the mediastinum, as this is considered to be too aggressive. (6)


Fourth branchial pouch anomalies are more common than previously believed and can present in many different ways, ranging from deep neck infections to being asymptomatic. Identification of a piriform sinus aperture, either by barium swallows or direct examination, is key in the diagnosis of these lesions. Definitive management is best performed by retrograde surgical excision of the sinus tract.
Table. Literature review of reports documenting fourth branchial
 No. Left Right
Authors patients anomaly anomaly

Tucker & Skolnick (2) 1 1
Takai et al (7) 7 7
Abe et al (8) 3 3
Taylor et al (9) 1 1
DeLozier & Sofferman (10) 1 1
Miller et al (11) 3 3
Shugar & Healy (12) 1 1
Ostfeld et al (13) 1 1
Narcy et al (14) 8 8
Doi et al (15) 1 1
Godin et al (3) 1 1
Sandborn & Shafer (16) 1 1
Roediger et al (17) 1 1
Andrieu-Guitrancourt et al (18) 7 7
Miyauchi et al (19) 8 8
Burge & Middleton (5) 1 1
Tovi et al (20) 1 1
Tanyel et al (21) 1 1
Rosenfeld & Biller (1) 2 1 1
Nicollas et al (22) 6 6
Sharma et al (23) 1 1
Jordan et al (24) 7 7
Link et al (25) 1 1

 Total 65 62 3


(1.) Rosenfeld RM, Biller HF. Fourth branchial pouch sinus: Diagnosis and treatment. Otolaryngol Head Neck Surg 1991:105:44-50.

(2.) Tucker HM, Skolnick ML. Fourth branchial cleft (pharyngeal pouch) remnant. Trans Am Acad Ophthalmol Otolaryngol 1973;77:368-71.

(3.) Godin MS, Kearns DB, Pransky SM, et al. Fourth branchial pouch sinus: Principles of diagnosis and management. Laryngoscope 1990; 100:174-8.

(4.) Liston SL. Fourth branchial fistula. Otolaryngol Head Neck Surg 1981;89:520-2.

(5.) Burge D, Middleton A. Persistent pharyngeal pouch derivatives in the neonate. J Pediatr Surg 1983:18:230-4.

(6.) Takimoto T, Yoshizaki T, Ohoka H, Sakashita H. Fourth branchial pouch anomaly. J Laryngol Otol 1990; 104:905-7.

(7.) Takai SI, Miyauchi A, Matsuzuka F, et al. Internal fistula as a route of infection in acute suppurative thyroiditis. Lancet 1979; 1: 751-2.

(8.) Abe K, Fujita H, Matsuura N, et al. A fistula from pyriform sinus in recurrent acute suppurative thyroiditis. Am J Dis Child 1981;135: 178.

(9.) Taylor WE, Jr., Myer CM III, Hays LL, Cotton RT. Acute suppurative thyroiditis in children. Laryngoscope 1982;92:1269-73.

(10.) DeLozier HL, Sofferman RA. Pyriform sinus fistula: An unusual cause of recurrent retropharyngeal abscess and cellulitis. Ann Otol Rhinol Laryngol 1986:95:377 82.

(11.) Miller D, Hill JL, Sun CC, et al. The diagnosis and management of pyriform sinus fistulae in infants and young children. J Pediatr Surg 1983:18:377-81.

(12.) Shugar MA, Healy GB. The fourth branchial cleft anomaly. Head Neck Surg 1980;3:72-5.

(13.) Ostfeld E, Segal J, Auslander L, Rabinson S. Fourth pharyngeal pouch sinus. Laryngoscope 1985:95:1114-7.

(14.) Narcy P, Aumont-Grosskopf C, Bobin S, Manac'h Y. Fistulae of the fourth endobranchial pouch. Int J Pediatr Otorhinolaryngol 1988:16:157-65.

(15.) Doi O, Hutson JM, Myers NA, McKelvie PA. Branchial remnants: A review of 58 cases. J Pediatr Surg 1988:23:789-92.

(16.) Sandborn WD, Shafer AD. A branchial cleft cyst of fourth pouch origin. J Pediatr Surg 1972:7:82.

(17.) Roediger WE, Kalk F, Spitz L, Schmaman A. Congenital thyroid cyst of ultimobranchial gland origin. J Pediatr Surg 1977:12:575-6.

(18.) Andrieu-Guitrancourt J, Amstutz I, Buffet X, et al. [Recurrent laterocervical suppurations: Role of fistulae and cysts of the 4th branchial pouch.] Ann OtoLaryngol Chir Cervicofac 1988:105: 189-92.

(19.) Miyauchi A, Matsuzuka F, Takai S, et al. Piriform sinus fistula. A route of infection in acute suppurative thyroiditis. Arch Surg 1981: 116:66-9.

(20.) Tovi F, Gatot A, Bar-Ziv J, Yanay I. Recent suppurative thyroiditis due to fourth branchial pouch sinus. Int J Pediatr Otorhinolaryngol 1985:9:89-96.

(21.) Tanyel FC, Calmak O, Caglar M, Balkanci F. Fourth pharyngeal pouch remnant presented as a neck mass. Z Kinderchir 1986;41: 360-1.

(22.) Nicollas R, Ducroz V, Garabedian EN, Triglia JM. Fourth branchial pouch anomalies: A study of six cases and review of the literature. Int J Pediatr Otorhinolaryngol 1998:44:5-10.

(23.) Sharma HS, Razif A, Hamzah M, et al. Fourth branchial pouch cyst: An unusual cause of neonatal stridor. Int J Pediatr Otorhinolaryngol 1996;38:155-61.

(24.) Jordan JA, Graves JE, Manning SC, et al. Endoscopic cauterization for treatment of fourth branchial cleft sinuses. Arch Otolaryngol Head Neck Surg 1998;124: 1021-4.

(25.) Link TD, Bite U, Kasperbauer JL, Harner SG. Fourth branchial pouch sinus: A diagnostic challenge. Plast Reconstr Surg 2001;108: 695-701.

From the Department of Otolaryngology, University of Rochester, Strong Memorial Hospital, Rochester, N.Y.

Reprint requests: Anita Jeyakumar, MD, Department of Otolaryngology, University of Rochester, Strong Memorial Hospital, Rochester, NY 14624. Phone: 585-275-2222; fax: 585 271-8552; e-mail:
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Author:Hengerer, Arthur S.
Publication:Ear, Nose and Throat Journal
Geographic Code:1USA
Date:Sep 1, 2004
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