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Fourth branchial pouch sinus: A case report
Fourth Branchial Pouch Sinus: A Case Report Produl Hazarika, MS, DLO, FACS, Dipak Ranjan Nayak, MS, FICS, Ramaswamy Balakrishnan, MS, DNB, Anjana Vishvanathan, MS, and Kailesh Pujary, MS The fourth branchial pouch sinus is a very rare developmental anomaly; it usually presents as a recurring abscess in the left side of the neck. A high index of suspicion, combined with Barium swallow and computed tomography scan, aids in diagnosis. A case of branchial pouch anomaly is reported here for its rarity and late presentation. Complete excision of the entire epithelial tract combined with ipsilateral thyroid lobectomy remains the mainstay of management. (Am J Otolaryngol 2001;22:435-438. Copyright © 2001 by W.B. Saunders Company) (Editorial Comments: The authors report on a rare congenital anomaly. The difficulty of diagnosis and management in these patients are well illustrated.)
The most frequent developmental anomalies of the branchial apparatus are second cleft remnants, which constitute almost 95% of all anomalies. The remaining 5% are comprised almost exclusively of first and third arch remnants. The fourth arch remnants are exceedingly rare and often prove to be a diagnostic dilemma to the otolaryngologist. Reported here is one such case for its rarity and late presentation. CASE REPORT A 31-year-old man presented to the Department of Otolaryngology, Kasturba Hospital, Manipal, India, with a history of throat pain with difficulty swallowing of 5 days duration associated with fever with rigor. He also had a gradually enlarging swelling in the left side of the neck at the lower part of the sternocleidomastoid muscle, which was fluctuant and tender. Indirect laryngoscopic findings were normal. Blood counts showed an elevated leu-
From the Department of ENT—Head & Neck Surgery, Kasturba Medical College, Manipal, India. Address reprint requests to Produl Hazarika, MS, DLO, FACS, Department of ENT—Head & Neck Surgery, Kasturba Medical College, Manipal 576119, Karnataka, India. Copyright © 2001 by W.B. Saunders Company 0196-0709/01/2206-0013$35.00/0 doi:10.1053/ajot.2001.28065
kocyte count, and Barium swallow showed a 1-cm long tract from the left pyriform fossa apex. Computed tomography (CT) scan showed an abscess extending from the posterior wall of the left pyriform fossa down to the thyroid gland, pushing it forward (Fig 1). A provisional diagnosis of suppurative lymphadenopathy or infected branchial sinus was made. The patient was treated conservatively with antibiotics and analgesics, after which the dysphagia improved. In view of the barium swallow findings, he was taken up for hypopharyngoscopy under general anesthesia, which showed a small opening in the left pyriform fossa apex (Fig 2). Methylene blue dye was injected through the opening, and cannulation was attempted, but failed. Hence, no further treatment was performed. The patient was discharged with a provisional diagnosis of fourth internal branchial sinus. The patient came back to us the following year with complaints of foreign body sensation and throat pain accompanied by recurrent left neck swelling. Barium swallow at this visit showed a sinus tract from the left pyriform fossa apex. After a course of antibiotics, the patient was taken up for excision of the sinus by left lateral pharyngotomy approach under general anesthesia. The mouth of the sinus was visualized endoscopically, and cannulation was attempted, which failed to identify the definitive tract. Exploration of the tract from the neck was also attempted. An oblique skin crease incision was given in the region of the mid third of the sternocleidomastoid muscle. Dissection was performed to expose the inferior constrictor muscle. Because the
American Journal of Otolaryngology, Vol 22, No 6 (November-December), 2001: pp 435-438
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Fig 1. CT scan showing the abscess posterior to the thyroid gland, pushing it anteriorly.
search for the tract proved futile, the neck wound was closed after giving random sutures in the area of the inferior constrictor to encourage fibrosis to help close the tract. The patient was discharged with antibiotics, only to appear again after a few days with a recurrent swelling in the region of the lower third of the sternocleidomastoid (Fig 3). The swelling was diagnosed to be a left paratracheal abscess secondary to the fourth branchial sinus. After conservative treatment, barium swallow was performed. At this visit, it showed the presence of a definitive sinus tract from the left pyriform fossa to the abscess cavity in the neck (Fig 4). Under antibiotic cover, revision left pharyngotomy approach was performed to explore the abscess cavity. Excision of the upper pole of the thyroid lobe
Fig 3. Recurrent swelling in the region of the lower third of the sternocleidomastoid muscle with scar from previous incision.
enabled us to visualize the sinus tract clearly. Retrograde cannulation was performed with a catheter from the abscess cavity site to the left pyriform fossa. The tract was excised externally, and the mouth of the sinus in the left pyriform fossa was closed. The wound was closed in layers with suction drains. The postoperative period was uneventful. The patient was discharged and has had no recurrence as of the last follow-up. DISCUSSION
Fig 2. Left pyriform fossa, with the opening of the tract near the apex.
Branchial apparatus anomalies include branchial cysts, fistulae, and sinuses. The branchial sinuses can open externally or internally. Second arch anomalies are the most common, followed by first and third. The fourth arch anomaly is very rare, and few reports are available in the literature. Tucker and Skolnick reported the first such case in 1973.1 No single author has presented a case series to decide on an ideal treatment modality. The remnant of the fourth branchial cleft
FOURTH BRANCHIAL POUCH SINUS
Fig 4. Barium swallow (anteroposterior view) showing the sinus tract leading from the left pyriform fossa to the abscess cavity (ST, sinus tract; AB, abscess cavity).
usually tracks from the pyriform sinus along the tracheo-esophageal groove posterior to the upper pole of the thyroid lobe. In 1981, Liston proposed that the tract would begin at the apex of the pyriform sinus and proceed inferiorly to exit the pharynx caudal to the superior laryngeal nerve, cricothyroid muscle, and thyroid cartilage, all of which are fourth arch structures.2 After exiting just caudal to the cricothyroid muscle, the tract would continue to course inferiorly lateral to the trachea and the recurrent laryngeal nerve, which is of sixth arch origin. On the left side, the fistula would continue inferiorly until it reached the posterior aspect of the aortic arch, which is also of fourth branchial arch origin. It would then loop forward under the aorta and course superiorly in the neck, just posterior to the common carotid artery. The fistula would loop over the hypoglossal nerve before redescending to open in the skin of the neck at the anterior aspect of the lower portion of the sternocleidomastoid muscle as a remnant of
437
the primordial sinus of His. If the fistula were on the right side, which would be rare, it would pass forward underneath the right subclavian artery before ascending in the neck. Fourth branchial sinuses almost always occur on the left side, as in our case. Most investigators believe that a complete fourth arch fistula may never be surgically shown.2-4 Almost all reported cases of fourth branchial sinus were symptomatic in childhood. As the sinus tract communicates with the pyriform fossa, it is exposed to secretions and food particles; hence, they present as recurrent left neck abscesses within the first 10 years of life. This is unlike our case, which had a late presentation. A fourth branchial sinus may also present as recurrent acute suppurative thyroiditis, because of its close relation to the left lobe of thyroid. De Lozier et al have reported this anomaly presenting as recurrent retropharyngeal abscess and cellulitis in an infant.5 The differential diagnosis of these lesions, according to Godin et al, includes secondary lymphadenitis, cystic hygroma, atypical thyroglossal duct remnants, thymic cyst, ectopic thyroid, tuberculous adenitis, lymphoma, hemangioma, branchiogenic carcinoma, metastatic malignant neoplasms, carotid body tumor, and subcutaneous bronchogenic cyst, to name some of the possibilities.6 A high index of suspicion of this anomaly is necessary, especially when patients present with recurrent left neck abscesses. Barium swallow is an effective means of showing the presence of the anomalous tract.1,5,8,9 CT scan of the neck in our case showed an abscess cavity pushing the left thyroid anterolaterally. CT scan as an adjuvant to Barium swallow is a useful investigative tool in aiding the diagnosis by showing the posterior relation of the tract to the left thyroid lobe. Endoscopy will show the pharyngeal opening of the sinus at the apex of the pyriform fossa. Cannulation of the tract is often difficult because of its tortuous course, but, when possible, palpation of the catheter in the tract during surgery will facilitate complete excision. Injection of methylene blue through the internal opening has been attempted by a few investigators, without much success.5 Surgical excision remains the mainstay of treatment, in addition to antibiotics to control the infection. In our experience, identification of the tract was
438
more difficult in the quiescent stage, compared with the abscess stage. The predominant opinion of investigators is that a complete fourth arch fistula may never be surgically shown. Nevertheless, an attempt should be made to completely expose and excise the tract; its opening in the pyriform fossa should be ligated and transected with purse string sutures. Some cases may require a partial lobectomy, as was performed in our case, if the tract courses into the thyroid gland. Because of its proximity to the superior and recurrent laryngeal nerves, the safest course of action would be to expose the structures at risk before venturing into complete excision.9 CONCLUSION Fourth branchial remnants are very rare and are found along the left thyrotracheal axis. They usually present as recurrent abscesses in the left neck. A high index of clinical suspicion, aided by barium swallow, CT, and en-
HAZARIKA ET AL
doscopy, assists with diagnosis. Complete excision remains the mainstay of treatment. REFERENCES 1. Tucker HM, Skolnick ML: Fourth branchial cleft (Pharyngeal pouch) remnant. Trans Pa Am Acad Ophthalmol Otolaryngol 77:368-371, 1973 2. Liston SL: Fourth branchial fistula. Otolaryngol Head Neck Surg 89:520-522, 1981 3. Shugar MA, Healey GB: The fourth branchial cleft anomaly. Head Neck Surg 3:72-75, 1980 4. Ostfeld E, Segal J, Auslander L, et al: Fourth pharyngeal pouch sinus. Laryngoscope 95:1114-1117, 1985 5. DeLozier HL, Sofferman RA: Pyriform sinus fistula: An unusual cause of recurrent retropharyngeal abscess and cellulitis. Ann Otol Rhinol Laryngol 95:377-382, 1986 6. Godin MS, Kearns DB, Pransky SM: Fourth branchial pouch sinus: Principles of diagnosis and management. Laryngoscope 100:174-178, 1990 7. Abe K, Fujita H, Matsuura N, et al: A fistula from pyriform sinus in recurrent acute suppurative thyroiditis. Am J Dis Child 135:178, 1981 8. Miller D, Hill JL, Chen-Chih S, et al: The diagnosis and management of pyriform sinus fistulae in infants and young children. J Pediatr Surg 18:377-381, 1983 9. Narcy P, Aumont-Grosskopf C, Bobin S, et al: Fistulae of the fourth endobranchial fistula. Int J Pediatr Otorhinolaryngol 16:157-165, 1988
The most frequent developmental anomalies of the branchial apparatus are second cleft remnants, which constitute almost 95% of all anomalies. The remaining 5% are comprised almost exclusively of first and third arch remnants. The fourth arch remnants are exceedingly rare and often prove to be a diagnostic dilemma to the otolaryngologist. Reported here is one such case for its rarity and late presentation. CASE REPORT A 31-year-old man presented to the Department of Otolaryngology, Kasturba Hospital, Manipal, India, with a history of throat pain with difficulty swallowing of 5 days duration associated with fever with rigor. He also had a gradually enlarging swelling in the left side of the neck at the lower part of the sternocleidomastoid muscle, which was fluctuant and tender. Indirect laryngoscopic findings were normal. Blood counts showed an elevated leu-
From the Department of ENT—Head & Neck Surgery, Kasturba Medical College, Manipal, India. Address reprint requests to Produl Hazarika, MS, DLO, FACS, Department of ENT—Head & Neck Surgery, Kasturba Medical College, Manipal 576119, Karnataka, India. Copyright © 2001 by W.B. Saunders Company 0196-0709/01/2206-0013$35.00/0 doi:10.1053/ajot.2001.28065
kocyte count, and Barium swallow showed a 1-cm long tract from the left pyriform fossa apex. Computed tomography (CT) scan showed an abscess extending from the posterior wall of the left pyriform fossa down to the thyroid gland, pushing it forward (Fig 1). A provisional diagnosis of suppurative lymphadenopathy or infected branchial sinus was made. The patient was treated conservatively with antibiotics and analgesics, after which the dysphagia improved. In view of the barium swallow findings, he was taken up for hypopharyngoscopy under general anesthesia, which showed a small opening in the left pyriform fossa apex (Fig 2). Methylene blue dye was injected through the opening, and cannulation was attempted, but failed. Hence, no further treatment was performed. The patient was discharged with a provisional diagnosis of fourth internal branchial sinus. The patient came back to us the following year with complaints of foreign body sensation and throat pain accompanied by recurrent left neck swelling. Barium swallow at this visit showed a sinus tract from the left pyriform fossa apex. After a course of antibiotics, the patient was taken up for excision of the sinus by left lateral pharyngotomy approach under general anesthesia. The mouth of the sinus was visualized endoscopically, and cannulation was attempted, which failed to identify the definitive tract. Exploration of the tract from the neck was also attempted. An oblique skin crease incision was given in the region of the mid third of the sternocleidomastoid muscle. Dissection was performed to expose the inferior constrictor muscle. Because the
American Journal of Otolaryngology, Vol 22, No 6 (November-December), 2001: pp 435-438
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HAZARIKA ET AL
Fig 1. CT scan showing the abscess posterior to the thyroid gland, pushing it anteriorly.
search for the tract proved futile, the neck wound was closed after giving random sutures in the area of the inferior constrictor to encourage fibrosis to help close the tract. The patient was discharged with antibiotics, only to appear again after a few days with a recurrent swelling in the region of the lower third of the sternocleidomastoid (Fig 3). The swelling was diagnosed to be a left paratracheal abscess secondary to the fourth branchial sinus. After conservative treatment, barium swallow was performed. At this visit, it showed the presence of a definitive sinus tract from the left pyriform fossa to the abscess cavity in the neck (Fig 4). Under antibiotic cover, revision left pharyngotomy approach was performed to explore the abscess cavity. Excision of the upper pole of the thyroid lobe
Fig 3. Recurrent swelling in the region of the lower third of the sternocleidomastoid muscle with scar from previous incision.
enabled us to visualize the sinus tract clearly. Retrograde cannulation was performed with a catheter from the abscess cavity site to the left pyriform fossa. The tract was excised externally, and the mouth of the sinus in the left pyriform fossa was closed. The wound was closed in layers with suction drains. The postoperative period was uneventful. The patient was discharged and has had no recurrence as of the last follow-up. DISCUSSION
Fig 2. Left pyriform fossa, with the opening of the tract near the apex.
Branchial apparatus anomalies include branchial cysts, fistulae, and sinuses. The branchial sinuses can open externally or internally. Second arch anomalies are the most common, followed by first and third. The fourth arch anomaly is very rare, and few reports are available in the literature. Tucker and Skolnick reported the first such case in 1973.1 No single author has presented a case series to decide on an ideal treatment modality. The remnant of the fourth branchial cleft
FOURTH BRANCHIAL POUCH SINUS
Fig 4. Barium swallow (anteroposterior view) showing the sinus tract leading from the left pyriform fossa to the abscess cavity (ST, sinus tract; AB, abscess cavity).
usually tracks from the pyriform sinus along the tracheo-esophageal groove posterior to the upper pole of the thyroid lobe. In 1981, Liston proposed that the tract would begin at the apex of the pyriform sinus and proceed inferiorly to exit the pharynx caudal to the superior laryngeal nerve, cricothyroid muscle, and thyroid cartilage, all of which are fourth arch structures.2 After exiting just caudal to the cricothyroid muscle, the tract would continue to course inferiorly lateral to the trachea and the recurrent laryngeal nerve, which is of sixth arch origin. On the left side, the fistula would continue inferiorly until it reached the posterior aspect of the aortic arch, which is also of fourth branchial arch origin. It would then loop forward under the aorta and course superiorly in the neck, just posterior to the common carotid artery. The fistula would loop over the hypoglossal nerve before redescending to open in the skin of the neck at the anterior aspect of the lower portion of the sternocleidomastoid muscle as a remnant of
437
the primordial sinus of His. If the fistula were on the right side, which would be rare, it would pass forward underneath the right subclavian artery before ascending in the neck. Fourth branchial sinuses almost always occur on the left side, as in our case. Most investigators believe that a complete fourth arch fistula may never be surgically shown.2-4 Almost all reported cases of fourth branchial sinus were symptomatic in childhood. As the sinus tract communicates with the pyriform fossa, it is exposed to secretions and food particles; hence, they present as recurrent left neck abscesses within the first 10 years of life. This is unlike our case, which had a late presentation. A fourth branchial sinus may also present as recurrent acute suppurative thyroiditis, because of its close relation to the left lobe of thyroid. De Lozier et al have reported this anomaly presenting as recurrent retropharyngeal abscess and cellulitis in an infant.5 The differential diagnosis of these lesions, according to Godin et al, includes secondary lymphadenitis, cystic hygroma, atypical thyroglossal duct remnants, thymic cyst, ectopic thyroid, tuberculous adenitis, lymphoma, hemangioma, branchiogenic carcinoma, metastatic malignant neoplasms, carotid body tumor, and subcutaneous bronchogenic cyst, to name some of the possibilities.6 A high index of suspicion of this anomaly is necessary, especially when patients present with recurrent left neck abscesses. Barium swallow is an effective means of showing the presence of the anomalous tract.1,5,8,9 CT scan of the neck in our case showed an abscess cavity pushing the left thyroid anterolaterally. CT scan as an adjuvant to Barium swallow is a useful investigative tool in aiding the diagnosis by showing the posterior relation of the tract to the left thyroid lobe. Endoscopy will show the pharyngeal opening of the sinus at the apex of the pyriform fossa. Cannulation of the tract is often difficult because of its tortuous course, but, when possible, palpation of the catheter in the tract during surgery will facilitate complete excision. Injection of methylene blue through the internal opening has been attempted by a few investigators, without much success.5 Surgical excision remains the mainstay of treatment, in addition to antibiotics to control the infection. In our experience, identification of the tract was
438
more difficult in the quiescent stage, compared with the abscess stage. The predominant opinion of investigators is that a complete fourth arch fistula may never be surgically shown. Nevertheless, an attempt should be made to completely expose and excise the tract; its opening in the pyriform fossa should be ligated and transected with purse string sutures. Some cases may require a partial lobectomy, as was performed in our case, if the tract courses into the thyroid gland. Because of its proximity to the superior and recurrent laryngeal nerves, the safest course of action would be to expose the structures at risk before venturing into complete excision.9 CONCLUSION Fourth branchial remnants are very rare and are found along the left thyrotracheal axis. They usually present as recurrent abscesses in the left neck. A high index of clinical suspicion, aided by barium swallow, CT, and en-
HAZARIKA ET AL
doscopy, assists with diagnosis. Complete excision remains the mainstay of treatment. REFERENCES 1. Tucker HM, Skolnick ML: Fourth branchial cleft (Pharyngeal pouch) remnant. Trans Pa Am Acad Ophthalmol Otolaryngol 77:368-371, 1973 2. Liston SL: Fourth branchial fistula. Otolaryngol Head Neck Surg 89:520-522, 1981 3. Shugar MA, Healey GB: The fourth branchial cleft anomaly. Head Neck Surg 3:72-75, 1980 4. Ostfeld E, Segal J, Auslander L, et al: Fourth pharyngeal pouch sinus. Laryngoscope 95:1114-1117, 1985 5. DeLozier HL, Sofferman RA: Pyriform sinus fistula: An unusual cause of recurrent retropharyngeal abscess and cellulitis. Ann Otol Rhinol Laryngol 95:377-382, 1986 6. Godin MS, Kearns DB, Pransky SM: Fourth branchial pouch sinus: Principles of diagnosis and management. Laryngoscope 100:174-178, 1990 7. Abe K, Fujita H, Matsuura N, et al: A fistula from pyriform sinus in recurrent acute suppurative thyroiditis. Am J Dis Child 135:178, 1981 8. Miller D, Hill JL, Chen-Chih S, et al: The diagnosis and management of pyriform sinus fistulae in infants and young children. J Pediatr Surg 18:377-381, 1983 9. Narcy P, Aumont-Grosskopf C, Bobin S, et al: Fistulae of the fourth endobranchial fistula. Int J Pediatr Otorhinolaryngol 16:157-165, 1988