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First branchial arch fistula: diagnostic dilemma and improvised surgical management
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American Journal of Otolaryngology–Head and Neck Medicine and Surgery 32 (2011) 617 – 619 www.elsevier.com/locate/amjoto
First branchial arch fistula: diagnostic dilemma and improvised surgical management Vinod Prabhu, MBBS, MRCS, DLO, DOHNS⁎, Duncan Ingrams, FRCS (ORL-HNS) Department of ENT, “E” block, Royal Gwent Hospital, Newport, UK Received 26 August 2010
Abstract
First branchial cleft anomalies are uncommon, and only sporadic case reports are published in the literature. They account for 1% to 8% of all the branchial abnormalities. The often variable presentation and tract siting of first arch fistulae have led to misdiagnosis. The misdiagnosis results in inappropriate/ineffective treatment and recurrence of the sinus tract. We present a 19-year-old woman who presented to the ENT outpatient department with episodic discharge from a longstanding fistula anterior to the left sternomastoid muscle. This was associated with repeated episodes of ipsilateral tonsillitis. In relation to the history and because of the position of the fistula, a diagnosis of second branchial arch fistula was made. An attempt at excision was unfortunately followed by early recurrence of discharge. At review following the procedure, a defect of the left tympanic membrane in the form of a fibrous band was noted, and a revised diagnosis of first branchial arch sinus was made. Wide surgical excision of the tract with partial parotidectomy was performed. An uneventful postoperative course followed, with no recurrence of symptoms after 24 months of review. We discuss the case, the diagnostic pathway, and the wide local excision technique used for removal of branchial fistulae. © 2011 Elsevier Inc. All rights reserved.
1. Introduction The branchial system plays a significant role in the embryologic development of the many internal and external human body structures. With development, the planes separating arches obliterate leaving seamless junctions. Failure of complete obliteration of these junctions and the interposition of rest cells within the tissues is considered to be the likely cause [1] for the branchial system anomalies . The chance of malformations occurring in and around the ear, including the parotid gland, is possible due to the developmental nature of the cleft, that is, from ventral to dorsal and the interaction with the developing hillocks of Hiss. First branchial arch fistulae are the rarest of the recognized branchial fistulae occurring in between 1% and 8% of all types [2]. Second arch is the most common,
with a tract classically seen with an external opening at the junction of the lower third of the anterior border of sternomastoid muscle. Complete excision is the only treatment for fistulae [2,3], and any residual tract has a chance of recurrence varying from 3% in primary cases to 20% in revision cases [4]. Because of the recurrence rate, many surgical techniques are described to excise sinuses and fistulas in head and neck site [5-9]. This includes the sinus/fistula recurrence after Sistrunk procedure for thyroglossal tract sinuses and for preauricular sinuses. We describe a wide local excision technique to reduce recurrence in first branchial fistula. This technique has been used successfully in managing recurrent thyroglossal cyst sinuses [7] after a Sistrunk procedure.
2. Case report ⁎ Corresponding author. Department of ENT, “E” block, Royal Gwent Hospital, Newport NP20 2UB, UK. E-mail address: [email protected] (V. Prabhu). 0196-0709/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2010.09.008
A 19-year-old woman patient presented to the ENT outpatient department with discharging pit on the left side of
618
V. Prabhu, D. Ingrams / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 32 (2011) 617–619
her neck. She was extremely concerned about this because she planned to go to college to study performing arts, and this unsightly lesion would reduce her chances of progress in the chosen field. Associated with this was a history of repeated episodes of tonsillitis, which she described as being worse on the left side. Initially, she sought help from her general practitioner who treated the problem with oral antibiotics, which reduced the discharge for a few weeks before recurrence. On examination, the patient had a discharging pit lateral to the midline on the left side at the level of the thyroid cartilage. Full ENT examination was undertaken, and no other abnormality could be detected. In view of a classical history and examination, a diagnosis of second branchial fistula was made. The episodes of left tonsillitis were thought to concur with second arch fistula, as the internal opening would be within the tonsil fossa. The patient underwent tonsillectomy and excision of the left branchial sinus tract under general anesthesia. Although the operation was uneventful, the tract was followed to a blind end approximately 5 mm from the ipsilateral tonsil but was not found to communicate with the tonsillar fossa. The tonsil was also removed, and the pillars were sutured together. The patient was discharged home the following day. In the prevailing weeks, the patient had further episodes of neck swelling (Fig. 1) and discharge. At review, incomplete excision was considered and a further ENT examination revealed a defect in the left tympanic membrane described as a bar extending from the floor of the ear canal to the tympanic membrane (Fig. 2). A methylene blue fistula test was undertaken, which revealed communication with this defect and the neck pit. A second diagnosis of first arch fistula was made; this was confirmed with magnetic resonance imaging fistulography. Wide local excision of the fistulous tract with cuff of healthy tissue was undertaken. The postoperative period was uneventful.
Fig. 1. The arrow shows the cystic lesion in the neck.
Fig. 2. Ear canal and the bulging tympanic membrane. The arrow shows the fibrous first branchial arch fistulous tract in the ear canal.
Follow-up for 24 months postoperatively has revealed no evidence of recurrence, and she is well and continuing with her career. This case has been discussed for a variety of reasons: first, the rarity of the diagnosis, the obvious problems with diagnosis, and treatment and a technique to remove en bloc tissue surrounding the tract to reduce recurrence.
3. Discussion First branchial cyst and sinus within the ear canal or postauricular area are classified into 2 types by Jakubikova et al [10]: 1) Type I defect is medial to the concha, and opening of the sinus tract usually situated inferior or posterior to the pinna or concha. The track normally ends in a blind pouch on a bony plate at the level of mesotympanum. 2) Type II lesions pass through the parotid gland and may lie either medial or lateral to the facial nerve or may even cross several branches of the facial nerve. This sinus track may end either in the cartilaginous external auditory canal or extend to the face or upper neck. Type I defect that usually needs a simple excision without facial dissection can often suffice [10]. Surgical treatment of type II anomalies depends on the nature of the intimate association of the lesion with the facial nerve and parotid gland. These anomalies often require superficial parotidectomy and the facial nerve identification. Surgeons who are unaware of these lesions may be tempted to perform a limited resection that inevitably leads to recurrence [2]. Most common complications after such excision include infection, damage to the facial nerve, and lower cranial nerves. Recurrence rates after excision vary depending on
V. Prabhu, D. Ingrams / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 32 (2011) 617–619
the clinical diagnosis, primary or revision surgery, and experience of the surgeon involved [4]. Wide local excision of the lesion in persistent recurrence of the sinuses has been successfully used in treating failed Sistrunk procedure [7] and in preauricular sinus excision [8]. In our case discussed above, the history led us to diagnose it to be a second branchial fistula/sinus. A radiologic investigation like fistulogram was not carried out initially, as there is very little evidence in the published literature about its usefulness [3], but later aided in our diagnosis of the first branchial fistula. We used a modified Blair incision to approach the problem. The core of fistula was excised, including the surrounding normal tissue. As part of the procedure, a partial parotidectomy was performed and a cuff of auricular cartilage excised. The tract was medial to the facial nerve and extended into the ear canal. The facial nerve was preserved, and the tract was excised en bloc. The postoperative period was uneventful, with no evidence of recurrence after 24-month follow-up. This en bloc excision is a modified technique, and this is the only case mentioned in the literature involving the first branchial fistula. The complications of this technique should not be more than usual if the surgeon is familiar with the anatomy of the head and neck region.
4. Conclusion Branchial cleft anomalies are usually not associated with other facial malformations; thus, recognition of the first branchial cleft anomalies can be difficult and can lead to misdiagnosis and inappropriate treatment. A high index of suspicion and appropriate history, examination, and investigation should confirm a correct diagnosis and management.
619
We present this case for the diagnosis dilemma we had after the clinical history and the improvised surgical technique of wide local excision to cure the problem. Acknowledgment We are extremely grateful to Professor David Howard, Consultant and Senior Lecturer of Otorhinolaryngology, Royal National Throat, Nose and Ear Hospital, London; for his guidance in managing this case. References [1] Gaisford JC, Anderson VS. First Branchial cleft cysts and sinuses. Plastic Reconstruct Surg 1975;55:299-304. [2] D'Souza AR, Uppal HS, De R, et al. Updating concepts of first branchial cleft defects: a literature review. Int J Pediatric Otorhinolaryngol 2002;62:103-9. [3] Rattan KN, Rattan S, Parihar D, et al. Second branchial cleft fistula: is fistulogram necessary for complete excision. Int J Pediatr Otorhinolaryngol 2006;70:1027-30. [4] Chandler RJ, Mitchell B. Branchial cleft cysts, sinuses and fistulas. Otolaryngol Clin N Am 1981;14:175-86. [5] Cox EF. Excision of branchial sinus and fistula tracts using arterial intimal strippers. Surg, Gynecol Obstet 1963;117:767-8. [6] Oshio T, Nakamiza H, Yoshikawa K, et al. A new fistulectomy method for the second pharyngeal arch remnants. J Pediatr Surg 2005;40:1784-7. [7] Patel NN, Hartley BEJ, Howard DJ. Management of thyroglossal tract disease after failed Sistrunk's procedure. J Laryngol Otol 2003;117:710-2. [8] de Jong RJB, Leiden MD. A new surgical technique for treatment of preauricular sinus. Surgery 2005;137:567-70. [9] Lam HC, Soo G, Wormald PJ, et al. Excision of the preauricular sinus: a comparison of two surgical techniques. Laryngoscope 2001;111:317-9. [10] Jakubikova J, Stanik R, Stanikova A. Malformations of the first branchial cleft: duplication of the external auditory canal. Int J Pediatr Otorhinolaryngol 2005;69:255-61.
American Journal of Otolaryngology–Head and Neck Medicine and Surgery 32 (2011) 617 – 619 www.elsevier.com/locate/amjoto
First branchial arch fistula: diagnostic dilemma and improvised surgical management Vinod Prabhu, MBBS, MRCS, DLO, DOHNS⁎, Duncan Ingrams, FRCS (ORL-HNS) Department of ENT, “E” block, Royal Gwent Hospital, Newport, UK Received 26 August 2010
Abstract
First branchial cleft anomalies are uncommon, and only sporadic case reports are published in the literature. They account for 1% to 8% of all the branchial abnormalities. The often variable presentation and tract siting of first arch fistulae have led to misdiagnosis. The misdiagnosis results in inappropriate/ineffective treatment and recurrence of the sinus tract. We present a 19-year-old woman who presented to the ENT outpatient department with episodic discharge from a longstanding fistula anterior to the left sternomastoid muscle. This was associated with repeated episodes of ipsilateral tonsillitis. In relation to the history and because of the position of the fistula, a diagnosis of second branchial arch fistula was made. An attempt at excision was unfortunately followed by early recurrence of discharge. At review following the procedure, a defect of the left tympanic membrane in the form of a fibrous band was noted, and a revised diagnosis of first branchial arch sinus was made. Wide surgical excision of the tract with partial parotidectomy was performed. An uneventful postoperative course followed, with no recurrence of symptoms after 24 months of review. We discuss the case, the diagnostic pathway, and the wide local excision technique used for removal of branchial fistulae. © 2011 Elsevier Inc. All rights reserved.
1. Introduction The branchial system plays a significant role in the embryologic development of the many internal and external human body structures. With development, the planes separating arches obliterate leaving seamless junctions. Failure of complete obliteration of these junctions and the interposition of rest cells within the tissues is considered to be the likely cause [1] for the branchial system anomalies . The chance of malformations occurring in and around the ear, including the parotid gland, is possible due to the developmental nature of the cleft, that is, from ventral to dorsal and the interaction with the developing hillocks of Hiss. First branchial arch fistulae are the rarest of the recognized branchial fistulae occurring in between 1% and 8% of all types [2]. Second arch is the most common,
with a tract classically seen with an external opening at the junction of the lower third of the anterior border of sternomastoid muscle. Complete excision is the only treatment for fistulae [2,3], and any residual tract has a chance of recurrence varying from 3% in primary cases to 20% in revision cases [4]. Because of the recurrence rate, many surgical techniques are described to excise sinuses and fistulas in head and neck site [5-9]. This includes the sinus/fistula recurrence after Sistrunk procedure for thyroglossal tract sinuses and for preauricular sinuses. We describe a wide local excision technique to reduce recurrence in first branchial fistula. This technique has been used successfully in managing recurrent thyroglossal cyst sinuses [7] after a Sistrunk procedure.
2. Case report ⁎ Corresponding author. Department of ENT, “E” block, Royal Gwent Hospital, Newport NP20 2UB, UK. E-mail address: [email protected] (V. Prabhu). 0196-0709/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2010.09.008
A 19-year-old woman patient presented to the ENT outpatient department with discharging pit on the left side of
618
V. Prabhu, D. Ingrams / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 32 (2011) 617–619
her neck. She was extremely concerned about this because she planned to go to college to study performing arts, and this unsightly lesion would reduce her chances of progress in the chosen field. Associated with this was a history of repeated episodes of tonsillitis, which she described as being worse on the left side. Initially, she sought help from her general practitioner who treated the problem with oral antibiotics, which reduced the discharge for a few weeks before recurrence. On examination, the patient had a discharging pit lateral to the midline on the left side at the level of the thyroid cartilage. Full ENT examination was undertaken, and no other abnormality could be detected. In view of a classical history and examination, a diagnosis of second branchial fistula was made. The episodes of left tonsillitis were thought to concur with second arch fistula, as the internal opening would be within the tonsil fossa. The patient underwent tonsillectomy and excision of the left branchial sinus tract under general anesthesia. Although the operation was uneventful, the tract was followed to a blind end approximately 5 mm from the ipsilateral tonsil but was not found to communicate with the tonsillar fossa. The tonsil was also removed, and the pillars were sutured together. The patient was discharged home the following day. In the prevailing weeks, the patient had further episodes of neck swelling (Fig. 1) and discharge. At review, incomplete excision was considered and a further ENT examination revealed a defect in the left tympanic membrane described as a bar extending from the floor of the ear canal to the tympanic membrane (Fig. 2). A methylene blue fistula test was undertaken, which revealed communication with this defect and the neck pit. A second diagnosis of first arch fistula was made; this was confirmed with magnetic resonance imaging fistulography. Wide local excision of the fistulous tract with cuff of healthy tissue was undertaken. The postoperative period was uneventful.
Fig. 1. The arrow shows the cystic lesion in the neck.
Fig. 2. Ear canal and the bulging tympanic membrane. The arrow shows the fibrous first branchial arch fistulous tract in the ear canal.
Follow-up for 24 months postoperatively has revealed no evidence of recurrence, and she is well and continuing with her career. This case has been discussed for a variety of reasons: first, the rarity of the diagnosis, the obvious problems with diagnosis, and treatment and a technique to remove en bloc tissue surrounding the tract to reduce recurrence.
3. Discussion First branchial cyst and sinus within the ear canal or postauricular area are classified into 2 types by Jakubikova et al [10]: 1) Type I defect is medial to the concha, and opening of the sinus tract usually situated inferior or posterior to the pinna or concha. The track normally ends in a blind pouch on a bony plate at the level of mesotympanum. 2) Type II lesions pass through the parotid gland and may lie either medial or lateral to the facial nerve or may even cross several branches of the facial nerve. This sinus track may end either in the cartilaginous external auditory canal or extend to the face or upper neck. Type I defect that usually needs a simple excision without facial dissection can often suffice [10]. Surgical treatment of type II anomalies depends on the nature of the intimate association of the lesion with the facial nerve and parotid gland. These anomalies often require superficial parotidectomy and the facial nerve identification. Surgeons who are unaware of these lesions may be tempted to perform a limited resection that inevitably leads to recurrence [2]. Most common complications after such excision include infection, damage to the facial nerve, and lower cranial nerves. Recurrence rates after excision vary depending on
V. Prabhu, D. Ingrams / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 32 (2011) 617–619
the clinical diagnosis, primary or revision surgery, and experience of the surgeon involved [4]. Wide local excision of the lesion in persistent recurrence of the sinuses has been successfully used in treating failed Sistrunk procedure [7] and in preauricular sinus excision [8]. In our case discussed above, the history led us to diagnose it to be a second branchial fistula/sinus. A radiologic investigation like fistulogram was not carried out initially, as there is very little evidence in the published literature about its usefulness [3], but later aided in our diagnosis of the first branchial fistula. We used a modified Blair incision to approach the problem. The core of fistula was excised, including the surrounding normal tissue. As part of the procedure, a partial parotidectomy was performed and a cuff of auricular cartilage excised. The tract was medial to the facial nerve and extended into the ear canal. The facial nerve was preserved, and the tract was excised en bloc. The postoperative period was uneventful, with no evidence of recurrence after 24-month follow-up. This en bloc excision is a modified technique, and this is the only case mentioned in the literature involving the first branchial fistula. The complications of this technique should not be more than usual if the surgeon is familiar with the anatomy of the head and neck region.
4. Conclusion Branchial cleft anomalies are usually not associated with other facial malformations; thus, recognition of the first branchial cleft anomalies can be difficult and can lead to misdiagnosis and inappropriate treatment. A high index of suspicion and appropriate history, examination, and investigation should confirm a correct diagnosis and management.
619
We present this case for the diagnosis dilemma we had after the clinical history and the improvised surgical technique of wide local excision to cure the problem. Acknowledgment We are extremely grateful to Professor David Howard, Consultant and Senior Lecturer of Otorhinolaryngology, Royal National Throat, Nose and Ear Hospital, London; for his guidance in managing this case. References [1] Gaisford JC, Anderson VS. First Branchial cleft cysts and sinuses. Plastic Reconstruct Surg 1975;55:299-304. [2] D'Souza AR, Uppal HS, De R, et al. Updating concepts of first branchial cleft defects: a literature review. Int J Pediatric Otorhinolaryngol 2002;62:103-9. [3] Rattan KN, Rattan S, Parihar D, et al. Second branchial cleft fistula: is fistulogram necessary for complete excision. Int J Pediatr Otorhinolaryngol 2006;70:1027-30. [4] Chandler RJ, Mitchell B. Branchial cleft cysts, sinuses and fistulas. Otolaryngol Clin N Am 1981;14:175-86. [5] Cox EF. Excision of branchial sinus and fistula tracts using arterial intimal strippers. Surg, Gynecol Obstet 1963;117:767-8. [6] Oshio T, Nakamiza H, Yoshikawa K, et al. A new fistulectomy method for the second pharyngeal arch remnants. J Pediatr Surg 2005;40:1784-7. [7] Patel NN, Hartley BEJ, Howard DJ. Management of thyroglossal tract disease after failed Sistrunk's procedure. J Laryngol Otol 2003;117:710-2. [8] de Jong RJB, Leiden MD. A new surgical technique for treatment of preauricular sinus. Surgery 2005;137:567-70. [9] Lam HC, Soo G, Wormald PJ, et al. Excision of the preauricular sinus: a comparison of two surgical techniques. Laryngoscope 2001;111:317-9. [10] Jakubikova J, Stanik R, Stanikova A. Malformations of the first branchial cleft: duplication of the external auditory canal. Int J Pediatr Otorhinolaryngol 2005;69:255-61.