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Third branchial cleft anomalies
Operative Techniques in Otolaryngology (2017) ], ]]]–]]]
Third branchial cleft anomalies Janet W. Lee, MD Jamie L. Funamura, MD From the Department of Otolaryngology—Head and Neck Surgery, UC Davis Health System, Sacramento, California KEYWORDS Congenital neck anomalies; open and endoscopic approach; sinus tract obliteration; laryngoscopy
Third branchial cleft anomalies are rare congenital abnormalities with a complicated anatomical course. The traditional standard of treatment is complete open excision of the entire tract, but newer endoscopic and combined approaches are becoming more common. We summarize the anatomy, presentation, and evaluation of these anomalies. A review of the literature regarding the various surgical options and their associated risks and benefits is discussed. We then review the details of open excision, endoscopic treatment, and combined open or endoscopic surgical options. Given the higher risk of complications with open excision and the similar rates of recurrence with open and endoscopic procedures, we recommend endoscopic management of third branchial cleft anomalies as the first-line treatment. Published by Elsevier Inc.
Introduction Third branchial cleft anomalies are rare, representing 2%8% of branchial cleft anomalies.1,2 Left-sided presentation is more common, with reported rates ranging from 89%-100%, compared to second branchial cleft cysts that are more common on the right side.1,3,4 Distinguishing between third and fourth branchial cleft anomalies can be clinically challenging; therefore, third and fourth branchial cleft anomalies are sometimes grouped together as “pyriform sinus fistulas” or related terms.1,3,5-7
Anatomy Anomalies of the third branchial cleft may present as cysts, sinuses, or fistulae, with sinuses being the most common. The anatomical course of these anomalies is quite complex, as determined both from intraoperative findings and cadaver dissections (Figure 1).2,4,8-10 An opening into the base of the pyriform sinus is characteristic of third branchial cleft Address reprint requests and correspondence: Jamie Funamura, MD, Department of Otolaryngology—Head and Neck Surgery, UC Davis Health System, 2521 Stockton Blvd, Ste 7200, Sacramento, CA 95817. E-mail address: [email protected] http://dx.doi.org/10.1016/j.otot.2017.05.014 1043-1810/Published by Elsevier Inc.
anomalies and is often considered diagnostic. From the point where this tract begins in the base of the pyriform sinus, it pierces through the thyrohyoid membrane, then turns superiorly to run parallel to the superior laryngeal nerve. The tract continues posterior to the internal or common carotid artery before coursing anterior to the vagus and superior laryngeal nerves. It then turns laterally to pass between the glossopharyngeal (cranial nerve [CN] IX) and hypoglossal nerves (CN XII) before looping inferiorly and passing superficial to the hypoglossal nerve. The tract continues inferiorly along the internal jugular vein and travels just deep to the sternocleidomastoid along its anterior border. Finally, the tract travels superficially through the strap and platysmal muscles. If there is a cutaneous opening, this will typically be located at the anterior border of the sternocleidomastoid muscle at the junction of its middle and inferior thirds. Although difficult to determine preoperatively, third branchial cleft anomalies may be differentiated from fourth branchial cleft anomalies in several ways. The general opinion is that fourth branchial cleft anomalies arise from the apex of the pyriform sinus rather than its base, which may be determined at the time of diagnostic rigid endoscopy. Fourth branchial anomalies also course caudal to the superior laryngeal nerve, whereas third branchial anomalies pass cranial to this nerve.4,6,8 In addition, fourth branchial
Operative Techniques in Otolaryngology, Vol ], No ], ] 2017
2
Stylohyoid Posterior belly of digastric
border of the lower sternocleidomastoid.3-7,9 Acute suppurative thyroiditis may be seen in 33%-42% of cases.1 In neonates, these lateral neck cysts or abscesses may cause airway obstruction, though this is quite rare.1,2
CN XII SLN
Carotid Thyroid cartilage
Cyst and tract
Figure 1 Anatomical relationships of third branchial cleft anomalies. The course of a third branchial cleft anomaly is shown relative to important anatomical structures. If a piriform sinus fistula is present, the tract will next pierce the thyrohyoid membrane and course parallel to the superior laryngeal nerve before passing between the glossopharyngeal and hypoglossal nerves and extending inferiorly, superficial to the internal or common carotid.
cleft anomalies exit the larynx near the cricothyroid joint, whereas third branchial anomalies pierce the thyrohyoid membrane.
Histology The histologic components of third branchial cleft anomalies are variable. Both squamous and lymphoid elements may be present, as well as endocrine tissue.6,10 A systematic review of 72 cases of third branchial cleft anomalies identified significant variability in their contents on histology: 58% contained stratified squamous epithelium, 35% contained respiratory ciliated epithelium, 40% demonstrated thymic tissue, 19% showed thyroid tissue, and 10% contained parathyroid tissue. Fifty-four percent of the lesions identified were described as cystic.1 The presence of thyroid, parathyroid, or thymic tissue, or all of these on histology does not differentiate a third from a fourth branchial cleft anomaly.2
Presentation The most common presentation of a third branchial cleft anomaly is recurrent left-sided neck abscess at the anterior
Evaluation In children with a history of recurrent lateral neck infection, especially on the left side, third or fourth branchial cleft anomalies should be suspected. Similarly, a history of even a single episode of acute suppurative thyroiditis, draining left neck infection, or lateral neck mass should raise clinical suspicion. Many patients will present with a history of prior incision and drainage, aspiration of a fluid-filled mass, or attempt at previous excision.3,4 A small case series demonstrated that 7 of 8 patients had a history of previous surgical therapy, with a mean number of 6 prior surgeries.4 In a larger case series of 27 children, 16 of them had been treated with previous drainage surgeries and 2 had prior attempts at excision.3 Physical examination findings will vary based on the severity of infection and the extent of the branchial anomaly. In the setting of active infection, findings are consistent with that of any lateral neck abscess, including pain, fever, erythema, skin warmth, swelling, induration, and possibly fluctuance. If there is a sinus or fistula opening into the piriform sinus, patients may present with respiratory symptoms, odynophagia, or dysphagia, or all of these. Outside of active infection, many patients do not have specific physical examination findings. In some cases a draining fistulous tract may be present, though this is atypical. Bedside flexible laryngoscopy may be performed to evaluate for piriform sinus swelling, erythema, or purulence, or all of these. In the setting of infection, the presence of a pyriform sinus opening is unlikely to be seen and operative direct laryngoscopy affords better visualization of this area.3-7 Although preoperative imaging is recommended, it is rare that a definitive diagnosis of a third branchial cleft anomaly can be made based on imaging along. Barium swallow can reveal the presence of a sinus or fistula tract extending from the pyriform sinus, but is not highly sensitive, especially in the setting of acute infection.3,4,6,7,11 X-ray fistulography can be done preoperatively or intraoperatively, and can reveal the presence of a sinus tract, but does not show 3-dimensional relationships between the tract and surrounding structures. Computed tomography, magnetic resonance imaging, and ultrasound can be useful for more cystic masses or if there is air present in the sinus tract, but often the tract cannot be easily followed on crosssectional imaging.3,5,7 Computed tomography may show loss of contrast enhancement within the ipsilateral thyroid lobe, a low-density area in the thyroid parenchyma, or thyroid/perithyroid inflammation (Figure 2).7 When used in combination with barium swallow or injection of contrast through the cutaneous opening of the fistula, if present, the sensitivity of cross-sectional imaging improves.7 Reactive
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Figure 2 Infected third branchial cleft cysts. Coronal computed tomography scans with contrast are shown of 2 different patients. Classically, abscesses of third branchial cleft cysts will appear to involve the left thyroid lobe.
lymphadenopathy may be seen in the setting of active infection but is not always present. If visualized, the tract may run adjacent to or through the thyroid lobe, anterior to the sternocleidomastoid muscle, and ascend along the carotid sheath.
Treatment options Most patients with third branchial cleft anomalies present with infection resulting in lateral neck abscess, acute suppurative thyroiditis, or both. Adequate resolution of the infection should be the primary goal, with a secondary goal of avoiding open incision and drainage if possible to facilitate delayed definitive treatment. Antibiotics are the mainstay of medical therapy and should include coverage for common respiratory pathogens. Avoidance of surgical intervention in the acute infectious state is preferred; however, surgery is indicated for infections that do not resolve or progress despite medical management. In some cases, needle aspiration or open incision and drainage cannot be avoided, but it is important to be aware that this can create scarring, which could complicate subsequent surgery. There is also the risk of iatrogenic fistula formation. In the setting of active infection, open resection is not recommended given the increased risk and difficulty of performing a complete excision of the tract although tissues are significantly inflamed. Delayed definitive treatment can be categorized into the following 3 approaches: (1) endoscopic, (2) traditional open resection, or (3) a combined endoscopic and open approach.
Indications for surgery As mentioned previously, definitive diagnosis of a third branchial anomaly almost always requires a surgical procedure, as direct laryngoscopy is often confirmatory of a fistula tract that is rarely identifiable on imaging or bedside
flexible laryngoscopy. In cases in which direct laryngoscopy is being performed for diagnostic purposes, endoscopic management of the pyriform sinus opening can be performed under the same anesthetic, even in the setting of active infection. Concurrent needle aspiration or open incision and drainage of abscesses may also be necessary.9 If the diagnosis has already been established and the acute infection resolved, definitive surgical management is indicated. The 3 aforementioned approaches of endoscopic vs open vs combined are described in detail later.
Surgical technique Endoscopic approach After induction of general anesthesia, the patient is turned 901 from the anesthetist in the standard fashion for microdirect laryngoscopy. If there is no plan for a concomitant approach from the neck, the patient can be draped and the procedure performed using clean technique. If there is to be a lateral neck approach performed in the same setting, sterile technique should be used. An appropriately sized laryngoscope and suspension apparatus should be chosen for exposure. Depending on the choice of the method of treatment, visualization should be aided with an operating microscope or telescope. The procedure can be performed with the patient breathing spontaneously or intubated with a small endotracheal tube. Once the patient has been optimally positioned and the sinus tract has been identified, obliteration is performed (Figure 3). Described methods of obliteration include the use of electrocautery, trichloroacetic acid, silver nitrate, CO2 laser, thalium laser, endoscopic oversewing, and fibrin glue.3,5,7,9,11 Combination techniques such as CO2 laser obliteration of the opening of the tract in the pyriform sinus under visualization by microdirect laryngoscopy, followed by oversewing with a chromic suture have also been described.5 Whichever method is chosen, care should be
4
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Figure 3 Endoscopic approach for third branchial cleft anomalies. With the patient in suspension, the hypopharynx is inspected with either a telescope or microscope (A). One method of obliteration is shown here: the fistula tract is identified (B), a Bugbee electrode is introduced (C), and the tract cauterized (D). (Color version of figure is available online.)
taken to treat the internal opening circumferentially to maximize the chance of complete closure of the tract opening.
Open approach Before surgery, potential risks of surgery should be discussed with the anesthesiology team including the risk of injury to the great vessels of the neck. Long-acting paralytic medications should be avoided so that intraoperative nerve stimulation can be performed if necessary. Under general anesthesia, the patient is turned 1801 from the anesthetist. Use of recurrent laryngeal nerve monitoring may be considered, especially if a hemithyroidectomy is planned. If a combined approach is to be used (as described later), the endoscopic portion of the procedure should precede the open portion. A shoulder roll is placed to facilitate neck extension. The patient is prepped and draped in a sterile fashion and the remainder of the procedure should be performed using sterile technique. A transverse skin incision over the central portion of the cyst and along a natural skin crease should be marked and infiltrated using local anesthetic with epinephrine. The position of the skin incision will vary according to the lesion, as isolated cysts will require less exposure than true sinus or fistula tracts. If a cutaneous opening is present, the incision can be modified into an elliptical incision around the tract opening. A “stepladder” incision may be used to gain increased exposure along the tract and to avoid a vertical incision or a much larger horizontal incision.8 If there is a cutaneous opening, this can be incorporated into one of the skin incisions and excised in an elliptical fashion (Figure 4). The incision is taken through the skin, subcutaneous tissues and the platysma muscle. Subplatysmal flaps are then raised. The anterior border of the sternocleidomastoid muscle is identified and its fascia incised to allow lateral rotation of the muscle, as the tract will ascend deep to the anterior border of this muscle. The carotid sheath is exposed and the tract carefully dissected free of the surrounding tissues as it ascends along the internal jugular vein. Dissection along the tract must be performed carefully as it passes superficially over the hypoglossal nerve, then dives medially between the hypoglossal (CN XII) and glossopharyngeal (CN IX) nerves. Care must be taken to avoid
Primary incision centered over cyst
Stepladder incision
Elliptical incision
Figure 4 Types of incisions for open cervical approaches to third branchial cleft anomalies. The primary cervical incision will typically be transverse in orientation and placed in an existing or future neck crease. Individual characteristics of the anomaly will dictate whether the incision is centered over the cyst vs creating an elliptical incision excising the fistula. A stepladder incision may be used for better access to the tract without needing to extend the primary incision.
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undue traction to the hypoglossal nerve as dissection continues along the tract, which will pass over the hypoglossal nerve, between the vagus and superior laryngeal nerves posteriorly and the internal carotid artery anteriorly. The tract will then turn again to course inferiorly and medially, parallel to the superior laryngeal nerve. The surgeon will next approach the tract origin where it pierces through the thyrohyoid membrane, still traveling superior to the superior laryngeal nerve, to end in the pyriform sinus. Exposure of the pyriform sinus using an oblique thyrotomy has been described,6 but this approach is not routinely performed. The pharyngeal communication is closed using 4-0 chromic suture. Whether or not a thyroid lobectomy is necessary or advisable during an open resection is at the discretion of the surgeon. A small penrose drain or suction drain is then placed into the wound and the wound is closed in a layered fashion using the surgeon's preferred method for platysmal and skin closure. The age of the patient should be taken into account, as nonabsorbable suture material may not be appropriate for younger children who may not tolerate suture removal. An area of evolving technique is the endoscope-assisted open treatment of branchial cleft cysts. One described modified open technique as that of a single elliptical incision made around the cutaneous tract opening and traced along its path in the traditional fashion until the limit of visualization of the tract through the initial skin incision.12 At that point, rather than making a second “stepladder” incision, an endoscope is used to continue dissection through the single incision. Just before the tract opens into the laryngeal mucosa, it is ligated with 2 titanium clips and the stump treated with bipolar cautery. A series of similar endoscope-assisted techniques showed no change in operative time or postoperative complications when compared to the traditional open approach.13
Combined approach Various combinations of endoscopic and open approaches have been reported. Some of these approaches use catheter, probe, glue, or dye insertion endoscopically through the pyriform sinus opening of the tract to aid in localization of the anomaly.3,4,6,9,11,14 Two distinct advantages of the combined approach exist. One advantage is that cannulation or dye application enabled by the endoscopic approach can help identify the tract, which can be elusive or challenging, or both to dissect, especially in a field where previous infection and surgery have resulted in extensive scarring. A second advantage is that endoscopic cauterization may be performed in this same setting, decreasing the likelihood of recurrence. A combined approach may be particularly beneficial where a prior endoscopic or open approach has failed (Figure 5).
Postoperative care Patients should be admitted to an inpatient unit postoperatively for monitoring. Perioperative use of steroids for
5
Figure 5 Combined approach for third branchial cleft cyst surgery. A combined endoscopic and open cervical approach addresses both the internal piriform sinus fistula as well as the branchial cleft tract and cyst in the neck.
edema is reasonable if there are no contraindications. In the setting of an acute infection, additional perioperative antibiotics are necessary. If there is no infection present, the procedure can be performed without perioperative antibiotics. Patients treated endoscopically should be kept nil per os and given intravenous hydration until postoperative day 1 or 2, at which time an oral diet can be resumed.1,3 Those treated with an open or combined approach may follow a similar postoperative course, though a longer nil per os period may be considered if the pharyngeal defect created during excision is larger. If an oral diet is well tolerated, endoscopically treated patients may be discharged the next day. Patients treated via an open approach may need continued inpatient care until their drains are removed. Frequently, drains will be kept in place until resuming an oral diet in order to facilitate monitoring for a pharyngeal leak.
Complications The most common complication of both endoscopic and open treatment of third branchial cleft anomalies is recurrence. Incision and drainage alone (often before a diagnosis of a branchial cleft anomaly has been made) leads to recurrence rates nearing 90%.1 Endoscopic treatment alone has been shown to have a recurrence rate of 18%25%1,5 but can only be used in cases in which an opening is present in the pyriform sinus. Open treatment with or without thyroid lobectomy results in recurrence rates from 14%-15%.1 With regards to whether or not a thyroid lobectomy should be performed at the time of open resection, a systematic review found no significant improvement in recurrence rates when a hemithyroidectomy was incorporated into the procedure.1 Recurrences after endoscopic management can be treated with repeat endoscopic
6 sinus obliteration (which is no more challenging than the initial procedure), vs an open or combined approach. Lesions that recur after open excision are more challenging to treat with repeat open excision because of postoperative scarring.6,9 Because endoscopic treatment is less likely to result in complications,1 some authors recommend initial treatment using an endoscopic approach, reserving the open approach for recurrent cases.9,11 Multiple methods of endoscopic obliteration have been described, but so far there is no significant evidence to advocate for one method over the others.1,5 Other complications from the treatment of a third branchial cleft anomaly include wound infection; bleeding; salivary fistula; vocal cord paresis or paralysis; injury to the superior laryngeal, hypoglossal, facial, or glossopharyngeal nerves; and Horner's syndrome. These are more common with an open approach than with an endoscopic approach, although exact incidence is unknown.1 Of patients treated with an open neck approach, it was found that there was a higher rate of complications in children 8 years old or younger.1 This finding has prompted some surgeons to consider open resection only after the age of 8 years.1
Conclusions Third branchial cleft anomalies are rare congenital abnormalities that are usually diagnosed in the setting of an acute or recurrent neck infection. Although open surgical excision of the entire tract remains the standard, there is movement toward endoscopic treatment of the opening of the sinus, which prevents infection by eliminating the source of contamination. Various treatment algorithms have been proposed, but currently there is no consensus regarding the appropriate situations in which to use endoscopic versus open management. To date, studies have shown that endoscopic management is comparable in efficacy with a lower risk of complications as compared to open excision and therefore may be a reasonable first approach. The most common complication of surgical management of third branchial cleft anomalies is recurrence, which can be treated via open, endoscopic, or combined means.
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Disclosure The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article.
References 1. Nicoucar K, Giger R, Jaecklin T, et al: Management of congenital third branchial arch anomalies: A systematic review. Otolaryngol Head Neck Surg 142(1). 21-28.e22, 2010 2. Mirilas P: Lateral congenital anomalies of the pharyngeal apparatus: Part II. Anatomy of the abnormal for the surgeon. Am Surg 77 (9):1243-1256, 2011 3. Hwang J, Kim SC, Kim DY, et al: Excision versus trichloroacetic acid (TCA) chemocauterization for branchial sinus of the pyriform fossa. J Pediatr Surg 50(11):1949-1953, 2015 4. Rea P, Hartley B, Bailey C: Third and fourth branchial pouch anomalies. J Laryngol Otol 118(01):19-24, 2004 5. Josephson GD, Black K: A review over the past 15 years of the management of the internal piriform apex sinus tract of a branchial pouch anomaly and case description. Ann Otol Rhinol Laryngol 124 (12):947-952. http://dx.doi.org/10.1177/0003489415593554, 2015 6. Pereira KD, Losh GG, Oliver D, et al: Management of anomalies of the third and fourth branchial pouches. Int J Pediatr Otorhinolaryngol 68 (1):43-50, 2004 7. Chauhan NS, Sharma YP, Bhagra T, et al: Branchial fistula arising from pyriform fossa: CT diagnosis of a case and discussion of radiological features. Clin Imaging 36(5):591-594, 2012 8. Mirilas P: Lateral congenital anomalies of the pharyngeal apparatus: Part III. Cadaveric representation of the course of second and third cleft and pouch fistulas. Am Surg 77(9):1257-1263, 2011 9. Wong PY, Moore A, Daya H: Management of third branchial pouch anomalies—An evolution of a minimally invasive technique. Int J Pediatr Otorhinolaryngol 78(3):493-498, 2014 10. Joshi M, Provenzano M, Smith R, et al: The rare third branchial cleft cyst. Am J Neuroradiol 30(9):1804-1806, 2009 11. Goff CJ, Allred C, Glade RS: Current management of congenital branchial cleft cysts, sinuses, and fistulae. Curr Opin Otolaryngol Head Neck Surg 20(6):533-539, 2012 12. Barrett G, Toynton S: Endoscopically assisted branchial fistula excision. Int J Pediatr Otorhinolaryngol 77(6):1048-1050, 2013 13. Teng SE, Paul BC, Brumm JD, et al: Endoscope-assisted approach to excision of branchial cleft cysts. Laryngoscope 126(6):1339-1342. http://dx.doi.org/10.1002/lary.25711, 2016 14. Piccioni M, Bottazzoli M, Nassif N, et al: Intraoperative use of fibrin glue dyed with methylene blue in surgery for branchial cleft anomalies. Laryngoscope 126(9):2147-2150. http://dx.doi.org/10.1002/lary. 25833, 2016
Third branchial cleft anomalies Janet W. Lee, MD Jamie L. Funamura, MD From the Department of Otolaryngology—Head and Neck Surgery, UC Davis Health System, Sacramento, California KEYWORDS Congenital neck anomalies; open and endoscopic approach; sinus tract obliteration; laryngoscopy
Third branchial cleft anomalies are rare congenital abnormalities with a complicated anatomical course. The traditional standard of treatment is complete open excision of the entire tract, but newer endoscopic and combined approaches are becoming more common. We summarize the anatomy, presentation, and evaluation of these anomalies. A review of the literature regarding the various surgical options and their associated risks and benefits is discussed. We then review the details of open excision, endoscopic treatment, and combined open or endoscopic surgical options. Given the higher risk of complications with open excision and the similar rates of recurrence with open and endoscopic procedures, we recommend endoscopic management of third branchial cleft anomalies as the first-line treatment. Published by Elsevier Inc.
Introduction Third branchial cleft anomalies are rare, representing 2%8% of branchial cleft anomalies.1,2 Left-sided presentation is more common, with reported rates ranging from 89%-100%, compared to second branchial cleft cysts that are more common on the right side.1,3,4 Distinguishing between third and fourth branchial cleft anomalies can be clinically challenging; therefore, third and fourth branchial cleft anomalies are sometimes grouped together as “pyriform sinus fistulas” or related terms.1,3,5-7
Anatomy Anomalies of the third branchial cleft may present as cysts, sinuses, or fistulae, with sinuses being the most common. The anatomical course of these anomalies is quite complex, as determined both from intraoperative findings and cadaver dissections (Figure 1).2,4,8-10 An opening into the base of the pyriform sinus is characteristic of third branchial cleft Address reprint requests and correspondence: Jamie Funamura, MD, Department of Otolaryngology—Head and Neck Surgery, UC Davis Health System, 2521 Stockton Blvd, Ste 7200, Sacramento, CA 95817. E-mail address: [email protected] http://dx.doi.org/10.1016/j.otot.2017.05.014 1043-1810/Published by Elsevier Inc.
anomalies and is often considered diagnostic. From the point where this tract begins in the base of the pyriform sinus, it pierces through the thyrohyoid membrane, then turns superiorly to run parallel to the superior laryngeal nerve. The tract continues posterior to the internal or common carotid artery before coursing anterior to the vagus and superior laryngeal nerves. It then turns laterally to pass between the glossopharyngeal (cranial nerve [CN] IX) and hypoglossal nerves (CN XII) before looping inferiorly and passing superficial to the hypoglossal nerve. The tract continues inferiorly along the internal jugular vein and travels just deep to the sternocleidomastoid along its anterior border. Finally, the tract travels superficially through the strap and platysmal muscles. If there is a cutaneous opening, this will typically be located at the anterior border of the sternocleidomastoid muscle at the junction of its middle and inferior thirds. Although difficult to determine preoperatively, third branchial cleft anomalies may be differentiated from fourth branchial cleft anomalies in several ways. The general opinion is that fourth branchial cleft anomalies arise from the apex of the pyriform sinus rather than its base, which may be determined at the time of diagnostic rigid endoscopy. Fourth branchial anomalies also course caudal to the superior laryngeal nerve, whereas third branchial anomalies pass cranial to this nerve.4,6,8 In addition, fourth branchial
Operative Techniques in Otolaryngology, Vol ], No ], ] 2017
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Stylohyoid Posterior belly of digastric
border of the lower sternocleidomastoid.3-7,9 Acute suppurative thyroiditis may be seen in 33%-42% of cases.1 In neonates, these lateral neck cysts or abscesses may cause airway obstruction, though this is quite rare.1,2
CN XII SLN
Carotid Thyroid cartilage
Cyst and tract
Figure 1 Anatomical relationships of third branchial cleft anomalies. The course of a third branchial cleft anomaly is shown relative to important anatomical structures. If a piriform sinus fistula is present, the tract will next pierce the thyrohyoid membrane and course parallel to the superior laryngeal nerve before passing between the glossopharyngeal and hypoglossal nerves and extending inferiorly, superficial to the internal or common carotid.
cleft anomalies exit the larynx near the cricothyroid joint, whereas third branchial anomalies pierce the thyrohyoid membrane.
Histology The histologic components of third branchial cleft anomalies are variable. Both squamous and lymphoid elements may be present, as well as endocrine tissue.6,10 A systematic review of 72 cases of third branchial cleft anomalies identified significant variability in their contents on histology: 58% contained stratified squamous epithelium, 35% contained respiratory ciliated epithelium, 40% demonstrated thymic tissue, 19% showed thyroid tissue, and 10% contained parathyroid tissue. Fifty-four percent of the lesions identified were described as cystic.1 The presence of thyroid, parathyroid, or thymic tissue, or all of these on histology does not differentiate a third from a fourth branchial cleft anomaly.2
Presentation The most common presentation of a third branchial cleft anomaly is recurrent left-sided neck abscess at the anterior
Evaluation In children with a history of recurrent lateral neck infection, especially on the left side, third or fourth branchial cleft anomalies should be suspected. Similarly, a history of even a single episode of acute suppurative thyroiditis, draining left neck infection, or lateral neck mass should raise clinical suspicion. Many patients will present with a history of prior incision and drainage, aspiration of a fluid-filled mass, or attempt at previous excision.3,4 A small case series demonstrated that 7 of 8 patients had a history of previous surgical therapy, with a mean number of 6 prior surgeries.4 In a larger case series of 27 children, 16 of them had been treated with previous drainage surgeries and 2 had prior attempts at excision.3 Physical examination findings will vary based on the severity of infection and the extent of the branchial anomaly. In the setting of active infection, findings are consistent with that of any lateral neck abscess, including pain, fever, erythema, skin warmth, swelling, induration, and possibly fluctuance. If there is a sinus or fistula opening into the piriform sinus, patients may present with respiratory symptoms, odynophagia, or dysphagia, or all of these. Outside of active infection, many patients do not have specific physical examination findings. In some cases a draining fistulous tract may be present, though this is atypical. Bedside flexible laryngoscopy may be performed to evaluate for piriform sinus swelling, erythema, or purulence, or all of these. In the setting of infection, the presence of a pyriform sinus opening is unlikely to be seen and operative direct laryngoscopy affords better visualization of this area.3-7 Although preoperative imaging is recommended, it is rare that a definitive diagnosis of a third branchial cleft anomaly can be made based on imaging along. Barium swallow can reveal the presence of a sinus or fistula tract extending from the pyriform sinus, but is not highly sensitive, especially in the setting of acute infection.3,4,6,7,11 X-ray fistulography can be done preoperatively or intraoperatively, and can reveal the presence of a sinus tract, but does not show 3-dimensional relationships between the tract and surrounding structures. Computed tomography, magnetic resonance imaging, and ultrasound can be useful for more cystic masses or if there is air present in the sinus tract, but often the tract cannot be easily followed on crosssectional imaging.3,5,7 Computed tomography may show loss of contrast enhancement within the ipsilateral thyroid lobe, a low-density area in the thyroid parenchyma, or thyroid/perithyroid inflammation (Figure 2).7 When used in combination with barium swallow or injection of contrast through the cutaneous opening of the fistula, if present, the sensitivity of cross-sectional imaging improves.7 Reactive
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Figure 2 Infected third branchial cleft cysts. Coronal computed tomography scans with contrast are shown of 2 different patients. Classically, abscesses of third branchial cleft cysts will appear to involve the left thyroid lobe.
lymphadenopathy may be seen in the setting of active infection but is not always present. If visualized, the tract may run adjacent to or through the thyroid lobe, anterior to the sternocleidomastoid muscle, and ascend along the carotid sheath.
Treatment options Most patients with third branchial cleft anomalies present with infection resulting in lateral neck abscess, acute suppurative thyroiditis, or both. Adequate resolution of the infection should be the primary goal, with a secondary goal of avoiding open incision and drainage if possible to facilitate delayed definitive treatment. Antibiotics are the mainstay of medical therapy and should include coverage for common respiratory pathogens. Avoidance of surgical intervention in the acute infectious state is preferred; however, surgery is indicated for infections that do not resolve or progress despite medical management. In some cases, needle aspiration or open incision and drainage cannot be avoided, but it is important to be aware that this can create scarring, which could complicate subsequent surgery. There is also the risk of iatrogenic fistula formation. In the setting of active infection, open resection is not recommended given the increased risk and difficulty of performing a complete excision of the tract although tissues are significantly inflamed. Delayed definitive treatment can be categorized into the following 3 approaches: (1) endoscopic, (2) traditional open resection, or (3) a combined endoscopic and open approach.
Indications for surgery As mentioned previously, definitive diagnosis of a third branchial anomaly almost always requires a surgical procedure, as direct laryngoscopy is often confirmatory of a fistula tract that is rarely identifiable on imaging or bedside
flexible laryngoscopy. In cases in which direct laryngoscopy is being performed for diagnostic purposes, endoscopic management of the pyriform sinus opening can be performed under the same anesthetic, even in the setting of active infection. Concurrent needle aspiration or open incision and drainage of abscesses may also be necessary.9 If the diagnosis has already been established and the acute infection resolved, definitive surgical management is indicated. The 3 aforementioned approaches of endoscopic vs open vs combined are described in detail later.
Surgical technique Endoscopic approach After induction of general anesthesia, the patient is turned 901 from the anesthetist in the standard fashion for microdirect laryngoscopy. If there is no plan for a concomitant approach from the neck, the patient can be draped and the procedure performed using clean technique. If there is to be a lateral neck approach performed in the same setting, sterile technique should be used. An appropriately sized laryngoscope and suspension apparatus should be chosen for exposure. Depending on the choice of the method of treatment, visualization should be aided with an operating microscope or telescope. The procedure can be performed with the patient breathing spontaneously or intubated with a small endotracheal tube. Once the patient has been optimally positioned and the sinus tract has been identified, obliteration is performed (Figure 3). Described methods of obliteration include the use of electrocautery, trichloroacetic acid, silver nitrate, CO2 laser, thalium laser, endoscopic oversewing, and fibrin glue.3,5,7,9,11 Combination techniques such as CO2 laser obliteration of the opening of the tract in the pyriform sinus under visualization by microdirect laryngoscopy, followed by oversewing with a chromic suture have also been described.5 Whichever method is chosen, care should be
4
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Figure 3 Endoscopic approach for third branchial cleft anomalies. With the patient in suspension, the hypopharynx is inspected with either a telescope or microscope (A). One method of obliteration is shown here: the fistula tract is identified (B), a Bugbee electrode is introduced (C), and the tract cauterized (D). (Color version of figure is available online.)
taken to treat the internal opening circumferentially to maximize the chance of complete closure of the tract opening.
Open approach Before surgery, potential risks of surgery should be discussed with the anesthesiology team including the risk of injury to the great vessels of the neck. Long-acting paralytic medications should be avoided so that intraoperative nerve stimulation can be performed if necessary. Under general anesthesia, the patient is turned 1801 from the anesthetist. Use of recurrent laryngeal nerve monitoring may be considered, especially if a hemithyroidectomy is planned. If a combined approach is to be used (as described later), the endoscopic portion of the procedure should precede the open portion. A shoulder roll is placed to facilitate neck extension. The patient is prepped and draped in a sterile fashion and the remainder of the procedure should be performed using sterile technique. A transverse skin incision over the central portion of the cyst and along a natural skin crease should be marked and infiltrated using local anesthetic with epinephrine. The position of the skin incision will vary according to the lesion, as isolated cysts will require less exposure than true sinus or fistula tracts. If a cutaneous opening is present, the incision can be modified into an elliptical incision around the tract opening. A “stepladder” incision may be used to gain increased exposure along the tract and to avoid a vertical incision or a much larger horizontal incision.8 If there is a cutaneous opening, this can be incorporated into one of the skin incisions and excised in an elliptical fashion (Figure 4). The incision is taken through the skin, subcutaneous tissues and the platysma muscle. Subplatysmal flaps are then raised. The anterior border of the sternocleidomastoid muscle is identified and its fascia incised to allow lateral rotation of the muscle, as the tract will ascend deep to the anterior border of this muscle. The carotid sheath is exposed and the tract carefully dissected free of the surrounding tissues as it ascends along the internal jugular vein. Dissection along the tract must be performed carefully as it passes superficially over the hypoglossal nerve, then dives medially between the hypoglossal (CN XII) and glossopharyngeal (CN IX) nerves. Care must be taken to avoid
Primary incision centered over cyst
Stepladder incision
Elliptical incision
Figure 4 Types of incisions for open cervical approaches to third branchial cleft anomalies. The primary cervical incision will typically be transverse in orientation and placed in an existing or future neck crease. Individual characteristics of the anomaly will dictate whether the incision is centered over the cyst vs creating an elliptical incision excising the fistula. A stepladder incision may be used for better access to the tract without needing to extend the primary incision.
Lee and Funamura
Third branchial anomalies
undue traction to the hypoglossal nerve as dissection continues along the tract, which will pass over the hypoglossal nerve, between the vagus and superior laryngeal nerves posteriorly and the internal carotid artery anteriorly. The tract will then turn again to course inferiorly and medially, parallel to the superior laryngeal nerve. The surgeon will next approach the tract origin where it pierces through the thyrohyoid membrane, still traveling superior to the superior laryngeal nerve, to end in the pyriform sinus. Exposure of the pyriform sinus using an oblique thyrotomy has been described,6 but this approach is not routinely performed. The pharyngeal communication is closed using 4-0 chromic suture. Whether or not a thyroid lobectomy is necessary or advisable during an open resection is at the discretion of the surgeon. A small penrose drain or suction drain is then placed into the wound and the wound is closed in a layered fashion using the surgeon's preferred method for platysmal and skin closure. The age of the patient should be taken into account, as nonabsorbable suture material may not be appropriate for younger children who may not tolerate suture removal. An area of evolving technique is the endoscope-assisted open treatment of branchial cleft cysts. One described modified open technique as that of a single elliptical incision made around the cutaneous tract opening and traced along its path in the traditional fashion until the limit of visualization of the tract through the initial skin incision.12 At that point, rather than making a second “stepladder” incision, an endoscope is used to continue dissection through the single incision. Just before the tract opens into the laryngeal mucosa, it is ligated with 2 titanium clips and the stump treated with bipolar cautery. A series of similar endoscope-assisted techniques showed no change in operative time or postoperative complications when compared to the traditional open approach.13
Combined approach Various combinations of endoscopic and open approaches have been reported. Some of these approaches use catheter, probe, glue, or dye insertion endoscopically through the pyriform sinus opening of the tract to aid in localization of the anomaly.3,4,6,9,11,14 Two distinct advantages of the combined approach exist. One advantage is that cannulation or dye application enabled by the endoscopic approach can help identify the tract, which can be elusive or challenging, or both to dissect, especially in a field where previous infection and surgery have resulted in extensive scarring. A second advantage is that endoscopic cauterization may be performed in this same setting, decreasing the likelihood of recurrence. A combined approach may be particularly beneficial where a prior endoscopic or open approach has failed (Figure 5).
Postoperative care Patients should be admitted to an inpatient unit postoperatively for monitoring. Perioperative use of steroids for
5
Figure 5 Combined approach for third branchial cleft cyst surgery. A combined endoscopic and open cervical approach addresses both the internal piriform sinus fistula as well as the branchial cleft tract and cyst in the neck.
edema is reasonable if there are no contraindications. In the setting of an acute infection, additional perioperative antibiotics are necessary. If there is no infection present, the procedure can be performed without perioperative antibiotics. Patients treated endoscopically should be kept nil per os and given intravenous hydration until postoperative day 1 or 2, at which time an oral diet can be resumed.1,3 Those treated with an open or combined approach may follow a similar postoperative course, though a longer nil per os period may be considered if the pharyngeal defect created during excision is larger. If an oral diet is well tolerated, endoscopically treated patients may be discharged the next day. Patients treated via an open approach may need continued inpatient care until their drains are removed. Frequently, drains will be kept in place until resuming an oral diet in order to facilitate monitoring for a pharyngeal leak.
Complications The most common complication of both endoscopic and open treatment of third branchial cleft anomalies is recurrence. Incision and drainage alone (often before a diagnosis of a branchial cleft anomaly has been made) leads to recurrence rates nearing 90%.1 Endoscopic treatment alone has been shown to have a recurrence rate of 18%25%1,5 but can only be used in cases in which an opening is present in the pyriform sinus. Open treatment with or without thyroid lobectomy results in recurrence rates from 14%-15%.1 With regards to whether or not a thyroid lobectomy should be performed at the time of open resection, a systematic review found no significant improvement in recurrence rates when a hemithyroidectomy was incorporated into the procedure.1 Recurrences after endoscopic management can be treated with repeat endoscopic
6 sinus obliteration (which is no more challenging than the initial procedure), vs an open or combined approach. Lesions that recur after open excision are more challenging to treat with repeat open excision because of postoperative scarring.6,9 Because endoscopic treatment is less likely to result in complications,1 some authors recommend initial treatment using an endoscopic approach, reserving the open approach for recurrent cases.9,11 Multiple methods of endoscopic obliteration have been described, but so far there is no significant evidence to advocate for one method over the others.1,5 Other complications from the treatment of a third branchial cleft anomaly include wound infection; bleeding; salivary fistula; vocal cord paresis or paralysis; injury to the superior laryngeal, hypoglossal, facial, or glossopharyngeal nerves; and Horner's syndrome. These are more common with an open approach than with an endoscopic approach, although exact incidence is unknown.1 Of patients treated with an open neck approach, it was found that there was a higher rate of complications in children 8 years old or younger.1 This finding has prompted some surgeons to consider open resection only after the age of 8 years.1
Conclusions Third branchial cleft anomalies are rare congenital abnormalities that are usually diagnosed in the setting of an acute or recurrent neck infection. Although open surgical excision of the entire tract remains the standard, there is movement toward endoscopic treatment of the opening of the sinus, which prevents infection by eliminating the source of contamination. Various treatment algorithms have been proposed, but currently there is no consensus regarding the appropriate situations in which to use endoscopic versus open management. To date, studies have shown that endoscopic management is comparable in efficacy with a lower risk of complications as compared to open excision and therefore may be a reasonable first approach. The most common complication of surgical management of third branchial cleft anomalies is recurrence, which can be treated via open, endoscopic, or combined means.
Operative Techniques in Otolaryngology, Vol ], No ], ] 2017
Disclosure The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article.
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