- Email: [email protected]
First branchial cleft anomalies
Author’s Accepted Manuscript First Branchial Cleft Anomalies Andrea D. Olivas, Jonathan M. Sherman
www.techgiendoscopy.com
PII: DOI: Reference:
S1043-1810(17)30067-2 http://dx.doi.org/10.1016/j.otot.2017.05.012 YOTOT775
To appear in: Operative Techniques in Otolaryngology - Head and Neck Surgery Cite this article as: Andrea D. Olivas and Jonathan M. Sherman, First Branchial Cleft Anomalies, Operative Techniques in Otolaryngology - Head and Neck Surgery, http://dx.doi.org/10.1016/j.otot.2017.05.012 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
First Branchial Cleft Anomalies Andrea D. Olivas1 and Jonathan M. Sherman2
1
Department of Pathology, University of Chicago, Chicago, IL
2
Department of Pediatric Otolaryngology, Advocate Children’s Hospital, Oak Lawn, IL
Jonathan Sherman [email protected]
Abstract First branchial cleft anomalies are rare congenital malformation of the head and neck. They occur above the level of the hyoid bone and have a close anatomic relationship to the facial nerve due to their embryologic origin. In this article, we will review the development and derivatives of the first branchial apparatus followed by the epidemiology and classification of these lesions. The treatment of choice for first branchial anomalies is surgical resection, which will be the focus of this article.
Embryology, Anatomy, and Classification Six branchial arches, separated externally by clefts and internally by pouches, form during the fourth week of embryologic development. The first branchial arch gives rise to the maxillary artery, trigeminal nerve, muscles of mastication, malleus, incus, sphenomandibular ligament, and Meckel’s cartilage. Derivatives of the first branchial pouch are the eustachian tube and middle
ear cleft, while derivatives of the first cleft include the external auditory canal, conchal cartilage, and external layer of the tympanic membrane (Figure 1). Anomalies of the first branchial cleft occur when there is incomplete fusion of the ventral part of the first and second arches, which typically takes place by the seventh week of development. Fusion occurs during the same time as the development of the parotid gland and the facial nerve, resulting in an intimate relationship between these structures and first branchial cleft anomalies. In the early 1970s, two classification systems of first branchial cleft anomalies were proposed. Arnot suggested that type 1 defects occur in the parotid region and type 2 defects occur in the anterior cervical triangle superior to the hyoid bone, often with a connection to the external auditory canal.1 Later, Work suggested a histological classification.2 Type 1 defects were believed to be strictly ectodermal in origin, essentially creating a duplicate external auditory canal which runs parallel and superior to the true external auditory canal. Type 2 defects, on the other hand, included both ectoderm and mesoderm and therefore involved both skin and underlying cartilage. Ultimately, the two systems define the same subsets among first branchial cleft anomalies. Almost a decade after these two classification systems were established, Olsen et al suggested a more clinically oriented classification which is similar to other branchial cleft anomalies.3 In this classification system, a first branchial cleft anomaly is a cavity lined with squamous epithelium occurring in an anatomical space between derivatives of the first and second branchial arches (which occupies a triangular region defined by vertices at the external auditory canal, the mental symphysis, and the lateral aspect of the hyoid bone – Figure 2). Such an enclosure without connections to the external environment is a first branchial cleft cyst; a sinus is one that consists of a single connection via a tract, typically to the external
auditory canal; and a fistula has two connections, most often to the external auditory canal and the submandibular skin. The formation of either a cyst, sinus, or fistula and the location of the defect depends on the timing of arrested embryologic development.
Epidemiology While branchial cleft anomalies as a general class are the second most common cause of congenital neck masses, first branchial cleft anomalies account for a small fraction of these.4 Estimates of that fraction vary; some report nearly a quarter of all defects arising from the first cleft,5 while most others suggest that these account for less than 10%.3,6 Some authors have suggested that there may be a female predominance. While there is no obvious predilection to one side of the neck for sinus tracts and cysts, there may be a left sided predominance for fistulae.7,8 Type 2 defects are substantially more common than type 1.6 The estimated incidence of first branchial cleft anomalies is 1 per million births.9
Natural history and Clinical Presentation Diagnosis of first branchial anomalies is challenging, as these are uncommon lesions. In one of the largest literature reviews of this pathology, age at time of diagnosis ranged from 20 days to 82 years, with the mean age at diagnosis being greater than 18 years.8 Treatment is also difficult, lending to the fact that there is a 3 to 4 year delay between identification of the problem and definitive treatment, as suggested by multiple retrospective series.7,10 The most common presenting sign of a first branchial cleft anomalies is a visible sinus tract in the skin. Sinus tracts are most frequently seen in the external auditory canal, followed by the neck, the concha, and the postauricular area.8 The second most common presenting symptom
is recurrent periauricular swelling. Patients have typically been classified as presenting with either cervical (40%), parotid (35%), or periauricular (25%) swelling.11 Otorrhea is present in a significant fraction of patients. Forty-four percent of patients with first branchial cleft anomalies have identifiable openings into the external auditory canal at the time of surgery, but many of these are not obvious on initial physical exam.12 The opening in the canal is most commonly found at the junction of the bony and cartilaginous canal but can also be seen throughout the length of the canal. Myringeal webs are not frequently seen, but this finding is highly suggestive of a first branchial cleft anomaly.7 Typically, there is a bridge of thickened tissue between the inferior canal and the umbo (Figure 3). It has been suggested that this is an associated, but not directly related, abnormality that does not require excision.12 First branchial cleft lesions are distinct from preauricular cysts and sinus tracts. The latter arise from failure of fusion of the auricular hillocks of His instead of the branchial arches, are vastly more common than first branchial cleft lesions, and have connections to the preauricular skin, outside of the typical triangle where first branchial cleft lesions are most commonly seen.13
Diagnosis and Work up Careful physical examination focusing on the external auditory canal is generally accepted as the most helpful tool in timely diagnosis of first branchial cleft anomalies. Perhaps the most specific finding is a myringeal web, but in the largest single institution case series, only 10% of patients were found to have this on exam.7 In this series, half of patients underwent previous unsuccessful treatment before diagnosis. This estimate may be low, as other authors have reported greater than 90% of patients with previous incision and drainage14 and the mean number of operations before cure to be 2.4.10
Although imaging studies for first branchial cleft anomalies, like skin defects, are not often specific, they are helpful in making a diagnosis. A tract near the external canal can often be seen on computed tomography (CT) scan. The most common finding on CT scan is a thinrimmed cystic lesion in the parotid gland without significant post-contrast rim enhancement.15 While a CT scan is the most common radiographic study obtained for diagnosis and surgical planning, a recent study showed CT imaging led to correct diagnosis in 95% and 81% of cysts and sinuses, respectively, but just half of fistulae.16 MRI can show the relationship to the parotid gland with greater detail. Fistulogram may help in showing the extent of a sinus tract or fistula leading to more complete excision, and some authors have suggested that CT fistulogram with coronal reconstruction is particularly helpful.17
Management of Type 2 First Branchial Anomalies The fundamental principle of treatment of first branchial cleft anomalies is the same as that of all other branchial cleft anomalies: cure is only achieved with complete surgical excision. The intimate relationship between this anomaly and the facial nerve, at least in type 2 lesions, makes identification and preservation of that nerve the focus of surgical management (Figure 4). This can be made challenging by the fact that diagnosis is often only made after at least one attempted incision and drainage, which leads to increased scarring. The rate of iatrogenic facial nerve palsy resulting from treatment has been shown to be related to the frequency of previous surgical intervention before definitive excision.7 Many authors have attempted to define the relationship between first branchial cleft anomalies and the facial nerve. This is the main objective of studies by D’Souza, and while most isolated cysts seem to be lateral to the nerve, fistulas clearly have a variable presentation, with
41% being lateral, 37% being medial, and the remaining 12% passing between branches.8 It is this variable relationship that lends to the general suggestion that type 2 lesions should be approached via a parotidectomy incision with complete dissection of the facial nerve from the main trunk. Type 1 lesions, which tend to be superior and posterior to the branches of the facial nerve, can typically be removed without this dissection and managed through a retroauricular incision, sparing the meatal skin of the external auditory canal. Surgical resection of type 2 anomalies is performed under general anesthesia. Longacting paralytics should be avoided by the anesthetist to allow for facial nerve monitoring. A shoulder roll is placed for gentle neck extension and the patient’s head is turned to allow for access to the lesion. The patient is prepped in the standard fashion for a parotidectomy with the operative field extending from the suprauricular area to the clavicle. The lateral face is left exposed to monitor for facial muscle twitches during the dissection. An incision is planned beginning at the preauricular skin crease and extending posteriorly into the retroauricular space and then inferiorly into a neck skin crease. If there is an external skin opening, then an ellipse is fashioned around the opening such that it is excised en bloc with the lesion. The planned incision is infiltrated with local anesthesia mixed with 1:100,000 or 1:200,000 epinephrine. A 15 blade scalpel is used to incise the skin and subcutaneous tissue. A skin flap is raised anteriorly within the superficial facial layer. Inferiorly, a subplatysmal flap is elevated. Next, the posterior border of the parotid gland is identified and bluntly dissected free from the external auditory canal cartilage. The sternocleidomastoid muscle is identified as well as the greater auricular nerve, which is identified as it travels anterosuperiorly over the muscle. The posterior belly of the digastric muscle is identified next as it inserts onto the mastoid tip. This is
used as a landmark along with the tragal pointer for the main trunk of the facial nerve. The nerve is identified just inferior to the pointer and a facial nerve stimulator can be used to confirm its location. Dissection then proceeds laterally along the facial nerve branches until the boundaries of the branchial anomaly are completely exposed and known to be free from the nerve. The lesion is then removed en bloc including any connection with the external canal. The wound is irrigated and a bulb suction drain is placed within the wound. This is secured to the skin with a single stitch. The soft tissue is closed in layers using 4.0 vicryl for the playsmal/fascial layers and 5.0 vicryl for the deep dermal layer. Skin is reapproximated using absorbable suture in a subcuticular fashion to avoid later stitch removal. A compression dressing is applied to the face and the patient is awoken from anesthesia. The patient is monitored overnight and the drain is removed the following day at the surgeon’s discretion. Activity is restricted for 1 week postoperatively.
Complications Hematoma and seroma formation, wound site infection, and wound dehiscence are complications of any surgical excision. Rate of injury to the facial nerve is high, even with careful dissection and facial nerve monitoring, and temporary palsy occurs in 18% of cases.8 To achieve complete excision, the surgeon should also be prepared to address the external canal and resect skin and cartilage.18 First bite and Frey’s syndrome are rare complications of parotidectomy that caregivers should be counseled about pre-operatively.
Conclusion First branchial cleft anomalies are rare, and their diagnosis is often delayed. Treatment is complicated by both the challenging and variable anatomic relationship to the facial nerve and the fact that most are mischaracterized and treated inappropriately with incision and drainage prior to formal excision. A high level of suspicion for first branchial cleft anomalies in patients with sinus tracts in the affected region or otorrhea without middle ear disease is necessary to expedite diagnosis, avoid inappropriate surgical management, and safely complete surgical excision with facial nerve identification and preservation.
REFERENCES 1.
Arnot RS. Defects of the first branchial cleft. S Afr J Surg. 1971;9(2):93-98.
2.
Work WP. Newer concepts of first branchial cleft defects. Laryngoscope. 1972;82(9):1581-1593.
3.
Olsen KD, Maragos NE, Weiland LH. First branchial cleft anomalies. Laryngoscope. 1980;90(3):423-436.
4.
Mounsey RA, Forte V, Friedberg J. First brachial cleft sinuses: an analysis of current management strategies and treatment outcomes. J Otolaryngol. 1993;22(6):457-461.
5.
Choi SS, Zalzal GH. Branchial anomalies: a review of 52 cases. Laryngoscope. 1995;105(9 Pt 1):909-913.
6.
Finn DG, Buchalter IH, Sarti E, Romo T, Chodosh P. First branchial cleft cysts: clinical update. Laryngoscope. 1987;97(2):136-140.
7.
Triglia JM, Nicollas R, Ducroz V, Koltai PJ, Garabedian EN. First branchial cleft anomalies: a study of 39 cases and a review of the literature. Arch Otolaryngol Head Neck Surg. 1998;124(3):291-295.
8.
D'Souza AR, Uppal HS, De R, Zeitoun H. Updating concepts of first branchial cleft defects: a literature review. Int J Pediatr Otorhinolaryngol. 2002;62(2):103-109.
9.
Arndal H, Bonding P. First branchial cleft anomaly. Clin Otolaryngol Allied Sci. 1996;21(3):203-207.
10.
Ford GR, Balakrishnan A, Evans JN, Bailey CM. Branchial cleft and pouch anomalies. J Laryngol Otol. 1992;106(2):137-143.
11.
Nicollas R, Guelfucci B, Roman S, Triglia JM. Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol. 2000;55(2):117-124.
12.
Sichel JY, Halperin D, Dano I, Dangoor E. Clinical update on type II first branchial cleft cysts. Laryngoscope. 1998;108(10):1524-1527.
13.
Hickey SA, Scott GA, Traub P. Defects of the first branchial cleft. J Laryngol Otol. 1994;108(3):240-243.
14.
Aronsohn RS, Batsakis JG, Rice DH, Work WP. Anomalies of the first branchial cleft. Arch Otolaryngol. 1976;102(12):737-740.
15.
Fordham MT, Lambert PR. First Branchial Cleft Anomalies. In: Kountakis SE, ed. Encyclopedia of Otolaryngology, Head and Neck Surgery. Berlin, Heidelberg: Springer Berlin Heidelberg; 2013:944-951.
16.
Schroeder JW, Jr., Mohyuddin N, Maddalozzo J. Branchial anomalies in the pediatric population. Otolaryngol Head Neck Surg. 2007;137(2):289-295.
17.
Whetstone J, Branstetter BFt, Hirsch BE. Fluoroscopic and CT fistulography of the first branchial cleft. AJNR Am J Neuroradiol. 2006;27(9):1817-1819.
18.
Stulner C, Chambers PA, Telfer MR, Corrigan AM. Management of first branchial cleft anomalies: report of two cases. Br J Oral Maxillofac Surg. 2001;39(1):30-33.
Figure Legends Figure 1. Nerves associated with the branchial arches in the embryo (A) and fetus (B). Note that cranial nerve V (the trigeminal nerve) is associated with the first branchial arch and therefore the muscles that it innervates (C) are the muscular derivatives of this arch. Bony and cartilaginous derivatives of the first branchial arch (D) include the maxillary process, zygoma, malleus (except manubrium), incus (except long process), sphenomandibular ligament, and Meckel’s cartilage. Figure 2. Triangle outlining the anatomic space where first branchial cleft anomalies occur. This space is defined by vertices at the external auditory canal, the mental symphysis, and the lateral aspect of the hyoid bone. Figure 3. Transcanal microscopic view of a myringeal web, a bridge of thickened tissue between the inferior canal and the umbo. This finding is highly suggestive of a first branchial cleft anomaly. Figure 4. Illustration depicting the differences between type 1 and 2 first branchial cleft anomalies.
www.techgiendoscopy.com
PII: DOI: Reference:
S1043-1810(17)30067-2 http://dx.doi.org/10.1016/j.otot.2017.05.012 YOTOT775
To appear in: Operative Techniques in Otolaryngology - Head and Neck Surgery Cite this article as: Andrea D. Olivas and Jonathan M. Sherman, First Branchial Cleft Anomalies, Operative Techniques in Otolaryngology - Head and Neck Surgery, http://dx.doi.org/10.1016/j.otot.2017.05.012 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
First Branchial Cleft Anomalies Andrea D. Olivas1 and Jonathan M. Sherman2
1
Department of Pathology, University of Chicago, Chicago, IL
2
Department of Pediatric Otolaryngology, Advocate Children’s Hospital, Oak Lawn, IL
Jonathan Sherman [email protected]
Abstract First branchial cleft anomalies are rare congenital malformation of the head and neck. They occur above the level of the hyoid bone and have a close anatomic relationship to the facial nerve due to their embryologic origin. In this article, we will review the development and derivatives of the first branchial apparatus followed by the epidemiology and classification of these lesions. The treatment of choice for first branchial anomalies is surgical resection, which will be the focus of this article.
Embryology, Anatomy, and Classification Six branchial arches, separated externally by clefts and internally by pouches, form during the fourth week of embryologic development. The first branchial arch gives rise to the maxillary artery, trigeminal nerve, muscles of mastication, malleus, incus, sphenomandibular ligament, and Meckel’s cartilage. Derivatives of the first branchial pouch are the eustachian tube and middle
ear cleft, while derivatives of the first cleft include the external auditory canal, conchal cartilage, and external layer of the tympanic membrane (Figure 1). Anomalies of the first branchial cleft occur when there is incomplete fusion of the ventral part of the first and second arches, which typically takes place by the seventh week of development. Fusion occurs during the same time as the development of the parotid gland and the facial nerve, resulting in an intimate relationship between these structures and first branchial cleft anomalies. In the early 1970s, two classification systems of first branchial cleft anomalies were proposed. Arnot suggested that type 1 defects occur in the parotid region and type 2 defects occur in the anterior cervical triangle superior to the hyoid bone, often with a connection to the external auditory canal.1 Later, Work suggested a histological classification.2 Type 1 defects were believed to be strictly ectodermal in origin, essentially creating a duplicate external auditory canal which runs parallel and superior to the true external auditory canal. Type 2 defects, on the other hand, included both ectoderm and mesoderm and therefore involved both skin and underlying cartilage. Ultimately, the two systems define the same subsets among first branchial cleft anomalies. Almost a decade after these two classification systems were established, Olsen et al suggested a more clinically oriented classification which is similar to other branchial cleft anomalies.3 In this classification system, a first branchial cleft anomaly is a cavity lined with squamous epithelium occurring in an anatomical space between derivatives of the first and second branchial arches (which occupies a triangular region defined by vertices at the external auditory canal, the mental symphysis, and the lateral aspect of the hyoid bone – Figure 2). Such an enclosure without connections to the external environment is a first branchial cleft cyst; a sinus is one that consists of a single connection via a tract, typically to the external
auditory canal; and a fistula has two connections, most often to the external auditory canal and the submandibular skin. The formation of either a cyst, sinus, or fistula and the location of the defect depends on the timing of arrested embryologic development.
Epidemiology While branchial cleft anomalies as a general class are the second most common cause of congenital neck masses, first branchial cleft anomalies account for a small fraction of these.4 Estimates of that fraction vary; some report nearly a quarter of all defects arising from the first cleft,5 while most others suggest that these account for less than 10%.3,6 Some authors have suggested that there may be a female predominance. While there is no obvious predilection to one side of the neck for sinus tracts and cysts, there may be a left sided predominance for fistulae.7,8 Type 2 defects are substantially more common than type 1.6 The estimated incidence of first branchial cleft anomalies is 1 per million births.9
Natural history and Clinical Presentation Diagnosis of first branchial anomalies is challenging, as these are uncommon lesions. In one of the largest literature reviews of this pathology, age at time of diagnosis ranged from 20 days to 82 years, with the mean age at diagnosis being greater than 18 years.8 Treatment is also difficult, lending to the fact that there is a 3 to 4 year delay between identification of the problem and definitive treatment, as suggested by multiple retrospective series.7,10 The most common presenting sign of a first branchial cleft anomalies is a visible sinus tract in the skin. Sinus tracts are most frequently seen in the external auditory canal, followed by the neck, the concha, and the postauricular area.8 The second most common presenting symptom
is recurrent periauricular swelling. Patients have typically been classified as presenting with either cervical (40%), parotid (35%), or periauricular (25%) swelling.11 Otorrhea is present in a significant fraction of patients. Forty-four percent of patients with first branchial cleft anomalies have identifiable openings into the external auditory canal at the time of surgery, but many of these are not obvious on initial physical exam.12 The opening in the canal is most commonly found at the junction of the bony and cartilaginous canal but can also be seen throughout the length of the canal. Myringeal webs are not frequently seen, but this finding is highly suggestive of a first branchial cleft anomaly.7 Typically, there is a bridge of thickened tissue between the inferior canal and the umbo (Figure 3). It has been suggested that this is an associated, but not directly related, abnormality that does not require excision.12 First branchial cleft lesions are distinct from preauricular cysts and sinus tracts. The latter arise from failure of fusion of the auricular hillocks of His instead of the branchial arches, are vastly more common than first branchial cleft lesions, and have connections to the preauricular skin, outside of the typical triangle where first branchial cleft lesions are most commonly seen.13
Diagnosis and Work up Careful physical examination focusing on the external auditory canal is generally accepted as the most helpful tool in timely diagnosis of first branchial cleft anomalies. Perhaps the most specific finding is a myringeal web, but in the largest single institution case series, only 10% of patients were found to have this on exam.7 In this series, half of patients underwent previous unsuccessful treatment before diagnosis. This estimate may be low, as other authors have reported greater than 90% of patients with previous incision and drainage14 and the mean number of operations before cure to be 2.4.10
Although imaging studies for first branchial cleft anomalies, like skin defects, are not often specific, they are helpful in making a diagnosis. A tract near the external canal can often be seen on computed tomography (CT) scan. The most common finding on CT scan is a thinrimmed cystic lesion in the parotid gland without significant post-contrast rim enhancement.15 While a CT scan is the most common radiographic study obtained for diagnosis and surgical planning, a recent study showed CT imaging led to correct diagnosis in 95% and 81% of cysts and sinuses, respectively, but just half of fistulae.16 MRI can show the relationship to the parotid gland with greater detail. Fistulogram may help in showing the extent of a sinus tract or fistula leading to more complete excision, and some authors have suggested that CT fistulogram with coronal reconstruction is particularly helpful.17
Management of Type 2 First Branchial Anomalies The fundamental principle of treatment of first branchial cleft anomalies is the same as that of all other branchial cleft anomalies: cure is only achieved with complete surgical excision. The intimate relationship between this anomaly and the facial nerve, at least in type 2 lesions, makes identification and preservation of that nerve the focus of surgical management (Figure 4). This can be made challenging by the fact that diagnosis is often only made after at least one attempted incision and drainage, which leads to increased scarring. The rate of iatrogenic facial nerve palsy resulting from treatment has been shown to be related to the frequency of previous surgical intervention before definitive excision.7 Many authors have attempted to define the relationship between first branchial cleft anomalies and the facial nerve. This is the main objective of studies by D’Souza, and while most isolated cysts seem to be lateral to the nerve, fistulas clearly have a variable presentation, with
41% being lateral, 37% being medial, and the remaining 12% passing between branches.8 It is this variable relationship that lends to the general suggestion that type 2 lesions should be approached via a parotidectomy incision with complete dissection of the facial nerve from the main trunk. Type 1 lesions, which tend to be superior and posterior to the branches of the facial nerve, can typically be removed without this dissection and managed through a retroauricular incision, sparing the meatal skin of the external auditory canal. Surgical resection of type 2 anomalies is performed under general anesthesia. Longacting paralytics should be avoided by the anesthetist to allow for facial nerve monitoring. A shoulder roll is placed for gentle neck extension and the patient’s head is turned to allow for access to the lesion. The patient is prepped in the standard fashion for a parotidectomy with the operative field extending from the suprauricular area to the clavicle. The lateral face is left exposed to monitor for facial muscle twitches during the dissection. An incision is planned beginning at the preauricular skin crease and extending posteriorly into the retroauricular space and then inferiorly into a neck skin crease. If there is an external skin opening, then an ellipse is fashioned around the opening such that it is excised en bloc with the lesion. The planned incision is infiltrated with local anesthesia mixed with 1:100,000 or 1:200,000 epinephrine. A 15 blade scalpel is used to incise the skin and subcutaneous tissue. A skin flap is raised anteriorly within the superficial facial layer. Inferiorly, a subplatysmal flap is elevated. Next, the posterior border of the parotid gland is identified and bluntly dissected free from the external auditory canal cartilage. The sternocleidomastoid muscle is identified as well as the greater auricular nerve, which is identified as it travels anterosuperiorly over the muscle. The posterior belly of the digastric muscle is identified next as it inserts onto the mastoid tip. This is
used as a landmark along with the tragal pointer for the main trunk of the facial nerve. The nerve is identified just inferior to the pointer and a facial nerve stimulator can be used to confirm its location. Dissection then proceeds laterally along the facial nerve branches until the boundaries of the branchial anomaly are completely exposed and known to be free from the nerve. The lesion is then removed en bloc including any connection with the external canal. The wound is irrigated and a bulb suction drain is placed within the wound. This is secured to the skin with a single stitch. The soft tissue is closed in layers using 4.0 vicryl for the playsmal/fascial layers and 5.0 vicryl for the deep dermal layer. Skin is reapproximated using absorbable suture in a subcuticular fashion to avoid later stitch removal. A compression dressing is applied to the face and the patient is awoken from anesthesia. The patient is monitored overnight and the drain is removed the following day at the surgeon’s discretion. Activity is restricted for 1 week postoperatively.
Complications Hematoma and seroma formation, wound site infection, and wound dehiscence are complications of any surgical excision. Rate of injury to the facial nerve is high, even with careful dissection and facial nerve monitoring, and temporary palsy occurs in 18% of cases.8 To achieve complete excision, the surgeon should also be prepared to address the external canal and resect skin and cartilage.18 First bite and Frey’s syndrome are rare complications of parotidectomy that caregivers should be counseled about pre-operatively.
Conclusion First branchial cleft anomalies are rare, and their diagnosis is often delayed. Treatment is complicated by both the challenging and variable anatomic relationship to the facial nerve and the fact that most are mischaracterized and treated inappropriately with incision and drainage prior to formal excision. A high level of suspicion for first branchial cleft anomalies in patients with sinus tracts in the affected region or otorrhea without middle ear disease is necessary to expedite diagnosis, avoid inappropriate surgical management, and safely complete surgical excision with facial nerve identification and preservation.
REFERENCES 1.
Arnot RS. Defects of the first branchial cleft. S Afr J Surg. 1971;9(2):93-98.
2.
Work WP. Newer concepts of first branchial cleft defects. Laryngoscope. 1972;82(9):1581-1593.
3.
Olsen KD, Maragos NE, Weiland LH. First branchial cleft anomalies. Laryngoscope. 1980;90(3):423-436.
4.
Mounsey RA, Forte V, Friedberg J. First brachial cleft sinuses: an analysis of current management strategies and treatment outcomes. J Otolaryngol. 1993;22(6):457-461.
5.
Choi SS, Zalzal GH. Branchial anomalies: a review of 52 cases. Laryngoscope. 1995;105(9 Pt 1):909-913.
6.
Finn DG, Buchalter IH, Sarti E, Romo T, Chodosh P. First branchial cleft cysts: clinical update. Laryngoscope. 1987;97(2):136-140.
7.
Triglia JM, Nicollas R, Ducroz V, Koltai PJ, Garabedian EN. First branchial cleft anomalies: a study of 39 cases and a review of the literature. Arch Otolaryngol Head Neck Surg. 1998;124(3):291-295.
8.
D'Souza AR, Uppal HS, De R, Zeitoun H. Updating concepts of first branchial cleft defects: a literature review. Int J Pediatr Otorhinolaryngol. 2002;62(2):103-109.
9.
Arndal H, Bonding P. First branchial cleft anomaly. Clin Otolaryngol Allied Sci. 1996;21(3):203-207.
10.
Ford GR, Balakrishnan A, Evans JN, Bailey CM. Branchial cleft and pouch anomalies. J Laryngol Otol. 1992;106(2):137-143.
11.
Nicollas R, Guelfucci B, Roman S, Triglia JM. Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol. 2000;55(2):117-124.
12.
Sichel JY, Halperin D, Dano I, Dangoor E. Clinical update on type II first branchial cleft cysts. Laryngoscope. 1998;108(10):1524-1527.
13.
Hickey SA, Scott GA, Traub P. Defects of the first branchial cleft. J Laryngol Otol. 1994;108(3):240-243.
14.
Aronsohn RS, Batsakis JG, Rice DH, Work WP. Anomalies of the first branchial cleft. Arch Otolaryngol. 1976;102(12):737-740.
15.
Fordham MT, Lambert PR. First Branchial Cleft Anomalies. In: Kountakis SE, ed. Encyclopedia of Otolaryngology, Head and Neck Surgery. Berlin, Heidelberg: Springer Berlin Heidelberg; 2013:944-951.
16.
Schroeder JW, Jr., Mohyuddin N, Maddalozzo J. Branchial anomalies in the pediatric population. Otolaryngol Head Neck Surg. 2007;137(2):289-295.
17.
Whetstone J, Branstetter BFt, Hirsch BE. Fluoroscopic and CT fistulography of the first branchial cleft. AJNR Am J Neuroradiol. 2006;27(9):1817-1819.
18.
Stulner C, Chambers PA, Telfer MR, Corrigan AM. Management of first branchial cleft anomalies: report of two cases. Br J Oral Maxillofac Surg. 2001;39(1):30-33.
Figure Legends Figure 1. Nerves associated with the branchial arches in the embryo (A) and fetus (B). Note that cranial nerve V (the trigeminal nerve) is associated with the first branchial arch and therefore the muscles that it innervates (C) are the muscular derivatives of this arch. Bony and cartilaginous derivatives of the first branchial arch (D) include the maxillary process, zygoma, malleus (except manubrium), incus (except long process), sphenomandibular ligament, and Meckel’s cartilage. Figure 2. Triangle outlining the anatomic space where first branchial cleft anomalies occur. This space is defined by vertices at the external auditory canal, the mental symphysis, and the lateral aspect of the hyoid bone. Figure 3. Transcanal microscopic view of a myringeal web, a bridge of thickened tissue between the inferior canal and the umbo. This finding is highly suggestive of a first branchial cleft anomaly. Figure 4. Illustration depicting the differences between type 1 and 2 first branchial cleft anomalies.