First Branchial Cleft Anomalies Otologic Manifestations and Treatment Outcomes

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Original Research—Otology and Neurotology First Branchial Cleft Anomalies: Otologic Manifestations and Treatment Outcomes Otolaryngology– Head and Neck Surgery 2015, Vol. 152(3) 506–512 Ó American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0194599814562773 http://otojournal.org Justin R. Shinn 1 , Patricia L. Purcell, MD 1 , David L. Horn, MD 1,2 , Kathleen C. Y. Sie, MD 1,2 , and Scott C. Manning, MD 1,2 Sponsorships or competing interests that may be relevant to content are dis- closed at the end of this article. Abstract Objective. This study describes the presentation of first bran- chial cleft anomalies and compares outcomes of first bran- chial cleft with other branchial cleft anomalies with attention to otologic findings. Study Design. Case series with chart review. Setting. Pediatric tertiary care facility. Methods. Surgical databases were queried to identify children with branchial cleft anomalies. Descriptive analysis defined sample characteristics. Risk estimates were calculated using Fisher’s exact test. Results. Queries identified 126 subjects: 27 (21.4%) had first branchial cleft anomalies, 80 (63.4%) had second, and 19 (15.1%) had third or fourth. Children with first anoma- lies often presented with otologic complications, including otorrhea (22.2%), otitis media (25.9%), and cholesteatoma (14.8%). Of 80 children with second branchial cleft anomalies, only 3 (3.8%) had otitis. Compared with chil- dren with second anomalies, children with first anomalies had a greater risk of requiring primary incision and drai- nage: 16 (59.3%) vs 2 (2.5%) (relative risk [RR], 3.5; 95% confidence interval [CI], 2.4-5; P \ .0001). They were more likely to have persistent disease after primary exci- sion: 7 (25.9%) vs 2 (2.5%) (RR, 3; 95% CI, 1.9-5; P = .0025). They were more likely to undergo additional sur- gery: 8 (29.6%) vs 3 (11.1%) (RR, 2.9; 95% CI, 1.8-4.7; P = .0025). Of 7 persistent first anomalies, 6 (85.7%) were medial to the facial nerve, and 4 (57.1%) required ear- specific surgery for management. Conclusions. Children with first branchial cleft anomalies often present with otologic complaints. They are at increased risk of persistent disease, particularly if anomalies lie medial to the facial nerve. They may require ear-specific surgery such as tympanoplasty. Keywords branchial cleft, otitis, congenital neck mass, pediatric otolaryngology Received August 22, 2014; revised November 10, 2014; accepted November 14, 2014. B ranchial cleft anomalies (BCAs) are the second most common type of congenital neck mass after thyro- glossal duct cyst. 1 Branchial cleft anomalies can result from duplication of the first branchial cleft, or ear canal, or from failure of fusion of the second, third, or fourth branchial arches. 2 Most anomalies occur as second branchial cleft sinuses and cysts. 3 Third or fourth branchial anomalies, also referred to as piriform sinus fistulae, are the least common, 4 while first branchial anomalies account for about 10% of cases. 5 Because first BCAs are rare, attempts to describe the characteristics and management of these anoma- lies have historically been limited. Previous case series have raised concerns regarding the frequency of misdiagnosis and complications with treatment of first BCAs. 6 Previous studies have often focused on complications related to injury of the facial nerve, which can be found in close association to these lesions. 7 In 1972, Work 8 proposed a classification system for first BCAs. Work type I lesions are cysts that lie superficial to the facial nerve in closer proximity to the pinna, while Work type II anomalies communicate with the external auditory canal (EAC) or tympanic membrane (TM) and often lie medial to the facial nerve. Olsen et al 9 then introduced a clas- sification of the defects into cysts, sinuses, and fistulas based on the number of surface openings present. The TM is considered a fusion point for the 3 primary vestigial layers of the first branchial apparatus; therefore, abnormal embryologic development in this region often leads to otologic abnormalities. 10 Considering this embryologic 1 Department of Otolaryngology, University of Washington, Seattle, Washington, USA 2 Division of Pediatric Otolaryngology, Seattle Children’s Hospital, Seattle, Washington, USA This article was presented at the 2014 AAO-HNSF Annual Meeting & OTO EXPO SM ; September 21-24, 2014; Orlando, Florida. Corresponding Author: Patricia L. Purcell, Department of Otolaryngology, University of Washington, 1959 NE Pacific St, Box 356515, Seattle, WA 98195-6515, USA. Email: [email protected] at IMSS on May 26, 2015 oto.sagepub.com Downloaded from

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MALFORMACIONES OTOLOGICAS

Transcript of First Branchial Cleft Anomalies Otologic Manifestations and Treatment Outcomes

  • Original ResearchOtology and Neurotology

    First Branchial Cleft Anomalies: OtologicManifestations and Treatment Outcomes

    OtolaryngologyHead and Neck Surgery2015, Vol. 152(3) 506512 American Academy ofOtolaryngologyHead and NeckSurgery Foundation 2014Reprints and permission:sagepub.com/journalsPermissions.navDOI: 10.1177/0194599814562773http://otojournal.org

    Justin R. Shinn1, Patricia L. Purcell, MD1, David L. Horn, MD1,2,Kathleen C. Y. Sie, MD1,2, and Scott C. Manning, MD1,2

    Sponsorships or competing interests that may be relevant to content are dis-

    closed at the end of this article.

    Abstract

    Objective. This study describes the presentation of first bran-chial cleft anomalies and compares outcomes of first bran-chial cleft with other branchial cleft anomalies withattention to otologic findings.

    Study Design. Case series with chart review.

    Setting. Pediatric tertiary care facility.

    Methods. Surgical databases were queried to identify childrenwith branchial cleft anomalies. Descriptive analysis definedsample characteristics. Risk estimates were calculated usingFishers exact test.

    Results. Queries identified 126 subjects: 27 (21.4%) hadfirst branchial cleft anomalies, 80 (63.4%) had second, and19 (15.1%) had third or fourth. Children with first anoma-lies often presented with otologic complications, includingotorrhea (22.2%), otitis media (25.9%), and cholesteatoma(14.8%). Of 80 children with second branchial cleftanomalies, only 3 (3.8%) had otitis. Compared with chil-dren with second anomalies, children with first anomalieshad a greater risk of requiring primary incision and drai-nage: 16 (59.3%) vs 2 (2.5%) (relative risk [RR], 3.5; 95%confidence interval [CI], 2.4-5; P \ .0001). They weremore likely to have persistent disease after primary exci-sion: 7 (25.9%) vs 2 (2.5%) (RR, 3; 95% CI, 1.9-5; P =.0025). They were more likely to undergo additional sur-gery: 8 (29.6%) vs 3 (11.1%) (RR, 2.9; 95% CI, 1.8-4.7; P =.0025). Of 7 persistent first anomalies, 6 (85.7%) weremedial to the facial nerve, and 4 (57.1%) required ear-specific surgery for management.

    Conclusions. Children with first branchial cleft anomalies oftenpresent with otologic complaints. They are at increased risk ofpersistent disease, particularly if anomalies lie medial to thefacial nerve. They may require ear-specific surgery such astympanoplasty.

    Keywords

    branchial cleft, otitis, congenital neck mass, pediatricotolaryngology

    Received August 22, 2014; revised November 10, 2014; accepted

    November 14, 2014.

    Branchial cleft anomalies (BCAs) are the second most

    common type of congenital neck mass after thyro-

    glossal duct cyst.1 Branchial cleft anomalies can

    result from duplication of the first branchial cleft, or ear

    canal, or from failure of fusion of the second, third, or fourth

    branchial arches.2 Most anomalies occur as second branchial

    cleft sinuses and cysts.3 Third or fourth branchial anomalies,

    also referred to as piriform sinus fistulae, are the least

    common,4 while first branchial anomalies account for about

    10% of cases.5 Because first BCAs are rare, attempts to

    describe the characteristics and management of these anoma-

    lies have historically been limited. Previous case series have

    raised concerns regarding the frequency of misdiagnosis and

    complications with treatment of first BCAs.6 Previous studies

    have often focused on complications related to injury of the

    facial nerve, which can be found in close association to these

    lesions.7

    In 1972, Work8 proposed a classification system for first

    BCAs. Work type I lesions are cysts that lie superficial to the

    facial nerve in closer proximity to the pinna, while Work

    type II anomalies communicate with the external auditory

    canal (EAC) or tympanic membrane (TM) and often lie

    medial to the facial nerve. Olsen et al9 then introduced a clas-

    sification of the defects into cysts, sinuses, and fistulas based

    on the number of surface openings present.

    The TM is considered a fusion point for the 3 primary

    vestigial layers of the first branchial apparatus; therefore,

    abnormal embryologic development in this region often

    leads to otologic abnormalities.10 Considering this embryologic

    1Department of Otolaryngology, University of Washington, Seattle,

    Washington, USA2Division of Pediatric Otolaryngology, Seattle Childrens Hospital, Seattle,

    Washington, USA

    This article was presented at the 2014 AAO-HNSF Annual Meeting & OTO

    EXPOSM; September 21-24, 2014; Orlando, Florida.

    Corresponding Author:

    Patricia L. Purcell, Department of Otolaryngology, University of

    Washington, 1959 NE Pacific St, Box 356515, Seattle, WA 98195-6515,

    USA.

    Email: [email protected]

    at IMSS on May 26, 2015oto.sagepub.comDownloaded from

  • source, the primary objective of the current study was to

    describe the presentation of first BCAs at our institution,

    with attention to otologic manifestations of disease. A

    second objective was to compare treatment outcomes of

    children with first BCAs to children with other types of

    BCAs to determine the extent to which children with first

    anomalies are at greater risk of delayed diagnosis or per-

    sistent disease. Our hypothesis was that children with per-

    sistent disease would often require ear-specific surgery

    for definitive management.

    Methods

    This investigation is a case series that received institutional

    review board approval from Seattle Childrens Hospital, a

    pediatric tertiary care facility. All children and adolescents

    who were surgically treated for congenital neck masses between

    2004 and 2013 were identified through query of the facilitys

    surgical database using the following Current Procedural

    Terminology (CPT) codes: 21556, 21557, 38510, 42408, 42815,

    60280, and 60281.

    A retrospective chart review was performed to identify

    subjects from age birth to 21 years who were treated for

    BCA. Diagnosis of branchial anomaly was made on the

    basis of clinical evaluation, imaging characteristics, and sur-

    gical pathology.

    First BCAs were defined by a pit or cyst identified in

    level I or level II or the postauricular region with imaging

    and surgical confirmation of a tract leading toward the ear

    canal. Second branchial cleft sinuses and fistulae were

    defined by a congenital pit along the anterior border of the

    sternocleidomastoid muscle with surgical confirmation of a

    tract heading partially or completely to the tonsillar fossa.

    Second branchial cleft cysts were defined by an isolated

    level II cyst lined by ciliated columnar epithelium. Third or

    fourth branchial anomalies were defined by level III or level

    IV neck inflammation, thyroid involvement, and/or a pit in

    the ipsilateral piriform sinus. In all cases, diagnosis of bran-

    chial cleft anomaly was confirmed by review of final

    pathology reports.

    Data were collected regarding demographic characteris-

    tics, presenting signs and symptoms, procedures performed,

    and treatment outcomes. Clinical records of children with

    first and second BCAs were reviewed with attention to oto-

    logic manifestations of disease.

    Statistical Analysis

    Univariate analyses were carried out to obtain descriptive

    statistics such as means, confidence intervals, and frequen-

    cies for both groups. To make comparisons between the

    groups, we performed inferential testing using one-way

    analysis of variance. Risk estimates were obtained through

    calculation of risk ratios using Fishers exact test. P \ .05was considered statistically significant. Stata 13.1 (StataCorp,

    College Station, Texas) statistical software was used for all

    analyses.

    Results

    Review identified 126 patients with BCAs. Of these, 27

    (21.4%) had first BCAs, 80 (63.4%) had second BCAs, and

    19 (15.1%) had third or fourth branchial anomalies.

    Clinical characteristics and presenting symptoms of the sub-

    jects are summarized in Table 1. Subjects with first BCAswere more likely to be female (63%) and to have left-sided

    lesions (63%). Patients with second BCAs were more likely to

    have right-sided lesions (63.7%), while patients with third or

    fourth BCAs had predominantly left-sided lesions (89.5%).

    While most children were correctly diagnosed with BCA,

    chart review also identified a subgroup of patients who did

    not have BCA but rather the more commonly encountered

    entity of preauricular pits. These children most commonly

    presented with infected pits or abscesses anterior to the

    tragus or helical root; these lesions were identified on the

    right, left, or bilaterally nearly in equal numbers (5 bilater-

    ally, 4 on the left, and 6 on the right). Findings during surgi-

    cal excision included a pit, mass, or skin tag anterior to the

    tragus or helical root in all cases.

    Children with first and second BCAs had similar age at

    diagnosis, 2.08 years versus 1.51 years (P = .4, not signifi-

    cant). In contrast, children with third or fourth BCAs were

    significantly older with a mean age of 6.37 years (P \.001). Children with first BCAs most commonly presented

    with otologic complaints (40.7%), while those with second

    BCAs were most likely to present with draining sinus or fis-

    tulae (62.5%). All children with third or fourth BCAs pre-

    sented with neck mass or abscess.

    Table 2 lists otologic examination findings of the sub-jects. Although a small number of patients with second

    BCAs had otitis media or EAC deformity, an otologic prob-

    lem was not given as the primary complaint for any of these

    Table 1. Characteristics of Different Types of Branchial Cleft Anomalies.

    Laterality, No. (%) Presenting Symptom, No. (%)

    Type of

    Anomaly No.

    Age at Diagnosis,

    Mean (95% CI), y

    Female Sex,

    No. (%) Left Right Bilateral

    Draining

    Pit

    Neck

    Abscess

    Otologic

    Problem

    First 27 2.08 (1.07-3.08) 17 (63) 17 (63) 10 (37) 0 6 (22.2) 10 (37) 11 (40.7)

    Second 80 1.51 (0.67-2.36) 34 (42.5) 24 (30) 51 (63.7) 5 (6.3) 50 (62.5) 30 (37.5) 0

    Third 19 6.37 (4.12-8.62) 10 (52.6) 17 (89.5) 2 (10.5) 0 0 19 (100) 0

    Abbreviation: CI, confidence interval.

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  • patients. In contrast, patients with first BCAs had a variety of

    otologic complications, ranging from EAC cyst to cholestea-

    toma. Even if not the presenting complaint, the presence of

    otitis media was noted from clinical examination records.

    Seven of 27 children with first BCAs and 3 of 80 children

    with second BCAs were noted to have otitis media (relative

    risk [RR], 2.8; 95% confidence interval [CI], 1.6-4.7; P =

    .006). Among the 7 children with first BCAs who had otitis, 4

    had a history of acute otitis media, while 3 had a history of

    chronic otitis media with effusion. None had TM perforation.

    An EAC web, or abnormal connection between the

    anterior-inferior or posterior-inferior canal wall and TM,

    can signal the presence of a first branchial cleft anomaly; 6

    of our subjects (22.2%) were discovered to have such a web

    on evaluation. In addition, children with first BCAs dis-

    played a range of other EAC deformities: 5 (18.5%) had an

    EAC mass or cyst, while 5 (18.5%) had EAC duplication.

    In comparison, 3 (3%) of the children with second BCAs

    displayed EAC deformities, which were associated with

    hemifacial microsomia or Down syndrome.

    Table 3 contains a comparison of outcomes amongpatients with first and second BCAs. Compared with chil-

    dren with second BCAs, children with first BCAs had a

    greater incidence of primary incision and drainage: 16

    (59.3%) vs 2 (2.5%) (RR, 3.5; 95% CI, 2.4-5; P\ .0001).

    Of the 16 children with first BCAs who underwent primary

    incision and drainage, 11 presented with an acute abscess,

    while 5 children presented with a draining pit. After resolu-

    tion of their acute infection, the children underwent primary

    excision. Compared with children with second BCAs, chil-

    dren with first BCAs were more likely to have persistent

    disease following primary excision: 7 (25.9%) vs 2 (3.8%)

    (RR, 3; 95% CI, 1.9-5; P = .0025). They were also more

    likely to undergo additional surgery: 8 (29.6%) vs 3

    (11.1%) (RR, 2.9; 95% CI, 1.8-4.7; P = .0025). There was

    not a significant difference in rates of persistent disease

    between children with first and children with third or fourth

    BCAs. Of the 19 children with third or fourth BCAs, 11

    (57.9%) underwent incision and drainage prior to excision,

    7 (36.8%) experienced persistent disease, and 7 (36.8%)

    required additional surgery.

    Length of follow-up period was not significantly differ-

    ent among the anomaly types. Children with first BCAs had

    a mean follow-up period of 16 months (95% CI, 6-27

    months), children with second BCAs had a mean follow-up

    length of 8 months (95% CI, 4-12 months), and those with

    third or fourth anomalies had a follow-up of 11 months

    (95% CI, 1-21 months).

    Table 4 describes the surgical management of the 27patients with first BCAs. Thirteen subjects (48.1%) underwent

    Table 3. Comparison of Surgical Outcomes among First and Second Branchial Cleft Anomalies.

    Type of Anomaly Primary I&D Persistent Disease Multiple Surgeriesa (Excluding Primary I&D)

    First, No. (%) 16 (59.3) 7 (25.9) 8 (29.6)

    Second, No. (%) 2 (2.5) 2 (2.5) 3 (11.1)

    Relative risk (95% CI) 3.5 (2.4-5) 3 (1.9-5) 2.9 (1.8-4.7)

    P value \.0001 .0025 .0025

    Abbreviations: CI, confidence interval; incision and drainage.aMultiple surgeries: includes both repeat excision and treatment of complication.

    Table 4. Surgical Management of Patients with First Branchial Cleft Anomalies.

    Procedure(s) Performed, No. (%)

    Local Excision Superficial Parotidectomy Meatoplasty/Tympanomastoidectomy

    Primary excision (n = 27) 13 (48.1) 13 (48.1) 5 (18.5)

    Repeat excision (n = 7) 1 (14.3) 2 (28.6) 4 (57.1)

    Table 2. Otologic Examination Findings of Patients with First and Second Branchial Cleft Anomalies.

    Type of

    Anomaly

    Otorrhea,

    No. (%)

    Acute Otitis

    Media, No. (%)

    Chronic Otitis

    Media, No. (%)

    Cholesteatoma,

    No. (%)

    EAC Web,

    No. (%)

    Other EAC

    Deformity, No. (%)

    First 6 (22.2) 4 (14.8) 3 (11.1) 4 (14.8) 6 (22.2) 13 (48.1)

    Second 0 2 (2.5) 1 (1.3) 0 0 3 (3)

    Abbreviation: EAC, external auditory canal.

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  • superficial parotidectomy on primary excision, with 4 of these

    patients also undergoing tympanoplasty or tympanomastoidect-

    omy. One patient underwent tympanomastoidectomy alone

    after the identification of an EAC duplication, a type of first

    branchial cleft anomaly. The duplication was located superfi-

    cial to the facial nerve. The remainder of children underwent

    simple excision at the time of primary surgery. Among the 7

    patients who required revision surgery, more than half (57.1%)

    underwent otologic surgery, either tympanoplasty or tympano-

    mastoidectomy. There were 9 patients (33.3%) who had Work

    type II anomalies that coursed medial to the facial nerve; all

    eventually required parotidectomy either at primary or repeat

    excision. Of the 7 patients who required additional surgery, 6

    (85.7%) had anomalies medial to the facial nerve.

    Facial nerve complications were also evaluated among

    the patients with first BCAs. Only 1 patient (3.7%) had con-

    firmation of permanent facial nerve weakness, an adolescent

    who presented to our facility with facial nerve palsy after

    undergoing multiple procedures at outside facilities. In addi-

    tion, 3 patients (11.1%) developed temporary weakness of

    the marginal mandibular nerve that completely resolved,

    while 2 (7.4%) patients experienced weakness of unknown

    duration due to loss to follow-up. Among the 5 cases that

    occurred at our institution, the first branchial cleft anomaly

    was identified medial to the facial nerve during the excision

    procedure.

    Discussion

    Children with first BCAs often undergo multiple procedures

    to treat infectious complications and ultimately remove

    these lesions. In the current study, more than half of the

    children were treated with incision and drainage prior to

    excision, and over a quarter required repeated excision pro-

    cedures. Risk of persistent disease and repeat surgery were

    much higher for these children than for children with

    second BCAs. This indicates that the diagnosis of first BCA

    may not be recognized until the anomaly is infected and

    requires incision and drainage. Interestingly, our findings

    suggest that children with third or fourth branchial anoma-

    lies also have high rates of persistent disease, which is con-

    sistent with previously published literature11 and should be

    explored further in future investigations.

    Careful otologic evaluation can assist providers in

    making correct diagnosis of first BCAs.12 Previous case

    series have described otologic complications, including cho-

    lesteatoma13; however, we were surprised by the high fre-

    quency of children who presented with a primary otologic

    complaint. Otologic complications were the most frequent

    presenting symptoms, occurring in nearly half of the cases.

    Otorrhea and otitis media were identified in about one-

    fourth of the cases, and 15% of patients were found to have

    cholesteatoma. Seven children with first BCAs had otitis

    media, of whom 4 had a history of acute otitis media and 3

    had a history of chronic otitis media with effusion. None of

    these children had tympanic membrane perforation. In addi-

    tion, more than half of the patients were noted to have an

    EAC anatomical deformity.

    Perhaps the most complicated case encountered was a 2-

    year-old male who presented with a left level II neck abscess

    and purulent otorrhea. The patient was ultimately diagnosed

    with both Work type I and Work type II anomalies. During

    the childs initial incision and drainage procedure, pressure

    on the neck abscess resulted in copious expression of pus

    from the ear canal (Figure 1), confirming the diagnosis of aWork type II anomaly. After antibiotic therapy, the patient

    had persistent erythema and drainage at the original incision

    site and in the postlobular crease (Figure 2). Otoscopyrevealed both anterior-inferior and posterior-inferior TM

    webs along with some persistent granulation at the anterior

    inferior sulcus (Figure 3). The 2 first branchial anomalieswere then excised via a superficial parotidectomy approach

    with facial nerve dissection (Figure 4). The proximal cartila-ginous portion of the main fistula, which ran deep to the

    facial nerve, was shaved off at the bony cartilaginous junc-

    tion of the anterior ear canal. The patient experienced con-

    tinued wound infection at the incision area directly inferior

    to the lobule. Cultures grew Pseudomonas, indicating a

    likely continued connection to the ear canal or middle ear.

    Otoscopy demonstrated a persistent area of granulation at

    the point of origin of the anterior web at the anterior

    sulcus. A tympanoplasty was performed with excision of

    the anterior-inferior TM, curettage of the pit at the anterior

    sulcus, and reconstruction with fascia. The infection

    resolved, and the ear canal and tympanic membrane healed

    (Figure 5). This is a rare case of 2 first branchial anoma-lies in 1 patient and emphasizes the point that Work type

    II fistulae can present at a young age with a sinus tract

    running deep to the facial nerve. Failure to adequately

    address the otologic component of the lesion can result in

    persistent infection, as was the case for this patient.

    The approach to a child with a first branchial cleft anom-

    aly lies in accurate diagnosis; Figure 6 contains a potentialalgorithm for management. First anomalies should be among

    Figure 1. Left level II cervical abscess. Application of pressure pro-duces otorrhea.

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  • the differential diagnoses considered when children present

    with a pit or inflammatory response in neck level I, high in

    level IIa/IIb, on the face, or in the postauricular area. A thor-

    ough otoscopic examination is indicated, with the use of

    binocular microscopy if available. The examiner should look

    specifically for a canal web, granulation tissue, or middle ear

    disease. Evidence of purulent expression from the ear with

    simultaneous pressure on the neck or infected lesion indicates

    a communication between the lesion and the EAC. Imaging,

    either with contrast computed tomography (CT) or magnetic

    resonance imaging (MRI), can help clarify the diagnosis. If a

    child presents with acute infection, incision and drainage and

    antibiotics are indicated prior to definitive excision.

    When deciding the optimal approach for primary exci-

    sion, patients who present with a pit or abscess anterior to

    the angle of mandible, at cervical level I or II, are more

    likely to have an anomaly deep or medial to the facial nerve

    (Work type II). In our study, all of these patients required

    parotidectomy, and many required otologic surgery. If there

    is any suspicion of Work type II anomaly, then imaging

    should be performed to assist with operative planning and

    to determine where the tract lies in relation to the facial

    nerve. A parotidectomy approach with preliminary identifi-

    cation of the facial nerve and excision of the entire tract is

    indicated. Concurrent tympanoplasty or canalplasty should

    be performed if a connection between the superficial skin

    and EAC or TM is seen or if a cyst involves the external

    ear. Facial nerve monitoring should be considered in all

    cases. In the current study, all patients with facial nerve

    weakness had a Work type II anomaly medial to the nerve.

    If the child has a Work type I anomaly, indicated by a

    pit or cyst around the lobule, then imaging may not be

    Figure 4. Parotidectomy approach. Ruptured abscess and earcanal duplication (*) are delivered from a location medial to thefacial nerve trunk, which is elongated. The anomaly is then excisedfrom the anterior ear canal.

    Figure 5. Otoscopy after tympanoplasty showing healing of ante-rior ear drum and ear canal. Posterior web remains. Umbo indi-cated by (*).

    Figure 3. Otoscopy showing anterior inferior granulation (*) andanterior (#) and posterior ($) webs.

    Figure 2. Left level II incision and drainage site as a sign of Worktype II anomaly; persistent postlobule infection as evidence ofWork type I anomaly.

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  • required, although preferences will likely be institution spe-

    cific. Surgical excision of the tract, superficial to the facial

    nerve, is performed with a mini-facelift incision behind

    the tragus and around the lobule with facial nerve monitor-

    ing. If there is a visible connection or cyst on the tympanic

    membrane or external auditory canal, then concurrent tym-

    panoplasty or canalplasty should be performed.

    Children with preauricular pits may be misdiagnosed as

    having a first BCA; however, we found these children to

    always present with a lesion near the helical root. These

    children most commonly presented with infected pits or

    abscesses and were identified on the right, left, or bilaterally

    nearly in equal numbers. Preauricular pits are a distinct

    diagnosis and should not be confused with BCA. They

    occur anterior to the tragus or helical root, often become

    infected, and do not involve the facial nerve. Imaging prior

    to surgical excision is often not necessary.

    As a retrospective study, this investigation has a number

    of limitations, including incomplete records, loss to follow-

    up, and potential misclassification of anomaly type.

    However, the large difference in persistent disease between

    first and second BCAs strongly suggests that there is a need

    for improvement in surgical management of first BCAs.

    Misdiagnosis and inadequate treatment of first BCAs

    have the potential to result in recurrent infection, scarring

    due to multiple procedures, and facial nerve injury. For chil-

    dren with chronic or recurrent upper neck infections,

    especially in the setting of ipsilateral ear disease, providers

    should strongly consider the possibility that the subject may

    have a first branchial cleft anomaly. Children with anoma-

    lies that occur medial to the nerve are especially at risk for

    persistent disease. Preoperative imaging should be per-

    formed to define this relationship, so that families can be

    counseled appropriately. If otologic involvement is sus-

    pected, then it should be addressed with otologic surgery at

    the time of primary excision if possible.

    Conclusion

    First BCAs are associated with a wide range of otologic

    manifestations. These children, particularly those with Work

    type II lesions medial to the facial nerve, experience a rela-

    tively high frequency of persistent disease, and otologic sur-

    gery may be required for definitive surgical management.

    Acknowledgment

    We thank Rose Jones-Goodrich, clinical research associate, for

    administrative support of this project.

    Author Contributions

    Justin R. Shinn, study design, data collection, manuscript prepara-

    tion; Patricia L. Purcell, study design, data analysis, manuscript

    revision; David L. Horn, data collection, manuscript revision;

    Kathleen C. Y. Sie, data collection, manuscript revision; Scott C.

    Figure 6. Algorithm for management of first branchial cleft anomalies. BCA, branchial cleft anomaly; CT, computed tomography; MRI, mag-netic resonance imaging.

    Shinn et al 511

    at IMSS on May 26, 2015oto.sagepub.comDownloaded from

  • Manning, study conceptualization, study design, data collection,

    manuscript preparation and revision.

    Disclosures

    Competing interests: None.

    Sponsorships: None.

    Funding source: Patricia L. Purcell is completing a research fel-

    lowship supported by grant 2T32DC000018, the Institutional

    National Research Service Award for Research Training in

    Otolaryngology from the National Institute on Deafness and Other

    Communication Disorders.

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