Joshua Gurberg1*, John Paul Moxham2, and Jeffrey P Ludemann3
1The University of British Columbia, Division of Otolaryngology, Diamond Health Care Centre, 4th Floor, 2775 Laurel Street, Vancouver, British Columbia, Canada
2Children’s Hospital, Vancouver, British Columbia, Canada
3Children’s Hospital, Vancouver, British Columbia, Canada
Received: 10 April, 2017; Accepted: 04 May, 2017; Published: 06 May, 2017
Joshua Gurberg, University of British Columbia, Division of Otolaryngology, Diamond Health Care Centre, 4th Floor, 2775 Laurel Street, Vancouver, British Columbia, Canada, E-mail:
Gurberg J, Moxham JP, Ludemann JP (2017) First Branchial Cleft Anomalies: Managing Various Cervico Facial Anomalies and Otologic Malformations at a large Canadian Referral Centre. Arch Otolaryngol Rhinol 3(2): 046-50. DOI: 10.17352/2455-1759.000044
© 2017 Gurberg J, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Pediatric; Pediatrics; First Branchial Cleft Anomalies: Managing Various Cervico-Facial Anomalies and Otologic Malformations at a large Canadian Referral Centre
Background:Branchial cleft anomalies account for approximately 17% of pediatric neck masses and 30% of all congenital neck lesions; of these, less than 1% involve the first branchial cleft. We report several novel otologic malformations encountered in children with first branchial cleft anomalies; as well as our surgical techniques and outcomes.
Methods:We conducted a retrospective chart review of all patients consecutively referred to the senior authors at a tertiary-care Pediatric Otolaryngology clinic for first branchial cleft anomalies over a 14-year period. Patients underwent computerized tomography scan with contrast, otoscopy plus tympanic microsurgery, when indicated (29%), methylene blue mapping of the cutaneous opening, when present (57%), intra-operative facial nerve monitoring, and anterograde or retrograde facial nerve dissection, when necessary (86%).
Results:Seven patients presented with first branchial cleft anomalies, at an average age of 3.9 years. Five of seven patients (71%) presented with cervico-facial infection. Four of the anomalies were sinuses, 2 were cysts and 1 was a fistula. Two patients had a classic myringeal web. One patient had a large posterior intratympanic keratoma, partial myringeal duplication, and wax-pocket; while another patient had external auditory canal triplication and a small intratympanic keratoma. In 6 cases (86%) there was distortion of anatomical landmarks and retrograde facial nerve dissection was therefore employed. In one patient, no branches of the facial nerve were identified during excision. In two patients, re-anastomosis of a small distal branch of the facial nerve was performed. Five patients (71%) experienced transient facial nerve weakness. However, with a mean length of follow-up of 7.5 years, no permanent facial nerve weakness, recurrence, or hearing loss has been identified.
Conclusions:We present several novel otologic first branchial cleft findings, as well as the experience of a two-surgeon team using methylene blue mapping plus monitored and primarily retrograde facial nerve dissection. This approach appears relatively safe and effective in children with these highly variable anomalies.
FBCA: First branchial cleft anomaly; CT: Computed tomography
The branchial arches appear as 4 paired arches of tissue at 4 weeks gestational age. The branchial clefts are composed of ectoderm, the pouches are formed of endoderm, while the mesenchyme of the arch will form an artery, nerve, cartilaginous bar, and muscle. With regards to the first branchial arch, the specific structures that develop are the maxillary artery, trigeminal nerve, muscles of mastication, mylohyoid, anterior belly of digastric muscle, tensor tympani, tensor veli palatini, maleus, incus, and a portion of the mandible. The first branchial cleft forms the external auditory canal and the first branchial pouch forms the Eustachian tube, middle ear cavity, and mastoid air cells. Formation of these structures occurs early and rapidly in gestation; as such this process is predisposed to error and resultant congenital anomalies.
Branchial cleft malformations account for approximately 17% of neck masses and 30% of all congenital neck lesions in children, second only to thyroglossal duct cysts . First branchial cleft anomalies (FBCA) account for only 1% of all branchial cleft malformations; however, making them exceedingly rare . They tend to present as a cyst, sinus, or fistula occurring in a region known as Pochet’s triangle, which is bounded by the external auditory canal superiorly, the mental region anteriorly, and the hyoid bone inferiorly . Symptoms include drainage from a pit-like depression in this region, submandibular adenitis, a parotid mass, or otorrhea. 10% of lesions are also associated with an asymptomatic membranous attachment between the tympanic membrane and floor of the external auditory canal [2,3]. These lesions are not to be confused with pre-auricular sinuses, which by definition are superficial to the first branchial cleft (and were excluded from this study).
Historically, FBCA have been divided into type I and type II lesions by means of a histopathological classification system . Type I lesions are made up of ectoderm exclusively, while type II lesions contain ectoderm and mesoderm . Several authors have reported on the relationship of these types of lesions to the facial nerve, with type I tending to be superficial and type II tending to be deep to the nerve [1,3,4]. It has been recognized however that, in many cases, these anomalies do not abide to these classifications nor anatomical relationships .
Our objective was to describe our surgical approach and outcomes with these rare and challenging congenital lesions as well as report on several novel associated otologic malformations encountered in our clinical experience. We have also reviewed the relevant literature.
After obtaining IRB approval, the charts of all patients consecutively referred to a two-surgeon team over a 14-year period for FBCA were retrospectively reviewed. 7 patients were identified; 6 female and 1 male. The mean age at presentation was 3.9 years. 5 patients presented with active infection. No associated craniofacial anomalies or syndromes were identified and there was no pre-operative facial nerve dysfunction. Six patients were imaged with computed tomography scan (some after initial ultrasound) and one patient had an MRI due to parent refusal. Figures 1,2 are illustrative.