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International Journal of Pediatric Otorhinolaryngology 75 (2011) 1020–1023

Contents lists available at ScienceDirect

International Journal of Pediatric Otorhinolaryngology

journ al hom epage: www.elsevier.com/locat e/ijpo r l

Branchial anomalies in children

Y. Bajaj , S. Ifeacho, D. Tweedie, C.G. Jephson, D.M. Albert, L.A. Cochrane, M.E. Wyatt,

N. Jonas, B.E.J. Hartley

Department of Otolaryngology, Great Ormond Street Hospital, Great Ormond Street, London LS17 7WA, United Kingdom

A B S T R A C T

Background: Branchial cleft anomalies are the second most common head and neck congenital lesions

seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95% of the

branchial anomalies. This article analyzes all the cases of branchial cleft anomalies operated on at Great

Ormond Street Hospital over the past 10 years.

Methods: All children who underwent surgery for branchial cleft sinus or fistula from January 2000 to

December 2010 were included in this study.

Results: In this series, we had 80 patients (38 female and 42 male). The age at the time of operation varied

from 1 year to 14 years. Amongst this group, 15 patients had first branchial cleft anomaly, 62 had second

branchial cleft anomaly and 3 had fourth branchial pouch anomaly. All the first cleft cases were operated

on by a superficial parotidectomy approach with facial nerve identification. Complete excision was

achieved in all these first cleft cases. In this series of first cleft anomalies, we had one complication

(temporary marginal mandibular nerve weakness. In the 62 children with second branchial cleft

anomalies, 50 were unilateral and 12 were bilateral. In the vast majority, the tract extended through the

carotid bifurcation and extended up to pharyngeal constrictor muscles. Majority of these cases were

operated on through an elliptical incision around the external opening. Complete excision was achieved

in all second cleft cases except one who required a repeat excision. In this subgroup, we had two

complications one patient developed a seroma and one had incomplete excision. The three patients with

fourth pouch anomaly were treated with endoscopic assisted monopolar diathermy to the sinus opening

with good outcome.

Conclusion: Branchial anomalies are relatively common in children. There are three distinct types, first

cleft, second cleft and fourth pouch anomaly. Correct diagnosis is essential to avoid inadequate surgery

and multiple procedures. The surgical approach needs to be tailored to the type of anomaly of origin of

the anomaly. Complete excision is essential for good outcomes.

ß 2011 Elsevier Ireland Ltd. All rights reserved.

1. Introduction

Branchial cleft anomalies are the second most common head

and neck congenital lesions seen in children. They present as cysts,

fistulas, sinuses or cartilaginous remnants. The term branchial cyst

was first used by Ascherson in 1832(1). By the 4th week of

embryonic life, six branchial arches are recognisable. Five pairs of

ectodermal clefts (grooves) and five endodermal branchial

pouches separate these six arches, with a membrane located at

the interface between the pouch and the cleft [1].

Various theories proposed for the development of these

anomalies are branchial apparatus theory, cervical sinus theory,

thymopharyngeal theory and inclusion theory [2]. The most widely

* Corresponding author. Tel.: +44 7769686449; fax: +44 1132663305.

E-mail address: ybajaj@hotmail.co.uk (Y. Bajaj).

0165-5876/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved.

doi:10.1016/j.ijporl.2011.05.008

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Y. Bajaj et al. / International Journal of Pediatric Otorhinolaryngology 75 (2011) 1020–1023 1021

Fig. 3. Fourth arch sinus opening in left pyriform fossa.

upper division of the facial trunk. All these cases had macroscopic

evidence of skin or cartilage in the lining of the tract. Four of the

cases were completely duplicated ear canals with circumferential

cartilage lined with hair bearing skin. Complete excision was

achieved in all these cases. The majority of patients (12/15) stayed

in the hospital for one night and the other three for two nights. In

this series we had one complication (temporary marginal

mandibular nerve weakness). All these patients were followed

up in the clinic for at least 6 months post-operatively. Good

outcome was achieved in all these cases, and there were no

recurrences.

In the 62 children (30 male and 32 female) with second

branchial cleft anomalies, 50 were unilateral (34 right and 16 left)

and 12 were bilateral. The extent of the tract was variable in these

74 sinus/fistula tracts. In 6/74, the tract was short and extended

just up to sternomastoid muscle. In 14/74, the tract stopped short

of the carotid artery. In the vast majority (50/74), the tract

extended through the carotid bifurcation and extended up to

pharyngeal constrictor muscles in 44/74 and into tonsil fossa in 7/

74 cases. The majority of these cases (55/62) were operated on via

an elliptical incision around the external fistula opening under

general anaesthetic. Seven patients underwent a stepladder

incision (second separate horizontal incision) to excise the tract

completely. Complete excision was achieved in all cases except one

who required a repeat excision. A drain was inserted in only 13/62

cases. The majority of the patients stayed in the hospital less than

24 h (Daycase – 11, Overnight – 38, two nights – 11, three nights –

2). In this subgroup, we had two complications (3.2%) – one patient

developed a seroma and one had incomplete excision. Patients

were reviewed in the clinic 6 weeks after the excision. Good

outcome was defined as complete excision of the disease and no

recurrence. This was achieved in all these patients (Fig. 2).

In this series, we had three patients with fourth branchial pouch

anomaly. All of them had a sinus opening in the left pyriform fossa

(Fig. 3), which was confirmed on microlaryngobronchoscopy

examination. They were all operated on less than 1 year of age.

These patients had monopolar diathermy applied endoscopically

to the sinus opening using a suspension laryngoscope and a

diathermy needle. They were observed in the hospital overnight. A

repeat laryngoscopy was performed 6 weeks later to confirm that

the sinus opening had sealed off. They were followed up in the

clinic and a good outcome was achieved in all these patients.

4. Discussion

Various terms have been used in the literature to describe

branchial anomalies. There are some important definitions which

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1022 Y. Bajaj et al. / International Journal of Pediatric Otorhinolaryngology 75 (2011) 1020–1023

need to be clarified. A sinus is a blind ending tract. Sinuses arising are useful investigations to confirm the diagnosis and define the

from the branchial apparatus may connect with either the skin or extent of the lesion. The radiological appearances of branchial

the pharynx. Those connecting to skin are termed branchial cleft anomalies have been reported [14]. The scan may also help to

sinuses and those connecting with the pharynx are termed delineate the sinus tract or a deep seated cyst associated with the

branchial pouch sinuses [6]. Fistula, by definition is a communica- sinus tract. A scan can also be useful in evaluating the relation of

tion between two epitheliazed surfaces. A congenital branchial facial nerve to the first cleft anomaly. In the cases of suspected

fistula would require a communication between a persistent pouch fourth pouch anomalies, a sinogram or barium contrast study can

and a cleft. A pseudo-fistula may form between a pouch internally be useful [10]. In this series, majority of the cases had a scan done

and an external opening produced secondary to an infection or pre-operatively. All the first cleft anomalies underwent MRI scan.

iatrogenic. Surgical excision is the definitive treatment for branchial

Branchial anomalies can be divided into first cleft, second cleft, anomalies. These lesions do not regress spontaneously and have a

third and fourth pouch anomaly. The embryonic origin of these high rate of recurrent infections [15]. The reported recurrence rate

lesions ultimately dictates their presentation, diagnosis and after primary excision is 3%, but can be 14–22% after surgical

correct surgical treatment. Each branchial arch, poch and cleft excision after previous infection or previous incomplete excision

complex develops into specific structures in the head and neck [16]. Recurrences are usually seen with first cleft, third and fourth

region. The branchial anomaly and its associated tract lies inferior pouch anomalies [16]. The aim should be to achieve complete

to all the derivatives of its associated arch and superior to all surgical excision, preserving the normal structures.

derivatives of the next arch [7]. The ideal treatment for first cleft anomalies is by a superficial

The first cleft anomalies have been classified by Work into two parotidectomy approach with facial nerve exposure. All the cases

types on the basis of clinical features and histopathology [8]. Type I in this series were managed by this approach, with facial nerve

anomalies are purely ectodermal and present as a cystic mass. monitoring. The external appearance suggests a minor abnormali-

Histologically these have squamous epithelium, but no skin ty, however very significant cutaneous tract exists. Clinicians may

adnexa or cartilage. Type II anomalies present as a cyst, sinus or therefore easily underestimate the extent of the problem. Facial

fistula tract and are of ectodermal and mesodermal origin. These nerve injury is a major risk factor in the excision, especially so if the

on histology have squamous epithelium with skin adnexa or patient has a past history of multiple infections or incomplete

cartilage. surgical excisions [17]. Facial nerve injury was reported in 6 of the

The second branchial cleft anomalies are identified along the 39 cases by Triglia et al. [18] and 2 of the 5 patients by Ford et al.

anterior border of the sternomastoid muscle. The extent of the [19]. In this series we had temporary marginal mandibular

sinus or the location of the cyst can be anywhere along the course weakness in 1 out of 15 patients.

of the second branchial fistula, which proceeds from the skin of Second cleft sinuses/fistulas also need to be completely

neck, between the internal and external carotid arteries and into excised for good outcomes [7]. The usual approach is a

the palatine tonsil. The second branchial cleft sinuses/cysts are horizontal incision encompassing the external sinus opening

classified into four types by Bailey [9]. Type I is the most superficial and dissecting the sinus tract completely. Sometimes a second

and lies along the anterior surface of the sternomastoid muscle, incision is required for completing the excision. Similar

just deep to the platysma. Type II is the most common and is found approach was used in this series. Care must be taken to avoid

along the anterior surface of the sternomastoid, lateral to carotid injury to hypoglossal and vagus nerves [3]. In this series we had

space and posterior to submandibular gland. Type III extends good overall complications. We had two complications in the 62

medially between the bifurcation of the internal and external second arch patient subgroup- one had a seroma and an

carotid arteries to lateral pharyngeal wall. Type IV opens into the incomplete excision in another patient.

pharynx and is lined with columnar epithelium. Treatment of congenital fourth arch sinuses from historical

Most third and fourth pouch anomalies are sinuses and not times has been surgical, in the form of formal excision of the whole

congenital fistulae. A third branchial pouch sinus might originate sinus or fistula tract as well as thyroid lobectomy for thyroid

in the rostral end of pyriform fossa, would be most likely to pass involvement as curative treatment [20–23]. However due to the

cranial to superior laryngeal nerve before turning inferiorly anatomical course of the tract, complete resection remains

between the common carotid artery and the vagus nerve to end challenging [24,25]. Successful outcomes have been achieved

lateral to thyroid gland [10]. A fourth branchial pouch sinus would more recently by endoscopic techniques, which is reliable, quick

exit the hypopharynx, in a similar manner behind the thyroid ala, and simple with less post operative complications [26,27]. A

descending caudal to the superior laryngeal nerve and cranial to recent review of management of fourth branchial arch anomalies

the recurrent laryngeal nerve and terminating in the thyroid gland in children, recommended treatment with cauterisation rather

or parathracheal position [10]. Fourth branchial pouch anomalies than open neck surgery to reduce complications [22]. Recurrence

are extremely rare and constitute less than 3% of all branchial rates also were similar with both procedures. All three cases with

anomalies [11]. The consistent feature of these fourth branchial fourth pouch anomaly were treated with cauterisation with good

pouch anomalies is that they are predominantly (95–97%) left end result. There were no complications in this subgroup of

sided [12], and a clear reason for this is still not understood. It is patients.

thought to be due to the more complex and longer pathway of the Recurrence rates are increased when there are multiple

fourth branchial tract on this side compared with the right side. preoperative infections and when there is no epithelial lining

Some authors have suggested it is a result of asymmetrical vascular identified in the excised specimen [7].

agenesis during embryogenesis [13].

Correct diagnosis and appropriate surgical management of 5. Conclusions

these brachial anomalies depends on the understanding of

anatomy and high index of suspicion. The diagnosis of branchial Branchial anomalies are relatively common in children. There

anomalies depends on good history and examination in addition to are three distinct types, first cleft, second cleft and fourth pouch

having high index of suspicion and clinical awareness. Branchial anomaly. Correct diagnosis is essential to avoid inadequate surgery

anomalies should be suspected for any unexplained neck masses or and multiple procedures. The surgical approach needs to be

recurrent neck spaces infections. Evaluation of congenital neck tailored to the suspected arch of origin of the anomaly. Definitive

lesions is aided by radiological investigations [14]. CT/MRI scans excision is essential for good outcomes.