International Journal of Pediatric Otorhinolaryngology 78 (2014) 1503–1507
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Second branchial cleft fistulae: Patient characteristics and surgical outcome Lauri Kajosaari a, *, Antti Mäkitie a , Päivi Salminen b , Tuomas Klockars a a Department of Otorhinolaryngology—Head and Neck Surgery, Helsinki University Central Hospital and University of Helsinki, P.O. Box 220, FI-00029 HUCH, Helsinki, Finland b Department of Pediatric Surgery, Helsinki University Central Hospital, Helsinki, Finland
A R T I C L E I N F O
A B S T R A C T
Article history: Received 3 May 2014 Received in revised form 10 June 2014 Accepted 14 June 2014 Available online 21 June 2014
Backgrounds: Second branchial cleft anomalies predispose to recurrent infections, and surgical resection is recommended as the treatment of choice. There is no clear consensus regarding the timing or surgical technique in the operative treatment of these anomalies. Our aim was to compare the effect of age and operative techniques to patient characteristics and treatment outcome. Methods: A retrospective study of pediatric patients treated for second branchial sinuses or fistulae during 1998–2012 at two departments in our academic tertiary care referral center. Comparison of patient characteristics, preoperative investigations, surgical techniques and postoperative sequelae. Results: Our data is based on 68 patients, the largest series in the literature. One-fourth (24%) of patients had any infectious symptoms prior to operative treatment. Patient demographics, preoperative investigations, use of methylene blue, or tonsillectomy had no effect on the surgical outcome. There were no re-operations due to residual disease. Three complications were observed postoperatively. Conclusions: Our patient series of second branchial cleft sinuses/fistulae is the largest so far and enables analyses of patient characteristics and surgical outcomes more reliably than previously. Preoperative symptoms are infrequent and mild. There was no difference in clinical outcome between the observed departments. Performing ipsilateral tonsillectomy gave no outcome benefits. The operation may be delayed to an age of approximately three years when anesthesiological risks are and possible harms are best avoided. Considering postoperative pain and risk of postoperative hemorrhage a routine tonsillectomy should not be included to the operative treatment of second branchial cleft fistulae. ã 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Branchial anomalies Surgery
1. Introduction Facial and neck structures are formed from five pairs of branchial arches separated by branchial clefts externally and branchial pouches internally. The pouches continue to develop into tympanic cavity, eustachian tube, tonsils, thymus and parathyroid glands. The first cleft forms the external auditory meatus, while the other clefts are obliterated by overgrowth of the second branchial arch. If this obliteration process is incomplete, branchial cleft anomalies are formed [1,2]. These anomalies include sinuses (blind end with a connection to the skin or pharynx), fistulas (communication between skin and pharynx), cysts and remnants of cartilage. Second branchial cleft anomalies represent 80–95% and first cleft anomalies 4–20% of cases while third and fourth pouch anomalies are rare [3,4] (Fig. 1).
* Corresponding author. Tel: +358 415457071. E-mail address: lauri.kajosaari@hus.fi (L. Kajosaari). http://dx.doi.org/10.1016/j.ijporl.2014.06.020 0165-5876/ ã 2014 Elsevier Ireland Ltd. All rights reserved.
Second branchial cleft fistula opens to the skin near the junction of lower and middle thirds of the anterior border of sternocleidomastoid muscle. The fistula travels beneath the sternocleidomastoid muscle, passes between the carotid bifurcation and then reaches the pharynx close to the tonsil [5,6]. A second branchial sinus represents an incomplete form of fistula. The second branchial cleft cysts can be located anywhere along the described fistula course. In a series of 51 patients, the distribution of second cleft anomalies was: sinus 45%, cyst 27% and fistula 27% [7]. The diagnosis of a second branchial cleft sinus or fistula can usually be made clinically. The average age of diagnosis of branchial cleft anomalies has been reported to be around 3–4 years, with a third of patients having a history on infections [7]. A branchial cleft sinus or fistula is usually noted in neonates or at an early age [8]. Ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) may be warranted if there is a palpable neck mass in association with the anomaly. A fistulogram can be used to assess extent of fistulous anomalies, but in a series of 63 fistulae in 52 patients the use of fistulogram was not found to give
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to compare patient numbers, characteristics, surgical techniques and outcome in two separate departments of the HUCH: the Department of Otorhinolaryngology and Head and Neck Surgery (ORL) and Department Pediatric Surgery (Ped Surg). 1.1. Patients and methods
Fig. 1. A complete second branchial cleft fistula.
advantage [17,18]. In addition to imaging, laryngoscopy may be needed to rule out a pyriform fossa fistula or sinus suggestive of a third pouch anomaly [9]. As branchial anomalies may lead to recurrent infections, the standard treatment is complete surgical excision of the sinus and fistulous tract, usually as open surgery from a lateral neck incision [10]. The extent of the surgery can range from simple excision of a short sinus to excising a complete fistulous tract from the lower neck to the pharynx. Recurrences after primary operative treatment are reported to be around 3%. However, the recurrence rate may be as high as 22% if there is a history of infections or incomplete operation [11]. Our objective is to describe the management of second branchial sinuses and fistulas in Helsinki University Central Hospital (HUCH). In addition, our study design gives a possibility
HUCH is a tertiary care referral center attending specialist level healthcare to approximately 1.5 million people in Southern Finland. Electronic hospital records were searched to identify all pediatric patients (age < 16 years) who had been examined or operated for second branchial fistulae or sinuses at ORL or Ped Surg during years 1997–2012. The patients were identified using the diagnostic codes from the WHO International Classification of Diseases and Nordic Medico-Statistical Committee classification. Data on demographics, symptoms, infections and preoperative investigations and imaging as well as operative details were gathered. The extent of the sinus or fistula tract was determined from the surgical notes. Due to the retrospective nature of the study, the Bailey classification for branchial fistulae could not be used as such, but the fistulae were classified to represent Bailey I, Bailey I–II or Bailey III–IV (Table 1). The need for re-operation and postoperative complications were analyzed. All statistical calculations were performed by an independent statistician (Datawell Oy, Helsinki, Finland). Possible differences in patient age between the two departments were analyzed using the
Table 1 Patient characteristics. Ped Surg
ORL
Total
Patient characteristics Number of patients (female + male) Mean age at diagnosis (range) Mean age at operation (range)
20 (10 + 10) 1.8 (0–12.2) 2.8 (0.4–12.6)
48 (19 + 29) 3.9 (0–16.0) 4.7 (1.0–16.2)
68 (29 + 39) 3.2 (0–16.0) 4.1 (0.4–16.2)
Side of anomaly Right Left Bilateral Total number of anomalies Preoperative symptoms
12 (60%) 6 (30%) 2 (17%) 22 4 (20%)
37 (77%) 7 (15%) 4 (8%) 52 12 (25%)
49 (72%) 13 (19%) 6 (9%) 74 16 (24%)
Preoperative investigations None Ultrasound Fistulography
19 (95%) 0 1 (5%)
35 (73%) 9 (19%) 4 (8%)
54 (79%) 9 (13%) 5 (7%)
9 (41%) 3 (14%) 0
45 (87%) 6 (12%) 24a (46%)
54 (73%) 9 (12%) 24 (32%)
Operative details Blue dye used Blue dye to pharynx Tonsil removed Sinus/fistula staging Sinus/fistula Extends to platysma (Bailey I)c Pierces platysma (Bailey I–II) Extends to pharynx (Bailey III–IV) Postoperative period Cosmetically disturbing scar Post-tonsillectomy haemorrhage Wound infection Residual disease Reoperation for residual disease
22 20 (91%) 19 (86%) 17 (77%)
52 41 (79%) 38 (73%) 31 (59%)
74 61 (82%) 57 (77%) 48 (65%)
0 0 0 0 0
1b 2 0 0 0
1 2 0 0 0
I: along anterior surface of sternocleidomastoid muscle just deep to platysma. II: along anterior surface of sternocleidomastoid muscle, lateral to carotid space and posterior to submandibular gland. III: extends medially between the bifurcation of internal and external carotid arteries to lateral pharyngeal wall. IV: opens into the pharynx and lined with columnar epithelium. a In one case tonsillectomy was performed because of tonsillar hypertrophy. In two cases where the fistula tract reached pharynx tonsillectomy had been previously performed.bHypertrophic scar correction 1 year after primary operation.cBailey classification of second branchial cleft sinuses (Cheng and Elden, 2012) [21].
L. Kajosaari et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1503–1507
Mann–Whitney U-test, a non-parametric test of the null hypothesis that two populations are the same against an alternative hypothesis. Statistical comparison of surgical technique (with or without tonsillectomy) and the use of methylene blue dye between the two departments were done using Fisher’s exact test, a statistical significance test used in the analysis of contingency tables. Institutional review board approval was obtained for this retrospective study based on hospital records only.
2. Results A total of 68 patients with second branchial cleft sinus or fistulae were identified. Majority of patients were treated at ORL (48/68, 71%) compared to Ped Surg (20/68, 29%). Majority of patients were male (57%). Of the patients, 62 (91%) had unilateral and six (9%) bilateral anomaly, resulting in a total of 74 branchial fistulae or sinuses. Of the unilateral anomalies, 79% (49/62) were right-sided. The mean age at the first visit for branchial sinus/fistula was 3.2 years (range 0–16 years). The mean age was 1.8 years for Ped Surg (range 0–12 years) and 3.9 years for ORL (range 0–16 years). The difference is statistically significant (p = 0.000677). The mean age at operation was 4.1 years (range 0–16 years). The mean age at operation was 2.8 years for Ped Surg (range 0–12 years) and 4.7 years for ORL (range 1–16 years). The difference is statistically significant (p = 0.000529). The mean time difference between the first visit and the operation was 0.8 years (range 0–7 years). This time difference was 1.0 years for Ped Surg (range 0–7 years) and 0.7 years for ORL (range 0–5 years). The difference is statistically significant (p = 0.030732). One-fourth of patients (24%, 16/68) had symptoms prior to operative treatment. These consisted of purulent discharge, local swelling, and infection of surrounding tissue or skin irritation. The proportion of symptomatic cases was relatively similar (25%, 12/48, for ORL and 20%, 4/20, for Ped Surg). The mean age of symptomatic patients at the first visit was 3.5 years (1.3 years for Ped Surg and 4.2 years for ORL). There were no cases of hospitalization or operative treatment before definite treatment. The mean age of patients without infection symptoms was 3.3 years (2.2 years for Ped Surg and 3.8 years for ORL). Most patients (79%, 54/68) had no radiological investigations prior to the operation. This proportion was 95% (19/29) for Ped Surg and 73% (35/48) for ORL . Preoperative ultrasound was performed to 13% (9/68) of patients (0% for Ped Surg, 19% for ORL). Preoperative fistulography was performed to 7% (5/68) of patients (5% for Ped Surg and 8% for ORL). Based on surgical notes, 82% (61/74) of the sinuses/fistulae extended at least to the level of platysma (91%, 20/22 for Ped Surg and 79%, 41/52 for ORL). 77% (57/74) extended beyond the platysma level (86%, 19/22 for Ped Surg and 73%, 38/52 for ORL). The sinus/fistula extended to the pharynx or its immediate
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proximity in 65% (48/74) of cases (77%, 17/22 for Ped Surg and 59%, 31/52 for ORL). Methylene blue dye was used intraoperatively in 73% (54/74) of the operations. The percentage was 41% (9/22) for Ped Surg and 87% (45/52) for ORL. The difference is statistically significant (p = 0.000061). In 17% of these cases (9/54) the dye was also visible orally, consistent with a complete and open fistula. Ipsilateral tonsillectomy was performed in one third of the cases (32%, 24/74). All tonsillectomies were performed at ORL where 46% (24/54) of the operations included an ipsilateral tonsillectomy. No tonsillectomies were performed at Ped Surg (0%, 0/22). The difference is statistically significant (p = 0.000028). In one patient with a second branchial cleft sinus only extending to platysma, a tonsillectomy was simultaneously performed because of tonsillar hypertrophy. In two patients with a complete fistula, tonsillectomy had been performed prior to the diagnosis of second branchial cleft sinus/fistula for other indications. There were no postoperative wound infections or re-operations for residual disease. Postoperative hemorrhage was present in 8% (2/24) of tonsillectomies. One patient was operated due to a hypertrophic scar one year after primary surgery. In two cases, there was a perioperative suspicion of incomplete removal of the sinus/fistula tract based on surgical notes. However, these patients were symptom free postoperatively and no re-operations were performed. A summary of the patient numbers, characteristics and operative techniques is presented in Table 1. 3. Discussions Congenital neck anomalies represent more than half of cervical masses operated in children. Of these approximately 20% represent branchial anomalies of which the second cleft anomalies are most frequent [12]. Adding our series of 68 patients to the previously reported total of 207 patients enables analyses of patient characteristics and surgical outcomes more reliably than previously (Table 2). Second branchial cleft anomalies are in the interface between the specialties of pediatric surgery and otorhinolaryngology, especially in countries like Finland where pediatric otorhinolaryngology is not a clinical subspecialty. At the Helsinki University Central Hospital these patients are managed at two different departments, Department of Otorhinolaryngology and Head and Neck surgery (ORL) and Department of Pediatric Surgery (Ped Surg). The selection between the two departments is made by the referring physician. Our series enables a possibility to compare patient characteristics and surgical techniques between these two departments. A branchial cleft sinus or fistula is usually noted in newborns, but the correct diagnosis is often delayed [8]. In our patient series, the mean age at the first visit was 3.2 years. This was 1.8 years for Ped Surg and 3.9 years for ORL (p = 0,000677). The mean age at operation was 2.8 years for Ped Surg and 4.7 years for ORL. In both departments the mean time difference between the first visit and
Table 2 Second branchial sinuses and fistulae: review of reported patient series since year 2000. Rattan et al., 2006 [18] Bajaj et al., 2011 [4] Maddalozzo et al., 2012 [23] Guldfred et al., 2012 [24] Cheng et al., 2012 [21] Our series Total
Patients 52 62 28 13 32 68 255
Males 38 (73%) 30 (48%) 11 (28%) 7 (54%) 23 (72%) 39 (57%) 148 (58%)
Anomalies 63 74 31 13 36 74 291
Unilateral 41 50 25 13 27 62 218 (85%)
Bilateral 11 12 3 0 5 6 37 (15%)
Complications 0 1 0 1 0 3 5 (1.7%)
Recurrences 2 1 0 1 1 0 5 (1.7%)
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the operation was relatively equal (although the difference is statistically significant), thus the difference in the mean age at operation seems to be mostly explained by the referring physician rather than suitable age for operation as defined by the operating surgeon. In our sample, there was no difference in the clinical outcome between the observed departments. The age at operation did not influence the clinical outcome of operative treatment. Some authors advocate surgical treatment of branchial anomalies at approximately 1 year of age [7]. This view is supported by the increase of recurrences if infections precede operative treatment [11]. Another view is to postpone the operation until 2–3 years of age [9,13]. A review of 249 deep neck infections revealed a congenital cause in 39 cases (16%) [14]. No second branchial cleft sinuses/fistulae were observed, indicating that these sinuses and fistulae are extremely rare cause of deep neck infections. Milder infections are reported to be relatively frequent – Schroeder et al. stated that 67% of branchial cleft sinuses/fistulae were infected prior to surgery [7]. However, only one-fourth (24%) of our patients had any infectious symptoms prior to operative treatment. In addition, the preoperative symptoms were mild; none of the patients required hospitalization or drainage of an abscess. It seems appropriate that in patients with no infectious sequalae the operation may be delayed to a later age of approximately three years when anesthesiological risks and possible harms are best avoided [15,16]. Forthemajorityofcasesinourseries,nopreoperative imagingwas performed. There was no statistically significant difference in preoperative imaging between the two departments. Obviously, the use for radiological investigations has to be decided individually based on the degree of certainty of the diagnosis. If there is no doubt about the diagnosis of second branchial cleft sinus/fistula, the operationcan besafely performedwithoutanypreoperative imaging. The operative technique was markedly different between pediatric surgeons and otolaryngologists. Both groups used intraoperative methylene blue dye, but its use was more regular among otolaryngologists (87% vs. 41%, (p = 0.000061). Ipsilateral tonsillectomy was performed in almost half of the operations performed by otolaryngologists (46%, 24/54), but in none of those performed at Ped Surg (0%, 0/22; p = 0.000028). Tonsillectomy had no effect on the outcome – there were no postoperative wound infections or re-operations for residual disease in either of the departments. This is in concordance with a recent report on second branchial arch anomalies consisting of 20 cysts, 29 sinuses and 36 fistulae by Cheng and Elden, who found no difference in outcome whether ipsilateral tonsillectomy was performed or not. However, Cheng and Elden discussed that based on power analyses, 154 patients would have been needed in each group to achieve a power of 80% to detect a recurrence rate of 5% [21]. Thus, performing ipsilateral tonsillectomy in association with the fistula operation does not give any outcome benefits to these patients. Considering postoperative pain and hemorrhage, a routine tonsillectomy should not be included to second branchial cleft fistula operation. In a patient series of 88 second branchial cleft anomalies (sinus, fistula and cyst), postoperative complications included wound infection in 5.7% and seroma in 1% of patients, all of which resolved without operative treatment [7]. The need for reoperation due to residual disease is typically 3–6% [7,18]. History of several infections seems to increase the risk for incomplete surgical and even figures up to 22% have been described [11]. The only reoperation in our material was due to a cosmetically disturbing scar formation. Postoperative hemorrhage was present in 8% (2/24) of tonsillectomies, which is in line with the rates noted in pediatric tonsillectomies in general [22]. No residual disease requiring surgery was reported. As the two centers are the only ones operating these anomalies in children in the Helsinki hospital district and minimum of 6 months (and up to 17 years) has passed
after the operation, it is highly likely that if residuals were frequent we would have observed them. Complete second branchial cleft fistulae have been considered so exceptional, that even case reports have been published [19,20,23,24]. However, in a recent series of 62 second branchial cleft anomalies with a total of 74 fistulae/sinuses almost 70% (50/74) were reported to be complete [4]. This finding is confirmed by our series: the fistula extended to the pharynx or its immediate proximity in 65% (48/74) of cases. If methylene blue dye was used, the dye was observed intraorally in almost one-fifth of patients (17%, 9/54) consistent with open fistula. 4. Conclusions Our patient series of second branchial cleft sinuses/fistulae is the largest so far and enables analyses of patient characteristics and surgical outcomes more reliably than previously. Preoperative symptoms are infrequent and mild. The operation may be delayed to an age of approximately three years when anesthesiological risks are and possible harms are best avoided. Considering postoperative pain and risk of postoperative hemorrhage a routine tonsillectomy should not be included to the operative treatment of second branchial cleft fistulae.
Conflicts of interest The authors report no conflicts of interest.
References [1] M. Mitroi, D. Dumitrescu, C. Simionescu, et al., Management of second branchial cleft anomalies, Rom. J. Morphol. Embryol. 49 (2008) 69–74. [2] J.H. Waldhausen, Branchial cleft and arch anomalies in children, Semin. Pediatr. Surg. 15 (2006) 64–69. [3] R.A. Mounsey, V. Forte, J. Friedberg, First brachial cleft sinuses: an analysis of current management strategies and treatment outcomes, J. Otolaryngol. 22 (1993) 457–461. [4] Y. Bajaj, S. Ifeacho, D. Tweedie, et al, Branchial anomalies in children, Int. J. Pediatr. Otorhinolaryngol. 75 (2011) 1020–1023. [5] P. Mirilas, 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 (2011) 1257–1263. [6] A.H. Ang, K.P. Pang, L.K. Tan, Complete branchial fistula. Case report and review of the literature, Ann. Otol. Rhinol. Laryngol. 110 (2001) 1077–1079. [7] J.W. Schroeder Jr., N. Mohyuddin, J. Maddalozzo, Branchial anomalies in the pediatric population, Otolaryngol. Head Neck Surg. 137 (2007) 289–295. [8] J.R. Chandler, B. Mitchell, Branchial cleft cysts, sinuses, and fistulas, Otolaryngol. Clin. North Am. 14 (1981) 175–186. [9] C.J. Goff, C. Allred, R.S. Glade, Current management of congenital branchial cleft cysts, sinuses, and fistulae, Curr. Opin. Otolaryngol. Head Neck Surg. 20 (2012) 533–539. [10] V.M. Vermeire, J.J. Daele, Second branchial cleft-pouch set fistulae, sinuses and cysts in children, Acta Otorhinolaryngol. Belg. 45 (1991) 437–442. [11] S.S. Choi, G.H. Zalzal, Branchial anomalies: a review of 52 cases, Laryngoscope 105 (1995) 909–913. [12] G.D. Albers, Branchial anomalies, JAMA 183 (1963) 399–409. [13] G.R. Ford, A. Balakrishnan, J.N. Evans, C.M. Bailey, Branchial cleft and pouch anomalies, J. Laryngol. Otol. 106 (1992) 137–143. [14] Y.A. Nour, M.H. Hassan, A. Gaafar, A. Eldaly, Deep neck infections of congenital causes, Otolaryngol. Head Neck Surg. 144 (2011) 365–371. [15] E.A. Olsen, A.M. Brambrink, Anesthesia for the young child undergoing ambulatory procedures: current concerns regarding harm to the developing brain, Curr. Opin. Anaesthesiol. 26 (2013) 677–684. [16] R. Miller, Miller’s Anesthesia, seventh ed., Elsevier, Churchill Livingstone, 2009. [17] J.L. Guarisco, A. Fatakia, Intraoperative fistulograms in the management of branchial apparatus abnormalities in children, Int. J. Pediatr. Otorhinolaryngol. 72 (2008) 1777–1782. [18] K.N. Rattan, S. Rattan, D. Parihar, J.S. Gulia, S.P. Yadav, Second branchial cleft fistula: is fistulo-gram necessary for complete excision, Int. J. Pediatr. Otorhinolaryngol. 70 (2006) 1027–1030. [19] V.G. Shankar, T.A. Babu, H.B. Swami, A rare case of complete second arch branchial fistula in a 7-year-old child, Natl. J. Maxillofac. Surg. 3 (2012) 226–228.
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[23] J. Maddalozzo, J.C. Rastatter, H.F. Dreyfuss, R. Jaffar, B. Bhushan, The second branchial cleft fistula, Int. J. Pediatr. Otorhinolaryngol. 76 (2012) 1042– 1045. [24] L.A. Guldfred, B.B. Philipsen, C. Siim, Branchial cleft anomalies: accuracy of pre-operative diagnosis, clinical presentation and management, J. Laryngol. Otol. 126 (2012) 598–604.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Second branchial cleft fistulae: Patient characteristics and surgical outcome Lauri Kajosaari a, *, Antti Mäkitie a , Päivi Salminen b , Tuomas Klockars a a Department of Otorhinolaryngology—Head and Neck Surgery, Helsinki University Central Hospital and University of Helsinki, P.O. Box 220, FI-00029 HUCH, Helsinki, Finland b Department of Pediatric Surgery, Helsinki University Central Hospital, Helsinki, Finland
A R T I C L E I N F O
A B S T R A C T
Article history: Received 3 May 2014 Received in revised form 10 June 2014 Accepted 14 June 2014 Available online 21 June 2014
Backgrounds: Second branchial cleft anomalies predispose to recurrent infections, and surgical resection is recommended as the treatment of choice. There is no clear consensus regarding the timing or surgical technique in the operative treatment of these anomalies. Our aim was to compare the effect of age and operative techniques to patient characteristics and treatment outcome. Methods: A retrospective study of pediatric patients treated for second branchial sinuses or fistulae during 1998–2012 at two departments in our academic tertiary care referral center. Comparison of patient characteristics, preoperative investigations, surgical techniques and postoperative sequelae. Results: Our data is based on 68 patients, the largest series in the literature. One-fourth (24%) of patients had any infectious symptoms prior to operative treatment. Patient demographics, preoperative investigations, use of methylene blue, or tonsillectomy had no effect on the surgical outcome. There were no re-operations due to residual disease. Three complications were observed postoperatively. Conclusions: Our patient series of second branchial cleft sinuses/fistulae is the largest so far and enables analyses of patient characteristics and surgical outcomes more reliably than previously. Preoperative symptoms are infrequent and mild. There was no difference in clinical outcome between the observed departments. Performing ipsilateral tonsillectomy gave no outcome benefits. The operation may be delayed to an age of approximately three years when anesthesiological risks are and possible harms are best avoided. Considering postoperative pain and risk of postoperative hemorrhage a routine tonsillectomy should not be included to the operative treatment of second branchial cleft fistulae. ã 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Branchial anomalies Surgery
1. Introduction Facial and neck structures are formed from five pairs of branchial arches separated by branchial clefts externally and branchial pouches internally. The pouches continue to develop into tympanic cavity, eustachian tube, tonsils, thymus and parathyroid glands. The first cleft forms the external auditory meatus, while the other clefts are obliterated by overgrowth of the second branchial arch. If this obliteration process is incomplete, branchial cleft anomalies are formed [1,2]. These anomalies include sinuses (blind end with a connection to the skin or pharynx), fistulas (communication between skin and pharynx), cysts and remnants of cartilage. Second branchial cleft anomalies represent 80–95% and first cleft anomalies 4–20% of cases while third and fourth pouch anomalies are rare [3,4] (Fig. 1).
* Corresponding author. Tel: +358 415457071. E-mail address: lauri.kajosaari@hus.fi (L. Kajosaari). http://dx.doi.org/10.1016/j.ijporl.2014.06.020 0165-5876/ ã 2014 Elsevier Ireland Ltd. All rights reserved.
Second branchial cleft fistula opens to the skin near the junction of lower and middle thirds of the anterior border of sternocleidomastoid muscle. The fistula travels beneath the sternocleidomastoid muscle, passes between the carotid bifurcation and then reaches the pharynx close to the tonsil [5,6]. A second branchial sinus represents an incomplete form of fistula. The second branchial cleft cysts can be located anywhere along the described fistula course. In a series of 51 patients, the distribution of second cleft anomalies was: sinus 45%, cyst 27% and fistula 27% [7]. The diagnosis of a second branchial cleft sinus or fistula can usually be made clinically. The average age of diagnosis of branchial cleft anomalies has been reported to be around 3–4 years, with a third of patients having a history on infections [7]. A branchial cleft sinus or fistula is usually noted in neonates or at an early age [8]. Ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) may be warranted if there is a palpable neck mass in association with the anomaly. A fistulogram can be used to assess extent of fistulous anomalies, but in a series of 63 fistulae in 52 patients the use of fistulogram was not found to give
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to compare patient numbers, characteristics, surgical techniques and outcome in two separate departments of the HUCH: the Department of Otorhinolaryngology and Head and Neck Surgery (ORL) and Department Pediatric Surgery (Ped Surg). 1.1. Patients and methods
Fig. 1. A complete second branchial cleft fistula.
advantage [17,18]. In addition to imaging, laryngoscopy may be needed to rule out a pyriform fossa fistula or sinus suggestive of a third pouch anomaly [9]. As branchial anomalies may lead to recurrent infections, the standard treatment is complete surgical excision of the sinus and fistulous tract, usually as open surgery from a lateral neck incision [10]. The extent of the surgery can range from simple excision of a short sinus to excising a complete fistulous tract from the lower neck to the pharynx. Recurrences after primary operative treatment are reported to be around 3%. However, the recurrence rate may be as high as 22% if there is a history of infections or incomplete operation [11]. Our objective is to describe the management of second branchial sinuses and fistulas in Helsinki University Central Hospital (HUCH). In addition, our study design gives a possibility
HUCH is a tertiary care referral center attending specialist level healthcare to approximately 1.5 million people in Southern Finland. Electronic hospital records were searched to identify all pediatric patients (age < 16 years) who had been examined or operated for second branchial fistulae or sinuses at ORL or Ped Surg during years 1997–2012. The patients were identified using the diagnostic codes from the WHO International Classification of Diseases and Nordic Medico-Statistical Committee classification. Data on demographics, symptoms, infections and preoperative investigations and imaging as well as operative details were gathered. The extent of the sinus or fistula tract was determined from the surgical notes. Due to the retrospective nature of the study, the Bailey classification for branchial fistulae could not be used as such, but the fistulae were classified to represent Bailey I, Bailey I–II or Bailey III–IV (Table 1). The need for re-operation and postoperative complications were analyzed. All statistical calculations were performed by an independent statistician (Datawell Oy, Helsinki, Finland). Possible differences in patient age between the two departments were analyzed using the
Table 1 Patient characteristics. Ped Surg
ORL
Total
Patient characteristics Number of patients (female + male) Mean age at diagnosis (range) Mean age at operation (range)
20 (10 + 10) 1.8 (0–12.2) 2.8 (0.4–12.6)
48 (19 + 29) 3.9 (0–16.0) 4.7 (1.0–16.2)
68 (29 + 39) 3.2 (0–16.0) 4.1 (0.4–16.2)
Side of anomaly Right Left Bilateral Total number of anomalies Preoperative symptoms
12 (60%) 6 (30%) 2 (17%) 22 4 (20%)
37 (77%) 7 (15%) 4 (8%) 52 12 (25%)
49 (72%) 13 (19%) 6 (9%) 74 16 (24%)
Preoperative investigations None Ultrasound Fistulography
19 (95%) 0 1 (5%)
35 (73%) 9 (19%) 4 (8%)
54 (79%) 9 (13%) 5 (7%)
9 (41%) 3 (14%) 0
45 (87%) 6 (12%) 24a (46%)
54 (73%) 9 (12%) 24 (32%)
Operative details Blue dye used Blue dye to pharynx Tonsil removed Sinus/fistula staging Sinus/fistula Extends to platysma (Bailey I)c Pierces platysma (Bailey I–II) Extends to pharynx (Bailey III–IV) Postoperative period Cosmetically disturbing scar Post-tonsillectomy haemorrhage Wound infection Residual disease Reoperation for residual disease
22 20 (91%) 19 (86%) 17 (77%)
52 41 (79%) 38 (73%) 31 (59%)
74 61 (82%) 57 (77%) 48 (65%)
0 0 0 0 0
1b 2 0 0 0
1 2 0 0 0
I: along anterior surface of sternocleidomastoid muscle just deep to platysma. II: along anterior surface of sternocleidomastoid muscle, lateral to carotid space and posterior to submandibular gland. III: extends medially between the bifurcation of internal and external carotid arteries to lateral pharyngeal wall. IV: opens into the pharynx and lined with columnar epithelium. a In one case tonsillectomy was performed because of tonsillar hypertrophy. In two cases where the fistula tract reached pharynx tonsillectomy had been previously performed.bHypertrophic scar correction 1 year after primary operation.cBailey classification of second branchial cleft sinuses (Cheng and Elden, 2012) [21].
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Mann–Whitney U-test, a non-parametric test of the null hypothesis that two populations are the same against an alternative hypothesis. Statistical comparison of surgical technique (with or without tonsillectomy) and the use of methylene blue dye between the two departments were done using Fisher’s exact test, a statistical significance test used in the analysis of contingency tables. Institutional review board approval was obtained for this retrospective study based on hospital records only.
2. Results A total of 68 patients with second branchial cleft sinus or fistulae were identified. Majority of patients were treated at ORL (48/68, 71%) compared to Ped Surg (20/68, 29%). Majority of patients were male (57%). Of the patients, 62 (91%) had unilateral and six (9%) bilateral anomaly, resulting in a total of 74 branchial fistulae or sinuses. Of the unilateral anomalies, 79% (49/62) were right-sided. The mean age at the first visit for branchial sinus/fistula was 3.2 years (range 0–16 years). The mean age was 1.8 years for Ped Surg (range 0–12 years) and 3.9 years for ORL (range 0–16 years). The difference is statistically significant (p = 0.000677). The mean age at operation was 4.1 years (range 0–16 years). The mean age at operation was 2.8 years for Ped Surg (range 0–12 years) and 4.7 years for ORL (range 1–16 years). The difference is statistically significant (p = 0.000529). The mean time difference between the first visit and the operation was 0.8 years (range 0–7 years). This time difference was 1.0 years for Ped Surg (range 0–7 years) and 0.7 years for ORL (range 0–5 years). The difference is statistically significant (p = 0.030732). One-fourth of patients (24%, 16/68) had symptoms prior to operative treatment. These consisted of purulent discharge, local swelling, and infection of surrounding tissue or skin irritation. The proportion of symptomatic cases was relatively similar (25%, 12/48, for ORL and 20%, 4/20, for Ped Surg). The mean age of symptomatic patients at the first visit was 3.5 years (1.3 years for Ped Surg and 4.2 years for ORL). There were no cases of hospitalization or operative treatment before definite treatment. The mean age of patients without infection symptoms was 3.3 years (2.2 years for Ped Surg and 3.8 years for ORL). Most patients (79%, 54/68) had no radiological investigations prior to the operation. This proportion was 95% (19/29) for Ped Surg and 73% (35/48) for ORL . Preoperative ultrasound was performed to 13% (9/68) of patients (0% for Ped Surg, 19% for ORL). Preoperative fistulography was performed to 7% (5/68) of patients (5% for Ped Surg and 8% for ORL). Based on surgical notes, 82% (61/74) of the sinuses/fistulae extended at least to the level of platysma (91%, 20/22 for Ped Surg and 79%, 41/52 for ORL). 77% (57/74) extended beyond the platysma level (86%, 19/22 for Ped Surg and 73%, 38/52 for ORL). The sinus/fistula extended to the pharynx or its immediate
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proximity in 65% (48/74) of cases (77%, 17/22 for Ped Surg and 59%, 31/52 for ORL). Methylene blue dye was used intraoperatively in 73% (54/74) of the operations. The percentage was 41% (9/22) for Ped Surg and 87% (45/52) for ORL. The difference is statistically significant (p = 0.000061). In 17% of these cases (9/54) the dye was also visible orally, consistent with a complete and open fistula. Ipsilateral tonsillectomy was performed in one third of the cases (32%, 24/74). All tonsillectomies were performed at ORL where 46% (24/54) of the operations included an ipsilateral tonsillectomy. No tonsillectomies were performed at Ped Surg (0%, 0/22). The difference is statistically significant (p = 0.000028). In one patient with a second branchial cleft sinus only extending to platysma, a tonsillectomy was simultaneously performed because of tonsillar hypertrophy. In two patients with a complete fistula, tonsillectomy had been performed prior to the diagnosis of second branchial cleft sinus/fistula for other indications. There were no postoperative wound infections or re-operations for residual disease. Postoperative hemorrhage was present in 8% (2/24) of tonsillectomies. One patient was operated due to a hypertrophic scar one year after primary surgery. In two cases, there was a perioperative suspicion of incomplete removal of the sinus/fistula tract based on surgical notes. However, these patients were symptom free postoperatively and no re-operations were performed. A summary of the patient numbers, characteristics and operative techniques is presented in Table 1. 3. Discussions Congenital neck anomalies represent more than half of cervical masses operated in children. Of these approximately 20% represent branchial anomalies of which the second cleft anomalies are most frequent [12]. Adding our series of 68 patients to the previously reported total of 207 patients enables analyses of patient characteristics and surgical outcomes more reliably than previously (Table 2). Second branchial cleft anomalies are in the interface between the specialties of pediatric surgery and otorhinolaryngology, especially in countries like Finland where pediatric otorhinolaryngology is not a clinical subspecialty. At the Helsinki University Central Hospital these patients are managed at two different departments, Department of Otorhinolaryngology and Head and Neck surgery (ORL) and Department of Pediatric Surgery (Ped Surg). The selection between the two departments is made by the referring physician. Our series enables a possibility to compare patient characteristics and surgical techniques between these two departments. A branchial cleft sinus or fistula is usually noted in newborns, but the correct diagnosis is often delayed [8]. In our patient series, the mean age at the first visit was 3.2 years. This was 1.8 years for Ped Surg and 3.9 years for ORL (p = 0,000677). The mean age at operation was 2.8 years for Ped Surg and 4.7 years for ORL. In both departments the mean time difference between the first visit and
Table 2 Second branchial sinuses and fistulae: review of reported patient series since year 2000. Rattan et al., 2006 [18] Bajaj et al., 2011 [4] Maddalozzo et al., 2012 [23] Guldfred et al., 2012 [24] Cheng et al., 2012 [21] Our series Total
Patients 52 62 28 13 32 68 255
Males 38 (73%) 30 (48%) 11 (28%) 7 (54%) 23 (72%) 39 (57%) 148 (58%)
Anomalies 63 74 31 13 36 74 291
Unilateral 41 50 25 13 27 62 218 (85%)
Bilateral 11 12 3 0 5 6 37 (15%)
Complications 0 1 0 1 0 3 5 (1.7%)
Recurrences 2 1 0 1 1 0 5 (1.7%)
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the operation was relatively equal (although the difference is statistically significant), thus the difference in the mean age at operation seems to be mostly explained by the referring physician rather than suitable age for operation as defined by the operating surgeon. In our sample, there was no difference in the clinical outcome between the observed departments. The age at operation did not influence the clinical outcome of operative treatment. Some authors advocate surgical treatment of branchial anomalies at approximately 1 year of age [7]. This view is supported by the increase of recurrences if infections precede operative treatment [11]. Another view is to postpone the operation until 2–3 years of age [9,13]. A review of 249 deep neck infections revealed a congenital cause in 39 cases (16%) [14]. No second branchial cleft sinuses/fistulae were observed, indicating that these sinuses and fistulae are extremely rare cause of deep neck infections. Milder infections are reported to be relatively frequent – Schroeder et al. stated that 67% of branchial cleft sinuses/fistulae were infected prior to surgery [7]. However, only one-fourth (24%) of our patients had any infectious symptoms prior to operative treatment. In addition, the preoperative symptoms were mild; none of the patients required hospitalization or drainage of an abscess. It seems appropriate that in patients with no infectious sequalae the operation may be delayed to a later age of approximately three years when anesthesiological risks and possible harms are best avoided [15,16]. Forthemajorityofcasesinourseries,nopreoperative imagingwas performed. There was no statistically significant difference in preoperative imaging between the two departments. Obviously, the use for radiological investigations has to be decided individually based on the degree of certainty of the diagnosis. If there is no doubt about the diagnosis of second branchial cleft sinus/fistula, the operationcan besafely performedwithoutanypreoperative imaging. The operative technique was markedly different between pediatric surgeons and otolaryngologists. Both groups used intraoperative methylene blue dye, but its use was more regular among otolaryngologists (87% vs. 41%, (p = 0.000061). Ipsilateral tonsillectomy was performed in almost half of the operations performed by otolaryngologists (46%, 24/54), but in none of those performed at Ped Surg (0%, 0/22; p = 0.000028). Tonsillectomy had no effect on the outcome – there were no postoperative wound infections or re-operations for residual disease in either of the departments. This is in concordance with a recent report on second branchial arch anomalies consisting of 20 cysts, 29 sinuses and 36 fistulae by Cheng and Elden, who found no difference in outcome whether ipsilateral tonsillectomy was performed or not. However, Cheng and Elden discussed that based on power analyses, 154 patients would have been needed in each group to achieve a power of 80% to detect a recurrence rate of 5% [21]. Thus, performing ipsilateral tonsillectomy in association with the fistula operation does not give any outcome benefits to these patients. Considering postoperative pain and hemorrhage, a routine tonsillectomy should not be included to second branchial cleft fistula operation. In a patient series of 88 second branchial cleft anomalies (sinus, fistula and cyst), postoperative complications included wound infection in 5.7% and seroma in 1% of patients, all of which resolved without operative treatment [7]. The need for reoperation due to residual disease is typically 3–6% [7,18]. History of several infections seems to increase the risk for incomplete surgical and even figures up to 22% have been described [11]. The only reoperation in our material was due to a cosmetically disturbing scar formation. Postoperative hemorrhage was present in 8% (2/24) of tonsillectomies, which is in line with the rates noted in pediatric tonsillectomies in general [22]. No residual disease requiring surgery was reported. As the two centers are the only ones operating these anomalies in children in the Helsinki hospital district and minimum of 6 months (and up to 17 years) has passed
after the operation, it is highly likely that if residuals were frequent we would have observed them. Complete second branchial cleft fistulae have been considered so exceptional, that even case reports have been published [19,20,23,24]. However, in a recent series of 62 second branchial cleft anomalies with a total of 74 fistulae/sinuses almost 70% (50/74) were reported to be complete [4]. This finding is confirmed by our series: the fistula extended to the pharynx or its immediate proximity in 65% (48/74) of cases. If methylene blue dye was used, the dye was observed intraorally in almost one-fifth of patients (17%, 9/54) consistent with open fistula. 4. Conclusions Our patient series of second branchial cleft sinuses/fistulae is the largest so far and enables analyses of patient characteristics and surgical outcomes more reliably than previously. Preoperative symptoms are infrequent and mild. The operation may be delayed to an age of approximately three years when anesthesiological risks are and possible harms are best avoided. Considering postoperative pain and risk of postoperative hemorrhage a routine tonsillectomy should not be included to the operative treatment of second branchial cleft fistulae.
Conflicts of interest The authors report no conflicts of interest.
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