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First branchial cleft fistula: A case report
International Journal of Pediatric Otorhinolaryngology Extra (2008) 3, 174—176
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CASE REPORT
First branchial cleft fistula: A case report I. Fernandez-Pineda a,*, J.M. Hernandez-Guisado b, A. Gracia a, J.C. de Agustin a a b
Department of Pediatric Surgery, Virgen del Rocı´o Children’s Hospital, Sevilla, Spain Department of Oral and Maxillofacial Surgery, Virgen del Rocı´o Children’s Hospital, Sevilla, Spain
Received 19 February 2008; received in revised form 13 March 2008; accepted 22 March 2008 Available online 5 May 2008
KEYWORDS Branchial cleft fistula; Parotidectomy; Facial nerve
Summary First branchial cleft anomalies are a rare group of congenital malformations, accounting for about 5% of branchial cleft defects. The diagnosis may not be clear since their presentation is similar to other clinical conditions such as lymphadenitis, dermoid cyst, cystic hygroma, lymphangioma, primary parotid tumor, etc. Surgical excision requires a wide exposure so as not to damage the facial nerve. We report a case of a 2-year-old boy with a first branchial cleft fistula, in which a seropurulent discharge was observed through the cleft and along the ear canal. # 2008 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
2. Case report
Embryological anomalies of the first branchial cleft are a rare condition accounting for about 5% of branchial cleft defects [1]. Fistulae usually extend from the external auditory canal to the skin of the neck below the angle of the mandible. Embryological theory is based on the fact that branchial apparatus is a transient structure that is observed between the 4th and the 7th week of fetal development and whose incomplete obliteration results in the formation of a cyst, fistula or sinus [2]. We present the case of a 2-year-old boy who showed a first branchial cleft fistula.
A fit 2-year-old boy was referred to the Department of Pediatric Surgery because of an enlarging tender mass over the left side of his neck and fever. He was treated by intravenous antibiotics and an appropriate drainage was performed. Cultures grew Streptococcus beta-hem group A and were negative for acid fast bacilli. Postoperatively, the incision site failed to close and a seropulent discharge persisted. Drainage was also found in the ear external canal that helped the diagnosis of first branchial cleft fistula. Two months after drainage a superficial lobe parotidectomy (Fig. 1) was performed with facial nerve dissection. A fistula was noted to run from the ear external canal to the left angle of the mandible (Fig. 2). The tract passed deep to the facial nerve. Fistula excision was performed and postoperative time was uneventful. 6-Methylprednisolone
* Corresponding author at: Department of Pediatric Surgery, Hospital Infantil Universitario Virgen del Rocı´o, Av. Manuel Siurot s/n, Sevilla 41013, Spain. Tel.: +34 955 012956. E-mail address: [email protected] (I. Fernandez-Pineda).
1871-4048/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2008.03.006
First branchial cleft fistula
Fig. 1
Superficial lobe parotidectomy.
Fig. 2
Fistula tract.
(1 mg/kg) was administered after surgery in order to improve facial paresis during 8 weeks. Two months after operation facial nerve weakness had completely resolved and rehabilitation was not necessary. Histological examination showed keratizing stratified squamous epithelium with adnexal structure.
175 First branchial cleft anomalies are often misdiagnosed since their presentation may be similar to other conditions and the differential diagnosis of masses in this area include lymphadenitis, dermoid cyst, cystic hygroma, lipoma, lymphangioma and other tumoral conditions [6,7]. The age of presentation ranges between birth and the 2nd decade. The common clinical symptom is swelling in the periauricular, parotid or cervical regions. There may be external ear discharge and an otitis externa can be misdiagnosed. There is often a history of recurrent infection requiring antibiotics, incision and drainage. Recurrent surgical intervention before the correct procedure was reported in 35—48% of cases [8,9]. Diagnosis is mainly clinical, although radiologic exams like CT may be a useful tool to confirm or suggest other conditions, and to define the location and extent of the lesion [10]. Magnetic resonance is specially indicated in parotid gland anomalies [11]. In the management of these lesions, it is important to avoid any excisional procedure in the acute phase. Antibiotics, incision and drainage are the principles of the first treatment. After resolution, a parotidectomy with a wide exposure is essential for both the complete excision and preservation of the facial nerve. Any sinus, fistula or other conditions should be excised. The relation between the facial nerve and the sinus or fistulous tract seems to be variable; a review of 73 patients showed that the majority of anomalies passed superficial to the nerve (63%). In our case, the fistulous tract ran deep to the facial nerve [12]. Recurrence rate after operation is about 5% and may reach 20% in case there had been previous infection and operation.
4. Conclusion 3. Discussion Since James Paget, in 1878 [3], published the first case in the literature, not many cases of first branchial cleft fistula have been reported. In 1972 Work [4] classified first branchial cleft anomalies as Type I, composed of ectoderm only and generally parallel to the external ear canal, and Type II which tend to develop from the external canal to the neck and are composed of ectoderm and mesoderm. The majority of branchial arch anomalies are 2nd branchial cleft defects; first branchial cleft forms from 1 to 8% of all of them [5]. Cysts represent more than 50% and the rest are fistulas, sinuses, etc.
The diverse presentations of first branchial cleft anomalies lead to misdiagnosis and inadequate surgical treatment. Any patient with a history of recurrent periauricular swelling, a sinus in the neck or external ear drainage, should be studied looking for a first cleft branchial anomaly. A wide exposure is needed to avoid the damage of the facial nerve and minimize the recurrence.
References [1] K.D. Olsen, N.E. Maragos, L.H. Weiland, First branchial cleft anomalies, Laryngoscope 90 (1980) 423—436.
176
I. Fernandez-Pineda et al.
[2] D.B. Wilson, Embryonic development of the head and neck. Part 2. The branchial region, Head Neck Surg. 2 (1979) 59— 66. [3] J. Jakubikova, R. Stanik, A. Stanikova, Malformations of the first branchial cleft: duplication of the external auditory canal, Int. J. Pediatr. Otorhinolaryngol. 69 (2) (2005) 255— 261. [4] W.P. Work, Newer concepts of first branchial cleft defects, Laryngoscope 82 (1972) 1581—1593. [5] A.R. D’Souza, H.S. Uppal, R. De, H. Zeitoun, Updating concepts of the first branchial clefts defect: a literature review, Int. J. Pediatr. Otorhinolaryngol. 62 (2003) 103—109. [6] K.G. Mc Rae, K.J. Lee, E. Goertzen, First branchial cleft anomalies and the facial nerve, Otolaryngol. Head Neck Surg. (1983) 197—202.
[7] F. Noel, A. Leung, First branchial cleft fistula: case reports and literature review, J. Otolaryngol. 16 (4) (1987) 247—251. [8] J.M. Triglia, R. Nicollas, V. Ducroz, P. Koltai, E. Garabedian, First branchial cleft anomalies: a study of 39 cases and a review of the literature, Arch. Otolaryngol. Head Neck Surg. 124 (1998) 291—295. [9] S.S. Choi, G.H. Zalzal, Branchial anomalies: a review of 52 cases, Laryngoscope 105 (1995) 909—913. [10] C. Stulner, Management of first branchial anomalies: report of two cases, Br. J. Oral Maxillofac. Surg. 39 (2001) 30—33. [11] Y. Ariyoshi, Determining whether a parotid tumor is in the superficial or deep lobe using magnetic resonance imaging, J. Oral Maxillofac. Surg. 56 (1998) 23—26. [12] P.D. Miller, Surgical excision of first cleft branchial fistulae, Br. J. Surg. 71 (1984) 696—697.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORT
First branchial cleft fistula: A case report I. Fernandez-Pineda a,*, J.M. Hernandez-Guisado b, A. Gracia a, J.C. de Agustin a a b
Department of Pediatric Surgery, Virgen del Rocı´o Children’s Hospital, Sevilla, Spain Department of Oral and Maxillofacial Surgery, Virgen del Rocı´o Children’s Hospital, Sevilla, Spain
Received 19 February 2008; received in revised form 13 March 2008; accepted 22 March 2008 Available online 5 May 2008
KEYWORDS Branchial cleft fistula; Parotidectomy; Facial nerve
Summary First branchial cleft anomalies are a rare group of congenital malformations, accounting for about 5% of branchial cleft defects. The diagnosis may not be clear since their presentation is similar to other clinical conditions such as lymphadenitis, dermoid cyst, cystic hygroma, lymphangioma, primary parotid tumor, etc. Surgical excision requires a wide exposure so as not to damage the facial nerve. We report a case of a 2-year-old boy with a first branchial cleft fistula, in which a seropurulent discharge was observed through the cleft and along the ear canal. # 2008 Elsevier Ireland Ltd. All rights reserved.
1. Introduction
2. Case report
Embryological anomalies of the first branchial cleft are a rare condition accounting for about 5% of branchial cleft defects [1]. Fistulae usually extend from the external auditory canal to the skin of the neck below the angle of the mandible. Embryological theory is based on the fact that branchial apparatus is a transient structure that is observed between the 4th and the 7th week of fetal development and whose incomplete obliteration results in the formation of a cyst, fistula or sinus [2]. We present the case of a 2-year-old boy who showed a first branchial cleft fistula.
A fit 2-year-old boy was referred to the Department of Pediatric Surgery because of an enlarging tender mass over the left side of his neck and fever. He was treated by intravenous antibiotics and an appropriate drainage was performed. Cultures grew Streptococcus beta-hem group A and were negative for acid fast bacilli. Postoperatively, the incision site failed to close and a seropulent discharge persisted. Drainage was also found in the ear external canal that helped the diagnosis of first branchial cleft fistula. Two months after drainage a superficial lobe parotidectomy (Fig. 1) was performed with facial nerve dissection. A fistula was noted to run from the ear external canal to the left angle of the mandible (Fig. 2). The tract passed deep to the facial nerve. Fistula excision was performed and postoperative time was uneventful. 6-Methylprednisolone
* Corresponding author at: Department of Pediatric Surgery, Hospital Infantil Universitario Virgen del Rocı´o, Av. Manuel Siurot s/n, Sevilla 41013, Spain. Tel.: +34 955 012956. E-mail address: [email protected] (I. Fernandez-Pineda).
1871-4048/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2008.03.006
First branchial cleft fistula
Fig. 1
Superficial lobe parotidectomy.
Fig. 2
Fistula tract.
(1 mg/kg) was administered after surgery in order to improve facial paresis during 8 weeks. Two months after operation facial nerve weakness had completely resolved and rehabilitation was not necessary. Histological examination showed keratizing stratified squamous epithelium with adnexal structure.
175 First branchial cleft anomalies are often misdiagnosed since their presentation may be similar to other conditions and the differential diagnosis of masses in this area include lymphadenitis, dermoid cyst, cystic hygroma, lipoma, lymphangioma and other tumoral conditions [6,7]. The age of presentation ranges between birth and the 2nd decade. The common clinical symptom is swelling in the periauricular, parotid or cervical regions. There may be external ear discharge and an otitis externa can be misdiagnosed. There is often a history of recurrent infection requiring antibiotics, incision and drainage. Recurrent surgical intervention before the correct procedure was reported in 35—48% of cases [8,9]. Diagnosis is mainly clinical, although radiologic exams like CT may be a useful tool to confirm or suggest other conditions, and to define the location and extent of the lesion [10]. Magnetic resonance is specially indicated in parotid gland anomalies [11]. In the management of these lesions, it is important to avoid any excisional procedure in the acute phase. Antibiotics, incision and drainage are the principles of the first treatment. After resolution, a parotidectomy with a wide exposure is essential for both the complete excision and preservation of the facial nerve. Any sinus, fistula or other conditions should be excised. The relation between the facial nerve and the sinus or fistulous tract seems to be variable; a review of 73 patients showed that the majority of anomalies passed superficial to the nerve (63%). In our case, the fistulous tract ran deep to the facial nerve [12]. Recurrence rate after operation is about 5% and may reach 20% in case there had been previous infection and operation.
4. Conclusion 3. Discussion Since James Paget, in 1878 [3], published the first case in the literature, not many cases of first branchial cleft fistula have been reported. In 1972 Work [4] classified first branchial cleft anomalies as Type I, composed of ectoderm only and generally parallel to the external ear canal, and Type II which tend to develop from the external canal to the neck and are composed of ectoderm and mesoderm. The majority of branchial arch anomalies are 2nd branchial cleft defects; first branchial cleft forms from 1 to 8% of all of them [5]. Cysts represent more than 50% and the rest are fistulas, sinuses, etc.
The diverse presentations of first branchial cleft anomalies lead to misdiagnosis and inadequate surgical treatment. Any patient with a history of recurrent periauricular swelling, a sinus in the neck or external ear drainage, should be studied looking for a first cleft branchial anomaly. A wide exposure is needed to avoid the damage of the facial nerve and minimize the recurrence.
References [1] K.D. Olsen, N.E. Maragos, L.H. Weiland, First branchial cleft anomalies, Laryngoscope 90 (1980) 423—436.
176
I. Fernandez-Pineda et al.
[2] D.B. Wilson, Embryonic development of the head and neck. Part 2. The branchial region, Head Neck Surg. 2 (1979) 59— 66. [3] J. Jakubikova, R. Stanik, A. Stanikova, Malformations of the first branchial cleft: duplication of the external auditory canal, Int. J. Pediatr. Otorhinolaryngol. 69 (2) (2005) 255— 261. [4] W.P. Work, Newer concepts of first branchial cleft defects, Laryngoscope 82 (1972) 1581—1593. [5] A.R. D’Souza, H.S. Uppal, R. De, H. Zeitoun, Updating concepts of the first branchial clefts defect: a literature review, Int. J. Pediatr. Otorhinolaryngol. 62 (2003) 103—109. [6] K.G. Mc Rae, K.J. Lee, E. Goertzen, First branchial cleft anomalies and the facial nerve, Otolaryngol. Head Neck Surg. (1983) 197—202.
[7] F. Noel, A. Leung, First branchial cleft fistula: case reports and literature review, J. Otolaryngol. 16 (4) (1987) 247—251. [8] J.M. Triglia, R. Nicollas, V. Ducroz, P. Koltai, E. Garabedian, First branchial cleft anomalies: a study of 39 cases and a review of the literature, Arch. Otolaryngol. Head Neck Surg. 124 (1998) 291—295. [9] S.S. Choi, G.H. Zalzal, Branchial anomalies: a review of 52 cases, Laryngoscope 105 (1995) 909—913. [10] C. Stulner, Management of first branchial anomalies: report of two cases, Br. J. Oral Maxillofac. Surg. 39 (2001) 30—33. [11] Y. Ariyoshi, Determining whether a parotid tumor is in the superficial or deep lobe using magnetic resonance imaging, J. Oral Maxillofac. Surg. 56 (1998) 23—26. [12] P.D. Miller, Surgical excision of first cleft branchial fistulae, Br. J. Surg. 71 (1984) 696—697.
Available online at www.sciencedirect.com