Diagnosis and treatment of branchial cleft cysts and fistulae. A retrospective study of 183 patients

Int. Z Oral Maxillofac. Surg. 1996; 25:449-452 Printed in Denmark. All rights reserved

Copyright 9 Munksgaard 1996 InternationalJournal of

Ord &

Maxillofacial Surgery ISSN 0901-5027

Pathology

Diagnosis and treatment of branchial cleft cystsand fistulae. A retrospectivestudy of 183 patients

Francisco C. Agaton-Bonilla 1,2, Cosme Gay-Escoda 1 1Department of Oral and MaxillofaeialSurgery, School of Dentistry, Division of Health Sciences, University of Barcelona, Barcelona; 2Hospital de Mollet, Barcelona, Spain

E C. Agaton-Bonilla, C. Gay-Escoda: Diagnosis and treatment of branchial cleft cysts and fistulae. A retrospective study of 183 patients. Int. J. Oral Maxillofac. Surg. 1996; 25: 449-452. 9 Munksgaard, 1996 Abstract. A retrospective analysis of 183 patients undergoing surgical treatment for branchial cleft cysts and fistulae between 1970 and 1990 was carded out. There were 148 (80.8%) cases of branchial cleft cysts and 35 (19.2%) cases of branchial cleft fistulae. In patients with branchial cysts, the most frequent clinical presentation consisted of a left-sided, painless, cervical mass. Patients with a branchial fistula presented with persistent mucous discharge from a skin opening in the neck. The introduction of ultrasonography, computed tomography, and magnetic nuclear resonance imaging has improved the accuracy of traditional radiologic methods such as parotid sialography and fistulography. Complete excision under general anesthesia is the treatment of choice, and the procedure is associated with a low incidence of local complications and neurologic sequelae. The overall recurrence rate was 4.9% after a follow-up of 2 years.

Cysts and fistulae arising from the first and second branchial arches are relatively uncommon anomalies of the embryonic development,, involving soft tissues of the neck 5'15'16. A cyst, sinus, or fistula lying between the external auditory canal and the submandibular area originates from the first branchial cleft. -A fistula or cyst originating low in the anterior lateral region of the neck usually results from a malformation of the second branchial cleft. Typically, the fistula extends from the skin of the lower part of the neck upward along the anterior border of the sternocleidomastoid muscle, and then inward between the bifurcation of the carotid artery, to enter the posterolateral pharynx just below the tonsillar fossa. Cysts are typically found only in the

lower end of the tract just above the clavicle 1. The histogenesis of the branchial cleft cyst has been a matter of controversy. The thymic duct hypothesis appears to be unlikely because of the absence of unequivocal thymic tissue in the walls of most branchial cysts and the wide anatomic distribution of these cysts, particularly in the upper neck region 1~ In addition to the commonly accepted branchial theory, it has been postulated that branchial cysts arise from cystic alterations of epithelium derived from the parotid gland or from structures arising in the pharyngeal pouches trapped in cervical nodes. However, the nature of this epithelium has never been clarified 3. Although these developmental le-

Key words: first branchial cleft; second branchial cleft; cyst; fistulae; congenital anomalies. Accepted for publication 23 July 1996

sions are present at birth, they are most frequently diagnosed during the third and second decades and occur predominantly in women. No familial clustering has been described, and bilaterality has been reported in 1-30% of cases 11'17. The main clinical features of the branchial cleft cyst are the appearance of a left-sided, painless, cervical mass, episodes of inflammation, and, in the case of a branchial cleft fistula, the presence of persistent mucous discharge from the skin opening and occasional swelling. In some cases, the skin opening can be located in the external acoustic meatus, causing otorrhea or chronic external otitis. The treatment of choice is surgical excision under general anesthesia. A retrospective analysis of 183 pa-

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Fig. 1. Bilateral fistula of second branchial cleft.

tients with branchial cysts and fistulae was carried out. The main clinical features, diagnostic procedures, and surgical treatment are discussed.

Student's t-test was used to compare variables such as age, sex, time course, and clinical signs and symptoms. Data were analyzed with the Microsoft statistical package. Statistical significance was set at P<0.05.

Material and methods

Results

Between 1970 and 1990, 183 patients (48 men, 135 women) with congenital anomalies of the first and second branchial arches were diagnosed and treated at the departments of general surgery and maxillofacial surgery of four teaching hospitals in Barcelona, Spain. A total of 148 patients had branchial cleft cysts (42 men, 102 women), and 35 patients had branchial cleft fistulae (six men, 29 women). Their mean age was 23.8 years (range 12-58, SD 6.9). The clinical records and histopathologic reports of all patients were retrospectively reviewed. All patients underwent full clinical examination, as well as plain radiographic examination of the neck. All patients with fistulae underwent fistulography, but cystography was not performed in any patient. Sialography of the parotid gland was also performed, and, as of 1985, ultrasound examination, computed tomography (CT), and magnetic resonance imaging (MRI) were performed as indicated. Ultrasound examination was done in 50% of patients with cystic lesions and sialography in 60% of patients with lesions of the first branchial cleft. In order to confirm the diagnosis, CT scans were obtained in 44% of patients and MRI in 13%. Fistulography was combined with MRI in 15% of patients with fistulae. All patients underwent surgery. According to the clinical and surgical findings, the lesions were categorized as first or second branchial cleft cysts or fistulae. The patients were followed up for 2 years after surgery.

According to the type and origin of the anomaly, 35 patients (seven men, 28 women) had first branchial Cleft cysts and 113 patients (35 men, 78 women) had second branchial cleft cysts. Eleven patients (two men, nine women) had first branchial cleft fistulae, and 24 patients (four men, 20 women) had second branchial cleft fistulae. The mean age of patients with cysts was 23.6 years (SD 7.1), and that of patients with fistulae was 24.6 years (SD 6.1). Although there was no statistically significant difference with regard to age, there was a clear female predominance. However, a relationship between sex and type of lesion was not found. The mean period that the lesion was present was 18.3 months (range 1-84 months) for all patients. There were two cases of bilateral second branchial cleft fistulae (Fig. 1). No cases of familial clustering were detected. Seven (20%) patients with branchial fistulae had been previously operated and had presented with recurrences, but none of the patients with branchial cleft cysts had a history of previous surgery. This difference was not statistically significant.

The most frequent clinical features were a left-sided, painless, cervical mass which underwent episodes of inflammation, and the presence of persistent mucous discharge from a skin opening and occasional swelling. In the present series, no patient had otologic manifestations. The diagnosis of first branchial arch anomalies was mainly based on anatomic and clinical criteria including the position of the lesion and mode of presentation (cystic mass, persistent or intermittent drainage through a cutaneous opening), and radiologic criteria including sialography of the parotid gland and ultrasound examination. Since 1985, sonograms were systematically obtained in all patients. CT scans and MRI studies were usually performed, although not systematically (due to cost constraints), to confirm the diagnosis or to define the topographic relationships of a lesion. A similar diagnostic workup was used for second branchial arch anomalies, although studies of the parotid gland and ear-related symptoms were not considered. Fistulography was carried out in all patients with fistulae of either the first or second branchial arches. Although clinical presentation and radiologic examination were diagnostic in most patients, ultrasound-guided aspiration was carried out in 35 (23.6%) patients; it was diagnostic in 30 (85.7%)

Fig. 2. Fistulogram of bilateral fistula of second branchial cleft.

Branchial cleft cysts patients and inconclusive in five (14.3%). CT scans were done in 65 (44%) patients, and MRI was done in eight (12.3%) patients. All patients underwent surgical excision through a wide, transverse cervicotomy incision under general anesthesia with orotracheal or nasotracheal intubation. A drain was left in place for 72 h in all patients. Of the 148 branchial cysts, 47 (31.8%) were located on the right side and 101 (68.2%) on the left side. Of the 35 branchial fistulae, 11 (31.4%) were located on the right side, 22 (62.8%) on the left side, and two (5.7%) were bilateral (Fig. 2). The histopathologic reports confirmed the epithelial and benign nature of excised specimens. No signs of malignancy or degenerative changes were observed in any case (Fig. 3). Most cystic lesions were lined with stratified squamous epithelium and contained a brownish fluid with crystals of cholesterol. Columnar epithelial tissue was seen in some cases. In 10% of the cases, the wall contained lymphoid tissue arranged in a follicular pattern. There were no major complications related to surgery. Eighteen patients with branchial cysts and seven patients with branchial fistulae presented minor complications consisting of local infection (nine) and seroma/hematoma formation (16). Other postoperative complications were neurologic and aesthetic sequelae. Among the patients undergoing surgery for first branchial arch anomalies, two had facial nerve injury and one lingual nerve injury. Neurologic impairment was resolved in all three patients after 15 days to 3 months. Among the patients undergoing surgery for second branchial arch anomalies, four patients with branchial fistulae had neurologic damage (two had hypoglossal nerve injury and two had upper laryngeal nerve injury) that spontaneously resolved after an interval of 15 days to 2 months. Healing was aesthetically satisfactory in 147 (80.3%) patients. A visible scar was present in 36 (19.7%) patients. Red, hypertrophic scars were a cause of great concem to six (3.3%) young women. The mean length of hospital stay was 5 days (range 3-12 days) days for patients with brat~chial cysts and 7 days (range 4--15 days) for patients with branchial fistulae. The differences were not statistically significant.

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Fig. 3. A) Branchial cyst lined with stratified squamous epithelium and subepithelial lymphoid tissue (HE • B) inflammatory infiltrate intermingled with epithelial cells (HE •

The recurrence rate was slightly lower for branchial cysts (six patients, 4%) than for branchial "fistulae (three patients, 8.6%), but the difference was not statistically significant. The overall recurrence rate was 4.9%. Discussion

Age and sex distribution, period that the lesion was present, and rate of recurrence observed in the present study were similar to those reported by others 2'5'6. Cysts and fistulae of the first and second branchial arches were prev-

alent among women of a mean age at diagnosis of 23.8 years. Branchial cysts were more prevalent (80.8%) than branchial fistulae (19.2%). The diagnosis of first and second branchial arch anomalies has improved with the introduction of ultrasonography, CT, and MRI. The use of these tools has been strongly recommended 4'7, as they improve diagnostic accuracy and anatomic definition. Cytologic examination of aspiration samples obtained by ultrasound-guided puncture has also been recommended by s o m e authors 9'15A6. Such examina-

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tion greatly assists in the preoperative diagnosis, allowing us to exclude the presence of a metastatic or inflammatory adenopathy that would indicate an ill-advised surgical intervention. In the present series, no signs of malignant transformation or degenerative changes in the cytologic examination of aspiration samples were observed. For lesions of the first branchial cleft, a surgical incision like that used for the excision of the parotid gland is recommended, whereas a wide cervicotomy incision remains the method of choice for lesions of the second branchial cleft. In some first branchial arch anomalies, the resection of a small portion of the cartilage tissue of the external acoustic meatus is necessary to prevent recurrence. Other therapeutic modalities, such as the use of sclerotic agents or aspiration, may result in recurrence and may complicate the definitive surgical treatment 5. Dissection should be accurate in order to prevent nerve injury. Postoperative complications described in the literature include facial nerve injury, fistula formation, injury of the internal jugular vein, local infection, seroma or hematoma formation, and an unsightly scar9'12'16. In the present series, seven (3.5%) patients developed neurologic damage that spontaneously resolved after 2 months in all cases, and there were no cases of fistula formation or vascular injury. In two patients undergoing surgery for second branchial fistulae, exploration of a track into the pharynx caused hypoglossal nerve injury. Six young woman (3.3%) developed an unsightly scar that was a cause of great concern. Recurrence rates reported in the literature vary from 2% to 18% 13,17

With regard to pathogenesis, the branchial sinus concept does not seem to be likely because a track into the pharynx was not found in any of the patients with branchial cysts. Although most authors believe that lymph follicles are present in the walls of these cysts 3'10'14, the epithelial inclusion theory also appears to be unlikely since the presence of lymphoid tissue arranged in a follicular pattern was found in only 10% of the presented branchial cysts.

8. 9.

10. 11.

Acknowledgment. We are indebted to Marta Pulido, MD, for editorial assistance and copy-editing.

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Address:

Cosme Gay-Escoda Department of Oral and Maxillofacial Surgery School of Dentistry University of Barcelona Feixa Llarga s/n E-08907 L'Hospitalet de Llobregat Barcelona Spain