I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
RUNNING TITLE:
Complete branchial cleft fistula: diagnosis and surgical management AUTHORS: *
I. J. Keogh, M.D, FRCS (ORL-HNS), Senior Registrar, Otolaryngology / HNS
*
S. G. Khoo, AFRCSI, Specialist Registrar, Otolaryngology / HNS
K. Waheed, FRCS, Registrar, Otolaryngology / HNS C. Timon, FRCS (ORL-HNS), Professor of Otolaryngology / HNS * IJK & SGK contributed equally to this paper INSTITUTION: Department of Otolaryngology, Head and Neck Surgery (HNS), Royal Victoria Eye and Ear Hospital, Adelaid Road, Dublin 2, IRELAND. CORRESPONDING AUTHOR: Mr. Ivan J. Keogh, 243 Ros Caoin, Roscam, Galway, Ireland. Email:
[email protected] Tel: 00 353 87 2344399
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
SUMMARY: Branchial cleft fistulae are rare congenital abnormalities that arise from the abnormal persistence of branchial apparatus remnants. A complete fistula is a tract that has an internal opening and an external opening. Second branchial cleft fistulae pass deep to second arch structures and over third arch structures, in a direction extending from the anterior border of sternocleidomastoid (SCM) muscle to the upper pole of the ipsilateral tonsil fossa. Because of this anatomical route, these long tubular structures are intimately associated with major neuro-vascular structures in the neck. Fistulae are usually clinically apparent after birth with up to 80% being diagnosed before age 5 years. There may be an obvious opening in the anterior neck between the hyoid bone and suprasternal notch. Recurrent mucoid discharge becoming purulent during acute infection or associated with upper respiratory tract infection is the usual course. Treatment is complete surgical excision of all abnormally placed epithelium, while preserving surrounding neurovascular structures, and using cosmetically acceptable incisions. Complete fistulae in adults are rare and diagnosis can be difficult. We present the clinical presentation and surgical management of a long (14 cm) second branchial cleft fistula, in a 25-year-old female. Preoperative MRI scan of the neck clearly demonstrated the fistula. We recommend a “stepladder” or Mc Fee incision and intraoral pull-through fistulectomy to allow safe and complete excision.
Key - words: Branchial apparatus, branchial fistula, MRI scan, fistulectomy
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
INTRODUCTION: Von Baer first described the branchial apparatus in 1827 (1). The branchial apparatus appears during the fourth week of gestation and contributes to the major structural development of the head and neck. It consists of four pairs of well-defined mesodermal arches numbered sequentially from cranial to caudal. The arches are divided by endothelial-lined pouches internally and ectodermal lined clefts (grooves) externally. Ascherson described the first branchial cleft lesion in 1832 (2). Failure of resolution of the embryonic branchial apparatus leads to developmental abnormalities such as branchial cysts, sinuses and fistulae (2, 3). Cysts usually present as congenital lateral neck masses are lined by epithelium and have no external opening. Sinuses are blind pockets that extend a variable distance up the neck and are usually lined by respiratory epithelium. Sinuses may open internally (persistence of a pouch) or externally (persistence of a cleft). Fistulae are less common than sinuses and result from persistence of both branchial pouch and cleft elements (4). Structurally they are muscular tubes of variable lengths lined with respiratory or squamous epithelium. There is a slight female and right side predominance (5). Internal relations are dependent on the structures derived from the source branchial arch. Knowledge of these associated structures is critical for the safe removal of these lesions. Anomalies of the second cleft and pouch represent 60% to 90% of all branchial defects and approximately 10% are bilateral (5). Second branchial fistulae extend from the antero-lateral neck towards the pharynx and are rare (3).
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
Second branchial fistulae run through the platysma muscle and deep to the SCM, following the carotid sheath upward to the level of the hyoid bone. They then turn medially to pass over the hypoglossal nerve, under the posterior belly of the digastric muscle, between the internal and external carotid arteries, over the glossopharyngeal nerve and towards an internal opening at the upper pole of the tonsil (2, 3, 5). Complications are usually precipitated by respiratory tract infections (3, 6). Recurrent infection and or discharge are the usual indications for surgical excision (2, 3).
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
PATIENT AND METHODS: A 25-year-old female presented with a 5-year history of recurrent painless discharge from a skin pit overlying the right lower SCM. This discharge was noted to become more copious and purulent during respiratory tract infections. There was no history of childhood problems and she was medically fit otherwise. On clinical examination a firm cord like tract was palpable radiating from the skin pit inferiorly towards the right parapharyngeal space. Magnetic resonance imaging (MRI) clearly demonstrated a long tract extending up the right neck towards the ipsilateral parapharyngeal region (Photo 1). Clinical and radiological findings were consistent with a complete second branchial fistula. Because of the history of recurrent discharge and intermittent inflammation, a decision was taken with the patients consent, to excise the fistula tract under general anaesthetic. A metal probe was passed along the tract and an elliptical incision was made around the skin pit (Photo 2), dissection then proceeded through the subcutaneous tissues. The fistula was traced deep to the platysma muscle and the superficial layer of the deep cervical fascia. A second incision was made about the level of the hyoid bone (along Langer lines) and the tract was threaded through it. This approach allows identification and preservation of important neurovascular structures (Photo 3). The tract was then followed between the carotid arteries, superficial to the right hypoglossal nerve and into the oropharynx. By gently pulling on the fistula a small pit became apparent on the upper pole of the tonsil. The fistula was delivered through the right tonsil fossa after excision of a large portion of the tonsil (Photo 4).
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
The tract was dissected out in toto (14 cm), preserving the marginal mandibular, hypoglossal and glossopharyngeal nerves (Photo 5). A small redivac neck drain was placed deep to platysma and secured. Incisions were closed in layers, with skin clips externally. A temporary nasogastric feeding tube was passed. The drain was removed and oral feeding commenced after 48 hours. There were no postoperative complications and the patient was discharged home on full diet six days postoperatively.
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
DISCUSSION: Complete branchial cleft fistulae, with a deep parapharyngeal portion and a superficial infrahyoid portion are rare, and comprise 2 % of all branchial anomalies (1,3,7,8). The majority (80%) of fistulae are diagnosed before five years of age (5, 6). Persistent or recurrent discharge from the neck opening, with intermittent inflammation of the surrounding skin is the usual course. Preoperative imaging (CAT, MRI) is frequently unhelpful, however injecting the tract with contrast material (fistulogram) may demonstrate the extent of the tract (5, 9). Complete fistulae in adults are extremely rare and consequently a full differential diagnosis with appropriate imaging must be considered. What was unusual in this case was the total absence of symptoms until the patient reached her early 20’s. MRI scan was very helpful and clearly demonstrated a tubular structure in the right neck (Photo 1). It was possible to follow the course of the fistula and define important neurovascular relationships in the neck. It is possible that the maturity and length of the tract made it more apparent on the MRI scan. Complete surgical excision of all the offending epithelium is the treatment of choice (1, 3, 5, 9). Surgery is usually delayed until the infant is 2-3 years old (9). Acute infections should always be treated and resolved before any surgical intervention. Repeated infections and inadequate surgeries lead to more scarring and a more difficult definitive procedure. The cosmesis of surgical access in these young patients is an important issue. Vertical incisions should be avoided, as scarring can be unsightly. Small transverse incisions planned along Langer lines give a much better cosmetic outcome. With long fistulas, a “stepladder” or Mc Fee incision should allow
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
adequate exposure. Dissection can be aided by inserting a probe (Photo 2) or by injecting the tract with methylene blue dye. It is important to remove an ellipse of skin around the skin pit in continuity with the tract. In order to keep recurrence rates low it is vital to remove all epithelium (5, 9). This can be accomplished by removing a cuff of supporting connective tissue along with the tract (Photo 5). Despite the predictability of the eventual course of the fistula based on embryological development, different anatomical presentations must be considered. The pharyngeal opening can be identified by gently pulling on the fistula once it is dissected from the neck; this will reveal a small pit in the tonsil fossa. Tonsillectomy is performed and the fistula can be delivered via a pull-through intraoral fistulectomy (8). Complications include trauma to and dysfunction of any of the important neurovascular structures the fistula encounters travelling from neck to pharynx. The overall recurrence rate has been quoted as 8.6% after a 2-year follow-up, and is increased seven fold especially in those who have had previous surgery and recurrent infection (2, 3, 9). A functional neck dissection has been suggested as a treatment option for recurrent cervical branchial fistulae (2). This allows for the en bloc resection of epithelial remnants including the surrounding scar tissue.
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
CONCLUSION: Complete second branchial fistula are rare and usually diagnosed at birth. Magnetic resonance imaging of the neck may aid diagnosis. We recommend a stepladder excision and an intraoral pull-through fistulectomy for complete and safe removal.
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
REFERENCE: 1. DE PR, MIKHAIL T. A combined approach excision of branchial fistula. J LARYNGOL OTOL. 1995; 109: 999-1000.
2. BLACKWELL KE, CALCATERRA TC. Functional neck dissection for treatment of recurrent branchial remnants. ARCH OTOL HEAD NECK SURG. 1994; 120 (4): 417421.
3. ANG AH, PANG KP, TAN LK. Complete branchial fistula: case report and review of the literature. ANN OTOL RHINOL LARYNGOL. 2001; 110 (11): 1077-1079.
4. CHOI SS, ZALZAL GH. Branchial anomalies: a review of 52 cases. LARYNGOSCOPE. 1995; 105: 909-913.
5. J HOUCK. Excision of branchial cysts. OP TECH OTOL H N SURG. 2005 Sep; 16 (3): 213-222.
6. MARAN AG, BUCHANAN DR. Branchial cysts, sinuses and fistulae. CLIN OTOLARYNGOL. 1978; 3 (1): 77-92.
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
7. DE CALUWE D, HAYES R, MCDERMOTT M, CORBALLY MT. Complex branchial fistula: a variant arch anomaly. J PED SURG. 2001; 36 (7): 1087-1088.
8. TALAAT M. Pull-through branchial fistulectomy: a technique for the otolaryngologist. ANN OTOL RHINOL LARYNGOL. 1992; 101 (6): 501-502.
9. AGATON-BONILLA FC, GAY-ESCODA C. Diagnosis and treatment of branchial cleft cysts and fistulae, a retrospective study of 183 patients. INT J ORAL MAXILLOFAC SURG. 1996; 25 (6): 449-452.
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
LEGENDS FOR ILLUSTRATIONS Photo 1, MRI neck (axial): demonstrating the fistula tract on right (arrow).
Photo 2, Planned incisions with metal probe inserted in tract. Note elliptical incision planned around skin pit.
Photo 3, Stepped incisions in right neck. Fistula will be threaded through upper Mc Fee incision using an artery clip
Photo 4, Intra-oral delivery of the tract through the tonsillar fossa. A Boyle Davis Gag is used to keep the mouth open.
Photo 5, Complete branchial fistula (14 cm) with cuff of supporting tissue, ellipse of skin on left and tonsil tissue on right.
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
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I J Keogh et al, Complete branchial cleft fistula: diagnosis and surgical management
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