International Journal of Pediatric Otorhinolaryngology Extra
Volume 3, Issue 1 , Pages 20-23, January 2008

Third branchial cleft fistula infected with Actinomyces

  • Kay W. Chang

      Affiliations

    • Stanford University School of Medicine, Department of Otolaryngology - Head and Neck Surgery, 801 Welch Road, Stanford, CA 94305, United States
    • Corresponding Author InformationCorresponding author. Tel.: +1 650 736 1314; fax: +1 650 498 2734.
    • ,
  • Brian G. Lee

      Affiliations

    • Keck School of Medicine, University of Southern California, LAC USC Medical Center, Department of Otolaryngology, General Hospital 4136, 1937 Hospital Place, Los Angeles, CA 90031, United States
    • ,
  • Kathleen M. Gutierrez

      Affiliations

    • Stanford University School of Medicine, Division of Pediatric Infectious Disease, 300 Pasteur Drive Rm G312, Stanford, CA 94305, United States
    • Tel.: +1 650 723 5682; fax: +1 650 725 8040.

Received 2 August 2007; received in revised form 25 August 2007; accepted 28 August 2007. published online 08 October 2007.

Article Outline

Summary 

A 16-year-old male with a 10-year history of recurrent left lateral neck swelling and an associated draining fistula was taken to surgery for excision of the fistula. Histologic examination of the surgical specimen demonstrated findings consistent with Actinomyces species. We suspect that this patient had a third branchial cleft fistula that was infected with Actinomyces.

Keywords: Branchial cleft, Actinomyces

 

Branchial anomalies arise from the abnormal persistence of branchial apparatus remnants [1], and account for 17% of all pediatric cervical masses [2]. Third and fourth arch anomalies, which open into the piriform sinus, account for 3–10% of all branchial anomalies [1], [3]. For unknown reasons, these remnants almost always occur on the left side [4].

A complete third branchial cleft fistula follows a theoretic course which begins at the piriform sinus, pierces the thyrohyoid membrane, and travels inferiorly along the carotid sheath posterior to the internal carotid artery [3]. A fourth branchial cleft fistula would emerge from the piriform sinus between the thyroid and cricoid cartilages, descend in the tracheoesophageal groove, loop around the aorta, ascend and form a second loop around the hypoglossal nerve, and finally exit on the lateral aspect of the neck [5]. The key difference between the two lies in their association with the superior and recurrent laryngeal nerves. Third branchial fistulas pass superficial to both nerves whereas fourth branchial fistulas pass deep to the superior laryngeal nerve and superficial to the recurrent laryngeal nerve [3].

These lesions most commonly present as a recurrent lateral neck swelling and abscess, but can also present as suppurative thyroiditis [2]. Neck infections caused by branchial anomalies tend to recur despite adequate treatment with antibiotics [6]. Patient history, physical exam, a high index of suspicion, and clinical awareness are all essential in diagnosing branchial anomalies [7].

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1. Case report 

The patient is a 16-year-old male with a 10-year history of recurrent swelling on the left side of his neck. These episodes would resolve with antibiotic therapy. Two years before presentation at our clinic, the patient underwent an attempted excision of a possible thryoglossal duct cyst. At the time, there was no swelling present and the surgeon was unable to clearly identify a mass. In the following 2 years, the patient had three more episodes of swelling, one resulting in abscess.

On presentation, he was afebrile and clinically stable with no complaints of pain, cough, dysphagia, or hoarseness. Physical exam demonstrated bilateral thyroid fullness but without appreciable nodules or masses. There was a 3.5cm area of erythema on the skin overlying the left side of the thyroid. An MRI was performed, demonstrating a triangular area of increased signal and enhancement in the subcutaneous soft tissues along the left anterior neck, centered at the level of the thyroid, measuring 2.0cm×1.2cm. The enhancement appeared to extend into and involve the superior aspect of the left thyroid lobe, tracking superiorly along the medial margin of the left carotid sheath to the level of the piriform sinus (Fig. 1). These findings were most consistent with a third branchial cleft anomaly with inflammation involving the left piriform sinus and the superior aspect of the left thyroid lobe.

  • View full-size image.
  • Fig. 1. 

    Pre-treatment, axial, T1 weighted, post-gadolinium contrast MRI scans showing the fistula tract. The thick arrow shows the tract coursing towards the external ostium. The thin arrow shows the tract as it heads towards the left piriform sinus.

On examination in clinic, the patient had fullness over the left thyroid with a more palpable cystic mass, measuring approximately 1.5cm in diameter. There was a small dimple over the skin, just left of the midline, with no obvious drainage. The patient reported that this dimple had drained thick, yellowish fluid in the past. Flexible laryngoscopy revealed a dimpled area in the left piriform sinus not present on the right, which was thought to possibly represent the internal opening of a third branchial cleft fistula.

The patient underwent an excision of the suspected third branchial cleft fistula. A left partial hemithyroidectomy was also performed due to severe, chronic inflammation involving the thyroid. The dissection proved to be difficult due to the large amount of scar tissue and the lack of clear fascial planes. However, the entire anomaly was successfully excised.

Histologic sections of the surgical specimens (Fig. 2) revealed fibrofatty tissue with abscess formation and bacterial organisms morphologically consistent with Actinomyces species. Serial sectioning failed to show any areas of epithelialization. Cultures grew 2+ Streptococcus anginosus, and 1+ Eikenella corrodens, but no Actinomyces sp. were isolated. Acid fast and silver stains were negative.

  • View full-size image.
  • Fig. 2. 

    Histologic section from the surgical specimen. Actinomycosis is characterized by a mixed suppurative and granulomatous inflammatory reaction, connective tissue proliferation, and the presence of sulfur granules. The sulfur granules are practically pathognomonic for this infection. Low power view of a hematoxylin-eosin stained sulfur granule.

The patient was started on amoxicillin and referred to infectious disease clinic. Because of the recurrent and chronic nature of the patient's infection, the histologic findings consistent with Actinomyces species, and culture results showing likely polymicrobial infection, a percutaneous intravenous central catheter was placed and the patient was started on intravenous ceftriaxone, 2g/day. After 6 weeks, ceftriaxone was discontinued and the patient was started on oral amoxicillin, 500mg TID for 10 months. A post-treatment MRI with gadolinium contrast showed that the previously visualized left piriform sinus tract was no longer present. The patient remains well 5 years after treatment was discontinued.

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2. Discussion 

We suspect that this patient had a third branchial cleft fistula that was infected by Actinomyces spp. Unfortunately, a precise epithelium-lined tract was not identified in the surgical specimen. Due to recurrent abscesses and previous surgery, it is likely that postinfectious fibrosis obscured some of these anatomic relationships. Furthermore, it is generally recognized that the epithelium lining these tracts may be destroyed by recurrent infections [4].

Cervicofacial actinomycosis should also be included in the differential diagnosis of any head and neck mass. An alternative hypothesis in this particular patient is that he developed a suppurative thyroiditis [8]. However, due to the MRI findings of an enhancing tract extending from the skin to the left piriform sinus region, we favored the third branchial anomaly diagnosis.

Actinomyces israelii is the most common causative bacteria in human cases of actinomycosis, with the most common site of infection being the cervicofacial region (55%) [9]. It is a gram-positive, non-spore forming, facultative anaerobic bacillus that is part of the normal oral and gastrointestinal flora [10]. Positive cultures of this organism are only obtained in fewer than 50% of cases [11]. Poor recovery rate is attributed to the fastidious nature of the organism, improper culture conditions, and prior antibiotic use. Because microbiologic identification is so difficult to obtain, the diagnosis of actinomycosis is often based on clinical findings and histologic examination demonstrating characteristic sulfur granules. These granules are aggregates of branching bacteria [9]. Infections are often polymicrobial. Other bacteria, including E. corrodens and Streptococcus species have been designated as companion microbes [12], [13], [14].

Prolonged initial intravenous therapy is often necessary to eradicate infection caused by Actinomyces spp. Penicillin or ampicillin intravenously for 4–6 weeks, followed by oral penicillin for 6–12 months is usually the recommendation [15]. Ceftriaxone has been used successfully to treat actinomycetes, based on in vitro susceptibility testing of Actinomyces spp. and susceptibility patterns of “companion microbes” including E. corrodens, Streptococcus sp. and A. actinomycetemcomitans [16], [17].

A lateral neck mass in the pediatric patient could represent acute cervical adenitis with or without suppuration, a branchial cleft anomaly, a thyroid abscess, mycobacterial lymphadenitis, or actinomycosis. When it is associated with a draining sinus fistula, a branchial anomaly or actinomycosis is more likely. Because cultures of Actinomyces are poorly sensitive, and imaging cannot differentiate the two conditions, surgery may be the only definitive way to diagnose either by demonstration of a tract with the appropriate anatomic relationships or the characteristic histologic appearance of Actinomyces.

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References 

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PII: S1871-4048(07)00071-8

doi:10.1016/j.pedex.2007.08.007

International Journal of Pediatric Otorhinolaryngology Extra
Volume 3, Issue 1 , Pages 20-23, January 2008

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