International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 4 , Pages 301-305, December 2006

Buccal salivary cysts in association with a congenital parotid salivary fistula

  • Devraj Basu

      Affiliations

    • Department of Otorhinolaryngology-Head and Neck Surgery, University of Pennsylvania Health System, 3400 Spruce Street, 5 Ravdin, Philadelphia, PA 19104, USA
    • Corresponding Author InformationCorresponding author. Tel.: +1 215 349 5390; fax: +1 215 349 8953.
    • ,
  • Frances V. White

      Affiliations

    • Department of Pathology and Immunology, Washington University School of Medicine, Box 8118, 660 S. Euclid Avenue, St. Louis, MO 63110, USA
    • ,
  • David W. Molter

      Affiliations

    • Department of Otolaryngology-Head and Neck Surgery, Washington University School of Medicine, Box 8115, 660 S. Euclid Avenue, St. Louis, MO 63110, USA

Received 27 June 2006; received in revised form 8 September 2006; accepted 8 September 2006.

Article Outline

Summary 

Congenital parotid salivary fistulas are unusual entities that can arise from accessory parotid glands or, even more infrequently, from normal parotid glands via an aberrant Stensen's duct. A unique case of a congenital parotid salivary fistula presented in a four-week-old infant as polycystic swelling of the buccal region, with cyst contents draining to a cutaneous pit near the oral commissure. This patient offered an initial diagnostic challenge until it became evident that the drainage represented a salivary fistula. The case is used here to highlight the overlapping clinical and pathologic features shared between type I branchial cleft anomalies and congenital salivary cysts. This case is the second report of buccal salivary cysts in structural association with a congenital parotid salivary fistula and the first such case in which a normal Stensen's duct orifice has been preserved.

Keywords: Congenital malformation, Salivary cyst, Branchial cleft cyst, Salivary fistula, Parotid fistula, Parotid duct malformation, Buccal cyst, Parotid fistula, CT sialography

 

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1. Introduction 

The differential for benign congenital cysts of the parotid region includes type I branchial cleft cysts, lymphatic malformations, salivary cysts, and dermoid cysts. The association of a connected draining cutaneous pit suggests a diagnosis of a type I branchial cleft anomaly when such lesions are encountered. Congenital salivary fistulas draining to the skin are more unusual entities than type I branchial cleft anomalies and are often not associated with cysts. They present as cutaneous pits arising from the parotid gland, submandibular gland, or ectopic salivary tissue [1], with labial pits usually associated with minor salivary glands. Occasional salivary fistulas can arise from accessory parotid glands, which are themselves common entities, present in 21% of anatomic specimens [2]. Normally these accessory glands merely drain to the Stensen's duct via two or three direct connections. However, multiple case reports exist of accessory parotid glands giving rise to congenital salivary fistulas to the cheek skin and various other sites [3], [4], [5].

Congenital salivary fistulas arising directly from the parotid gland are exceedingly rare, and their structural association with congenital salivary cysts outside the parotid gland is nearly unprecedented in the literature. Here we present a unique case of a congenital parotid salivary fistula presenting with pronounced polycystic swelling of the cheek and draining to a cutaneous pit near the oral commissure. The diagnostic evaluation, treatment planning, and histologic interpretation of this lesion all posed challenges that are described here. The case is reviewed in context of the scant existing literature on congenital salivary cysts and fistulas.

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2. Case report 

A four-week-old healthy term female infant with unremarkable prenatal history presented to our emergency department one day following acute onset of large right cheek swelling in association with an exam finding of a cutaneous pit just lateral to the right oral commissure (Fig. 1). There were no signs of infection noted, and an ultrasound demonstrated three submucosal cystic structures within the left buccal region. The patient was managed conservatively, with followup planned pending further imaging. However, two days later, the patient was admitted with purulent drainage from the right cheek pit, from which cultures grew P. mirabilis and community-acquired oxacillin-resistant S. aureus.

Treatment with intravenous antibiotics and expression of purulence from the cyst produced rapid resolution of infection, although residual buccal swelling anterior to the Stensen's duct orifice persisted. Magnetic resonance imaging (MRI) was performed, revealing three buccal cysts located anterior to the ramus of the mandible at a submucosal location. The favored diagnoses at this point included an unusually placed first branchial cleft anomaly versus a lymphatic malformation with an atypical drainage tract.

Despite resolution of infection, these cysts continued daily to accumulate fluid, which could be expressed as a turbid liquid resembling saliva. An amylase level was thus sent on the fluid and was assayed at 1300IU/L. This finding prompted a computed tomography (CT)-sialogram to define the structural relationships between the cysts, the pit, the native Stensen's duct, and the parotid gland. Cannulation and injection of either the native Stensen's duct or the cheek skin pit directly filled the same three buccal submucosal cysts with contrast material (Fig. 2). Further injection of the cysts caused retrograde filling of a normal-appearing Stensen's duct proximal to the cyst complex with contrast material (Fig. 3). This proximal duct extended to the hilum of a normal-appearing parotid gland.

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  • Fig. 3. 

    CT sialogram showing further contrast injection causes retrograde filling of a normal proximal Stensen's duct (arrow) extending to the hilum of the parotid gland.

Given the fluid reaccumulation and associated likelihood of recurrent infection, the patient was taken to the operating room at six weeks of life to undergo conservative excision of this complex malformation. The pit was cannulated and used to distend the cyst complex with saline solution, and the Stensen's duct was cannulated as a landmark. The two more anterior cysts appeared to lie entirely anterior to the Stensen's duct, while the posterior-most cyst was in close association with the duct. The anterior-most cyst was marsupialized into the oral cavity and then excised in continuity with both its tract to the cheek skin and the communicating middle cyst. The posterior cyst was identified and, after ligation of its communication with the middle cyst, was left in situ because of concerns regarding both injury risk to distal facial nerve branches and the intimate relation of the posterior cyst to the Stensen's duct. The patient's postoperative course was unremarkable, and she has not had recurrent infection or swelling of the remaining salivary cyst after 15 months of followup.

Microscopic examination of the pathologic specimen revealed fragments of cyst wall lined predominantly by squamous mucosa without adnexal structures. A small portion of cyst wall contained pseudostratified epithelium with ciliated columnar cells. Salivary gland acini, cuboidal ductal epithelium, and lymphoid tissue were not present (Fig. 4).

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  • Fig. 4. 

    (A) The cyst wall is lined by both squamous epithelium and pseudostratified columnar epithelium. (B) A higher magnification of the pseudostratified columnar epithelium shows focal ciliated cells.

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3. Discussion 

Branchial cleft cysts and salivary cysts have overlapping pathologic features, thus sometimes requiring interpretation using the anatomic context from which they were excised. As is seen in our patient's salivary cysts, branchial cleft cysts are also typically lined by squamous epithelium, pseudostratified ciliated columnar epithelium or a mixture of both types of epithelium. Most congenital salivary cysts, in contradistinction to our specimen, occupy a glandular intraparenchymal location. Salivary cysts may be congenital or acquired and have been categorized as salivary duct cysts, lymphoepithelial cysts, and dysgenetic congenital cysts [6]. So-called lymphoepithelial cysts are designated based on the presence of significant lymphoid tissue in the cyst wall, as can also be present in branchial cleft cysts. Dysgenetic congenital cysts designate congenital complexes of cysts, as present in our patient. Salivary cysts may contain cuboidal ductal epithelial epithelium but may also bear metaplastic squamous linings resembling both those of branchial cleft cysts and the lining of our specimen.

This inherent confusion between salivary cysts and type I branchial cleft cysts is apparent in the old literature, where cases of congenital intraparotid cysts without draining tracts have been reported as branchial cleft cysts [7], [8]. In modern context, these cystic malformations could easily be reinterpreted as salivary cysts. Our initial misinterpretation of our case as an unusual type I branchial cleft cyst was supported by the extraglandular location of the cysts and the presence of a draining cutaneous pit. Type I branchial cleft anomalies are variable in their cutaneous connections; external openings are found at postauricular locations, at angle of mandible, lateral to body of mandible, or even below the mandible [9]. However, a branchial cleft anomaly tract opening at the oral commissure would be unprecedented in the literature.

The application of CT sialography allowed us to characterize our lesion as comprised of salivary cysts in association with a parotid salivary fistula. While congenital cutaneous salivary fistulas typically arise from minor salivary tissue, several cases of accessory parotid tissue giving rise to salivary fistulas have also have been reported. However, congenital cutaneous fistulas arising from the parotid gland proper are truly rare entities. Rare case reports do exist of congenitally aberrant Stensen's ducts with cutaneous orifices [10], [11]. One of these reports [11] included a fistula with a cystic component: a seven-year-old male with a parotid fistula draining at a labial pit and comprised of an aberrant Stensen's duct in association with a buccal submucosal cyst. In that report, the authors propose an embryologic mechanism involving the parotid bud becoming associated with the labial sulcus before the gland's normal posterior migration into the mesenchyme of the first and second arches. While this patient's anatomic traits closely resemble our patient's lesion, our case is differentiated by the additional preservation of a native functional Stensen's duct orifice. Although other congenital duplication anomalies of Stensen's duct have been described [12], an associated complex cystic malformation with a widely separated normal and aberrant Stensen's duct orifices has not previously been reported.

Surgical strategies for managing congenital salivary fistulas can involve either excising the fistula tract or translocating the fistula orifice into the oral cavity [3], [4], [5]. Excision was favored in our case because of both the presence of a normal Stensen's duct orifice and the need to remove the cystic structures in communication with the salivary fistula. Our intraoral approach was facilitated by the immediately submucosal location of the two more anterior cysts, and the most posterior cyst was left in situ because of its intimate relation to the normal Stensen's duct as well as distal facial nerve branches. While complete excision would have been optimal, our initial conservative resection minimized risk of injury to these structures and has not led to recurrence of swelling or infection to date.

In summary, here we report a diagnostically challenging case that highlights the overlapping clinical and pathologic features between type I branchial cleft cysts and congenital salivary cysts. While most congenital cutaneous salivary pits arise from minor salivary glands, rare congenital salivary fistulas can arise from either accessory parotid glands or normal parotid glands via aberrant Stensen's ducts. Here we have reported the second case of buccal salivary cysts in structural association with a parotid salivary fistula and the first such case in which a normal Stensen's duct orifice is simultaneously present.

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References 

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PII: S1871-4048(06)00088-8

doi:10.1016/j.pedex.2006.09.003

International Journal of Pediatric Otorhinolaryngology Extra
Volume 1, Issue 4 , Pages 301-305, December 2006

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