Recurrent Acute Suppurative Thyroiditis in an Adult Due to a Fourth Branchial Pouch Fistula

Jane A. Cases, Bruce M. Wenig, Carl E. Silver and Martin I. Surks

Departments of Medicine (J.A.C., M.I.S.), Pathology (B.M.W.), and Surgery (C.E.S.), Montefiore Medical Center and The Albert Einstein College of Medicine, Bronx, New York 10467

Address correspondence and requests for reprints to: Martin I. Surks, M.D., 111 East 210th Street, Bronx, New York 10467. E-mail: msurks{at}westnet.com


    Introduction
 Top
 Introduction
 Clinical Presentation
 Discussion
 Conclusion
 References
 
Acute suppurative thyroiditis is an uncommon infectious disease affecting mainly children and young adults (1). Recurrent acute suppurative thyroiditis due to persistent pyriform sinus-thyroid fistula is rare. There have been 109 cases of suppurative thyroiditis reported, mainly in the pediatric and otolaryngologic literature (2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40), since its first description in 1933 (2). Only 14 adult patients have been described (3, 4, 5, 6, 7, 8).


    Clinical Presentation
 Top
 Introduction
 Clinical Presentation
 Discussion
 Conclusion
 References
 
An otherwise healthy 25-yr-old female was referred for evaluation of recurrent left thyroid swelling and pain for 6 months. She first presented to her primary care physician with acute and painful thyroiditis that was preceded by recurrent left otalgia and pharyngitis. She was treated with antibiotics and nonsteroidal anti-inflammatory drugs with resolution of symptoms. Three similar episodes of acute left thyroiditis occurred at 1–2-month intervals. The last episode was significant for persistence of the swelling despite treatment with antibiotic and nonsteroidal anti-inflammatory drugs. On presentation, she denied recent fever or pain on the left side of the neck. She had no significant past medical history or history of radiation exposure. There was no family history of cancer or thyroid disease. On examination, she was afebrile and vital signs were normal. The right thyroid lobe appeared normal, but a 2.5 x 3.0-cm nontender, left thyroid mass was palpated that was very firm, nonerythematous, and relatively fixed. There was no cervical lymphadenopathy. She was clinically euthyroid. Laboratory tests, especially the thyroid function tests, were all within the normal range, and thyroid antibodies were absent. Fine-needle aspiration biopsy obtained about 0.1 mL-thick yellowish fluid. The fluid was acellular. No culture of the fluid was done. Thyroid scan revealed diminished uptake of [123I] throughout the left lobe, with normal uptake in the right lobe (Fig. 1Go).



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Figure 1. Thyroid scan revealed diminished uptake of [123I] throughout the left lobe, with normal uptake in the right lobe.

 
Barium swallow demonstrated a fistulous tract, approximately 2 cm in length, extending inferiorly from the inferior aspect of the left pyriform sinus (Fig. 2Go). Direct laryngoscopic examination revealed a small opening at the apex of the left pyriform sinus. One week after the biopsy, she had another episode of acute suppurative thyroiditis. The mass in the area of the left lobe of the thyroid was exquisitely tender and fluctuant. The overlying skin was erythematous. This was empirically treated with amoxicillin/clavulanic acid, and the mass was incised and drained. Two weeks later, left hemithyroidectomy and fistulectomy via a left lateral pharyngotomy approach was carried out. The resected specimen included a left lobe of the thyroid with attached skeletal muscle and a fistulous tract. The latter measured 1.6 cm in length and 0.2 cm in diameter. The tract was probe patent along most of its length, but not into the thyroid gland. The resected thyroid lobe measured 3.8 x 2.4 x 2.1 cm and was grossly unremarkable. The light microscopic features included the presence of a squamous epithelial-lined fistulous tract. The wall of the fistulous tract was fibrotic and included a dense mixed chronic inflammatory cell infiltrate composed of mature lymphocytes, histiocytes, and scattered plasma cells. An acute inflammatory cell infiltrate was not seen. Focally, the fistulous tract superficially entered the thyroid gland with close approximation of the fistulous tract epithelium to thyroid follicular epithelium (Fig. 3Go). Histologically, the thyroid gland showed lymphocytic thyroiditis, including mature lymphocytes and germinal centers. In addition, a focus of solid cell nests (Fig. 4Go) representing a remnant of the ultimobranchial body was identified near to where the fistulous tract approximated the thyroid gland. Immunohistochemical stains showed the fistulous tract epithelium to be reactive with kermix (combined low- and high-molecular weight keratin) and with the low-molecular weight keratin CAM 5.2; this epithelial lining was not reactive with thyroglobulin, calcitonin, or neuroendocrine markers (e.g. chromogranin). The thyroid follicular epithelium showed immunoreactivity with thyroglobulin. In addition, calcitonin- and chromogranin-positive C cells were seen near the fistula. The C cells were inconspicuous by light microscopy. The postoperative course was uneventful, and the patient remains asymptomatic to date.



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Figure 2. Barium swallow (frontal view) demonstrating a sinus tract (F, middle arrow) extending inferiorly from the inferior aspect of the left pyriform sinus (P, top arrow). E, Esophagus (bottom arrow).

 


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Figure 3. Histological specimen of a pyriform sinus-thyroid fistula demonstrating a squamous epithelial-lined fistulous tract (A) extending into the superior pole of the left lobe of the thyroid gland (B). Hematoxylin and eosin.

 


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Figure 4. Solid cell nests are seen (arrows) representing a remnant of the ultimobranchial body. The solid cell nests were located near to where the fistulous tract approximated the thyroid gland. Hematoxylin and eosin.

 

    Discussion
 Top
 Introduction
 Clinical Presentation
 Discussion
 Conclusion
 References
 
Clinical findings

Eighty percent of patients with recurrent acute suppurative thyroiditis due to persistent pyriform sinus-thyroid fistula present during the first decade of life (mean age, 7.6 yr; range, birth to 56 yr) (7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40); 30% between birth and 2 yr (2, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 24, 25, 26); and 8% during adulthood (3, 4, 5, 6, 7, 8). One third of adults recalled similar illness during childhood (4, 5). Children and adults usually have painful neck swelling accompanied by fever that is preceded by upper respiratory infection, otalgia, and odynophagia (3, 4, 5, 7, 8, 14, 15, 16, 17, 18, 19, 22, 23, 24, 25, 29, 30, 31, 32, 34, 35, 36). Neonates and infants may have respiratory distress, sometimes with stridor, due to tracheal compression by the abscess (2, 8, 9, 10, 11, 20, 25). The neck mass presents as acute suppurative thyroiditis in 67% of cases (3, 4, 5, 6, 7, 9, 10, 14, 15, 16, 19, 20, 22, 24, 25, 29, 30, 31, 32, 33, 35, 36, 39), the remainder either a lateral neck mass or a cervical fistula. Ninety-two percent of cases involve the left thyroid lobe (2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18), 6% the right lobe (8, 18, 20, 21, 22, 23), and 2% are bilateral (25, 26). The left-sided predominance may be due to embryological asymmetry of the transformation of the fourth branchial arch to form the aortic and innominate arteries (41) or to poor development of the ultimobrachial body on the right side of the embryo (42). About 50% of patients have three or more episodes of thyroiditis before the diagnosis is made (3, 4, 5, 16, 17, 18, 24, 25, 26, 29, 30, 35). Laboratory findings are significant for leucocytosis and normal thyroid function. Normal oropharyngeal flora has frequently been cultured from the infected thyroid, suggesting communication with the oropharynx (4, 5, 6, 11, 13, 14, 18, 19, 24, 27, 30, 32, 35, 36). The internal opening of the fistula is at the apex of the pyriform fossa (43), a recess in the pharynx that is exposed to secretions in the normal state and during upper respiratory tract infections.

The sinus tract courses antero-inferiorly from the pyriform sinus and ends blindly either in the perithyroid tissues or in the parenchyma of the thyroid (4, 43). In the former case, the tract causes an abscess in the soft tissues of the neck by contiguous spread that may involve the thyroid and other spaces of the neck including the retropharynx (25). In the latter case, as in the patient described in this report, the presentation is acute thyroiditis.

Embryology

There are four well-defined pairs of pharyngeal pouches and a fifth pair that is absent or rudimentary and is incorporated into the fourth pouch. The embryological precursor of the pyriform sinus fistula has been considered to be either a remnant of the third or fourth pouch. Proponents of third pouch origin rely heavily on the histologic presence of thymus tissue within the wall of the fistulous tract because the third pouch gives rise to the inferior parathyroid glands and the thymus (4, 24, 44).

The ultimobranchial body is a remnant of the fourth branchial pouch. This migrates caudally and laterally fusing with the lateral lobe of the thyroid, giving rise to C cells and compact cell nests (Fig. 5Go) (44, 45, 46). Miyauchi et al. (44) in 1992 demonstrated aggregates of many C cells in the thyroid near the fistula and a few within the fistula. Similar findings were identified in our case. These findings, along with the anatomical relation of the fistula to major structures of the neck and its migration path, strongly suggest that the fistula is a remnant related to the ultimobrachial body of the fourth branchial pouch (7, 44, 47).



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Figure 5. Schematic horizontal section illustrating the adult derivatives of the pharyngeal pouches (II, second pharyngeal pouch; III, third pharyngeal pouch; IV, fourth pharyngeal pouch) and migration path of the developing ultimobranchial body (arrows) into the neck in a 7-week embryo (adapted from Ref. 47).

 
Diagnosis

The diagnosis of a persistent pyriform sinus-thyroid fistula requires an index of suspicion. A recurrent left neck abscess or acute thyroiditis that does not respond to appropriate antibiotic and surgical therapy should raise suspicion of this entity. Fine-needle aspiration biopsy should be the initial diagnostic test, especially when malignancy is being considered. Culture of the aspirate should be done to guide antimicrobial therapy. Barium swallow has been advocated as an effective means of demonstrating the presence of an anomalous tract. It has a sensitivity of 80% (3, 4, 5, 6, 11, 13, 14, 15, 17, 18, 19, 20, 22, 23, 24, 27, 28, 30, 34, 35, 36). False negative results are mostly due to edema around the orifice of the tract (5, 8, 9, 15, 16, 22, 23, 25). Edema occurs during acute infection and may prevent contrast medium from entering the sinus tract (24). Thus, the optimal time for this study is the quiescent stage after antibiotic therapy. Further confirmation of a sinus tract may be obtained by direct laryngoscopy (5, 8, 14, 15, 16, 17, 18, 29, 31, 34, 39, 48). Computed tomography scan is the technique of choice to assess the extent of infection (8, 49).

Treatment

Cure is achieved only by complete resection of the sinus tract, including that portion of the thyroid where the sinus tract terminates (3, 5, 18, 41, 48). A Fogarty catheter may be used to cannulate the tract (5, 14, 17, 18, 29, 39), and dye studies using methylene blue (17) may be used to facilitate identification and dissection of the tract when fascial planes are altered by recurrent infection or previous unsuccessful surgeries. Incision and drainage and antibiotic treatment may be used as temporizing measures before definitive surgery.


    Conclusion
 Top
 Introduction
 Clinical Presentation
 Discussion
 Conclusion
 References
 
Recurrent acute suppurative thyroiditis due to persistent pyriform sinus-thyroid fistula is likely more common than previously believed and usually becomes symptomatic before age 10. We suggest that clinicians should suspect this disorder whenever an inflammatory process or abscess is present in the lower neck, especially when recurrent and on the left side. There are two aspects to consider in the investigation of a pyriform sinus fistula. First, fine-needle aspiration biopsy and culture of the tissue should be done to confirm the presence of acute suppurative thyroiditis. Second, barium esophagography should be done to define the presence of a pyriform sinus fistula during periods of quiescence. These may be complemented with direct laryngoscopy. Total excision of the sinus tract is the definitive treatment.

Received September 29, 1999.

Revised November 23, 1999.

Accepted December 6, 1999.


    References
 Top
 Introduction
 Clinical Presentation
 Discussion
 Conclusion
 References
 

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