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
|
---|
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
|
---|
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 12-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. 1
).

View larger version (159K):
[in this window]
[in a new window]
|
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. 2
). 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. 3
). Histologically, the thyroid gland
showed lymphocytic thyroiditis, including mature lymphocytes and
germinal centers. In addition, a focus of solid cell nests (Fig. 4
) 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.

View larger version (97K):
[in this window]
[in a new window]
|
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).
|
|

View larger version (108K):
[in this window]
[in a new window]
|
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.
|
|

View larger version (164K):
[in this window]
[in a new window]
|
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
|
---|
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. 5
) (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).

View larger version (40K):
[in this window]
[in a new window]
|
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
|
---|
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
|
---|
-
Berger SA, Zonszein J, Villamena P, Mittman N. 1983 Infectious disease of the thyroid gland. Rev Infect Dis. 5:108122.[Medline]
-
Raven RW. 1933 Pouches of the pharynx and
esophagus with special reference to the embyological and morphological
aspects. Br J Surg. 21:235256.
-
Takai S, Matsuzuka F, Miyauchi A, Kuma K, Kosaki
G. 1979 Internal fistula as a route of infection in acute
suppurative thyroiditis. Lancet. 1:751752.[Medline]
-
Miyauchi A, Matsuzuka F, Takai S, Kuma K, Kosaki
G. 1981 Piriform sinus fistula. Arch Surg.116:6669.
-
Nonomura N, Ikarashi F, Fujisaki T, Nakano Y. 1993 Surgical approach to pyriform sinus fistula. Am J
Otolaryngol.14:111115.
-
Yamashita J, Ogawa M, Yamashita S, Saishoji T, Nomura
K, Tsuruta J. 1994 Acute suppurative thyroiditis in an asymptomatc
woman: an atypical presentation simulating thyroid carcinoma. Clin
Endocrinol. 40:145150.[Medline]
-
Himi T, Kataura A. 1995 Distribution of C cells in
the thyroid gland with pyriform sinus fistula. Otolaryngol Head Neck
Surg.112:268273.
-
Bar-Ziv J, Slasky BS, Sichel JY, Lieberman A, Katz
R. 1996 Branchial pouch sinus tract from piriform fossa causing
acute suppurative thyroiditis, neck abscess, or both: CT appearance and
the use of air as a contrast agent. Am J
Radiol.167:15691572.
-
Burge D, Middleton A. 1983 Persistent pharyngeal
pouch derivatives in the neonate. J Pediatr Surg.18:230234.
-
Roediger WEW, Kalk F, Spitz L, Schmaman A. 1977 Congenital thyroid cyst of ultimobranchial gland origin. J Pediatr
Surg. 12:575576.[Medline]
-
Tucker HM, Skolnick ML. 1973 Fourth branchial cleft
(pharyngeal pouch) remnant. Trans Am Acad Ophthalmol
Otol.77:368371.
-
Sandborn W, Shafer A. 1972 A branchial cleft cyst
of fourth pouch origin. J Pediatr Surg. 7:82.[Medline]
-
Tovi F, Gatot A, Bar-Ziv J, Yanay I. 1985 Recurrent
suppurative thyroiditis due to fourth branchial pouch sinus. Int J
Pediatr Otolaryngol. 9:8996.[Medline]
-
Hirata A, Saito S, Tsuchida Y, et al. 1984 Surgical
management of piriform sinus fistula. Am Surg. 50:454457.[Medline]
-
Narcy P, Aumont-Grosskopf C, Bobin S, Manach Y. 1988 Fistulae of the fourth endobronchial pouch. Int J Pediatr
Otolaryngol. 16:157165.[Medline]
-
Edmonds JL, Girod DA, Woodroof JM, Bruegger DE. 1997 Third branchial anomalies. Arch Otolaryngol Head Neck Surg. 123:438441.[Medline]
-
Kubota M, Suita S, Kamimura T, Zaizen Y. 1997 Surgical strategy for the treatment of pyriform sinus fistula. J
Pediatr Surg. 32:3437.[Medline]
-
Makino S, Tsuchida Y, Yoshioka H, Saito S. 1986 The
endoscopic and surgical management of pyriform sinus fistulae in
infants and children. J Pediatr Surg.21:398401.
-
Taylor WE, Myer CM, Hays LL, Cotton RT. 1982 Acute
suppurative thyroiditis in children. Laryngoscope. 92:12691273.[Medline]
-
Rosenfeld RM, Biller HF. 1991 Fourth branchial
pouch sinus: diagnosis and treatment. Otolaryngol Head Neck
Surg.105:4450.
-
Doi O, Hutson JM, Myers NA, Mckelvie PA. 1988 Branchial remnants: a review of 58 cases. J Pediatr Surg. 23:789792.[Medline]
-
Ahuja AT, Griffiths JF, Roebuck DJ, et al. 1998 The
role of ultrasound and oesophagography in the management of acute
suppurative thyroiditis in children associated with congenital pyriform
fossa sinus. Clin Radiol. 53:209211.[Medline]
-
Katz R, Bar-Ziv J, Preminger-Shapiro R, Ben-Tovim R,
Rechnic Y. 1989 Pyogenic thyroiditis due to branchial pouch sinus. Isr J Med Sci. 25:641644.[Medline]
-
Miller D, Hill JL, Sun C, OBrien DS, Haller JA. 1983 The diagnosis and management of pyriform sinus fistulae in infants
and young children. J Pediatr Surg.18:377381.
-
DeLozier HL, Sofferman RA. 1986 Pyriform sinus
fistula: an unusual cause of recurrent retropharyngeal abscess and
cellulitis. Ann Otol Rhinol Laryngol. 95:377382.[Medline]
-
Shaw A. 1979 Acute suppurative thyroiditis. Am
J Dis Child. 133:757.[CrossRef]
-
English JS, Al-Hussani A. 1983 Recurrent
suppurative thyroiditis due to pyriform fossa-thyroid fistula. J
Laryngol Otol. 97:557559.[Medline]
-
Abe K, Fujita H, Matsuura N. 1981 A fistula from
pyriform sinus in recurrent acute suppurative thyroiditis. Am J
Dis Child. 135:178.
-
Godin MS, Kearns DB, Pransky SM, Seid AB, Wilson
DB. 1990 Fourth branchial pouch sinus: principles of diagnosis and
management. Laryngoscope.100:174178.
-
Lucaya J, Berdon WE, Enriquez G, Regas J, Carreno
JC. 1990 Congenital pyriform sinus fistula: a cause of acute
left-sided suppurative throiditis and neck abscess in children. Pediatr
Radiol. 21:2729.[Medline]
-
Goudreau E, Comtois R, Bayardelle P, Beauregard H,
Larochelle D. 1985 Capnocytophaga ochracea and group F
ß-hemolytic streptococcus suppurative thyroiditis. J Otolaryngol. 15:5961.
-
Montgomery GL, Ballantine TVN, Kleiman MB, Wright JC,
Reynolds J. 1982 Ruptured branchial cleft cyst presenting as acute
thyroid infection. Clin Pediatr. 21:380383.[Medline]
-
Ostfeld E, Segal J, Auslander L, Rabinson S. 1985 Fourth pharyngeal pouch sinus. Laryngoscope. 95:11141117.[Medline]
-
Schneider U, Birnbacher R, Schick S, Ponhold W, Schober
E. 1995 Recurrent suppurative thyroiditis due to pyriform sinus
fistula: a case report. Eur J Pediatr. 154:640642.[CrossRef][Medline]
-
Skuza K, Rapaport R, Fieldman R, Goldstein S, Marquis
J. 1991 Recurrent acute suppurative thyroiditis. J Otolaryngol. 20:126129.[Medline]
-
Hsin MKY, Barker GJ, Spitz L. 1998 Recurrent left
cervical abscess secondary to persistent pyriform sinus fistula. J R
Coll Surg Edinb. 43:125126.[Medline]
-
El-Naggar M, Flood LM, Naisby G, Wight R. 1997 Acute thyroid abscess in infancy as a complication of pharyngeal
fistula. J Otolaryngol. 26:136138.[Medline]
-
Karlan MS, Michel SL, Snyder WH. 1965 Branchiogenic
cysts: congenital or acquired. Am J Surg. 110:615619.[Medline]
-
Liu KKW, van Hasselt A. 1993 Piriform sinus
fistula- the role of endoscopy in its management. Otolaryngol Head Neck
Surg. 108:378379.[Medline]
-
Lin JN, Wang KL. 1991 Persistent third branchial
apparatus. J Pediatr Surg. 26:663665.[Medline]
-
Miyauchi A, Matsuzuka F, Kuma K, Takai S. 1990 Piriform sinus fistula: an underlying abnormality common in patients
with acute suppurative thyroiditis. World J Surg. 14:400405.[Medline]
-
Kingsbury BF. 1935 On the fate of the
ultimobranchial body within the human thyroid gland. Anat Rec. 61:155167.
-
Liston SL. 1981 Fourth branchial fistula. Otolaryngol Head Neck Surg. 89:520522.[Medline]
-
Miyauchi A, Matsuzuka F, Kuma K, Katayama S. 1992 Piriform sinus fistula and the ultimobranchial body. Histopathology. 20:221227.[Medline]
-
Janzer R, Weber E, Hedinger C. 1979 The relation
between solid cell nests and C cells of the thyroid gland: an
immunohistochemical and morphometric investigation. Cell Tissue Res. 197:295312.[Medline]
-
Williams ED, Toyn CE, Harach HR. 1989 The
ultimobranchial gland and congenital thyroid abnormalities in man.
J Pathol.159:135141.
-
Moore KL, Persaud TVN. 1998 The developing human:
clinically oriented embryology, 6th ed. Philadelphia: W.B. Saunders;
215233.
-
Goldman N. 1993 Piriform sinus fistula: the role of
endoscopy in its management. Otolaryngol Head Neck Surg. 108:378379.[Medline]
-
Bernard PJ, Som PM, Urken ML, Lawson W, Biller HF. 1988 The CT findings of acute thyroiditis and acute suppurative
thyroiditis. Otolaryngol Head Neck Surg. 99:489493.[Medline]