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Congenital Foregut Duplication Cysts of the Anterior Tongue
Debbie Eaton, MD;
Kathleen Billings, MD;
Charles Timmons, MD, PhD;
Timothy Booth, MD;
J. Michael J. Biavati, MD
Arch Otolaryngol Head Neck Surg. 2001;127:1484-1487.
ABSTRACT
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Objective To review our experience with foregut duplication cysts of the anterior
tongue, an unusual and rarely encountered mass in this location.
Design A retrospective review of patients with anterior tongue foregut duplication
cysts identified between 1990 and 2000.
Setting Academic, tertiary care children's medical center.
Patients Six pediatric patients (5 boys and 1 girl) ranging in age from birth
to 8 months at diagnosis.
Intervention Three patients underwent preoperative magnetic resonance imaging (MRI).
All 6 patients underwent excisional biopsy.
Main Outcome Measures Clinical description of foregut duplication cysts, ability to make the
diagnosis preoperatively, and recurrence rates.
Results No patient presented with respiratory compromise, despite the large
size of the anterior tongue masses (range, 1.5-2.4 cm). An MRI study was performed
in 3 patients, all given a presumptive diagnosis of dermoid cyst based on
the radiographic findings. No patient was diagnosed correctly prior to surgical
excision. All patients underwent surgical excision, and the average time from
birth to surgical excision was 11 months (range, 3 days to 3.7 years). Surgical
pathologic findings were reported as a foregut duplication cyst (enterocystoma)
in all patients, with 3 specimens containing foci of gastric mucosa. No recurrence
has occurred at 1-month follow-up.
Conclusions Foregut duplication cysts rarely present in the anterior tongue and
are easily misdiagnosed preoperatively. An MRI study is helpful in preoperative
planning, although all lesions were radiologically indistinguishable from
dermoid cysts. These masses may be an underappreciated entity in the differential
diagnosis of congenital anterior tongue masses.
INTRODUCTION
FOREGUT (enteric) duplication cysts are classified as choristomas containing
heterotopic islands of gastrointestinal mucosa. They consist of a cystic wall
composed partly of stratified squamous epithelium and partly of gastrointestinal
(columnar) epithelium. Variable amounts of parietal or chief cells, goblet
cells, argentaffin cells, and Paneth cells are present.1
Smooth muscle is usually identified surrounding the cyst. Though enteric duplication
cysts occur anywhere from the oral cavity to the rectum, they are rare in
the oral cavity; only 21 cases have been reported.2-3
Most present asymptomatically shortly after birth, but the location of these
lesions has the potential to cause respiratory and feeding difficulties. Magnetic
resonance imaging (MRI) is the study of choice, but the diagnosis is made
only with excisional biopsy. Treatment is surgical excision, with no reported
recurrences. The cases presented here demonstrate the difficulty in making
the diagnosis preoperatively.
PATIENTS AND METHODS
This was a retrospective medical record review of children who underwent
excision of foregut duplication cysts of the tongue. Between 1990 and 2000,
6 patients at Children's Medical Center in Dallas, Tex, underwent excision
of anterior tongue cysts determined postsurgically to be enterocystomas.
Hospital, clinic, and surgical records were reviewed for age at diagnosis,
presenting symptoms, sex, site of lesion, age at surgical excision, histopathologic
and preoperative imaging findings, complications, and recurrences. All procedures
were performed in the operating room at Children's Medical Center in Dallas.
All patients were extubated postoperatively and had no postoperative complications.
No recurrences were identified at 1-month follow-up.
RESULTS
Table 1 summarizes the pretreatment
and histopathologic patient data. Five patients presented at birth with a
mass in the anterior tongue/floor of the mouth (FOM) region (Figure 1). Two of these patients were asymptomatic, and 3 presented
with difficulty feeding. The sixth patient was noted at birth to have a large
protruding tongue, but an anterior FOM cyst was not recognized until age 8
months. No patient presented with respiratory compromise, despite the large
size of the anterior tongue masses (range, 1.5-2.4 cm).
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Pretreatment and Histopathologic Characteristics of Patients With Foregut
Duplication Cysts of the Anterior Tongue*
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Figure 1. Patient is a 2-day-old boy with
a cystic mass on the anterior floor of the mouth.
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The differential diagnosis included dermoid cyst, ranula, cystic hygroma,
hemangioma, and thyroglossal duct cyst. An MRI study was performed in 3 patients
preoperatively. In all patients, the mass was hyperintense on T2 and short
T1 inversion recovery images (consistent with a dermoid cyst). All cysts were
nonenhancing under intravenous contrast, but variable intensities on the T1-weighted
images were noted (Figure 2 and Figure 3).
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Figure 2. Midline sagittal T1-weighted image
of a 10-month-old boy. A well-defined mildly and uniformly hyperintense mass
is present within the anterior floor of the mouth (arrow).
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Figure 3. Midline sagittal T1-weighted image
of a 2-day-old boy. A predominantly isointense mass is present within the
anterior tongue, and a focus of increased signal (arrow) appears within the
anterior aspect of the mass, corresponding to dermal elements found pathologically.
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The average time from birth to surgical excision was 11 months (range,
3 days to 3.7 years). All patients were found to have foregut duplication
cysts, with 3 specimens containing foci of gastric mucosa (Figure 4 and Figure 5).
Patient 2 was found to have a coexisting dermoid in the sublingual area, corresponding
to an area of increased signal in the anterior aspect of the mass noted on
the T1-weighted image (Figure 3).
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Figure 4. Intraoperative dissection showing
a well-defined, cystic mass on the floor of the mouth.
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Figure 5. Gross specimen of a congenital
foregut duplication cyst, cut open, revealing smooth mucosal lining. The units
indicated on the ruler are centimeters.
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COMMENT
Enteric duplication cysts are cystic lesions containing a gastrointestinal
mucosal lining. Some consist of only a mucosal lining, and others have a multilayered
wall of mucosa, submucosa, and muscularis propria (Figure 6 and Figure 7).
Three lesions in this series contained foci of gastric mucosa. They may be
multiple and occur anywhere from the oral cavity to the anus, though they
are rare in the oral cavity, with only 0.3% reported in the tongue.4
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Figure 6. Gastric foveolar epithelium (hematoxylin-eosin,
original magnification x10).
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Figure 7. Respiratory epithelium, mucous
glands, inner layer of smooth muscle, and outer layer of skeletal muscle (hematoxylin-eosin,
original magnification x4).
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The mucosa observed in the cyst may not correspond to the normal gastrointestinal
mucosa at the anatomic level of the cyst, and mixed mucosal types within a
single cyst are common. The endodermal derivation of the respiratory mucosa
is reflected in the frequent presence of ciliated columnar epithelium in these
cysts. Thus, although pure esophageal, gastric, and intestinal duplications
are seen, the cysts often defy anatomic categorization and are more conveniently
designated by the generic name of foregut duplication or foregut cyst. Bronchogenic
cysts belong to this same group, being distinguished mainly by the presence
of hyaline cartilage in the wall.
The embryology of duplications in the tongue and FOM region is unclear,
and several theories on the pathogenesis have been proposed. Tongue development
begins in the fourth week of gestation with the formation of the tuberculum
impar (median tongue bud) and lateral lingual swellings from the first branchial
arch mesenchyme. The lateral lingual swellings ultimately overgrow the tuberculum
impar and form the anterior two thirds of the tongue. The posterior one third
of the tongue is formed from third and fourth branchial arch mesenchyme. The
mucosa of the anterior two thirds of the tongue is of ectodermal origin, whereas
the posterior one third is of endodermal origin.4
Four main theories have been proposed to explain how gastric mucosa
(endoderm) becomes incorporated into the tongue. Gorlin and Jirasek1 suggested in 1970 that heterotopic gastric mucosa
may be derived from entrapped embryonic gastrointestinal epithelium or ectoderm
from the primitive stomodeum. This theory fails to explain the presence of
esophageal, intestinal, or colonic mucosa anterior to this area.
Another theory, developed by Daley et al5
in 1984, proposed that endodermal cell rests of the stomodeum became trapped
by the lateral lingual swellings and were subject to inductive influences
causing differentiation into gastrointestinal epithelia. In 1988, Woolgar
and Smith6 performed a mucin histochemical
study of an enteric duplication cyst of the tongue and found well-differentiated
columnar and goblet cells. These did not correspond precisely to normal gastrointestinal
epithelium. This supported the theory that these cysts arose from primitive
endodermal gastric mucosa subjected to inductive influences.
Veeneklaas7 initially proposed the currently
accepted explanation in 1952, when he observed vertebral clefts and rib anomalies
in association with intestinal duplications. He suggested that a disturbance
in the development of the notochord and surrounding structures accounted for
misplaced segments of gastrointestinal mucosa. In this situation, adherent
endodermal cells became caught during the infolding of the notochordal plate.
This split notochord syndrome is now the favored developmental theory.
The differential diagnosis includes dermoid cyst, cystic hygroma, hemangioma,
ranula, and thyroglossal duct cyst. Most are diagnosed in asymptomatic infants,
but they have the potential to go undetected for years if the cyst is small
and asymptomatic.8 Difficulty with feeding
is not unusual, as reported in our series. Large cysts in the newborn may
initially cause airway obstruction, and needle aspiration may be used as a
temporary measure to maintain the airway. Prenatal ultrasound has led to early
diagnosis, and 2 cases of intraoral enteric duplication cysts have been detected
in utero.2 In both cases, the cysts were initially
decompressed and the airway secured prior to division of the placental cord.
These patients were later treated with definitive surgical excision.
Magnetic resonance imaging and/or computed tomographic scans are helpful
in determining the extent of the cyst. An MRI study, with its lack of ionizing
radiation and superior soft tissue resolution, is the imaging study of choice.
It allows an accurate assessment of the extent of tumor infiltration for preoperative
planning. As demonstrated in this case series, enteric duplication cysts are
often indistinguishable from dermoids on MRI because of the presence of proteinaceous
fluid. They appear as cystic lesions that do not enhance with intravenous
contrast. They may be hyperintense on T1-weighted images, depending on the
amount and distribution of proteinaceous contents. They uniformly appear hyperintense
on T2 and short T1 inversion recovery sequences.
CONCLUSIONS
The rare occurrence of foregut duplications in the oral cavity mandates
vigilance with respect to the airway. An MRI study is a recommended part of
the preoperative evaluation but cannot be relied on for definitive diagnosis
because these lesions appear similar to dermoids. Complete cyst excision with
removal of the mucosal lining is the treatment of choice. Aspiration alone
results in recurrence, and the functional mucosa continues to secrete mucus
if left intact. Also, there is a risk of ulceration and bleeding in the presence
of acid-secreting gastric mucosa. The long-term prognosis is excellent, with
no recurrences reported and complete recovery expected. This report comprises
the largest series of these unusual lesions at a single institution.
AUTHOR INFORMATION
Accepted for publication July 17, 2001.
Presented as a poster at the American Society of Pediatric Otolaryngology
meeting, Scottsdale, Ariz, May 9-11, 2001.
Corresponding author and reprints: Michael J. Biavati, MD, Pediatric
ENT Associates, 8325 Walnut Hill, No. 100, Dallas, TX 75231 (e-mail: pedi-ent{at}mindspring.com).
From the Departments of Otolaryngology (Drs Eaton and Billings), Pathology
(Dr Timmons), and Radiology (Dr Booth), Children's Medical Center, The University
of Texas Southwestern Medical Center, and Pediatric ENT Associates (Dr Biavati),
Dallas, Tex.
REFERENCES
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1. Gorlin FJ, Jirasek JE. Oral cysts containing gastric or intestinal mucosa: unusual embryological
accident or heterotopia. Arch Otolaryngol. 1970;91:594-597.
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2. Chen MK, Gross E, Lobe TE. Perinatal management of enteric duplication cysts of the tongue. Am J Perinatol. 1997;14:161-163.
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3. Willner A, Feghali J, Bassila M. An enteric duplication cyst occurring in the anterior two-thirds of
the tongue. Int J Pediatr Otorhinolaryngol. 1991;21:169-177.
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4. Lipsett J, Sparnon AL, Byard RW. Embryogenesis of enterocystomas-enteric duplication cysts of the tongue. Oral Surg Oral Med Oral Pathol. 1993;75:626-630.
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5. Daley TD, Wysocki GP, Lovas GL, et al. Heterotopic gastric cyst of the oral cavity. Head Neck Surg. 1984;7:168-171.
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6. Woolgar JA, Smith AJ. Heterotopic gastrointestinal cyst of oral cavity: a developmental lesion? Oral Surg Oral Med Oral Pathol. 1988;66:223-225.
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7. Veeneklaas GMH. Pathogenesis of intrathoracic gastrogenic cysts. AJDC. 1952;83:500-507.
8. Velcek FT, Klotz DH, Hill CH, et al. Tongue lesions in children. J Pediatr Surg. 1979;14:238-245.
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