CLINICAL LETTERS

Prenatal Diagnosis of Congenital Epulis: otorhinolaryngology in the delivery room for assis-
Implications for Delivery tance with neonatal airway management, if necessary.
At 32 weeks 6 days, the patient developed cramping
Oral masses are uncommon in the fetus.1 The differen- and bleeding. Fetal heart tracing showed moderate vari-
tial diagnosis is broad and includes both benign and ability with recurrent variable decelerations, and a plan
malignant conditions. Oral masses may disrupt fetal was made to proceed with delivery. The female neonate
swallowing and can affect neonatal breathing and feed- was born by a classical cesarean delivery because of the
ing, thus potentially affecting immediate management of breech presentation, prematurity, and an undeveloped
the neonate.1 Although most oral masses are diagnosed lower uterine segment and was handed off to the
postnatally, some are detected prenatally.2 Accurate pre- awaiting neonatology and pediatric otorhinolaryngology
natal diagnosis is important for antenatal counseling and teams. The neonate was found to have a 2-cm soft tissue
delivery planning, which may include the addition of sur- mass arising from the left maxillary gingiva (Figure 1C).
gical specialists at the delivery and, in some cases, use of She did not have respiratory distress. Given prematurity,
the ex utero intrapartum treatment (EXIT) procedure.3 she was transferred to the neonatal intensive care unit
We present a case of a fetal oral mass, thought to be con- for stabilization and was scheduled for mass excision on
genital epulis, which led to multidisciplinary delivery day 2 of life. Histologic findings of the excised mass
room management at the time of birth and subsequent were consistent with congenital epulis.
planned surgical excision. Congenital epulis, also known as a congenital gingi-
A 32-year-old woman, gravida 2, para 0, presented val granular cell tumor, is a rare benign tumor observed
to obstetric triage at a gestational age of 30 weeks 5 days only in fetuses and neonates.4,5 This tumor, which can
with a report of leaking fluid. Her prenatal course had range in size from 1 mm to 9 cm, tends to arise from the
been otherwise uncomplicated except for placenta previa anterior alveolar ridge, with the maxilla involved twice as
diagnosed at her 18-week anatomy scan; the previa had often as the mandible.4 This tumor has the potential for
resolved on follow-up sonography, and there was no evi- obstruction of the oral cavity, leading to polyhydramnios
dence of polyhydramnios during her prenatal course. prenatally and respiratory and feeding difficulty postna-
Her medical and surgical histories were unremarkable. tally.4 As such, when technically possible, surgical exci-
Preterm premature rupture of membranes was sion is performed.2
confirmed on the basis of a physical examination, vagi- The etiology of congenital epulis is unknown,
nal pH, and microscopic evaluation. The patient started although various theories of development have been
receiving latency antibiotics, was given a course of beta- postulated. A hormonal etiology has been suspected, as
methasone, and was admitted to the hospital for it is seen 10 times more often in female fetuses. It is typi-
expectant management. Sonography performed on cally found in chromosomally normal neonates without
hospital day 2 estimated the fetal weight at 1719 g other congenital anomalies, although it has been associ-
(52nd percentile), in a female fetus who was in a frank ated with 47,XXX6 and polydactyly.5 Given that there
breech presentation with an amniotic fluid index of 7.6. have been no case reports of congenital epulis before 26
Sonography showed a 1- to 2-cm spherical homogene- weeks, these tumors are thought to develop and grow in
ous solid and vascular mass protruding from the fetus’s the late second and third trimesters.7 It is notable that in
mouth (Figure 1A). Fetal magnetic resonance imaging our case, 3 prior prenatal sonographic examinations, per-
(MRI) performed the same day to examine the airway formed at 18, 22, and 28 weeks, did not show the mass;
and further evaluate the mass showed a 1.6-cm soft tis- our review of the images showed normal profile views at
sue mass originating specifically from the gingiva of the the first 2 scans; however, views of the fetal lips and oral
left side of the maxilla, near the future site of the maxil- cavity were not obtained at the 28-week scan.
lary canine or lateral incisors (Figure 1B). No other First identified by Neumann in 1871, this tumor has
abnormalities were detected. Based on the morpho- been described in more than 200 case reports, document-
logic characteristics and location on MRI, the diagnosis ing its clinical features, natural history, and surgical exci-
of congenital epulis was made. sion.8 Until recently, the tumor was primarily identified at
Although the mass was considered to be small the time of birth, but in the setting of improved ultra-
and unlikely to lead to substantial airway obstruction, sound technology, prenatal diagnosis is increasingly more
there was concern for its growth before delivery. common.7 Prenatal diagnosis allows for optimal multidis-
Thus, a plan was made for the presence of pediatric ciplinary delivery management and timely treatment of

C 2016 by the American Institute of Ultrasound in Medicine | J Ultrasound Med 2017;36:449–451 | 0278-4297 | www.aium.org
V
Clinical Letters

the neonate,2 although a definitive diagnosis can be made epulis, which has more organized blood flow similar to
only on surgical pathologic examination.5 that seen in this case, with a single or branched feeding
The differential diagnosis of a fetal oral mass vessel, a hemangioma can be distinguished by its disor-
includes macroglossia, teratoma, hemangioma, a lym- ganized high-flow vascularization.9 Homogeneity can
phatic malformation, a dermoid cyst, a duplication cyst, also distinguish congenital epulis from other lesions,
and other benign or malignant soft tissue masses.1 Dif- such as teratomas, which tend to be more heterogeneous
ferentiating between these possibilities by sonography and hypoechoic.9 Nevertheless, sonography has fairly
can become increasingly difficult in later gestation.8 low specificity. Magnetic resonance imaging has been
The blood flow pattern can help differentiate con- shown to be helpful, with its improved ability to delin-
genital epulis from other oral masses. Unlike congenital eate soft tissue structures and more accurately identify

Figure 1. Congenital epulis of the fetal maxilla noted prenatally at 31 weeks’ gestation. A, Coronal 2-dimensional sonogram of the mass (arrow)
seen protruding from the fetal upper lip. B, Axial MRI of the mass (arrow) slightly to the left of midline, emanating from the fetal gum. Note the arc
of the tooth-bearing maxilla behind the mass (3.0-T Skyra; Siemens Medical Solutions, Mountain View, CA; slice thickness, 2–3 mm). C, Neonatal
findings at the time of birth.

450 J Ultrasound Med 2017; 36:449–451

the anatomic relationship of the mass with other Katherine M. Johnson, MD, Scott A. Shainker, DO, MS,
structures.9 Judy A. Estroff, MD, Steven J. Ralston, MD, MPH
Case reports describe MRI findings of a nonenhanc- Department of Obstetrics and Gynecology (K.M.J.)
ing mass with smooth borders originating from the gin- Division of Maternal-Fetal Medicine (S.A.S., S.J.R.)
giva. Congenital epulis tends to display isointensity to Beth Israel Deaconess Medical Center
muscle on T1-weighted imaging and isogeneous to het- Boston, Massachusetts USA
erogeneous signal intensity on T2-weighted imaging.9,10 Department of Obstetrics, Gynecology, and Reproductive Biology
The diagnostic accuracy of MRI is often better than that Harvard Medical School
of sonography by virtue of its more precise anatomic Boston, Massachusetts USA (K.M.J., S.A.S., S.J.R.)
definition; however, it can be more difficult on MRI to Department of Radiology, Advanced Fetal Care Center
perform a functional assessment of fetal swallowing, Boston Children’s Hospital
which is important in determining the likelihood of oral Boston, Massachusetts USA (J.A.E.)
and airway obstruction and consequently the need for doi:10.7863/ultra.16.03055
an EXIT procedure or additional resources and staffing
at the time of delivery (eg, flexible bronchoscopy and Special thanks to Jenny Powers, RDMS, for the original detec-
pediatric otorhinolaryngology).1 Functional characteris- tion of this mass.
tics on sonography that are of concern for obstruction
include the absence of a stomach bubble and nonvisuali- References
zation of bidirectional flow of amniotic fluid through the
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