Congenital Dacryocystocele Prenatal 2- and 3-Dimensional Sonographic Findings Case Series

Case Series
Congenital Dacryocystocele
Prenatal 2- and 3-Dimensional Sonographic
Findings
Waldo Sepulveda, MD, Adriana B. Wojakowski, MD,
Diego Elias, MD, Lucas Otaño, MD, Jorge Gutierrez, MD
Objective. The purpose of this series is to present our experience with cases of dacryocystocele diagnosed prenatally. The role of prenatal 3-dimensional sonography, as an adjunct to 2-dimensional
sonography, in the prenatal assessment of these cases is emphasized. Methods. A retrospective review
of cases was conducted. Information was obtained by reviewing the sonographic reports and medical
records. Outcomes were obtained from the referring obstetricians or directly from the parents.
Results. Ten fetuses had the diagnosis of a congenital dacryocystocele at a median gestational age of
30.1 weeks (range, 27–33 weeks). In 6 cases, the cystic lesion was unilateral, and in 4 it was bilateral, with a mean largest diameter at the time of diagnosis of 7.5 mm (range, 4–11 mm). There were
no other associated findings. Three-dimensional sonography, carried out in 3 cases, clearly depicted
the anomaly, the degree of intranasal extension, and swelling below the medial canthal area.
Spontaneous resolution was documented prenatally in 5 fetuses, and 1 additional case resolved
between the last prenatal scan and the delivery. There were no reported long-term complications associated with this finding, although 1 infant required probing at 2 months of age to resolve the dacryocystocele. Conclusions. Prenatal diagnosis of dacryocystocele is straightforward. A considerable
number of lesions are bilateral, and many resolve in utero spontaneously or neonatally after minimal
intervention. For those not resolving by the time of the delivery, ophthalmologic or rhinologic consultation is warranted because of potential complications. Three-dimensional sonography may provide a
noninvasive method for evaluating these cystic masses and may contribute to the avoidance of additional diagnostic techniques in the neonatal period. Key words: dacryocystocele; prenatal diagnosis;
prenatal sonography; 3-dimensional sonography.
Abbreviations
3D, 3-dimensional; 2D, 2-dimensional
Received October 18, 2004, from the Fetal Medicine
Center, Department of Obstetrics and Gynecology,
Clinica Las Condes, Santiago, Chile (W.S.); Fetal
Medicine Unit, San Jose Hospital, University of
Santiago de Chile, Santiago, Chile (W.S., J.G.); and
Department of Diagnostic Imaging (A.B.W., D.E.)
and Fetal Diagnosis and Therapy Unit (A.B.W., D.E.,
L.O.), Hospital Italiano, Buenos Aires, Argentina.
Revision requested October 27, 2004. Revised
manuscript accepted for publication November 3,
2004.
This work was supported by the Sociedad
Profesional de Medicina Fetal "Fetalmed" Limitada,
Chile.
Address correspondence and reprint requests to
Waldo Sepulveda, MD, Fetal Medicine Center,
Clinica Las Condes, Casilla 208, Santiago 20, Chile.
E-mail: [email protected]
C
ongenital dacryocystocele is a rare condition
characterized by cystic distension of the nasolacrimal sac due to obstruction of the lacrimal
drainage system, both above and below the sac.1,2
In neonates it can appear with a variable range of clinical
manifestations; mild forms of the disease are usually overlooked and frequently resolve spontaneously, whereas
large dacryocystoceles typically appear at birth as a tense,
bluish swelling located just below the medial canthal area.
Although congenital dacryocystocele is generally considered a benign condition, those with bilateral involvement
and substantial intranasal extension might be severe
enough to cause respiratory distress syndrome requiring
surgical intervention because neonates are obligate nasal
breathers.2–4
Prenatal diagnosis of dacryocystocele by 2-dimensional
(2D) sonography has been reported by several authors,
mostly as isolated case reports.5–18 In this report, we
© 2005 by the American Institute of Ultrasound in Medicine • J Ultrasound Med 2005; 24:225–230 • 0278-4297/04/$3.50
Congenital Dacryocystocele
describe a large series of congenital dacryocystoceles diagnosed prenatally. The clinical and prenatal sonographic findings, including those
obtained by means of 3-dimensional (3D) sonography, as well as their natural history and outcome, are reviewed.
Information on clinical findings, prenatal features,
pregnancy outcome, and follow-up was obtained
from the sonographic reports, medical records,
referring obstetricians, or patients themselves.
Materials and Methods
A total of 10 fetuses had the diagnosis of dacryocystocele at a mean gestational age of 30.1
weeks (range, 27–33 weeks). The mean maternal
age was 30.6 years (range, 24–39 years), and 6
women (60%) were nulliparous. Two cases were
referred from another institution after the detection of an anechoic mass in the fetal face, and the
remaining 8 were detected incidentally in our
inner general population undergoing routine
third-trimester sonography to assess fetal growth.
One woman had a previous child with congenital
dacryocystocele, and 1 had epilepsy treated with
carbamazepine. The other 8 had unremarkable
medical histories, and the pregnancies were otherwise uncomplicated. Characteristics of the
fetuses are given in Table 1.
The dacryocystoceles were unilateral in 6 cases
and bilateral in 4 (Figure 1). The mean diameter at
the time of detection was 7.5 mm (range, 4–11
mm). Eight fetuses (80%) were female. There were
no other associated findings, and the amniotic
fluid volume was normal in all cases. Threedimensional sonography was carried out to further characterize the lesion in 3 cases, which
clearly depicted the anomaly, its degree of
intranasal extension, and its communication
between the orbit and the nasal cavity (Figure 2).
Surface-rendered imaging showed swelling below
the medial canthal area in all 3 cases (Figure 3).
Cases of congenital dacryocystocele diagnosed
prenatally by sonography were identified by
review of a fetal medicine database from 3 referral
centers for prenatal diagnosis. The diagnosis was
established by the detection of a cystic lesion in
relation to the medial and inferior aspects of the
fetal orbit. Color Doppler sonography was used to
confirm the absence of blood flow signals within
and around the mass in all cases. Three-dimensional sonography, when available, was performed with Voluson 730 ultrasound equipment
(GE Healthcare, Milwaukee, WI). Briefly, once the
area of interest was identified with conventional
2D sonography, the image was enhanced by
adjustment of the gray scale and tissue harmonic
imaging settings. Volume data were then acquired
with a mechanical probe using an angle sweep of
50° to 65°. Digital information was stored for postprocessing and posterior analysis using the builtin hardware, which included simultaneous
orthogonal views, multiplanar plane slicing and
rotation, and surface rendering.
When the diagnosis was established, the parents were counseled regarding the probable
benign prognosis of the condition, although the
need for ophthalmologic or rhinologic consultation in the neonatal period was mentioned.
Results
Table 1. Prenatal Diagnosis of Congenital Dacryocystocele
MA, y
GA at
Diagnosis,
wk
1
34
27
Right
2
3
4
5
6
7
8
9
10
33
34
39
27
32
26
30
24
27
31
33
28
30
32
30
31
30
29
Right
Left
Bilateral
Bilateral
Bilateral
Right
Left
Left
Bilateral
Case
Dacryocystocele
Side
Size, mm
Prenatal
Resolution
7
Yes
7
8
7/7
7/4
9/7
8
10
7
11/6
Yes
Yes
Yes
Yes
Yes
No
No
No
No
Outcome and Remarks
Term delivery, healthy neonate, previous
infant with dacryocystocele
Term delivery, healthy neonate
Term delivery, healthy neonate
Term delivery, healthy neonate
Term delivery, healthy neonate
Term delivery, healthy neonate
Term delivery, nasolacrimal probing
Term delivery, resolution after massage
Term delivery, resolution after massage
Term delivery, spontaneous resolution,
unilateral epiphora
GA indicates gestational age; and MA, maternal age.
226
J Ultrasound Med 2005; 24:225–230
Sepulveda et al
Follow-up sonography was performed in all
cases. Spontaneous resolution was documented
prenatally in 5 fetuses, 3 with unilateral lesions
and 2 with bilateral lesions, at a mean gestational age of 35.5 weeks (range, 34–37 weeks). Among
the 5 other cases, the dacryocystocele remained
unchanged in size in 2, increased from 8 to 12
mm in 1, decreased on one side and remained
unchanged on the other side in 1, and resolved
on one side and remained unchanged on the
other side in 1. In 1 case of bilateral dacryocystoceles, particulate matter was also found within
a dacryocystocele on the follow-up scans at 34
and 37 weeks. Prenatal diagnosis was confirmed at birth in 4 of the 5 cases with persistent
dacryocystocele, 3 with a palpable paraocular
mass and 1 with persistent unilateral epiphora.
Among them, 1 resolved spontaneously; 2
resolved after repeated local pressure decompression; and 1 resolved after nasolacrimal duct
probing at 2 months of age. The remaining case
presumably resolved between the last prenatal
scan and delivery. Overall, none of the neonates
had complications in the neonatal period, and
all were discharged in good condition. There
were no reported long-term sequelae associated
with this finding.
Figure 1. A, Transverse view of the fetal eyes at 27 weeks
shows unilateral dacryocystocele. B, Swelling of the subcutaneous tissue is evident (arrows). C, Bilateral dacryocystoceles at
32 weeks.
A
B
Discussion
In this report, we describe a large series of dacryocystoceles diagnosed prenatally by sonography.
In neonates, congenital obstruction of the nasolacrimal drainage system can appear either as
dacryocystocele or dacryostenosis, depending
on the presence or absence of distension of
the nasolacrimal sac, respectively.1–4 Potential
complications of this condition include persistent epiphora, dacryocystitis, conjunctivitis,
facial cellulitis, and upper airway obstruction.
Nevertheless, most cases resolve spontaneously
or after gentle digital massage, although, occasionally, intranasal probing and marsupialization may be necessary if the dacryocystocele
persists or complications arise.1–4
Despite the fact that sonography has been used
extensively to evaluate the fetal face in the second and third trimesters, prenatal diagnosis of
dacryocystocele has been scarcely reported in
the literature.5–16 When detected in the fetus,
other causes of midfacial tumors can be ruled
out confidently, preventing unnecessary diagnostic procedures after birth. The list of differenJ Ultrasound Med 2005; 24:225–230
C
227
Congenital Dacryocystocele
A
B
Figure 2. A, Multiplanar views of the dacryocystocele shows the characteristic location and sonographic appearance. B, After slicing, the communication between the cystic paraocular mass and the orbit is evident.
tial diagnoses is extensive and includes anterior
encephalocele, dermoid cysts, and facial hemangioma.5,9,10 However, our experience is in keeping
with other authors in that the prenatal features of
dacryocystocele are characteristic in terms of its
location and sonographic findings.5–16 Indeed,
the sonographic detection of a cystic mass medial and inferior to the fetal orbit can be considered
diagnostic of dacryocystocele. Occasionally, particulate matter due to the presence of mucous
material and debris can be visualized within the
mass,16 but only 1 of our 10 cases had this prena-
tal sonographic feature. In a previous series of 6
cases, Sharony et al12 reported an association
between dacryocystocele and genetic conditions
such as Canavan disease and polycystic kidneys.
However, in all our cases and the ones reported
in the literature, the fetuses had had no other
structural abnormalities, although polyhydramnios had been noted previously in a few cases.5,11
Another important feature of dacryocystocele
is that it typically appears in the third trimester,
probably because of its embryologic basis.5,10,12
The nasolacrimal duct develops from an epithe-
Figure 3. A, Surface-rendered image shows the dacryocystocele in the inner canthal area. B, Neonate at the time of delivery with findings similar to
those shown on prenatal 3D sonography.
A
228
B
J Ultrasound Med 2005; 24:225–230
Sepulveda et al
lial cord, which appears around the sixth week of
gestation. Canalization of the cord starts from
the ocular to the nasal end by 12 weeks, and it
should be completed by the eighth intrauterine
month. Persistence of a thin mucosal membrane
between the duct and the nasal cavity, known as
the Hasner valve, allows accumulation of fluid
within the lacrimal sac. Another valve mechanism at the proximal end, known as the
Rosenmüller valve, which in the normal condition prevents reflux of fluid from the lacrimal sac
to the canaliculi, further causes distension of the
sac, leading to the formation of the dacryocystocele. If disruption of the Hasner valve occurs, the
nasolacrimal duct becomes patent, resulting in
spontaneous resolution either prenatally or after
birth. Our series shows that a substantial number
of cases resolve spontaneously in utero, which
has also been reported previously.6 Persistence of
the dacryocystocele until delivery, however, does
not carry a poorer prognosis because most will
resolve spontaneously in the neonatal period or
after minimal intervention such as local digital
massage. In the only other large prenatal series of
dacryocystoceles to our knowledge, Sharony et
al12 noted persistence until delivery in all 6 of
their cases, with 2 resolving spontaneously after
birth, 2 after digital massage, and 2 after surgical
drainage, 1 of which had dacryocystitis. In our
series of 10 cases, 7 resolved spontaneously (6
prenatally and 1 postnatally); 2 resolved after digital massage; and only 1 case persisted beyond
the first month of age, requiring probing as the
definitive treatment.
We performed 3D sonography in only 3 of our
cases because this technology was not available
at the time of the diagnosis of the other cases.
This allowed us to determine both the precise
location of the cystic lesion and the degree of
intranasal extension. To our knowledge, on the
basis of a MEDLINE search, the use of 3D sonography in this condition has been reported only
once previously.17 The prenatal findings in that
case, however, were limited because this technology was only used to relieve parental concerns about facial distortion by bilateral
dacryocystoceles. In contrast, we were able to
obtain high-quality images of the dacryocystocele and fetal face for diagnostic purposes, which
were easily understood by both the sonologist
and the prospective parents. Recently, Bianchini
et al18 reported the use of magnetic resonance
imaging in 3 fetuses with dacryocystocele. In a
J Ultrasound Med 2005; 24:225–230
comparison of both techniques, it seems that the
quality is similar, with 3D sonography having the
additional advantages of lower costs, no need for
manipulating the patient, and no potential for
radiation exposure. We therefore think that 3D
sonography should be the investigation of
choice if additional imaging beyond standard 2D
sonographic techniques is required for the prenatal diagnosis of dacryocystocele.
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