International Journal of Pediatric Otorhinolaryngology Extra 9 (2014) 97–99
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International Journal of Pediatric Otorhinolaryngology
Extra
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Case Report
Bilateral congenital nasolacrimal duct cysts mimicking bilateral choanal atresia
C¸ag˘das¸ Elsu¨ rer *, Mete Kaan Bozkurt
Department of Otorhinolaryngology, Selc¸uk University Medical School, Turkey
A R T I C L E I N F O A B S T R A C T
Article history: Congenital nasolacrimal duct cyst (CNLDC) is a rare neonatal condition that can be life threatening when
Received 12 December 2013
it occurs bilaterally. Herein we report a neonate with bilateral CNLDC that caused respiratory distress
Received in revised form 19 March 2014
and was treated successfully with surgery. Post surgery, the patient was discharged from hospital
Accepted 20 March 2014
without any complications. The case details, differential diagnosis, and management of CNLDC are
discussed, along with a review of the relevant literature.
Keywords:
ß 2014 Elsevier Ireland Ltd. All rights reserved.
Congenital nasolacrimal duct cyst
Choanal atresia
Nasal endoscopy
Congenital nasolacrimal obstruction
Newborn respiratory distress
1. Introduction the valve of Hasner, and functional obstruction of the common
canaliculus or valve of Rosenmuller. CNLDCs are more common in
Nasal obstruction in neonates is caused by numerous condi- females and non-Hispanic Caucasians. Familial cases have been
tions, including choanal atresia, pyriform aperture stenosis, described only sporadically. Common signs are a benign, bluish-
nasopharyngeal teratomas, hemangiomas, dermoids, nasal glio- gray mass in the inferomedial canthus, dacryocystitis, facial
mas, and meningoencephaloceles [1]. In rare instances congenital cellulitis, respiratory distress, and feeding difficulty [2,3,5]. CNLDC
nasolacrimal duct anomalies with intranasal cystic swelling of the is diagnosed based on bilateral nasal endoscopic examination.
nasolacrimal apparatus can cause unilateral or bilateral nasal Current treatment modalities for CNLDC include nasolacrimal
obstruction in children, especially neonates [2]. The lacrimal probing, placement of a silastic stent, and intranasal endoscopic
system begins to develop during the 5th week of fetal develop- cyst marsupialization [2]. Herein we report a neonatal case of
ment, and formation of a lumen in the lacrimal cord occurs during CNLDC that mimicked bilateral choanal atresia.
the 10th week, which coincides with cavitation of the inferior
meatal lumen. Via canalization of the lacrimal cord, communica-
2. Case report
tion with the nasal inferior meatus is completed from the 6th fetal
month to beyond term [3].
An 11-h-old female was referred to our clinic with cyanosis
Congenital nasolacrimal duct cyst (CNLDC), also known as
while breastfeeding, respiratory dysfunction, and a feeding tube
congenital dacryocystocele, is a focal dilation of the nasolacrimal
inserted via both nasal cavities. Physical examination showed
drainage system [4]. Epidemiologically, CNLDCs are relatively
respiratory dysfunction and stridor. Endoscopic nasal examination
uncommon, and are estimated to occur in 1/3884 births. CNLDCs
showed bilateral inferior meatal cystic masses and intact bilateral
are thought to be the result of a persistent membrane at the level of
choanal atresia. MRI showed bilateral cystic masses in the inferior
meatus, measuring 12 mm T 9 mm on left side and 9 mm T 7 mm
on the right side (Fig. 1a and b). The patient underwent endoscopic
marsupialization of the bilateral nasal cysts (Fig. 2a and b), and the
* Corresponding author at: Department of Otorhinolaryngology, Selc¸uk Univer-
obtained biopsy specimens were consistent with dacryocystocele.
sity Medical School, Selc¸uklu, Konya, Turkey. Tel.: +90 332 2244422;
The patient was discharged from the neonatal intensive care unit
fax: +90 332 241 21 84.
E-mail address: [email protected] (C¸. Elsu¨ rer). 24 h after surgery.
http://dx.doi.org/10.1016/j.pedex.2014.03.007
1871-4048/ß 2014 Elsevier Ireland Ltd. All rights reserved.
98 C¸. Elsu¨rer, M.K. Bozkurt / International Journal of Pediatric Otorhinolaryngology Extra 9 (2014) 97–99
Fig. 2. (a) Nasal endoscopic view shows the right inferior meatal cyst obstructing
the right nasal cavity. (b) Nasal endoscopic view shows the left inferior meatal cyst
obstructing the left nasal cavity.
3. Discussion
Congenital nasal obstruction is an uncommon, but important
entity, as it can result in neonatal respiratory distress because
neonates are obligate nasal breathers. Nasal obstruction can occur
due to choanal atresia, congenital pyriform aperture stenosis
(CPAS), midnasal stenosis, encephalocele, nasal/nasopharyngeal
dermoid, glioma, teratoma, and CNLDC [8]. Choanal atresia is the
most common congenital nasal anomaly, affecting 5000–8000 live
births. Choanal atresia shows a female predominance, is more
frequently unilateral than bilateral, and in unilateral cases the right
side is most commonly involved. Among patients with choanal
atresia, 47–74% have a comorbid congenital anomaly [7,8]. CPAS is
a very rare condition characterized by bony overgrowth of the
medial process of the maxillary bone that results in neonatal nasal
obstruction. Most studies on CPAS are case reports and case series;
therefore, calculation of its prevalence is virtually impossible due
to the extreme rarity of the condition. Diagnostic criteria for CPAS,
which are based on CT findings, are not definitive. In cases of
moderate-severe stenosis, treatment is surgical and involves
pyriform aperture enlargement to reshape the stenotic area with
burs via an endo-oral sub-labial approach [9,10].
Midnasal stenosis is a rare clinical entity that occurs secondary
to bilateral bony overgrowth midway through the nasal cavity. It
usually occurs in children with midface hypoplasia, such as those
with fetal alcohol syndrome, but isolated cases have been
described. Midnasal stenosis is diagnosed based on CT or nasal
endoscopy findings. Treatment is conservative, with frequent nasal
saline irrigation and suctioning until 6 age months, at which point
the midface has grown sufficiently to relieve the obstruction, or
dilatation followed by stenting [6,11].
Nasal dermoids, gliomas, and encephaloceles are categorized as
Fig. 1. (a) MRI shows bilateral dacryocystoceles. (b) MRI shows bilateral inferior
meatal cystic masses. congenital midline nasal masses; they occur in 1/30,000 live births
and are more common in Asian populations, occurring in 1/6000
births. Nasal dermoids are the most common of the congenital
midline nasal masses. Diagnosis of nasal dermoids is based on
physical exam and MRI findings. Small intranasal dermoids can be
C¸. Elsu¨rer, M.K. Bozkurt / International Journal of Pediatric Otorhinolaryngology Extra 9 (2014) 97–99 99
accessed via an endoscopic approach, whereas large lesions may 4. Conclusion
require a lateral rhinotomy or external rhinoplasty. Encephaloceles
are caused by defective development of the skull and herniation of In conclusion, the present case report described a patient with
intracranial contents; their treatment is similar to that of other bilateral CNLDCs that mimicked bilateral choanal atresia. CNLDC
congenital midline nasal masses [6]. should be a diagnostic consideration in neonates with nasal
CNLDC, especially bilateral, causes nasal obstruction in obstruction, despite its rarity.
neonates. The nasolacrimal duct (NLD) is a canalized tract that
allows tears to drain from the medial canthus to the inferior Conflict of interest statement
meatus of the nasal cavity. The NLD develops from the ectodermal
surface located between the nasal and maxillary processes at 32 d The authors state that there are no sponsorships or conflicts of
of gestation. Canalization begins at the punctum in the eyelid interest relevant to the materials resented herein, and that the
during the third month of gestation and extends toward the nose study did not receive any financial assistance.
[2]. Congenital NLD obstruction has been reported with an
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