International Journal of Pediatric 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 mimicking bilateral choanal atresia

C¸ag˘das¸ Elsu¨ rer *, Mete Kaan Bozkurt

Department of Otorhinolaryngology, Selc¸uk University , 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 (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 . 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

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, , 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, , 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 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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