Bilateral Congenital Nasolacrimal Duct Cysts Mimicking Bilateral Choanal Atresia
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International Journal of Pediatric Otorhinolaryngology Extra 9 (2014) 97–99 Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology Extra jo urnal homepage: www.elsevier.com/locate/ijporl 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 incidence of 35–73%; the majority of cases resolve spontaneously References within the first week following birth [3]. CNLDC is an infrequent [1] H. Coates, Nasal obstruction in the neonate and infant, Clin. Pediatr. 31 (1992) 25– severe variant of congenital nasolacrimal duct obstruction 29. (NLDO), occurring in 0.1% of infants with NLDO. The cause of [2] E. Dogan, N.G. Yu¨ ksel, M.C. Ecevit, A. Yaman, A.T. Berk, S. Su¨ tay, Microdebrider NLDO is valve of Hasner or valve of Rosenmuller dysfunction. assisted endoscopic marsupialization of congenital intranasal nasolacrimal duct cysts, Int. J. Pediatr. Otorhinolaryngol. 76 (April (4)) (2012) 488–491. , http:// Congenital dacryocystoceles have a significant female predomi- dx.doi.org/10.1016/j.ijporl.2011.12.031. nance (55–75% of cases are female), with a higher incidence in [3] H.P. Barham, J.M. Wudel, R.W. Enzenauer, K.H. Chan, Congenital nasolacrimal duct Caucasians [2–4]. To date, 4 cases of familial NLDO have been cyst/dacryocystocele: An argument for a genetic basis, Allergy Rhinol. 3 (2012) reported. The 1st case is a mother and daughter with branchio- e46–e49. , http://dx.doi.org/10.2500/ar.2012.3.0024. [4] J.C. Wang, M.J. Cunningham, Congenital dacryocystocele: is there a familial oculo-facial syndrome, the 2nd case is a brother and sister with predisposition? Int. J. Pediatr. Otorhinolaryngol. 75 (2011) 430–432. , http:// congenital NLDO due to lacrimal puncta agenesis, the 3rd case is dx.doi.org/10.1016/j.ijporl.2010.11.026.