Congenital Nasal Stenosis: a Report of Two Cases Samy El-Wanya, Diaa M
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234 Case report Congenital nasal stenosis: a report of two cases Samy El-Wanya, Diaa M. El-Hennawib, Mohamed R. Ahmedb, Ashraf S. Abou-Halawab, Yasser T. Madianb, Ibrahim H. Ibrahimb aOtorhinolaryngology Department, Faculty of The human neonate is an obligate nasal breather for a variable period of time after Medicine, Alexandria University, Alexandria, birth. Congenital nasal airway anomalies are potentially dangerous if they are not bOtorhinolaryngology Department, Faculty of Medicine, Suez Canal University, Ismailia, immediately recognized at birth and if appropriate intervention is not instituted. Egypt Here, we report two clinical cases of congenital nasal stenosis seen in the Suez Canal University Hospital in Ismailia in 2016. Approval from our institutional ethics Correspondence to Mohamed R. Ahmed, MD, Otolaryngology Department, Faculty of committee and consent from parents of the patients to publish the cases and their Medicine, Suez Canal University, Ismailia, photos were obtained. Level of Evidence: 3b. Egypt. Tel: +20 128 504 3825; fax: +20 663 415 603; Keywords: e-mail: [email protected] congenital-C Nose, neonates, stenosis Received 8 December 2018 Egypt J Otolaryngol 35:234–238 Accepted 14 February 2019 © 2019 The Egyptian Journal of Otolaryngology The Egyptian Journal of Otolaryngology 1012-5574 2019, 35:234–238 reported that with any falling on the face, the child Introduction got the lower lip injured by the mere size of this tooth. The human neonate is an obligate nasal breather for a Thehardpalatewasnarrowed,andthetongueand variable period of time after birth [1]. Congenital nasal oropharynx were within normal. airway anomalies are potentially dangerous if they are not immediately recognized and if appropriate X-ray nasopharynx lateral view showed normal adenoid intervention is not instituted [2]. Asphyxia and shadow. Computed tomography (CT) revealed cyanosis in the first few minutes after birth may lead narrowing of the pyriform nasal aperture. Choanae to brain insult [3]. A simple oral airway will bypass the were patent bilaterally. nasal airway and keep the neonate orally breathing. If successful in bypassing the airway obstruction, this also Surgical correction was planned. Under general excludes the more serious lower airway anomalies, for anesthesia, a sublabial approach was used to drill example, laryngeal and tracheal anomalies, pulmonary the bony narrowing at the pyriform aperture using a hypoplasia and diaphragmatic hernia [4]. With this diamond bit. Drilling was performed only at the right life-saving oral airway in place, definitive surgical and left corners, keeping away from the nasal floor and intervention can be planned. In this work, we report preserving the vestibular mucosa intact. two congenital nasal airway problems. Postoperatively, the child had marked improvement of her nasal breathing with disappearance of sleep Case #1 apnea. She was referred to the dentist who decided to A 2-year-old girl from a poor uneducated family came remove the abnormally big central incisor. with nasal obstruction since birth. A catheter failed to pass through the nose during her visits to other ENT Case #2 clinics. Nasal airway is somehow satisfactory except A neonate was born to a 29-year-old primigravida during the common cold episodes when the child had following 40 weeks of gestation. The mother’s complete nasal stuffiness, nasal discharge, snoring, history did not include exposure to viral infection, and apneic spells during sleep. The mother was sex hormones, teratogens or ionizing radiation sometimes obliged to stay awake beside the child to during pregnancy. Following normal vaginal delivery, ensure normal oral breathing. When seen in the clinic, the neonate was resuscitated for delayed onset of crying the child was asleep and snoring. Apneic pauses of and respiration. Apgar score was unknown. several seconds were noticed and relieved through Ambiguous genitalia were diagnosed when nonfused awakening. Nasal examination revealed mucopurulent discharge. The nose was cleared with suction, and an ear speculum was inserted into the This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 right and left nostrils. Bony narrowing just beyond the License, which allows others to remix, tweak, and build upon the work nostril was noticed bilaterally. Oral examination non-commercially, as long as appropriate credit is given and the new revealed a single big central incisor. Mother creations are licensed under the identical terms. © 2019 The Egyptian Journal of Otolaryngology | Published by Wolters Kluwer - Medknow DOI: 10.4103/ejo.ejo_106_18 Congenital nasal stenosis El-Wany et al. 235 ‘scrotal’ folds and curved phallus between them were Discussion noticed. Moreover, it was noticed that there was left- sided coloboma irides in the form of an inferior During the third and fourth weeks of intrauterine life, triangular defect but no ear deformities. No finger, the nose starts to form nasal placodes, which are toes or other limb defects were noticed. She was sent to thickenings of the surface ectoderm on the lateral the neonatal ICU on oral airway. surface of the head of the embryo. They gradually sink into depressions and migrate medially to In the neonatal ICU, failure to pass a catheter through become the nostrils. Thereafter, the tissue the nose into the nasopharynx suggested possible surrounding them creates the medial and lateral bilateral choanal atresia. CT axial cuts confirmed nasal prominences. The medial nasal processes form the atretic plate at the choana bilaterally. The the anterior nasal septum, whereas the nasofrontal neonate received feeding through an Nasogastric process forms the posterior nasal septum and the tube (NG) tube through the mouth. ethmoid, nasal, and premaxillary bones. Finally, the Echocardiography and abdominal ultrasound were oronasal membrane is resorbed to form the primitive free. Karyotyping and pelvic ultrasound confirmed choanae [1]. female sex, and a girl’s name was given. Congenital nasal anomalies are considered errors in The neonate was then referred to our department on embryogenesis or are a result of intrauterine events the 12th day. Under General anesthesia (GA) with affecting embryonic and fetal growth. They may be ET intubation, nasal endoscopy was performed with associated with other craniofacial anomalies [2]. a1.9mm0° otoendoscope. Using otologic instruments, an 8 F metal suction was used to Congenital nasal pyriform aperture stenosis is a rare perforate the atretic plate bilaterally. Bone cause of nasal airway obstruction due to bony fragments were noticed to be very thin and were overgrowth of the nasal process of the maxilla and easily removed using otologic crocodile forceps. its clinical picture is similar to choanal atresia [3,4]. Using a pediatric backbiter, the posterior end of Manifestations in such conditions are apneic crisis, the nasal septum was removed. There was minimal episodic cyanosis, and inability to nurse the newborn bleeding. No attempt was made to create mucosal [5]. Its etiology is unknown, but it arises in the fourth flaps. A 2–0 silk suture was passed into one nasal month of fetal development due to an overgrowth of cavity to the nasopharynx and then retrieved from the the nasal process of the maxilla, and may present as an opposite side around the posterior end of the septum. isolated condition or in association with other Two #3 Polyvinyl chloride (PVC) tracheal tube were congenital disorders such as holoprosencephaly, cleft used as a stent. Two months after surgery, the patient palate, and early presence of maxillary central incisors had widely patent choanae (Figs 1–3). [6]. Inability to pass a fine catheter through the very Figure 1 Showing iris defect. 236 The Egyptian Journal of Otolaryngology, Vol. 35 No. 2, April-June 2019 Figure 2 Showing genitalia defect. Figure 3 Computed tomography showed bilateral atresia. nostril should raise suspicion. Investigations depend satisfactory enlargement. Drilling of the nasal floor mainly on CT. A radiographically measured pyriform must be avoided to prevent damage to the tooth opening of less than 8–10 mm in a full-term infant is buds [6]. considered diagnostic [7]. Immediate management is to establish a secure airway with endotracheal Congenital midnasal stenosis is a midline developmental intubation. Treatment using a nonsurgical approach defect. A missense mutation in the SHH gene at 7q36 using silastic stents in the nasal cavity and/or local has been incriminated [10]. The most significant decongestants is preferable [8]. However, in moderate findings are a single central incisor tooth located or severe cases, surgery through a sublabial approach is precisely in the midline of the maxillary alveolus, and essential. This provides good bilateral exposure of the congenital pyriform aperture stenosis with midnasal pyriform aperture with minimal visible scarring [9]. stenosis [11]. In addition, it could be associated with Using burrs, the stenotic area can be widened with holoprosencephaly and CHARGE syndrome intact mucoperiosteum. A passage for a 3.5-mm (colobomata, heart defects, atrasia choanae, retarded endotracheal tube stent is made to ensure growth, genital hypoplasia, and ear abnormalities) Congenital nasal stenosis El-Wany et al. 237 [12] or VACTERL association (vertebral anomalies, fewer subsequent surgical debridements with topical anal atresia, cardiac malformations, tracheoesophageal