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26 June 2023: Articles  Japan

Successful Noninvasive Respiratory Management of an Infant with Bilateral Choanal Atresia and a Supernumerary Nostril Located on the Columella by a Mouthpiece: A Case Report

Unusual or unexpected effect of treatment, Rare disease

Nami Nakamura12ABE, Hiroki Kakita13ABCDEF*, Mizuki Takagi2AB, Shimpei Asai1AB, Takafumi Asai1AD, Mari Mori1AB, Satoru Takeshita13BDF, Hiroko Ueda1D, Mineyoshi Aoyama3DE, Mayuko Kishimoto4B, Kunihiro Ito5B, Yasumasa Yamada1DE

DOI: 10.12659/AJCR.939642

Am J Case Rep 2023; 24:e939642

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Abstract

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BACKGROUND: Choanal atresia with a supernumerary nostril located on the columella is extremely rare. Infants are obligate nasal breathers because the oral airway is invariably blocked during calm respiration. Infants breathe through the mouth only during crying, and they only have nasal breathing until 5 months of life. Congenital nasal anomalies have been reported to be fatal from birth, requiring tracheal intubation or tracheostomy in the early postnatal period. In these cases, it is crucial to maintain an adequate airway.

CASE REPORT: A 2948-g female infant was born at 40 weeks by normal vaginal delivery. Her Apgar scores were 9 and 9 at 1 and 5 min, respectively. She had retractive breathing, cyanosis, and a supernumerary nostril at birth. She had no other anomalies. Computed tomography showed bilateral membranous choanal atresia. She needed nasal continuous positive pressure or a high-flow nasal canula for oxygen desaturation during crying, apnea, and dyspnea. However, her respiratory symptoms did not improve completely. On day 25 of life, she was given a mouthpiece to support mouth breathing. Her respiratory symptoms improved gradually, and she was discharged on day 73 of life with a mouthpiece.

CONCLUSIONS: A very rare case of choanal atresia with a supernumerary nostril located on the columella was described. A mouthpiece was effective for breathing, obviating the need for emergency surgical intervention in the early postnatal period. Emergency procedures were avoided, probably because this case involved incomplete bilateral membranous choanal atresia rather than complete bony atresia.

Keywords: Choanal Atresia, Infant, Newborn, Humans, Infant, Female, Nasal Septum, Dyspnea, Tomography, X-Ray Computed, Tracheostomy

Background

Choanal atresia is an uncommon congenital disorder in which the back of the nasal passage (choana) is blocked, usually by abnormal bony or soft tissue (membranous) due to failed recanalization of the nasal fossae during fetal development [1–3]. The incidence of congenital choanal atresia is about 1: 5000 to 1: 8000 [1,2]. A supernumerary nostril is a much rarer congenital anomaly with an unknown etiology [4]. A third nostril in most supernumerary nostril cases is on the right or left side of a normal nostril [4]. Cases with a third nostril on the columella are very rare, with only 3 previous cases reported [4]. In addition, choanal atresia with a supernumerary nostril is extremely rare [5]. Choanal atresia can also be associated with other malformations or chromosomal anomalies [4]. There have been only 2 reported cases [5,6] of choanal atresia with a supernumerary nostril. One case had the third nostril on the side of the left normal nostril and the other on the right [5,6]. There were no cases with choanal atresia and a supernumerary nostril located on the columella. The exact underlying pathogenesis of these congenital malformations is still unclear [1,2,4].

Congenital nasal anomalies such as choanal atresia have been reported to be fatal from birth. Thus, it is crucial to maintain an adequate airway. Severe cases require tracheal intubation followed by tracheostomy and stenting in the early postnatal period [6]. However, these procedures, especially tracheostomy, have adverse effects and result in a lower quality of life [7].

A very rare case of choanal atresia with a supernumerary nostril located on the columella is described. In this case, a mouthpiece was effective for breathing and resulted in the avoidance of early emergency surgical intervention.

Case Report

A 2948-g female infant was born at 40 weeks by normal vaginal delivery. There was no family history and her mother did not have any prenatal maternal risk factors. Her Apgar scores were 9 and 9 at 1 and 5 min, respectively. Her cyanosis did not improve and her retractive breathing gradually worsened after 5 min of life; therefore, she needed oxygen therapy and mask continuous positive pressure. She had a supernumerary nostril at birth (Figure 1A) and did not have any other congenital malformations. Her chest X-ray and cardiac and abdominal ultrasound were normal. Transnasal endoscopic examination showed that the right nostril was open, but the middle nostril was a blind end, and the left nostril was stenotic due to compression by the middle nostril. In addition, computed tomography showed bilateral membranous choanal atresia (Figure 2).

Magnetic resonance imaging did not show any cranial dysplasia or intracranial abnormalities.

Figure 3 show her clinical course. She needed nasal continuous positive pressure or a high-flow nasal cannula for oxygen desaturation during crying, apnea, and dyspnea. An attempt was made to insert a nasal airway, but it failed due to nasal obstruction with secretions. Oxygen desaturation below 80% with apnea, and crying occurred more than 10 times a day after birth. Dyspnea did not improve. The occurrence of oxygen desaturation decreased gradually, but it did not disappear completely. On day 23 of life, the venosus blood gas examination showed respiratory acidosis (pH, 7.251; PaO2, 43.8 Torr; PaCO2, 63.8 Torr; HCO3, 30.5 mEq/L; base excess, 2.1 mEq/L). On day 25 of life, she was given a mouthpiece to support mouth breathing (Figure 1A, 1B). The mouthpiece was effective for maintaining an adequate airway via the oral route. Her respiratory symptoms improved gradually, and she was able to feed orally. In addition, her respiratory acidosis improved on day 26 of life (pH, 7.373; PaO2, 51.6 Torr; PaCO2, 41.8 Torr; HCO3, 23.8 mEq/L; base excess, −1.6 mEq/L). She was discharged on day 73 of life without invasive procedures such as tracheal intubation, tracheostomy, or stenting. She did not have any abnormal results on neonatal mass screening for congenital metabolic disorders or on the hearing screening test. Repair of bilateral choanal atresia was performed at 6 months of life, followed by 4 weeks nasal stenting. Her development and growth were good at 10 months of life. The supernumerary nostril repair was planned at 1 year old.

Discussion

Supernumerary nostril may be associated with other malformations or disease [1,8] According to Franco et al, other congenital malformations were found in 45% of patients with a supernumerary nostril [8]. The coincident malformations included complete unilateral cleft lip, congenital cataract, esophageal atresia, imperforate anus and patent ductus arteriosus, osteoma of the ethmoid sinus, and congenital adrenal hyper-plasia [1,8]. Choanal atresia can also be associated with other malformations or disease [3,9]. Coincident anomalies with choanal atresia could include polydactyly, nasal-auricular and palatal deformities, Crouzon syndrome, craniosynostosis, microencephaly, meningocele, meningoencephalocele, facial asymmetry, hypoplasia of the orbit and midface, hypertelorism and cleft palate, CHARGE (coloboma, cardiovascular malformations, growth and mental retardation, urogenital and ear anomalies) syndrome, and chromosomal anomalies [3,9]. The CHARGE association is seen in around 20% to 50% of infants with choanal atresia [3,9]. In addition, chromosomal anomalies are found in 6% of infants with choanal atresia [3,9]. Approximately two-thirds of choanal atresia cases are unilateral, and 65% to 75% of unilateral cases and 75% of bilateral cases have other anomalies [3]. In 30% of cases, the atretic plate is bony, and in 70%, it is mixed with bone and membranous tissue [3]. It is recommended that geneticists evaluate patients diagnosed with choanal atresia, especially if it is bilateral [3,9,10]. The present infant did not undergo genetic evaluation, because there were no anomalies other than the supernumerary nostril and choanal atresia, and her parents did not agree to genetic evaluation.

Infants are obligate nasal breathers because the oral airway is invariably blocked during calm respiration [11]. Infants breathe through the mouth only during crying, and infants have only nasal breathing until 5 months of life [11]. A one-third reduction in the diameter of the nasal airway can increase nasal airway resistance 81-fold, resulting in severe distress in neonates [11]. Bilateral choanal atresia is a clinical emergency that requires surgical treatment [11–14]. In the present case, these emergency procedures were avoided, probably due to the presence of incomplete bilateral membranous choanal atresia rather than complete bony atresia. These cases need tracheal intubation followed by surgical treatment, such as transnasal endoscopic surgery, tracheostomy, or stenting [11–14]. Transnasal endoscopically assisted choanal atresia repair has become the preferred procedure [12–14]. Bilateral choanal atresia, young age, and low birth weight are listed as possible risk factors for surgical failure, although complications are rare and morbidity is low [11–14]. In addition, surgical treatment such as tracheostomy or stenting in the early postnatal period has adverse effects, such as nasopharyngeal reflux and gastroesophageal reflux, and decreases the infant’s quality of life [7]. Thus, we decided to avoid surgical intervention in the early postnatal period.

Before surgical treatment, there are conservative treatments, such as nasal drops and McGovern pacifiers, in addition to the treatment option of a nasal airway, which is noninvasive, low-cost, and can be applied easily and quickly [15,16]. The goal of initial treatment for these patients is to maintain an adequate airway via the oral route. The preferred oral airway is a McGovern nipple, an intraoral nipple with a large opening by cutting its end off, secured in the mouth with ties around the infant’s occiput [15,16]. In the present case with a good outcome, the mouthpiece stabilized the baby’s respiratory status, and invasive treatment could be avoided in the early postnatal period.

Conclusions

We reported a rare case of choanal atresia and a supernumerary nostril located on the columella in which a mouthpiece was found to be effective for breathing, thus allowing avoidance of emergency surgical intervention. Noninvasive respiratory management by mouthpiece might be an effective treatment option until repair of choanal atresia.

References:

1.. Alqabbani AA, Alateeq MF, Alshalaan Z, Isolated congenital supernumerary nostril is an adult patient: Ear Nose Throat J, 2021; 100(10); 1038S-40S

2.. Paradis J, Dzioba A, EI-Hakim H, A national study of choanal atresia in tertiary care centers in Canada – part II: Clinical management: J Otolaryngol Head Neck Surg, 2021; 50(1); 46

3.. Abdul Cader SH, Shah FA, Reghunandanan N, Clinical retrospective analysis of 15 cases of chroanal atresia – our experience: World J Otorhinolaryngol Head Neck Surg, 2019; 5(4); 188-92

4.. Gulasi S, Turhan AH, Celik Y, Accessory nostril: Rare congenital nasal anomaly: J Craniofac Surg, 2015; 26(7); e602-3

5.. Shanmuganathan P, Chong DK, Accessory nostril associated with choanal atresia: J Craniofac Surg, 2021; 32(3); 2845-47

6.. Li XZ, Cai XL, Zhang L, Bilateral congenital choanal atresia and osteoma of ethmoid sinus with supernumerary nostril: A case report and review of the literature: J Med Case Rep, 2011; 20(5); 583

7.. Kwong KM, Current updates on choanal atresia: Front Pediatr, 2015; 3; 52

8.. Franco D, Medeiros J, Faveret P, Supernumerary nostril: Case report and review of the literature: J Plast Reconstr Aesthet Surg, 2008; 61(4); 442-46

9.. Duncan NO, Miller RH, Catlin FI, Choanal atresia and associated anomalies: The CHARGE association: Int J Pediatr Otorhinolaryngol, 1998; 15(2); 129-35

10.. Manica D, Schweiger C, Netto CCS, Retrospective study of a series of choanal atresia patients.: Int Arch Otorhinolaryngol, 2014; 18(1); 2-5

11.. Sinha V, Umesh ST, Jha SG, Choanal atresia: Birth without breath: Indian J Otolaryngol Head Neck Surg, 2018; 70(1); 53-58

12.. Leedderose GJ, Havel M, Leddeose C, Endoscopic endonasal repair of complete bilateral choanal atresia in neonates: Eur J Pediatr, 2021; 80(7); 2245-51

13.. Baldovin M, Cazzador D, Zanotti C, Bilateral choanal atresia and endoscopic surgery: A chance for CHARGE patients: J Clin Med, 2021; 10(13); 2951

14.. Saafan ME, Endoscopic management of congenital bilateral posterior choanal atresia: Value of using stents: Eur Arch Otorhinolaryngol, 2013; 270(1); 129-34

15.. Kumar S, Jain M, Sogi S, McGovern nipple: An alternative for nose breathing in newborn with CHARGE syndrome, having bilateral choanal atresia: J Indian Soc Pedod Prev Dent, 2020; 38(2); 204-7

16.. Knegt-Junk KJ, Bos CE, Berkovits RN, Congenital nasal stenosis in neonates: J Laryngol Otol, 1998; 102(6); 500-2

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923