Is isolated bilateral choanal atresia a real emergency? Two case reports Al Kindy SA

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Year : 2013 | Volume : 2 | Issue : 1 | Page : 58-60

Is isolated bilateral choanal atresia a real emergency? Two case reports

Sami A Al Kindy
Department of Surgery, College of Medicine, Taif University, Taif 21947, Kingdom of Saudi Arabia

Date of Web Publication

29-May-2013

Correspondence Address:
Sami A Al Kindy
Department of Surgery, College of Medicine, Taif University, PO Box 888, Taif 21947
Kingdom of Saudi Arabia

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2278-0521.112633

Abstract

Bilateral choanal atresia (BCA) in newborns is thought to be a potentially life threatening anomaly. Unlike unilateral choanal atresia, its' early detection and management is considered to be an emergency. The aim of the present case presentation is an attempt to explain how a newborn, who is an obligatory nasal breather can survive this anomaly and adapt a new pattern of breathing. In this study, we present two rare cases of isolated BCA. According to our knowledge, only few similar cases were reported in the literature. The first one is a 60-year-old female that was discovered accidentally during clinical examination for a different complaint. The second case is a 24-year-old male presenting with bilateral nasal obstruction since childhood. The female declined the offer of surgical interference while the male underwent endoscopic drilling of the atretic plate, no stent was required post-operative. He was followed-up for more than a year with no evidence of restenosis. It can be concluded that isolated missed BCA in infants is compatible with life. Why these patients survive this anomaly, is a topic for discussion. We propose a trial of pacifier as a first line of management to allow the newborn with isolated BCA to adapt a new pattern of mouth breathing, thus avoiding early surgical interference with its risk of complications.

Keywords: Adults, bilateral choanal atresia, CHARGE syndrome, complications, surgery

How to cite this article:
Al Kindy SA. Is isolated bilateral choanal atresia a real emergency? Two case reports. Saudi J Health Sci 2013;2:58-60

How to cite this URL:
Al Kindy SA. Is isolated bilateral choanal atresia a real emergency? Two case reports. Saudi J Health Sci [serial online] 2013 [cited 2020 Nov 28];2:58-60. Available from: https://www.saudijhealthsci.org/text.asp?2013/2/1/58/112633

Introduction

Choanal atresia (CA) is not an uncommon congenital malformation caused by failure of buccopharyngeal membrane to break in intrauterine life. It may be unilateral or bilateral, membranous or bony, females being slightly dominant. It is usually associated with coloboma, heart defects, atresia choanal, retarded growth and development, genital hypoplasia, ear deformities (CHARGE) syndrome. Bilateral choanal atresia (BCA) presents at birth with cyclic respiratory distress, pallor, cyanosis relieved only by crying and with feeding difficulty. This anomaly is confirmed by computed tomography (CT) scan which is the investigation of choice. ^[1],[2]

CA was first described in 1755 by Roederer. ^[3] The theory behind this anomaly is thought to be abnormality in the migration and development of the neural crest during embryogenesis. ^[4] Others believe that it's a combination of incomplete resorption of nasopharyngeal mesoderm and the persistence of either the nasobuccal membrane or the buccopharyngeal membrane of the foregut, thus, contributing to the anomaly occurring between the 4 ^th and 11 ^th weeks of the intrauterine life. ^[5]

It has been reported with Cranio-facial- dysmorphism including Down, Treacher-Collin, Crouzon, Apert, Pierre-Robin and orofaciodigital syndromes. ^[6] This suspected, but not proven, life threatening condition requires urgent intervention in the form of mask ventilation, oral airway, endotracheal intubation or rarely tracheostomy, ^[7] till definitive management is carried out. The reported cases of BCA in adults was an isolated anomaly, ^{[8],[9],[10],[11],[12],[13],[14]} with the exception, of the case with multiple congenital anomalies that included congenital right accessory nasal deformity, osteoma of the left ethmoidal sinus and congenital keratoleukoma of the right eye. ^[15] In spite of the suspected morbid outcome of isolated BCA in newborns, unless treated, this has not been verified by any published reports. The only reported death of BCA case was its association with CHARGE syndrome. ^[16]

Case Reports

Case 1

A 60-year-old diabetic female presented to our ENT unit with a complaint of dysphagia for solid meals of 1 year duration. It was of a gradual onset and associated with hoarseness. Clinically she was fairly obese with short and thick neck and no palpable mass or neck nodes. Naso-laryngoscopy, with astonishment, showed BCA, prompting us to perform oro-laryngoscopic examination, revealing right vocal cord palsy. CT of paranasal sinuses [Figure 1] and neck confirmed BCA and showed enlarged thyroid gland extending posterior to the esophagus [Figure 2], respectively. That later proved to be papillary thyroid cancer with lung metastases. Although, the patient could not breathe through her nose for as long as she could remember, it never bothered her, therefore, she never sought medical attention. It has been part of her normal life. Regarding her thyroid, the patient was treated accordingly while her BCA was left in status quo.

Figure 1: Axial computed tomography of sinuses showing bilateral choanal atresia in a 60-year-old female patient presented with dysphagia for sold meals (white arrows)

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Figure 2: Axial computed tomography of the neck showing tumour of the right thyroid lobe (arrow)

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Case 2

A 24-year-old male presented with bilateral chronic nasal blockage for as long as he remembers during which he was a mouth breather. This was associated with recurrent thick nasal discharge. He denied any history of nasal trauma or surgery. There was no other associated complaint. His other sibling had a diagnosis of unilateral choanal atresia.

Endoscopic nasal examination showed BCA with inability to visualise the postnasal space. Other ENT examination was essentially normal and there were no signs of other congenital anomalies.

The diagnosis of bilateral mixed CA [Figure 3] was confirmed by CT scan. The patient underwent transnasal drilling of the bony atretic plate without stenting. He had an uneventful post-operative recovery. One year follow-up showed no evidence of recurrence.

Figure 3: Axial computed tomography sinuses showing bilateral choanal atresia in a 24-year-old male patient presented with chronic bilateral nasal blockage (arrows)

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Discussion

The presentation here clearly showed that there is no fatal outcome of isolated BCA. The explanation given by Baker et al., ^[17] of newborns with BCA to quickly learn and adopt mouth breathing is sound and can be considered an acceptable theory. This may explain the survival of our two adult cases, delivered by a local housewife at home who was not familiar with the anomaly, for obvious reasons.

These patients definitely had respiratory problems at birth. However, the instinct to survive compelled them to quickly learn and adapt breathing through the mouth. Thus, challenging this "suspected" potentially fatal anomaly. However, later in life, according to the first patient, she had uneventful childhood, adolescence, and adulthood until the complaint of dysphagia that appeared at the sixth decade, the oldest yet recorded. Her nasal blockage never bothered her. Further, proving BCA morbidity is subjective as she declined treatment. While the second patient, had nasal breathing problems and sought medical attention at a much earlier age. Worth nothing is the normal development of the paranasal sinuses in both cases further confirming independence of nasal airway patency from BCA as previously reported. ^[18],[19]

In the newborns, suckling requires appropriate integration and timing of movements of the lips, cheeks, tongue, and palate in order to draw milk into the mouth, form a bolus, and propel it to the back of the pharynx to initiate the swallowing reflex. ^[20] This probably has no relation with nasal obstruction. Once this mechanism is not impaired in isolated BCA, survival is recordable. Surgery of BCA in a newborn, who is usually underweight or premature in occasions, carry many hazards including, nasal septal perforation, ^[21] palatal perforation, columella ulceration/necrosis ^[22] and bleeding rarely leading to death ^[23] in addition to restenosis.

The treatment options are multiple, however, in our case we chose to go transnasal and drill out the atresia under direct vision, after gently dissecting mucosa covering the bone, with a good result. The procedure was simple and straight forward. No stent was fixed or Mitomycin C applied as reported in similar cases respectively, ^[9],[15] to prevent stenosis.

Conclusion

We believe isolated BCA in a newborn is compatible with life, if chance is given to overcome the respiratory stress and adopt the new breathing pattern with little help by a pacifier, as it enhances the infant's ability to maintain adequate oral airflow. ^[24] This proposed first line of management, especially for inexperienced surgeons, does not only avoid the risks of surgery mentioned above, but allows the baby to gain weight and size for future plan of surgery. Furthermore, it allows ample time to exclude other associated anomalies. This approach in management, however, requires further studies.

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