A Startling Revelation Behind a Stifled Breath: A Unique Case Report

INTRODUCTION

Otorhinolaryngology contains a wide spectrum of congenital anomalies, with choanal atresia being one of them. The incidence is about 8,000 live births, with unilateral being two times commoner than bilateral and more likely to be on the right side.¹

The atresia can be bony, membranous, or mixed. CHARGE syndrome is the commonest association composed of coloboma, heart defects, choanal atresia, growth retardation, and ear anomalies. Choanal atresia may occur even without any associations, which is seen in most cases of unilateral variety. It was Johann George Roederer who first described this anomaly in 1755, after which several surgeons such as Haag, Hubert Von Luschka, Kiel, Freer, and many others continued to research this anomaly.²

Choanal atresia is attributed to the persistence of the buccopharyngeal membrane from the foregut or to mesodermal adhesions in the nasochoanal region, and another theory suggests a misdirection of neural crest cell migration.

Patients with unilateral choanal atresia often present late in childhood and rarely in adulthood with unilateral nasal obstruction, mucopurulent discharge, and sinusitis-like features. Making a diagnosis with such a presentation of choanal atresia requires meticulous examination and suspicion. Surgical intervention is necessary for the creation of a neochoana.^3,4

We hereby present one such unique case that went undiagnosed for 22 years and have discussed the surgical management and outcome of the same.

CASE DESCRIPTION

A 22-year-old male patient presented to the Otorhinolaryngology OPD with complaints of right nasal obstruction since childhood. It was aggravated by exposure to a cold climate and episodes of URTI and was not relieved by nasal drops, sprays, or oral medications. No H/o headache, fever, facial pain, or facial swelling; no H/o repeated bouts of sneezing or itching of the eyes; or watery nasal discharge. The patient had no known comorbidities and no history of previous surgeries or chronic medications. The general physical examination was normal, and vitals were stable.

On examination, the external appearance of the nose was normal. A cold spatula test showed absent misting on the right side. On anterior rhinoscopy, there was a left anteriorly deviated nasal septum and a right nasal cavity filled with mucoid discharge. There was no paranasal sinus tenderness. The oral cavity and oropharynx were normal. The systemic examination was normal.

On diagnostic nasal endoscopy, in the right nasal cavity, thick mucoid discharge was present; suction clearance was done. The right gross bony deviation with spur was present, due to which, beyond the middle meatus, we were unable to pass the endoscope to visualize the nasopharynx. The left anterior cartilaginous deviation of septum with antero-inferior spur was present.

A CT scan of the paranasal sinuses was done, which showed opacification in the right nasal cavity with a gross right bony deviated nasal septum, and the paranasal sinuses were clear (Fig. 1).

The patient was planned for surgery; septoplasty was done under GA, and the gross bony and cartilaginous deviations were corrected. The middle meatus was examined, and the osteomeatal complex was found to be normal. Following which we could navigate the scope beyond the middle meatus, and that is when, to our surprise, we found there was no patency of choana suggestive of choanal atresia (Fig. 2). Upon probing, it was membranous, and a decision was taken on the table for microdebrider-assisted widening atresia, and a neochoana was created.

To maintain the patency, a nasal stent was placed and secured with sutures to the ala. We confirmed position by passing the scope from the opposite nasal cavity (Fig. 3). Postoperatively, the patient was given decongestant nasal sprays and frequent nasal washes to prevent crusting. He showed good symptomatic relief immediately postoperatively. The stent was in situ for 3 weeks, as shown in image 1, after which a diagnostic nasal endoscopy was done, and then the patency was confirmed and the stent was removed. No synechia or restenosis was seen at 4 and 6 weeks of follow-up, and the patient was completely relived symptomatically (Fig. 4).

DISCUSSION

Choanal atresia is an uncommon congenital anomaly of the nasal airway characterized by complete obliteration of the choana. It is due to the persistence of the naso-buccal membrane. It was first described by Roederer in 1755. Unilateral choanal atresia is more common as compared to bilateral choanal atresia and can present later in life with unilateral nasal obstruction, recurrent rhinorrhea, and foul-smelling nasal discharge. Choanal atresia is of the bony type in 30% of cases; only less than about 10% of cases are membranous, and 60–70% of cases, it is mixed type.^1,2

Neonates with bilateral choanal atresia, by virtue of being obligate nasal breathers, present immediately after birth with respiratory distress, aspiration, and even cardiac complications, which require immediate surgical intervention to secure the nasal airway. Whereas unilateral choanal atresia often gets missed and has a delayed presentation in childhood or early adulthood.^3,5–9

Computed tomography of paranasal sinuses is a gold standard tool for imaging in such cases, but, again, it is difficult to look for membranous defects, as seen in our scenario, where the atresia was only noted on endoscopy. There have been several approaches described for choanaoplasty: transnasal, transseptal, transpalatal, and transantral.

In only two decades, Stankiewicz performed a successful endoscopic repair, after which blind procedures are now diminishingly used. Endoscopic surgeries have very good visualization, and with precise powered instruments such as microdebriders and drills, the removal of atretic segments can be done efficiently.⁹ Other approaches, such as transpalatal resection, also offer good exposure, but this method has multiple complications, such as palate flap necrosis, fistula, and crossbite defects. We performed microdebrider-assisted repair without posterior septectomy and were able to successfully correct the anomaly.^9,10

Restenosis of the choanae is a major problem in surgery for choanal atresia. The literature varies on surgical failure, as the rate of restenosis ranges from 0 to 85% of the cases. Surgical failure is considered when choanal restenosis is more than 50% of the diameter. Controversy exists over the use of stenting as well as the use of fibroblast inhibitors (Mitomycin C).^7,10

Various methods of stenting have been described by multiple authors. The materials used as stents are modified endotracheal tubes, nasopharyngeal airways, silastic stents, polyvinyl chloride stents, Teflon, silicone, and even the newer steroid-eluting stents.^7,11 In our case, we used the commonly available portex nasopharyngeal airway, refashioned it into the appropriate dimensions, and used it for stenting after creating the neochoana, giving us equivalent outcomes to commercially available stents. Portex stents have been used by others too and have the advantage of being resilient and yet malleable, hence lasting longer with assured patency and being comfortable for bilateral and unilateral repairs in adults as well as children. Stents can be regularly instilled with saline and suctioned to prevent crusting.^12,13 Traditionally, stents are left in situ for 3–6 weeks for re-epithelialization of the neochoana. Stents do pose a possible complication of granulation tissue formation and skin or alar necrosis in children, but in adults, if sutured without tension, they have been seen to give a good outcome.¹⁴ In our study, we did not have any complications with respect to stent placement.

ORCID

Chenchulakshmi Vasudevan https://orcid.org/0000-0002-8127-9706

REFERENCES

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