Surgical Odyssey in Treating Recurrent Congenital Nasal Pyriform Aperture Stenosis

Abstract

We present a case report of congenital nasal pyriform aperture stenosis, in which the child was managed with a series of less invasive surgical procedures, which were eventually followed by repair via a sublabial approach. This case report aims to highlight that in severe stenosis, earlier open surgical intervention with bone drilling is suggested, as dilatation alone will result in recurrent stenosis. Open surgery with bone drilling not only facilitates faster recovery but also aids in oxygen weaning, shortens hospitalization duration, and reduces the risk of complications.

Introduction

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition of the newborn, characterized by the narrowing of the nasal pyriform aperture due to excessive bone growth at the nasal process of the maxilla. The presentation of CNPAS varies depending on the degree of obstruction. Since infants are obligate nasal breathers, increased airway resistance results in functional airway obstruction and respiratory distress. The child may present with cyclical cyanosis, respiratory distress and inability to wean from assisted airways in severe cases.

Case Report

A baby girl was born late, premature at 36 weeks gestation, following failed induction of labour due to oligohydramnios. The Apgar score was 9 at 1 min and 10 at 5 min. However, at the tenth minute of life, she developed respiratory distress, necessitating immediate intubation. Despite an initial extubation attempt, she required reintubation at 30 h of life due to worsening respiratory distress. Failure to insert suction catheter size 6Fr into the bilateral anterior nasal vestibule raised suspicion of CNPAS. The introduction of a flexible scope was attempted; however, there was difficulty in advancing the scope. To confirm the diagnosis, a computed tomography scan of the paranasal sinuses (CT PNS) was conducted, revealing inward bowing and thickening of the nasal processes of the medial maxilla, with narrowing of the nasal pyriform aperture with a transverse diameter of 4.4 mm. A solitary median maxillary central incisor was also visualized in the CT scan. Magnetic resonance imaging (MRI) of the brain and pituitary was done to exclude solitary median maxillary incisor syndrome (SMMCI), which revealed normal brain and pituitary structures (Fig. 1a and b).

Fig. 1a.

shows an inward bowing and thickening of the nasal processes of the medial maxilla, with a narrowing of the nasal pyriform aperture with a transverse diameter of 4.4 mm

Fig. 1b shows a solitary median maxillary incisor (indicated by the orange colour arrow)

At 24 days of life, weighing 2.9 kg, the patient underwent a direct laryngoscope, examination under anaesthesia of the nose, submucosal diathermy of the bilateral inferior turbinate and stent insertion. Nasopharyngeal airway size 3.0 was used as stents and kept for two weeks. Unfortunately, following surgery, the child had recurrent pneumonia with restenosis of CNPAS and was thus reintubated. Due to multiple issues, the child was stabilized by the paediatric team. At three months old, another endoscopic dilatation and stent placement were done. Intraoperatively, synechia (adhesions) and bony stenosis were observed at the anterior nasal aperture. Dilatation was carried out, and an endotracheal tube (ETT) size 3.0 was used as a stent. Following the surgery, the child was successfully extubated and remained comfortable while breathing room air. Regular suctioning was performed to prevent blockage of the nasal stent.

Unfortunately, the bilateral nasal stent was dislodged five days after insertion, and restenosis occurred. Additionally, the child developed a new onset of pneumonia, necessitating the need for intubation again. At this stage, we decided on curative CNPAS repair via a sublabial approach to prevent recurrent stenosis. Intraoperatively, synechia was present between the bilateral inferior turbinate and septum, which was released with microscissors. Nasal cavities were then dilated using bougie size 12,14,16. The sublabial flap was raised, and the bilateral nasal process of the maxilla was drilled using a coarse diamond burr size 1.0 (Fig. 2). Bilateral nostrils were stented using ETT size 3.0. The child was successfully extubated to room air postoperatively, and the stent was kept for two weeks. Upon review in our clinic, the child was comfortable, with no sign of respiratory distress. However, we gave her two monthly appointment dates to observe and intervene in case restenosis occur.

Fig. 2.

Sublabial flap raised until the anterior nasal spine and bilateral nasal processes of maxilla are exposed

Discussion

Neonates born with CNPAS usually present with cyclical cyanosis. In severe cases, the child may present with respiratory distress that requires intubation and failure to wean off from the ventilator since birth [1]. In less severe cases, they may present later with feeding difficulties and failure to thrive. Failure to pass suction catheter size 5Fr (1.65 mm)/ 2.2 mm endoscope over the anterior nasal vestibule may suggest choanal atresia or CNPAS, depending on the level of obstruction. An axial CT scan helps to confirm the diagnosis with an aperture of width less than 11 mm at the level of the inferior meatus. A single central incisor may be seen in the CT scan of a few patients diagnosed with CNPAS, which may suggest an association with holoprosencephaly. This finding in CT scan warrants magnetic resonance imaging (MRI) of the brain for further evaluation of the central nervous system, particularly the hypothalamic-pituitary-thyroid axis [2]. Although the child has a central megaloincisor, this may be a sporadic case as all investigations, including radiological and hypopituitary workup, revealed normal findings. Both parents were normal and healthy, and the family has no history of consanguinity or hereditary disease.

Based on a case series of 26 patients, it is found that an aperture of less than 5.7 mm almost always requires surgery [3]. This is consistent with our case, whereby stenting without bone drilling is futile. In another case series, four babies with a mean pyriform aperture width of 4.5 mm were successfully treated with stenting and dilatation. However, 50% needed repeated dilatation [4]. This suggests that in severe cases of CNPAS, single-stage nasal dilation and stenting may not be adequate. As there are limited cases that can be found where a child with severe CNPAS requires multiple-stage surgeries (more than two surgeries), this case report can be a stepping stone for future reference. To prevent restenosis, severe CNPAS with significant airway compromise should be considered for an earlier sublabial approach with bone drilling and stent placement to relieve airway opening. The postoperative regimen should be adhered to, which includes regular nasal saline washes, careful suction not more than 1 cm beyond the stent to prevent trauma, and the use of oxy hood for airway humidification. Stent removal at post-op day five to prevent restenosis and mucosal dehiscence with an additional 48 h of inpatient observation post-stent removal is essential to ensure no complication prior to discharge [5].

Conclusion

For patients with severe narrowing of the nasal pyriform aperture, a single approach might not be enough; open surgical intervention with the drilling of the nasal process of the maxilla may hasten the recovery, aid in oxygen weaning, shorten the hospitalization period and reduce complications. Thus, open surgery with drilling may provide faster relief of stenosis and reduce the risk of ventilator-associated complications that can be observed in our case. As there are limited cases that can be found where a child with severe CNPAS required multiple-stage surgeries (more than two surgeries), this case report can be a steppingstone for future references when dealing with CNPAS with recurrent restenosis.

Funding

No funds, grants, or other support was received.

Declarations

Ethical Approval

All procedures performed in the study were in accordance with the ethical standards of the institution and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from the individual included in the study.

Conflict of interest

The authors have no conflicts of interest to declare that are relevant to the content of this article.