Congenital Nasal Vestibular Stenosis in an 11-Year-Old: Case Report, Surgical Management, and a Proposal for Refined Nomenclature

Introduction

Congenital nasal vestibule stenosis (CNVS) is an uncommon problem that results in severe nasal obstruction, mouth breathing, and cosmetic abnormalities in infancy. Most of the earlier studies have focused on acquired nostril or nasal vestibule stenosis due to burns, infections, trauma, or iatrogenic harm, and congenital cases are rarely mentioned, typically in neonates or infants [1–5]. Various methods, such as Y-V- and W-plasties, starplasty, local flaps, skin grafts, and serial dilatations, have been used [6–10]. Nonetheless, it is not yet possible to have a universally agreed terminology that can be used to differentiate between the severity and magnitude of the obstruction of the nasal vestibule, and there is a lack of agreement on the best surgical management approach in older children. This report presents the case of an 11-year-old boy with chronic nasal deformities who underwent surgical treatment, and provides a tentative descriptive framework of CNVS [11,12].

Case Report

PROCEDURE:

The patient was positioned on his back and given general anesthesia through transoral endotracheal intubation. The incision pattern created was a longitudinal S-shape made based on the small existing orifice at midline of the stenotic tissue of the right nostril. To attain hemostasis, local infiltration of 1: 400 000 epinephrine in saline was performed, which resulted in tissue blanching.

The dissection was done along the atretic membrane. The intraoperative examination revealed that the posterior area of the stenosed tissue was composed of skin tissue and small quantity of subcutaneous fat tissue. A sharp dissection of the membrane was done in the subcutaneous fat that divided the membrane into anterior and posterior portions. On the side of the septum, the posterior part of the membrane was resected, and the remaining anterior flap was trimmed and stitched to the edge of the septal wound. Conversely, on the alar side, the anterior layer was removed, but the posterior flap was left, trimmed, and sutured to the border of the alar defect. Postoperatively, the inner surface of the nasal vestibule was flat and smooth, with the suture being in several planes instead of a single linear plane. The stenosis was effectively relieved, and the nasal mucosa was regained with nasal patency. The same operation was repeated on the left nostril. Povidone-iodine gauze-coated silicone tubes were employed as postoperative packing material.

After 7 days after surgery, the cuts had properly healed, the 2 nostrils looked almost exactly alike, and the transnasal airflow was sufficient under moderate-intensity exercise such as fast walking (Figure 3). The patient was advised to breathe through his nose to assist growth of cartilage and nasal development. At the 3-month follow-up, the functional and aesthetic outcomes were still satisfying (Figure 4). Correction of the low nasal tip, short columella, and abnormality in nostril configuration were postponed until adulthood. Figure 5 is a schematic drawing of the incised design, flap transposition, and the end result.

Discussion

Etiology and Pathogenesis: CNVS is believed to develop due to improper prenatal development of the nasal vestibule. In a normal embryo, temporary epithelial plugs occlude the front of the nasal chamber and later dissolve to form a patent air passage. Maldevelopment or incomplete development of this mechanism can result in membranous tissue remaining in place in the vestibule, producing stenosis or atresia. Other possible etiologies are hypoplasia of the alar cartilage, abnormal attachment of the alar base, and abnormal development of the columella. This membranous obstruction in the anterior nostril or vestibule has been documented newborns and infants [1–3]. Our patient had bilateral membranous atresia and associated alar chondrodysplasia and septal deviation suggestive of a developmental- as opposed to trauma-related cause. However, the exact pathophysiology of CNVS is unknown and is probably multifactorial.

Nomenclature: Nasal vestibule is the part of the nasal airway that lies between the external nostril and the nasal valve. Medially, it forms the membranous nasal septum and laterally it is made of alar soft tissues consisting of alar cartilage and the superficial layer of skin. Previous works have referred to congenital narrowing of this area with various names, including congenital anterior nostril stenosis, congenital nasal vestibular stenosis, and congenital nostril atresia. The terminology does not clearly differentiate between the obstruction of the external nostril, the middle vestibule, or the innermost nostril near the valve. To indicate the location of the disease in the anatomy, we have chosen to use the term congenital nasal vestibule stenosis (CNVS) in this report and specify the level of stenosis as anterior, middle, or inner nasal vestibule, if needed.

From a pragmatic point of view, we suggest a descriptive classification based on dividing CNVS into partial and complete nasal vestibule stenosis. Partial CNVS is the involvement of 1 portion of the vestibule and may be subdivided into anterior (external nostril), intermediate (as with our patient, about 3 mm into the nostril), or internal (close to the nasal valve). Total CNVS means total blockage of the vestibular duct. The functional severity of stenosis can be defined as a mild, moderate, or severe, depending on the symptoms and exercise tolerance, such as constriction to quiet breathing, dyspnea during exercise, or inability to breathe through the nose. The intent in this framework is to provide an easy-to-use descriptive system to enhance communication and surgical planning and not to be considered a formal classification system. It has its foundation on limited clinical experience and must be confirmed in large series.

Clinical Manifestations: People affected by CNVS usually manifest chronic nasal obstruction, mouth breathing, snoring, and sometimes inability to feed properly or failure to thrive. Caregivers might also observe that their child is prone to repeated upper respiratory tract infections or otitis media because of poor nasal breathing. Examination shows that the nose tends to be small and flat, and the nostrils are narrowed or asymmetrical and that the nasal apex is lower. It is possible to see membranous tissue in the anterior nostril or vestibule at times as a tiny perforation that permits slight air passage. The prolonged obstruction causes mouth breathing, malocclusion, and psychological and social issues associated with facial appearance.

Diagnosis: To diagnose CNVS, a thorough history and careful physical examination should be combined with a nasal endoscopy and imaging. Lifelong nasal obstruction and mouth breathing, especially without allergic symptoms, is a condition that needs to evoke suspicion. Basal and frontal images are used to determine the size and shape of the nostrils and their symmetry and intranasal exploration can detect membranous tissue inside the vestibule. Endoscopy of the nose aids in directly examining the amount of narrowing and serves to rule out intranasal masses or choanal atresia. Computed tomography and MRI provide more detail on the structure of bone and soft tissue, such as the alar cartilage, the nasal septum, and paranasal sinuses, and can be helpful in surgical planning in older children.

Management: Early surgery should be offered as soon as CNVS leads to clinically meaningful obstruction, feeding difficulties, or growth impairment. The main concept is to excise the membranous or overly-developed soft tissue causing the stenosis and re-create a non-contracting and well-epithelialized nasal vestibule. Many different methods have been published, such as the release and serial stenting, starplasty, modified Y-V or W-plasty, local flap transfers, and skin grafts, most of them created in patients with acquired nostril stenosis [5–11]. Treatment selection is based on the degree and severity of stenosis, condition of the neighboring tissues, and patient age.

In such cases, our strategy is to increase the middle vestibule area and protect the shape of the nose externally by performing bilateral intranasal S-shaped incisions and opposing local flaps. The method does not involve external scars, provides control over the size of the vestibular canal, and creates an epithelial layer with similar tissue properties. To reduce the risk of restenosis postoperative, nasal packing, topical medication, and periodic monitoring are necessary. Although our patient had steady improvement in nasal airflow and appearance at 3 months after treatment, a longer duration of observation is required to assess the growth of the nose over time and the risk of late contracture.

Constraints and other methods: We present the case of 1 patient who was followed up for only 3 months, which is inadequate to monitor for late scar contraction, restenosis, or developmental variations of the septum and external nose. Outcomes were assessed mainly according to clinical examination, and objective functional measures (eg, standardized endoscopic scoring, acoustic rhinometry, or validated symptom scales) were not used. CNVS is heterogeneous; therefore, this intranasal S-incision vestibular plasty with opposing flaps might not be effective in individuals with large lining or skin shortage, circumferential scars, or total or nearly total vestibular atresia, where it can be possible to reconstruct using grafts. Published alternatives to treat vestibular stenosis are long-term stenting combined with serial dilation, starplasty, modified Y-V/W-plasty, local perialar flaps, or split or full-thickness skin grafts, depending on the degree of stenosis and quality of tissue [12]. Definitive open septorhinoplasty and fixing of residual tip/columellar abnormalities in growing children are usually postponed to adulthood so as not to interfere with the developmental processes of the nasal cavity.

Conclusions

Congenital nasal vestibule stenosis (CNVS) is a rare condition that causes nasal obstruction and facial malformation in children. This report demonstrates that nasal patency and nasal symmetry in an older child can be achieved by careful relief of membranous stenosis with restoration of the nasal vestibule. Endoscopy, CT, and MRI are very helpful in determining the degree and range and evaluating the adjacent nasal tissues, thus helping select the best surgical plan. Based on this case and a cursory examination of the literature, we suggest that using the unifying terminology of CNVS, tentatively separate partial nasal vestibule stenosis (anterior, middle, or inner), and total nasal vestibule stenosis as a simple descriptive scheme, not as a formal classification. Early surgical treatment of vestibular stenosis is possible once the diagnosis is clear, but revision surgery of the remaining nasal tip and columella deformities can be reasonably postponed until the patient is an adult, to achieve the most cosmetically pleasing result.