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BACKGROUND Congenital nasal vestibule stenosis (CNVS) is a rare condition often inconsistently termed "congenital anterior nostril stenosis" in the literature. This nomenclatural ambiguity complicates anatomical description and surgical planning. We report the successful management of a late-presenting pediatric case and propose a standardized nomenclature to improve clinical categorization. CASE REPORT An 11-year-old boy presented with a lifelong history of nasal deformity and mouth breathing. Physical examination revealed a flattened nasal tip and bilateral, small, oval nostrils. Each nostril was obstructed 3 mm posterior to the rim by a membranous diaphragm with a 1-mm residual gap. Magnetic resonance imaging (MRI) demonstrated bilateral anterior vestibular narrowing, alar cartilage hypoplasia, and septal deviation. Treatment involved diaphragm excision and vestibular reconstruction using bilateral intranasal Z-plasties, avoiding external incisions. Postoperatively, nasal symmetry, aesthetics, and transnasal airflow significantly improved and remained stable at 3-month follow-up. CONCLUSIONS This case demonstrates that intranasal Z-plasty effectively minimizes external scarring in membranous CNVS for children over 9 years old. We advocate for the unified term "CNVS" and propose a classification based on anatomical location (anterior, middle, or inner) and extent (partial or total). Prioritizing skin conservation, we recommend Z-plasty for partial stenosis and skin grafting for total stenosis to optimize long-term outcomes. Further studies are needed to evaluate the impact on adolescent nasal development.