Reconstruction of Congenital Arhinia With Stereolithographic Modeling: Case Correlate and Literature Review

Abstract

Complete congenital arhinia is a rare defect of embryogenesis leading to the absence of the external nose and airway. We report our novel multistaged reconstructive approach and literature review. Nasal methyl methacrylate prosthesis was created from a stereolithographic model for use as a temporary prosthesis and tissue expander. Lefort 1 with cannulization was utilized for midface advancement and airway formation. External framework was reconstructed with bilateral conchal bowl cartilage and rib osteocartilagenous grafts. Patient was pleased with the aesthetics and had safe decannulation with the ability to breathe through the nose and airway.

A Clinical Report of the Complete Nasal Agenesis: Reconstruction of Congenital Arhinia and Review of the Literature

“…16 Arhinia can occur due to premature fusion of medial nasal process, abnormal migration of neural crest cells, growth failure of the medial and lateral nasal processes, and resorption deficiency of nasal epithelial plugs. 10,16 Additionally, signaling of the fibroblast growth factor is one of the important pathways for nasal development and its deficiency may cause induce midfacial malformations. 14 To our best knowledge, 44 cases of arhinia have been previously reported in English through 2021.…”

“…Although the genetic pathways involved in nasal development have been investigated, the genetic factor of arhinia has yet to be proven. 16 Congenital arhinia may be accompanied by other associated malformations such as ocular, ear, palate, and gonadal. 16 Therefore, it is recommended to evaluate CT/magnetic resonance imaging (MRI) in the postnatal period.…”

“…16 Congenital arhinia may be accompanied by other associated malformations such as ocular, ear, palate, and gonadal. 16 Therefore, it is recommended to evaluate CT/magnetic resonance imaging (MRI) in the postnatal period. Additionally, a radiological evaluation helps nasal reconstruction by documenting changes in nasal and maxillary anatomy over time.…”

Two Forehead Flaps for Nasal Reconstruction of a Patient with Arhinia

“…The exact pathophysiology of congenital arhinia is unclear, however, several causes such as defective growth of the medial and lateral nasal processes, overgrowth and early fusion of the nasal medial processes, and defective resorption of the nasal epithelial plugs have been suggested 2 .…”

“…Congenital arhinia is usually affiliated with other congenital abnormalities such as the ears, eyes, and palate. Owing to the absence of nose and nasal cavities, the patients may encounter difficulties with breathing and feeding 2 .…”