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Nasal Reconstruction in a Rare Case of Congenital Arhinia

Lucas Harrison BS, David Jerkins MD, Salim Mancho MD, Steven Schmidt MD
Wright State University Department of Plastic Surgery
2018-01-30

Presenter: Lucas Harrison

Affidavit:
This project will be presented by a medical student and not the resident.

Director Name: Michael Johnson, MD

Author Category: Medical Student
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial

PURPOSE:
Congenital arhinia is a rare defect of embryogenesis leading to absence of the external nose and nasal airway often linked with other disorders of the face such as the eyes, ears, and palate. This study reports on the presentation, treatment, and outcomes of a spontaneous case without associated syndrome.

METHODS:
Retrospective chart review was performed for one patient from 1994 – 2017. The collected information included the age, gender, medical comorbidities, etiology of the defect, prior surgical operations, and outcomes. Patient underwent several staged reconstructions over more than a decade.

RESULTS:
A 22-year-old male underwent a two stage procedure for congenital arhinia. Patient and initially refused large flap reconstruction early in childhood so patient underwent local flap rearrangement for external nares and nasal dorsum follow years later by a Lefort 1 Maxillary advancement with recreation of nasal airway lined with skin graft. This was followed by a nasal reconstruction with a cantilever rib graft and bilateral ear cartilage alar grafts. Arterial embolization was performed of two medial maxillary that passed through the normal location of the nasal airway. There were no surgical complications or flap loss. The patient's tracheostomy following birth was finally de-canulated after stable nasal airway was constructed. Patient did not have audible velopharyngeal insufficiency after creation of nasal airway.

CONCLUSION:
Construction of both the nasal internal and external architecture is important in the treatment of congenital arhinia and in this study we present a novel approach for surgical intervention.

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