Ortho-surgical management of Arhinia syndrome: A case report

Introduction and importance: Arhinia syndrome (AS) is an extremely rare malformation characterized by typical clinical signs such as a lack of nose, with difficulty in breathing and inability to feed due to airway obstruction. The ortho-surgical management of AS is a big challenge and it requires an interdisciplinary relationship between the orthodontist and a maxillofacial surgeon. The authors reported a case of AS. Case presentation: The only case in Morocco among fewer than 30 cases described in the whole world, male child, 7-year-old. Clinical findings and investigations: Complete absence of the external nose, nasal cavities, and other characteristic signs. This diagnosis was genetically confirmed. Interventions and outcome: A LeFort II maxillary osteotomy was done in combination with a facemask and miniscrew followed by a parietal bone graft and maxillary expansion with a customer expander. An internal and external nasal reconstruction was undergone with orthodontic management of unerupted teeth. An acceptable morphology for the newly created external nose was obtained, this reconstruction was viable and esthetically acceptable. A slight external restenosis of a left nave was observed. This result was stable over three years. Relevance and impact: AS is a rare condition with a pathogenesis not been fully understood. The ideal treatment should have the lowest possible morbidity and a low relapse rate. The most difficulty in the approach is the creation and maintenance of a nasal cavity. These therapeutic aspects will be discussed through this case. The use of facemask therapy with a miniscrew potentiated the effect of osteotomy and stabilized the parietal bone graft which allowed nasal reconstruction and orthodontic management of unerupted teeth in this case.