Background: After presenting two sisters with the rare form of congenital arrhinia, this syndrome is reviewed, an explanation of the pathogenesis is offered and the therapeutic options of the functional and aesthetic reconstruction are discussed.
Discussion: In cases of congenital arrhinia different degrees of respiratory distress, cyanotic episodes, and impaired food intake are described. Therefore after birth respiration and food intake need to be monitored to alleviate the situation through intubation or tracheotomy. The following conclusions could be made based on the literature overview. Little is known about the pathophysiology and a great variety of therapeutic interventions and reconstruction solutions with a wide spectrum of complications are described. Due to the numerous forms of complications, which need to be compared with the reconstructive results, indications for surgical reconstruction of the airway and plastic reconstruction of the nose during childhood must be defined very stringently.
Conclusion: One method to achieve a satisfactory plastic result is with an osseointegrated prosthesis. This facial prosthesis can be inserted without complications and can guarantee an adequate result, whereas no impairment of maxillofacial development was noted.