مراحل بازسازي عدم تشكيل مادرزادي يكطرفه صورت و بيني: گزارش يك مورد نادر ، (Tessier Cleft No.1)

Congenital Unilateral Arhinia (Tessier Cleft No.1), A very rare anomaly

Background: We report 2 cases of partial arhinia (hemiarhinia) with its complete manifestations, which is a very rare congenital abnormality. Reviewing the literature showed that there were only three cases were reported. This defect can be classified as Cleft no.l, rare craniofacial clefts which were reported by Paul Tessier on 1976. Methods: The patient was treated continuously for past 9y. and different reconstructive procedure was carried out on him to get more symmetric and natural looking appearance. Results: In our opinion, we could not reach a favorable outcome due to complex and multi organ anomaly. Since these cases refer to physicians at early ages, reconstruction with complex flaps such as forehead is far from satisfactory due to a lack of available tissue. Conclusions:These patients suffer from their congenital anomalies , mean while reconstruction of absence of half of the nasal cavity could complicated by injuries on eyes and check bones. Multi specialty teams to cover all anomalies are mandatory and staged reconstruction to have more available tissue and better results are recommended.