Case Report
Volume 21 Issue 8 - 2022
Bosma Arhinia Microphthalmia Syndrome: A Rare Case
Abdelali Halimi1*, Loubna Bahije1, Choumi Faissal2, Alae Guerrouani3, Mohamed El-Okeily4, Hicham Benyahia1 and Fatima Zaoui1

1Department of Orthodontics and Dentofacial Orthopedics, Research Team of Biotech, MMB, Drug Science Center, Mohamed-V University (UM5) in Rabat, Rabat City, Morocco

2Faculty of Medicine and Pharmacy of Fez, Department of Stomatology and Maxillofacial Surgery, Mohamed-V University (UM5) in Rabat, Morocco

3Oral and Maxillofacial Surgeon, Al Farabi Hospital, Oujda, Morocco

4Chirurgien Esthétique, SOFCPRE - Société Française de Chirurgie Plastique, Reconstructrice et Esthétique, Bordeaux, France

*Corresponding Author: Abdelali Halimi, Professor in Orthodontic, Department of Orthodontics and Dentofacial Orthopedics, Research Team of Biotech, MMB, Drug Science Center, Mohamed-V University (UM5) in Rabat, Rabat City, Morocco.
Received: June 04, 2022; Published: August 29, 2022




Abstract

Purpose: Bosma Arhinia Microphthalmia Syndrome (BAMS) is a very complex and rare malformation, and only few cases are described in the literature and there is no standardized surgical protocol. This article describes a typical BAMS patient and presents the treatment plan of this patient underlining the key role of protocol in obtaining satisfactory results.

Methods: We observed a Moroccan male infant, 7-year-old presented a typical appearance of BAMS, clinically observed and genetically confirmed. He was treated by a 3-step surgical protocol. During the first phase, a Le Fort II maxillary osteotomy with a bicortical approach under submental intubation was done. The effect of this osteotomy was maintained and potentiated by the Delaire Face Mask. In a second step, the patient benefited from a parietal bone graft. In 3rd step, the patient underwent an internal and external nasal reconstruction.

Results: An acceptable morphology for the newly created external nose was obtained, this reconstruction was viable and esthetically acceptable. No internal nose restenosis, a slight external restenosis of a left nave was observed. This result was stable over 1 years.

Conclusion: On the basis of our experience, a multidisciplinary team is very important to succeed the treatment BAMS. Maxillary osteotomy should be considered part of an this approach with external traction of the maxilla, cortical graft and reconstruction of internal and external nose.

 

Keywords: Arhinia; Congenital; Reconstruction

References

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Citation: Abdelali Halimi., et al. “Bosma Arhinia Microphthalmia Syndrome: A Rare Case” EC Dental Science 21.9 (2022): 71-81.

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