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


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


  1. Sato D., et al. “Congenital arhinia: Molecular-genetic analysis of five patients”. The American Journal of Medical Genetics - Part A 143 (2007): 546.
  2. Goyal A., et al. “Congenital arhinia: A rare case”. Journal of Indian Association of Pediatric Surgeons 13 (2008): 153.
  3. Meng L., et al. “Congenital arhinia: One case report”. Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 44 (2009): 343.
  4. Brusati R., et al. “Management of a case of arrhinia”. Journal of Plastic, Reconstructive and Aesthetic Surgery 62 (2009): e206.
  5. Thornburg LL., et al. “Prenatal diagnosis of total arhinia associated with normal chromosomal analysis: A case report”. The Journal of Reproductive Medicine 54 (2009): 579.
  6. Zhang MM., et al. “Congenital arhinia: A rare case”. American Journal of Case Reports 15 (2014): 115-118.
  7. Brusati R and Colletti G. “The Role of Maxillary Osteotomy in the Treatment of Arhinia”. Journal of Oral and Maxillofacial Surgery 70 (2012): e361-e368.
  8. Gordon CT., et al. “De novo mutations in SMCHD1 cause Bosma arhinia microphthalmia syndrome and abrogate nasal development”. Nature Genetics 2 (2017): 249-255.
  9. Albernaz VS., et al. “Congenital arhinia”. American Journal of Neuroradiology 17 (1996): 1312.
  10. Akkuzu G., et al. “Congenital partial arhinia: A case report”. Journal of Medical Case Reports 1 (2007): 97.
  11. Mathur NN., et al. “Arhinia”. International Journal of Pediatric Otorhinolaryngology 69 (2005): 97.
  12. Hülya Kılıçoğlu., et al. “Evaluation of Skeletal and Dental Effects of Modified Jasper Jumper Appliance and Delaire Face Mask with Pancherz Analysis”. Turkish Journal of Orthodontics 1 (2017): 6-14.
  13. Thornburg LL., et al. “Prenatal diagnosis of total arrhinia associated with normal chromosomal analysis: a case report”. The Journal of Reproductive Medicine 9 (2009): 579-582.
  14. Feledy JA., et al. “Vertical facial distraction in the treatment of arrhinia”. Plastic and Reconstructive Surgery 7 (2004): 2061-2066.
  15. Galetti R., et al. “[Considerations concerning respiratory physiopathology in a case of total arhinia]”. ACTA Otorhinolaryngologica Italica 1 (1994): 63-69.
  16. Hou JW. “Congenital arhinia with de novo reciprocal translocation, t (3;12) (q13.2;p11.2)”. The American Journal of Medical Genetics - Part A 2 (2004): 200-203.
  17. Fakhraee SH., et al. “Congenital arhinia: case report of a rare congenital anomaly”. Archives of Iranian Medicine 5 (2011): 355-356.
  18. Gifford GH., et al. “Congenital absence of the nose and anterior nasopharynx. Report of two cases”. Plastic and Reconstructive Surgery 50 (1972): 5.
  19. Galetti R., et al. “Considerations concerning respiratory physiopathology in a case of total arrhinia”. ACTA Otorhinolaryngologica Italica 14 (1994): 63.
  20. Olsen ØE., et al. “Congenital absence of the nose: A case report and literature review”. Pediatric Radiology 31 (2001): 225.
  21. Meyer R. “Total external and internal construction in arrhinia”. Plastic and Reconstructive Surgery 99 (1997): 534.
  22. Feledy JA., et al. “Vertical facial distraction in the treatment of arrhinia”. Plastic and Reconstructive Surgery 113 (2004): 2061.
  23. Shino M., et al. “Congenital arhinia: A case report and functional evaluation”. Laryngoscope 115 (2005): 1118.
Citation: Abdelali Halimi., et al. “Bosma Arhinia Microphthalmia Syndrome: A Rare Case” EC Dental Science 21.9 (2022): 71-81.

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