The Role of Maxillary Osteotomy in the Treatment of Arhinia

doi:10.1016/j.joms.2012.01.009

Journal of Oral and Maxillofacial Surgery

Volume 70, Issue 5, May 2012, Pages e361-e368

https://doi.org/10.1016/j.joms.2012.01.009 Get rights and content

Purpose

Arhinia is a very rare malformation, and only 41 cases are described in the literature. Given its rarity, there is no standardized surgical protocol. This article describes our preferred treatment, which underlines the importance of maxillary osteotomy for obtaining satisfactory results.

Methods

We observed 3 girls with arhinia, 2 of whom were treated by a 2-step surgical protocol. During the first phase, the patients underwent maxillary osteotomy with the creation of a new epithelium-lined nasal cavity. A skin expander was also placed in the forehead. During the second step, an external nose was created in both patients from the expanded forehead flap with local perinasal flaps and costochondral grafts.

Results

Both reconstructions were viable and esthetically acceptable. No internal nose restenosis was observed.

Conclusions

On the basis of our experience, maxillary osteotomy should be considered part of an integrated approach in treating arhinia.

Section snippets

Case Reports

Patient 1 was born in Morocco to healthy unrelated parents. At birth, she showed no signs of respiratory distress, but she had some difficulty feeding and required an orogastric tube for a few weeks. Her psychophysical development was normal when we first examined her at the age of 6 years. She showed all the typical signs of arhinia associated with a minor form of Treacher Collins syndrome: the external nose, nasal cavity, and paranasal sinuses were absent. In addition, the maxilla was

Discussion

There are 2 goals in the treatment of arhinia. The first is to manage any peripartum complications. The literature includes some reports of neonatal respiratory distress accompanying this disease. This concurs with the dogma of newborns being obligatory nasal breathers. Remarkably, none of our 3 patients showed signs of respiratory distress at birth. This, along with other reports,11, 12, 13 should lead to reconsideration of the principle stating that newborns are absolutely required to breathe

Acknowledgments

The authors thank Dr Paul E. Gravem from Bergen, Norway, for his invaluable help in managing the difficult case of patient 2.

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Citation Excerpt :
Multiple procedures of nasal reconstruction refinements can be necessary during the development, based on the child's growth. Free flaps (e.g. femoral medial condyle flap or iliac crest flap) can be also considered, especially during the patient's growth [35,70,71]. Treatment of congenital nasal septum deviation is, above all, medical.
Congenital bony nasal cavity stenosis is caused by alterations of the normal embryological development of the nasal cavity. Depending on the site of the obstruction, the most important types of stenosis are: choanal atresia and stenosis, congenital nasal pyriform aperture stenosis, congenital midnasal stenosis, arhinia and nasal septum deviation. Although they are uncommon, they could be potentially life-threatening conditions that require early diagnosis and proper treatment. In case of neonatal nasal obstruction, appropriate differential diagnosis with other causes, such as rhinitis and sinonasal masses, are performed by nasal endoscopy and radiological exams. Treatment strategy consisting of medical nasal therapies and endoscopic or open nasal surgery should be tailored according to the types and the degree of the stenosis. When indicated, endoscopic endonasal approach is considered the most effective technique in neonates warranting minimal surgical invasiveness and maximum effect. In order to promote the management of these rare yet clinically relevant neonatal nasal breath disorders, we review the current trends in diagnosis and treatment of congenital bony nasal cavity stenosis.
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A Clinical Report of the Complete Nasal Agenesis: Reconstruction of Congenital Arhinia and Review of the Literature
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Craniomaxillofacial deformities/cosmetic surgeryThe Role of Maxillary Osteotomy in the Treatment of Arhinia

Purpose

Methods

Results

Conclusions

Section snippets

Case Reports

Discussion

Acknowledgments

J Plast Reconstr Aesthet Surg

Int J Pediatr Otorhinolaryngol

J Plast Reconstr Aesthet Surg

Congenital arhinia: Molecular-genetic analysis of five patients

Am J Med Genet A

Congenital arhinia: A rare case

J Indian Assoc Pediatr Surg

Congenital arhinia: One case report

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi

Prenatal diagnosis of total arhinia associated with normal chromosomal analysis: A case report

J Reprod Med

Congenital arhinia

J Paediatr Child Health

Craniomaxillofacial deformities/cosmetic surgery
The Role of Maxillary Osteotomy in the Treatment of Arhinia