• Users Online: 365
  • Print this page
  • Email this page


 
 
Table of Contents
CASE REPORT
Year : 2022  |  Volume : 24  |  Issue : 2  |  Page : 85-87

Congenital vomer agenesis


1 Department of Otolaryngology, Ahad Rufaida General Hospital, Ministry of Health, Aseer Region, Saudi Arabia
2 Department of Otolaryngology, King Faisal Medical City, Saudi Arabia

Date of Submission30-Mar-2022
Date of Decision11-May-2022
Date of Acceptance14-May-2022
Date of Web Publication28-Jun-2022

Correspondence Address:
Dr. Abdullah Al-Shahrani
Department of Otolaryngology, Ahad Rufaida General Hospital, Ministry of Health, Aseer Region
Saudi Arabia
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sjoh.sjoh_14_22

Rights and Permissions
  Abstract 


The nasal septum is composed of the quadrangular septal cartilage, crests of the maxillary and palatine bones, the perpendicular plate of the ethmoid bone and the vomer. vomer agenesis is a very rare condition in which misinterpreted as other causes including pervious trauma, infection, neoplasia, and Granulomatous and Systemic Diseases Affecting the Nose. This is a 35-year-old male diagnosed with chronic rhinosinusitis with nasal polyposis (CRSWNP). While performing a preoperative CT scan, there was a defect in the vomerian bony part of the nasal septum. Congenital vomer agenesis is a rare condition and few reports are mentioned in the literature. It can be associated with other head and neck diseases. A careful history and physical examination are needed to exclude other causes of the septal defect. The utilization of imaging studies and endoscopic examinations will reveal more cases in the future.

Keywords: Congenital vomer agenesis, endoscopic examination, immature ossification, nasal septum


How to cite this article:
Al-Shahrani A, Alshehri A. Congenital vomer agenesis. Saudi J Otorhinolaryngol Head Neck Surg 2022;24:85-7

How to cite this URL:
Al-Shahrani A, Alshehri A. Congenital vomer agenesis. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2022 [cited 2022 Dec 5];24:85-7. Available from: https://www.sjohns.org/text.asp?2022/24/2/85/348718




  Introduction Top


The nasal septum is composed of the quadrangular septal cartilage, crests of the maxillary and palatine bones, perpendicular plate of the ethmoid bone, and vomer.[1] In the first 28 days of gestation, the face develops from five swellings: paired maxillary and mandibular processes and unpaired frontonasal process.[2] During the 6th week, the medial nasal processes fuse to form the intermaxillary process, which is the primordia of the septum and the bridge of the nose. The palatal shelves shift on either side of the inferior edge of the precartilaginous nasal septum with the nasopalatine nerve in the 8th gestational week.[3] During development, the vomer mineralization foci begin to appear after the fusion of the cartilaginous nasal septum with the palatal shelves. Vomer agenesis (VA) is a very rare condition that is misinterpreted as other causes, including previous trauma, infection, neoplasia, and granulomatous and systemic diseases affecting the nose.[4]


  Case Report Top


A 35-year-old man presented with bilateral nasal obstruction and no history of nasal trauma, septal surgery, neoplasia, infection, or chronic inflammatory diseases affecting the septum. Clinical endoscopic examination revealed bilateral nasal polyposis and an anterior septal spur on the left side. Further examination of the ear and oral cavity revealed no abnormalities, and indirect laryngoscopy results were normal. On a preoperative computed tomography scan, we found a left-sided septal spur, bilateral hypertrophied inferior turbinates, defect in the posteroinferior portion of the septum, and bilateral sinonasal polyposis [Figure 1].
Figure 1: Computed tomography scan of the peripheral nervous system. (a) Coronal and (b) axial view showing a posteroinferior nasal septum defect in the proposed vomerine part

Click here to view


After endoscopic sinus surgery and polypectomy, we were able to identify the defect as agenesis of the vomer bone of the nasal septum. The mucosa was intact and normal [Figure 2].
Figure 2: Intraoperative image showing the absence of the vomer bone with intact mucosa around it

Click here to view



  Discussion Top


The vomer and perpendicular plate of the ethmoid form the bony part of the nasal septum. During development, the vomer's mineralization foci appear after the fusion of the cartilaginous nasal septum with the palatal shelves. Several hypotheses have been proposed as possible causes of the isolated congenital absence of the vomer.[5],[6] The “immature ossification center” and “incomplete downward growth” theories by Mohri and Amatsu suggest that an immature ossification center could lead to underdevelopment of the vomer. Other theories, such as “the incomplete touch theory,” state that the incomplete union of the ossification line of the cartilaginous septum inserted in the vomerine line with the surrounding soft tissues is responsible for VA.[7] According to Verim et al.,[6] VA can be classified into two types. In type 1 aplasia, the vomer is absent from the middle turbinate to the choanae, whereas in type 2 aplasia, only the caudal vomer portion is absent (partial aplasia).

Literature reviews frequently describe how VA can be associated with other concomitant findings. Most of these are located in the head-and-neck subsites. It can be found in conjunction with different types of cleft palate or may be seen with severe and complex craniofacial anomalies.[6] In our patient, we observed compensatory hypertrophy of both inferior turbinates. Other authors have described lower turbinate hypertrophy in VA.[6],[8],[9],[10] In a literature review, including our case, six of 21 cases had otological pathologies (Mohri and Amatsu, Doğru et al., and Yilmaz and Altuntas).[5],[8],[9] Other reports by Lee et al. and Kang et al.[11],[12] have shown that VA developed concomitantly with sinonasal diseases; their findings were consistent with those in our case. Other reported cases are summarized in [Table 1]. The genetic nature of VA was studied by Verim et al.[6] in their case series. However, to date, no study has approved this effect in the literature, since it carries no significant medical problems.
Table 1: A list of reported vomer agenesis cases in the literature

Click here to view


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Davies J, Duckert L. Embryology of the head, neck, face, palate, nose and paranasal sinuses. In: Paparella MM, Shumrick DA, Gluckman JL, Meyeroff WM, Editors. Otolaryngology. 3th ed., Philadelphia: W.B. Saunders; 1991. p. 59-90.  Back to cited text no. 1
    
2.
Pansky B. Review of Medical Embryology [E-book]. McGraw-Hill Education. 1982.  Back to cited text no. 2
    
3.
Sandikcioglu M, Mølsted K, Kjaer I. The prenatal development of the human nasal and vomeral bones. J Craniofac Genet Dev Biol 1994;14:124-34.  Back to cited text no. 3
    
4.
Dudek RW. Embryology BRS. 5th ed., Philadelphia: Lippincott Williams and Wilkins; 2009. p. 1-240.  Back to cited text no. 4
    
5.
Mohri M, Amatsu M. Congenital defects of the vomer. Ann Otol Rhinol Laryngol 2000;109:497-9.  Back to cited text no. 5
    
6.
Verim A, Faruk Çalım Ö, Yenigün A, Kocagöz GD, Kökten N, Özkul H. Hereditary characteristic of isolated congenital vomer aplasia. J Craniomaxillofac Surg 2012;40:e392-6.  Back to cited text no. 6
    
7.
Takahashi R. The formation of the nasal septum and the etiology of septal deformity. The concept of evolutionary paradox. Acta Otolaryngol Suppl 1987;443:1-160.  Back to cited text no. 7
    
8.
Yilmaz MD, Altuntaş A. Congenital vomeral bone defect. Am J Otolaryngol 2005;26:64-6.  Back to cited text no. 8
    
9.
Doğru H, Yasan H, Tüz M. Congenital vomeral bone defect in two thalassemia trait cases. Eur Arch Otorhinolaryngol 2004;261:136-8.  Back to cited text no. 9
    
10.
Uçar S, Aydın Ö, Öztürk M. Congenital vomer agenesis. Kulak Burun Bogaz Ihtis Derg 2015;25:182-4.  Back to cited text no. 10
    
11.
Lee JH. Congenital vomeral bone defect: report of two cases and a review of the literature. ActaOtolaryngol 2006;126:1229-31.  Back to cited text no. 11
    
12.
HeeJoon K, Hyun-Woo L, Jae HS, Heung-Man L. Congenital defect of the vomer bone: A rare cause of septal perforation. Int J of PedOtorhinolaryngol Extra 2007;2:17–9.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures
Article Tables

 Article Access Statistics
    Viewed294    
    Printed28    
    Emailed0    
    PDF Downloaded48    
    Comments [Add]    

Recommend this journal