Congenital vomer agenesis

Abdullah Al-Shahrani; Anas Alshehri

doi:10.4103/sjoh.sjoh_14_22

CASE REPORT

Year : 2022 | Volume : 24 | Issue : 2 | Page : 85-87

Congenital vomer agenesis

Abdullah Al-Shahrani¹, Anas Alshehri²
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 Submission	30-Mar-2022
Date of Decision	11-May-2022
Date of Acceptance	14-May-2022
Date of Web Publication	28-Jun-2022

Correspondence Address:
Dr. Abdullah Al-Shahrani
Department of Otolaryngology, Ahad Rufaida General Hospital, Ministry of Health, Aseer Region
Saudi Arabia

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sjoh.sjoh_14_22

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 2023 Jan 30];24:85-7. Available from: https://www.sjohns.org/text.asp?2022/24/2/85/348718

Introduction

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 6^th 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 8^th 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

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

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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

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Discussion

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

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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.

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