Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol Vol: 2 Issue: 4

Congenital Os Vomer Agenesis: A Case Report

Isa Ozbay1*, Hasan Huseyin Balikci2, Turgut Yüce3, Reşit Murat Açikalin3, Ahmet Alp4 and Burak Dikmen3
1Department of ORL, Dumlupinar University, Kütahya, Turkey
2Department of ORL, Susehri Government Hospital, Sivas, Turkey
3Department of ORL, Haseki Research and Training Hospital, Istanbul, Turkey
4Department of ORL, Eyüp Government Hospital, Istanbul, Turkey
Corresponding author : Isa Ozbay
Department of Otolaryngology, Medicine Faculty of Dumlupinar University, Kütahya, Turkey
Tel: +9005059311869; Fax: +9004122580055
E-mail: [email protected]
Received: August 26, 2013 Accepted: October 29, 2013 Published: November 04, 2013
Citation: Ozbay I, Balikci HH, Yüce T, Açikalin RM, Alp A, et al. (2013) Congenital Os Vomer Agenesis: A Case Report. J Otol Rhinol 2:4. doi:10.4172/2324-8785.1000138

Abstract

Congenital Os Vomer Agenesis: A Case Report

The os vomer forms the posteroinferior part of the nasal septum. Defects of the nasal septum can occur as a result of a variety of causes, including infection, irritation, trauma, iatrogenic factors, tuberculosis and neoplasia. Congenital os vomer agenesis is a very rare cause of nasal septal defects. Here, we report the case of a 14-year-old girl with agenesis of the os vomer. The patient presented with complaints of nasal obstruction. We discovered the septal defect on a routine endoscopic examination, and the diagnosis was confirmed by computed tomography. In this report, we present this patient’s case and review the literature on this rare anomaly.

Keywords: Vomer; Agenesis; Congenital; Obstruction; Septum; Defect

Keywords

Vomer; Agenesis; Congenital; Obstruction; Septum; Defect

Introduction

The nasal septum is composed of the os vomer, the crests of the palatine and maxillary bones, the perpendicular lamina of the ethmoid bone and the quadrilateral cartilage [1]. Defects of the nasal septum may lead to a wide variety of nasal symptoms with different degrees of severity depending on the location and size of the defect. Isolated vomeral defects may impair nasal airflow and disturb eustachian tube function and middle ear ventilation [2]. Congenital vomer agenesis is very rare, and it causes a defect of the posteroinferior part of the nasal septum. Here, we present the case of a patient who presented with nasal obstruction due to congenital vomer agenesis.

Case Report

A 14-year-old girl was admitted to our clinic with complaints of nasal obstruction and dryness. She had no history of maxillofacial trauma, nasal surgery, tuberculosis or syphilis, and had not received medical treatment for any nasal problem. The results of laboratory tests were normal. Endoscopic nasal examination revealed a defect in the posteroinferior part of the nasal septum, at the location of the os vomer (Figure 1). The margins of the defect were smooth, and the color of the overlying mucosa was normal. Further examination revealed that the external ears and tympanic membranes were bilaterally normal. There were no anomalies in the craniofacial region; such as submucous cleft palate, cleft lips, aural agenesis and so on. A computed tomography imaging study revealed the complete absence of the vomer bone (Figure 2). There was no complication of this situation. There was no symptom of patient’s relatives and also the endoscopic nasal examination of them was normal. As a treatment, we did not find any treatment modalities for vomer agenesis in literature. For nasal obstruction, we applied radiofrequence therapy to inferior turbinates because there was hypertrophy of inferior turbinates of patients that contributed nasal obstruction of the patient.
Figure 1: Endoscopic view of the vomer agnesis.
Figure 2: Paranasal BT of the patient.

Discussion

The osseous nasal septum is composed of the perpendicular plate of the ethmoid and the vomer. The ethmoid bone forms via endochondral ossification, wherein mesodermal cells first transform into hyaline cartilage and then ossify. In contrast, the vomer develops via intramembranous ossification, which involves direct ossification of mesodermal cells [3]. In the eighth week of gestation, intramembranous ossification begins within the membrane that covers the cartilaginous nasal capsule. During this phase, two ossification centers are present in the posteroinferior part of the nasal septum; they are located between the nasal septal cartilage medially and the nasopalatine nerves laterally [4]. These centers begin to take on a Y-shaped morphology until they become completely fused at approximately 17 weeks of gestation and join the rostral edge of the sphenoid. At the onset of puberty, the laminae are almost completely fused. The correct formation and union of the palatal bones and the palatal apophyses of both maxillary bones are essential for the complete development of the vomer [5].
In 2000, Mohri and Amatsu reported the first cases of congenital agenesis of the os vomer [6]. They proposed two theories to explain the etiopathogenesis of vomer agenesis. One is the “immature ossification center” theory, which proposes that the presence of an incomplete or immature ossification center leads to incomplete development and/ or later absorption of the posterior part of the vomer. The other is the “incomplete downward growth” theory, which suggests that the posterior extension and downward growth of the primary nasal septum is stunted, so that the caudal part of the septum cannot reach the palate when the right and left palatal shelves fuse completely [6].
Doğru et al. reported congenital vomeral defects in two patients with the thalassemia trait [7]. They suggested that thalassemia might have produced this defect via an unknown mechanism. Our patient, however, did not have thalassemia.
This malformation is shown to be a genetically transmitted disease without evidence of other associated anomalies. The disease is not recognised as a syndrome by the genetic department, so further chromosome analysis to identify the disease’s specific gene or genes was assumed to be unnecessary [2]. In the present case, patient had no associated anomalies and examination of patient’s relatives was normal. Therefore, we did not need to examine genetic analysis of the patient.
In conclusion, nasal septal defects can be a result of congenital defects of the vomer bone. To arrive at a diagnosis of vomer bone agenesis, it is necessary to rule out all the causes of nasal septal defects, such as traumatic injury, history of surgery, abuse of inhaled substances, infections, inflammatory processes and neoplasias.

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