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CASE REPORT
Year : 2018  |  Volume : 6  |  Issue : 1  |  Page : 14-16

Nonfamilial unilateral aplasia of the submandibular gland: A rare finding


Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Web Publication26-Apr-2018

Correspondence Address:
Neeraj Kumar Dhiman
Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jomr.jomr_38_17

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  Abstract 


Congenital absence of the major salivary gland is a rare disorder, and the cause of absence has not been determined yet. In literature, disorder has been described to affect the submandibular and parotid gland along with multiple other facial developmental anomalies. Most commonly, congenital aplasia of submandibular gland is associated with compensatory hypertrophy of contralateral submandibular gland. Clinically, patients may be asymptomatic or may present with dryness of the mouth, difficulty in chewing and swallowing, and dental caries. We present a case of 34-year-old female with right submandibular gland aplasia associated with stone in the left submandibular duct causing proximal dilatation incidentally detected in the computed tomography.

Keywords: Aplasia, congenital developmental anomalies, submandibular


How to cite this article:
Dhiman NK, Vishwakarma AK, Verma V, Singh S. Nonfamilial unilateral aplasia of the submandibular gland: A rare finding. J Oral Maxillofac Radiol 2018;6:14-6

How to cite this URL:
Dhiman NK, Vishwakarma AK, Verma V, Singh S. Nonfamilial unilateral aplasia of the submandibular gland: A rare finding. J Oral Maxillofac Radiol [serial online] 2018 [cited 2018 Nov 20];6:14-6. Available from: http://www.joomr.org/text.asp?2018/6/1/14/231362




  Introduction Top


The term “aplasia” (Greek) is defined as “defective developmental or congenital absence of tissue or organ.” In the present context, it is described as the total or partial agenesis of the gland. Analysis of these abnormalities suggests that the aplasia of salivary glands likely results from the disturbances during fetal development of the first and second branchial arches but the exact etiology is unknown.[1] Any one or group of salivary glands may be absent, unilaterally or bilaterally. Aplasia occurs for unknown reasons as an isolated finding, and most of the cases of congenital absence of the salivary gland may be associated with other developmental anomalies of the face such as hemifacial microsomia, lacrimo-auricular-dento-digital syndrome, and mandibulofacial dysostosis.[2],[3] The first case was presented in 1885 by Gruber and was a bilateral submandibular gland aplasia.[4] Aplasia of the gland manifests with the development of xerostomia and its sequelae. Unilateral submandibular gland aplasia is even rarer with <10–14 cases have been described. Most of them were incidental findings because of the asymptomatic conditions.[5]

The purpose behind presenting this case report is not only as a rare incidental finding on radiograph but also as an isolated phenomenon of early fetal development.


  Case Report Top


A 34-year-old female presented with a chief complaint of pain and tenderness over left submandibular region during mastication for 2 years. The patient also complains of decreased salivation. The medical history showed nothing significant. She had no history of swelling of the submandibular region with any palpable cervical lymph nodes, and no other significant findings were evident on physical examination. The oral mucosa and submandibular papilla on both sides were normal. Computed tomography (CT) revealed a calculus of size 5 mm × 3 mm in mid part of the left duct causing proximal dilatation [Figure 1] with incidentally discovering the absence of the right submandibular gland [Figure 2] secondary to the sialolithiasis. No evidence of any mass or mucosal thickening was seen. Contralateral submandibular gland was normal in size with no compensatory hypertrophy, and no changes were seen in bilateral sublingual gland as well. The presence of subcentimeter size lymph node in submental and bilateral submandibular region is clinically insignificant. The stone was removed surgically without any complications. Postoperative period was uneventful.
Figure 1: Calculus in the left submandibular duct

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Figure 2: Missing right submandibular salivary gland

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  Discussion Top


The development of major salivary glands initiates about 6–8 weeks of gestation. The placodes of major salivary gland initiate as thickening of the oral ectoderm. Salivary gland biogenesis is characterized by branching morphogenesis of epithelium, associated with developing nerves and vasculature to form a branched glandular structure of ducts with terminal buds that become acini by around 14th week. By 13th–16th week, submandibular gland become well differentiated.[6] The cause of aplasia of the salivary glands is still unknown and involves multiple major salivary glands.[7] The aplasia is likely due to arrest in organogenesis but the exact etiology is unknown.[8] Agenesis of major salivary glands may be associated with ectodermal defects of the first and second branchial arches, lacrimal punctum aplasia or hypoplasia and agenesis of lacrimal glands and may be the result of some disturbing influence in early fetal development.[9]

The parotid gland is most commonly affected than the submandibular glands.[10] Isolated unilateral major salivary gland aplasia is a rare entity with about 40 cases reported in the literature till date.[11]

Major salivary gland aplasia may be hereditary or syndromic with an incidence of 1 in 5000 births. Rarely, salivary gland aplasia develops in the absence of any familial history and may exist with no associated anomalies. In 1885, Gruber first time described the aplasia of major salivary gland. Nonfamilial presentation of complete agenesis of unilateral submandibular salivary glands as seen in our cases is rare although agenesis is reported in a variety of unilateral and bilateral combinations.[12]

Unilateral submandibular gland aplasia has been reported to be associated with ipsilateral sublingual gland hypertrophy may also be associated with severe caries in mandibular permanent incisor teeth as well as other signs of salivary hypofunction such as dry mouth, disturbed oral sensation, oral infections,[9] and ectodermal dysplasia.[13] In this case, there was a calculus in the duct of the left submandibular gland causing pain and discomfort. Salivary gland aplasia can be diagnosed with a variety of imaging techniques which include CT, magnetic resonance imaging, sonography, sialography, or nuclear medicine (technetium T99m pertechnetate) studies. Among these methods, ultrasonography is cheap, noninvasive, and widely available and thus very important.[2],[4],[5]

Cone-beam CT (CBCT) equipment is smaller in size and cheaper compared to conventional CT. CBCT provides images of high contrast structures and is therefore particularly more suited for the imaging of osseous structures of the maxillofacial area. Comparison with patient dose reported for maxillofacial imaging by conventional CT (approximately 2000 mSv) indicates that CBCT provides substantial dose reductions of between 98.5% and 76.2%.[14],[15],[16]

In our case, the patient comes with CT which was already done from referred center, and a sialolith in the left submandibular gland was incidentally detected along with right submandibular gland aplasia that might be secondary to sialolithiasis of the left submandibular gland.

Unilateral submandibular gland aplasia is a rare disorder. When such a case is encountered, symptoms and findings should be reevaluated, and the clinician should look for additional deformities. The family history and evaluation should be done for other cases in the family. The etiopathogenesis of this anomaly is still unclear. In this case, the aplasia of the submandibular gland was not associated with any other developmental anomalies of the face. There was no information about the incidence of the congenital absence of the submandibular gland as these are mostly asymptomatic and detected incidentally.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mathison CC, Hudgins PA. Bilateral submandibular gland aplasia with hypertrophy of sublingual glands. Otolaryngol Head Neck Surg 2008;138:119-20.  Back to cited text no. 1
    
2.
Srinivasan A, Moyer JS, Mukherji SK. Unilateral submandibular gland aplasia associated with ipsilateral sublingual gland hypertrophy. AJNR Am J Neuroradiol 2006;27:2214-6.  Back to cited text no. 2
    
3.
Rajendran A, Sivapathasundharam B. Developmental diturbances of oral and paraoral structures. Shafer's Textbook of Oral Pathology. 7th ed. Elsevier India; 2012. p. 36.  Back to cited text no. 3
    
4.
Yilmaz MD, Yücel A, Dereköy S, Altuntaş A. Unilateral aplasia of the submandibular gland. Eur Arch Otorhinolaryngol 2002;259:554-6.  Back to cited text no. 4
    
5.
Roh JL. Unilateral submandibular gland aplasia: An isolated phenomenon of early fetal development. Otolaryngol Head Neck Surg 2006;135:332-4.  Back to cited text no. 5
    
6.
Knox S, Hoffman MP. Salivary gland development and regeneration. In: Wong DT, editor. Saliva Diagnostics. Ames, IW: Black-Well Publications; 2008. p. 3-13.  Back to cited text no. 6
    
7.
Herrera-Calvo G, García-Montesinos-Perea B, Saiz-Bustillo R, Gallo-Terán J, Lastra-García-Barón P. Unilateral submandibular gland aplasia with ipsilateral sublingual gland hypertrophy presenting as a neck mass. Med Oral Patol Oral Cir Bucal 2011;16:e537-40.  Back to cited text no. 7
    
8.
Wiedemann HR. Salivary gland disorders and heredity. Am J Med Genet 1997;68:222-4.  Back to cited text no. 8
    
9.
Fracaro MS, Linnett VM, Hallett KB, Savage NW. Submandibular gland aplasia and progressive dental caries: A case report. Aust Dent J 2002;47:347-50.  Back to cited text no. 9
    
10.
Gangidi S, Cunliffe D. Hyperplastic sublingual gland in association with a congenitally missing ipsilateral submandibular gland. J Surg Case Rep 2011;2011:5.  Back to cited text no. 10
    
11.
Kara M, Güçlü O, Dereköy FS, Resorlu M, Adam G. Agenesis of submandibular glands: A report of two cases with review of literature. Case Rep Otolaryngol 2014;2014:569026.  Back to cited text no. 11
    
12.
Mohan RP, Verma S, Chawa VR, Tyagi K. Non-syndromic non-familial agenesis of major salivary glands: A report of two cases with review of literature. J Clin Imaging Sci 2013;3:2.  Back to cited text no. 12
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13.
Singh P, Warnakulasuriya S. Aplasia of submandibular salivary glands associated with ectodermal dysplasia. J Oral Pathol Med 2004;33:634-6.  Back to cited text no. 13
    
14.
Schulze D, Heiland M, Thurmann H, Adam G. Radiation exposure during midfacial imaging using 4- and 16-slice computed tomography, cone beam computed tomography systems and conventional radiography. Dentomaxillofac Radiol 2004;33:83-6.  Back to cited text no. 14
    
15.
Scaf G, Lurie AG, Mosier KM, Kantor ML, Ramsby GR, Freedman ML, et al. Dosimetry and cost of imaging osseointegrated implants with film-based and computed tomography. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;83:41-8.  Back to cited text no. 15
    
16.
Dula K, Mini R, van der Stelt PF, Lambrecht JT, Schneeberger P, Buser D, et al. Hypothetical mortality risk associated with spiral computed tomography of the maxilla and mandible. Eur J Oral Sci 1996;104:503-10.  Back to cited text no. 16
    


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