Case Report Agenesis of Submandibular Glands: A Report of Two Cases with Review of Literature Medine Kara, 1 OLuz Güçlü, 1 Fevzi Sefa Dereköy, 1 Mustafa Resorlu, 2 and Gürhan Adam 2 1 Department of Otolaryngology, Canakkale Onsekiz Mart University Medical Hospital, Canakkale, Turkey 2 Department of Radiology, Canakkale Onsekiz Mart University Medical Hospital, Canakkale, Turkey Correspondence should be addressed to Medine Kara; medinekara@gmail.com Received 20 May 2014; Revised 16 August 2014; Accepted 22 August 2014; Published 1 September 2014 Academic Editor: Kenichi Takano Copyright © 2014 Medine Kara et al. Tis is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Background. Congenital absence of the submandibular gland (SMG) is a rare condition. Although complaints such as dry mouth, dental problems, or difculty in swallowing may be seen, the subjects may also be asymptomatic. Te absence of the SMG may be associated with hypertrophy of the contralateral SMG. Case Report. We report the case of a 44-year-old woman with incidentally detected lef SMG aplasia, with contralateral SMG hypertrophy mimicking a mass, and the case of a 46-year-old woman with incidentally detected bilateral SMG aplasia, demonstrated by computerized tomography (CT) and magnetic resonance imaging (MRI). Conclusion. It is important for the clinician to know that this very rare abnormality may exist. When such a case is encountered, symptoms and fndings should be reevaluated and, if necessary, conservative therapy should be initiated. Te possibility of observing additional deformities should be kept in mind and an evaluation should be done for other cases in the family. 1. Introduction Congenital absence of the submandibular gland (SMG) is a rare condition. Te term “aplasia” is described as the total or partial agenesis of the gland [1]. In the English medical literature, approximately 40 cases have been reported to date. Te frst case was presented in 1885 by Gruber and was a bilateral SMG aplasia [2]. Although its etiology is not known, it is thought to result from a defect that occurs during fetal development. In such subjects, additional developmental deformities may also be observed. Clinical syndromes, such as lacrimo-auriculo-dento-digital syndrome and mandibulo- facial-dysostosis (Treacher-Collins syndrome), may also be seen [3]. Unilateral SMG aplasia is ofen asymptomatic and is usually discovered incidentally through imaging [4]. Due to an inadequate amount of saliva in these cases, some symptoms such as dry mouth, dental problems, and difculty swallowing are seen. However, while asymptomatic subjects constitute approximately half of the cases, there are also subjects that are arbitrarily diagnosed. In this study, two cases—a patient with bilateral subman- dibular aplasia and a patient with unilateral submandibular aplasia—are reported and discussed based on the literature. 2. First Case A 46-year-old female patient was presented to our clinic with a palpable mass. In the physical examination, her thyroid gland was palpated as bilateral hypertrophic. In the palpation, nodular formations were detected in both lobes. Te thyroid hormone levels were within normal limits. In the ultrasound (US) examination, the right lobe of the thyroid was measured at 13 × 4 × 4 cm and the lef lobe was measured at 9 × 3 × 3 cm. Additionally, the isoechoic nodular structures were observed, the largest of which was on the right with a diameter of 32 mm while the lef had a diameter of 26 × 19 mm with retrosternal extension. In the nodular structures, peripheral and intranodular bleeding was observed. A fne-needle aspi- ration biopsy was performed and the result showed a benign cytology. To determine its relation with the surrounding tissue, a computerized tomography (CT) of the neck was requested. In the CT scan, it was observed that the thyroid gland extended into the submandibular area and the SMG was bilaterally absent (Figures 1 and 2). Tereafer, the patient was asked if she had a dry mouth, difculty swallowing, or dental problems, which may occur due to the absence of salivary glands. Te oral cavity was reevaluated and the Hindawi Publishing Corporation Case Reports in Otolaryngology Volume 2014, Article ID 569026, 5 pages http://dx.doi.org/10.1155/2014/569026