case_images: PMC3716019_01
case_images: Case 1: 15-year-old male child: (a) Intra-oral photograph shows chipping of incisal and cuspal surfaces of incisors best described as "chipping" defects. (b) Panoramic radiograph demonstrates extensive loss of tooth structure. (c) Longitudinal ultrasonogram demonstrates small atrophied echogenic tissue of bilateral parotid glands (white arrow). (d) Longitudinal ultrasonogram reveals small atrophied echogenic tissue of bilateral submandibular glands (white arrow).
case_images: JCIS-3-2-g002.jpg
case_images: PMC3716019_01_JCIS-3-2-g002.jpg
case_images: F1
case_images: These areas do not normally become carious [Figure 1a]. Panoramic radiography revealed extensive loss of teeth structure [Figure 1b]. Further investigations included an ultrasound scan [Figure 1c and d] and contrast enhanced computed tomography in which the submandibular and parotid glands were not visualized [Figure 2a and b].
case_images: Case 1: (a and b) Non-contrast computed tomography axial sections demonstrate bilateral absence of parotid glands (long arrows) and bilateral submandibular glands (Figure a, small arrows). (c) Multiplanar Magnetic Resonance (MR) image, coronal sections demonstrates absence of bilateral submandibular glands (small arrows). (d) Multiplanar MR axial sections shows absence of bilateral parotid glands (small arrows).
case_images: JCIS-3-2-g003.jpg
case_images: PMC3716019_01_JCIS-3-2-g003.jpg
case_images: F2
case_images: Further investigations included an ultrasound scan [Figure 1c and d] and contrast enhanced computed tomography in which the submandibular and parotid glands were not visualized [Figure 2a and b]. Magnetic resonance imaging (MRI) was performed which revealed complete absence of all major salivary glands [Figure 2c and d].
case_images: PMC3716019_02
case_images: Case 2: An 18-year-old female: a) Intra-oral photograph shows extensive chipping of teeth, mainly incisors and canines. (b) Multiplanar MR coronal sections demonstrates agenesis of bilateral submandibular glands (small arrows). (c) Panoramic radiograph reveals multiple missing teeth and extensive destruction of teeth mainly incisors and canines. (d) Multiplanar MR axial sections reveals agenesis of bilateral parotid glands (small arrows).
case_images: JCIS-3-2-g004.jpg
case_images: PMC3716019_02_JCIS-3-2-g004.jpg
case_images: F3
case_images: Intra-oral examination revealed signs of xerostomia, reduced salivary flow, dry erythematous oral mucosa, and extensive chipping of teeth, particularly incisors and canines [Figure 3a]. Panoramic radiography revealed extensive destruction of teeth, particularly incisors and canines [Figure 3c]. MRI scan was performed, which confirmed the agenesis of all the major salivary glands [Figure 3b and d].