Unilateral Submandibular Gland Aplasia Mimicking Nodal Metastasis.

Aplasia of salivary glands is rare and usually involves several major salivary glands. Isolated unilateral submandibular gland aplasia or aplasia accompanied by hypertrophy of contralateral submandibular gland or sublingual glands is a rare entity. The majority of cases are incidentally detected findings in asymptomatic patients; however, imaging findings may mimic mass-like lesions, posing diagnostic challenges in cancer patients. We presented 2 cases, a case with melanoma and an asymptomatic case, who had asymmetric lesion at left submandibular space, demonstrated to represent left submandibular gland accompanied by right submandibular gland aplasia. We aimed to highlight the imaging features for correct identification of these mass-like lesions as normal contralateral salivary gland accompanied by unilateral submandibular gland aplasia.

Postnatal changes in the development of rat submandibular glands in offspring of diabetic mothers: Biochemical, histological and ultrastructural study.

Development and maturation of submandibular salivary glands are influenced by intrauterine diabetic environment. Several studies investigated the effects of diabetes on the salivary glands. However, the effects of maternal diabetes on the submandibular glands of the offspring was not properly examined. Therefore, the present study was designed to describe the changes in the development of the submandibular glands of the offspring of diabetic mothers. The submandibular glands of the offspring of Streptozotocin (STZ)-induced diabetic female rats were examined at two and four weeks after birth. Detection of mRNA demonstrated that maternal diabetes affects the level of different indicators. The reduction of expression of epidermal growth factor (EGF); a protein mitogen, cytokeratin 5 (CK5); an epithelial cell progenitor, CK7 and aquaporin 5 (AQP5); differentiation markers and B cell lymphoma 2 (Bcl2); an antiapoptotic marker were found. Increase in Bcl2-associated X protein (Bax); an apoptotic marker was detected. These changes indicate their effects on saliva secretion, glands tumorigenesis, growth of normal oral flora and oral microbes, with decreased protein synthesis and production of xerostomia and dental caries. Loss of normal glandular architecture, significant increase in fibrosis, by the detection of collagen fibers, and stagnation of secretory granules were found with atrophic changes in the acinar cells. Marked defect of polysaccharides in the acinar cells, denoting functional changes, was manifested by significant reduction of the intensity of periodic acid-Schiff (PAS) reaction. The positive immunoreactivity of caspase-3, denoting cellular apoptosis, and minimal reaction of alpha-smooth muscle actin (α SMA) and proliferating cell nuclear antigen (PCNA) were evident in the offspring of diabetic mothers. We conclude that maternal diabetes produces degenerative effects in the structure and function of the submandibular salivary glands of the offspring, reflecting possible influences on their secretory activity affecting oral and digestive health.

Maldevelopment of the submandibular gland in a mouse model of apert syndrome.

BACKGROUND: Apert syndrome is characterized by craniosynostosis and bony syndactyly of the hands and feet. The cause of Apert syndrome is a single nucleotide substitution mutation (S252W or P253R) in fibroblast growth factor receptor 2 (FGFR2). Clinical experience suggests increased production of saliva by Apert syndrome patients, but this has not been formally investigated. FGFR2 signaling is known to regulate branching morphogenesis of the submandibular glands (SMGs). With the Apert syndrome mouse model (Ap mouse), we investigated the role of FGFR2 in SMGs and analyzed the SMG pathology of Apert syndrome. RESULTS: Ap mice demonstrated significantly greater SMG and sublingual gland (SMG/SLG complex) mass/body weight and percentage of parenchyma per unit area of the SMG compared with control mice. Furthermore, gene expression of Fgf1, Fgf2, Fgf3, Pdgfra, Pdgfrb, Mmp2, Bmp4, Lama5, Etv5, and Dusp6 was significantly higher in the SMG/SLG complex of Ap mice. FGF3 and BMP4 exhibited altered detection patterns. The numbers of macrophages were significantly greater in SMGs of Ap mice than in controls. Regarding functional evaluations of the salivary glands, no significant differences were observed. CONCLUSIONS: These results suggest that the gain-of-function mutation in FGFR2 in the SMGs of Ap mice enhances branching morphogenesis. Developmental Dynamics 247:1175-1185, 2018. © 2018 Wiley Periodicals, Inc.

Congenital dilatation of the submandibular duct.

Congenital dilatation of the submandibular duct also known as imperforate submandibular duct is a rare condition of unknown etiology, clinically characterized as a well-defined, fluctuant swelling in the floor of the mouth, and microscopically defined as a cystic cavity covered with pseudostratified columnar epithelium consistent with a dilated salivary duct. A 1-month-old female presented with a translucent, smooth cystic swelling in the floor of the mouth causing breastfeeding difficulty. The lesion was surgically excised and microscopically, a cystic cavity lined with pseudostratified columnar epithelium was observed. The final diagnosis was congenital dilatation of the submandibular duct. Dentists and otolaryngologists should consider congenital dilatation of the submandibular duct when evaluating fluctuant swelling in the floor of the month of infants.

Lingual Nerve Entrapment in Fused Submandibular and Sublingual Salivary Glands: A Unique Finding.

Lingual nerve (LN) is one among the 3 branches of posterior division of mandibular nerve. It might get entrapped in the infratemporal fossa by lateral pterygoid muscle, pterygospinous ligament, or lateral pterygoid plate. Its entrapment in the submandibular region has not been reported yet. A unique entrapment of LN in the fused submandibular and sublingual salivary glands in a cadaver was reported. The deep parts of the submandibular and sublingual salivary glands were completely fused with each other. The LN passed through the center of the fused part. Histologically both submandibular and sublingual salivary glands had both mucous and serous acini. Though this entrapment might not cause any functional problems, it might get injured during various surgical procedures of the submandibular region.

Accessory submandibular duct sialolithiasis identified during sialendoscopy.

We describe a unique case of a 62-year-old patient with recurrent right submandibular sialadenitis. He initially appeared to have extensive sialolithiasis of the right submandibular duct on computed tomography imaging and examination, but sialendoscopy demonstrated a normal-appearing right submandibular duct. An accessory duct posterior and parallel to the normal duct was identified at the time of sialendoscopy and was found to have extensive sialolithiasis, which required removal by both an endoscopic and intraoral technique. To the best of our knowledge, this is the first report of sialolithiasis of an accessory submandibular duct identified at the time of sialendoscopy; the other few reported cases in the literature are based primarily on findings from traditional sialography or magnetic resonance sialography. Identification of accessory salivary ducts requires intraoperative consideration of this entity during sialendoscopy. Open approaches to sialolithiasis, however, may be aided by appropriate preoperative imaging.

Accessory Submandibular Salivary Gland Forming a "Horseshoe" With the Main Submandibular Salivary Gland: A Unique Variation.

Presence of accessory submandibular salivary gland (ASSG) is an extremely rare variation. Knowledge of its relations could be very useful to oral and maxillofacial surgeons, head and neck surgeons, and radiologists. During dissection classes, an ASSG was noted between the mylohyoid and hyoglossus muscles. The main submandibular salivary gland had superficial and deep parts. The deep part was narrow and measured about 5 cm. The lingual nerve passed between the superficial and deep parts. The accessory submandibular gland was situated below and parallel to the deep part of SSG. It also measured 5 cm. The ASSG had its own duct, which joined the duct of main gland. The ASSG and the deep part of the SSG were united at the lateral border of geniohyoid muscle to give a characteristic "horseshoe" appearance. The ASSG overlapped both lingual and hypoglossal nerves.

Chronic sialadenitis due to the stone inside the accessory duct of submandibular gland.

PURPOSE: Sialolithiasis is the most common cause of chronic sialadenitis. In this case report, intraoperative finding of an accessory submandibular duct, obstructed with stone, originating from the same gland nearby the main Warthon's duct, is presented. CASE REPORT: A 22-year-old male patient, suffering from eating-related pain and swelling in his left submandibular region, was diagnosed with left sublandibular gland sialadenitis with radiologically manifested sialolithiasis, and gland excision was advised. Surgery was performed under general anesthesia. When the full anatomical scenery was delineated before excision of the gland, we surprisingly encountered two submandibular ducts originating from ipsilateral gland, one of them was obstructed with stone. After two ducts were ligated, the gland with sialolith was excised. According to histopathologic examination, the duct obstructed with stone was identified as the accessory duct and the other one was the main Wharton's duct. Postoperative days were uneventful; no neurologic complication was observed. CONCLUSIONS: Otolaryngologists should be aware of anatomic variations of the submandibular duct(s) to avoid possible complications, especially intraoperatively, because rutine preoperative radiologic preparation does not include investigation of possible accessory ducts.

Are the patients with anatomic variation of the sublingual/Wharton's duct system predisposed to ranula formation?

OBJECTIVE: To evaluate ranula development according to anatomic variation of the ductal system of sublingual gland (SLG), especially the presence of Bartholin's duct. METHODS: The anatomic variation of SLG duct was prospectively investigated and compared between 55 consecutive patients with ranulas treated by SLG excision (group 1) and another 15 consecutive patients undergoing similar surgeries for other conditions (group 2). The ductal structures of SLGs and submandibular glands (SMG) were also compared between the pediatric patients and adult patients with ranulas. RESULTS: In 32 of 55 patients with ranulas (58.2%) and 1 of 15 patients without ranulas (6.7%), the SLG showed an anatomic variation of the main duct called Bartholin's duct structure (P<0.01). Seventeen of 22 (77.3%) pediatric patients with ranulas had Bartholin's ducts and 15 of 33 (45.5%) adult patients with ranulas had Bartholin's ducts (0.01

Large tonsillolith associated with the accessory duct of the ipsilateral submandibular gland: support for saliva stasis hypothesis.

Tonsillolith is a calcified mass in the tonsil and/or its surrounding tissue, which is considered to be caused by chronic tonsillitis. However, here we hypothesized that a tonsillolith can also be formed by chronic saliva stasis in the tonsillar tissue, without any signs of chronic inflammation. We present the case of a 32-year-old male patient with a large tonsillolith. We reviewed his medical files, pre-operative imaging and intraoperative findings. During a standard tonsillectomy, we encountered a large tonsillolith measuring 3.1 × 2.6 cm. Additionally, a careful dissection of the lower pole of the remaining tonsillar tissue revealed a large fistulous tract filled with saliva. Post-operative examination of the pre-operative CT scan found a hypodense fistulous tract extending from the lower tonsillar pole towards the left submandibular gland, measuring 36 mm in length, which was diagnosed as an accessory duct of the submandibular gland. To our knowledge, this is the first case of a large tonsillolith associated with the accessory duct of the ipsilateral major salivary gland. Furthermore, from the aetiopathological view, this finding supports the saliva stasis hypothesis for formation of the tonsillolith. However, larger studies, including a detailed radiological analysis as in our case, are needed to further investigate this possible aetiology of tonsilloliths.

A case of imperforate Wharton duct.

Congenital oral masses are rare entities. The establishment of formal fetal diagnostic teams has led to an increased antenatal detection of such lesions. The congenital ranula is a distinct entity from the more familiar variant presenting later in life. The congenital variant may result from an anomaly of the Wharton duct with subsequent dilation of the duct. The variant presenting later in life is the more familiar mucous extravasation phenomenon in the floor of the mouth. Management of the congenital ranula is distinct from its noncongenital counterpart and more conservative and is discussed in the present report.

Dynamic sonography and CT findings of unilateral submandibular gland agenesis associated with herniated hypertrophic sublingual gland.

Congenital agenesis of the submandibular gland is uncommon. We report dynamic sonography and CT findings of the unilateral submandibular gland agenesis associated with herniated hypertrophic sublingual gland tissue through the mylohyoid gap. The dynamic sonography examination applied at rest and during the modified Valsalva maneuver demonstrated hypertrophied sublingual gland prolapsing to the anterior part through the mylohyoid gap. This may be the first reported case describing dynamic sonography findings of this entity. CT confirmed the agenesis of the left submandibular gland and compensatory hypertrophy of the ipsilateral sublingual gland.

The Hippo signaling pathway is required for salivary gland development and its dysregulation is associated with Sjogren's syndrome.

Sjogren's syndrome (SS) is a complex autoimmune disease that primarily affects salivary and lacrimal glands and is associated with high morbidity. Although the prevailing dogma is that immune system pathology drives SS, increasing evidence points to structural defects, including defective E-cadherin adhesion, to be involved in its etiology. We have shown that E-cadherin has pivotal roles in the development of the mouse salivary submandibular gland (SMG) by organizing apical-basal polarity in acinar and ductal progenitors and by signaling survival for differentiating duct cells. Recently, E-cadherin junctions have been shown to interact with effectors of the Hippo signaling pathway, a core pathway regulating the organ size, cell proliferation, and differentiation. We now show that Hippo signaling is required for SMG-branching morphogenesis and is involved in the pathophysiology of SS. During SMG development, a Hippo pathway effector, TAZ, becomes increasingly phosphorylated and associated with E-cadherin and α-catenin, consistent with the activation of Hippo signaling. Inhibition of Lats2, an upstream kinase that promotes TAZ phosphorylation, results in dysmorphogenesis of the SMG and impaired duct formation. SMGs from non-obese diabetic mice, a mouse model for SS, phenocopy the Lats2-inhibited SMGs and exhibit a reduction in E-cadherin junctional components, including TAZ. Importantly, labial specimens from human SS patients display mislocalization of TAZ from junctional regions to the nucleus, coincident with accumulation of extracellular matrix components, fibronectin and connective tissue growth factor, known downstream targets of TAZ. Our studies show that Hippo signaling has a crucial role in SMG-branching morphogenesis and provide evidence that defects in this pathway are associated with SS in humans.

Complete agenesis of major salivary glands.

A 4 year-old female patient was treated for persistent right-sided dacryocystitis and xerostomia. MRI was performed to screen for a dry syndrome; which resulted in the diagnosis of agenesis of the parotid and submandibular glands as well as lacrimal duct malformation. An MRI of each parent was normal. The mother's history revealed 4 days of pyrexia during the 8th week of amenorrhea. This was an isolated case, with no family history, characterized by a febrile episode during pregnancy at the period of main salivary gland genesis. Epigenetic mechanisms could be implicated.