Unilateral submandibular gland agenesis associated with capillary hemangioma of the cartilaginous nasal septum

Congenital agenesis of the submandibular gland with compensatory hypertrophy of the contralateral gland is an extremely rare disorder and only few cases are reported in the literature. To our knowledge the reported cases of submandibular agenesis have been asymptomatic and associated with facial anomalies. In our study we present the imaging features of the right submandibular gland agenesis with associated capillary hemangioma of the cartilaginous nasal septum in a middle-aged man who presented with a right-sided nasal block.


Introduction
Congenital absence of submandibular gland is an infrequent disorder and is usually associated with contralateral submandibular gland hypertrophy [1,2]. Major salivary gland aplasia are seen in congenital disorders such as lacrimoauriculodentodigital syndrome, mandibulofascial dysostosis, and ectodermal dysplasia in association with dysplasia or aplasia of lacrimal or thyroid glands [3,4]. To the best of our knowledge, this is the fi rst case of submandibular gland agenesis associated with capillary hemangioma of the cartilaginous nasal septum. In this case report we present the computed tomographic (CT) fi ndings in a 48-year-old man with right submandibular gland agenesis associated with left side capillary hemangioma of the cartilaginous nasal septum.

Case report
A 48-year-old man came with complaints of left-sided nasal block for 1 month, without any history of pain, fever or of salivary hypofunction. On examination the patient had a nasal mass that had minimal bleeding on touch. Th e patient underwent nasal endoscopy, which showed polypoidal mass lesion in the anteroinferior nasal cavity. Th e patient then underwent contrastenhanced CT of the paranasal sinus, which showed absent right submandibular gland with compensatory hypertrophy of the left submandibular gland (Fig. 1a-c). Th ere was also a well-defi ned polypoidal mass lesion in the anteroinferior nasal cavity in close proximity with the cartilaginous nasal septum that showed intense and persistent contrast enhancement suggestive of capillary hemangioma (Fig. 2a-c). Th e patient was counseled and then his consent for the surgery was obtained. Th e polypoidal mass lesion was surgical excised and sent for histopathological analysis. Histopathology detected thin-walled, small-and medium-sized capillaries lined by a single layer of endothelium with intervening fi brous stroma consistent with capillary hemangioma of the nasal septum (Fig. 3), thus confi rming the radiological diagnosis.

Discussion
Th e major salivary glands sequentially develop between 4th and 12th week of the fetal development as solid epithelial buds from the primary oral cavity. Th ese primodal buds grow and extend into the underlying mesenchymal tissue as solid core of cells, which then undergoes extensive branching and eventually develops into lumina. Th e submandibular gland arises at the 6th week of gestation [5,6].
Agenesis of the major salivary gland is a rare disorder. Although the etiology is unknown, it is thought to occur because of the defects that occur during early fetal development [6,7].
Th e clinical symptoms related to salivary gland agenesis are varied. In most of the cases dry mouth, diffi culty in swallowing, and dental problems can be seen. But in our case there were no such symptoms. Absence of salivary gland may also be seen as part of the Unilateral submandibular gland agenesis associated with capillary hemangioma of the cartilaginous nasal septum Janani Moorthy a , Subramanian Ilanchezhian b , Rajoo Ramachandran a , Periasamy V. Prithiviraj a Congenital agenesis of the submandibular gland with compensatory hypertrophy of the contralateral gland is an extremely rare disorder and only few cases are reported in the literature. To our knowledge the reported cases of submandibular agenesis have been asymptomatic and associated with facial anomalies. In our study we present the imaging features of the right lacrimoauriculodentodigital syndrome, characterized by hypoplasia, aplasia, or atresia of the lacrimal system, deafness and ear malformations, and dental and digital anomalies [8]. Th ere were no such anomalies present in our patient. Th e lack of salivary gland related symptoms was probably because of the secretions of the other salivary glands compensating for this.
Salivary gland aplasia can be diagnosed with a variety of imaging techniques that include ultrasonography, CT, MRI sialography or scintigraphy. In our patient we were able to confi rm the diagnosis with the help of a CT scan. Th e characteristic imaging feature in CT is the nonvisualization of the submandibular gland, which is often replaced by fatty tissue, the margins of which are well defi ned with no signs of invasion of the adjacent soft tissue or bone and compensatory hypertrophy of the contralateral submandibular gland, as seen in our case.    Nasal lobular capillary hemangioma is a rare benign tumor of the paranasal sinuses. Th e most common location of lobular capillary hemangioma is the head and neck region. Nasal lobular capillary hemangioma occurs at all ages but is more commonly seen in middle-aged adults, with a slight predilection toward women than men [9,10]. In children nearly 76.9% of the cases occur in the gingiva, lips, and tongue and only few cases are seen in the nasal cavity [11,12].
Nasal lobular capillary hemangiomas usually arises from the nasal septum, predominantly from the little area and/or from the roof of the nasal cavity and turbinates or in the maxillary sinus [13].
Th e exact etiology of lobular capillary hemangioma is still unknown. Recurrent nose picking or nasal packing plays a signifi cant role in the development of lobular capillary hemangioma resulting in excessive growth of the granulation tissue [10,12,14]. It is commonly found in pregnant women and in women on oral contraceptive pills, and its development has a strong association with trauma and hormonal infl uences [14]. Epistaxis and nasal obstruction are the most common symptoms of lobular capillary hemangioma.
On plain CT, nasal lobular capillary hemangioma appear as a well-defi ned soft-tissue mass that shows intense enhancement on post contrast study [15]. Th e diff erential diagnosis of capillary hemangioma is juvenile nasal angiofi broma, which are commonly seen in adolescent men. Other diff erential diagnoses are angiomatous polyp and some uncommon malignant lesions such as nasopharyngeal carcinoma or nasopharyngeal teratoma.
On MRI, these lesions have an intermediate signal on T1-weighted and heterogeneous signal with areas of fl ow void on T2-weighted sequences showing intense enhancement post gadolinium.
Angiography is useful for preoperative embolization as it signifi cantly reduces the occurrence of bleeding during the operation.
Th e management of capillary hemangioma consists of preoperative embolization of the tumor followed by complete surgical resection of the mass as in our case. Nasal lobular capillary hemangioma associated with pregnancy may regress following childbirth. Th e lesions that do not regress completely may require surgery and the patients are generally advised to follow-up.

Conclusion
Unilateral submandibular gland agenesis is an extremely rare disorder and its incidence is unknown.
To the best of our knowledge this is the fi rst case report of submandibular gland agenesis and capillary hemangioma of the nasal septum seen in the same patient. However, no established association has been mentioned so far.

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Con icts of interest
Th ere are no confl icts of interest.