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SALIVARY GLANDS - APLASIA OCTOBER 1998 Aplasia (agenesis) of the salivary glands is an occasional finding seen in the Salivary Gland Center (SGC). Any one or group of salivary glands may be absent, unilaterally or bilaterally. Aplasia becomes manifest with the development of xerostornia and its sequelae. A diagnosis of salivary gland aplasia is made after exclusion of the common causes of xerostomia - medications, Sjogren's syndrome and radiation. The CT scan or MRI will indicate the gland's absence and its replacement by fat and fibrous tissue. Scintiscanning with a radioisotope (TPT) will confirm the initial diagnosis. The absence of the salivary duct orifice/papilla is an additional clue. Aplasia occurs for unknown reasons as an isolated finding or in conjunction with other developmental defects such as hemifacial microsomia, the LADD syndrome and mandibulo-facial dysostosis (Treacher Collins). In the more severe cases, the ensuing xerostomia causes clinical problems. Salivary loss leads to increased caries, burning sensations, oral infections, taste aberrations and difficulty with denture retention.
LADD is a hereditary autosomal dominant syndrome.The lacrimal (L) apparatus usually demonstrates occlusion of the lacrimal puncta, nasolacrimal duct obstruction with overflow of tears (epiphora), lacrimal sac inflammation (dacrocystitis) and lacrimal gland aplasia. The auricles (A) are deformed with the ear having a cup-shaped appearance. There is some hearing loss. Dentally (D), peg-shaped teeth, hypodontia, and enamel hypoplasia are noted. Various combinations of salivary gland agenesis, with varying degrees of xerostomia, are present. Digital (D) deformities are manifested by deviation of the fingers medially or laterally (clinodactyly). Mandibulo-facial dysostosis is familial in origin. Patients have a symmetric notching of the lower eyelids with the eyes slanting downward at the lateral borders. Maldevelopment of the mandible and maxilla, a defective development of the malar bones, and often salivary aplasia are seen. A bird-like appearance results. Obviously, treatment for salivary gland agenesis is supportive and directed at relieving xerostomia and its effects. salivary substitutes, frequent mouth washes, comprehensive dental care, fluoride therapy, and good oral hygiene all play a role in successful management. The Salivary Gland Center (SGC) was developed because a void existed in the diagnostic and comprehensive care of patients with salivary gland problems and/or secretory dysfunction. Since the diversity of these salivary conditions presents challenges to the clinician, the SGC is available for referrals. Louis Mandel, DDS Director, Salivary Gland Center (212) 305-9982
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Hemifacial
microsomia, a relatively frequent entity (I in 3500 births), is
characterized by an asymmetric mild to severe underdevelopment of
the craniofacial skeleton, the external ear, and facial soft tissues
including the parotid gland. It involves structures derived from
the first and second branchial arches. The majority of cases occur
sporadically but rare familial cases have been reported.