ABSTRACT
Schwannomas are a type of nerve sheath tumours predominant in the soft tissues of the head and neck. They commonly present as slow growing, painful swellings and may rarely be accompanied by paresthesia. Less than 1% of schwannomas are intraosseous with affliction to the mandible over maxilla. Only 13 cases of maxillary schwannomas have been reported till date. This article documents a rare case of intramaxillary schwannoma that was disclosed during an incidental radiographic examination. It also provides a review of the literature on central schwannomas affecting the maxilla which suggests its affliction to females in the second decade with equal preference to both anterior and posterior segments of the jaw. It also highlights that intraosseous schwannomas may be considered in the differential diagnosis of periapical lesions with nonspecific clinical and radiographic features.
ABSTRACT
Hunter syndrome, or mucopolysaccharidosis type II (MPS II), is a member of a group of inherited metabolic disorders together termed mucopolysaccharidosis (MPSs). It is a rare, X-linked disorder caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase. The prevalence of this syndrome is 1:100,000 births. Insufficient enzyme activity results in accumulation of glycosaminoglycans (GAGS) in the lysosomes of various tissues and organs and leading to progressive multisystem pathologies. Here, we report a case of 13-year-old boy who presented with typical facial, skeletal and dental features without corneal clouding. It is possible that thorough and systematic clinical and radiological examination alone can help in diagnosis of this complex disorder.
