Open AccessMultiple dentigerous cysts as a rare presentation of maroteaux– Lamy syndrome
Author(s) -
Aasim Farooq Shah,
Tajamul Hakim,
Shahid Farooq,
Mubashir Younis,
Shamina Kosar,
Aijaz Ahmad Malik,
Latief Najar
Publication year - 2017
Publication title -
annals of maxillofacial surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.365
H-Index - 6
ISSN - 2231-0746
DOI - 10.4103/ams.ams_13_17
Subject(s) - medicine , organomegaly , pathology , mucopolysaccharidosis , tongue , presentation (obstetrics) , dermatology , disease , surgery
Maroteaux-Lamy syndrome is one of the genetic disorders involving disturbances in mucopolysaccaride metabolism, due to deficiency of aryl sulfatase-B which leads to accumulation of dermatan sulfate in tissues and their excretion in urine. The diseases has several oral and dental manifestations, is first diagnosed on the basis of clinical findings. It is characterized by coarse facial features, normal intelligence, organomegaly, enlarged head, short neck, corneal clouding, enlarged tongue, and prominent metachromatic inclusions in leukocytes. Death is usually a result of either respiratory tract infection or cardiac disease, which is caused by the deposition of mucopolysaccharides. An 18-year-old with Maroteaux-Lamy syndrome is described in this article with multiple dentigerous cysts as the first presentation.