Oral anomalies associated with the oculocerebrorenal syndrome of Lowe: case report with multiple unerupted teeth and pericoronal radiolucencies.
The oculocerebrorenal syndrome of Lowe (OCRL) is a rare X-linked recessive disorder, chiefly characterized by ocular involvement, mental retardation, and kidney disease. A literature review is provided, detailing the diversity of oral anomalies associated with the OCRL syndrome. Reported abnormalities include delayed tooth eruption, odontogenic cyst formation, and constricted dental arches. In addition, we present an unusual case of an 18-year-old male affected with the OCRL syndrome and fetal alcohol syndrome. The oral radiographic examination was significant for multiple impacted permanent teeth, many with pericoronal radiolucencies, and an underdeveloped mandible.
Brooks
John K
JK
Department of Oncology and Diagnostic Sciences, University of Maryland Dental School, Baltimore, Maryland 21201-1586, USA. oralpath5@comcast.net
Ahmad
Rizwan
R
D000293:Adolescent; D003744:Dental Enamel Hypoplasia / Q000209:etiology; D006801:Humans; D008297:Male; D008334:Mandible / Q000473:pathology*; D008844:Micrognathism / Q000209:etiology; D009800:Oculocerebrorenal Syndrome / Q000150:complications*; D009807:Odontogenic Cysts / Q000000981:diagnostic imaging / Q000209:etiology*; D024343:Open Bite / Q000209:etiology*; D011859:Radiography; D014097:Tooth, Unerupted / Q000000981:diagnostic imaging / Q000209:etiology*