Biomaterial Database

Axenfeld-Rieger syndrome (ARS): A review and case report. 2010

Jennie M Waldron, and Clare McNamara, and Antonia R Hewson, and C M McNamara

Dental Department, Community Services, Mayo, Ireland.

Axenfeld-Rieger syndrome (ARS) is a rare, autosomal dominant condition characterized by ocular, craniofacial, dental, and periumbilical abnormalities. Relatively little information exists on this syndrome within the dental literature despite the fact that midface hypoplasia and maxillary hypodontia are classical presenting features of this syndrome. This is a case report of a 7-year-old Caucasian female with ARS who presented with significant ocular and dental anomalies. She was also found to have osteopenia. Her dental condition is described, her immediate treatment is shown, and her long-term treatment needs are discussed.

UI	MeSH Term	Description	Entries
D007180	Incisor	Any of the eight frontal teeth (four maxillary and four mandibular) having a sharp incisal edge for cutting food and a single root, which occurs in man both as a deciduous and a permanent tooth. (Jablonski, Dictionary of Dentistry, 1992, p820)	Incisors
D008437	Maxilla	One of a pair of irregularly shaped bones that form the upper jaw. A maxillary bone provides tooth sockets for the superior teeth, forms part of the ORBIT, and contains the MAXILLARY SINUS.	Maxillae,Maxillary Bone,Bone, Maxillary,Bones, Maxillary,Maxillary Bones,Maxillas
D009295	Nasal Bone	Either one of the two small elongated rectangular bones that together form the bridge of the nose.	Nasal Bones,Bone, Nasal,Bones, Nasal
D009910	Oral Hygiene	The practice of personal hygiene of the mouth. It includes the maintenance of oral cleanliness, tissue tone, and general preservation of oral health.	Dental Hygiene,Hygiene, Dental,Hygiene, Oral
D010347	Patient Care Planning	Usually a written medical and nursing care program designed for a particular patient.	Nursing Care Plans,Goals of Care,Plans, Nursing Care,Care Goal,Care Goals,Care Plan, Nursing,Care Planning, Patient,Care Plans, Nursing,Nursing Care Plan,Plan, Nursing Care,Planning, Patient Care
D010895	Pit and Fissure Sealants	Agents used to occlude dental enamel pits and fissures in the prevention of dental caries.	Dental Sealant,Dental Sealants,Fissure Sealant,Fissure Sealants,Pit And Fissure Sealant,Pit Fissure Sealant,Sealants, Tooth,Pit Fissure Sealants,Sealants, Dental,Fissure Sealant, Pit,Fissure Sealants, Pit,Sealant, Dental,Sealant, Fissure,Sealant, Pit Fissure,Sealant, Tooth,Sealants, Fissure,Sealants, Pit Fissure,Tooth Sealant,Tooth Sealants
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003481	Cuspid	The third tooth to the left and to the right of the midline of either jaw, situated between the second INCISOR and the premolar teeth (BICUSPID). (Jablonski, Dictionary of Dentistry, 1992, p817)	Canine Tooth,Canine Teeth,Cuspids,Teeth, Canine,Tooth, Canine
D003832	Denture, Partial, Removable	A partial denture designed and constructed to be removed readily from the mouth.	Removable Partial Denture,Denture, Removable Partial,Dentures, Removable Partial,Partial Denture, Removable,Partial Dentures, Removable,Removable Partial Dentures
D005124	Eye Abnormalities	Congenital absence of or defects in structures of the eye; may also be hereditary.	Abnormalities, Eye,Abnormality, Eye,Eye Abnormality