Biomaterial Database

Orthognathic surgery in osteogenesis imperfecta: a case report with management considerations. 1995

I W Ormiston, and H Tideman

Department of Oral & Maxillofacial Surgery, University of Hong Kong.

Osteogenesis imperfecta is a disease of connective tissues with additional metabolic defects. It is associated with recognisable facial disproportion and sometimes warrants surgical intervention for aesthetic, functional and psychological reasons. A severe case of osteogenesis imperfecta type 3 is presented to illustrate the feasibility of bimaxillary surgery using a mandibular body step osteotomy and maxillary down grafting at the Le Fort I level. The medical, anaesthetic, surgical and specific maxillofacial implications of surgery in these patients are discussed.

UI	MeSH Term	Description	Entries
D007569	Jaw Abnormalities	Congenital absence of or defects in structures of the jaw.	Abnormalities, Jaw,Abnormality, Jaw,Jaw Abnormality
D008334	Mandible	The largest and strongest bone of the FACE constituting the lower jaw. It supports the lower teeth.	Mylohyoid Groove,Mylohyoid Ridge,Groove, Mylohyoid,Grooves, Mylohyoid,Mandibles,Mylohyoid Grooves,Mylohyoid Ridges,Ridge, Mylohyoid,Ridges, Mylohyoid
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
D010013	Osteogenesis Imperfecta	COLLAGEN DISEASES characterized by brittle, osteoporotic, and easily fractured bones. It may also present with blue sclerae, loose joints, and imperfect dentin formation. Most types are autosomal dominant and are associated with mutations in COLLAGEN TYPE I.	Fragilitas Ossium,Lobstein Disease,Brittle Bone Disease,Lobstein's Disease,Osteogenesis Imperfecta Tarda,Osteogenesis Imperfecta with Blue Sclerae,Osteogenesis Imperfecta, Type 1,Osteogenesis Imperfecta, Type I,Disease, Lobstein,Disease, Lobstein's,Lobsteins Disease,Ossiums, Fragilitas,Osteogenesis Imperfecta Tardas
D010027	Osteotomy	The surgical cutting of a bone. (Dorland, 28th ed)	Osteotomies
D002508	Cephalometry	The measurement of the dimensions of the HEAD.	Craniometry
D003811	Dentinogenesis Imperfecta	An autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. The dentin develops poorly with low mineral content while the pulp canal is obliterated.	Hereditary Opalescent Dentin,Capdepont Teeth,Dentinogenesis Imperfecta 1,Dentinogenesis Imperfecta without Osteogenesis Imperfecta,Dentinogenesis Imperfecta, Shields Type 2,Dentinogenesis Imperfecta, Shields Type II,Opalescent Dentin,Opalescent Teeth without Osteogenesis Imperfecta,Dentin, Opalescent,Opalescent Dentin, Hereditary,Teeth, Capdepont
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults