The history of the main compliant was as follows: the patient reported getting into a physical altercation over a girlfriend, when he was then hit in the face; this resulted in his 11 being fractured as well as a significant laceration to his upper lip. (A scar and haematoma were both still present at the initial visit; however the patient refused any further investigations pertaining to his lip.)
Osteogenesis imperfecta is a genetic bone disorder, where people have either defective connective tissue or lack the ability to make it. Recent works suggest that Osteogenesis imperfecta must be understood as a multi-scale phenomenon, which involves understanding the mechanisms at the genetic, nano, micro and macro level of tissues.
Osteogenesis imperfecta is an autosomal dominant defect. Most people with Osteogenesis imperfecta inherit it from a parent but it can be a sporadic mutation too. In this case, he appears to have inherited it from a parent, his mother and sisters are both more severely affected, as well as having dentinogenesis imperfecta.
About half of patients that suffer from Osteogenesis Imperfecta (OI) have dentinogenesis imperfecta most commonly identified by blue or brown teeth that are either opaque or milky in appearance. (O'Connel, 2003) Osteogenesis Imperfecta is a genetic autosomal dominant disorder affecting bone. The patients are usually born with a deficiency of Type 1 collagen, and are therefore unable to make connective tissue. (Cawson, 2002)
The fact that these patients are deficient in Type 1 collagen occurs because of an amino acid substitution of glycine to amino acids in the collagen triple helix structure. This creates a steric obstruction which in turn creates a swelling in the collagen complex which influences both the molecular nanomechanics and the interaction between molecules. They are both compromised, and this is shown by hydrolyzing the improper collagen structure and if the body does not destroy this, then bones become brittle. (Carranza, 2002)
The latest time that a dentist should see a child with confirmed Osteogenesis imperfecta is six months after eruption of the first primary tooth, as their teeth require special care and attention. (Thompson, 2006) The main reasons for the early care of primary teeth is that they are important for many essential functions including chewing, speaking, holding space for the permanent teeth, and growth of the mandible and maxilla. (O'Connel, 2003)
Parents should regularly brush and floss the teeth of young children, they should then teach the children to do it themselves as well as continue checking they use the correct techniques as they grow older. (Thompson, 2006) The dentist seeing to these young children should initially show the parents how to correctly brush the child's teeth, and then when the child is old enough reinforce oral hygiene instructions to them. (Carranza, 2002)
Starting when the child is 7 years old, it is recommended that an orthodontist should check their bite for evidence of an open bite or Class III malocclusion, as these malocclusions are common in children with osteogenesis imperfecta. (Thompson, 2006) At this time early orthodontic interventions in children who are developing a relatively small maxilla compared to the mandible may help decrease the need for orthognathic surgery or expensive fixed appliances later on. (Bell, 2000)
When treating a patient with osteogenesis imperfecta, the treating dentist needs to give special considerations as to whether or not the impacted teeth should be left alone or extracted, or if an attempt should be made to move them into a more functional position in the mouth. (O'Connel, 2003) To move a tooth, there needs to be communication between the oral surgeon and the orthodontist to surgically uncover the impacted tooth and glue an attachment onto the tooth so that light force from the braces can be used to bring the tooth into the proper position. (O'Connel, 2003) The orthodontist may also use braces prior to surgery to be sure there is space to bring the impacted tooth into the proper position. (Lee, 2003) (Bell, 2000)
The class of drugs known as bisphosphonates is being used as a treatment for many bone diseases. There have been reports in medical journals suggesting a link between bisphosphonates and areas of osteonecrosis. (Lee, 2003) Good oral hygiene along with regular dental care to prevent infections or periodontal disease lowers risk. When possible, required dental surgery should be scheduled prior to starting bisphosphonate treatment. (O'Connel, 2003) Bisphosphonate treatment should not be restarted until after the surgical area is healed. Elective jaw surgery, including dental implants, should be avoided during intravenous bisphosphonate therapy. Extraction of third molars should be postponed until more information is available. (Lee, 2003)
