Retreatment of a patient with Marfan syndrome and severe root resorption

This case report describes the retreatment of a patient with Marfan syndrome whose earlier orthodontic and surgical treatment had been unsuccessful. Marfan syndrome is an inherited connective tissue disorder transmitted as an autosomal dominant trait. The disorder results from molecular defects in the fibrillin gene that are responsible for the impaired structural integrity of the skeletal, ocular, and cardiovascular systems. When she sought retreatment, the patient had an open bite, mandibular anterior crowding, severe root resorption, and temporomandibular joint derangement with some resorption of the condyles. The second treatment, which included extractions and surgery, resulted in balanced and harmonious facial proportions, and a Class I occlusion with normal overjet and overbite. There was no further loss of condylar tissue, and the temporomandibular joints were asymptomatic. More root resorption on the mandibular left canine and the left second premolar was evident after the second treatment.

A young woman needed orthodontic retreatment for an open bite, crowded mandibular incisors, and temporomandibular joint (TMJ) derangement with some flattening (resorption) of the condyles. She had a history of previous orthodontic and orthognathic surgical treatment. She has Marfan syndrome and extensive root resorption. Did the earlier treatment cause the flattening of the condyles and root resorption or was there a genetic predisposition, or both? Why did the first treatment fail? Was retreatment worth the risk?

History and etiology

The patient was a white woman, aged 28 years 5 months, with a history of orthodontic and orthognathic surgical treatment that began at age 13 and lasted for 5 years, culminating with orthognathic surgery at age 18. Her medical history confirmed that she had Marfan syndrome, a genetic disorder. She had arachnodactyly with positive wrist (Walker) and thumb (Steinberg) signs ( Fig 1 ). She was taking a beta-adrenergic blocker to control blood pressure in hopes of preventing aortic dissection, because she had evidence of aortic dilatation and mitral valve prolapse. This disorder weakens the connective tissue of the aorta as it enters the heart. She had dural ectasis, hypermobility of her joints, osteoarthritis of her knees, and scoliosis. She had a Class II malocclusion complicated by a 5-mm open bite, 6 mm of mandibular anterior crowding, and severe root resorption. She had a long lower anterior facial height. Her chief concerns were her “crooked teeth, open bite, and facial appearance.” The discomfort she was experiencing in her TMJs had been somewhat relieved with splint therapy.

Apr 14, 2017 | Posted by in Orthodontics | Comments Off on Retreatment of a patient with Marfan syndrome and severe root resorption
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