Tricho-dento-osseous syndrome

Tricho-dento-osseous syndrome, often shortened to TDO, is a rare inherited disorder that affects the hair, the teeth and the bones. It follows an autosomal dominant pattern, is present from birth, and is caused by a mutation in the DLX3 gene, which helps control how hair follicles differentiate and how bone forms. Everyone with the syndrome has enamel hypoplasia and taurodontism, so the teeth are discolored, thin, pitted or fragile and may be malformed inside and out. Hair is rough and sheds heavily, often curly in infancy and straighter later. Some people have an underbite, a prominent lower jaw and longer bones. Cases have clustered in families and in nearby areas of Virginia, Tennessee and North Carolina. Care is team based, drawing on dentists, physical therapists and oral and maxillofacial surgeons, with genetic counseling and emotional support recommended. This record is a reference summary of what is documented, not medical advice.