Binder Syndrome is a rare congenital disease affecting the face that results in undergrowth of the central face that may include elements of the nose and upper jaw.
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SYMPTOMS |
The primary physical characteristic of Binder Syndrome is a flat, underdeveloped midface (midfacial hypoplasia) and flattened nose. The nasal deformity is caused by an absence of the anterior nasal spine that supports the nose and specifically the nasal tip in normal development. The nasal deformity is characterized by a shortened columella and underdeveloped nasal bridge. The nostrils in children with Binder syndrome are characteristically comma-shaped and the bony tissue at the base of the columella (the anterior nasal spine) is absent. If the upper jaw is set back and the teeth retropositioned, the typical treatment is to wait until your child’s facial bones have stopped growing, usually around age 15-19, before surgery is performed.
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TREATMENT & MANAGEMENT |
Surgery for a recessed upper jaw usually involves cutting and sliding the upper jaw forward, a procedure known as a Le Fort I osteotomy or advancement. This is typically performed by a plastic and reconstructive surgeon. Surgery usually follows a period of orthodontic therapy to better align the teeth. In mild cases, surgery to advance the jaw may not be required and your child will be treated by orthodontic therapy alone.
Surgery for the nose depends on how severely it is affected. For mild defects surgery may not be needed. For more severe defects, nose reshaping using cartilage grafts taken from the ribs can add to both the bridge and columella to reshape the nose. This procedure is usually done after your child has reached skeletal maturity to reduce the risk that he will outgrow these grafts.
Once jaw and nasal reconstruction are performed in adolescence, few if any additional procedures will be required. Most patients will experience long-term improved nasal breathing and appearance, and a functionally normal upper jaw and bite.