About Binder Syndrome

Binder syndrome is a rare congenital disease affecting the face. Also called nasomaxillary hypoplasia or maxilla-facial dysplasia, the condition results in undergrowth of the central face and may include elements of the nose and Upper Jaw.

The primary physical characteristic of Binder Syndrome is a flat, underdeveloped midface (midfacial hypoplasia) and flattened nose associated with the absence of the anterior nasal spine that supports the nose in normal development. Your child may appear to have an underdeveloped Upper jaw and facial imbalance.

Binder’s Syndrome/Binder Syndrome (Maxillo-Nasal Dysplasia) is a developmental disorder primarily affecting the anterior part of the maxilla and nasal complex (nose and jaw). It is a rare disorder and the causes are unclear.

The characteristics of the syndrome are typically visible. The syndrome involves hypoplasia of variable severity of cartilaginous nasal septum and premaxilla. It includes complete total absence of the anterior nasal spine. There are also associated anomalies of muscle insertions of the upper lip and the nasal floor and of the cervical spine.

Affected individuals typically have an unusually flat, underdeveloped midface (midfacial hypoplasia), with an abnormally short nose and flat nasal bridge. They have an underdeveloped upper jaw, relatively protruding lower jaw with anterior mandibular vertical excess and a Class III skeletal and dental (reverse overjet) profile. They have a small frontal sinus and global facial imbalance.

Treatment is encouraged as early as possible with posteroanterior traction on the maxilla and, at about age 8, reinsertion of the nasolabial muscles onto the anterior border of the cartilaginous system. Many who have a severe case of the disorder undergo plastic surgery or orthodontic treatment for cosmetic reasons.

Discussion

The facial features of maxillonasal dysplasia are well described. The midface appears flattened, the columella is short and there is backward slanting of the upper lip. In most reports on Binder’s syndrome, the local maxillary hypoplasia of the floor and walls of piriform aperture have been corrected with bone grafts.Various methods of correcting the deformity associated with the Binder’s syndrome have been mentioned in the literature, although no rigid protocols for treatment are followed.

The nasal deformity can be corrected with bone grafts, cartilage grafts or the alloplastic materials. Similarly, paranasal onlay grafting or a Le Fort I or II osteotomy has been described for the correction of the midface hypoplasia and malocclusion. Converse used the oral vestibular approach to insert a shell-like segment of iliac bone, and Ragnell applied cancellous bone chips to the anterior surface of the maxilla through a median incision at columellar base.

In our cases, we have used onlay bone grafts as well as alloplastic implants to correct the hypoplasia in patients with class I occlusion. An upper vestibular approach was utilised to place the graft in the paranasal region and columellar incision was used to place nasal dorsal graft. The split calvarial bone graft was harvested in the shape required for the onlay graft. The split calvarial bone graft has the advantage of concealed scar site and has comparatively lesser pain as compared to other bone harvest sites.

Symptoms

Patients with the most severe form of Binder syndrome have a tiny nose and recessed upper jaw, creating an underbite (malocclusion). In milder forms, the position of the upper teeth may be normal and the only difference visible may be the bony deficiency on either side of the nose.

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.

Although researchers have been able to establish characteristic or “core” symptoms, much about Binder type nasomaxillary l dysplasia is not fully understood. Several factors including the small number of identified affected individuals, the lack of large clinical studies, and the possibility of other genes influencing the disorder prevent physicians from developing an accurate picture of associated symptoms and prognosis.

The characteristic finding of the disorder is the abnormal development (dysplasia) of the central or mid portion of the face. The midface appears abnormally flattened. In some patients the frontal sinuses may be underdeveloped or absent. Affected individuals have a short nose and flattened bridge of the nose. The nasal bones may be underdeveloped or abnormally positioned.

The bottom of the sheet of cartilage and bone (nasal septum) that separates the right and left nostrils is known as the columella. The columella is abnormally short and the nostrils have a half-moon or comma-shaped appearance. In cases where the columella is severely short, the nostrils may appear triangular. The upper lips may be slanted backward. Despite the various nasal abnormalities, the sense of smell is unaffected.

Underdevelopment (hypoplasia) upper jaw (maxillary bone) is another key feature of Binder type nasomaxillary dysplasia. The maxillae are the large bones of that form the upper jaw and assist in the formation of the nasal cavities, the bony cavities of the eyes (orbits), and the roof of the mouth (palate). The maxillae also contain the sockets of the upper teeth.

Hypoplasia of the upper jaw may cause the lower jaw (mandible) to appear to protrude or stick out (relative prognathism). However, in some individuals, the mandible may actually be longer than normal (true prognathism). Affected individuals also develop malocclusion, a condition in which the upper teeth are improperly positioned in relation to the lower teeth.

More specifically, affected individuals may be predisposed to a reverse overbite (class III malocclusion), in which the lower jaw is too far forward, the cusps of the lower back teeth are abnormally positioned in front of the corresponding upper teeth, and the lower front teeth (incisors) meet or lie in front of the corresponding upper incisors.

In some cases, additional symptoms and physical findings have been reported in association with this condition. Individuals with Binder type nasomaxillary dysplasia seem to be at an increased risk of various malformations of the spine (vertebrae).

Less often, affected individuals exhibit hearing impairment, incomplete closure of the roof of the mouth (cleft palate), misalignment of the eyes (strabismus), structural malformations of the heart (congenital heart defects), mild intellectual disability, and other features. However, the exact relationship between these findings and Binder type nasomaxillary dysplasia is unknown and they may not represent symptoms of the disorder.

Diagnosis

Binder syndrome is diagnosed based on your child’s appearance. Supplemental tests including X-rays and CT scans can be used to confirm the diagnosis.

A diagnosis of Binder syndrome is based upon identification of characteristic symptoms, a detailed patient history, and a thorough clinical evaluation. Certain specialized tests can be used to confirm the diagnosis.

Clinical Testing and Workup

Specialized imaging techniques may be used to help obtain a diagnosis of Binder syndrome. Such tests include computerized tomography (CT) scanning and magnetic resonance imaging (MRI).

During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues.

Such exams may yield specific findings including underdevelopment or absence of the bony protrusion that projects from the base of the nasal septum to join with the middle part of the upper jaw (anterior nasal spine); thinness of a portion of the upper jaw known as the alveolar bone, which forms the dental arch over the upper incisors; underdevelopment or absence of the frontal sinuses; and/or certain abnormalities detected with cephalometric studies, which are scientific measurements of particular craniofacial dimensions.

Treatment

If the upper jaw is set back and the teeth retropositioned, the typical approach to treatment is to wait until your child’s facial bones have stopped growing, usually around age 15-19, before surgery is performed.

Surgery for a recessed upper jaw usually involves cutting and repositioning the jaw forward, a procedure known as a Le Fort I osteotomy or advancement.

This will be performed by your child’s plastic and reconstructive surgeon. Surgical intervention is usually preceded by a period of orthodontic therapy. In mild cases, surgery to advance the jaw may not be required and your child will be treated by orthodontic therapy alone.

In both cases, bony deficiency along the side of the nose may require the placement of bone grafts or synthetic implants.

If your child’s nose is more mildly affected, he may not require any additional treatment. For others, nose augmentation 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.

For patients who have functional appearance-related concerns at a younger age, temporary artificial implants, usually silicone, may be placed. The implants are replaced with larger implants as your child grows. It is usually best to use cartilage as the definitive correction. Cartilage grafts are better tolerated than artificial implants and have fewer long-term complications.

If your child has functional nasal obstruction due to the small size of the nose, surgery of the septum and turbinate membranes may be required. The turbinate membranes are fleshy membranes on the inside of the nose that warm and humidify air but are not functional if severe obstruction occurs.

Even minor degrees of septal deviation and turbinate membrane enlargement can compromise the nose. In this surgical procedure, your plastic surgeon will go through the inside of the nose to remove or straighten the septum and remove a portion of the turbinate membranes. This type of procedure is generally performed on an outpatient basis, and your child can go home the same day.