Binder syndrome



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BINDER SYNDROME


  • Described by Von Binder in 1962 (although earlier descriptions exist).

  • Nasomaxillary dysplasia


Incidence

  • M=F

  • sporadic developmental deformity, although familial recurrence has been described (autosomal recessive)




  • Aetiology unknown. Two major theories:

  1. Inhibition of the ossification centre that would normally have formed the lateral and inferior borders of the pyriform aperture during the 5th and 6th gestational week (Holström)

  2. A form of arhinencephalic malformation (Binder)




  • Result of hypoplasia (depression) of the anterior nasal floor (fossa prenasalis) and localized symmetric maxillary hypoplasia of the alar rim regions.


FEATURES

  1. midface hypoplasia, flattened nose, convex upper lip, broad philtrum, crescent-shaped nostrils, and a deep fold or fossa between nose and upper lip.

  2. Short small nose with a flat bridge and a short columella

  • most striking characteristics of the nose are vertical shortening, lack of tip projection, perialar flattening, half-moon shaped nostrils and an acute nasolabial angle.

  1. Maxillary length short in the AP direction with a degree of maxillary retrusion - para-alar flatness

  2. Absent / recessed anterior nasal spine

  3. Hypoplastic posterior nasal spine

  4. Absent frontonasal angle (angle of almost 180°)

  5. Hypoplasia of the nasal sill

  6. Scaphoid depression of the anterior nasal floor

  7. atrophy/limited nasal mucosa

  • absence of the frontal sinus is seen in some (not obligatory)

  • Occlusion usually near normal or class III (this varies from 15% in Ortiz-Monasterio’s series of 27 patients to 54% in Holmstrom’s series), but usually within the scope of orthodontic treatment

  • The children grow at a normal rate.

  • The deformity is maintained and does not worsen with growth.







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