| Apert syndrome is also known as Apert-Crouzon syndrome
or Acrocephalic syndactyly.
It is a rare developmental condition characterized
by early synostosis of the sutures, producing alterations
in growth. It
presents with associated brachicephalia and a protuberant
frontal region and ridge which may pass to the root
of the nose, syndactyly
of the hands and feet, malformation of the nasal structures,
megalocornea, divergent strabismus, orbital hypoplasia
and other malformations such as maxillary hypoplasia
with a relative mandibular prognatism, and a high arched
palate which is sometimes cleft.
Dental crowding with delayed eruption is common
in this condition.
The nose is small and has a parrot beak appearance.
It is believed to be transmitted in an autosomal
dominant fashion.
The panoramic radiograph of the patient shows
marked dental crowding
in the maxilla and a supernumerary mesiodens.
The hands and feet have been operated to correct the
syndactylies. |