The professional staff of the Dallas Craniofacial Center has prepared the following information for people investigating the treatment of major craniofacial birth defects. It is intended to help you better understand these defects, their cause and their treatment. Illustrations have been simplified to represent a generalization of these deformities and surgical procedures.

Birth defects occur in both sexes and in all ethnic groups. Generally, they have a wide range of variability between patients. Therefore, it is important to consider this information as a generalization. It should not necessarily be considered valid for diagnosis or treatment of any specific patient. Only competently trained medical professionals can appropriately diagnose and treat someone with these craniofacial birth defects.

Abnormal Skull Shapes

Plagiocephaly
Skull and facial asymmetry. At the front, asymmetry is caused by the premature fusion of the coronal suture on one side of the skull. At the back of the skull, occipital asymmetry is caused by a prematurely fused lambdoid suture.

Trigonocephaly
Wedge-shaped head. A bony ridge at the front of the head gives it a wedge shape. This is caused by premature fusion of the metopic suture.

Scaphocephaly
Boat-shaped head. One of the more common skull deformities caused by the early closure of the fontanelle and premature fusion of the sagittal suture.

Brachycephaly 

Wide-shaped head. This skull deformity results when both coronal sutures fuse prematurely.

Malposition of the Orbit
The orbit is the bony area surrounding the eyeball. The eye grows for the first two years of life, causing the orbit to expand. Abnormal growth of the skull can also cause displacement of the orbit.

Hypertelorism
Increased distance between the eyes. This deformity may result from an encephalocele, Apert Syndrome or other craniofacial problem. When the eyes are positioned too close together, it is known as hypotelorism.

Orbital Encephalocele
When brain tissue herniates or pushes through a skull defect at the orbits. This can be caused by abnormal skull growth.

Asymmetry of Orbits
Unequal height. This can be due to craniofacial microsomia or unilateral plagiocephaly.

Facial Asymmetry
Facial asymmetry, a noticeable difference in features from one side of the face to the other, is one of the more common craniofacial birth defects. The unilateral form of hemifacial microsomia, a deformity of the ear and lower jaw, is the second most common craniofacial birth defect after cleft lip and palate. Different facial structures may be involved from patient to patient. Seen less commonly is hemifacial atrophy, where the soft tissues on one side of the face slowly waste away. Hemifacial hypertrophy is characterized by an enlargement of one side of the face. Other parts of the body on the affected side may also be involved.

Treacher Collins Syndrome

The skull is generally normal, but the ridges over the eyes may be underdeveloped. The cheekbones are underdeveloped or missing completely. Eyelids slope downwards, ears are often abnormal in shape and impaired hearing is usually present. The lower jaw is small and angles down, contributing to an open bite.

Combined Deformities of the Skull and Face



Apert Syndrome 

A rare craniofacial development deformity characterized by a number of deformities, including an abnormally shaped head, small upper jaw, hypertelorism and a normal lower jaw. The fingers and toes are fused together.

Crouzon Syndrome
A common craniofacial syndrome characterized by a combination of skull and facial deformity with hypertelorism and protruding eyeballs. Premature fusion of several sutures causes a short, wide head. The upper jaw is underdeveloped.

Clefts

Lip and Palate


A cleft of the lip and palate can be complete or incomplete. In an isolated cleft palate, which involves only the palate, little aesthetic deformity may be present. However, functional problems can be severe, especially speech difficulties. In Pierre Robin Syndrome, a cleft palate and small lower jaw combine to cause backward displacement of the tongue, which may affect breathing or eating in the first few months of life. Surgery to repair a cleft lip can take place at around three months. Cleft palate repair can be scheduled when the infant is about a year old.

Rare Facial Clefts
In rare facial clefts, there is extensive clefting of the face and skull. This condition is classified on a scale from zero to 14 in a system devised by craniofacial surgery founder Paul Tessier.

Craniofacial Deformities

Dental Malocclusion 

When the teeth do not meet or align properly, it is known as malocclusion. This deformity can seriously affect eating, oral hygiene and speech.

Binder Syndrome
Flat midface and small, flat-tipped nose. The upper jawbone is set back, frequently resulting in a bite that positions the upper teeth behind the lower.

Small Lower Jaw
The most common jaw deformity, which results in a severe overbite.

Long Face

This deformity is characterized by a long face with an excessive amount of upper teeth and gums showing. This may result in a severe overbite.