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Conditions & Treatments

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 that gives it a wedge shape. This is caused by premature fusion of the metopic suture.

Scaphocephaly

Boat-shaped head. This is one of the more common skull deformities. It is 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 ORBITS

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 downward and ears are often abnormal in shape with impaired hearing 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. Apert syndrome is 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 0 to 14 in a system devised by craniofacial surgery founder Paul Tessier.

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.

Tumors

Recent advances in craniofacial surgery have made possible procedures to remove tumors that only a few years ago were considered inoperable. Among the most dramatic of these is the surgery involving a facial split.

This major breakthrough is an application of craniofacial surgery that has enabled neurosurgeons to reach inoperable or poorly accessible tumors at the base of the skull.

Other techniques that combine the skill of the craniofacial surgeon and neurosurgeon include:

Fibrous dysplasia – A tumor involving the craniofacial area. Usually benign, it occurs when normal bone-forming substance produces a form of fibrous connective tissue. It may involve one or multiple bones. Any bone of the skull may be involved. The dysplasia advances slowly, but may present danger to vital areas such as the optic nerve or middle ear.

Neurofibromas – Abnormal tissue growing from a nerve sheath. When present in the craniofacial region, neurofibromas are most often found in or near the orbits. Skin pigmentation, skeletal deformities and central nervous system involvement may also be present.

Surgery for Facial Fractures

Surgery for Facial Fractures

Advancements in craniofacial surgery and new techniques for viewing facial fractures now permit surgeons to correctly repair such injuries.

The best time to correct a craniofacial fracture is within the first few days of the injury. Old, misaligned fractures also can be improved with the latest craniofacial techniques.