Renaissance Plastic Surgical Arts - Jeffrey W. Colyer, M.D.
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Craniofacial Procedures

Cleft Lip and Palate - Craniosynotosis
Hemangiomas
- Plagiocephally
- Apert Syndrome - Abnormal head Shape

Craniosynostosis

What Is Craniosynostosis?

Craniosynostosis is a congenital anomaly characterized by the premature closure of one or more cranial sutures (the fibrous joints between the bones of the skull). The disorder results in an abnormal skull and head shape. Most cases are sporadic, although there are several genetic syndromes with Craniosynostosis. Disorders of the fibroblast growth factor receptor molecule have been recently found in some cases of hereditary Craniosynostosis.

What Are The Symptoms Of Craniosynostosis?

Most children with Craniosynostosis do not have any symptoms; however, the head shape is abnormal, and the face may be malformed in certain cases. Occasionally, symptoms from the increased pressure in the head such as headaches, decreased appetite, vomiting, or even developmental retardation may occur.

How Does A Craniofacial Anomaly Occur?

Five separate bones make up the cranium of the head: the occipital bone (back portion of the skull); two parietal bones (on either side of the skull); and the two frontal bones (front portion of the skull). The bones, which are in place by the 5th month of gestation, are like pieces of a puzzle, and are initially separated by sutures (fibrous joints). The skull bones coupled with the sutures form a protective shield for the brain. The sutures, which act as expansion joints, allow the skull to mold for birthing and to expand with the rapidly growing brain. At approximately 12 months of age the sutures will fuse together and connect the once free floating bones. As the brain grows, the sutures allow the skull to expand rapidly. If for any reason one or more of the sutures closes too early, the skull grows in a different direction where the bones are not resisting growth, thereby resulting in skull deformities. While many infants are born with an abnormal head shape due to the trip through the narrow birth canal, most will correct themselves within six weeks following birth. When an abnormal head shape persists or is not noticed until 3 to 6 months, it is important to determine the cause. A correct diagnosis is essential and should be made by a qualified specialist.

What Treatments Are Available?

Craniosynostosis surgery is commonly offered to correct the cosmetic deformity of the skull and face. Rarely is surgery necessary to reduce intracranial pressure and allow for brain growth; however, it is sometimes performed when multiple sutures are involved. The timing and type of surgery is very much dependent on the type of synostosis and the preferences of the surgeon. Most often, surgery for craniosynostosis is performed during the first several months of life. Helmets or headband therapy may also be used as an adjunct to surgery for selected cases. Some neurosurgeons use headbands for occipital plagiocephaly. In this type of therapy, the child wears a customized, external helmet or headband for a prescribed period to direct proper growth.

When Is Craniofacial Surgery Necessary?

The surgery is necessary in an infant who has Craniosynostosis when the bones of the skull fuse together, or a craniofacial syndrome, which causes fusion of the bones of the head and also abnormalities of the skull and face.

What Is The Ideal Age For A Child To Have Surgery For Craniosynostosis?

Surgery should be done within the first two weeks of life on those infants born with multiple fused sutures. When the saggital suture is involved, surgery is done at three months of age. For fusion of the remaining sutures, surgery is done at six months.

Will One Operation Be Sufficient For Craniosynostosis?

Most children who have a single suture involvement require only one surgical procedure. However, for those with multiple suture involvement and those with craniofacial abnormalities associated with the Craniosynostosis, multiple procedures are necessary.

What Are The Risks To The Child In This Type Of Surgery?

For healthy children with no other major medical problems, the risks are minimal. The major concern during surgery is blood loss because an infant's blood volume is low and the loss of a small amount may require replacement.

Do Infants Suffer Brain Damage When Nothing Is Done For A Misshapen Head?

In those who have Craniosynostosis or a craniofacial syndrome, there is a small group of 7 to 14 percent, who have increased intracranial pressure which may result in brain damage if not treated.

Do The Deformities Cause Other Problems With Ears Or Eyes?

Children with Craniosynostosis, craniofacial syndromes, and positional deformities may have problems with their eyes and ears. Those with Craniosynostosis and positional deformities have trouble with vision as a result of an imbalance in their ocular muscles. Children with craniofacial syndromes have problems with their ears and eyes and often have a history of ear abnormalities in conjunction with the bulging of the eyes and corneal exposure problems.

Why Are Both A Plastic Surgeon And Neurosurgeon Involved In The Child's Care?

The plastic surgeon reconstructs the facial deformityand the neurosurgeon is primarily involved with the cranial deformity. The combined expertise of the plastic surgeon and neurosurgeon yields optimal results.

If One Child in the Family Has A Deformity Of The Head Will Another Have The Same Problem?

In infants with Craniosynostosis and positional deformities, there is no evidence of genetic transmission. In infants who have abnormal head shapes from craniofacial syndrome, there is a history of genetic transmission.

Is There Any Scarring From The Surgery And Will It Be Apparent Later In Life?

The only visible scar is the scar in the hairline, which is usually covered nicely as the hair grows and the child ages.

What Kind Of Post-Surgical Care Is Needed?

As a parent, you will need to keep your child's suture line clean. This involves removing all crusts with peroxide and antibiotic ointment and shampooing the infant's hair with baby shampoo.

 

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