Craniosynostosis
Craniosynostosis involves a premature fusion or closure of the cranial sutures in children. Craniosynostosis can occur along a single suture line or several suture lines. The primary feature of craniosynostosis is that of a misshapen head. When children have craniosynostosis, pressure on the brain can occur and can be harmful to its growth and development.
Craniosynostosis should be repaired before damage to the brain occurs in the young children afflicted with the condition. After an evaluation, a CT scan is performed to prove the exact nature of the craniosynostosis. After the completed evaluation, the doctor will talk to the parents about the sutures involved in craniosynostosis and about the recommended treatment for the craniosynostosis the child has.
Scope of Craniosynostosis
Craniosynostosis can be isolated or associated with other syndromes. Examples of syndromic craniosynostosis include Apert, Crouzon’s, Pfeiffer, Carpenter, Saethre-Chotzen and Keeblattschadel Syndromes. The severity of the craniosynostosis depends on the syndrome and on the child. Our doctors will explain the nature of the craniosynostosis and the methods of treatment for the various types of craniosynostosis.
Should you decide to have your child treated for craniosynostosis, a surgery date will be scheduled. Your child will have a preoperative examination to see if he or she is healthy enough for craniosynostosis surgery. On the day of the craniosynostosis procedure, the doctors will mark areas on the child’s head which are involved in craniosynostosis and then will proceed with surgery.
After the craniosynostosis surgery is over, your child’s head will be bandaged and he or she will be monitored for potential surgery complications. When the child is stable after the craniosynostosis procedure, his or her head should look more normal and the brain and skull should grow in an acceptable fashion.
In rare situations, craniosynostosis surgery may need to be done in stages. The more serious sutures are released at first and the other, less serious sutures will be addressed in surgery later. If the child has more than just craniosynostosis, other facial procedures may also need to be done. Generally, it is less important to do the facial surgeries than it is to perform the craniosynostosis procedures, because of the impact of craniosynostosis on the growth and development of the brain.
Craniosynostosis Surgery
Craniosynostosis surgery is a treatment for a birth defect that affects the brain and skull known as craniosynostosis such as the case with single or multiple suture synostosis. Surgery is performed to correct the misshapen head and face resulting from this birth defect. To understand craniosynostosis surgery, knowledge of the skull’s makeup is necessary. There are five bones in the skull, and between these bones are fibrous joints called sutures. Sutures normally remain open so that the brain has room to expand in all directions until it stops growing. When sutures close too early, the brain compensates by continuing to grow along the path of least resistance, causing the skull to have an abnormal shape, which must be corrected by craniosynostosis surgery. Craniosynostosis surgery relieves pressure on the brain and cranial nerves, since beyond the abnormal proportions and shape of the head, problems with vision, cognition, and breathing may result from the brain compression associated with craniosynostosis. Surgery to correct the condition is usually performed when a child is between three and six months old.
In craniosynostosis surgery, doctors remove the sutures so the brain can grow normally. Sutures may close before birth or at any time before brain growth is completed. Craniosynostosis surgery may be performed on a single suture, which is the usual case, or several sutures may need closing through craniosynostosis surgery.
Multiple Suture Synostosis
There are two categories of craniosynostosis. In single suture craniosynostosis, only one of the seams between the skull bones closes prematurely. With multiple suture synostosis, several of the fibrous joints required treatment. Problems arising in cases of multiple suture synostosis vary. There are over seventy kinds of multiple suture synostosis, and each must be treated according to its unique nature.
The two most common types of multiple suture synostosis are:
- Apert syndrome: A misshapen skull, wide-set eyes, shallow orbits, and an underdeveloped upper jaw characterize this form of multiple suture synostosis.
- Crouzon syndrome: A head that is wider than usual, with a high and prominent forehead and small eye sockets is typical in this type of multiple suture synostosis.
In treating multiple suture synostosis, doctors correct the shape of the head during the child’s first year. They then address other issues associated with multiple suture synostosis, such as breathing problems, during the later childhood years. An additional craniosynostosis surgery may be performed to improve the appearance and to correct other effects of multiple suture synostosis in adolescence.
Craniosynostosis
Craniosynostosis is a birth defect in which the fibrous seams, or sutures, between the five bones of a baby’s skull close up prematurely, compressing the brain and resulting in head and face deformities. The brain of a child grows very fast. It reaches seventy percent of its adult size within one year. The required skull expansion can occur because the sutures let the skull bones push apart. New bone then forms in the seams. Craniosynostosis actually refers to a group of conditions in which there is abnormal head growth because these sutures close too early. These conditions can be corrected through traditional surgical methods or with a relatively new and less invasive technique, endoscopic-guided surgery for craniosynostosis.
In traditional craniosynostosis surgery, surgeons make an incision across the scalp from ear to ear, and then move the scalp to expose the skull bone. Craniosynostosis surgeons open the suture or sutures affected to give the brain room to grow. They also reshape the head to correct the skull and facial deformities that have occurred and replace the excised bone with suitable material.
This traditional type of craniosynostosis surgery lasts between three hours and seven hours, and it always requires a blood transfusion. Craniosynostosis patients undergoing this procedure typically stay in the hospital for three to five days. There is often extensive swelling in the craniosynostosis patient after the traditional surgery, and this causes some discomfort.
Endoscopic-Guided Surgery for Craniosynostosis
Endoscopic-guided surgery for craniosynostosis is much less invasive. The procedure to correct craniosynostosis is performed using an endoscope, a flexible medical device that uses fiber optics to allow craniosynostosis surgeons to see what they are doing inside the body. They perform the craniosynostosis correction procedure by inserting the endoscope through one or two scalp incisions, each of which is about one inch long. The suture or sutures that are causing the craniosynostosis are removed, or opened, and the brain can again grow in a normal fashion.
Endoscopic-guided surgery for craniosynostosis reduces:
- The need for blood transfusions for craniosynostosis patients
- The length of craniosynostosis surgery to about one hour
- The cost of hospitalization for craniosynostosis treatment
Additionally, nearly all craniosynostosis patients can be discharged from the hospital the day after their surgery.
Endoscopic-guided surgery for craniosynostosis is best performed on babies who are under the age of six months. The preferred age of candidates for endoscopic surgery is three months.
Brachycephaly
A brachiocephalic deformity consists of an abnormally broad head with a high forehead. The broad nature of the shape causes the length of the skull to be shortened in the front to back plane. Brachycephaly is associated with the early closure of the coronal sutures and is also present in many syndromal abnormalities, such as Aperts, Crouzons, Pfeiffer, Saethre-Chotzen and Carpenters syndromes.
Kleeblattschadel
Term used to describe a trilobular head or cloverleaf deformity of the skull which occurs when all the sutures are closed. Seen from the front, the skull shape resembles that of a cloverleaf. Surgical procedures by a skilled craniofacial surgeon can give these patients a more normal appearing head.
Oxycephaly
Oxycephaly is a form of craniosynostosis which describes an abnormally high conical head shape due to the fusion of both the coronal and sagittal sutures.
Plagiocephaly
This type of craniosynostosis results in both skull and facial asymmetry and the head shape is often described as “lopsided” in appearance. It is caused in the front by the premature fusion of one of the coronal sutures. At the back of the skull, the occipital asymmetry is produced when the lambdoid suture closes early.
Scaphocephaly
This term describes a boat-shaped head which is associated with the early closure of the fontanelle (soft spot) and sagittal suture. This is the most common form of craniosynostosis. It is usually relatively benign condition in that intracranial pressure is usually in the normal range. Cranial surgery is usually recommended, however, to give a more normal appearance.
Trigonocephaly
Trigonocehpaly is a head deformity which gives the front of the head a wedge-shaped appearance. It consists of a narrow pointed forehead with close-set eyes. The overhead view shows this “wedge-shaped” forehead region and a bony ridge running up from the root of the nose. This deformity is associated with premature fusion of the metopic suture and can be corrected with surgical intervention.
