At Plastic and Craniofacial Surgery for Infants and Children, we offer advanced treatment options for common craniofacial disorders. If you are the parent of a child with one of these challenging conditions, request a consultation online or call us at (469) 375-3838 to schedule a time to meet with our skilled team. You can also review online resources about:
- Distraction Osteogenesis
- Apert Syndrome and Crouzon Syndrome
- Craniosynostosis Surgery
- Deformational Plagiocephaly
The purpose of the distraction device to treat children with certain craniofacial conditions affecting the growth of the jaw is to gradually lengthen the space between the two cut ends so that new bone is formed to fill in the gap. During the surgical procedure, a cut is made in the jawbone. A small incision is made in the skin for pin placement, and the distraction or expansion rod is connected to the pins on the outside of the child’s jaw.
The area is allowed to heal for about one week before “turning,” or lengthening, is begun. The turning is done by the surgeon for the first time, and parents are then taught the technique so that they may continue expansion at home. Pin turning lasts for about 3 to 4 weeks, or until the desired length is achieved. The appliance then remains in place for an additional 6 to 8 weeks to ensure that the gap is filled-in with stable new bone. It is removed in the operating room under sedation.
Parents report that their children tolerate the pin turning without pain. Children may go to school and participate in their regular activities; however they may not swim or play contact sports. They should also be kept on a soft, mushy diet during this time.
Advantages of Procedure
In the past, surgery to lengthen the jaw involved a longer procedure that required bone grafts from the rib or hip. This distraction procedure is simpler, does not require a blood transfusion, and obtains very good results. There is also less chance of relapse. There is a small scar on the jawline, and the child may receive some curious looks or questions while wearing the device, however these drawbacks are minor compared with the long-term benefits of this procedure.
Crouzon and Apert Syndrome
As with any craniofacial disorder, treatment by a multidisciplinary team working together with the family provides the best results. In the newborn period some potential problems that may need to be addressed include respiratory difficulties, feeding problems, neurologic complications such as hydrocephalus, and the potential risk of developmental delay.
The infant’s shallow midface and/or small or partially obstructed nasal passages can cause respiratory problems and also contribute to feeding difficulty. Evaluations by the ENT specialists are important and a tracheostomy may be necessary to relieve the airway problems.
In Crouzon and Apert syndromes synostosis of 2 or more cranial sutures may be involved; therefore there can be a risk for increased intracranial pressure. There is also a greater chance of hydrocephalus in these infants than those with single suture fusion and evaluation and close monitoring by the team neurosurgeon is important.
The reported incidence of mental retardation in patients with Crouzon syndrome is between 0% and 20% while a higher incidence (approximately 20 to 30%) has been reported with Apert syndrome. Referral to an infant stimulation program may be helpful.
After the newborn period, the multidisciplinary team continues to evaluate the child’s needs. Audiology (hearing) and speech evaluations are important to ensure good speech and language development. Assessment by the opthalmologist is important, especially if the eyelids are not protecting the eyes completely or if there are eye muscle problems.
Orthodontic evaluation should begin at age 3 to 4 in these children. The upper jaw is usually set back and small in all dimensions. This results in severe crowding of the permanent teeth, as well as a significant “underbite,” a condition where the top teeth meet inside of the bottom teeth. The palate is constricted and high.
Delayed dental eruption is common, and extra or missing teeth have been noted on occasion. Treatment requires extraction of some permanent teeth to alleviate the crowding, as well as expansion of the maxilla, either surgically or through the use of orthodontic devices.
Treatment in infancy is directed at correction of the suture fusion and resultant misshapen head.
Surgical treatment of the midface deformity is usually done during the preschool period (age 4 to 6 years).
Conventional surgical advancement of the midface requires numerous cuts of the facial bones and advancing the midface region to a predetermined level. This usually requires bone grafts. Plates and screws are used to stabilize the new position.
The past several years have seen a significant increase in treatment using a technique called osteogenic distraction. The same surgical boney cuts are performed and an expansion device is inserted, where by gradual advancement of the midface region can be obtained. Research has indicated this may provide a more stable correction.
Treatment is aimed at restoring the normal shape and relationship of the forehead and orbital rims. While the deformity may vary from very mild to severe, the psychological effect of a noticeably misshapen forehead and face can cause great problems and is a very important reason for seeking treatment. The child must also be evaluated for any signs of increased intracranial pressure due to restricted skull growth.
The type and timing of surgery varies with the child and diagnosis. However, there are some common elements.
The surgeons make an incision or opening in the skull behind the hairline so that afterwards the scar is covered by the hair. The head is not shaved. The bone in the area of the defect is reshaped or replaced with bone from another area of the skull. The surgery is usually very successful, and most children go on to lead normal lives with no residual effects of their craniosynostosis.
There is a chance that your child may need blood during surgery or the recovery period. There is a Designated Donor Program available whereby family members or friends can donate blood in advance. We can help you with these arrangements before surgery.
The total hospital stay is usually 3 to 4 days. Your child will have a preoperative workup in the Day Surgery Unit the day before surgery. This involves completing the necessary paperwork, a physical examination by a nurse practitioner, and lab work. A tour of the operating room and hospital by a Child Life Specialist can also be arranged. On the morning of surgery you will report back to the Day Surgery Area. There is a Family Waiting Room nearby where you can wait during the operation and receive progress reports from the OR nurses.
After surgery your child will spend the night in the Intensive Care Unit in order to be closely watched. Parents are able to visit as much as possible. While in the ICU, your child will have several IV lines and monitors, as well as a dressing covering the head. Most of these will be removed before your child leaves the ICU.
There will be some swelling of the forehead and eyes after the operation. This usually increases after the drain is removed, and the eyes will swell shut for a few days.
The day after surgery, your child will be able to start having liquids and will gradually progress back to a regular diet.
You are probably worried about the amount of pain your child may experience during this time. Every effort is made to keep them comfortable and pain medication is available to ease their irritability. All in all you will be surprised at how well your child tolerates the operation and recovery.
Your child will not require any special care after discharge except for common sense safety precautions. The hair may be washed and the stitches are dissolvable. A follow-up visit is scheduled for approximately 2 weeks after discharge.
Office visits, hospitalization and surgery can be emotionally and financially difficult for the family. We realize this and have professionals such as Child Life Specialists, Financial Counselors, and Social Workers available to help. There is also a parent support network that provides an opportunity for parents to meet and talk with other families in the same situation.
What is the treatment for deformational plagiocephaly?
After your health care provider has determined the severity of the deformational plagiocephaly, a specific treatment plan will be made. This may include frequent position changes of your baby’s head, alternating sleep positions, keeping your baby off of the flat areas of the head as much as possible, and not putting your baby on his back when he is awake. Tummy time is also important. If torticollis is diagnosed, a referral for physical therapy will be made. Depending on the severity of the head shape, a cranial remolding band or helmet or orthosis (these are the same) may be prescribed to reshape the head.
How does a cranial remolding band or helmet or orthosis work?
Cranial remolding bands or helmets are custom-made orthotic devices, fabricated out of a hard outer shell with an inner foam lining. These orthotic devices work by providing mild constant pressure to the prominent parts of the skull and allowing the natural growth of the baby’s head to fill in the flat areas. There is no pain. Adjustments to the helmet or band need to be made every 1 to 2 weeks as the baby’s head grows very quickly. Average length of wear is 3 to 6 months, depending on the baby’s age, the growth of the baby, and the severity of the deformational plagiocephaly. Sometimes a second helmet or band may be necessary if the baby grows out of his first one prior to completion of treatment. Cranial remolding therapy is most effective when started by about 6 months of age; however, older babies also benefit from treatment.