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Craniofacial Procedures

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

Plagiocephaly

What is Positional Plagiocephaly?

An infant's skull is made up of free-floating bones that are separated by sutures. 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. 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. The diagnosis most often given is of a Positional Head Deformity. Due to the malleable nature of an infant's skull, it is possible for external pressures to cause skull deformity. The most common Positional Head Deformity is Positional Plagiocephaly. Positional Plagiocephaly is caused when repeated external pressure is applied to one side of the occiput (the back of the head) and a flat spot occurs. The side of the occiput that is flattened will often be accompanied by a prominent forehead, which when viewed from above will give the head a parallelogram shape instead of a normal symmetric oval shape. It is also common for an infant with Positional Plagiocephaly to have misaligned ears (the ear on the affected side may be pulled forward and down and be larger then the unaffected ear) and facial asymmetry, with the affected side of the face having a fuller cheek, and a more prominent appearance. Facial asymmetry on the affected side can also include a jawbone that is tilted, and an eye that appears displaced and mismatched in size.

Why is the Word Positional Used?

The term Positional is used to differentiate from true Synostosis, which is a premature fusion of one or more of the skull sutures (Craniosynostosis) and requires surgery to repair. When the term Positional is used, it means that there is NO premature suture fusion and that an external pressure on the skull caused the deformity.

Is Positional Plagiocephaly the only Head Deformity?

Positional Plagiocephaly is not the only head deformity, though it is by far the most common. Positional Brachycephaly is when the entire back of the head is flat and the head has the appearance of being wide and short. Positional Brachycephaly is most often seen when a child sleeps entirely on the back of his head. Positional Scaphocephaly is when the head is long and narrow. Positional Scaphocephaly can result when the infant is in a breech position during the pregnancy and the head becomes wedged underneath the mother's ribcage. Prematurity can also be a contributing factor in Positional Scaphocephaly.

What is Facial Asymmetry?

The external forces that push and mold the skull can also affect the features of the face causing facial asymmetry. Facial asymmetry is when one side of the face does not match up with the other side of the face. Most often the affected side will have a more pronounced forehead and possibly a cheek that is fuller then the other cheek. It is also possible to see the eye on the affected side appear displaced and mismatched in size and for the jaw to appear tilted. All or some of these factors can make the face appear lopsided.

How do I see Facial Asymmetry?

It can be very difficult to see facial asymmetry. Most of the parents have the feeling that something just does not look right. If you cannot readily see facial asymmetry place you baby in front of a mirror and look at your baby's reflection. Facial asymmetry becomes very apparent using this method because the mirror will help show the baby's face in a two dimensional plane.

Is Positional Plagiocephaly Just Cosmetic?

It was believed that Positional Plagiocephaly was only a cosmetic disorder because it does not restrict brain growth or cause brain damage. However, there is now increasing evidence that other problems can arise, especially when facial asymmetry is present. Some of the problems that can be experienced due to facial abnormalities include problems with chewing and eating. Difficulty with vision could also become an issue and require corrective surgery to repair. While head shape abnormalities can possibly cause temporormandibular joint syndrome (The temporormandibular joint or TMJ is the joint that operates the jaw.). Furthermore, the American Medical Association defines that a cosmetic procedure changes normal structure of the body in order to improve appearance, while a reconstructive procedure is performed on an abnormal structure of the body to improve function, or return it to normal. The possibility of psychological disturbances due to an abnormal appearance also weighs heavily on parent's minds. Knowing how conscientious children can be of their appearance, especially during teenage years, is cause for concern for many parents that have an infant with Positional Plagiocephaly.

Who Do I See To Diagnosis Positional Plagiocephaly?

In the majority of cases, concerns of Positional Plagiocephaly is first brought to the attention of the child's pediatrician. While it is not unusual for a pediatrician to refer a child to a pediatric neurologist, a pediatric neurosurgeon, or a pediatric craniofacial plastic surgeon, it is becoming more common for a pediatrician alone to make the diagnosis, and suggest treatments. However, it is essential to deal with a knowledgeable physician to obtain a proper diagnosis. If there is any doubt on the part of the parents then seek another professional opinion, preferably with a doctor experienced with Positional Plagiocephaly.

What Kind Of Tests Will Be Run?

The most common tests performed are X-Rays, CT-Scans and MRI's. Though in most cases only X-Rays will be performed, it is possible to have a combination of just two or of all three tests. Testing is typically done to confirm that the head deformity is not caused by a true Synostosis (a premature fusion of one or more skull sutures). However, increasing evidence suggests that Positional Plagiocephaly differs from Craniosynostosis Plagiocephaly in outward appearance. This finding is leading more doctors who are well versed in Positional Plagiocephaly, to feel confident in making a diagnosis without additional testing. But again, it is important for the parents to feel confident with the doctor and seek other opinions if there is doubt or uncertainty.

What Causes Positional Plagiocephaly?

There are several possible causes of positional plagiocephaly. The most common causes are:

In Utero Constraint

When a baby develops in a womb that is constricted it can lead to Positional Plagiocephaly. Constriction of the womb can be caused by a variety of factors including: a multiple pregnancy with two or more fetuses, a small or malformed uterus, a small maternal pelvis, or an inappropriate amount of amniotic fluid. A baby that is in a breech position can also develop Positional Plagiocephaly if the baby's head becomes wedged under the mother's ribs.

Prematurity

The skull of a premature infant can be very soft and malleable making the head more susceptible to remolding due to external pressures.

Back Sleeping

In 1992 the American Academy of Pediatrics' (AAP) made the recommendation that infants should sleep on their backs to reduce the risks of SIDS (Sudden Infant Death Syndrome), which launched the "Back To Sleep Campaign". While the Back to Sleep Campaign has made tremendous strides in reducing SIDS, there has also been a dramatic increase of the number of infants suffering from Positional Plagiocephaly due to back sleeping. Parents were not given enough information regarding back sleeping and how the infant's sleeping position should be alternated to prevent constant sleeping on the same side, nor were parents informed of the importance of "tummy time" during play. Consequently, because of the fear that many parents have regarding SIDS, many infants spend almost 100% of their time on their backs. Between infant car seats, infant carriers, bouncy seats, infant swings and sleeping on a mattress at night an infant spends little or no time without external pressure applied to the back of the head which leads to a susceptibility of Positional Plagiocephaly.

Congenital Muscular Torticollis

Congenital Muscular Torticollis (CMT), also known as Twisted Neck or Wry Neck, is a condition that is usually caused when one or more of the neck muscles is shortened or tightened on one side of the neck. This causes the baby's head to tilt and/or turn in one particular direction. CMT can be very blatant or very subtle. Parents are usually the first to notice the more subtle cases. If your baby tends to always look in one direction then it is very possible that CMT could be the cause. If you suspect that your child may have CMT it is important to have it diagnosed by a physician and to have it treated. Treatment of CMT usually consists of physical therapy to lengthen and loosen that affected muscle(s). Only in VERY severe cases is surgery needed to repair the muscle(s). For more information on CMT please visit the Torticolliskids website at:
http://pages.ivillage.com/torticolliskids/torticolliskids

What Treatments Are Available for Positional Plagiocephaly?

Before discussing treatment methods, the factor that age plays in treatment must be explained. As mentioned earlier, an infant's skull is comprised of free-floating bones connected by sutures. At approximately 12 months of age, these sutures will fuse and connect the once free-floating bones. Once the skull is fused into one piece the following treatment methods will not be effective in correcting an abnormal head shape. The treatment methods use the malleable nature of the unfused skull to their advantage in achieving a normal head shape.

Unfortunately, once the skull is fused it is no longer malleable and the only option for correction is reconstructive surgery to repair the shifted bones. For this reason it is imperative for parents to seek medical attention as soon as possible, deal with a physician that is knowledgeable in Positional Plagiocephaly, and choose a treatment course that they are confident with. Currently the most common forms of treatment are reposition therapy and helmet or band therapy. Some doctors argue that Positional Plagiocephaly will correct on it's own as the child grows and becomes developmentally mature, resulting in less time on the back. This can be true in mild cases, but most parents that have children with moderate or severe cases are not willing to take that gamble and opt for treatment. Before treatment is decided upon it is best to consider all of the factors. It is commonly believed that aggressive reposition therapy is best utilized when a child is between the ages of 0-6 months depending on the severity of the Positional Plagiocephaly. After 6 months (or sooner depending on the child), it is much harder to control the side that the baby sleeps on so reposition therapy may not be as affective. Helmet or band therapy is also best started at an early age, usually around 5 months. Though some bands and helmets can be used as late as 18 months, effectiveness is greater when the baby is younger. Because of this, parents, along with doctors should make educated decisions based on the individual baby, the baby's age and the degree of Positional Plagiocephaly and facial asymmetry.

Reposition Therapy

Repositional therapy is simply the concept of repositioning the baby so that he does not rest on the flat spot. It is believed that by removing the external pressure, the flat area will "round out" as the head grows. Before trying reposition therapy it is important that the baby be cleared of Torticollis. If the baby has Torticollis it is important that parents discuss this with the baby's physician and utilize physical therapy to stretch the neck muscles. If reposition therapy is to be used, the baby's physician should be consulted on the best way to achieve this.

Some common practices of reposition therapy are placing a rolled towel or blanket beneath the back of the flattened side to prevent the baby from rolling onto the affected side. Another common tactic is placing crib toys on the opposite side of the flattening to make the child look in that direction.

There are several different methods that can be used for reposition therapy. A little bit of creativity is also of great benefit in this area. However, always keep safety in mind and never place an item in the crib that could be harmful. Corrective Helmets and Bands The first corrective device offered for Positional Plagiocephaly was the Molding Helmet. The original molding helmet introduced in 1979 was designed with the basic concept that if you surrounded the asymmetrical infant head with a symmetrical (normal) mold, the skull will grow into the mold and take on the shape of the mold resulting in a normal shaped head.

An alternative technique, Dynamic Orthotic Cranioplasty SM (Doc Band(r)), was subsequently developed as a more proactive approach to treatment of Positional Plagiocephaly. In this technique, the device was specifically designed to apply gentle pressure to the area of the head where growth was not wanted, while leaving space where growth was needed. With this technique, the child is seen on a weekly to biweekly basis to have adjustments made to the band to ensure proper growth of the head and optimum correction of the deformity.

Today we are seeing a large variety of bands and helmets, many of which take into consideration the concepts of the original molding helmets and the DOC Band. The child's physician will be able to direct parents on where to obtain a helmet or band. It is important that parents research the success rate of any helmet or band under
consideration. If a particular helmet or band does not have a good success rate then another should be found.

Bands and Helmets come in several different designs but two things that they all have in common is that they are all light-weight and they are all custom made to fit the individual baby's head. In order for the band or helmet to be custom fit, there must be a casting done of the child's head. The casting process takes only a few minutes and although most children do not take kindly to it, it is a painless procedure. The length of treatment time for a helmet or band is dependent on several things: the age at which the baby starts the therapy, the particular design of the band or helmet used, the rate at which the individual baby grows, and the degree of Positional Plagiocephaly, are all factors in determining the length of treatment necessary. Therefore, it cannot be stated how long therapy will last for an individual baby. However, on average most children will complete therapy within 2-6 months, but again this is only an average and it is possible for an individual baby to complete therapy earlier or later depending on the factors listed above.

Positional Plagiocephaly Prevention

The most disturbing fact about Positional Plagiocephaly is that the majority of cases are preventable. Although we as parents have been told time and time again to keep our babies on their backs because of SIDS, the one thing we were not told is to vary the baby's position so that the baby is not always resting on the same side of the occiput. The number one preventive measure of Positional Plagiocephaly is to vary the sleeping position of the baby. We as parent need to be aware of how our babies sleep and take steps to ensure that equal time is
spent on both sides of the occiput. In the early weeks, the baby will stay in the position that he is placed. For this reason, simply placing the baby on alternate sides, left side, back, right side, each night will reduce the risk of Positional Plagiocephaly.

Once it becomes difficult to dictate the baby's sleep position, crib toys should be placed on both sides of the crib. If this does not work and the child still favors looking in one direction, then alternate the sleeping position by placing the baby's head at the foot of the crib every other night. Most babies tend to turn in the direction that faces out of the crib toward the center of the room. Placing the baby's head at the foot of the bed will allow the baby to look to the center of the room, but do it on the other side of the occiput. Being able to recognize the signs of Congenital Muscular Torticollis (CMT) is also important in prevention of Positional Plagiocephaly. If the baby tends to always turn in one direction or the head is always tilted to one side it is possible that CMT would be the cause. Although it is true that not every baby with CMT will have Positional Plagiocephaly, there is a high enough incidence rate of a baby having both disorders that parents should be able to recognize the warning signs and address concerns with the baby's physician.

The next preventive measure is "tummy time" during play. We as parents have strayed from this important practice. When our baby is awake and is being supervised there is no reason why the child should not be allowed to lay on his or her stomach. When tummy time is first introduced to a baby, it is best done in short intervals of only about a minute or two at a time. As the baby gains muscle control and begins to enjoy tummy time, the intervals can be increased. Thisnot only has the benefit of allowing an infant to strengthen muscles that are not used when laying on the back, but it also allows the back of the head to have some time without external pressure pushing up against it.

Finally, another preventive measure concerns the extended use of car seats (used as a carrier), infant carriers, bouncy seats and infant swings. Be aware of how much time your baby spends in any of these devices. The convenience of these items should not tempt parents into using them for the majority of the baby's day. If your child spends close to 100% of his or her day in one of these devices then take appropriate steps to lessen this by holding the baby more and by including tummy time during the day. If these items must be used for extended periods of time be aware of the direction that the child looks in. If the child tends to look in one direction, change the placement of the device to offer more stimulus on the side that the baby tends to look.

Children and Surgery

Surgery can be a frightening experience for anyone but is especially scary for children. The following is a direct message to our young patients and explains in more detail what they can expect during their time with us at the hospital. It takes a lot of people to keep the operating room (OR) working. When you arrive at the hospital, someone at the reception desk will take your name, address, phone number, and your parents' names. Then you will meet a nurse, who will ask you and your parents all about your health: what the problem is, how you feel, and whether you're allergic to anything. You may also get a physical exam to check your temperature, heart rate, and blood pressure and make sure you are ready for surgery. The nurses and doctors you'll meet during this part of your time at the hospital are great resources - ask them any questions you may have. They know the hospital and OR better than anyone.

To keep the OR sterile, the people who work there wear caps over their hair and masks over their mouths and noses to avoid spreading germs. Some even wear booties over their shoes! They might all look alike, but everyone in the OR has a different job. Before the surgery, you'll see an anesthesiologist (say: an-es-thee-zee-aah-luh-gist). This person is specially trained to help you sleep during surgery so you won't feel a thing during the operation. You'll also see the surgeon right before you drift off to sleep. The surgeon is the doctor who will perform your operation.

The surgeon and the anesthesiologist aren't the only people you'll see in the OR. You'll also see more nurses and maybe even a few medical students (people who are going to school to learn to be doctors).

If you will be going to sleep for the surgery, you probably won't be able to eat breakfast. That's because having food - or even water- in your stomach can make it dangerous to give you anesthesia. You'll be told ahead of time what you can and can't eat or drink. Usually skipping a meal is not a good idea, but in this case it's the doctor's orders. Don't worry - as soon as you're awake and feeling better, you can get right back to those good breakfast habits!

Right before surgery, you'll meet with the anesthesiologist. Some hospitals allow parents to be with their kids at this point, and some do not. In either case, you'll know ahead of time if your mom or dad can come with you. If they can't come into the OR with you before the surgery, they'll be right outside the door. As for what happens during the surgery, well - you won't remember much, because you either will be sound asleep or won't be able to feel the part of your body they're operating on.

When you wake up, you'll be in a recovery room. This is the wake-up room, and that's exactly what you'll be doing there - waking up. A nurse will be there to see how you're doing as you wake up. If your parents aren't allowed to be in this room, they will be nearby and you'll see them soon. Once you're fully awake, you'll either be moved to a hospital room (if you're staying overnight) or to another post-op room, where you can wait with your parents while the doctors or nurses see how you're doing.

The OR may seem a little scary. It might help you prepare for your trip to the hospital if you can take a tour of the hospital and OR before you go. And remember - this place is full of people who want you to feel good. Be sure to ask any questions you have - when you don't know the answer to something, thingscan seem scarier than they really are.

It can also help to bring a little bit of home with you on your trip to the OR. Many hospitals have waiting rooms with TVs and toys, but you can also bring things from home that make you feel good, like a favorite stuffed animal, book, blanket, or game.

 

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