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Deformational plagiocephaly is a deformity of the skull that occurs as the result of a child repeatedly sleeping in the same position. It causes the soft bones of the skull to conform to an abnormal shape, typically presenting as flattening in the back of the head, or of one side or the other.
When an abnormal head shape is seen, it is imperative that craniosynostosis is ruled out as the cause. Therefore, a skull xray or head CT scan may be ordered to aid in this process.
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Common causes include back-sleeping due to the recommendations of the American Academy of Pediatrics for safe sleep practices and/or torticollis. Use of the swing, bouncy seat, car seat, stroller, in-utero positioning, multiple gestation and time spent in the NICU may exacerbate plagiocephaly.
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Torticollis is the congenital tightening of the sternocleidomastoid muscle in the neck. This condition presents as an abnormal tilt or abnormal rotation of the head to one side or the other. As torticollis is a common cause of plagiocephaly, it is essential that this condition is immediately examined and ruled out in any child who has plagiocephaly. If the torticollis is not treated, then subsequently the treatment for the plagiocephaly, including use of a helmet, will not be fully effective and the plagiocephaly will continue to persist. This condition is treated with physical therapy and an at-home stretching and exercise regimen.
Today, 1 out of every 50 newborns has plagiocephaly.
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The two methods of treatment include (1) re-positioning techniques to relieve pressure off the affected part of the skull and (2) use of an orthotic helmet.
Repositioning can be helpful if the condition is caught while the baby is very young while the bones are very soft. It consists of rotating the child in the crib from the back position to the side position, which complies with SIDS prevention guidelines.
The cranial molding orthosis (helmet) works by passively guiding the new growth of a baby's head into the flattened areas to reshape the skull. It also provides a rounded surface so that the baby won't prefer to lay into the flattened spot. The helmet must be adjusted by the provider every 2 weeks to accommodate the rapid growth of the baby's head.
Most cranial devices are fabricated from a plaster of Paris impression or a digitalized image of the child's head shape. The outer portion of the helmet is a semi-rigid shell, which is bonded to a foam lining on the inside. The lightweight cranial headband applies dynamic pressure to the elevated areas, leaving spaces for growth and remodeling of the flattened areas.
In order for it to be effective, it must be worn 23 hours per day. The one hour per day that it is off can be for swimming, cleaning of the helmet, bathtime, or pictures. The typical length of a cranial remodeling treatment is directly dependent on the age at which the child begins this therapy. It is also possible that more than one helmet may be needed to complete the therapy.
Dr. Stelnicki will decide with you when the treatment has been optimized. Careful measurements of your child's head shape will be taken at each visit to evaluate the results of the therapy.
In general, a helmet may be initiated from the time it is determined that the child has achieved good neck control to 18 months of age. However, the most effective time to begin helmet therapy is while the head circumference is growing most rapidly, which in most children begins at approximately four and a half months.
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It is essential that after your child has been diagnosed with plagiocephaly that you contact the provider of your helmet therapy as soon as possible. Some insurance companies will fight you on authorizing the treatment and call this a cosmetic concern. It is important to know that this is not a cosmetic problem. It is a deformity of the skull, which is abnormal and is a result of a medical recommendation. There are theoretical concerns about long-term problems with temporomandibular joint placement and astigmatism in the eyes. Try to get your pediatrician to write you a letter of support; this will help in most cases. Dr. Stelnicki, of course, will help you and support you in every way.
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Craniosynostosis is defined as the premature closure of the cranial sutures (see photo). The premature closure of these sutures, or what some people call soft spots, results in restricted and abnormal growth of the head. This restriction is frequently associated with eye and ear problems. Occasionally, defects with hands, feet, mouth, spine, heart, and kidneys are also seen. In some cases, this condition is part of a genetic syndrome. What this means is that the patient will have a certain appearance, which allows a geneticist to classify the constellation of problems. Recent advances in molecular biology around the country have determined that in many of these syndromes there are defects in specific regions of the patients DNA, which are associated with this problem. Click here to visit the NIDCR homepage. An example is a defect in a growth factor receptor in the developing head called the fibroblast growth factor receptor (FGFR). There are several subclasses of this receptor that, when altered, lead to craniosynostosis syndromes such as Apert's and Crouzon's.
Our team is one of the few in the country specializing in Endoscopic Craniosynostosis repair. This minimally invasive surgery is used for help correcting several types of craniosynostosis s with less scarring and without the need for metal plates and screws.
Dr. Stelnicki as performs Spring Skull reconstruction and Distraction Osteogenesis of the skull on selected patients. The springs can be placed in the skull, prior to one year in life, for the treatment of sagittal craniosynostosis. Springs are less invasive than open cranial vault remodeling, but more invasive than the endoscopic repair. They can be used on the patient who is older than 5 months of age, but less than one year old. The springs expand the elongated scaphocephalic skull slowly as the child grows. (see figure) There are placed through a relatively small incision with little skull dissection at the time of surgery. They are left in place for several months and then removed once the child has had correction of the scaphocephaly. No helmets are required for this therapy, making it useful for families who cannot come in frequently for helmet adjustments. Ask Dr. Stelnicki if this operation is the right one for your child.
Dr. Stelnicki also uses distraction Osteogenesis of the Skull in the treatment of craniosynotosis. During infancy, Dr. Stelnicki will utilize skull distraction when necessary for the treatment of coronal, metopic, and syndromal craniosynostosis as an adjunct to the endoscopic repair. The use of distraction osteogenesis at the time of endoscopic surgery involves the placement of a specialized distractor.
Dr. Stelnicki will also use distraction in early childhood for monoblock advancement.
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Dr. Stelnicki is the only surgeon in Florida routinely performing endoscopic craniosynostosis repair. In addition, our center is only one of a few in the entire country to perform this technique on a variety of craniosynostosis types. Our practice was recently honored by being able to present the first series of endoscopic craniosynostosis repair vs. traditional repair. This paper which given at the prestigious International Craniofacial Society meeting in Monterey California was the first to carefully analyze the advantages of endoscopic repair over the traditional open technique. This new technique for treatment of craniosynostosis is minimally invasive. It creates incisions smaller than 2 cm in length that are used to access the fused and deformed skull. A specialized set of endoscopic instruments designed by Dr. Stelnicki, are then inserted through these holes and used to completely reshape of the skull without performing a large ear-to-ear incision.
The advantages to endoscopic surgery are many. The surgery has less blood loss than the traditional repair and, in many cases, prevents the need for blood transfusion during the surgery. The endoscopic technique also results in less postoperative swelling, decreases operative time, and decreases hospital stay by several days. Postoperative pain is also significantly reduced by this operation. The other advantage to endoscopic surgery is that there are no internal screws or plates that are required to hold the bone in place after surgery. This decreases the risk of plate induced pain, infection, swelling, or allergic reaction in your child. Dr. Stelnicki would be glad to discuss the advantages of endoscopic craniosynostosis surgery versus the classic craniosynostosis repair with you at time of the initial consultation.
Endoscopic and surgical correction is available for any patient, under 5 months of age, with a craniosynostosis. Initially endoscopic craniosynostosis corrections were restricted to single suture fusions, such as sagittal craniosynostosis, coronal craniosynostosis, metopic craniosynostosis, or lamdoid craniosynostosis. However, we now know that is also a useful adjuvant in the treatment of multi-suture craniosynostosis. When possible, the endoscopic approach is used in the first stage of these patient's repairs.
Endoscopic craniosynostosis repair must be combined with the use of a molding band in order to normalize skull shape after the surgery. Immediately after the surgery, you will note a significant improvement in your child's head. However, the final shape will not bee seen until after 6-8 months of helmet therapy. The corrective helmets used in helmet therapy are FDA approved devices for molding and protecting the developing head. They have been used on multiple patients throughout the country following endoscopic craniosynostosis repair.
Dr. Stelnicki is also a member of an elite set of surgeons who have designed & created their own endoscopic craniosynostosis instruments. These tools, which bear our name and are sold by KLS Martin, are now available to any surgeon around the world for the treatment of this condition. Our research has helped in the creation of these instruments and will aid in developing even newer instruments to better treat children with craniosynostosis in a minimally invasive fashion.
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Apert's syndrome, Crouzon's syndrome, Pfeiffer's syndrome, Saethre Chotzen syndrome, and other forms of syndromic craniosynostosis.
Although most forms of craniosynostosis are sporadic and affect no other part of the developing infant, there are some rare forms of craniosynostosis that are syndromic. These patients have a very characteristic appearance. Their craniosynostosis usually involves multiple sutures, and in every case, more than just the shape of the skull is affected. These patients should always be treated by a craniofacial team, as their syndrome involves multiple regions of the body. Without a good team approach of addressing each problem in an organized way, the likelihood of a good treatment result is decreased.
Apert's syndrome is an autosomal dominant form of congenital craniosynostosis. It is thought to result from a mutation in a Fibroblast Growth Factor Receptor (FGFR I-IV) that is essential for proper head and extremity development.
Patients with Apert's syndrome typically have premature fusion (craniosynostosis) of multiple cranial sutures. The ones most commonly affected are the coronal sutures that extend from ear to ear. As a result of this fusion, the head of these patients is shortened from front to back (brachycephalic) and elongated from top to bottom (turricephalic). The eyes of these patients appear to "bulge out" due to the fact that their skull base and mid-face fails to grow in a normal fashion. The palate of these patients is typically high arched and narrows. Dental eruption is typically delayed.
Apert's children also always have some degree of hand and foot webbing called polysyndactyly. This can be of a minor type that only requires skin separation, or a more sever type where there is actual fusion of bone within the hands and feet.
Most Apert's children have normal intelligence. However, varying degrees of mental retardation have been seen with this condition. Effected areas in Apert's syndrome include:
Patients with Crouzon's Syndrome have a similar facial appearance to those with Apert's syndrome. However, there are several important distinctions. Crouzon's patients seldom have macrocephaly, in spite of the fact that they have multiple cranial suture fusions. They have a variable degree of midfacial deficiency and exorbitism. Their hands are completely normal. Their mental development is variable. And their teeth tend not to have the delayed eruption seen in patients with Apert's syndrome.
Like Apert's, children with Crouzon's typically have a genetic mutation in the FGFR gene. Mutations in FGFR I-IV have been seen, but FGFR I seem to be the most common. The phenotype of children with Crouzon's is highly variable. In some instances, only mild brachycephaly and exorbitism are seen. In other cases, sever brachy-turricephaly is evident and the eyes are wide set (hypertelorism).
The patient with Pfeiffer's syndrome is similar to the Apert's and Crouzon's patient. These patients also have a defect in the FGFR gene, which lead to a multi-suture craniosynostosis. However, their midface is usually more severely effected. In many cases, these patients will have severe exorbitism that puts them at risk for corneal exposure and damage. A full ophthalmologic evaluation by an experienced pediatric ophthalmologist is needed, and in some cases these patients need the lateral aspect of their eyelids sewn together to provide adequate protection for the eye (tarsorhaphy). Patients with Pfeiffer's syndrome also have thumb and great toe anomalies, which occasionally have to be treated by an experience hand or foot surgeon. All Pfeiffer's patients seen by our team are carefully evaluated by our pediatric hand surgeon and podiatry staff to ensure that proper growth is occurring in these areas.
There are several other syndromes involving craniosynostosis. Whether it is Saethre-Chotzen (a mutation in the Twist gene), Carpenters syndrome, or another rare form of early suture closure, our team carefully evaluates each patients to make sure that all their medical and developmental needs are met.
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The treatment of these syndromes begins at birth. Because the midface fails to grow, Apert's patients may have problems breathing. As a result, they should be watched closely at birth for signs of apnea or desaturations. If the breathing problem is severe, early intervention is required. Our nurse practitioner/feeding specialist works closely with each child to insure that nutrition is adequate. Our staff geneticist performs a full genetic evaluation, including FGFR receptor analysis to complete the diagnosis.
The child is then evaluated carefully by our pediatric neurosurgeon for signs of hydrocephalus or increased intracranial pressure. Based on this evaluation, plans are made to begin the skull remodeling surgery. Our goal is to normalize skull shape by the patient's first birthday. This is done by Dr. Eric Stelnicki working closely with one of the pediatric neurosurgeons. Care is taken to sequentially mold the front and back of the misshapen head, while decreasing intracranial pressure and allowing room for growth.
If a cleft palate is present, this is typically closed between 12-18 months of age. The decision to close the cleft is based on a close consultation between the Craniomaxillofacial surgeons, the team of speech therapist, the orthodontic staff, and the patient's family. The goal of this surgery is to close the hole in the roof of the mouth, while maximizing speech development and maxillary growth.
Treatment of the hands and feet begins as early as 2-3 months of age. Our team hand specialists are dedicated hand surgeons who specialize in the treatment of pediatric hand abnormalities. The type of surgery performed is based on the type of hand abnormality present in the patient. Severe deformities usually begin with a separation of the thumb so that the infant can hold objects. We also have hand therapist who will work with the child to maximize their function though out their development. Our team is one of the few in the country, which recognizes the importance of treating feet abnormalities in these patients. The syndactyly of the toes is treated by trained orthopedic and podiatric surgeons who understand the need for proper foot care and development. These are typically done in conjunction with the hand surgeries in order to decrease the total number of operations a patient with Apert's has during their lifetime. Other hand and foot abnormalities are addressed and treated by the team in an organized, staged fashion that optimizes form and function.
Speech development is carefully followed by our speech therapist. Early intervention is given as needed to get the best results. Occasionally speech surgery is required to normalize the production of sound. However, this is only utilized as a last result when conservative measures have not been successful.
Dental development is followed closely by our pedodontic and orthodontic teams. Treatment is adjusted based on the pattern of dental growth and the type orthodontic care needed to normalize tooth and gum position. Children with these syndromes frequently require a surgery to put the underdeveloped "mid-face" into a proper position. This is performed at age 5 using the newest, less invasive methods of distraction osteogenesis when possible. This technique maximizes facial position with the least amount of risk.
A careful eye exam is given at birth. Corneal exposure problems are treated immediately. Problems with eye muscle movement (strabismus) are treated by our pediatric ophthalmology specialist in order to normalize eye position and visual development. In patients with very wide set eyes (orbital hypertelorism) the position of the eyes is normalized at 4 years of age. This is done in conjunction with Dr. Stelnicki, the pediatric neurosurgeon, and the ophthalmologist in order to get the best final result under the safest conditions.
In the teen-age years, nasal and jaw deformities are again addressed by the team. Patients with these syndromes usually require orthognathic surgery to correct jaw position. This is done in conjunction with our orthodontic staff, which works closely with the patient to maximize dental growth and development. Any additional nasal surgery is also performed at this time. Recently, we have been utilizing virtual reality techniques to plan our operations at this stage. A CT scan of the patient's facial bones and skull is compared to a "normal" template. Based on this comparison, the facial bones are segmented and moved into their proper location. All of which significantly normalizes facial appearance.
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Deformational plagiocephaly is becoming a worldwide concern. It is a deformity of the skull that occurs as the result of the child sleeping the same position, causing the soft bones of the skull to become deformed and then conform to an abnormal shape.
Today, deformational plagiocephaly is seen in up to 1 in every 50 newborns. It has developed as a result of the recommendations of the American Academy of Pediatrics for children to sleep on their back as part of the "Back to Sleep" campaign. The Back to Sleep campaign is, of course, endorsed by ourselves and all pediatricians because it has been shown that by sleeping on a child's back, there can be a decrease in the risk of sudden infant death syndrome or SIDS. However, the fallout is that many children are developing flatness on the back of their head which is causing severe deformity of not only the skull shape but also the position of the ear, the position of the eye, and the position of the temporomandibular joint. Of long-term concern is how abnormal positioning of the eye and the temporomandibular joints may effect problems with reading, vision, astigmatism, and temporomandibular joint position and pain as this abnormal positioning of the joint may place abnormal forces on the TMJ and result in long-term pain and clicking in these children.
The correction of deformational plagiocephaly has also undergone an evolution over the past several years. Fifteen years ago, when a few cases were seen, it was deemed to be a neurosurgical problem. Neurosurgeons and plastic surgeons around the world were performing a skull reconstruction for this condition similar to that being performed in classic craniosynostosis. This is no longer the case.
Doctors now realize that this condition can be treated by nonsurgical methods that are as effective as traditional surgical therapies. The two treatments for deformational plagiocephaly are: 1) Positioning of the child. 2) Use of an orthotic remolding helmet. Positioning of the child can be effective if the abnormal skull shape is caught very early. The child can be positioned instead of constantly on the back, in rotating positions from back to side. This is within the guidelines for decreasing the risk of crib death and at the same time, the rotating position of pressure on the skull prevents or decreases the risk of flattening.
If your child has flattening, you should position your child on the opposite side away from the flattening as much as possible. In the car seat and in the stroller, a bump or blanket, which in no way would cover the child's face and cause suffocation, should be placed behind the flattened part of the head to actually force the head over toward the opposite direction. In addition, the child's position should be rotated nightly and wedges and positioners should be used in the bed to try and decrease the continual pressure in the flat spot. It is essential to also rule out torticollis as a cause of a contributing factor to the flatness. Many children will have stiffness in the neck, which is felt to be related to intrauterine positioning that keeps them from turning their head to one side or the other. Most children with this problem can be treated with physical therapy alone. However, the flattening of the head will persist until the torticollis has been treated. If you feel that there is any restriction in the motion of your child's head, then your child should be seen by a craniofacial specialist to rule out torticollis immediately. Physical therapy regimen will then be assigned that will augment any attempt at positioning or treatment with an orthotic band or helmet.
Dr. Stelnicki has successfully used cranial remodeling devices in order to treat deformational plagiocephaly that has not been responsive to positioning. Cranial remodeling devices can be used effectively up to a year and a half of age, and in rare patients even beyond that. There are several FDA approved devices, which have now been used on thousands of children across the world for treatment of deformational plagiocephaly. Dr. Stelnicki works with Star Band, Doc Band, Kinder Band, Hanger Bands, and others. These Cranial remodeling devices work in several ways. They provide a rounded surface that prevents the child from rolling into the flat spot on the back of the head. It is also a passive process that is adjusted weekly to control head growth. The head is held in areas where it is already pushed too far forward and there is space made in the areas of the flattening so that as the brain and skull grow, the flattening resolves. These cranial devices must be adjusted weekly or bi-weekly in order to work effectively. With the use of the helmets, we expect a 90-95% correction in head shape. All human beings have some asymmetry in their skull and it is unrealistic and unnatural to expect perfect, rounded symmetry following any treatment of head shape. The typical length of a cranial remodeling treatment is directly dependent on the age at which the child begins this therapy. It is also possible that more than one helmet may be needed to complete corrections. When the child outgrows his or her helmets, a new helmet could be reapplied after updated measurements are taken to fit the child with new proper fitting device.
It is essential that after you have been diagnosed with deformational plagiocephaly that you contact the provider of your helmet therapy as soon as possible. Some insurance companies will try and fight you on authorizing this band and call this a cosmetic concern. This is not a cosmetic problem! It is a deformity of the skull, which is abnormal and is a result of a medical recommendation. The skull is deformed and should be corrected by an FDA approved device. You may have to fight your insurance company. Try to get your pediatrician to write you a letter of support; this will help in most cases. Dr. Stelnicki, of course, will help you and support you in every way. Cranial remodeling devices are developed as a conservative treatment and are most effective early in life. The correction of this does not in any way effect the development of the brain; In fact, the brain will develop the same regardless of treatment. However, the remodeling of the head needs to be done early in order to be effective. Most cranial devices are fabricated from a plaster of Paris impression, or a digitalized image of the child's head shape using a semi-rigid outer shell, which is bonded to a foam inner lining. The lightweight cranial headband applies dynamic pressure to the elevated areas, leaving spaces for growth and remodeling of the flattened areas. For effectiveness, cranial devices need to be worn 23 hours a day with 1 hour taken off for skin care, hygiene and cleaning of the helmet. Dr. Stelnicki will decide with you when the treatment has been optimized. Careful measurements of your child's head shape will be taken to evaluate the results of the treatment.
Dr. Stelnicki also uses cranial remodeling devices in conjunction with his endoscopic surgical repair for craniosynostosis. This helmet will be worn for 6-8 months postoperatively depending on the age of the child at the time of craniosynostosis repair. It must be worn at all times with the exception of being taken off once a day for cleaning. The endoscopic repair success relies heavily on the use of the helmet and families who cannot commit to the postoperative helmet and the need for constant adjustments for 6-8 months should not undergo endoscopic cranial vault repair. With the helmet, however, the correction of craniosynostosis can be minimized to a much faster operation with much less blood loss, with much less time in the hospital and much less morbidity. There is also no need for placement plates and screws around the skull, as the cranial remodeling devices offers post surgical splints for molding the healing and regenerating skull bone.
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For other questions and concerns please consult Dr. Stelnicki in our office.