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Craniomaxillofacial surgery is a highly specialized branch of plastic surgery that focuses on the complex reconstruction of the jawbone, orbits, skull and the soft tissue of the face. Patients who need a craniomaxillofacial surgeon include children with cleft lip/palate, but really extend beyond this condition to very complex skull and facial problems such as, craniosynostosis or other facial malformations (orbital hypertelorism, Goldenhar's syndrome, Craniofacial Microsomia, Treacher Collins Syndrome, to name a few), tumor ablative surgery involving the skull, or victims of trauma whose facial skeleton has been severely disrupted by the injury, or any major deformity of the jaws. A craniomaxillofacial surgeon also treats complex soft tissue problems such as hemangiomas, vascular malformations, facial tumors, Bell's palsy and severe facial scarring. Surgeon's performing this type of surgery has completed specialized fellowships dedicated to restoring facial form and function.
Some craniofacial conditions treated by Dr. Stelnicki include:
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Dr. Stelnicki is an internally recognized Craniomaxillofacial surgeon, who lectures all over the world on new surgical techniques meant to improve the quality of treatment for patients undergoing craniofacial surgery. Lecture sites include Taipei, Taiwan, Bangkok, China, Milan, Italy, Namibia, South Africa, Philippians & many other sites. By traveling the world, Dr. Stelnicki brings home the latest and greatest innovations in the world of Craniomaxillofacial surgery for our patients here in South Florida.
Any good craniofacial surgeon will belong to or lead a craniofacial team. The team should be approved and recognized by the American Cleft Palate Association, which upholds the standards for cleft and craniofacial surgical care. In the state of Florida, such teams should also be certified by the Department of Health, Children's Medical Services in order to insure their quality. Do not go to a team that does not have both these qualifications. If the team is not certified by both the ACPA and the State of Florida CMS program, then they are likely not to have any significant experience in cleft and craniofacial care. Patients should also look for craniomaxillofacial surgeon who belongs to such a team because these individuals frequently have a high volume practice that is constantly undergoing an internal review by other members of the team. Without this type of internal review, quality care is not guaranteed.
A craniofacial team should include:
Such a team will provide the multidisciplinary care of patients with craniomaxillofacial problems in order to obtain optimal results. This type of coordinated care also allows patients to see multiple physicians during a single visit. When a patient comes to a craniofacial team, the nurse coordinator will arrange for the patient to see all doctors who need to evaluate the problem. Then, at the end of the visit, the entire team will discuss the patient's problem and generate a concise and focused treatment plan, which will optimize care. This type of focused care prevents miscommunication between referring physicians and ensures the patient will have a clear idea of what each doctor involved feels is the best treatment plan.
A good craniofacial team also collects and analyzes patient data, and presents this data at national meetings. In this way, the team is able to discuss their treatment method with other leading centers around the world, making sure they are providing state of the art therapy to their patients.
The craniofacial team, under the guidance of Dr. Stelnicki at Joe DiMaggio Children's Hospital, and the cleft team at CMS or St. Mary's in West Palm Beach meet all these requirements. To contact our team please call at the Cleft and Craniofacial Office: (954) 983-1899 or contact Joe Dimaggio at (954)986-6314 or CMS office in West Palm Beach. It is important to specifically request Dr. Stelnicki to be your doctor when you call if you want your child to see the surgeon with the greatest amount of experience and expertise in craniofacial care.
All initial patient visits are arranged thorough our nurse coordinator. Please plan to spend at least half a day with us on your first visit so that we can give you a full and thorough evaluation. Also, please bring with you any copies of old medical records or X-rays, which may be helpful to our evaluation.
Drs Stelnicki uses state of the art computer virtual reality surgery to plan the treatment of facial anomalies. He also uses computer generated 3-D models to construct personalized implants that restore lost pieces of bone. These specialized implants can also be used to place "bone" into areas where bone never properly developed. It is also the most accurate way of building the facial skeleton. The type of implant used varies from the patients' own bone, to high tech polymers which are customized to individual need
Dr. Stelnicki, in conjunction with the Nova Southeastern University Craniofacial Anomalies Lab, is actively pursuing research to reconstruction bone without implants and grafts. Use a variety of bone scaffolds and synthetic bone proteins, Dr. Stelnicki has developed ways to reconstruct bone in the facial skeleton without the need to take bone from another part of your child's body. This saves your child from additional pain and scarring that most surgeons cannot avoid. It also allows your child to make there own bone, without any artificial hardware in many cases. Ask Dr. Stelnicki if your child is a candidate for this type of therapy.
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Minimally invasive Orthognathic surgery
Dr. Stelnicki is the first craniofacial surgeon in the state of Florida to offer non-osteotomy facial reconstruction. This is a unique procedure developed by Dr. DE Clerk in Europe that has only recently been approved by FDA for use in the United States. Dr. Stelnicki, and the team of orthodontists on the Joe DiMaggio Craniofacial team are first in the area to offer this unique type of minimally invasive jaw surgery to your child.
In addition, because of the unique nature of Dr. Stelnicki's practice, Dr. Stelnicki is the only craniofacial surgeon to use this technique for the treatment of cleft palate and craniofacial related jaw deformities. All other surgeons place these plates in children without craniofacial anomalies.
Bollard plates are specially designed titanium plates that adhere to the maxilla and mandible of the pre-pubescent child, that allow the orthodontist to control bone growth. This is unique because in the past, orthodontists have only been able to adjust tooth position, with minimal effect on the growing bone. When an large Skeletal Class 2 or Class 3 misalignment occurred, and the teeth didn't fit together, may jaw surgery called orthognathic surgery or jaw distraction osteogenesis, would be required to properly align the teeth and achieve facial balance. These types of surgeries are known as Le Fort operations, or mandibular sagittal split osteotomies, and they were done typically at skeletal maturity around age 16-18 years. This meant that kids with major cross bites and jaw deformities had to live throughout there teen-age years looking different to others. As any parent knows, teenage years can be the toughest times on kids. Adding the stress of a major jaw deformity to the mix only complicated matters.
This new minimally invasive therapy offers a way to avoid all this. The devices, 2 of which are placed on the mandible and 2 of which are place on the maxilla, are surgically attached to the bone around age 11-12 years. This is the time when facial bone growth is about to take off, just prior to full-blown puberty (see figure). The plates are surgically implanted through minimally invasive incisions. A small corticotomy is also made in the bone to assist in the Le Fort type jaw advancement as well as the mandibular movement. The surgery takes less than one hour. It is done under general anesthesia as an out patient procedure. The incisions are closed with dissolving sutures.
Post operatively, patents are kept on a liquid diet for a few days then a soft diet for a week. They can resume normal activity almost immediately. Ice can be applied to the face to minimize swelling. 6 weeks later, if the plates are solidly fixated to the bone, then the orthodontist can start to move the bone through the use of elastics that distract and pull the bone into the desired direction. This process takes a few years and is done in conjunction with standard orthodontics. The devices look similar to tooth born appliances so kids tolerate them well.
These devices can move jawbone between 4-8 mm and can normalize facial appearance without the risk of major jaw surgery. At the end of the Jaw movement, usually in the teenage years, the devices can be removed with a quick operation. We feel this is a major advancement for our patients with and without craniofacial anomalies.
The newest minimally invasive device to treat mid-face hypoplasia in children 8-10 years. Mid face hypoplasia or a dished in face can occur at any age. It is frequently seen in children with cleft lip and palate. Up to now, no treatment except reverse pull head gear, which corrects only a few mm of deformity, or maxillary distraction osteogenesis, which is a major bone cutting Le Fort 1 operation, were available to help children in this age group.
Piriform plates have changed all that. Now, like the patients receiving Bollard plates, patients in the 8-10 year range can achieve major facial advancement through a small operation.
The surgery is an outpatient operation done under general anesthesia. The surgery is typically less than one hour. During the operation, through 2 minimal incisions in the gum, 2 plates are place on the bone along side the nose, called the piriform aperture. The plates are held in place and the incisions closed with dissolving sutures.
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Surgery is typically performed at age 5-10 years for piriform plates or with maxillary distraction osteogenesis. Bollard plates are placed between ages 10-14 years
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A recent history and physical documenting good health is required one week or less before the surgery. A pre operative orthodontic assessment is needed. In most cases a panorex x-ray and a cephalogram are obtined. No eating or drinking after midnight, the night before the operation unless otherwise instructed.
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The surgery typically takes 1 hour per ear depending of the degree of severity
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Typically this operation is performed under general anesthesia.
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Most patients only need Tylenol or Motrin dosed according to their weight for pain management.
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Complications following this operation are rare. However, like any surgery they can occur. The complications most commonly described are bleeding, infection, hematoma, plate breaking or becoming dislodged from the bone. If any of these complications occur, bring them to the attention of your surgeon immediately.
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You should see Dr. Stelnicki or his physician assistant 7-14 days after the initial operation. Additional follow-ups will be arranged at that time.
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Bathe and shower normally, rinse will mouth wash 3 times a day post op
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Post op there is the same diet as patient with the Bollard plates. 6 Weeks later, if the plates are stable, the orthodontist will attach the reverse pull headgear directly to the plate. This will perform a gentle Le Fort 1 distraction, bringing the upper jaw forward. This process will take several years. At the end, the plates will be removed and the child will hopefully have avoided a major jaw surgery.
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Treacher Collins Franceschetti Syndrome (TCFS) is an autosomal dominant mandibulofacial dysostosis caused by a mutation in the Treacle gene. This genetic defect produces a variably penetrant genetic phenotype. These patients typically have "avian-like" faces with colobomata of the lower eyelids, antimongoloid slanting of the palpebral fissures, malar deficiencies, macrostomia, and auricular defects. Severely effected patients can present with inner ear deformities, mental retardation, orbital hypertelorism, maxillary retrusion, cleft palate, and a variety of other soft tissue abnormalities. Treacher Collins Syndrome patients also frequently have some degree of bilateral mandibular hypoplasia. This can range from microgenia to true micrognathia in both the vertical and horizontal dimensions. The mandibles of these individuals typically contain an enlarged antigonial notch that can be easily seen on a lateral cephalogram. The gonial angles are obtuse compared to normal age, sex, and race matched individuals. All of which creates an anterior open bite and gives the appearance of lower face retrusion.
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Nager's syndrome is another autosomal dominant disorder characterized by patients with faces similar to individuals with Treacher Collins syndrome. These patients also have "bird-like" features and their mandibles are typically retrognathic with an obtuse gonial angle and an enlarged antegonial notch. Their distinction lies in the fact that they have more severe auricular deformities than patients with TCFS . They also have characteristic upper and lower extremity defects in a post-axial distribution. The genetic mutation associated with this deformity is currently unknown.
The treatment of the mandibular malformations in these patients varies based on the degree of underlying pathology. Patients with minimal deformity require either no treatment or a sliding genioplasty, in order to normalize facial appearance. The majorities of patients however, have more severe forms of micrognathia and require some form of bone grafting or orthognathic surgery to generate an adequate repair. Bone grafting of the mandible with costochondral rib grafts has traditionally been performed in the prepubescent years in order to augment mandibular projection. Definite orthognathic surgery is typically delayed until the post pubescent growth has been completed in order to decrease the incidence of skeletal relapse secondary to predictable postsurgical skeletal change.
Recently, patients with severe bilateral micrognathias have been treated at our institution by mandibular distraction osteogenesis. Distraction offers the advantage over traditional mandibular reconstructive approaches. It allows for the induction of in situ neo-osteogenesis. This bony regenerate has a very low relapse rate and the minimal invasiveness of the operation allows the physician to operate on these children at an earlier age. This frequently permits decannulation of a tracheostomy when retrognathia is the cause of the airway obstruction. Moreover, it limits donor site morbidity that has previously accompanied placement of autologous bone grafts.
Dr. Stelnicki has also assembled a unique team of specialists who have developed a unique procedure for the treatment of very severe micrognathia in patients with TCS. This procedure involves the microvascular transfer of living tissue to the face in order to create a normal sized mandible. This technology has allowed TCS children with a tracheostomy to be decanulated at an early age; thereby permitting normal speech & eating for the first time in their lives. Dr. Stelnicki has performed several of these procedures now, and with great success.
Patients with Treacher Collins Syndrome and Nager's Syndrome frequently also require lower eyelid and ear reconstructions. Ear reconstruction can begin at age 7-10 and are usually completed in 2-3 stages. We recommend the use of autogenous rib cartilage to carve and craft the new ear. In our hands, this produces the most acceptable, long lasting result. However, in rare cases, we also consider placing an osteointegrated ear prothesis to create the external ear.
Prior to these reconstructions, all patients will receive a full evaluation by our team audiologist, otolaryngologist, and speech therapist. A full examination of the middle and inner ear structures will be performed in order to develop a plan that will optimize future speech development. In many cases, a bone conducting hearing aide can be created which will solve the majority of the hearing problems. In those which it cannot, further treatment alternatives will be discussed and customized to meet each individuals needs.
Lower eyelid reconstruction can begin as early as age six. Simple procedures such as medial or lateral canthopexies can be used to correct abnormal lid position in many cases. However, in patients with severe underlying cheekbone deficiencies, bone grafting to the cheek is required to give the lid a good foundation on which to rest, prior to moving any soft tissue. Without this support, the lids themselves would sink back down to their pre-surgical position. Lastly, when virtually no lower lid is present, a vascularized transfer of upper lid skin and muscle to the lower lid is indicated. Children with Nager's syndrome usually have a variety of arm and hand anomalies which accompany their facial features. A skilled hand surgeon is required in order to properly address these problems at an early age. Our team has certified hand specialist trained in the treatment of these problems. Under there direction, surgery and physical therapy can be initiated which will significantly improve both the form and function of the effected hands. This will allow each patient to maximize the use of each extremity.
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Craniofacial microsomia has several names. Some call it hemifacial microsomia, others call it Goldenhar's syndrome, while even others refer to it as first and second brachial arch syndrome. Regardless of its name, the syndrome is a combination of mandibular, cheekbone, ear, mouth, eye, and skull hypoplasia. In some cases, fatty deposits called epibulbar dermoids develop on the eyes, and there are associated neck bone abnormalities such as vertebral fusions.
In addition, when there is a significant facial bone hypoplasia, patients can develop airway obstruction called sleep apnea. Untreated, this can lead to poor weight gain, small stature, cerebral hypoxia, and even death. As a result, all patients with craniofacial microsomia seen at our institution with any symptoms of sleep apnea receive a full sleep evaluation by our otolaryngology staff. If evidence of sleep apnea is discovered, then appropriate steps are immediately taken to insure the child's safety and optimize future development.
Most patients with craniofacial microsomia have some degree of mandibular hypoplasia which is seen clinically as a deviated chin and an asymmetry in the position of the corners of the mouth. Patients with mandibular growth disturbances can present at any age. The challenge in treating many of these patients, lies in the variability of age and associated pathology of other facial structures such as the maxilla, the muscles of mastication, the zygoma, etc. All of these elements have a well-orchestrated interplay with one another, and therefore, the type of treatment chosen to address the individual deformity must be specific to the patient's needs.
The classification system of mandibular hypoplasia most frequently used is that of Pruzansky: Grade 1 mandibles are normal in configuration, but reduced in size. Grade 2 mandibles demonstrate hypoplasia plus maldevelopment of the associated condyle and coronoid processes. Kaban, et al later sub-classified the latter group as either 2a or 2b. Grade 2a mandibles have hypoplastic and malformed condyles, but the condylar head/glenoid fossa spatial relationship is spatially maintained in the sagittal dimension similar to that of the contralateral side. In these patients the misshapen condyle is functional and can be used in the mandibular reconstruction. Grade 2b mandibles have a severely hypoplastic and malformed condyle, which is displaced outside of the sagittal plane of the contralateral temporal mandibular joint (TMJ). These patients frequently have restricted TMJ function. Grade 3 mandibles are severely hypoplastic and lack a condyle, coronoid process and glenoid fossa. This classification system can be applied to patients with unilateral or bilateral mandibular hypoplasia. Any treatment plan constructed for these patients must factor in the age of the patient and the degree of skeletal hypoplasia in order to optimize long-term results. Most frequently Mandibular Distraction and bone grafting we work to reconstruct the mandible.
Mandibular & Maxillary Distraction Osteogenesis in the Treatment of Craniofacial Microsomia
In a typical mandibular distraction, anesthesia can be administered by either oral or nasal endotracheal intubation, but nasal intubation is preferred. The mandibular border is outlined on the skin surface with a surgical marker as a point of reference. Based on the patient's pathologic anatomy, the decision is made whether to use an intraoral or extra oral distraction device. Patients who require only unidirectional lengthening and have adequate mandibular bone stock are ideal candidates for intraoral distraction. Patients with severe mandibular deficiencies require distraction in multiple dimensions and are best treated with an extra oral device. In addition, patients who have previous external scars from other procedures are treated with an extra oral device. With an extra oral approach, care is taken not to damage soft tissue that may be needed for future surgeries, such as external ear remnants or microvascular soft tissue augmentation. Any incisions are placed in areas that can incorporate the distraction pins, so a second percutaneous pin site is unnecessary. An intraoral mucosal incision along the oblique line of the ramus is used for placement of both intra and extra oral devices.
In the initial clinical series, a supraperiosteal dissection of the mandible was recommended, following the Ilizarov tenant of minimal sub periosteal dissection. This maneuver is difficult to perform and has now proven unnecessary. Currently sub periosteal dissection is employed to elevate the entire lateral periosteal surface with a sharp-ended elevator. After the region of the osteotomy is exposed, the reciprocating saw is used to create lateral, anterior, and posterior corticotomies. The direction of the osteotomy is based solely on the bony pathology as well as the position of tooth follicles. The vector of the distraction is also a variable. Distraction can occur in the vertical, horizontal, or oblique vectors (based on the relationship of the vector to the long axis of the mandibular body). A vertical vector of distraction is preferred for lengthening a deficient ramus in a vertical dimension or for transporting the condyle up into the glenoid fossa. The horizontal vector along the long axis of the mandible is chosen in order to lengthen the mandible in a purely horizontal plane, as in bilateral micrognathias whose deficiency is predominately in the mandibular body. If an oblique vector (a direction between the vertical and horizontal vectors) is chosen, the osteotomy is placed anterior to the coronoid in order to prevent impingement of the coronoid on the zygomaticomaxillary buttress during distraction. An oblique distraction vector not only lengthens, but also vertically elongates the mandible.
Before converting the corticotomy into an osteotomy, the pins are placed. If the intraoral device is used, a single percutaneous stab incision is made for the placement of the screwdriver. For the extra oral device, a two-holed trocar is used for percutaneous placement of the posterior pins. The second anterior pair of pins is placed so that the skin between the two pin sites is compressed, thereby reducing the amount of tension on the wound and the length of the scar. The device is attached to the pins. A 3 mm osteotome completes the medial wall osteotomy, liberating the mandibular segments for distraction. The wounds are closed in layers with absorbable sutures. A careful cleaning regimen is followed whereby the pin tracks are cleansed four times a day and, as needed for any blood or serous discharge with a dilute hydrogen peroxide solution. After a delay of 5 to 7 days (termed the latency period), distraction commences at a rate of 0.5 mm twice a day (termed the activation phase). This rate is continued until the mandibular length is overcorrected by several millimeters. During distraction, the vertical or oblique vector will typically become more horizontal, due to the counterclockwise pull of the muscles of mastication. At this time orthodontic intermaxillary elastics may be used to mold the regenerating bone and optimize the occlusion (termed molding the regenerate). The device is left in place to serve as an external fixator for 8 or more weeks, until there is radiographic evidence of mineralization. This stage is known as the consolidation phase.
In patients with unilateral craniofacial microsomia undergoing distraction, it is important that a dental impression be taken and a bite block placed in the surgically created posterior open bite when the device is removed. This will allow the orthodontist to level the maxillary occlusal plane by allowing for eruption of the ipsilateral maxillary dentoalveolar complex. Distraction will also affect the entire facial milieu: the soft tissue envelope bulk will increase due to a combination of soft tissue expansion and muscle hypertrophy and leveling of the oral commissure are usually noted.
Age is also a factor in developing a treatment plan. Under 2 years of age, mandibular distraction is not usually performed unless there is airway compromise. Soft tissue treatments such as cleft closure or preauricular skin tag removal are initiated. Cranial vault remodeling procedures are also performed at this age. Yet, mandibular surgery is avoided for several reasons. First, it is difficult to identify tooth buds at this age, and therefore permanent dental injury is a likely occurrence. Secondly, distraction at this age can be a daunting experience for the patient and the parents. The exception to this would be when early mandibular distraction is used to prevent tracheotomy in a newborn with micrognathia that is causing severe airway obstruction. We and others have successfully applied a modified distraction device to the infant pediatric mandible that distracts the bone at an increased rate of 2 mm/day and a decreased latency of 3 days. This relieves the airway obstruction within 10-14 days post-operatively, evading the need for early tracheostomy.
From ages 2-6 years mandibular distraction osteogenesis can be comfortably considered. Children with mild deformities, such as Pruzansky Type I mandibles and a horizontal occlusal plane should not be considered for distraction. However, orthodontic therapy can be initiated during this age period to maintain a level occlusal plane and prepare for the eventual osteotomies that will be required, using standard orthognathic techniques.
When a child presents with a more severe Pruzansky Type 1 or any Pruzansky Type 2 deformity, with associated sleep apnea (with or without a tracheostomy), distraction is initiated. It has been demonstrated that distraction will not only successfully expand the mandibular skeletal volume in all dimensions, but also positively augment the surrounding soft tissues and muscles of mastication. This is particularly important for patients with craniofacial microsomia who have a significant degree of soft tissue underdevelopment, in addition to a lack of mandibular growth. Distraction has the advantage over other techniques in that it requires minimal operative time, carries little risk, minimizes hospitalization time, obviates the need for blood transfusion, bone graft and intermaxillary fixation, and has minimal relapse rates. More importantly, decannulation of the tracheostomy is frequently feasible post-distraction. Parents should be warned that, if distraction occurs during this age interval, it is likely that a secondary distraction will be required following post-pubertal facial growth. It is unlikely that mandibular development will keep up with the growth of the remainder of the facial skeleton.
Children with Pruzansky Type 3 mandibular deformities (absent ramus, condyle, and/or glenoid fossa), are initially treated with an autogenous costochondral rib graft reconstruction at approximately 3-4 years of age (first stage). The costochondral graft will increase mandibular length, reconstruct the condyle, and form a pseudoarthrosis with the glenoid fossa. When the glenoid fossa is absent, a new one is constructed with rib grafts fixated to the zygomatic arch. In a second stage, at least 6 months after removal of the fixation, distraction of the rib graft can be performed. In rare cases, microvascular free tissue transfer is offered to create absent parts of the mandible. From age 6 to the teen years, during the period of mixed dentition, orthodontic treatment is needed to promote growth of the affected dentoalveolus and to aid in the proper eruption of the permanent teeth. Distraction would be considered during this time only if the patient had sleep apnea or never received any prior surgical treatment. Additionally, distraction could be performed if a patient has a significant growth deficiency in the mandible, after rib grafting.
Mandibular distraction during the teenage years should be postponed until the patient has reached skeletal maturity. In girls, this typically occurs at approximately age 15 and in boys at age 17. A wrist film is taken prior to any jaw surgery to confirm closure of the growth plates, signifying the end of endochondral bone development.
Indications for surgery in the teen years include: 1) residual post surgical skeletal deficiency due to surgical relapse or abnormal growth, 2) unsatisfactory bone contour, 3) malocclusion, or 4) absence of previous treatment. Any appropriately chosen maxillofacial surgical procedure could be performed during this time ranging from sagittal split osteotomies, to bone grafting, to distraction. In patients with minimal mandibular deformities, classic orthognathic procedures are indicated. Mandibular distraction should be considered in patients with moderate to severe skeletal deficiency, or bilateral disease, in where pressure from the soft tissues would significantly increase the risk of postoperative graft resorption or skeletal relapse.
Restricted mandibular growth is frequently associated with abnormal maxillary development. The ipsilateral maxilla and dentoalveolar processes are often deficient in the vertical dimension. In mild cases this can be treated with a bite block and orthodontic therapy as described above; however, in more severe circumstances a maxillary (Le Fort I) leveling procedure may be considered. Traditionally, this has involved a Le Fort I osteotomy followed by ipsilateral lengthening of the mandible with bone grafts and a contralateral impaction.
The deficient maxilla can be distracted in conjunction with the mandible. In this technique, a Le Fort 1 corticotomy is made at the time of the mandibular osteotomy and placement of the distraction device. The upper and lower jaws are wired into intermaxillary fixation. After a 5-day latency period, distraction is commenced at the rate of 1 mm/day. At the conclusion of maxillary/mandibular distraction, the device is left in place for 8 weeks to allow for bone consolidation. Using this technique, we have had excellent soft tissue and bony results with complete leveling of the dental occlusion. Intermaxillary fixation is not employed during the latency period, instead heavy guiding elastics are placed at the time of distraction. The bands are modified throughout the process to obtain optimal dental alignment.
Maxillary deficiency is also addressed in a similar manner. We use KLS Martin, the Red external Maxillary distraction & internal distraction devices to correct Maxillary hypoplasia. These surgeries correct both the aesthetic appearance of the child and function problems such as sleep apnea.
Following the correction of the skeletal abnormalities, we will perform microsurgery to normalize the soft tissues in the face as well. Based on techniques developed by Ian Taylor in Australia and the staff at New York University, we can now significantly improve facial asymmetry by taking tissue from the back and moving it to the face. This augments the lack of soft tissue in the area. With these methods spectacular end results can be achieved for patients with craniofacial microsomia, Treacher Collins syndrome, Nager's syndrome, and Romberg's Hemifacial atrophy. This will be discussed further in the section on Romberg's disease.
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Fibrous dysplasia is a benign tumor of the skeleton that can have devastating local effects. The tumor can be mono-ostotic (in one place) or poly ostotic ( in multiple places). In our practice we deal with fibrous dysplasia that effects the craniofacial skeleton.
(insert photo of skull with FD) Fibrous dysplasia can occur at any age, but typically is noticed during mid to late childhood. The most common area of the craniofacial skeleton to be affected is the orbit. However, lesions can develop in the skull, maxilla, mandible, zygoma, dental alveolus, or any other bone.
The tumor usually presents as a painless, firm mass or swelling. The mass is fixed and non-mobile. There is usually a slow growth process associated with these lesions. Families notice a swelling or asymmetry at was not there in pictures the year before, but now is more apparent.
(insert photo of patient) The diagnosis of Fibrous dysplasia is typically made via a CT Scan radiographic assessment of the lesion. The lesion erodes into the bone and give the bone a fibrotic type appearance. The bone lesion then expands beyond the normal boundaries of the bone and pushes out into the surrounding tissue. A biopsy in the operating room confirms the diagnosis of the lesion, but the lesion has such a typical appearance of CT scan that most places can make the diagnosis accurately without the biopsy. These lesions rarely convert to a cancerous or malignant tumor. However, they can cause significant problems due to local bone destruction and mass effect. The most dangerous complication of fibrous dysplasia is blindness. Blindness is caused by a growth of the tumor along the lateral orbit and sphenoid bone. As the tumor grows it can extend into the optic canal and compress the optic nerve, which is nerve responsible for vision. This risk must be carefully monitored by an ophthalmologist if the tumor is in this area. These lesion can also severely distort the face, compress sensory nerves in the face giving numbness or pain, or cause teeth to be lost due to involvement in the dental roots. Treatment of Fibrous dysplasia is primarily surgical. There are various chemotherapy drugs that effect osteoclast function that have been tried, but outcomes of drug therapy are mixed at best. Surgery can come in many forms, depending on the problem. Also, the timing of surgery can vary depending risk to vision, symptoms, and patient desire. If the tumor involves the optic canal and is putting the vision at risk, then surgical resection of the tumor (either total or subtotal resection) with bone graft reconstruction is required. This is done in combination with a neurosurgeon who is there protecting the brain and the optic nerve. If the lesion involves other bones and is relatively small, complete resection and reconstruction with bone or alloplastic implant can be warranted. Many time a CT scan is used to make a 3D model. From this model, the area of bone to be removed is outlined. Once the defect is created, a computer generated implant is then created to reconstruct the defect. Alternatively, in large lesions or in lesions that involve important structures such as the teeth, the fibrous dysplasia can be treatment with serial debridement rather than resection. This treatment is chosen when there is little to no risk of malignancy and the damage to the patient caused by a complete resection would be severe. Debridement and sub-total resection controls the growth of the tumor and restored facial form with little risk to many vital structures. (insert photo of tumor and implant) When the teeth are effected by this tumor, Dr. Stelnicki will work closely with Dr. Portnof (oral surgery) to outline a proper treatment plan. Debridement can be an option, however Virtual Surgical Planning (VSP) is also frequently employed to treat this disorder. VSP allows the Doctors to take a CT scan and convert it into a 3 D model for the purpose of surgical planning. With this model, the exact areas of the tumor to be removed can be outlined to the 1 mm. Then, surgical reconstruction with bone or a computer generated implant can be optimized. This allows for precise reconstruction of the anatomy destroyed by the tumor and preservation of structures such as important nerves and blood vessels in the face. If teeth must be removed or are lost due to the tumor growth, Dr. Portnof will work with the patient following surgery to replace the teeth with dental implants when this is appropriate. This treatment can restore an normal smile and provides strong teeth for eating. (pre and post op patient photo) Ask our office which type of treatment would be best for you and your family.
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The cause of Romberg's hemifacial atrophy is unknown. It is a self-limiting disorder associated with collagen vascular diseases such as Sjogren's and Scleroderma. The cause is thought to be autoimmune, however other etiologies cannot be ruled out. It produces a characteristic loss in the soft tissues below the skin, usually on one side of the face. The classic description is that of the "coup de sabre" deformity or what looks like a sabre cut across the center of the face. All of which is indicative of the underlying soft tissue loss. In some severe cases, the underlying bone is also affected.
Our policy is to treat this disorder with a microvascular free flap that restores lost soft tissue bulk once the disease has burned itself out and has stopped progressing. The microvascular tissue for transfer is usually taken from the back, in the region of the shoulder blade (scapula). This tissue can be contoured to fit almost any defect. Our team has world's experts in microvascular free tissue transfers to help with this repair. The transfer is performed in a single stage through a cosmetically acceptable facelift incision. Then, over the next years a few small touch up surgeries are performed to attempt to tailor the shape of the graft to each patient.
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