Showing papers in "Journal of Craniofacial Surgery in 2005"

International anthropometric study of facial morphology in various ethnic groups/races.

Abstract: When anthropometric methods were introduced into clinical practice to quantify changes in the craniofacial framework, features distinguishing various races/ethnic groups were discovered. To treat congenital or post-traumatic facial disfigurements in members of these groups successfully, surgeons require access to craniofacial databases based on accurate anthropometric measurements. Normative data of facial measurements are indispensable to precise determination of the degree of deviations from the normal. The set of anthropometric measurements of the face in the population studied was gathered by an international team of scientists. Investigators in the country of the given ethnic group, experienced and/or specially trained in anthropometric methods, carried out the measurements. The normal range in each resultant database was then established, providing valuable information about major facial characteristics. Comparison of the ethnic groups' databases with the established norms of the North America whites (NAW) offered the most suitable way to select a method for successful treatment. The study group consisted of 1470 healthy subjects (18 to 30 years), 750 males and 720 females. The largest group (780 subjects, 53.1%) came from Europe, all of them Caucasians. Three were drawn from the Middle-East (180 subjects, 12.2%), five from Asia (300 subjects, 20.4%) and four from peoples of African origin (210 subjects, 14.3%). Their morphological characteristics were determined by 14 anthropometric measurements, 10 of them used already by classic facial artists, Leonardo da Vinci and Albrecht Durer, complemented by four measurements from the nasal, labio-oral and ear regions. In the regions with single measurements, identical values to NAW in forehead height, mouth width, and ear height were found in 99.7% in both sexes, while in those with multiple measurements, vertical measurements revealed a higher frequency of identical values than horizontal ones. The orbital regions exhibited the greatest variations in identical and contrasting measurements in comparison to NAW. Nose heights and widths contrasted sharply: in relation to NAW the nose was very or extremely significantly wide in both sexes of Asian and Black ethnic groups. Among Caucasians, nose height significantly differed from NAW in three ethnic groups, with one shorter and two greater. In the Middle Eastern groups nose width was identical to those of NAW but the height was significantly greater. The present study, conducted by investigators working separately across the world and with small samples of the population, is clearly preliminary in nature and extent. Yet it may fulfill its mission if medical and anthropological investigators continue the work of establishing normative data of the face. These data are urgently needed by medical professionals but have been lacking up till now in western and northern Europe, Asia, and Africa.

Platelet rich plasma: biology and new technology.

Abstract: Platelets play a central role in hemostasis and wound healing. The latter is mediated by release of secretory proteins on platelet activation, which directly or indirectly influences virtually all aspects of the wound healing cascade. Studies in basic science have shown a dose-response relationship between the platelet concentration and levels of secretory proteins, as well as between platelet concentration and certain proliferative events of significance to the healing wound. Technologies to provide autologous platelet rich plasma to the repair site are now being used in a wide variety of clinical applications, with the majority of such studies suggesting a role in the surgeon's armamentarium. Little standardization in the field exists, which has made it difficult to fully evaluate the literature on the subject and unequivocally establish applications for which the technology truly has merit. This article presents fundamental background on platelet biology and the role of platelets in both hemostasis and wound healing, as well as methods of preparing, characterizing, and using platelet rich plasma, to provide the reader a foundation on which to critically evaluate prior studies and plan future work.

Allograft and alloplastic bone substitutes: a review of science and technology for the craniomaxillofacial surgeon.

Abstract: Bone healing is a complex and multifactorial process. As such, there are numerous steps in the process to which intervention can be directed. This has given rise to many bone graft technologies that have been used to regenerate bone, creating, perhaps, a bewildering array of options. The options that surgeons have the most familiarity with are the ones that have been available the longest (i.e., autograft and allograft). Although useful for the widest spectrum of clinical applications, limitations of these grafts has prompted the development of new materials. Demineralized bone matrix formulations and synthetic ceramic materials are now being used with greater frequency. These biomaterials have demonstrated their usefulness in facial plastic and reconstructive surgery with their ability to augment and replace portions of the craniofacial skeleton. The purpose of this article is to describe and discuss the allograft and alloplastic bone grafting technologies so that the reader can consider each in the context of the others and gain a better appreciation for how each fits into the universe of existing and emerging treatments for bone regeneration.

Clinical outcome of 285 Medpor grafts used for craniofacial reconstruction.

Abstract: Porous polyethylene (Medpor) is an alloplastic material worldwide used for craniofacial reconstruction To evaluate complications and risk factors associated with this synthetic graft, a retrospective study was performed A series of 285 Medpor grafts were placed in 187 patients Age, sex, diagnosis at admission, site, type of surgical insertion, type of fixation, and outcome (no complications, anesthesia, exposure, infection, and implant remodeling and removal) are considered By means of univariate and multivariate analyses, we detect variables most associated with poor outcome Univariate analysis showed that graft "survival" curves stratified according to (1) diagnosis at admission and (2) site are statistically significant Subsequently, a Cox analysis was performed: both variables are also predictors of graft outcome Porous polyethylene is a reliable alloplastic material that can be satisfactory used for craniofacial reconstruction However, some sites (ie, nose, maxilla, and ear) and diagnosis at admission (ie, syndromic patients previously operated) are related to an higher risk of implant failure

Repair of alveolar clefts with recombinant human bone morphogenetic protein (rhBMP-2) in patients with clefts.

Abstract: This article demonstrates the feasibility of using recombinant human bone morphogenetic protein (rhBMP-2) as a substitute for autogenous iliac crest bone for repair of congenital facial clefts in humans. In this series, 50 cleft sites were repaired in 43 patients using rhBMP-2 without the use of autogenous graft tissue. Successful osseous union was achieved in 49 of the 50 sites. In one patient, the graft failed to consolidate. Severe clefts were managed by combining distraction osteogenesis and rhBMP-2. Eliminating the need to harvest autogenous iliac crest bone resulted in substantial decrease in morbidity. The constructed alveolus performed clinically as normal bone and responded to natural tooth eruption and orthodontic movement. Histology of the tissue constructed showed normal, vital bone. Although additional investigation is warranted to determine the optimum protocol for the use of this material in alveolar cleft repair, the technique should be considered as a viable treatment option in cases in which avoiding iliac crest harvesting is desirable.

Evaluation of the Adjunctive Benefits of Platelet-Rich Plasma in Subantral Sinus Augmentation

Abstract: :Subantral sinus augmentation is often necessary to permit placement of endosseous implants. Recent efforts to improve wound healing have focused on autogenous sources of bioactive mediators, such as platelet-rich plasma (PRP), which offer the potential to enhance the biological activity of

Vulnerability of the inferior alveolar nerve and mental nerve during genioplasty: an anatomic study.

Abstract: Microgenia or "small chin" is corrected by various techniques, such as insertion of an alloplastic implant, cartilage or bone grafting, or horizontal advancement osteotomy. Horizontal recession osteotomy is used in macrogenia. Particularly in a microgenic mandible, the mental foramen is unexpectedly nearer to the inferior border of the body. During sliding horizontal osteotomy of the mentum, the inferior alveolar nerve (IAN) and mental nerve are vulnerable to an injury. Thirty fresh hemimandibles were used for a study of the IAN. The IAN course was traced by serial sections at intervals of 5 mm. In 50 dry specimens the direction of the mandibular canal was evaluated by the photographs with a stick put into the mental foramen. The IAN in mandibular canal runs above the lower one-third of the mandibular body. The terminal mandibular canal locates at an average of 4.5 mm under the mental foramen, advances 5.0 mm anteriorly, loops, and ends at the foramen. The direction of the mandibular canal at the mental foramen was 39.4 degrees lateral, 67.2 degrees superior, and 80.2 degrees posterior. It is advisable for surgeons to keep the level of sliding osteotomy of the mentum at least 4.5 mm below the mental foramen to spare the IAN.

Virtual planning of composite mandibular reconstruction with free fibula bone graft.

Abstract: Functional mandibular reconstruction after tumor resection is challenging. Currently, voxel-based craniofacial surgery and virtual planning of craniofacial surgical procedures are becoming increasingly popular. We report on a 56-year-old patient with an infiltrating recurrent squamous cell carcinoma of the mandible and buccal mucosa. Virtual resection of the mandible was accomplished using virtual reality techniques. Based on virtual geometric data, a metal template was configured for optimal contouration of a fibular bone graft while it was still pedicled in the donor field. Our operative procedure demonstrates the usefulness of voxel-based three-dimensional cephalometry in virtual planning of microsurgical bone transfer for mandibular reconstruction.

Producing accurate platelet counts for platelet rich plasma: validation of a hematology analyzer and preparation techniques for counting.

Abstract: Platelet rich plasma (PRP) has been shown to clinically accelerate healing of both soft and hard tissues. As a result, it has gained increasing popularity. However, the clinical effectiveness of each type of PRP preparation method can vary in technique and efficiency, and current methods to evaluate the platelet concentration efficiency of PRP systems have several limitations. Therefore, the purpose of this study was to validate an automated hematology analyzer, the Cell-Dyn 3700, to accurately count platelets in concentration ranges of approximately 2,000,000-4,800,000 platelets/microL. PRP platelets were counted by way of a manual counting method and on the Cell-Dyn 3700, and the statistical evaluation indicated no difference between the groups (P > 0.05). Dilution of the PRP was not required, and accurate platelet counts could be achieved up to platelet concentrations of 4,800,000 platelets/microL. PRPs must be resuspended on a rocker for at least 5 minutes before platelet counts, and the entire PRP sample must be resuspended to allow for equal distribution of platelets before counting. With use of the validated Cell-Dyn 3700, a platelet concentrate system was used to prepare 153 PRPs. The baseline whole blood platelet concentration (328,000 platelets/microL +/- 69,000 platelets/microL) and the average PRP samples (2,645,000 platelets/microL +/- 680,000 platelets/microL) were compared, resulting in an eightfold increase in concentration and an average platelet percent recovery of approximately 76%. Automated hematology analyzers can be used to accurately count platelets in PRP given the system has been validated appropriately and the PRP samples are prepared properly to provide adequate platelet suspension.

Three-dimensional osteotomy planning in maxillofacial surgery including soft tissue prediction.

Abstract: Preoperative planning of complex osteotomies in craniomaxillofacial surgery, in conjunction with a surgeon's expertise, is essential for achieving an optimal result. However, the soft tissue changes that accompany facial bone movements cannot yet be accurately predicted. Bony tissue, because of its

Apert and Crouzon Syndromes: Clinical Findings, Genes and Extracellular Matrix

Abstract: Apert and Crouzon syndromes are well known craniostenosis. In the last 10 years several studies were performed to provide a better understanding of the etiology and pathogenesis of these diseases. Both have an autosomal dominant mode of transmission, and a mutation in the gene encoding for the fibroblast growth factor receptor 2 (FGFR2) is the cause in most patients. However, the fact that the same mutation can produce a wide range of phenotypic expression makes the mechanism of anomalous development more complex. The extracellular matrix (ECM) is composed of proteins, glycosaminoglycans, and cytokines that are secreted in an autocrine and paracrine manner and are able to modify the ECM. Fibroblast growth factors are complexed with heparan sulfate, a component of the ECM, before binding the FGFR2. Data exist about different expressions of cytokines and ECM macromolecule in craniostenosis-derived fibroblasts and osteoblasts. Changes in ECM composition could explain the altered osteogenic process and account for pathologic variations in cranial development in addition to the FGFR2 mutations.

Three-dimensional surface imaging: limitations and considerations from the anthropometric perspective.

Abstract: The use of three-dimensional (3D) surface imaging for clinical measurement purposes has increased considerably as the technology has become more affordable. Like any measurement technique, 3D surface-based anthropometry is subject to a number of limitations and methodological caveats and thus should not be applied without sufficient consideration of its potential strengths and weaknesses. In this brief report, the authors consider 3D surface imaging from the anthropometric perspective, specifically focusing on issues related to the capture of reliable quantitative information from the head and face.

Retrospective study of 18 patients treated by maxillomandibular advancement with adjunctive procedures for obstructive sleep apnea syndrome.

Abstract: A surgical protocol in the treatment of obstructive sleep apnea syndrome (OSAS) is presented. Eighteen patients with particular craniofacial characteristics consisting in maxillomandibular retroposition or retromandibulism underwent bimaxillary advancement by way of Lefort I and bilateral sagittal r

Orbital floor restoration.

Abstract: Orbital blow-out fractures reconstruction aims to restore the continuity of the orbital floor, to provide support of orbital contents and prevent soft tissues' fibrosis. Different materials have been tested over the years to reach this purpose. Traditionally, autogenous grafts have been used as the material of choice; in recent years alloplastic materials have gained popularity because of their availability and ease of use. The purpose of this study was to review materials used in orbital floor reconstructive surgery at the Department of Maxillo-Facial Surgery of University of Rome "La Sapienza", with emphasis on their biocompatibility, their shaping features, and mechanical properties. This report presents the results obtained by the application of these products on 379 patients who underwent surgical treatment for blow-out fractures from 1995 to 2003: the diagnosis of fracture of the orbital floor was based on clinical symptoms and CT axial scanning through coronal reconstruction. Follow-up period spanned from 1 to 8 years.

Changes in pharyngeal airway space and hyoid bone positions after body ostectomies and sagittal split ramus osteotomies.

Abstract: UNLABELLED Although mandibular setback might lead to possible pharyngeal narrowing, the development of obstructive sleep-related breathing disorders may occur because of multiple factors. In this study, the changes in the pharyngeal airway space (PAS) and the hyoid bone position have been evaluated before and after surgery in patients who underwent body ostectomy (BO) and sagittal split ramus osteotomy (SSRO). In addition, the effects of the BO on PAS and on hyoid bone position have been compared with the effects of the SSRO. MATERIAL AND METHODS Thirty patients with mandibular prognathism underwent mandibular setback osteotomy. The patients were divided into two groups: BO group, 15 patients (5 men, 10 women) who underwent BO; and SSRO group, 15 patients (11 men, 4 women) who underwent SSRO. Changes in PAS and hyoid bone position were analyzed throughout the preoperative and early and long-term postoperative periods via lateral cephalometric radiographs. RESULTS A decrease has been observed in the PAS area and the anteroposterior dimension of the PAS detected in all patients in the long-term period. Hyoid bone revealed backward and downward movement during the early postoperative period; however, it showed a tendency to return to its original anatomic position during the long-term postoperative period. None of the patients had disturbances in respiration. CONCLUSION After BO and SSRO, a decrease in PAS area was observed, and this decrease also continued in the long-term period. Our results revealed a decrease in PAS area after BO, but the decrease was less than that in cases having SSRO.

Biomechanical testing of alloplastic PMMA cranioplasty materials.

Abstract: Alloplastic cranioplasty techniques using polyfmethylmethacrylate (PMMA) are a common method of restoring lost cranial bone Numerous compositions of PMMA exist for this application, using either intraoperatively cured solid compositions or preoperatively fabricated porous custom implants Although all of these PMMA materials feel solid by palpation, the amount of cranial protection that they provide has never been precisely evaluated Whether differences exist between the differing PMMA cranioplasty materials likewise is unknown An impact resistance test was performed on uniform-size blocks of differing PMMA materials of representative cranial thickness using American Society of Testing and Materials (ASTM) method D 3029-78 Based on mean failure weights of 39 lb (Cranioplast), 42 lb (Cranioplexx), and 40 lb (HTR polymer), all of these materials are comparatively the same in impact resistance Precured porous PMMA, despite having 30% to 40% less material, offered no less impact resistance Based on projections to cranial bone, PMMA materials appear to offer protection similar to that of native osseous tissue

Management of close-range, high-energy shotgun and rifle wounds to the face.

Abstract: Close-range, high-energy shotgun wounds of the face are life-threatening and devastating traumas of the face. Suicidal attempts are the main reason in the great majority of the patients in civilian life. There is no consensus on the timing of reconstruction for bone and soft tissue defects resulting from high-energy shotgun wounds. The conventional method is primary repair as soon as possible and serial debridements and definitive reconstruction in the delayed stage. An alternative to this approach is the immediate definitive surgical reconstruction of the patient during the first operation for acute management of trauma. We had 15 patients with close-range, high-energy shotgun wounds in 10 years. Six of 15 patients referred to our center for definitive reconstruction after the acute management of the patients were performed in another center and the rest were all admitted in the acute period. Either conventional approach with delayed reconstruction for 10 patients or immediate definitive surgical reconstruction for 5 patients was used. Immediate reconstruction eliminated disadvantages of the conventional method such as high infection and scarring rate and deformities resulting from contraction of tissues. The emotional conditions of the patients were evaluated and major depression signs were determined. Functional evaluation showed that there was great correlation between facial appearance after reconstruction and social activity level.

Hydroxyapatite cement in pediatric craniofacial reconstruction.

Abstract: The gold standard for closing small cranial defects is autogenous bone: iliac crest, rib, or split calvarial grafts. Autogenous grafts result in donor site morbidity, increased operative time, blood loss, and additional cost, and they are limited in quantity. Hydroxyapatite cements are alternative bone substitutes that eliminate these restrictions. Although the use of hydroxyapatite is well accepted in completely developed crania, its use in the growing pediatric skull is limited. The purpose of the current study was to address the safety and long-term efficacy of this bone substitute for the repair of craniofacial bone defects in the growing pediatric skull. Safety is measured dually by resistance of bone substitute to infection and its tolerability by lack of a foreign body reaction. Efficacy is evaluated as structural skull integrity, volume stability with time, aesthetic quality, and most importantly, the ability to integrate adequately into a growing cranium without subsequent deformity or complications. This is a retrospective review of all pediatric patients at the authors' institution who underwent reconstruction of cranial defects using hydroxyapatite cement between May 1997 and March 2001. Eight patients who underwent 11 operations between the ages of 25 and 100 months (mean, 55 months) were followed up between 23 and 72 months (mean, 38 months). No mortalities or significant morbidities were encountered in the study population. It has been the authors' experience that hydroxyapatite cement is both biocompatible and resistant to infection when used in sites not contiguous with sinus mucosa; and that it is a good alternative to autogenous bone in pediatric craniofacial reconstruction.

The anatomy of temporal hollowing: the superficial temporal fat pad.

Abstract: A coronal incision provides exposure to the lateral craniofacial skeleton for plastic surgeons, oral-maxillofacial surgeons, head and neck surgeons, neurosurgeons, and cosmetic surgeons. A common complication of this approach is hollowing of the temporal fossa. This hollowing results in a significant cosmetic deformity that affects the patient physically and psychologically. Current theories suggest that hollowing may result from atrophy of the superficial temporal fat pad caused by ischemia, displacement, or denervation of the fat pad. The purpose of this study is to identify the neurovascular supply and the supporting structures of the superficial temporal fat pad. Eight fresh-frozen cadaver heads were injected with latex to facilitate identification of vessels. Through coronal incisions, the anatomy of the vessels, nerves, and fascial network within the superficial temporal fat pads were recorded. The vascular supply of the superficial temporal fat pad includes branching perforators from the deep and middle temporal arteries that traverse through the substance of the fat pad. The branches of the zygomaticotemporal nerve travel through the superficial temporal fat pad. There is a network of septations that suspends the superficial temporal fat pad to the anterior fascia. This is the first step to understanding the etiology of postoperative temporal hollowing. This study provides the basis for a prospective randomized clinical trial investigating temporal hollowing after different surgical exposures of the area.

Long-term outcomes of ultra-thin porous polyethylene implants used for reconstruction of orbital floor defects.

Abstract: PURPOSE The objective of this article is to present the long-term outcomes of ultra-thin polyethylene implants used for orbital floor reconstruction in facial trauma patients. MATERIALS AND METHODS From 1998 to 2004, 38 patients underwent orbital floor reconstruction with porous polyethylene implants with a mean follow-up of 4 years. A subciliary incision and preexisting facial wounds or scars were used. The boundaries of the maxillofacial injury and the orbital volumes of both orbits were assessed by computed tomography images obtained pre- and postoperatively. In all patients, ultra-thin porous polyethylene implants in various sizes were used to reconstruct the orbital floor defect. RESULTS None of the patients needed removal of the implants during the follow-up. The volume increase of the traumatized orbits ranged from 0.04 to 6.18 (average 3.12 +/- 1.48) mL compared with the intact orbit (P 0.01). Postoperative ectropion in three cases was corrected under local anesthesia. Persistence of complications were as follows: enophthalmos, 3 in 28; diplopia, 1 in 16; dystopia, 1 in 4; and infraorbital nerve hypoesthesia, 3 in 31. One patient underwent late enucleation of the globe because of initial penetrating trauma. CONCLUSIONS We recommend the use of ultra-thin porous polyethylene implants in the reconstruction of the orbital floor defects in facial trauma patients. The implants are durable in the long-term and mimic the anatomy of the thin orbital floor and avoid the morbidity of autogenous bone grafts.

Ethmoido-orbital tumors: our experience.

Abstract: No data in the literature report the specific invasion of the orbit from ethmoidal tumors, although such a pattern of involvement of the orbit frequently occurs because of the thin lamina papyracea separating the two structures. From January 1986 to January 2003, 38 patients with untreated primary ethmoidal malignancies were observed at the Unit of Maxillo-Facial Surgery of the University "La Sapienza" in Rome. Personal data were obtained from review of the personal clinical file of each. Orbital invasion was present in 24 patients with ethmoidal malignancy. Three stages of orbital invasion were identified. The average overall survival, with the Kaplan-Meyer method, was 61.4% after 1 year and 51.1% at 5 and 10 years. Intracranial involvement is the main element for short-term negative prognosis. Orbital exenteration is fundamental if grade III orbital invasion occurs because it ensures an improvement of the disease-free survival.

Torticollis associated with positional plagiocephaly: a growing epidemic.

Abstract: The primary study aim was to identify characteristics of torticollis associated with plagiocephaly without synostosis (PWS) and to differentiate this from other forms of torticollis. The three commonest causes of infantile torticollis are idiopathic muscular causes such as sternocleidomastoid fibrosis, structural anomalies in the cervical vertebrae, and neurologic or ocular causes, such as certain types of strabismus. Recently, several units have reported increasing numbers of babies presenting with head tilt and reduced range of cervical motion, a form of torticollis apparently associated with PWS and unrelated to the etiologies enumerated above. A secondary aim was to identify local trends in referral patterns over the preceding 3 years. This was a retrospective review of 159 children attending a secondary referral center for management of positional plagiocephaly or torticollis. The data was collected in a university-affiliated child development clinic, and the records of one of three similar, community-based outpatient clinics, staffed by physical therapists and working in conjunction with our tertiary craniofacial surgery center were analyzed by medical staff. There was a persistent escalation in referrals for both PWS and associated torticollis (ie, not attributable to 1 of the 3 causes mentioned) over the study period. Anatomic and clinical features of PWS-associated torticollis were characterised and contrasted with the classic forms of torticollis. Current treatment strategies are outlined and discussed. Torticollis can predispose to PWS, but in a large proportion of our cases of PWS, torticollis appears to develop secondary to plagiocephaly, and the number of presentations is escalating.

Le Fort III advancement osteotomy in the growing child affected by Crouzon's and Apert's syndromes: presurgical and postsurgical growth.

Abstract: This is a longitudinal cephalometric study of patients with craniofacial synostosis (Crouzon's and Apert's) syndromes who underwent Le Fort III advancement during early childhood. The objectives of the study were to quantify presurgical growth, surgical results, and surgical stability as well as long-term postsurgical growth. Of the 31 patients operated on during their growth period (less than 10 years of age), 17 had sufficient records to be included in the study: at infancy, before the Le Fort III osteotomy (average age=7.3 years, range: 4.8-10 years), and immediately after surgery. Ten of the patients were followed radiographically long term for an average of 6.1+/-2.7 years (range: 4.5-10.8 years). The presurgical abnormal midfacial vertical growth pattern was accurately described with a progressive class III discrepancy and an increasing exophthalmos. Remarkable postoperative stability of the maxillary segment was observed. After surgery, growth of the midface was measured, together with minimal, if any, anterior growth, similar to the presurgical growth pattern and rate. No actual detrimental or beneficial effect of surgery on subsequent growth was seen. This study quantitatively confirms that the standard Le Fort III osteotomy is a stable and effective but generally not definitive procedure in childhood. This study might also serve as a control sample to compare with groups of patients undergoing distraction osteogenesis to verify the actual advantages and shortcomings of this alternative technique.

Congenital mandibular hypoplasia: analysis and classification.

Abstract: Mandibular hypoplasia is a frequently encountered craniofacial difference and can be classified into three groups: congenital, developmental, and acquired. The focus of this article is on the congenital group, the majority of which is associated with syndromes. There have been numerous publications on patients with syndromic congenital mandibular hypoplasia; however, there has been no investigation and differentiation of the "nonsyndromic" patients. The purpose of this study was to analyze this subgroup of patients with nonsyndromic congenital mandibular hypoplasia to determine incidence, clinical presentation, and treatment.A retrospective analysis of all children treated for congenital mandibular hypoplasia at the Children's Hospital of Philadelphia between 1975 and 2003 was performed. Two hundred sixty-six patients were identified during this 27-year period. Of these 266 patients, 148 presented with oculo-auriculo-vertebral (OAV) spectrum, 52 with mandibulofacial dysostosis, 31 with Pierre Robin sequence, and 17 with miscellaneous syndromes. The remaining 18 patients were identified as having congenital mandibular hypoplasia without any known syndrome. Of the 18 patients with nonsyndromic congenital mandibular hypoplasia, 17 had primary bilateral growth anomalies and 1 had a primary unilateral growth disturbance resulting in bilateral deformity. Seven patients were products of a complicated pregnancy, 10 patients required tracheotomy or prolonged intubations, and 7 required gastric tube feedings. Associated anomalies included temporomandibular joint ankylosis in five patients, aglossia/microglossia in three patients, and rare craniofacial clefts in three patients. The average number of procedures required to treat the mandibular deformity for each patient was six. Although mandibular hypoplasia is a common craniofacial anomaly, patients manifesting nonsyndromic congenital mandibular hypoplasia are a rare subgroup. Case reports illustrating the range of mandibular deformities are presented.

Giant Congenital Melanocytic Nevi of the Trunk and an Algorithm for Treatment

Abstract: Giant congenital melanocytic nevi (CMN) are rare, congenital, disfiguring lesions with a risk of degeneration to malignant melanoma. Giant CMN are associated with an increased risk of malignant degeneration. In a minority of cases, patients with giant CMN may have associated neurocutaneous melanosis with leptomeningeal involvement. Giant CMN of the trunk pose difficult diagnostic and reconstructive problems requiring complex multistage treatment. For high-risk cases, diagnostic evaluation in the form of neuro-imaging is an essential component of the planning phase. Although nonsurgical options for the treatment of giant CMN have been advocated, these modalities may decrease the burden of nevus cells but do not result in complete removal of these cells. The ability to monitor nevus cells that remain after nonsurgical management of giant CMN remains questionable. These nonsurgical options include dermabrasion, laser ablation, and chemical peel. In contrast, direct excision of the nevus is the mainstay of treatment of nonsurgical management of giant CMN. There are numerous surgical options to resurface the resultant cutaneous defect after excision of the nevus. The simplest of these options consists of serial excision and direct closure of the defect in stages. However, if the defect cannot be closed by direct cutaneous advancement, other options for wound resurfacing include split- or full-thickness skin graft, tissue expansion, and free tissue transfer. Tissue expansion should be viewed as a category of treatment options because expanders can be used to create an expanded full-thickness skin graft, local expanded flaps adjacent to the lesion, or expansion of a free tissue donor site. Given the diversity of reconstructive options that use tissue expansion, these techniques have evolved as the primary treatment method for giant CMN of the trunk. The authors outline an approach to the evaluation of giant CMN of the trunk, review the risks of melanoma and of neurocutaneous melanosis, describe their preferred treatment regimen, and offer a treatment algorithm for giant CMN of the trunk.

In situ osteogenesis: regeneration of 10-cm mandibular defect in porcine model using recombinant human bone morphogenetic protein-2 (rhBMP-2) and Helistat absorbable collagen sponge.

Abstract: Traditional bone grafting relies upon the incorporation of a bone-cell bearing structure into a recipient site. The graft serves as a scaffold that is eventually replaced and remodeled. This process is known as osteoconduction. Recombinant human bone morphogenetic protein-2 (rhBMP-2) is commercially

Application of the supraorbital endoscopic approach to tumors of the anterior cranial base.

Abstract: Access to tumors of the anterior cranial fossa traditionally has required wide exposure of the surgical field, along with prolonged retraction of the frontal lobes or potentially disfiguring transfacial approaches. These approaches subject patients to undesirable neurologic and cosmetic morbidity. W

Treatment of recalcitrant idiopathic muscular torticollis in infants with botulinum toxin type a.

Abstract: Congenital muscular torticollis (CMT) is the most common form of torticollis in children, significantly outnumbering orthopedic, neurologic, and ocular causes. CMT may present as a palpable sternomastoid tumor (SMT) or a simple tightness of the sternocleidomastoid muscle (SCM), designated as idiopathic muscular torticollis (IMT). Muscular torticollis has been associated with positional plagiocephaly in neonates who slept in the supine position. We have had difficulty in treating some of these combined cases by traditional methods such as physiotherapy, stretching exercises, and molding helmets. In November 2000, we began injecting botulinum toxin type A in cases in which there was persistent IMT, despite significant physical therapy input. The 15 patients included in this retrospective study all presented with IMT and positional plagiocephaly; all had responded poorly to conservative treatment, including physiotherapy, stretching exercises, or use of a helmet. In the attempt to avoid progression to surgical release, these patients were treated with botulinum toxin injected into the affected SCM and subsequent additional physiotherapy. All appeared to respond well, and a retrospective analysis of this treatment strategy was undertaken. Information gathered included a questionnaire, skull-shape tracings, and photographs. Independent outcome assessment data were then obtained from the regional child development teams and community physiotherapists. These results show that 14 of 15 children with recalcitrant IMT and positional plagiocephaly treated with botulinum toxin obtained sufficient improvement in neck range of motion and head position as to make surgical release of the muscle unnecessary. Our conclusion is that the use of botulinum toxin is a safe and effective adjunct to physical therapy in treating recalcitrant IMT; in selected cases, it may obviate the need for surgical release of a tight but nonfibrotic SCM.

FGFR3 P250R mutation increases the risk of reoperation in apparent 'nonsyndromic' coronal craniosynostosis.

Abstract: Many patients with a clinical diagnosis of "nonsyndromic" coronal craniosynostosis have been found to be heterozygous for the fibroblast growth factor receptor 3 (FGFR3) mutation Pro250Arg. The phenotype associated with this mutation is variable and lacks highly distinctive features, so it is difficult to diagnose on clinical examination alone. The authors present a retrospective study of 76 patients with isolated coronal synostosis who were operated on in a single dedicated craniofacial unit over 25 years. The authors investigated whether any single factor, including the presence of a FGFR3 Pro250Arg mutation, predisposed to an increased transcranial reoperation rate. Eight patients had repeat transcranial surgery for a functional indication. Heterozygosity for the FGFR3 Pro250Arg mutation, present in 29 patients in the cohort, was the only factor found to have a significant association (P=0.048) with the transcranial reoperation rate. Six patients (20.7%) with the mutation underwent reoperation on the basis of raised intracranial pressure, as compared with two patients (4.3%) without the mutation. This highlights the need for genetic analysis and long-term clinical follow-up in apparently "isolated" coronal synostosis.

Surgical Planning in Reconstruction of Mandibular Defect With Fibula Free Flap: 15 Patients

Abstract: The authors present a descriptive work on the reconstruction of large oromandibular defects with revascularized fibula free flaps in a group of patients treated by surgical resection of bony and soft tissues of the mandible. The principal objective is preoperative planning for the best morphological, functional, and aesthetic result. The authors report 15 reconstructions of wide oromandibular defects by revascularized fibula free flaps. The average patient age was 42 years. The mean follow-up period was 18 months. Correct surgical planning reduces surgical timing, minimizes the chance of failure during the reconstruction, and contemporaneously increases treatment efficacy and the best functional and aesthetic results.