Search

Central giant cell granuloma (CGCG) is a rare tumour. The aetiology remains controversial with immunohistological features suggestive of inflammatory, vascular and proliferative markers. It is most often diagnosed in children and young adults under the age of 30 years with a predilection for the mandible. We herein present a case in a paediatric age group of a maxillary lesion of CGCG with a novel genetic translocation. A combination of surgical intervention including enucleation and embolization produced a good result. The importance of long term follow up for facial growth and recurrence is terribly important for the child.

Objective: To evaluate the speech outcomes after primary cleft palate repair in a single tertiary medical institution of Thailand.Materials and Methods: A prospective cohort study was performed. Patients who had cleft palate with/without cleft lip and underwent primary cleft palate repair were included. Speech assessment was performed using the Pittsburgh weighted speech score (PWSS) by a speech-language pathologist.Results: Forty patients (21 males and 19 females) who underwent primary cleft palate repair at Siriraj Hospital were included. The median age at the time of speech evaluation was 7 years. The median age at primary cleft palate surgery was 12 months. The predominant cleft palate type was Veau 3 (47.5%). Oronasal fistula occurred 40%. Two-flap palatoplasty and intravelar veloplasty were the most common procedures. Median PWSS was 7, in which the competence velopharyngeal mechanism was found 5%, borderline competence 10%, borderline incompetence 32.5%, and incompetence velopharyngeal mechanism 52.5%. Among the velopharyngeal incompetence group, articulation disorder was the most common disorder with median score of 3. Besides, the median scores for hypo/hyper-nasality, nasal emission, phonation, and facial grimace disorder were 1, 2, 0 and 0, respectively. There was no statistically significant association between velopharyngeal incompetence and cleft types, age at primary surgery, type of operation, the width of cleft palate and prevalence of postoperative oronasal fistula or otitis media effusion.Conclusion: Velopharyngeal incompetence has been commonly identified after cleft palate repair in our institute. The articulation disorder is the most common characteristic.

To investigate the incidence of persistent, open metopic sutures in contemporary Australians aged 24 months and older. Metopic suture evaluation was conducted on retrospective cranial/cervical computed tomography scans of patients aged 24 to 252 months who presented to the Women’s and Children’s Hospital in Adelaide, Australia, between 2010 and 2020. Suture ossification was graded according to Lottering scoring system based on 4 stages, on three-dimensional volume-rendered reconstructions (stage 1: fibrous tissue interface, stage 2: commenced fusion, stage 3: complete fusion and stage 4: obliterated suture). The complete persistent sutures were classified as stage 1. Partially closed sutures were classified into stages 2 and 3, while completely closed sutures were defined as stage 4. One thousand thirty-four patients (61.2% male and 38.8% female) were included, with a mean age at scan of 66 months. More than half of patients were subject to scanning due to closed-head injuries. The incidence of persistent (completely open) metopic suture was 4.8% (2.3% in males and 2.5% in females). In comparison, a partially closed metopic suture was found in 6.3% of the study cohort, with the remaining sutures located along the metopic suture line, at the glabella, mid-part of the suture, bregma and glabella-bregma areas. The prevalence of persistent metopic sutures in our study of the Australian population is 4.8%, and it is equally distributed between the genders. The pattern of suture closure can commence from any location along the suture line, which is in contrast to the existing literature.

Craniosynostosis is a condition of premature fusion of the cranial sutures. Multi-suture craniosynostosis has been found to be associated with a number of syndromes and underlying gene mutations. Tumour necrosis factor receptor-associated factors (TRAFs) are a family of adaptor proteins interacting with cell surface receptors or other signalling molecules. TRAF7 is one of the factors involved in multiple biologic processes, including ubiquitination, myogenesis and toll-like receptor signalling. Here, we report a child who presented with multi-suture craniosynostosis and had the uncommon c.1570C>T (p.Arg524Trp) variant of TRAF7.

The management of sagittal craniosynostosis has evolved over the decades as teams seek to refine their surgical approaches to idealize head shape with the least possible morbidity. Here, the authors identify the incidence of raised intracranial pressure (ICP) and its risk factors, requiring secondary surgical intervention after cranial vault remodeling (CVR) procedure at a single tertiary referral craniofacial unit. A retrospective case-control study was performed on the patients with isolated non-syndromic sagittal craniosynostosis. All patients who underwent CVR in our unit and had a minimum of 1.5 years follow-up were included. One hundred and eighty-four patients (134 male and 50 female) who underwent primary CVR surgery for isolated sagittal craniosynostosis were included. Thirteen patients (7.07%) had clinical evidence of late raised ICP resulting in repeat CVR procedures. Higher incidence of raised ICP in patients who had primary surgery before 6 months than after or at 6 months of age (P = 0.001). There were 23.5%, 5.6%, 3.2%, and 1.9% of secondary raised ICP patients who underwent the primary surgery between 1999-2004, 2005-2010, 2011-2015 and 2016-2018, respectively (P = 0.024). The risk of secondary raised ICP was higher in patients with isolated sagittal craniosynostosis whose primary surgery occurred before the age of 6 months (two times more likely). More extensive CVR can be performed safely in sagittal synostosis with promising outcomes. The late presentation with raised ICP reinforces the importance of long-term multidisciplinary protocol-based follow-up.

Organised and non-organised sports have shifted greatly over the past decade with variations in epidemiological trends for maxillofacial trauma. Our aim is to describe epidemiological trends of sport-related facial fractures in adults outlining the differences amongst gender, age groups, types of sports, and how this impacts clinical outcomes and management. A retrospective review was conducted from January 2012 to January 2017 at the Royal Adelaide Hospital, Adelaide. All sport-related facial fractures, aged 18 years and above, that attended or were referred to the unit were included in this study. Three hundred five sport participants presented with a facial fracture with a strong male predominance (7.5:1). Australian football league (AFL) was the most common sport (39.0%) to result in a facial fracture followed by cycling (13.1%). Younger men aged 18–45 years played AFL, whereas females aged 18–55 years engaged in cycling. Orbitozygomatic fractures were the most common facial fractures (33.1%). Associated injuries occurred in 6.9% of the cohort. There were significant differences between men and women for age, age groups, seasons, and type of sport (p

Introduction Facial fractures of children are relatively infrequent compared to adults. There are variations in facial fractures depending on the socioeconomic, cultural, and educational factors of the country and time. Our aim is to describe epidemiological trends of facial fractures in the pediatric population, understand differences amongst the age groups, and the impact this has on clinical outcomes and management in South Australia. Methods A retrospective review was conducted from January 2012 to January 2017 at the Women and Children's Hosptial, Adelaide. All facial fractures of children, aged 16 years and below, that attended or were referred to the unit were included in this study. Results A total of 265 pediatric patients presented with a facial fracture with a male predominance. Some 49.1% occurred from a sports-related injury with bicycle motocross as the single most common type of sport. The mandible was the common fracture type with 21.9% of the total cohort having an associated injury. There were significant differences between boys and girls for age, age groups, mechanism of injury, and type of sport (P Conclusions There are differences amongst boys and girls and an understanding of these differences can aid the diagnosis and management in a growing child's face.

With an ageing population, the elderly will increasingly represent a growing subgroup of trauma. A high-risk group with multiple comorbidities, decreased physiological reserves and frailty, this contributes to the complexity of management in facial trauma. Falls represent the most common presentation for facial-related injuries, but there are variations amongst gender that is important to aid diagnosis, management, and treatment plans. Our aim is to identify epidemiological trends of facial fractures in the elderly, understand differences amongst gender and elderly age groups, and the impact this has on clinical outcomes and management. A retrospective review was conducted from January 2012 to January 2017 at the Royal Adelaide Hospital, Adelaide. All facial fractures of the elderly, 65 years and above, that attended or were referred to the unit were included in this study. Three hundred fifty-five elderly patients presented with a facial fracture with falls (82.5%) as the most common mechanism of injury. The orbitozygomatic fracture was the most common type of facial fracture with 48.7% of the cohort sustaining an associated injury. There were differences between men and women for age, age groups, socioeconomic status, season, alcohol, mechanism of injury, and facial fractures (p

BACKGROUND Animal-related facial fractures can affect livelihoods impacting the hard and soft tissues of the face. Animal husbandry and recreational sports have close relationships with animals, which can lead to injuries equivalent to those of road traffic accidents and assaults. Our aim is to describe epidemiological trends of animal-related facial fractures in the paediatric and adult age group across South Australia. METHODS A retrospective review from January 2012 to January 2017 at the Women and Children's Hospital and Royal Adelaide Hospital in Adelaide. All animal-related injuries that attended or were referred to the unit were included in this study. RESULTS There were 50 patients who presented with an animal-related facial fracture. There was an equal distribution of males to females with horses as the most common animal to cause injury from kicking. The orbit and orbitozygomatic fracture equally represented the most common type of facial fracture with 38% of all cases having an associated injury. The operative rate was 56% with four post-operative complications. There were differences between males and females for season, post-operative complications and length of stay (P

Cloverleaf skull deformity (CSD), or Kleeblattschadel, is a condition with severe and unpatterned multisuture craniosynostosis, resulting in a trilobar-shaped skull. This deformity mainly comprises a cranio-orbito-facial malformation that leads to a spectrum of multidisciplinary issues. Several syndromes are associated with CSD, such as Crouzon syndrome (CS). Here, we report the case of an infant with CS and the pathogenic c.1061C>G (p.Ser354Cys) variant of the fibroblast growth factor receptor 2 (FGFR2) gene. The child presented with the severe form of CSD despite having a normal, mid-trimester, sonographic scan.

Cleidocranial dysplasia (CCD) is a rare autosomal dominant disorder caused by mutations in the Runx2 gene. The CCD is characterized by frontal bossing, a patent anterior fontanelle, presence of Wormian bones, midface hypoplasia, multiple dental abnormalities, clavicular hypoplasia or aplasia, skeletal abnormalities, and short stature. The aims of this study are to report the phenotypic manifestations of all patients who presented with CCD and to review the multidisciplinary management of these patients. The longitudinal data of patients with a diagnosis of CCD treated at The Australian Craniofacial Unit from 1980 to 2019 were reviewed. Fourteen patients were identified for inclusion in this study. The age at referral to the unit ranged from 1 week old to 49 years old (mean 11.2 years old). All patients had clinical features of frontal bossing, a patent anterior fontanelle, multiple Wormian bones, midface hypoplasia, abnormal dentition, clavicular hypoplasia/aplasia, and normal intellect. Eleven patients had obstructive sleep apnea. Eight patients had positive family history. Speech issues were found in 6 patients and abnormal hearing was found in 4 patients. Seven patients who underwent skeletal survey were found to have skeletal abnormalities. All patients were evaluated and managed by the multidisciplinary team, which consisted of craniofacial surgeons, pediatric dentists, orthodontists, ENT surgeons, pediatricians, clinical geneticists, radiologists, orthopedic surgeons, and social workers. All patients were treated by dentists/orthodontists requiring multiple surgical interventions and orthodontic treatment. Seven patients who had recurrent ear infection underwent ventilation tube insertion. Seven of 11 patients who had obstructive sleep apnea underwent adenotonsillectomy. Four patients underwent orthognathic surgery to correct midface hypoplasia and malocclusion. Two patients had cranioplasty for correction of metopic depressions. The characteristic findings of patients with CCD involving multiple regions of the body should draw clinicians' attention to the need for multidisciplinary management of these patients.

The impact of fronto-orbital advancement (FOA) on frontal sinus development and function is anecdotally variable. The aim of this study was to assess the impact of FOA on development of frontal sinuses, and additionally to identify the complications that might arise out of such procedures. This was a retrospective case-control study. Non-syndromic and syndromic craniosynostosis patients (n = 58) who underwent FOA at an early age and also had a skull radiograph or CT scan after the age of 12 were selected. Age matched trauma patients with CT scans done beyond 12 years of age were used as controls. Age at first FOA surgery, total number of procedures and age at imaging was noted. Presence or absence of frontal sinuses was assessed using imaging studies initially. Patients with a formed frontal sinus and a CT scan were further chosen for volumetric studies. Complications related to frontal sinus and secondary surgeries were recorded. One of 27 non-syndromic patients had absent frontal sinuses. Seven of 31 syndromic patients had absent sinuses. Among 20 controls, only 1 patient did not develop frontal sinuses. The mean age at first FOA was 11.81 months and 18.25 months for non-syndromic and syndromic groups, respectively. The average number of procedures before 12 years of age was 1.25 and 1.51 for non-syndromic and syndromic patients, respectively. The mean age at imaging was 17.74, 20.96, and 20.25 years for non-syndromic, syndromic and control groups, respectively. The mean frontal sinus volumes were 13050.36, 15039.02, and 8459.48 mm for non-syndromic, syndromic and control groups, respectively. In conclusion, FOA does not seem to have an impact on rate of pneumatization in the background of similar rates in the non-syndromic and control groups. The low pneumatization rate in syndromic group might be a virtue of the disease itself. There were significant frontal sinus complications that occurred after fronto orbital advancement and this should be borne in mind during the surgical consenting process.

Metopic craniosynostosis is a condition in which the metopic suture is prematurely fused. Trigonocephaly and hypotelorism are the major abnormal findings associated with synostosis. Fronto-orbital advancement with cranial remodelling procedure is the widely practised method for correction of the deformities. Previously, a few studies have shown a low incidence of secondary raised intracranial pressure after the primary surgery. Thus, we reviewed our database to investigate the outcomes of metopic craniosynostosis treatment between 1999 and 2020 in Cleft and Craniofacial South Australia. One hundred twelve patients (77 males and 35 females) with metopic synostosis were operated. The mean age of primary surgery was 11.1 months (range 2.8-131.7 months). Ten patients (9%) who had secondary raised intracranial pressure underwent secondary surgery. Among those, the mean age of primary and secondary surgery were 5.4 and 57.1 months, respectively. Syndromic patients were significantly revealed to have a higher incidence of secondary raised intracranial pressure. There were three patients (2.7%) who had other secondary procedures (hardware removal and aesthetic contouring procedures). This cohort identified a higher incidence of secondary raised intracranial pressure requiring secondary surgery than previous studies. Syndromic patients were significantly related to secondary raised intracranial pressure. The patients who had raised intracranial pressure tended to have primary correction at younger age. Long-term multidisciplinary follow-up is mandatory. The demand for secondary surgery for metalwork complications or cosmetic contouring is uncommon.

INTRODUCTION The mandible is one of the most common facial bones to be injured with great clinical variability across countries caused by assaults, road traffic accidents, and falls. METHODS A retrospective review was conducted for adult mandibular fractures from January 2012 to January 2017 at the Royal Adelaide Hospital, Adelaide. Our aim was to describe epidemiological trends of mandibular fractures, differences for sex and age, and clinical outcomes. RESULTS Five hundred sixty five adult patients presented with a mandibular fracture with a male predominance (4.5:1). The mean age was 34.2 years with men 11.9 years younger than females (32.0 versus 44.1 years, P

Craniosynostosis is a condition where the cranial sutures are early fused. Sagittal suture synostosis is found to be the most prevalent. Many techniques ranging from simple suture excision to wide suturectomy have been developed for treating this condition. While repeated fusion of previously excised involved sutures is common, neosuture formation has been identified in many recent reports after craniosynostosis surgery. In this case report, the authors present a finding of the neosuture formation in a patient presented with sagittal craniosynostosis after wide suturectomy with total cranial vault remodeling so that the pathologically fused suture can be reversed.

The mastoid process, a pneumatized prominence of the temporal bone with air-filled air cells, grows with age. We present here a series of 4 patients with bilateral mastoid hypertrophy associated with severe trismus from different etiologies, and discuss the possible explanation of this observation.

Pfeiffer syndrome is one of the autosomal dominant craniofacial syndromes. Classical clinical manifestations are coronal suture synostosis causing brachycephaly, midface retrusion, airway compromise, broad thumbs, and toes. Pfeiffer syndrome type I (classic type) is associated with FGFR1 mutation. However, wide range of clinical manifestations, with and without craniosynostosis, have been reported. Here, we present a family of Pfeiffer syndrome across 3 generations with identical FGFR1: c.755C>G (p.Pro252Arg) mutation. Where the members of the youngest generation have no cranial involvement. Lastly, we propose a guideline management for familial Pfeiffer syndrome management.

BACKGROUNDSquamosal sutures are minor sutures of the human skull. Early isolated fusion of the sutures (squamosal synostosis) is rarely found.OBSERVATIONSThe authors report a case of a girl who presented with an abnormal head shape and bilateral squamosal synostosis. Genetic testing revealed a chromosome 1p12–1p13.3 deletion. She has been managed with conservative treatment of the synostosis. She has global developmental delay and multiple anomalies due to the chromosome abnormality.LESSONSIsolated squamosal suture synostosis could be an uncommon feature of chromosome 1p12–1p13.3 deletion.

Plastic and reconstructive surgical teams visiting from Australia, a high-income country, have delivered cleft surgical services to Timor Leste since 2000 on a volunteer basis. This paper aims to estimate the economic benefit of correcting cleft deformities in this new nation as it evolved its healthcare delivery service from independence in 1999. We have utilised a prospective database of all cleft surgical interventions performed during 44 plastic surgical missions over the last 18 years. The disability-adjusted life year (DALY) framework was used to calculate the total DALYs averted by primary cleft lip and palate repair. The 2004 global burden of disease disability weights were used. Economic benefits were calculated using the gross national income (GNI) and the value of a statistical life (VSL) methods for Timor Leste. Estimates were adjusted for treatment effectiveness, counterfactual cases, and complications. Cost estimates included the local hospitalisation costs, the foregone salaries of the visiting surgeons and nurses, other costs associated with providing surgical care, and an estimate for foregone wages of the patients or their carers. Sensitivity analysis was performed with income elasticity set to 0.55, 1.0, and 1.5. During 44 visiting plastic surgical missions to Timor Leste, 1500 procedures were performed, including 843 primary cleft lip and palate operations. The cleft procedures resulted in the aversion of 842 DALYs and an economic return to Timor Leste of USD 2.2 million (GNI-based) or USD 197,917 (VSL-based). Our programme cost USD 705 per DALY averted. The economic return on investment was 0.3:1 (VSL-based) or 3.8:1 (GNI-based). A sustained and consistent visiting team approach providing repair of cleft lip and palate defects has resulted in considerable economic gain for Timor Leste over an 18-year period. The training of a local surgeon and multidisciplinary team with ongoing support to the in-country cleft service is expected to reduce the cost per DALY averted once the surgeon and team are able to manage clefts independently.

Background Preservation of the anterior cerebral arteries (ACAs) is important in the surgical management of frontoethmoidal meningoencephalocele (FEME). This would avoid complications related to the loss of blood supply to the part of the brain supplied by the ACA. Previous reports have identified hydrocephalus, microcephaly, cerebral dysplasias amounting to a 15% to 20% prevalence of brain anomalies in patients with FEME. What has not been previously reported are cerebral vasculature changes in the frontal region in FEME and how these may impact on the surgical correction and clinical outcome. Methods Two patients of FEME that demonstrate cautionary radiologic findings in relation to the ACAs and anterior fossa anatomy are discussed. Results The ACA in a 4-year-old boy with FEME was displaced anteriorly with the long A1 segments that extend into the defect. In the 2nd patient, a 4-year-old girl, we report on the complications related to the injury of ACAs from a previously partially resected FEME. Conclusion The importance of the anterior cerebral vasculature around the FEME during surgery is crucial to prevent complications resulting from damage to a looping A1 segment of the ACA.

Purpose Apert syndrome is a rare congenital malformation with severe craniofacial anomalies. The aim of this study was to review the outcomes of craniofacial and neurosurgical interventions in Apert syndrome patients treated at a single institution. Materials and methods A retrospective review of all patient records with a diagnosis of Apert syndrome assessed and managed in the Australian Craniofacial Unit (ACFU) from 1985 to 2013 was conducted. Results A total of 94 patients were identified, and 130 transcranial procedures were performed. Of the patients, 83 underwent a fronto-orbital advancement (FOA) as their primary procedure, and 18 patients also underwent a posterior vault procedure. Twenty patients underwent a fronto-facial monobloc advancement. Overall, 70% of patients underwent at least 2 transcranial procedures. Shunts were inserted in 2 patients preoperatively and in 5 patients postoperatively for cerebrospinal fluid (CSF) leaks or acute hydrocephalus. Re-do FOAs were performed in 8 patients. Patients who underwent an FOA at the age of more than 18 months had no recurrence of raised intracranial pressure (ICP). Of 18 patients who also underwent a posterior vault procedure, 1 patient had recurrence of raised ICP. Midfacial surgery was performed early if there was evidence of obstructive sleep apnoea (OSA), but delayed midfacial surgery was preferred. Complications were reported in 18% of procedures. The most common complications were CSF leaks and acute hydrocephalus. Conclusion Shunting is rarely required in Apert syndrome patients, confirming a predominantly nonprogressive ventriculomegaly. FOA appears to be a more stable procedure when performed at an age of more than 18 months. Undergoing a posterior vault procedure may reduce the risk of recurrent raised ICP and lead to fewer transcranial procedures needed in childhood. Midfacial surgery should be delayed until adolescence where there is no evidence of OSA, psychological disturbance, or complications of exorbitism. Complications are rare when these patients are treated by an experienced craniofacial team.

The complex, progressive, multisystem nature of Apert syndrome presents many challenges to managing surgeons. Based on the pioneering work of Paul Tessier, the senior author developed a multidisciplinary birth to maturity management protocol for Apert syndrome. Between 1975 and 2014 the Australian Craniofacial Unit has treated 174 Apert syndrome patients and 28 have completed full protocol management. This paper reviews the scientific contribution made to the management of Apert syndrome by the Australian Craniofacial Unit, the development and evolution of the protocol and presents comprehensive data on the surgical and nonsurgical craniofacial interventions, and outcomes for the 28 patients who have completed the programme; 26 had normal visual acuity, 22 had normal hearing, 20 achieved normal or nearly normal speech, 24 a functional class I occlusion, 18 completed mainstream schooling of whom at least 8 went on to tertiary education, at least 13 gained employment and 15 developed good social groups. These outcomes equal or exceed those presented by other authors and provide compelling evidence of the value of protocol management in clinical outcomes, in addition to their value in international collaboration, and scientific development of future therapeutic strategies for the management of Apert syndrome.

Over the last 10 years, the concept of value has emerged in both business and public life as part of an important process of measuring, benchmarking, and assuring the resources we invest and the outcomes we generate from our activities. In the context of public life, value is an important measure on the contribution to business and social good of activities for which strict financial measures are either inappropriate or fundamentally unsound. A systematic, interdisciplinary examination of public value is necessary to establish an essential definition and up-to-date picture of the field. In reflecting on the ‘public value project’, this book points to how the field has broadened well beyond its original focus on public sector management; has deepened in terms of the development of the analytical concepts and frameworks that linked the concepts together; and has been applied increasingly in concrete circumstances by academics, consultants, and practitioners. This book covers three main topics; deepening and enriching the theory of creating public value, broadening the theory and practice of creating public value to voluntary and commercial organisations and collaborative networks, and the challenge and opportunity that the concept of public value poses to social science and universities. Collectively, it offers new ways of looking at public and social assets against a backdrop of increasing financial pressure; new insights into changing social attitudes and perceptions of value; and new models for increasingly complicated collaborative forms of service delivery, involving public, private, and not-for-profit players.

Conventional skin biopsy limits the clinical research that involves cosmetically sensitive areas or pediatric applications due to its invasiveness. Here, we describe the protocol for using an absorbent microneedle-based device, absorbent microbiopsy, for minimally invasive sampling of skin and blood mixture. Our goal is to help facilitate rapid progress in clinical research, the establishment of biomarkers for skin disease and reducing the risk for clinical research participants. In contrast to conventional skin biopsy techniques, the absorbent microbiopsy can be performed within seconds and does not require intensive training due to its simple design. In this report, we describe the use of absorbent microbiopsy, including loading and application, on a volunteer. Then, we show how to isolate RNA from the absorbed sample. Finally, we demonstrate the use of quantitative reverse transcription PCR (RT-qPCR) to quantify mRNA expression levels of both blood (CD3E and CD19) and skin (KRT14 and TYR). The methods that we describe utilize off the shelf kits and reagents. This protocol offers a minimally invasive approach for simultaneous sampling of skin and blood within the same absorbent microbiopsy matrix. We have found human ethics committees, clinicians and volunteers to be supportive of this approach to dermatological research. Refereed/Peer-reviewed

Apert syndrome characterized by acrocephalosyndactyly is a rare autosomal dominant congenital malformation with a prevalence of 1/65,000 births. With an extensive range of phenotypic and developmental manifestations, its management requires a multidisciplinary approach. A variety of craniofacial, central nervous system (CNS), and cervical spine abnormalities have been reported in these patients. This study aimed to determine the incidence of these CNS abnormalities in our case series. Retrospective review of Australian Craniofacial Unit (ACFU) database for Apert patients was performed. Data collected that included demographics, place of origin, age at presentation, imaging performed, and images were reviewed and recorded. Where available, developmental data was also recorded. Ninety-four patients seen and managed at the ACFU had their CNS and cervical spine abnormalities documented. The main CNS abnormalities were prominent convolutional markings (67 %), ventriculomegaly (48 %), crowded foramen magnum (36 %), deficient septum pellucidum (13 %), and corpus callosum agenesis in 11 %. Major C-spine findings were present in 50.8 % of patients and included fusion of posterior elements of C5/C6 (50 %) and C3/4 (27 %). Multilevel fusion was seen in 20 %. Other abnormalities were C1 spina bifida occulta (7 %) and atlanto-axial subluxation (7 %). Multiple CNS and cervical spine (c-spine) abnormalities are common in Apert syndrome. The significance of these abnormalities remains largely unknown. Further research is needed to better understand the impact of these findings on growth, development, and treatment outcomes.

Questions of how best to define the ends, justify the means, and measure the performance of governments have preoccupied political economists for centuries. Recently, the concept of public value—defined in terms of the many dimensions of value that a democratic public might want to see produced by and reflected in the performance of government—has been proposed as an alternative approach. This article develops three philosophical claims central to the practice of public value accounting: (1) when the collectively owned assets of government are being deployed, the appropriate arbiter of public value is the collectively defined values of a “public” called into existence and made articulate through the quite imperfect processes of democratic governance; (2) the collectively owned assets include not only government money but also the authority of the state; (3) the normative framework for assessing the value of government production relies on both utilitarian and deontological philosophical frameworks.

This article explores a special class of innovations - innovations in governance – and develops an analytical schema for characterizing and evaluating them. To date, the innovation literature has focused primarily on the private rather than the public sector, and on innovations which improve organizational performance through product and process innovations rather than public sector innovations which seek to improve social performance through re-organizations of cross-sector decision-making, financing and production systems. On the other hand, the governance literature has focused on social co-ordination but has not drawn on the innovation literature. The article uses four case studies illustratively to argue that innovations in governance deserve greater attention theoretically. Further, it argues that five inter-related characteristics distinguish public sector innovations in governance from private sector product and process innovations. Innovations in governance: go beyond organizational boun...

Background: Recurrent fractures of the previously treated fractured mandible are rare. Methods: In this retrospective study, 13 cases of repeated mandibular fractures were evaluated according to age, sex, cause, fracture localizations and patterns, treatment modalities; and complications. Results: All of the patients were men, with a mean age of 27.5 years and 32 years for the first and the subsequent fractures, respectively. The mean time interval between fractures was 4.3 years. In 10 cases, the cause for the second fracture was an assault, with 90 percent related to alcohol intake. Angle, body, and parasymphysis region of the mandible were the most common sites to be involved in the first occasion. In 10 cases, an internal rigid fixation procedure was performed for the treatment. In the recurrent injuries, none of the fractures occurred at exactly the same anatomical site. Fractures were either in the neighboring side or the contralateral side of the previously healed area of mandible. On the second occasion, angle fractures were common and body and subcondylar fracture rates increased. At second presentation, the complication rate increased from 23.1 percent to 53.8 percent and most commonly involving malocclusion. Conclusions: This study showed that assault is the major etiologic factor for recurrent mandible fractures and that these fractures occur in different locations from the original injury. They necessitate a cautious approach for the prevention of unfavorable outcomes. Further retrospective clinical and experimental studies are necessary to elucidate the fracture patterns and bone-healing quality of these recurrent mandible fractures.

Mandibular lengthening by distraction was performed in a 6-year-old severely affected Treacher-Collins syndrome patient who was tracheostomy dependent. As previously reported, this procedure permitted tracheostomy removal once distraction was complete. Now that the patient is skeletally mature, the long-term results of this intervention are reported with regard to his clinical outcome and an assessment of the anatomical changes in the upper airway during growth. Although the distraction could be considered a success in that it enabled permanent decannulation and improved the minimum cross-sectional area of the upper airway, there was no further increase in the minimum cross-sectional area of the upper airway during childhood growth. It is significant that the abnormal growth pattern of the mandible, which is characteristic of this syndrome, did not alter from its preoperative pattern once distraction was completed.

I Introduction Citizens have long wanted a reliable measure of police performance. They want to know whether the police are producing something that is valuable with the assets entrusted to them. They want to be able to hold the organization to account for its performance. Police managers, too, have needed a measure of police performance-partly to meet external demands for accountability, and partly to establish a form of accountability inside their organizations that could focus attention on achieving valuable results rather than simply reliably executing established policies and procedures. A crucially important task for both citizens and police managers, then, is to work out an understanding of how the "public value" produced by policing can be recognized and assessed. Generally speaking, developing appropriate measures of police performance has been treated as a managerial or technical problem to be solved through some combination of the practical methods of business management (develop a "bottom line" for policing) or the statistical methods of social science (conduct a "program evaluation" of police departments focusing on the ultimate outcomes produced by the police). And it is true, of course, that developing suitable performance measures do raise important managerial, technical, and scientific issues. The managerial issues focus on how different measurement systems can be used to guide, motivate, or enable the learning of an organization. The technical issues concern the development of the statistical measures and instruments that can reliably capture the dimensions of performance that the police deem important. The scientific inquiry focuses on the extent to which performance measurement systems can help the police find out what particular programs "work" to accomplish various police objectives. In the end, though, it seems self-evident that the development of police performance measures is also, and perhaps most fundamentally, a normative and political question; not only or even primarily a management or technical issue. After all, to develop a standard for assessing the performance of an organization as "good" or "bad," "improving" or "deteriorating," is to make a normative, value claim as well as a positive, scientific claim. One has to have an idea of the "good" or the "right" as it applies to police operations to be able to defend a standard of police performance. One also has to have a theory about whose views of the "good" and the "right" should count in setting a standard for policing--more particularly, whether it is the views of individuals deciding for themselves what they think is "good" and "right" as they encounter the police as individuals seeking assistance or individuals who are stopped, cited, or arrested; or whether the idea of the good and the right in policing emerge from some kind of collective political process in which the body politic gathers itself together to say what is good and right for the whole, and where that view trumps the views of individuals. One may also have to have an idea about the kinds of deliberative processes that should, ideally, go into the formation of a collective judgment about the good and the right as it applies to public police departments. In short, the important question that lies at the core of developing any adequate measure of police performance is for citizens and their elected representatives to decide what it is that is intrinsically valuable, or what it is that we as a political community value in the activities and operations of a public police department. That is simultaneously an issue for political philosophy and practical politics, as well as for professional management and science. Indeed, once one sees the issue as a normative, philosophical, and political question, a great many additional questions arise, including the following: * Who does the valuing of public sector operations? Are police operations properly evaluated by the "customers" of the police (those who receive services from the police and/or pay the costs of keeping the police operating)? …

Police performance measurement systems based on traditional indicators, such as arrest rates and response times, prevent police organizations from moving towards a strategy of community problem solving as there is no way to hold police departments externally accountable for addressing community concerns and no way to hold particular officers internally accountable for engaging community problem‐solving activities. In the absence of relevant measurement systems, police executives experience difficulty motivating their managers and line‐level officers to change their approach towards policing. A number of departments have made considerable progress in developing performance measurement systems that both address community concerns and drive their organizations towards a community problem‐solving strategy. This paper argues why police executives would want to measure performance, describes how measurement is important in driving organizational change, discusses what police departments should be measuring, and presents an exploratory qualitative analysis of the mechanisms at work in the New York Police Department’s Compstat and its application in six other police departments.

The case for focusing regulatory and enforcement efforts on the illegal supply of firearms to criminals rests on the belief that a supply-side approach has the potential to reduce the use of guns in violence. The case against this focus follows from the belief that guns in America are so readily available, and from such a variety of sources, that efforts to restrict the supply are futile. Individuals who are proscribed from buying guns legally (because of their criminal record or youth) tend to acquire firearms from "point" sources, such as illegal traffickers and scofflaw dealers, and "diffuse sources," including all sorts of informal transfers from the vast stock of weapons in private hands. Both are important. The mix within a jurisdiction appears to depend on the prevalence of gun ownership and the stringency of state regulations. A variety of promising supply-side measures are available, and some have been tried. Lessons have been learned-for example, that gun "buybacks" are ineffective-but for the m...

Increased prominence and greater influence expose international nongovernmental development and environment organizations (INGOs) to increased demands for accountability from a wide variety of stakeholders, including donors, beneficiaries, staffs, and partners. This article focuses on developing the concept of INGO accountability, first as an abstract concept and then as a strategic idea with very different implications for different INGO strategies. The authors examine implications for INGOs that emphasize service delivery, capacity building, and policy influence. They propose that INGOs committed to service delivery may owe more accountability to donors and service regulators, capacity-building INGOs may be particularly obligated to clients whose capacities are being enhanced, and policy influence INGOs may be especially accountable to political constituencies and influence targets. INGOs that are expanding their activities to include new initiatives may need to reorganize their accountability systems to implement their strategies effectively.

Intracranial volume comparisons of patients with craniosynostosis and normal have been contrary to expectations, leading to questioning of the validity of the current normal reference material. Computed tomography-determined intracranial volume is presented for a white normal population. Specifically, intracranial volumes for 157 subjects (82 female and 75 male) were measured from computed tomography data using the Cavalieri estimator: volume determination was based on measuring the area in each computed tomography section. Monomolecular and Gompertz models were applied to find curves of best fit to the intracranial volume as a function of the age. The best fit was obtained using the monomolecular model when the response variable was the logarithmically transformed intracranial volume, and the independent variable was the logarithm of the age from conception. For example, the mean (standard deviation) for male subjects at 1 year and 20 years were 1,125.6 (89.6) ml and 1,472.9 (117.2) ml, respectively, and for female subjects 1,024.9 (84.0) ml and 1,321.7 (108.3) ml, respectively. Although the shape and rate of increase of the female and male curves is similar, the female mean is 1.3 standard deviations below the male mean at 20 years. These curves were compared with the commonly referenced curves of Blinkov (1941), Lichtenberg (1960), and Dekaban (1977). Our male curve is substantially higher than these curves in the age range 8 months to 4 years. Our female curve, however, is approximately 1 standard deviation below Lichtenberg's curve from birth to 7 months. There are then only minor differences between our female curve and Lichtenberg's curve until his curve crosses ours at 41 months, where they significantly diverge from approximately 4.5 years. Our curves indicate that 95% of the final intracranial volume has been attained by 42 months for girls and 46 months for boys.

All organizations benefit from developing a strategy. The most well-developed strategy models come from the private sector and focus on markets, customers, and competition. Yet, these models fail to take account of two crucially important features of nonprofit organizations: (a) the value produced by nonprofit organizations lies in the achievement of social purposes rather than in generating revenues; and (b) nonprofit organizations receive revenues from sources other than customer purchases. An alternative strategy model developed for governmental managers focuses the attention on three key issues: public value to be created, sources of legitimacy and support, and operational capacity to deliver the value. This alternative strategy model resonates powerfully with the experience of nonprofit managers precisely because it focuses attention on social purposes and on the ways in which society as a whole might be mobilized to achieve them.

Adams-Oliver syndrome (AOS) is characterised by aplasia cutis congenita of the scalp and variable degrees of terminal transverse limb defects. Short fingers and hypoplastic nails also occur in this predominantly autosomal dominant syndrome which displays marked variability of expression and lack of penetrance in some cases. We describe a boy with AOS whose sister is also mildly affected. Their mother has hypoplastic fifth toenails which may represent very mild expression of the syndrome. Brain (computed tomography) imaging to investigate mild left hemiparesis in the boy demonstrated severe cortical dysplasia of central, occipital and anterior regions of the right cerebral hemisphere. A variety of brain and cranial malformations has been reported in AOS but dysplasia of the cerebral cortex has not been noted previously. In addition, the boy and his sister have apparent constriction rings present on the toes which are uncommon in AOS.

Tessier craniofacial clefts are among the most surgically challenging examples of craniofacial dysmorphology. These clefts are characterized by hypoplasia of soft-tissue and skeletal elements throughout the three-dimensional extent of the cleft. Whereas bone grafting and craniofacial osteotomies have been successful toward correcting the underlying skeletal abnormalities, the ultimate success of these reconstructions has been limited by the deficiency of skin and soft tissue. This deficiency demands reconstruction ideally with tissue of like texture, consistency, and, especially in the face, color. Craniofacial tissue expansion was used toward reconstructing these facial clefts with like-quality tissue, allowing for tension-free reconstruction after osteotomy and bone grafting. Seventeen patients with Tessier craniofacial clefts underwent preoperative craniofacial soft-tissue expansion in the surgical management of their clefts. Tissue expansion was used in the primary correction of facial clefts in eight patients, with nine patients undergoing expansion before secondary surgery. In this series, tissue expansion has evolved as an important element in overcoming the skin and soft-tissue deficiency associated with these clefts, allowing for tension-free closure and improved aesthetic results in these surgically challenging patients.

Nasofacial phycomycosis caused by Conidiobolus is a rare fungal infection most often encountered in the developing world in conditions of poverty. A clinical presentation of the natural history of this condition observed over a period of 6 years by a visiting volunteer surgical team demonstrates the role of multimodality surgical and antifungal drug treatment in producing a successful outcome. The particular sensitivity of this infection to the new generation antifungal Voriconazole is noted.

Muencke syndrome results from mutations in the FGFR3 gene, and although it is well recognized that the clinical presentation is variable, the important key finding includes coronal synostosis. We present a family where a mother with proven FGFR3 Pro250Arg mutation gave birth to identical twins both of whom had craniosynostosis but had coexisting bilateral cleft lip and palate. We believe that this is the first description of clefting occurring in conjunction with Muencke syndrome and so further extends the range of phenotypic variation that can occur in this syndrome.

The histologic findings from sutural specimens removed during early cranial surgery in Apert's syndrome infants have been reviewed. Coronal sutures are uniformly fused histologically in their inferolateral extent, showing a progression to lesser changes at the superior margin. These findings correspond with radiographic changes. The lambdoid sutures occasionally show histologic evidence of fusion, with the same pattern of inferior to superior progression, but a lack of objective radiographic changes.

The purpose of the present study was to retrospectively review the management and outcome of patients treated by the Australian Craniofacial Unit (ACFU) for isolated fractures of the medial orbital wall. A retrospective medical record review of patients treated between 2008 and 2012 was performed. Ethical approval was granted by the ethics committee of the Women's and Children's Hospital. Patient demographics, causes of injury, physical examination findings, management (conservative or surgical), and findings at follow-up were recorded. Computed tomographic scans were reviewed, and values for fracture area and volume of displaced tissue were calculated. Twenty-four patients with this injury were treated by the ACFU between 2008 and 2012. Eighteen were male, and 6 were female. Assault was the most common cause (15/24 patients). Fifteen patients were managed conservatively, and 9 were treated surgically. In those patients managed conservatively, the mean fracture area was 1.44 cm (0.47-2.47 cm), and the mean volume of displaced tissue was 0.48 mL (0.03-1.15 mL). In patients treated surgically, the mean fracture surface area was 2.32 cm (0.07-3.43 cm), and mean volume of displaced tissue was 0.94 mL (0.00-1.47 mL). No patients were found to have clinically significant enophthalmos at follow-up examination. The current practice of managing isolated fractures of the medial orbital wall at the ACFU has been successful in preventing significant enophthalmos. The thresholds for fracture area and, in particular, volume of displaced tissue, proposed by Jin et al (J Oral Maxillofac Surg 2000;58:617-620), show merit as a tool for determining patient management.

A review of Pfeiffer's syndrome patients presenting in infancy identifies characteristic patterns of onset and progression of premature sutural fusion. Classic Pfeiffer's syndrome manifests symmetrical bicoronal synostosis; all other sutures are normal. The remaining patients, with a more extreme phenotypic expression, have superimposed on bicoronal synostosis progressive involvement of other cranial sutures, frequent hydrocephalus and craniolacunae, suggesting craniostenosis and intracranial hypertension. Although similar in clinical features and outcome, these patients have been subgrouped according to the presence or absence of a cloverleaf skull anomaly.

Involvement of the craniofacial skeleton by the process of massive osteolysis has been infrequently recorded. We describe a patient whose extent of craniomaxillofacial bony loss defied combined reconstruction by combined autogenous tissue and alloplastic materials. The clinical and radiographic features and management approaches to massive osteolysis are reviewed.

Review of the spinal radiographs of a consecutive series of 11 patients with Pfeiffer syndrome presenting to the Australian Craniofacial Unit was performed. The prevalence of cervical spine fusions was high, and the pattern of fusion complex. Isolated anomalies were evident at lower levels, including two cases of sacrococcygeal eversion. Spinal anomalies occur more frequently in the more severely involved cases of Pfeiffer syndrome emphasizing the generalized dysostotic nature of this condition.

The combination of bicoronal craniosynostosis, broad thumbs and great toes, and partial variable soft tissue syndactyly of the hands and feet (i.e., Pfeiffer syndrome) classically followed a benign clinical course. A review of the clinical features of those Pfeiffer syndrome patients presenting to our unit confirm another subgroup in whom the craniofacial and associated manifestations are more extreme, with a significant risk of early demise. The early aggressive surgical management of craniostenosis, hydrocephalus, exorbitism, faciostenosis, and upper airway obstruction has provided the potential for prolonged useful survival in these cases.

America appears to have committed itself to a profound shift in its core policing strategy, from “reform policing” to “community policing.” This shift has been propelled by a powerful historical critique of the reform strategy; by an operational movement in police departments; and by political forces. Still unanswered is the question of whether community policing “works”; that is, is a more valuable organizational strategy than the reform strategy. Social science and evaluation research are turning to this question. Implicit in the approach of community policing is a belief that the values of social science should guide social decision making; that this is a specialized task for trained outside evaluators; that “crime” is the most critical performance dimension; and that programs rather than organizations are the proper units of analysis. The authors argue that this framework may hinder the full development of community-policing departments as “learning organizations”; that dimensions other than c...