Search

High-velocity and high-impact forces can yield life-threatening injuries that are not only limited to the face but can involve other regions of the body. Associated injuries are more frequent in the elderly impacting management and outcomes. The primary objective was to study the association between associated injuries and age groups by identifying epidemiological trends and differences among the elderly and younger population.A retrospective review was conducted from January 2012 to January 2017 at the Royal Adelaide Hospital, Adelaide. A comparative analysis for elderly and younger controls was assessed for associated injuries, etiology, management, and length of stay.There were 355 elderly and 1884 younger controls for the study. There were differences between gender, alcohol, socioeconomic status, type of facial fracture, mechanism of injury, and associated injuries (p0.05). The elderly were 3.6 times more likely to have an associated injury compared to the younger cohort (p0.05). Total limb injuries were 2.2 times more likely in the elderly compared to the younger cohort, and neurological related injuries were 2.7 times more likely (p0.05). Neurological injuries were most frequent in fall-related injuries, limb injuries in RTA; and soft-tissue injuries for assault, sport, animal, and work (p0.001). The elderly had longer hospital stays as compared with younger controls (p0.05).Associated injuries are more frequent in the elderly and impact other bodily systems leading to a longer length of stay. In the assessment and management of facial trauma, anatomical and disciplinary boundaries may cross requiring interdisciplinary care.

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.

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.

Alcohol outlet density and unrestricted trading hours are strongly predictive of domestic and non-domestic assault rates, respectively. international studies report a strong male predominance for facial fractures. Harm minimization policies such as lockout laws have gained considerable attention to reduce accessibility of alcohol consumption and drinking patterns.A retrospective review was conducted from January 2012 to January 2017 at the Women and Children's Hospital and Royal Adelaide Hospital, Adelaide. All maxillofacial fractures that attended or were referred to the unit were included in this study. The primary objective was to determine the impact of facial fracture presentations, particularly assaults, for the pre- and post-lockout periods.From 2012 to 2017, there was a 5.1% decrease in assaults per year ( P = 0.002). Assaults increased by 11.7% for women and alcohol related assaults decreased by 10.2% for men ( P 0.05). Assault and alcohol related assault rates decreased by 9.3% and 10.5%, respectively, between lockout periods ( P 0.001). Comparative analysis between Adelaide and control (inner regional city) showed assaults decreased by 9.8% between lockout periods (51.2% versus 41.4%, P 0.001). Multivariate analysis showed alcohol, open reduction internal fixation and men were associated with a lower risk of assault related fractures, but women and night time presentations had a higher risk ( P 0.05).The influence of lockout laws contributed to an overall decrease in assaults and operative interventions, particularly alcohol related incidences amongst men. Ongoing longitudinal surveillance should guide current evidence for policy developers to reduce the risk of harm.

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.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.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 0.05). Boys were 2.3 times more likely to have a sport-related facial fracture than girls. Early adolescence were 5.2 times more likely to have an orbitozygomatic fracture than children of early childhood (P 0.05). Age, associated injuries, and sport-related facial fractures were independently associated with increased length of stay (P 0.001).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.

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.

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.1570CT (p.Arg524Trp) variant of TRAF7.

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.

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.

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.

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.

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.

We report a boy with a nasal deformity, choanal atresia, bifid uvula, severe bilateral microphthalmia and a facial cleft who showed regression of development at the age of 2 years with subsequent improvement. We suggest he represents a further case of the rare Fryns "Anophthalmia-Plus" syndrome.

Although most interest centers on the craniofacial region in Crouzon's disease, noncraniofacial manifestations are important because they may complicate both diagnosis and management. A population of 59 patients with Crouzon's disease was reviewed to determine the frequency of these deformities. Stylohyoid ligament calcification (50%) and cervical spine (40%) and elbow (18%) abnormalities were the most common. Minor hand deformities (10%), other musculoskeletal deformities (7%), and visceral anomalies (7%) were also seen. Acanthosis nigricans was not present in this population. Recommendations are made for the assessment and management of Crouzon's disease with reference to these areas.

Mandibular and dentoalveolar deformities associated with cystic hygroma of the head and neck have previously been described. This small series has identified changes involving the entire craniofacial skeleton, attributable to both the local and distant effects of massive facial lymphangiomas, without evidence of any actual soft tissue ingrowth into bone. In view of the inability to excise such lesions and normalize the soft tissue anatomy, the surgical approach to the craniofacial skeletal abnormality should be to reserve the required osteotomies or ostectomies until the completion of facial growth.

Background: Following the vote for independence in 1999, this team commenced the first coherent reconstructive surgical service in East Timor. The aim of this paper is to report the cleft lip and palate surgical experience during the 4 years since independence. Methods: From June 2000, a record of all cleft surgical procedures carried out by our team in East Timor has been maintained. This has been reviewed to identify the clinical experience, procedures carried out and the outcomes of the cleft population in this, the poorest and newest, nation in Asia. Results: From the 519 reconstructive surgical procedures carried out by this team in East Timor during the first 4 years, 267 were for cleft lip and palate deformity. Cleft lip and nose repairs were most commonly undertaken, although over the period of the study increasing numbers of cleft palate repairs were evident, reflecting the developing confidence in the service by the local population. Conclusion: The establishment of a regular, consistent cleft lip and palate surgical team in East Timor has not only seen the successful correction of a large number of untreated cleft patients, but has also contributed to a restoration of trust in the newly developing health system in East Timor.

Given a lack of a comprehensive classification for the frontoethmoidal encephalomeningocele (FEEM), clinical, photographic, and computed tomography (CT) data of 23 nonoperated patients were reviewed. Extracranial pathological findings of interest included herniation masses, facial deformities, and frontonasal bone morphology. Intracranial pathological findings of interest included morphology of the anterior cranial floor and brain malformations. Stereographic software processed data from a new-generation CT scanner into three-dimensional pictures that revealed some interesting morphological findings not often appreciated (eg, herniation mass without underlying external bone defect; mass at location far from external bone defect ["sequestrated cephalocele"]; new type of external bone defect characterized by a combination of nasoethmoidal and naso-orbital defects; correlation between mass, external bone defect, and exit pathway of herniation). Given these observations plus current knowledge available in the medical literature, a new classification system was developed that covers phenotypes and severity of the disease. The "FEEM classification" is an alphanumeric system based on facial deformities, external bone defect, exit pathway of herniation, and malformation of brain. It was tested in 42 patients for usability and validity. When combined with a newly designed "FEEM diagram," relevant pathological findings can be recorded in an objective manner so that diagnosis becomes more precise and uniform and comparison of outcome is possible. It also emphasizes the fact that FEEM has a range of manifestations governed by dynamic interaction between structural defects and herniation. Each clinical entity is a final result of its own disease course (stable, progressive, or regressive FEEM), with a varying degree of communication between the external mass and the central nervous system.

Hand anomalies in the amniotic band sequence are frequently both complex and difficult to explain in terms of our conventional understanding of limb embryogenesis. A case manifesting pseudosyndactyly between nonadjacent fingers confirms the role of an in utero disturbance after the seventh week, in concert with close apposition between nearby but not adjacent parts.

A technique of preoperative localisation of pharyngeal wall implants for velopharyngeal incompetence is described. The nasal approach in the non-sedated patient permits precise and permanent posterior pharyngeal wall tattooing for accurate implant location.

We performed a prospective study of 100 consecutive patients presenting to our unit with complex maxillary fractures. Fracture severity was assessed according to the alpha-numeric scoring system of Cooter and David and correlated with outcome. Regional maxillary and total fracture scores demonstrated a positive correlation with complication rates, with correlation coefficients of 0.89 and 0.98 respectively. Ocular and orbital complications were most commonly seen with these complex injuries, but occlusal problems were much less common. Despite close consultant-led supervision in a specialised centre, these injuries frequently result in long term problems.

Sutural anomalies in conjunction with craniofacial clefting are unusual. A case of median frontal clefting is presented in which there was an absence of a normal metopic suture and replacement by paramedian frontal sutures. The association of an underlying brain anomaly, with attendant surgical difficulties, is noted, as are the radiological techniques of preoperative diagnosis.

Over the last 10 years, children with deformational (nonsynostotic) plagiocephaly have undergone computed tomography scans using the same protocols as children with lambdoid craniosynostosis because of their similarly distorted head shapes. These children are believed to have normal intracranial volume. Given the recent questioning of what is the normal range of intracranial volume in human populations, the authors have undertaken a comparison of the intracranial volume of children with deformational plagiocephaly and Lichtenberg's normal population (Lichtenberg R. Radiographie du crane de 226 enfants normaux de la naissance a 8 ans: Impressions digitiformes, capacite, angles et indices [thesis]. Paris: University of Paris, 1960). The intracranial volume was determined for 20 females and 46 males with deformational plagiocepahly ranging in age from 2.5 to 20.7 months using computed tomography scan data. Although no significant differences were found for the females, the authors found that the intracranial volume of the males with deformational plagiocephaly were significantly larger than Lichtenberg's population. The authors conclude that this is because Lichtenberg's data do not adequately reflect the normal range of intracranial volume for males ranging in age from 2.5 to 20.7 months, rather than gender differences associated with deformational plagiocephaly. Further, until a more suitable normal becomes available, the deformational (nonsynostotic) plagiocephaly data could be used as substitute normal reference material in the measured age range for assessment of the intracranial volumes of children with craniosynostosis.

A consistent pattern of craniosynostosis in the sagittal and bilateral lambdoid sutures is described in three patients. The external cranial ridging associated with fusion of these sutures produces a characteristic triradiate, or "Mercedes Benz," appearance to the posterior skull. Locally marked growth restriction is evident in the posterior fossa with compensatory secondary expansion of the anterior fossa manifesting a degree of frontal bossing which mimics bicoronal synostosis. Although this appearance could lead to inadvertent surgery in the frontal region, attention to the occipital region with wide early suture excision and vault shaping is indicated.

Growing skull fractures are reported in three infants undergoing cranial vault surgery for craniosynostosis. Although primary dural injury was overt in only one patient, an extensive dural defect with spatially coincident bony absence was identified in all patients at late exploration. The coincidence of dural injury, deficient overlying bone, and a pulsatile growing brain are necessary conditions for the development of growing skull fractures. With the advent of increasingly radical cranial vault procedures during infancy, the potential for this complication to arise may increase. Definitive correction requires autogenous tissue reconstruction of the dural and bony defect.

The pathologic cause of both rare craniofacial clefts and congenital limb ring constrictions is the subject of some debate. Uncommon though these two conditions are, they have often been anecdotally reported in association. This study sought to determine the frequency of congenital limb anomalies in general and limb ring constrictions in particular amongst a population of patients with rare craniofacial clefts. Eighty-five cases of nonsyndromal, rare, craniofacial cleft were identified from a population of 280 patients assessed at the Women's and Children's Hospital, North Adelaide, with a diagnosis of rare craniofacial cleft classifiable by the Tessier system. Twenty-two patients (25.9 percent) displayed congenital limb anomalies. Eleven of these (12.9 percent) showed evidence of limb ring constrictions, a frequency much greater than in the general population. The group with limb ring constrictions demonstrated a significantly greater complexity of involvement with craniofacial clefting than the non-limb ring constriction group (4.27 clefts/patient versus 2.3 clefts/patient, p < 0.01). The distribution of craniofacial cleft locations in patients with evidence of limb ring constrictions was found to differ significantly from those with other or no limb anomalies (p < 0.01). The clefts in cases in which limb ring constrictions coexist are largely restricted to the paramedian 2-12, 3-11, 4-12 axes. This study confirms an association between rare craniofacial clefts and limb ring constrictions. The two conditions may therefore possess common etiology.

A child with frontoethmoidal meningoencephalocele in whom localized facial vitiligo developed after surgical correction of the encephalocele is presented. The potential role of the ocular disease accompanying the encephalocele in the development of the hypopigmentation is discussed.