Background: The bilateral zygomatic “out-fracture” procedure is highly effective in correcting upper midfacial deficiency in combination with maxillary orthognathic surgery. The aim of this retrospective study is to describe this technique and demonstrate how virtual surgical planning is used to ensure accurate and symmetrical malar augmentation in conjunction with orthognathic surgery.

Objective Describe the intelligence quotient (IQ) of children with Pierre Robin sequence (PRS). Design Prospective cohort study. Setting Neurodevelopmental follow-up clinic within a hospital. Patients Children with PRS (n = 45) who had been in the Neonatal Intensive Care Unit (NICU) were classified by a geneticist into 3 subgroups of isolated PRS (n = 20), PRS-plus additional medical features (n = 8), and syndromic PRS (n = 17) based on medical record review and genetic testing. Main Outcome Measure Children with PRS completed IQ testing at 5 or 8 years of age with the Wechsler Preschool and Primary Scale of Intelligence, Third Edition (WPPSI-III) or Fourth Edition (WPPSI-IV) or the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV) or Fifth Edition (WISC-V). Results IQ scores were more than 1 to 2 standard deviations below the mean for 36% of the overall sample, which was significantly greater compared to test norms (binomial test P = .001). There was a significant association between PRS subtype and IQ (Fisher’s exact P = .026). While only 20% of children with isolated PRS were within 1 standard deviation below average and 35% of children with syndromic PRS were below 1 to 2 standard deviations, 75% of PRS-plus children scored lower than 1 to 2 standard deviations below the mean. Conclusion PRS subgroups can help identify children at risk for cognitive delay. The majority of children with PRS-plus had low intellectual functioning, in contrast to the third of children with syndromic PRS who had low IQ and the majority of children with isolated PRS who had average or higher IQ.

There is a low incidence of serious complications with the Le Fort I maxillary osteotomy, but avascular necrosis is a serious problem that occurs at a higher frequency in the cleft population compared to non-cleft patients. In this retrospective study, cleft patients who had exhibited any manifestation of avascular necrosis of the maxilla following a Le Fort I advancement were identified. Five patients were found to have had vascular compromise, ranging from loss of gingival tissue to necrosis of bone and loss of teeth. In each case, possible risk factors were assessed, and secondary revision palatal surgery prior to maxillary advancement, particularly closure of oronasal fistulae with transpositional flaps, was noted to be present. Drawing on this finding and a review of the literature, clinical recommendations are made for minimizing the risk of this major complication. The use of the 'delayed maxillary flap' technique is introduced and described as a potentially beneficial procedure for cleft patients with a high risk of vascular compromise.

Condylar hyperplasia is known to result in facial asymmetries and constitutes a well-recognized group of unilateral mandibular enlargements. Condylar hyperplasia has been sub-classified into hemimandibular hyperplasia and hemimandibular elongation. A much rarer disorder, hemifacial hyperplasia (or hemifacial hypertrophy) is a congenital malformation characterized by prominent unilateral overdevelopment of the hard and soft tissues of the face. The affected side grows at a faster rate than the non-affected side, creating a marked asymmetry that potentially involves the skeleton and teeth, as well as all components of the associated soft tissues. Hemifacial hyperplasia is usually identified at birth and progresses towards puberty, but is not thought to alter throughout the lifetime of affected individuals. A case series of five patients clinically diagnosed with hemifacial hyperplasia is presented, with the aim of reviewing the clinical features, discussing their individual surgical management, and summarizing the more recent identification of possible genetic mutations that may be responsible for hemifacial hyperplasia and related overgrowth disorders. It is speculated that depending on the genetic factors, the disorder may be progressive in specific cases.

Introduction: Neonates & infants with craniofacial anomalies often present with varying degrees of upper airway obstruction (UAO) due to micrognathia. Mandibular distraction osteogensis (MDO) has been described as an effective method of managing severe upper airway obstruction in selected patients. This study describes the outcome of the management of a sample of neonatal & infants who have undergone mandibular distraction. Methods: A retrospective review of the medical records of 73 patients under 5 years of age, who had undergone MDO between 2000 - 2012, was undertaken. Parameters recorded included airway status, feeding, laryngoscopy, time of intubation, medical conditions, weight and complications. Results: Of the 73 patients 42 were syndromic (TCS, Stickler, Craniofacial Microsomia & others). The mean age of distraction was 2.0 months for non-syndromic, 3.3 months for syndromic patients. Initial management with nasopharyngeal tubes (NPT) was performed in 69 %, non-invasive therapy 18% (prone positioning, CPAP) and 9 syndromic patients (13%) had tracheotomies. Post-distraction none of the NPT or CPAP-dependent patients required any further airway support. Of the tracheostomy dependent group, 56 % were successfully decannulated within one year. Most patients required supplemented feeding and 77% were oral feeding by the end of the first year. The majority of syndromic PEG patients were still being fed by PEG at 1-year. The average growth of patients followed within 1-centile of their birth projected trajectories but 10% of syndromic patients had a low birth weight. Two patients died at home: at tracheotomy tube change and aspiration & respiratory arrest. Conclusion: MDO is a predictable and effective technique for managing severe UAO in selected patients. Differences in the outcomes of the syndromic and non-syndromic patients were demonstrated. Although successful, MDO may not be appropriate for all syndromic patients as long-term airway management with tracheosotomy may still be indicated.

Background: Paediatric odontogenic myxoma (OM) is a rare pathological condition in the oral and maxillofacial region. There has been much debate in the literature regarding the preferred method of treatment; however due to the rare nature of this disease, definitive algorithms of management are yet to be determined.

Introduction: Treatment protocols for the skeletal management in patients with syndromic craniosynostoses usually involve either a fronto-orbital or monobloc advancement or posterior vault distraction during infancy to alleviate raised intracranial pressure. Advancement at the Le Fort III level by distraction techniques to address the deficient mid-face is also commonly undertaken during the prepubertal period to address relative globe proptosis, to miminize the Class III jaw relationship and to resolve obstructive sleep apnoea if present. Following post-pubertal growth, recurrent mid-facial retrusion may require further definitive corrective procedures towards skeletal maturity for the best outcomes. The modified Le Fort III (MLFIII) advancement, where the nasal complex is excluded from the segment, has proved to be a useful procedure in this cohort of patients to finally position the orbital margins as well as the maxillary segment for occlusal correction. The purpose of this paper is to evaluate patients who have undergone this procedure in the Melbourne Craniofacial Unit with respect to the indications for treatment, the timing of surgery and outcomes. Methods: Patients who had undergone a MLFIII advancement were identified from the database in our Unit. Primary surgical intervention and interim mid-facial advancements performed and any subsequent procedures were recorded. Intraoperative and postoperative complications were recorded and an assessment of the final occlusion and aesthetics was undertaken. Results: Ten patients underwent a MLFIII advancement (Crouzon syndrome-7, Saethre-Chotzen syndrome-1, Achondroplasia-1 and Acromegaly-1) There were 6 males and 4 females. The age range was 7– 21 years, with a mean age 17 years. Four patients underwent a fronto-orbital advancement and one monobloc in infancy and a pre-pubertal Le Fort III advancement in 3 patients. Final cranioplasties / septo-rhinoplasties were performed in 6 patients. There were no major complications but 5 patients had persistent infraorbital paraesthesia and in 2 patients there was residual asymmetry of zygomatic projection. All patients were satisfied with the aesthetic results related to orbital proptosis. Conclusion: The MLFIII advancement is a versatile procedure that achieves final positioning of the infraorbital/zygomatic prominence and thus corrects persistent mid-facial hypoplasia and relative orbital proptosis.

Background: Craniofacial microsomia (CFM) is one of the most common congenital conditions treated in craniofacial centers worldwide. This condition is variably associated with anomalies of the jaws, ears, facial soft tissue, orbits, and facial nerve function and can be associated with extracranial anomalies.

The aim of this study was to evaluate the long-term survival of craniofacial implants and prostheses and to identify factors associated with failure in a cohort of patients. A 25-year retrospective analysis was conducted at Royal Melbourne Hospital. Data included demographic characteristics, age, site and cause of the deformity, and number and survival of implants. Odds ratios were calculated and event-to-time Kaplan-Meier analyses performed. One hundred and ten patients were included (341 implants); their mean age was 46.2 years. The overall implant survival rate was 79.5% (mean follow-up 10.6 years). Temporal implants had the highest success rate (97.0%), followed by nasal implants (87.5%) and orbital implants (63.3%); differences were statistically significant (P < 0.0001 and P = 0.033, respectively). Kaplan-Meier analyses to determine long-term implant and prosthesis survival found temporal implants had the highest prosthetic (P < 0.0001) and implant survival (P < 0.0001). Patients with congenital deformities demonstrated the highest success rate. Radiotherapy was found to increase the risk of implant failure (P = 0.02). Craniofacial implant-retained prostheses are a reliable and effective option for the restoration of facial defects, with good long-term success rates. Orbital implants and those placed post oncological surgery have a higher failure rate.

Background: Vascular disorders of the maxillofacial skeleton vary from benign localised lesions to aggressive, proliferative high-flow malformations that present enormous challenges for the clinician. Objectives: This presentation will describe the classification of vascular malformations within the maxillofacial complex, their characteristics and management. The distinction between arteriovenous malformations (AVM) and haemangiomas will also be highlighted to emphasise that these lesions are separate pathological entities. Methods: A retrospective review of patients (adults and paediatric patients) with a diagnosis of a vascular lesion of the facial skeleton was undertaken in an attempt to classify the main categories. The implications for elective and curative surgery were to be considered and treatment protocols outlined. Findings: Of 13 patients with AVM's, 6 were syndromic low-flow arteriovenous malformations, 4 were nonsyndromic low-flow lesions and 3 were high-flow malformations. Sturge-Weber syndrome is a phakomatosis due to an embryonal developmental anomaly (mutations in the GNAQ gene) with several subtypes. Nonsyndromic low-flow intraosseous lesions can be incidentally diagnosed on routine imaging and some may present with contour deformities or the displacement of teeth. However, high-flow lesions are rare and potentially life threatening. They may present with a pulsatile, expanding bony cortex, interdental bleeding or massive socket haemorrhage. For low-flow lesions, resection with a small margin may be sufficient for resolution of the lesion. However, high-flow lesions may require embolisation, sclerosing solutions, surgical resection or a combination of these interventions. A number of cases will be presented to illustrate the presentation of AVM's and the approach to management.

Background: Infants and children with craniofacial anomalies often present with upper airway obstruction (UAO) due to micrognathia. Objectives: This study describes the outcome of the management of neonatal, infant and young children who have undergone mandibular distraction for the management of severe UAO. Methods: A retrospective review of 73 patients who underwent mandibular distraction osteogenesis (MDO) between 2000 and 2012, was undertaken and included airway status, feeding, postoperative course including time of intubation, medical conditions, weight and any complications. Findings: 42 were syndromic and 31 were nonsyndromic. The mean age of distraction was 2.0 months (nonsyndromic) and 3.3 months (syndromic). Initial management with nasopharyngeal tubes (NPT) was performed in 69% whereas 18% had noninvasive therapy and 9 syndromic patients had tracheotomies. Post-distraction, none of the NPT/continuous positive airway pressure-dependent patients required any further airway support. Of the tracheostomy dependent group, 56% were successfully decannulated within one year. Most patients required supplemented feeding prior to MDO, 77% were oral feeding by the end of the first year. The majority of tracheotomy patients were still percutaneous endoscopic gastrostomy fed. The average growth of patients followed within one centile of their birth projected trajectories but 10% of syndromic patients had a low birth weight. Two patients died at home: during tracheotomy tube change and following aspiration and respiratory arrest. Conclusion: MDO is an effective technique for managing severe UAO in selected infants and young children. Differences in the outcomes of the syndromic and nonsyndromic patients were demonstrated. In some syndromic patients management with tracheostomy is still indicated.

Secondary alveolar bone grafting is a key procedure in comprehensive cleft services, the main objective of which is to allow the canine to erupt through the bone. We have assessed the outcomes of the procedure at two units, one in Australia and one in the UK. Success was measured using standardised indexes for radiological assessment (Bergland and Kindelan) and clinically by noting eruption of canines through the grafted sites. The two-year review indicated that the two units had comparably high success rates. Canines erupted through cleft sites in 27/28 sites in patients in the UK, and 26/28 in patients in Australia, and the radiological success using the indexes was also high. These rates are in line with international benchmarks.

High-flow vascular malformations in the paediatric population are potentially life-threatening and are challenging to treat. This paper describes the management of three cases of mandibular arteriovenous malformations and reviews the contemporary management options for these serious lesions.

We read with interest the article by Lin et al., “Comparison of different hypotensive anaesthesia techniques in orthognathic surgery with regard to intraoperative blood loss, quality of the surgical field, and postoperative nausea and vomiting”. 1 Lin S. Chen C. Yao C.F. Chen Y.A. Chen Y.R. Comparison of different hypotensive anaesthesia techniques in orthognathic surgery with regard to intraoperative blood loss, quality of the surgical field, and postoperative nausea and vomiting. Int J Oral Maxillofac Surg. 2016; 45: 1526-1530 Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar It provoked a lot of discussion and an audit of our practice. The low incidence of postoperative nausea and vomiting (PONV) was particularly notable. In a group of similar cases of similar age and sex ratio, the incidence of PONV requiring intervention in the post anaesthesia care unit (PACU) was 35%, comparable to the sevoflurane group in the study.

The spectrum of pathology affecting the craniomaxillofacial skeleton in the young patient includes a range of benign, aggressive pathological entities that require resection with an adequate margin to reduce the risk of recurrence. Malignant craniofacial lesions in the paediatric patient differ from the adult in that they are usually sarcomas of hard and soft tissues, and are relatively rare. Giant cell lesion and unusual lesions such as infantile mandibular fibromatosis and juvenile mandibular chronic osteomyelitis are disorders where the biological behaviour is not predictable and treatment may vary from conservative to ablative surgery. Similarly, the treatment of fibro-osseous lesions may include observation, contouring or resection. Ameloblastomas and malignant tumours require resection with a margin. Unlike adult malignancy, multimodal therapy is required for sarcomas of the jaws in paediatric patients and reconstruction of the resultant defects must be planned for a full life-span. Iliac non-vascularised grafts or rib may readily reconstruct “short-span” mandibular segmental resections. For larger maxillary and mandibular segments including tooth-bearing segments, free vascularised grafts provide the most reliable reconstruction (fibula/ilium). Where possible, facial skin incisions are best minimised in young patients to avoid unnecessary aesthetic compromise from potentially poor scarring. Surgical access for resection can be often achieved intraorally with access to recipient vessels gained by limited incisions in a skin crease. The “neo-alveolus” needs optimal orientation and sufficient prosthetic space for future implant-supported bridges. Hemimandibular reconstructions are often too laterally placed making rehabilitation difficult or impossible. The purpose of this presentation is to review the spectrum of jaw lesions in the context of the paediatric population and to give our Unit's approach to management.

Care of the patient with cleft lip and/or palate remains complex. Prior attempts at aggregating data to study the effectiveness of specific interventions or overall treatment protocols have been hindered by a lack of data standards. There exists a critical need to better define the outcomes-particularly those that matter most to patients and their families-and to standardize the methods by which these outcomes will be measured. This report summarizes the recommendations of an international, multidisciplinary working group with regard to which outcomes a typical cleft team could track, how those outcomes could be measured and recorded, and what strategies may be employed to sustainably implement a system for prospective data collection. It is only by agreeing on a common, standard set of outcome measures for the comprehensive appraisal of cleft care that intercenter comparisons can become possible. This is important for quality-improvement endeavors, comparative effectiveness research, and value-based health-care reform.

Rhinoplasty is one of the most commonly performed procedures in facial aesthetic surgery and has generated an enormous literature. The secondary cleft nasal deformity is often the most prominent feature of an individual with a cleft anomaly and its correction is widely regarded as one of the most challenging procedures in which to achieve consistently good results. While some cleft surgeons recommend correction of the nasal tip during primary surgery, others prefer that nasal surgery be confined to the adolescent years when growth is almost complete. At this stage, key anatomical defects, such as displacement of the lateral crus of the alar cartilage are then stable and can be definitively repositioned and sculpted to a more normal position. An analysis of the deformity and an appreciation of the action of the muscular forces associated with the cleft explain the malposition of the tissues observed. In unilateral cases, the septum deviates to the greater segment and there is lateral displacement of the lateral crus. Septal deflection may extend posteriorly in continuity with the vomerine septum and may result in complete posterior obstruction. The markedly deviated nasal septum is also often accompanied by a hypertrophied turbinate on the concave side and relative obstruction on the convex side due the close proximity of the adjacent contralateral turbinate. The keystone for correcting a deviated nose and for providing sufficient support is the construction of a symmetrical robust septum. The bilateral cleft nasal deformity is more symmetrical but the nasal tip is drawn ventrally with a short columella. There is a lack of tip projection due to the depressed alar cartilages in addition to the flaring of the nasal alae that have a more horizontal orientation. In this presentation, the key features of the cleft nasal deformity and the approach to surgical management will be highlighted with case presentations.

Paediatric odontogenic myxoma (OM) is a rare pathological condition in the oral and maxillofacial region. There has been much debate in the literature regarding the preferred method of treatment; however due to the rare nature of this disease, definitive algorithms of management are yet to be determined. A case series of eight paediatric patients with OM is presented. Six of the lesions were in the maxilla and two were mandibular lesions. The patients were aged between 2 and 18 years. Treatment ranged from excision and the application of Carnoy's solution to segmental resection and reconstruction. From this case series it can be seen that even in situations where treatment was limited to excision and the application of Carnoy's solution, no recurrences occurred. As such the present authors favour an initially more conservative approach to the management of these lesions where possible and reserving conventional resective treatment for recurrences, lesions causing pathological fracture, and those in regions that are difficult to access.

Background: Mandibular distraction osteogenesis (MDO) is an effective method of treating upper airway obstruction (UAO) in micrognathic infants. The short-term outcomes include relief of UAO, avoidance of tracheostomy, and prompt discharge from hospital. However, it is a significant surgical procedure with potential associated morbidities. This study describes a cohort of infants managed using MDO over a twelve-year period. Methods: A retrospective chart review was undertaken for children who had MDO before the age of 5 years between 2000 and 2012. This was followed by a clinical review of the same cohort specifically looking for dental anomalies, nerve injuries, and scar cosmesis. Results: Seventy-three children underwent MDO at a mean age of 2 months [interquartile range (IQR), 1.7–4.2] for nonsyndromic infants and 3.3 months (IQR, 2.1–7.4) for those with syndromes. Infants were discharged from hospital, on average, 15 days after procedure. After MDO, of the 9 who were previously tracheostomy dependent, 5 (56%) were decannulated within 12 months and none of the nontracheostomy-dependent children required further airway assistance. The majority of children required supplemental feeding preoperatively but, 12 months postoperatively, 97% of the nonsyndromic infants fed orally. Thirty-nine children (53%) were reviewed clinically [median age, 5.1 y (IQR, 3.9–6.5)] with 18 being syndromic. Many of the mandibular first permanent and second primary molars had developmental defects, but there was a low rate of neurosensory deficit and good scar cosmesis. Conclusions: This study contributes further to the evidence base underpinning the management of micrognathic infants with UAO.