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Alcohol is a recognized teratogen, and alcohol exposure increases the risk for hemifacial microsomia (HFM) of the fetus during maternal pregnancy. The present study aimed to explore potential mechanisms and verify hub genes of HFM associated with alcohol by bioinformatics methods.First, HFM and alcohol pathogenic genes were obtained. Thereafter, a protein-protein interactional (PPI) network was constructed. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analyses and molecular complex detection were performed by Metascape. Finally, we used the cytoHubba plugin to screen the hub genes.A total of 43 HFM genes and 50 optimal alcohol candidate genes were selected. The PPI networks for pathogenic genes contained 93 nodes and 503 edges. Functional enrichment analysis largely focused on tissue formation and development. Two modules were identified from the PPI network, and 10 hub genes were screened out. The genes most relevant to alcohol-induced HFM pathogenesis included CTNNB1, TP53, MYC, HDAC1, and SOX2.This study identified some significant hub genes, pathways, and modules of HFM related to alcohol by bioinformatics analyses. Our results suggest that the CTNNB1, TP53, MYC, HDAC1, and SOX B1 gene subfamilies may have played a major role in alcohol-induced HFM.

Hemifacial microsomia (HFM), which involves multiple sites with different levels of severity, is the second most common congenital craniofacial deformity after cleft lip and palate. However, three-dimensional (3D) measurements of mandibular deformities have not yet been studied in detail. The objective of this study is to investigate the method of 3D measurements of mandibular deformities in HFM patients.A total of 48 HFM patients were included in this study. All clinical treatment for patients was performed in the Plastic Surgery Hospital of the Chinese Academy of Medical Sciences at Peking Union Medical College from June 2006 to June 2020. The patients' 3D computerized tomography scan data were processed using medical imaging software, following four iterative steps: 3D reconstruction, mirroring, differential analysis, and partition.The characteristics of the mandibular bone in HFM patients are mainly presented as follows: (1) compared to the normal side, the part of the bone body that extends from the ascending ramus to the pogonion (Po-NB) is analyzed using a dynamic process: less fullness-fullness-more fullness; (2) absences were frequently observed among the angular zones, that is, the height of the ascending ramus is deficient.HFM is a complicated condition with numerous variations in clinical presentation. We employed both 3D image reconstruction and computerization image processing techniques to investigate asymmetrical mandibular deformity in HFM patients in detail and with great accuracy. This will be of great use to clinicians for disease management.

The treatment for severe hemifacial microsomia (HFM), especially type III case, is extremely difficult. Mandibular distraction osteogenesis (MDO) was rarely used as the primary choice in the treatment of severe type cases. The authors sought to observe the short-term therapeutic outcomes of patients with severe unilateral HFM who underwent MDO first.A retrospective study of children underwent MDO or later received costochondral graft (CCG) for severe unilateral HFM from 2009 to 2019 was conducted. Cephalometric measurements and clinical variables were analyzed to evaluate the effectiveness of MDO first strategy for severe cases and compare disparity between Pruzansky-Kaban classification type IIb and type III groups.Thirty-six patients (23 males and 13 females) underwent MDO first for severe HFM were included for analysis in the present study. The average age at MDO was 8.33 ± 2.03 years. At the last follow-up, MDO acquired significant improvement in mandibular height, maxillary cant, chin deviation, lip commissural line tilt, and clinical chin deviation (P 0.05). Distraction results were stable during the short-term follow-up in terms of the mandibular height ratio and maxillary cant (P 0.05).MDO is a proper primary method for suitable type IIb and type III HFM cases. MDO can immediately and significantly improve the facial skeleton deficiency, extend the associated soft tissue at the same time, and lay foundation for secondary surgery. MDO can achieve the downgrade of HFM deficiency severity. MDO followed by costochondral graft can get satisfactory esthetic and structural consequence for type III patients.

A European guideline on craniofacial microsomia was developed within the European Reference Network for rare and/or complex craniofacial anomalies and ear, nose, and throat disorders and published in 2020. The guideline provides an overview of optimal care provisions for patients with craniofacial microsomia and recommendations for the improvement of care. This document seeks to provide a tailored overview of this guideline for patients and their families.

This is a retrospective study to evaluate the postsurgical position of the maxilla and mandible in 5 matured craniofacial patients with unilateral craniofacial microsomia who underwent 2 jaw surgical procedures using computerized surgical planning. The craniofacial surgeon and orthodontist completed the virtual surgical treatment plan with a biomedical engineer's assistance via a web meeting. The treatment plan of each patient included 2 jaw surgery with genioplasty. At the maxillary dental midline, the planned mean advancement was 4 mm; yaw, a rotational correction towards the unaffected side was 4.96 mm; and impaction was 2.74 mm. The mean advancement measured at point B was 10.5 mm, and the rotational correction towards the unaffected side was 6.58 mm. The mean advancement following genioplasty was 8.43 mm, and the mean transverse correction was 6.33 mm towards the midsagittal plane. The intermediate surgical splint, final surgical splint, bone graft templates, and cutting guides were constructed utilizing computer-aided design/computer-aided manufacturing technology. The surgeon executed the treatment plan in the operating room using appropriate computer-generated guides and splints. A postsurgical cone-beam computed tomography scan was obtained and superimposed on the surgical treatment plan using Simplant OMS 10.1 software. The cranial base was used as a reference for superimposition. Three-dimensional color-coded displacement maps were generated to visually and quantitatively assess the surgical outcome. There was a mean error of 0.88 mm (+0.30) for the position of the maxillary anatomical structures from the planned position, and the anterior mandibular anatomical structures were on average 0.96 mm (+0.26) from the planned position.

Temporomandibular joint (TMJ) reconstruction with alloplastic prosthesis is a commonly performed procedure, which can be used as a treatment for improving pathologic conditions affecting the TMJ. Depending of the severity of the condition, the patient's ability to eat can be impaired, which in turn can affect their quality of life. Several syndromes can affect the patient's facial features, and some of those also affect TMJ's. Use of alloplastic prosthesis in TMJ's affecting syndromes, by means of correcting TMJ conditions and mandibular deformities, is still rather uncommon. Purpose of our study is to review the use TMJ total prosthesis as a treatment modality for correcting TMJ conditions and the reconstruction of mandibular deformities in congenital syndromes affecting the facial features. This study consists of a retrospective cohort of patients treated with TMJ reconstruction with alloplastic prosthesis at the Helsinki University Hospital during the past decade. All subjects with congenital syndrome who underwent TMJ reconstruction were included the study. The cohort consisted of seven patients and ten joints treated. Temporomandibular joints affecting syndromes were Goldenhar syndrome, hemifacial macrosomia, Nager syndrome, and Treacher-Collins syndrome. The majority of the patients have had several previous operative treatments on the facial skeleton during their childhood, which in turn probably affected and compromised the TMJ surgery outcome. Mouth opening was sufficiently achieved as average maximal opening was 34 mm (range 24-42 mm) postoperatively. Temporomandibular joint reconstruction with alloplastic prosthesis can offer new tools and approaches for treatment of facial deformities in syndromes with craniofacial abnormalities. Impaired jaw functions and asymmetry can be addressed with the use of alloplastic prostheses, achieving sufficient mouth opening and restoring the symmetry of the lower facial skeleton.

Hemifacial microsomia is the second most frequent pathology in craniofacial malformations. Clinical findings are broad, mainly affecting the mandible. Several classifications of mandibular compromise exist that guide the best treatment option in each patient. The authors present a case of an unusual complication following fibular free flap mandibular reconstruction in a patient with hemifacial microsomia prada type IV, who presented with ankylosis at the skull base and simultaneous fibula pseudoarthrosis at the union with the residual mandible. These dual findings allowed the patient to have a functional mouth aperture, which give us time to let him grow and wait for final management. Treatment options and follow up are discussed, knowing that there is no literature to support any protocol with this patient, so we present his evolution.

INTRODUCTION Craniofacial microsomia (CFM) is caused by abnormalities in the development of the first and second pharyngeal arches. One-third to half of the patients with CFM also present with extra craniofacial (ECF) malformations. The knowledge of the visceral alteration related to CFM is vital for optimized care and a better prognosis. AIM To describe the incidence of ECF malformations in patients with CFM and to infer if there was a correlation between CFM and ECF malformations. MATERIALS AND METHODS The authors analyzed medical records of patients diagnosed with CFM from 1996 to 2006. The data collected included age, gender, category of craniofacial alteration, and the type of ECF malformation when present. The sample was inspected to find possible correlations between craniofacial abnormalities and ECF malformations. RESULTS The sample included 102 patients, with a mean age of 7 years and a predominance of males (61.8%). Ear malformations (93.1%) followed by mandible (59.8%) and facial nerve (10.8%) abnormalities were the most common CFM. Among patients with CFM, 37.2% had ECF involvement, mainly in vertebrae (20%), heart (11%), and limbs (9.8%). Multivariate analysis revealed that the presence of ear malformations was related to a higher incidence of nonspecific visceral malformations (P = 0.034) and that mandible malformation was related to an increased incidence of vertebral malformations (P = 0.008). CONCLUSION A significant percentage of patients with CFM presented associated ECF impairment. Ear and mandible involvement may be predictors of nonspecific visceral malformation and vertebral malformations, respectively.

SUMMARY Congenital heart disease (CHD) is one of the most common combined malformations of microtia. There is currently no specific study that investigates the relationship between microtia and CHD. METHODS This study collected microtia inpatients admitted from May 1, 2015 to July 31, 2016. The diagnosis of CHD was based on patient's symptoms, past history, and echocardiography. Pearson χ2 test was used to analyze the correlation between CHD and microtia. RESULTS A total of 30 cases (3.35%) were documented with CHD, including atrial septal defect (12/40.00%), ventricular septal defect (7/23.30%), patent ductus arteriosus (2/6.70%), complex congenital heart disease (3/10.00%), combined CHD (2/6.70%) and other malformations (4/13.30%). Analysis showed no statistically significant relation between CHD and the side of affected ear or gender. CONCLUSIONS The occurrence of CHD in microtia patients was higher than that in the general population. The relationship between them was explored mainly from the etiological perspective. Microtia and CHD were often combined in syndromes such as Goldenhar syndrome, 22q11 deletion syndrome, and CHARGE syndrome. Absence of genes or abnormal embryo development associated with these syndromes leads to the occurrence of both.

The purpose of this study is to describe the management of 2 dimorphic patients affected by Hemimandibular Hypoplasia with Condylar-Coronoid Collapse (HH-CCC), also called Pseudo Hemifacial Microsomia, where the orthopedic treatment gave an excellent long-term follow-up. The patients were a 7-year-old female and a 6-year-old male with a HH-CCC on the left side, an asymmetrical face with chin deviation, class II dental malocclusion and canting of the occlusal plane. An X-ray evaluation and clinical observation confirmed the unilateral mandibular deficiency and the collapse of the condyle on the coronoid process on the affected side. The treatment plan consisted of the use of an asymmetrical functional appliance with a vertical screw. The appliance was gradually activated on the vertical plane on one side by a screw incorporated on an acrylic plate. It was used for 22 hours/day, including sleep time but not during meals. This treatment improved the patient' s facial appearance. HH-CCC showed a positive response to functional therapy.

BACKGROUND The cost-effectiveness, utility outcomes, and most optimal timing of mandibular distraction osteogenesis (MDO) in craniofacial microsomia (CFM) continue to be a topic of debate, especially in the population of patients with mild or nonsignificant functional issues. The objective of this study was to quantitatively assess the burden of mandibular asymmetry in CFM, and to accurately evaluate the impact of early MDO on patients' perceived quality of life and social acceptance, in addition to examining the cost-effectiveness of early MDO in CFM. METHODS A validated crowdsourcing platform was utilized to recruit participants. Psychosocial acceptance and utility outcomes were assessed for patients with CFM. Participants were presented with health-state scenarios supplemented with pre- and postoperative images. Quality-adjusted life years (QALYs) were subsequently calculated and costs were estimated based on Medicare fee schedules using the Current Procedural Terminology codes. Incremental cost-effectiveness ratios for early MDO were calculated and plotted against a $100,000/QALY threshold. RESULTS: A total of 463 participants were included in the study. The mean visual analog scale score for untreated mandibular hypoplasia in CFM was 0.48 ± 0.24, which improved significantly (P < 0.0001) to 0.63 ± 0.20 following early MDO. Time trade-off scores for an imaginary surgery leading to perfect health with no complications were not statistically different from undergoing early MDO (P = 0.113). Early mandibular distraction decreased social distance in all 8 social situations assessed. Incremental cost-effectiveness ratios varied by the duration of time between early MDO and a potential second intervention, ranging from $148,142.09 per QALY gained at an interval of 1 year to $9876.14 per QALY gained after 15 years. Using a willingness-to-pay threshold of $100,000/QALY, early MDO in CFM becomes cost-effective when patients enjoy an improved health-state post-MDO for more than 1.48 years before a potential second intervention, if deemed necessary. CONCLUSION Early mandibular distraction may lead to tangible positive gains in CFM patients based on utility outcome scores, psychosocial acceptance, and social distance. Therefore, although further interventions may be needed at skeletal maturity, early MDO can improve the psychological well-being of CFM patients during their crucial developmental years in a cost-effective manner. The incremental cost per QALY gained post-early MDO correlates negatively with time until a second potential surgical intervention.

Computer-aided surgery has been widely used in treatment of hemifacial microsomia and matured in recent decades. These techniques include the computer-aided design, virtual surgical planning, modeling surgery, rapid prototyping techniques, intraoperative navigation and so on. The purpose of this article is to summarize the current application of computer-aided design/computer-aided manufacturing technology in the treatment for hemifacial microsomia during the last 5 years, as well as the views and discussions on some topics, and finally introduce a method of our team. Our effort is that using the holes predrilled in cutting guides, the pre-bent titanium plates are easily placed. Avoiding potential bone autorotation caused by unfitness between conventional titanium plates and bone surface, which keeps the bone fixation precisely in line with the preoperative virtual plan and reduce the movement of bone segments due to the undesirable stress of the plates.

Patients with craniofacial microsomia (CFM) may present with severely malformed mandibles resulting in secondary tongue-based airway obstruction. While surgical management of clinically significant airway obstruction in these patients often involves mandibular distraction osteogenesis (MDO), patients with Pruzansky III CFM typically require additional costochondral bone grafting. In this report, the authors present the successful surgical management of airway obstruction and severe obstructive sleep apnea using a cranio-mandibular fixator (Matthews device) combined with MDO in a 4-year-old female patient with Goldenhar syndrome and associated Pruzansky III CFM. The patient tolerated the procedure without complications and experienced a significant improvement in airway obstruction. With this approach, a severely deformed mandible can be successfully distracted while avoiding tracheostomy and disruption of native TMJ anatomy.

Craniofacial microsomia (CFM) is the second most common congenital craniofacial malformation characterized by asymmetric malformation of the ear and mandible. Numerous studies have reported the importance of child perspective and psychosocial issues in patients with craniofacial abnormalities. However, clinical tools to evaluate child and caregiver perspectives in patients with microtia with or without CFM have been limited or not reported in the literature. The authors aimed to (1) To develop a tool for measuring patient and caregiver evaluation of facial appearance as it relates to microtia and craniofacial microsomia (CFM). (2) To utilize this tool in comparing children, between 7 and 20 years of age, and caregiver perspectives towards facial appearance in patients with microtia with or without craniofacial microsomia (CFM). A prospective single center study conducted from 2016 to 2017 using newly developed 13-item (Microtia) and 27-item (CFM) 5-point Likert scale Likert scale questionnaires given to patients with CFM and caregivers at a craniofacial center. Aged 7 to 20 (N = 25) and their caregivers. A total of 25 patients (13 male, 12 female; mean age at time of survey 13.2 ± 3.7) met criteria for the study. The Likert scale developed and presented in this study may be a useful tool for clinical use in investigating patient and caregiver perspectives for planning surgical timeline. Based on our pilot data it is important to incorporate all voices into decision-making on timing.

This report describes a patient with Goldenhar syndrome undergoing anesthesia for whom Macintosh videolaryngoscopy failed, as the epiglottis was adhered to the posterior pharynx and could not be lifted with a tracheal introducer (Cormack-Lehane grade 3B). Hyperangulated videolaryngoscopy revealed only the arytenoids (Cormack-Lehane grade 2B), even after direct lifting of the epiglottis, and endotracheal tube advancement failed due to unclear tissue resistance. Hyperangulated videolaryngoscopy was combined with a tube-mounted camera (VivaSight single lumen tube). The combination of both camera perspectives was successfully used to allow placement of the endotracheal tube underneath the epiglottis and through the vocal cords.

Craniofacial microsomia (CFM) is a variable craniofacial malformation, related to the development of the structures originated from the first and second brachial arches, affecting skeletal tissue, soft tissue, and neuromuscular components. In the situation of subcutaneous tissue and chewing muscles hypoplasia, free tissue transfer is a treatment option. Dermal-fat graft allows easy modeling during surgery, volumetric gain and improvement of asymmetry. The aim of this study was to evaluate the facial contour and the percentage of symmetry after the use of dermal-fat graft in patients with CFM, who had already submitted to osteotomies, attended at the Associate Center for Cleft Lip and Palate (CAIF) during 2001 to 2018. For analysis, the authors selected 17 patients who fulfilled the above prerequisites. The symmetry study was done by the analysis of preoperative and postoperative photographs in the Image J software. Two standard points were used: nasal base and upper lip limit. On the nasal base, the preoperative analysis showed a hypoplastic side with a median of 93.00% of the normal side size, rising to 97.78% in the postoperative period. On the upper lip limit, the preoperative analysis showed a median of 87.80% and, in the postoperative period, 98.15%. Analysis of the interaction between the operative moments and the modified Pruzansky classification showed that there were no significant differences between grades. Long-term evaluation demonstrated that the use of a dermal-fat graft for correction of facial symmetry was effective and close to 100%, regardless of the degree of hypoplasia of the patient.

The purpose of this study was to assess the objective and subjective outcomes of lower face volumetric (contour) asymmetry correction with isolated fat grafting in skeletally immature patients.A prospective analysis was conducted of skeletally immature patients (n = 73) with craniofacial microsomia and Parry-Romberg syndrome who underwent isolated fat grafting (with no previous or concomitant bone surgery) using anatomical surgical principles (facial subunits and fat compartments) for the reconstruction of lower face volumetric asymmetry. Objective ultrasound and photogrammetric lower face symmetry analyses were blindly performed preoperatively and at 3 and 12 months postoperatively. A panel assessment of blinded surgical professionals and laypersons was also obtained to grade the subjective lower face symmetry.There were significant (all P0.05) postoperative objective and subjective lower face symmetry enhancements (preoperativepostoperative) after isolated fat grafting, with no differences (all P0.05) between 3 versus 12 months' postoperative comparisons.Growing patients with unilateral lower face volumetric asymmetries presented with improvement of objective and subjective symmetry after a single isolated fat grafting procedure.

Introduction Hemifacial microsomia is the most common congenital disorder of the face after cleft lip and palate. While treatment of the bony abnormalities has been well documented, less attention has been paid to correction of the soft tissue deformity. This study aimed to systematically review the literature addressing the techniques utilized to correct the soft tissue deformity in hemifacial microsomia. Materials and methods A comprehensive review of peer-reviewed literature regarding the management of soft tissue reconstruction in patients with hemifacial microsomia was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. Odds ratio was used to assess differences in complication rates between reconstructions performed with free tissue transfer and fat grafting. Results The literature search yielded 38 articles that were fully reviewed for data extraction. Articles were grouped into one of 5 reconstructive modalities-pedicled flap, microvascular free tissue transfer, structural fat grafting, alloplastic implant, and functional reconstruction. Microvascular free tissue transfer had a complication rate of 27.1%, while structural fat grafting had a complication rate of 4.2% (odds ratio 6.7, P = 0.0003). Conclusions Microvascular free tissue transfer likely provides greater volume than fat grafting at the cost of a higher complication rate. Mild to moderate soft tissue deformity can be corrected with serial fat grafting. However, severe soft tissue deformity is better corrected with microvascular free tissue transfer. When performing free tissue transfer, one should be prepared for recipient vessel anomalies. To provide adequate augmentation with isolated fat grafting, multiple sessions are likely required.

Human interactions begin with near-instantaneous visual evaluation of the face, modifying emotional responses and social behavior toward the person. Eye-tracking technology can provide a potential window into how humans undertake this evaluation, and the threshold of whether a face containing a craniofacial difference is perceived as "normal." This study's main questions were whether gaze patterns differ from normal when evaluating children with craniofacial differences, and whether these gaze patterns vary with different levels of craniofacial deformity. Two experiments tested the feasibility of using eye tracking to study facial evaluation and whether there was a difference in the gaze pattern and characteristics, correlating with the Asher McDade Aesthetic Index. Fifty-three participants' eye movements were recorded as they gazed at photographs of children either with hemifacial microsomia or repaired cleft lip, with repaired cleft lip digitally corrected to symmetry, or with no craniofacial disorder. Recruitment and participation for this study occurred in a pediatric plastic surgery clinic and in a medical school student center. Participants gazed longer on the lip in the cleft-lip photographs compared with control photographs in the first experiment (207 ms; SD 75 versus 145 ms; SD 61, respectively; P = 0.04). This gaze bias was confirmed in the second experiment and found to correlate with severity (r = 0.042). Gaze patterns differ when individuals look at photographs with or without craniofacial differences. The degree to which these eye movement patterns differ correlates with the severity of craniofacial deformity.

Multiple or large distance mandibular distraction osteogenesis (MDO) in the older child is often complicated by iatrogenic temporomandibular joint (TMJ) pathology. The transmission of significant force to the TMJ in these particular patients is due to the greater distance of distraction required and the relative inelasticity of the soft tissue envelope. The authors present a clinical report of a successful asymmetrically vectored large distance MDO in a 13-year-old female with bilateral craniofacial microsomia with Goldenhar syndrome. During distraction, the TMJ joints were effectively unloaded from the forces of distraction using external bilateral cranial anchored devices (Cranio-Mandibular Fixator; KLS Martin, Jacksonville, FL). Angle's occlusion, facial angle, and evidence of TMJ pathology were assessed.

Objective Few epidemiological studies have investigated the external ear malformations anotia and microtia. The authors' study aimed to investigate the relationships between age, sex, type of microtia, and birth place and associated malformation as seen in 1 private Japanese clinic. Methods Cases of anotia/microtia that presented in Nagata Microtia and Reconstructive Plastic Surgery Clinic (Saitama, Japan) between 2005 and 2018 were included in the study. The authors identified 1896 patients with anotia/microtia with or without associated malformation. Most were primary cases, with some secondary reconstruction cases wherein the primary surgery was performed at another hospital. Cases were classified with Nagata classification; lobule type, small concha type, concha type, and anotia. Cryptotia was also observed in this study. Results Among the patients, 61.1% were male, 85.4% had unilateral defects (69.0% LB), and 59.1% had a right-sided defect. Most patients were less than 1 year old (15.0%) or 8 to 10 years old (5.5%-6.3%) on first examination, while 58% were from the Kanto region, including Tokyo. Regarding concomitant disorders, 32.7% had an accompanying malformation, while 13.3% had associated syndromes (eg, craniofacial microsomia, Treacher Collins syndrome). To conclude, in the authors' clinic, most cases of anotia/microtia were LB, unilateral, and seen in male patients. Information regarding several characteristic clinical features was obtained, especially that clefts and musculoskeletal deformities of the skull/face and jaw were the main accompanying malformations.

Background Controversy exists regarding the treatment of mandibular hypoplasia in craniofacial microsomia patients, notably the role of mandibular distraction osteogenesis. The authors compared the need for orthognathic surgery in skeletally mature craniofacial microsomia subjects who either did (study group) or did not (control group) undergo early mandibular distraction osteogenesis. Methods A retrospective review was conducted of all craniofacial microsomia patients evaluated between January of 1993 and March of 2017. This study included patients with a Kaban-Pruzansky grade I to III mandible, and who were at least 14 years old at the time of the latest follow-up. Results Thirty-eight subjects met inclusion criteria: 17 who underwent mandibular distraction osteogenesis and 21 who did not (mean age, 18.95 ± 2.82 years versus 17.95 ± 2.14 years, respectively; p = 0.246). The degree of mandibular deformity was matched (distraction, 29.4 percent Kaban-Pruzansky grade IIb and 5.9 percent grade III; no distraction, 23.8 percent grade IIb and 9.5 percent grade III; p = 0.788). No significant difference was noted between the distraction and no-distraction cohorts with regard to need for orthognathic surgery [distraction, n = 10 (58.8 percent); no distraction, n = 8 (38.1 percent); p = 0.203]. Conclusions The results seem to suggest that there is no significant difference in orthognathic surgery rates at skeletal maturity between craniofacial microsomia subjects who underwent early mandibular distraction osteogenesis and those who did not. Subjects who undergo distraction may still ultimately require orthognathic surgery to correct facial asymmetry. Additional studies are required to determine the optimal timing and technique of distraction, the importance of orthodontic management during and after distraction, and the early psychosocial benefits of improved facial symmetry. Clinical question/level of evidence Therapeutic, III.

Although facial paralysis is a fundamental feature of hemifacial microsomia, the frequency and distribution of nerve abnormalities in patients with hemifacial microsomia remain unclear. In this study, the authors classified 1125 cases with microtia (including 339 patients with hemifacial microsomia and 786 with isolated microtia) according to Orbital Distortion Mandibular Hypoplasia Ear Anomaly Nerve Involvement Soft Tissue Dependency (OMENS) scheme. Then, the authors performed an independent analysis to describe the distribution feature of nerve abnormalities and reveal the possible relationships between facial paralysis and the other 4 fundamental features in the OMENS system. Results revealed that facial paralysis is present 23.9% of patients with hemifacial microsomia. The frontal-temporal branch is the most vulnerable branch in the total 1125 cases with microtia. The occurrence of facial paralysis is positively correlated with mandibular hypoplasia and soft tissue deficiency both in the total 1125 cases and the hemifacial microsomia patients. Orbital asymmetry is related to facial paralysis only in the total microtia cases, and ear deformity is related to facial paralysis only in hemifacial microsomia patients. No significant association was found between the severity of facial paralysis and any of the other 4 OMENS anomalies. These data suggest that the occurrence of facial paralysis may be associated with other OMENS abnormalities. The presence of serious mandibular hypoplasia or soft tissue deficiency should alert the clinician to a high possibility but not a high severity of facial paralysis.

Craniofacial microsomia covers a set of morphogenetic anomalies that affect structures arising from the first and second brachial arches. Due to the vast phenotypic variation and complexity of the malformation, a global treatment that is coordinated by a multidisciplinary team is imperative. Herein, the authors describe 3 clinical patients and discuss the different therapies used according to the type of microsomia present. It was concluded that early and integrated treatment, which considers all the affected and potentially affected soft and hard tissue, is needed, and that patients must be monitored until they have finished growing.

Aim The aim of this study is to present the middle fossa technique (MFT) as an alternative for patients who cannot undergo traditional surgery for active transcutaneous bone conduction implants (ATBCI) due to their altered anatomy or desire for future aesthetic reconstruction. Design A case series descriptive study was designed. The MFT was developed. Preoperative and postoperative information from 24 patients with external auditory canal atresia (EACA) and implanted with ATBCI was reviewed. Results A total of 24 children with bilateral EACA received implants in the middle cranial fossa. Their average age was 12. Of these patients, eight had an associated congenital disorder: Goldenhar Syndrome, Treacher Collins Syndrome or the Pierre Robin Sequence. The average follow-up was at 17 months (ranging from between 2- and 36 mo) and there were no major complications. Four patients showed skin erythema at the processor site after turn on, which was solved by lowering the magnet strength. One patient had a scalp hematoma that required puncture drainage. The hearing thresholds went down on average from 66.5 to 25.2 dB 1 month after turn on. Speech recognition improved respectively from 29.4% without and 78.9% with a bone conduction hearing aid to 96.4%. Conclusion MFT placement of the ATBCI was proven to be safe and effective and a viable option for treating pediatric patients with EACA who cannot receive implants at the sinodural angle or in the retrosigmoidal position because of their altered anatomy and/or desire for future aesthetic reconstruction.

Accessory auricles are rare congenital abnormalities embryologically derived from the first branchial arch. Presentation may be variable with locations grouped into anatomical zones based on the frequency of location found in the literature. This study reviewed the papers between 1981 and 2017. Findings included an association with syndromes including Goldenhar, VACTERL, Treacher-Collins, Townes-Brocks, and Wolf-Hirschhorn. Based on histological and embryological evidence, the term "accessory auricle" is best used as an umbrella term to define this difference. Finally, indications are provided for imaging and laboratory investigations to guide the physician treating patients with accessory auricles.

Le Fort II distraction with zygomatic repositioning introduced the ability to restore central midfacial height and convexity independent of changes in orbital morphology. This study analyzes midfacial and orbital morphology before and after Le Fort II distraction with zygomatic repositioning.All patients who underwent Le Fort II Distraction with zygomatic repositioning between 2013 and 2015 were included. Two- and 3-dimensional measurements were made using 3dMD Vultus software to assess canthal tilt, nasolabial angle, ratio of midfacial to lower facial height, and absolute change in nasal length. Presence of an open bite and Angle classification were assessed before and after surgery.Four patients underwent segmental midface advancement using Le Fort II distraction with zygomatic repositioning. Associated diagnoses included Apert syndrome, Goldenhar syndrome, and achondroplasia. Changes in facial dimensions included: 3.19° improvement in canthal tilt (range -4.7° to 8.4°), 9° change in nasolabial angle (range -1.0° to 19°), and 0.69 cm increase in absolute nasal length (range 0.2-0.94 cm). Mean ratio of midfacial to lower facial height was 0.79 preoperatively and 0.89 postoperatively. Preoperatively, all patients demonstrated Angle class III with 3 of 4 patients demonstrating anterior open bite. All achieved closure of open bite and demonstrated class I or II occlusion. No complications were observed.Le Fort II distraction with zygomatic repositioning resulted in normalization of midfacial soft tissue landmarks. This form of advancement demonstrates the ability to selectively improve midfacial height and canthal tilt while restoring normal occlusion.

Background There is controversy regarding the treatment of young patients with unilateral craniofacial microsomia and moderate dysmorphism. The relative indication for mandibular distraction in such patients poses several questions: Is it deleterious in the context of craniofacial growth and appearance? This study was designed to address these questions. Methods A retrospective review of patients undergoing mandibular distraction by a single surgeon between 1989 and 2010 was conducted. Patients with "moderate" unilateral craniofacial microsomia (as defined by Pruzansky type I or IIa mandibles) and follow-up until craniofacial skeletal maturity were included for analysis. Patients were divided into two cohorts: satisfactory and unsatisfactory results based on photographic aesthetic evaluation by independent blinded observers at the initial presentation and at the age of skeletal maturity. Clinical variables were analyzed to detect predictors for satisfactory distraction. Results Nineteen patients were included for analysis. The average age at distraction was 68.2 months and the average age at follow-up was 19.55 years. Thirteen patients (68.4 percent) had Pruzansky type IIA and six patients (31.6 percent) had Pruzansky type I mandibles. Twelve patients (63.2 percent) had satisfactory outcomes, whereas seven patients (36.8 percent) had unsatisfactory outcomes. Comparing the two cohorts, patients with satisfactory outcomes had distraction at an earlier age (56.4 months versus 89.8 months; p = 0.07) and a greater percentage overcorrection from craniofacial midline (41.7 percent versus 1.8 percent; p = 0.003). Conclusion Mandibular distraction is successful in patients with mild to moderate dysmorphism, provided that there is a comprehensive clinical program emphasizing adequate mandibular bone stock, proper vector selection, planned overcorrection, and comprehensive orthodontic management. Clinical question/level of evidence Therapeutic, III.

Patients with severely hypoplastic mandibles usually require condylar reconstruction. This study aimed to describe costochondral graft (CCG) for condylar reconstruction and report subsequent outcomes of these grafts in young children with Pruzansky/Kaban type IIB and type III mandibular hypoplasia. This study included 4 young children with type IIB and type III hemifacial microsomia treated with CCG to reconstruct the condyle at the Department of Oral and Maxillofacial Surgery in our hospital from March 2008 to March 2014. Radiographic measurements and clinical examinations were conducted. The mean age of patients at operation was 3.8 years, ranging from 2.8 to 5.3 years. The mean follow-up period was 43.5 months, ranging from 23 to 63 months. Functional improvement was observed in all patients. The ribs had grown in all patients to date. Three patients had clinically improved face appearance with no significant chin point deviation and canting of the occlusal plane. Although the other patient had partly improved face appearance compared with preoperative condition, he still showed clinically significant facial asymmetry and chin deviation. Our results showed that condylar reconstruction with CCG is a feasible method in the treatment of type IIB and type III hemifacial microsomia in young children. These results will provide early preliminary suggestions of growth and stability of CCG in patients

Surgical rehabilitation of orbital dystopia can be challenging. The authors demonstrate the effective use of spectacle lenses to visually correct misalignments of the globe and the orbit. Presented is a retrospective review of 4 patients undergoing aesthetic rehabilitation through use of spectacle lenses and in a number patients a cosmetic shell.Two patients with neurofibromatosis presented with inferior dystopia of the globe and orbit. A base-down prismatic lens applied to the spectacles in conjunction with a prosthetic shell successfully visually corrected the facial asymmetry and improved patients' aesthetic appearance. One patient with a history of traumatic retinal detachment, who did not want any surgical intervention, a "plus" (hypermetropic) lens was used to magnify the perceived image of an enophthalmic and phthisical globe, to enhance appearance and improve symmetry. In the fourth patient, with Goldenhar syndrome, the appearance of a hypotropia and concurrent esotropia was successfully treated with a Fresnel prism and a prosthetic shell.This case series illustrates the successful role of various refractive lenses often in conjunction with prosthetic shells in patients with reduced vision and orbital dystopia to improve facial symmetry. This conservative treatment is especially useful when surgery is not a desired or not considered a suitable option for the patient.

Goldenhar syndrome is characterized by the typical features of craniofacial microsomia (CFM) with the addition of epibulbar dermoids and vertebral anomalies. The aim of this study is to examine the objective differences between patients carrying a diagnosis of Goldenhar syndrome to those diagnosed with CFM. Thus, we performed an Institutional Review Board-approved retrospective chart review on all patients who presented with a diagnosis of CFM or Goldenhar syndrome from January 1990 to December 2012. Demographic, diagnosis, OMENS+ classification, accompanying diagnoses, and radiographic data were collected. For subjective analysis, subgroups were designed based on the diagnosis Goldenhar syndrome or CFM per history. For objective analysis, subgroups were designed based on the presence of epibulbar dermoids and/or vertebral anomalies. The cohorts were compared with respect to associated medical abnormalities and severity of CFM features. One hundred thirty eight patients met inclusion criteria. Epibulbar dermoids and vertebral anomalies were seen in 17% and 34% of the patients, respectively. Only 10 patients (7.2%) had both epibulbar dermoids and vertebral anomalies. The subjective "Goldenhar" group (N = 44, 32%) was found to have a higher percentage of bilaterally affected patients (P = 0.001), a more severe mandibular deformity (P =

OBJECTIVE The objective of this study was to identify the presence of gender and laterality bias in patients with hemifacial microsomia. METHODS We performed a systematic search of PubMed, EMBASE, Cochrane Central Register of Systematic Reviews, and Cochrane Central Register of Controlled Trials to identify trials published up to January 2014. Data extracted from the literature were analyzed using Review Manager 5.0.24. RESULTS Relevant data were extracted from 24 articles (1121 participants), and the finding revealed no significant difference in incidence according to gender with the male-to-female ratio being 1.09:1 (relative risk, 1.13; 95% confidence interval, 0.94-1.36; P < 0.00001; n = 908) There was no obvious difference in the laterality subgroup either, with the ratio of between the right and left sides of the head being 1.12 (relative risk, 1.08; 95% confidence interval, 0.94-1.24; P = 0.006; n = 977). CONCLUSIONS This meta-analysis reveals no difference in the incidence of hemifacial microsomia between male and female patients; in addition, there is no obvious difference in distribution between the left and right sides of the head.

Background Craniofacial microsomia encompasses a spectrum of diagnoses associated with variable degrees of mandibular hypoplasia, facial asymmetry, chin deviation, occlusal abnormalities, and potential airway compromise. This study presents one surgeon's experience with costochondral rib grafting for mandibular reconstruction in children with Pruzansky/Kaban type 2B and type 3 mandibular hypoplasia. Methods An institutional review board-approved retrospective chart review was performed of all patients with craniofacial microsomia who underwent costochondral rib grafting for mandibular reconstruction performed by the senior author (S.P.B.) at The Children's Hospital of Philadelphia from January of 1998 to September of 2013. Demographic information, surgical history, operative details, postoperative complications, and outcomes were recorded. Plain radiographs and preoperative and postoperative three-dimensional computed tomographic scans were reviewed. Results Two hundred fifty-five patients were diagnosed with craniofacial microsomia, and 22 patients met inclusion criteria. Twelve boys and 10 girls underwent grafting at an average age of 7.2 years. Thirty-three costochondral rib grafts were performed, 11 unilateral reconstructions and 11 bilateral reconstructions. Twelve hemimandibles were type 2B and 21 were type 3. No intraoperative complications were reported, and no incidence of graft resorption was noted. No additional procedures were required in 27 reconstructed hemimandibles (81.8 percent), whereas six (18.2 percent) required secondary distraction osteogenesis. Only one patient developed postoperative ankylosis. No malunion or nonunion was noted. Conclusion The approach described in this article allowed the authors to obtain reliably good results with costochondral rib grafting for type 2B and type 3 mandibular hypoplasia associated with craniofacial microsomia. Clinical question/level of evidence Therapeutic, IV.

Macrostomia is a rare facial cleft, with an incompletely described pathogenesis. This series highlights cases of isolated macrostomia presenting with several distinct phenotypes. We examine phenotypic differences in macrostomia patients, to further elucidate the etiopathogenesis.We performed a retrospective review of macrostomia patients evaluated during a 10-year period. Patient demographics and clinical features are reported.We identified 25 macrostomia patients (13M/12F). Right-sided macrostomia occurred in 15, left-sided macrostomia occurred in 6, and bilateral macrostomia occurred in 4 patients. Of the bilateral cases, 100% existed in isolation of craniofacial microsomia (CFM) or other craniofacial abnormalities. Twelve patients presented with macrostomia in isolation of CFM; in this subgroup, the male-to-female ratio was 1:1. Bilateral macrostomia was present in 33% of patients. Unilateral macrostomia occurred more often on the right (5:2). Phenotypes included simple unilateral or bilateral macrostomia (67%), macrostomia associated with severe diastasis of the cheek musculature (8%), macrostomia associated with lateral facial clefts (17%), and diastasis of cheek musculature without significant macrostomia (8%).Macrostomia seen in isolation of CFM presents in phenotypically distinct forms. It is unlikely that a single mechanism is responsible for this range of phenotypes. We believe that both intrauterine trauma and failure of fusion of the mandibular and maxillary processes secondary to an aberration in FGF8 function are responsible. Additionally, diastasis of facial musculature may result from delayed fusion and subsequent decreased mesodermal penetration of the mandibular and maxillary processes.

The purpose of this research was to simulate mandibular movement calculated from three-dimensional computed tomography (CT) data to determine the linear distractor position to correct mandibular deformities in a series of patients with hemifacial microsomia (HFM). Preoperative CT scans from 6 HFM patients were obtained and imported into a CT-based software program (Mimics) to produce three-dimensional images and data. After measurement of the mandibular deficiency in 3 dimensions (horizontal and vertical), the angle between the distraction device and the ramus was determined by a geometric calculation, and then the surgery was performed on the three-dimensional model constructed using the rapid prototyping technique. This planning method was finally used in the treatment of 6 HFM patients. The HFM was corrected, and a symmetrical facial contour obtained without any complications in these 6 patients. The results matched the expectation before surgery that the distractor elongation would range from 18 to 22 mm. The distraction tracing model fitted the actual postdistraction tracing to within 2 mm. The preoperative design and three-dimensional modeling technique are considered to be helpful in enhancing the predictability and improving the outcome of the treatment of distraction osteogenesis.

Various treatment strategies have been proposed to perform the aesthetic surgical correction of asymmetric deformities of the mandible. These techniques range from relatively simple to complex procedures including bimaxillary surgery associated with complex mandibular osteotomies. The authors describe a patient with grade III Goldenhar syndrome, treated by a "chin wing" mentoplasty as described by Triaca. These situations are classically treated with a bilateral sagittal splint osteotomy (BSSO) in combination with mentoplasty. However, because of a good occlusion with Angle's class I relation, slight imbalance of the occlusal plane with good function of the mouth opening, the patient refused to be treated with a BSSO, hence, a chin wing mentoplasty was performed. Size and stability of bone regeneration were evaluated by histological examination and dynamic-volume computed tomography (CT). Mature bone in the grafted areas was detected by histology and CT scan with stable results and a significant increase of facial aesthetics 1 year after surgery. The authors also demonstrated that the chin wing technique provided a little but significant breathing improvement as detected on CT scans and lateral X-ray cephalograms by measuring the breathing area. Chin wing mentoplasty is a moderately invasive technique that is able to improve the aesthetics of the face and patient breathing.

Hemifacial microsomia is the second most common congenital craniofacial malformation after cleft lip/palate with a wide variety of pathologic expression in jaws, skeletal components, ears, and soft tissues. Among the deformities, mandibular hypoplasia is the most common and is the main component that affects facial asymmetry. Mandibular distraction osteogenesis is the mainstay treatment; however, the vector of device and osteotomy lines need to be well designed. We utilized the sagittal split osteotomy for mandibular distraction with rapid prototyping surgical guide plate, making a successful outcome.Hemifacial microsomia with unilateral Pruzansky II mandibular hypoplasia were selected in this study. Three-dimensional CT reconstructive data was put into Proplan CFM for preoperative designing and then manufacturing the surgical guide plate. The mandibular osteotomy and implantation of the internal distractor were performed through an intraoral approach aided with the prefabricated guide plate. Distraction began 7 days postoperation with a frequency of 1 mm/d and the distractor was kept in place 6 to 10 months after the first operation, then the distractor was removed.From July 2012 to March 2014, 6 cases of Pruzansky II hemifacial microsomia aged from 7 to 11 years were treated with the technique mentioned above. The range of distraction extends from 20 to 30 mm. The facial asymmetry deformities were improved obviously and without any complication.Mandibular distraction osteogenesis by sagittal split osteotomy through rapid prototyping surgical guide plate provides certain advantages in the treatment of hemifacial microsomia.

Background The purpose of this study was to evaluate the results of long-term follow-up in patients with relatively severe unilateral craniofacial microsomia after mandibular distraction. Methods The sample consisted of 26 patients with an average age of 6.08 years at the time of distraction. All patients had nonsyndromic unilateral craniofacial microsomia (Pruzansky-Kaban types IIA, IIB, and III). Follow-up for all patients continued until the completion of growth. The 26 clinical records and posteroanterior cephalograms of the patients, taken before distraction (time 0) and approximately 1, 4, and 11 years (time 1, time 2, and time 3, respectively) after distraction, were used. Four items (i.e., supraorbital tilting angle and occlusal tilting angle to the horizontal reference line, and maxillary and mandibular tilting angles to the vertical reference line) were analyzed at each of the four time intervals. Results The overall mean distraction amount was 23 mm vertically and 21 mm horizontally. The average cephalometric follow-up was 131.7 months. In this study, the mandibular horizontal and vertical changes after distraction showed a gradual return of the asymmetry with growth in all patients. There were no statistically significant differences in any of the analyzed angles between the preoperative and final records, except for the supraorbital tilting angle. Conclusions The longitudinal results achieved by distraction osteogenesis were unstable and generally led to relapse, although they were very good in the early postoperative period. Therefore, further efforts are suggested to find a solution that prevents relapse. Clinical question/level of evidence Therapeutic, IV.

Objective To report preliminary results of a new closed-skin, transcutaneous bone conduction device (BCD) in 6 children with high-grade ear atresia. Settings Tertiary care center; prospective study; we evaluated the gain with masking of the contralateral ear and the benefit of hearing rehabilitation with the transcutaneous BCD in noise: speech-in-noise tests, conducted in real life condition (with contralateral ear unmasked and fitted with a hearing device if done before implantation), with and without BCD, with determination of the speech reception threshold (SRT). Children and parent's satisfaction was assessed. Results Patients' ages ranged from 6 to 9 years. All had high-grade ear atresia with a preoperative mean pure-tone average (PTA) loss of 71.46 +/- 6.59 dB on air conduction and 14 +/- 4.98 dB on bone conduction. At M6, all children used the implant 5 to 12 hours daily (mean, 10) without pain or cutaneous complications. At M6, the mean air conduction PTA with transcutaneous BCD was 28.45 +/- 1.68 dB, the mean gain 43 +/- 6.96 dB, and the mean SRT gain 33.33 +/- 10.75 dB. Using speech-in-noise tests in real-life conditions, the mean SRT was statistically improved with the transcutaneous BCD (-8 +/- 2.83 dB, p = 0.0313). Both children and parents reported being satisfied or very satisfied. Conclusion These preliminary results show satisfactory functional gain, cutaneous tolerance, and patients' satisfaction with the new transcutaneous BCD.

Moderate to severe soft tissue deficits can exist with craniofacial microsomia or progressive hemifacial atrophy. The authors reviewed the surgical correction of these defects, including serial autologous fat grafting and parascapular free tissue transfer.Recently treated patients at the Institute of Reconstructive Plastic Surgery at NYU Medical Center were identified. Patients with microvascular free flap underwent reconstruction with parascapular flaps. These flaps have been modified from previously reported inframammary extended circumflex scapular flaps. Demographic information, operative interventions, complications, and outcomes were reviewed and analyzed. The clinical outcomes of these patients were compared with previously reported patients who underwent serial autologous fat grafting.Five patients were recently treated with 7 parascapular flaps. The mean age of the patients at the time of parascapular flap reconstruction was 13.1 years. These were compared to those previously reported who have undergone serial autologous fat grafting. The mean number of procedures was less for the free tissue transfer cohort. There were no microvascular complications because all free flaps survived. One patient had wound dehiscence of the donor site managed with local wound care and healing by secondary intention.For patients undergoing multiple-stage reconstruction of craniofacial microsomia, serial fat grafting is a useful tool for soft tissue reconstruction. Alternatively, in those patients with isolated soft tissue hypoplasia, such as progressive hemifacial atrophy, microvascular free tissue transfer is a safe and efficient option.

Distraction osteogenesis of the mandible has become an alternative to tracheostomy in infants and children who present with upper airway obstruction due to micrognathia. To avoid prolonged intubation during distraction, we have implemented a protocol of immediate distraction at the time of distractor placement, which results in acute airway improvement. Over 2 years, 22 patients with micrognathia and severe airway obstruction have undergone mandibular distractor placement. Indications for surgery were apnea and desaturations with feeding. Resorbable distraction devices were placed bilaterally and activated to 5 to 8 mm. Recombinant human bone morphogenetic protein 2 was placed in the gap. Distraction was implemented at postoperative day 2 at 2 mm/d. Forty-four distraction devices were placed in 22 patients (68% male, 32% female) with a mean age of 24.1 months (range, 3 days to 5.5 years). The average distance of distraction performed in the operating room was 5 mm. The average total distraction was 24 mm performed over 12 days. Overall, 89% of patients were extubated after distractor placement in the operating room. Two patients with difficult intubations were extubated 7 days later in the operating room with otolaryngology. Of the 4 tracheostomy patients, 1 patient was decannulated, whereas 3 patients are pending postoperative sleep studies. One patient had a minor wound complication. Tracheostomy and prolonged intubation in patients with mandibular hypoplasia have significant morbidity and mortality. We have implemented a successful protocol of immediate distraction in the operating room with placement of bone morphogenetic protein. Immediate distraction appears to be an effective method of avoiding postoperative intubation and tracheostomy.

Objective Report outcomes of 2 first known cases using a cochlear implant (CI) and a contralateral auditory brainstem implant (ABI). Patients Two adult patients with postlingual sensorineural deafness. Intervention Both patients had unilateral CI insertion followed by contralateral ABI insertion. In 1 case, there was a large left vestibular schwannoma in the only hearing left ear. CI insertion was first performed in the right longstanding deaf ear. Shortly afterward during the left translabyrinthine surgery, a left ABI was simultaneously inserted. The second patient had Meniere's disease controlled initially by right translabyrinthine vestibular neurectomy but complicated by a right dead ear. When symptoms recurred she underwent left retrosigmoid vestibular neurectomy with auditory nerve preservation. This allowed left CI insertion and a subsequent right ABI insertion. Main outcome measures Pure tone audiometry (PTA), speech discrimination in quiet (SDq), speech discrimination in noise (SDn), and sound localization. Testing was performed with the following: 1) ABI activated alone, 2) CI activated alone, 3) CI and ABI activation (CI-ABI), and 4) CI linked to a contralateral routing of sound (CROS) hearing aid system (CI-CROS). Quality of life assessments were made using a validated questionnaire. Results PTA was worst with the ABI activated alone. SDq was best with the CI-CROS. Regarding SDn with noise coming from the CI side, the head shadow effect was only overcome by the CI-ABI; however, the CI-CROS worked best in the presence of noise opposite to the CI. The CI activated alone and CI-ABI produced useful sound localization. Quality-of-life assessments were best with the CI-CROS. Conclusion Bilateral sound detection seems more beneficial than unilateral in profoundly deaf cases with only 1 functioning auditory nerve. A CI-CROS may produce similar gains to a CI-ABI.

Patients with congenital craniofacial malformations present with complex challenges for reconstruction. Successful management requires individualized treatment often involving rebuilding the facial skeleton de novo, as well as correcting the overlying soft-tissue deficiencies in the final stages. At the University of California, Los Angeles (UCLA) Craniofacial Clinic, serial autologous fat transplantation performed during staged reconstruction is the preferred method.A total of 27 patients with a diagnosis of either craniofacial microsomia/Goldenhar (CM) (n = 19) or Treacher Collins syndrome (TC) (n = 8) were treated at the UCLA Craniofacial Clinic by autologous fat transfer between July 1999 and February 2009. Two-dimensional analysis was conducted on standardized preoperative and postoperative photographs to determine facial symmetry for every patient. Results was grouped based on pathology and analyzed by comparing adipocyte donor site (abdomen vs other).The mean ages at the time of first fat transfer were 17 years for the CM group and 15 years for the TC group. The average numbers of fat transfers per patient were 2.05 (CM) and 2.12 (TC). A mean of 3.74 procedures other than fat grafting was performed in each CM patient, whereas 4.38 other procedures were performed in each TC patient. In CM patients, less than 10.5 months between procedures resulted in improved symmetry. There was an average 6.63% improvement in facial symmetry in the CM group, and a 7.67% improvement in the TC group.Based on the UCLA experience, the durability of facial asymmetry and contour correction with fat transplantation is attainable in the craniofacial patient but may also require concomitant skeletal correction in the most severe cases.

Hereditary elliptocytosis is an inherited red blood cell membrane disorder characterized by typical peripheral blood smear findings of elliptocytes or rod-like red blood cells. Hemoglobin H disease is a form of α-thalassemia disease resulting in mild to moderate hemolytic anemia. The authors report 1 case of a girl who was diagnosed with oculo-auriculo-vertebral spectrum and a coinheritance of hereditary elliptocytosis and deletional hemoglobin H disease. She had moderate, non-transfusion-dependent anemia. The red blood cells showed marked poikilocytosis and fragmentation. The parents were α-thalassemia carriers and the father had the typical red blood cell morphology of common hereditary elliptocytosis.

In craniofacial microsomia, patients with severely hypoplastic mandibles (Pruzansky type III) require replacement of the ramus and condyle unit. Autogenous costocartilaginous rib graft and distraction osteogenesis are the most important techniques used, but long-term results need to be looked at to determine optimal management.Of the 485 patients with craniofacial microsomia and mandibular abnormality identified by the authors' craniofacial multidisciplinary clinic, 31 patients were identified with Pruzansky type III mandibles who underwent treatment and were available for study. Patients primarily had either costocartilaginous grafts or mandibular distraction after molar extraction. Outcomes assessed rib failure, undergrowth, or overgrowth. Reoperation included regrafting for graft failure, rib distraction for undergrowth, and mandibular setback for overgrowth. Details surrounding complications for each modality including osteotomy site were recorded.For primary mandibular reconstruction, 27 patients underwent costocartilaginous rib graft surgery (30 grafts, three bilateral) at 9.9 ± 4.1 years; four patients underwent mandibular distraction at 7.4 ± 2.3 years. Rib graft failure in seven of 30 cases (23 percent) required regrafting. Undergrowth in 17 cases (57 percent) required rib distraction. Overgrowth in three cases (10 percent) required correction at the time of orthognathic correction. For rib graft distraction, osteotomy site locations included native mandible (25 percent), rib-mandible junction (19 percent), and rib graft (56 percent). The rib-mandible junction site had graft-related complications (100 percent) that the other sites did not.For the severely hypoplastic mandibles (Pruzansky type III), costocartilaginous grafts are an accepted modality. However, when rib graft growth is insufficient, secondary distraction should be performed within the native mandible or rib graft and not at the rib graft-mandible junction site.

Background Hemifacial microsomia presents with abnormalities including short ramus, absence of condyle, abnormal canting, deviated chin, and facial asymmetry. Many studies about distraction osteogenesis have been published over the last 20 years, but without long-term follow-up. The aim of this study was to evaluate patients with unilateral craniofacial microsomia who were treated with mandible distraction and with follow-up of more than 5 years. Methods The following retrospective study was evaluated and approved by the Assistance Center for Cleft Lip and Palate. Data were compiled from the charts of 33 patients with hemifacial microsomia who underwent unilateral mandible distraction. Results Average age at time of procedure was 7.3 years, with an average degree of distraction of 20 mm. Seventy percent of cases were treated with internal distraction, 30% external. Follow-up varied between 5 and 15 years, with a mean follow-up of 9 years. Ninety percent of the 33 patients in the study had recurrence of their asymmetry. Mean time to postsurgical recurrence was 44 months. Thirty patients were referred for orthognathic surgery. Six patients have already undergone corrective bimaxillary surgery. One patient underwent genioplasty only, and 1 patient underwent genioplasty with orthognathic jaw surgery. Twenty-two patients are awaiting orthognathic surgery, including one with temporomandibular joint ankylosis. Only 3 subjects had good outcomes, without signs of recurrence. Conclusions Bone distraction once seemed a promising long-term option for treatment of craniofacial microsomia. However, this has not proven effective for all cases, and most patients needed subsequent orthognathic surgery.