Treatment of Facial Asymmetry Using Distraction Osteogenesis in a Mandible First Approach.

Background: Facial asymmetry includes several etiologies, among them trauma to the condylar area during early childhood and congenital malformations such as hemifacial microsomia. This article describes the management of facial asymmetry in adolescents and young adults using a mandible first approach by distraction osteogenesis, followed by maxillary Le-Fort I as a second stage. Methods: Eighteen patients 14-25 years of age presented with unilateral hypoplasia of the jaws which manifested clinically by deviation of the chin and canting of the occlusal plane. Etiology included hemifacial microsomia and trauma injuries at early childhood.All patients underwent orthodontic treatment and two phases of surgical treatment. Surgical treatment included unilateral mandibular distraction followed by Le-Fort I osteotomy for alignment of the maxilla. Additional bone graft in the affected side and sliding genioplasty were done as required. Results: Marked ramal elongation of 18.94 mm concomitant with mandibular forward traction of 12.5 mm was noted while achieving symmetry. In all cases, the maxilla was centered to the midline in proper occlusion. Post distraction, posteroanterior cephalometric radiographs demonstrated elongation of the affected ramus, improvement in facial symmetry, and correction of the occlusal canting. Relapse was minimal based on long-term follow-ups of 47.4 months. Conclusions: The two-stage surgical approach that includes elongation of the mandible as a first stage followed by adaptation of the maxilla is useful in correcting facial asymmetry. Using this protocol at the correct age (14-18) is very stable, as demonstrated by our results, yet one should always remember the transverse deficiency in the gonial angle requires additional bone grafting or patient specific implants.

Cloning, purification and characterization of human dentine matrix protein 1(DMP1).

Introduction: Human teeth are composed mainly of dentin, formed by the odontoblasts. Dentin matrix protein 1 (DMP1) is one of odontoblast differentiation's most important growth factors. Human DMP1 has yet to be completely identified or studied. This study aimed to clone and characterize human DMP1. Materials and methods: The DMP1 gene sequence was prepared and cloned by transfection of human 293 cells. Results: The recombinant DMP1 was purified and characterized. Conclusion: The results suggested its future use in dental tissue regeneration and tissue engineering.

Osteopontin Upregulation, Induced by the Continuous Mechanical Load in Adipose Tissue-Derived Mesenchymal Stem Cells, is Strongly Restricted in INF-γ/TNF-α/IL-22 Microenvironment.

The osteogenic potential of mesenchymal stem cells (MSc) in axial spondyloarthritis (AxSpA) depends on the interplay of inflammation and multiple hormonal and local mechanical factors. In this study, MCs, derived from the adipose tissue of a healthy donor, were cultured under or without continuous mechanical load in the osteogenic differentiation medium with or without the addition of testosterone, cocktail of INF-γ/TNF-α/IL-22, or both. Real-time PCR for osteogenic transcription factors demonstrated that in the absence of INF-γ/TNF-α/IL-22, mechanical load causes significant upregulation of SPP1 (osteopontin), while the presence of the inflammatory cytokines almost completely abolishes this effect. In addition, exposure to INF-γ/TNF-α/IL-22 slightly upregulated BMP2, but suppressed the expression of ALPL, Col1A1, and SPP1, reinforcing the hypothesis that the inflammatory environment allows MSc to commit toward the IL-22-driven osteogenic differentiation but can restrict the later stages of osteogenesis. In summary, osteopontin can play a role in the pathogenesis of AxSpA, linking between mechanical load and pathological bone formation.

Trends in cleft palate incidence in the era of obstetric sonography and early detection.

BACKGROUND: The effect of improvement in prenatal identification of cleft lip or palate (CL/P) on termination of pregnancy (TOP) worldwide is scarcely reported. Our aim was to assess changes in the prevalence of cleft palate attributed to the high access and availability of prenatal advanced screening and pregnancy termination in Israel. METHODS: A retrospective study was conducted on CL/P patients which were treated in our institute and born between January 2000 and December 2018. Clinical and demographic data were extracted from medical records. Data on TOP were collected based on accessible information from the Ministry of Health. Cleft palate severity was evaluated using the Veau Classification. RESULTS: The study was conducted on 258 patients. Higher incidence of Veau II and III was evident throughout the examined period (2000-2018). However, when evaluating the incidence per year, we found that the incidence of severe cases (Veau III and IV) decreased every year showing a major decline of 60% in the last decade, whereas mild cases (Veau type I and II) demonstrated a marked increase of 90%. Regarding the incidence of abortions in Israel, a decrease of 30% was observed in the last two decades, meanwhile a substantial increase was detected in the rate of abortions related to physical malformations of the fetus. Additionally, the number of late terminations due to physical malformations significantly increased in the last decade. CONCLUSIONS: Significant decrease was observed in the incidence of severe cleft palate cases in the last decade. Concurrently, we found a substantial increase in percentage of abortions performed due to physical malformations. We suggest that these changes might be attributed to the accessibility of advanced prenatal screening and pregnancy termination in Israel under the social healthcare system.

Orthodontic force and extracorporeal shock wave therapy: Assessment of orthodontic tooth movement and bone morphometry in a rat model.

OBJECTIVE: The objective was to investigate the effect of extracorporeal shock wave therapy (ESWT) on the magnitude of orthodontic tooth movement, in a rat model, based on a previously established treatment protocol. DESIGN: In conjunction with orthodontic force commencement, rats underwent ESWT. The amount of tooth movement along with different microarchitectural parameters were measured after three weeks by means of microcomputed tomography. In addition, the percentage of cells expressing vascular endothelial growth factor, the number of tartrate-resistant acid phosphatase (TRAP) positive cells/area and blood vessel density were evaluated both for the pressure and tension sides. RESULTS: The addition of ESWT to the orthodontic force after three weeks more than doubled the average tooth movement. The addition of ESWT on the pressure side induced a significant decrease in volumetric bone mineral density. Blood vessel density and the number of TRAP positive cells were higher after the application of ESWT. CONCLUSION: The induction of ESWT during orthodontic tooth movement in a rat model increases the rate of tooth movement by accelerating bone resorption on the pressure side and possibly enhances bone formation on the tension side.

Analytical methodology to measure periodontal bone morphometry following orthodontic tooth movement in mice.

INTRODUCTION: Basic research in orthodontics is commonly conducted in rodents. However, experimental studies on orthodontic tooth movement (OTM) lack a standard method to examine OTM and periodontal changes. This study describes a unifying protocol for the analysis of OTM and associated bone microarchitectural changes in mice using microcomputed tomography (µCT). METHODS: Mice (10 animals/group) were divided into control and OTM groups. OTM was generated by anchoring a nickel-titanium closed-coil spring to the upper incisors to pull the upper left first molar. A third group of TNFα -/- mice was added since these are known to have slower OTM. Using µCT, we implemented and tested a number of methods to measure OTM distance and examine 3D bone morphometric parameters associated with OTM in mice. RESULTS: In total, we tested five methods to measure the OTM distance in mice. The results indicated that measuring the intermolar diastema, and assessing tooth movement relative to the anterior root of the zygomatic arch, displayed the lowest standard deviation and enabled optimal detection of intergroup differences. We also developed two protocols for µCT analysis of the periradicular bone that yielded no false-positive results. Our results revealed that including the width of the periodontal ligament rather than excluding it from the region of interest in mice detected more statistically significant differences in the morphometric parameters between the OTM and control sides and between WT and TNFα -/- mice despite more subtle differences. CONCLUSIONS: We, therefore, propose new guidelines for a standardized µCT-based method to analyse OTM and the extent of the periradicular bone structural changes in mice.

Osteotomy of the Premaxilla in Bilateral Cleft Lip: A Useful Technique Following Failure of Primary Lip Closure.

OBJECTIVES: Successful aesthetic repair of the bilateral cleft lip and palate (BCLP) is one of the most challenging cases encountered by a surgeon. This challenge is heightened when the premaxilla is anteriorly protruded in relation lateral maxillary segments. Our aim is to demonstrate a useful technique for reducing the cleft size before secondary lip repair. We use a premaxillary osteotomy to posteriorly reposition the premaxilla between the lateral segments. METHODS: Six patients with BCLP underwent repositioning of the protruded premaxilla. Five patients had previous primary lip closure procedures with unsatisfactory results. The cleft size was reduced by premaxillary osteotomy from nasal septum and partial removal of the vertical vomer. The osteotomized premaxilla was then repositioned, fixed with a custom-made orthodontic appliance. Following this procedure, the lip was closed in a traditional fashion. RESULTS: All patients demonstrated satisfying lip closure without tension. In each case, the premaxilla was vital and exhibited partial stabilization during follow-up. CONCLUSIONS: In this study the authors demonstrate the usefulness of a premaxillary segment setback as an adjunct to improved function and aesthetics in bilateral lip repair. This method serves as useful technique for previously unsuccessful closure of BCLP.

Periodontal Status Among Schoolchildren in the Republic of Moldova: A Cross-Sectional Study Using the Pathfinder Study Design.

OBJECTIVES: To screen a sample of Moldavian schoolchildren to establish their periodontal condition using the Pathfinder study design of the World Health Organization (WHO). METHODS: Two cohorts - 12- and 15-year schoolchildren - were screened in 12 schools around the country: four schools in the capital city; four more schools in two other larger cities (two schools in each city); and four village schools (one school in each village). In addition to demographic data, the periodontal parameters dental plaque, calculus and bleeding on probing (BOP) were collected. Periodontal pocket depth (PPD) was also measured but only in the cohort of 15-year-old schoolchildren. Measurements were recorded for the six Ramfjord index teeth. RESULTS: In total, 720 children were surveyed: 365 (50.7%) were 12 years of age and 355 (49.3%) were 15 years of age; 351 (48.8%) were girls and 369 (51.2%) were boys; 490 (68%) lived in an urban area and 230 (32%) lived in a rural area. Only 4.5% of 15-year-old children presented with a pocket depth of ≥3.5 mm. Children who lived in rural areas had higher plaque scores than children who lived in urban areas (64.8% vs. 54.1%, P = 0.007). In addition, children who lived in urban areas had significantly less calculus (P = 0.047) and shallower PPDs (P = 0.019). Deeper PPD was associated with higher PI and calculus scores. CONCLUSIONS: Moderate-to-deep periodontal pockets were not uncommon in children in the 15-year-old cohort. Periodontal status was worse in children from rural areas than in children from urban areas. It is therefore suggested that an educational programme, together with preventive and interceptive protocols, should be adopted in early adolescence and especially in rural regions.

Orthodontists and lay people rate masculine soft tissue profiles similarly but feminine soft tissue profiles differently.

Objective: An esthetic facial soft tissue profile is an important objective of contemporary orthodontics. The extent to which clinicians and the public agree on profiles that are esthetically acceptable versus profiles recommended for treatment is unclear. Thus, this work examined the profile considered esthetic by laypeople and general dental practitioners compared to orthodontists.
Method and materials: An Israeli balanced male-female group comprising 284 participants (orthodontists [n = 86], general dental practitioners [n = 64], laypeople [n = 134]) rated the attractiveness of five standardized masculine and feminine profiles presented in standardized silhouettes ranging from concave to convex.
Results: Laypeople see no esthetic difference between masculine and feminine profiles, and prefer moderately concave over moderately convex and severely concave over severely convex profiles. While all raters preferred the straight silhouettes, orthodontists rated them significantly higher than general dental practitioners (P < .0001) or laypeople (P < .020). Similar to the general population, orthodontists gave higher ratings to concave profiles for masculine profiles, but significantly lower ratings for feminine profiles. The severely convex male profile was the least attractive to clinicians and laypeople. There was a significant difference in the attractiveness of the female profile between practitioners (P < .001 and P < .02 for general dental practitioners and orthodontists, respectively) and laypeople.
Conclusions: The findings suggest that orthodontic training and clinical adherence to strict beauty norms results in more critical judgment of female attractiveness, which may affect orthodontic treatment. They indicate trends that reflect changes in recent decades in the public perception of facial beauty, which may affect patient expectations of treatment outcomes.

Effects of Intraligamentary Injection of Osteogenic-Induced Gingival Fibroblasts on Cementum Thickness in the Dog Model of Tooth Root Resorption.

Tooth root resorption is an unwanted result of orthodontic tooth movement, and it can be expressed by a reduction in cementum thickness. The aim of this experimental study was to evaluate the effect of intraligamentary injection of osteogenic-induced gingival fibroblasts (OIGF) on cellular and acellular tooth root cementum thickness in modeled orthodontic tooth movement. Six beagle dogs were used in the study. All the upper and lower third and fourth premolars were subjected to mechanical loading for 4 weeks, which induced orthodontic tooth movement. Fifteen premolars were assigned to the OIGF group, which received a single OIGF injection through the periodontal ligament near the root apex (n = 7 teeth), and to the control group, which received a single injection of Dulbecco's modified eagle's medium in the periapical area (n = 8 teeth). The evaluation of histomorphometry was performed to assess the thicknesses of cellular and acellular cementum at the root apex and four bilateral sites distal to the apex. We found no statistically significant enhancing effects of gingival fibroblasts on either cellular or acellular cementum thicknesses when compared with the control group. We conclude that a single intraligamentary injection of OIGF does not stimulate the formation of tooth root cementum in the dog model of orthodontic tooth movement. Thus, OIGF is unlikely to prevent orthodontic-induced tooth root resorption.

Surgically Assisted Rapid Palatal Expansion to Correct Maxillary Transverse Deficiency.

BACKGROUND: Transverse maxillomandibular discrepancies are widespread. Treatment is comprised of orthodontic expansion in patients younger than 15 years or by surgically assisted rapid palatal expansion (SARPE) in skeletally mature patients where the possibility of successful orthodontic maxillary expansion decreases as sutures close and resistance to mechanical forces increases. AIM: To present our experience of treating transverse maxillary deficiency using a unique L-shaped osteotomy and to demonstrate stable results. PATIENTS AND METHODS: 32 patients aged between 19 and 54 years exhibiting transverse maxillary deficiency. L-shaped osteotomy was performed laterally from the pterygoid plate posteriorly to above the roots of the second incisive anteriorly continuing with a vertical osteotomy between the lateral incisive and canine teeth toward the horizontal osteotomy. In 18 patients with dysgnathia, bimaxillary surgery was performed one year following the SARPE procedure. RESULTS: Mean transverse maxillary expansion of 6.2mm at the canine incisal and 6.4mm at the first molar occlusal regions were obtained. One year postoperatively results were relatively stable, 5.8mm and 6.2mm respectively. The SARPE procedure resulted in overcoming the maxillary buttress resistance, expansion of the anterior dental arch and bilateral distraction creating bone on both sides of the premaxilla contributing to better alignment of the anterior teeth and superior stability. CONCLUSIONS: We conclude that SARPE is an effective and stable method for addressing severe maxillary transverse discrepancy in adults while the unique osteotomy performed allowed for maintaining proper position of the premaxilla and maxillary midline and allowing for division of the newly created bone bilaterally thus resulting in a more stable outcome.

Craniofacial Deformities in Patients With Beta-Thalassemia: Orthodontic Versus Surgical Correction-A Systematic Review.

Rapid blood cell turnover and bone marrow expansion caused by beta-thalassemia (ßT) result in craniofacial and dentoalveolar anomalies. This report presents a systematic review of the literature over the past 50 years on orthodontic and surgical considerations in the management of ßT-affected patients. Seventeen publications encompassed 24 patients, 11 male individuals and 13 female individuals, 7 to 43 years of age. Eleven patients underwent only surgical treatment, eleven combined orthodontic-surgical treatment, and 2 orthodontic treatment. Surgical treatment primarily addressed typical maxillary overgrowth by maxillary reshaping, premaxillary segmental repositioning, or complete Le Fort I impaction and set back osteotomy. In severe maxilla-mandibular discrepancy and/or increased lower facial height, a bilateral sagittal split mandibular osteotomy is the treatment of choice. Although surgery involves risks of excessive bleeding, morbidity, and impaired nasal esthetics, little attention is given to the orthodontic modality. In conclusion, the current literature recommends early interceptive orthodontics aimed to decrease dentoskeletal deformities, severe malocclusion, and soft tissue imbalance. Treatment includes maxillo-mandibular orthopedic and functional manipulation with dentoalveolar treatment, which might either prevent orthosurgical procedures later or reduce its extent. This suggested a multidisciplinary approach comprising a hematologist, a pediatrician, a pediatric dentist, and an orthodontist, which might also significantly improve the patient's quality of life.

Traumatic Dental Injuries among Adolescents In Republic of Moldova.

Objectives: The aim of this investigation was to evaluate the epidemiological data on dental injuries in permanent teeth among seventh and tenth grade schoolchildren in the Republic of Moldova. Study design: The pathfinder study model was chosen to evaluate the prevalence of Traumatic Dental Injuries (TDI). Schoolchildren ages 12 and 15 years old were examined: In the capital city (four schools), in two other major cities-(two schools each city), and in four villages (one per each village) in accordance with the WHO's recommendations. Results: A total of 720 seventh and tenth grade schoolchildren were examined. The overall prevalence of TDI was found to be 16.4% (total of 118 children). The prevalence of TDI was greater in the older age cohort (p<0.05). Children from rural areas presented with greater prevalence of TDI compared to children who live in urban region (p<0.03). The maxillary central incisors were found to be the most common teeth affected by trauma constituting 57.1% of the evaluated TDI cases. Enamel fracture was the main type of TDI (83%). Conclusions: The TDI mainly involved enamel fractures. Urban schoolchildren show lower rates of TDI compared to rural schoolchildren.

Orthodontic extraction space closure with and without socket preservation: a comparative case analysis.

Preorthodontic socket preservation after tooth extraction is intended to enhance favorable dentoalveolar ridge morphology and architecture, and facilitate orthodontic tooth movement (OTM) and extraction space closure. This 13-year-old skeletal Class II case presents a unique opportunity to evaluate and compare the OTM extraction space closure by means of a split-mouth analysis in a single patient. The comprehensive orthodontic-periodontal treatment included nonsimultaneous extraction of the bilateral periodontally compromised mandibular first molars and the eruption of bilateral impacted mandibular canines. While the right post-extraction space underwent a natural healing process, the left one was grafted using 4BONE BCH (hydroxyapatite ß-tricalcium phosphate [HA-ß-TCP], HA 60%, and ß-TCP 40%). The 3-year multidisciplinary treatment approach resulted in Class I relationships on the right side and Class II on the left side, improved facial appearance, and dento-skeletal jaw relationships. However, a 3-mm residual alveolar space remained unclosed on the grafted left-site, along with an unerupted left third molar. This split-mouth comparative analysis of the orthodontic space closure demonstrated a significant clinical difference in the outcome. Preorthodontic placement of HA-ß-TCP grafting material on the left segment, proved to be an obstacle for OTM extraction space closure, hindering the establishment of good occlusion.

Effects of Extracorporeal Shock Wave Therapy on Distraction Osteogenesis in Rat Mandible.

BACKGROUND: Distraction osteogenesis has widespread clinical use in the treatment of congenital and acquired craniofacial deformities. Nonetheless, during the prolonged consolidation period, the newly regenerated bone carries the risk of complications. A known method for enhancing bone healing is extracorporeal shock wave therapy, which has been shown to induce neovascularization and promote tissue regeneration. The authors investigated whether extracorporeal shock wave therapy can accelerate bony consolidation and regeneration in distraction osteogenesis of the rat mandible and at which stage of distraction osteogenesis it should be applied. METHODS: Twenty-four male Sprague-Dawley rats were subjected to distraction osteogenesis of the right mandible (latency period, 3 days; distraction period, 10 days; 0.5 mm/day). Experimental groups consisted of the following: group I (control), no extracorporeal shock wave therapy; group II, extracorporeal shock wave therapy (0.18 mJ/mm(2)) at the latency period; and group III, extracorporeal shock wave therapy (0.18 mJ/mm(2)) at the consolidation period. Explants were removed for evaluation after 4 weeks of consolidation. RESULTS: Histologic evaluation showed well-developed cortical cortex and a higher degree of bone formation and mature bone in group III; micro-computed tomography showed significantly increased bone mineral density, bone volume fraction, and trabecular thickness; immunohistochemistry demonstrated significantly increased expression of bone morphogenetic protein-2, vascular endothelial growth factor, and proliferating cell nuclear antigen. CONCLUSION: Extracorporeal shock wave therapy application at the consolidation period during distraction osteogenesis in the rat mandible enhances bone formation and osteogenic and angiogenic growth factors, improves bone mechanical properties, and accelerates bone mineralization.

Intermittent Hypoxia Induced Formation of "Endothelial Cell-Colony Forming Units (EC-CFUs)" Is Affected by ROS and Oxidative Stress.

Intermittent hypoxia (IH)-the hallmark of obstructive sleep apnea (OSA)-increases leukocyte activation, production of NADPH-oxidase dependent reactive oxygen species (ROS) and oxidative stress, affecting endothelial function. However, IH and oxidative stress can also stimulate adaptive-protective mechanisms by inducing the development of Endothelial Cell-Colony Forming Units (EC-CFUs), which are considered as a good surrogate marker for endothelial progenitor cells (EPCs), and likely reflect a reparatory response to vascular damage or tissue ischemia by leukocytes. Blood samples were obtained from 15 healthy consenting volunteers to evaluate the effects of IH and sustained hypoxia (SH) in vitro on EC-CFUs development and functions. The variables measured included: their numbers, the area, the proliferative capacity and ROS production. Additionally, NADPH-oxidase, VEGF and nuclear factor-erythroid 2 related factor 2 (Nrf2) expression, as well as their paracrine effects on endothelial tube formation were determined. The involvement of ROS was probed using the anti-oxidant N-acetylcysteine (NAC) and NADPH-oxidase inhibitors apocynin and diphenyl-iodide. Compared to normoxia, IH-dependent increases in EC-CFUs numbers were observed, showing an individual donor-dependent trait. Also, the expression of VEGF and gp91phox, a subunit of NADPH-oxidase, were significantly increased. ROS production and oxidative stress markers were also significantly increased, but Nrf2 expression and colony size were unaffected by IH. Additionally, conditioned media harvested from IH- and SH-treated mature EC-CFUs, significantly increased endothelial tube formation. These effects were markedly attenuated or diminished by the ROS and NADPH-oxidase inhibitors employed. In conclusion, we show here for the first time that IH-associated oxidative stress promotes EC-CFUs' vascular and paracrine capacities through ROS. However, the large inter-individual variability expressed in EC-CFUs numbers and functions to a given IH stimulus, may represent an individual trait with a potential clinical significance.

Cleft Palate Fistula Closure Utilizing Acellular Dermal Matrix.

Fistulas represent failure of cleft palate repair. Secondary and tertiary fistula repair is challenging, with high recurrence rates. In the present retrospective study, we review the efficacy of using acellular dermal matrix as an interposition layer for cleft palate fistula closure in 20 consecutive patients between 2013 and 2016. Complete fistula closure was obtained in 16 patients; 1 patient had asymptomatic recurrent fistula; 2 patients had partial closure with reduction of fistula size and minimal nasal regurgitation; 1 patient developed a recurrent fistula without changes in symptoms (success rate of 85%). We conclude that utilizing acellular dermal matrix for cleft palate fistula repair is safe and simple with a high success rate.

The pathfinder study among schoolchildren in the Republic of Moldova: dental caries experience.

OBJECTIVES: The aim of the present research was to evaluate the current caries experience of schoolchildren in the Republic of Moldova. METHODS: A pathfinder study was planned and executed according to the World Health Organization (WHO) recommendations. Caries experience was recorded in three large cities (the capital city and two other large cities) and four villages throughout the country. Within each site and age group, school clusters were randomly chosen. Three different age groups (6, 12 and 15 years) of schoolchildren were assessed. RESULTS: Overall, 1,100 children were examined: 724 from cities and 376 from the villages. Among the study population, 408 (37.1%) were female and 692 (62.9%) were male, 380 were 6 years of age, 365 were 12 years and 355 were 15 years. The prevalence of caries experience [decayed, missing and filled scores of > 1 for primary (dmft) and secondary (DMFT) teeth] was 87.4% (dmft score = 3.9 ± 3.0) for 6-year-old participants, 77.53% (DMFT score = 2.7 ± 2.3) for 12-year-old participants and 86.2% (DMFT score = 3.7 ± 2.9) for 15-year-old participants. For the 12-year group, a higher proportion of caries-free children were detected in urban areas than in rural areas (25.5% vs. 15.5%, P = 0.003). We found more caries-free participants among 12-year-olds than among 15-year-olds (22.5% vs. 13.8%, P = 0.004). CONCLUSIONS: These WHO key index age groups may be inserted into the global oral health databank and provide a 'pathfinder' baseline for formulating and conducting future public oral health programmes in the Republic of Moldova, with an emphasis on children in rural locations.

Management of Severely Atrophic Maxilla in Ectrodactyly Ectodermal Dysplasia-cleft Syndrome.

BACKGROUND: Ectrodactyly ectodermal dysplasia-cleft syndrome is a rare genetic syndrome with an incidence of 1/90,000 live births, characterized by cleft lip and palate, severely hypoplastic maxilla, and hypodontia. Patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome suffer from a severely hypoplastic maxilla that is highly difficult to treat using traditional orthognathic methods. In this study, we propose using distraction osteogenesis to achieve a major advancement while maintaining good stability and minimal relapse. To our knowledge, this is the first description of patients with this syndrome treated using distraction osteogenesis. METHODS: Five patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome were included in the study. All patients had been operated on according to the well-established protocol of cleft lip and palate reconstruction before maxillary distraction osteogenesis. Hard and soft-tissue changes were evaluated by cone beam computed tomography and lateral cephalograms before distraction osteogenesis (T1), at the postdistraction point (T2) and after 1 year of follow-up (T3). RESULTS: Examination revealed marked maxillary advancement in all our patients with a significant mean difference in hard tissue parameters (condylion to A point = 18 mm; nasion-sella line to A point = 15.2 degrees) and a notable improvement in facial convexity (20.9 degrees). One year follow-up measurements demonstrated mild relapse rates of 6% in the horizontal plane. CONCLUSIONS: We conclude that despite the challenging anatomic and physiological features of ectrodactyly ectodermal dysplasia-cleft patients, by enhancing current surgical techniques, there is promising potential for improved patient outcomes, achieving normognathic facial appearance with implant supported rehabilitation.