Expression of biological mediators during orthodontic tooth movement: A systematic review.

OBJECTIVES: The aim of the present systematic review was to offer a timeline of the events taking place during orthodontic tooth movement(OTM). MATERIALS AND METHODS: Electronic databases PubMed, Web of Science and EMBASE were searched up to November 2017. All studies describing the expression of signaling proteins in the periodontal ligament(PDL) of teeth subjected to OTM or describing the expression of signaling proteins in human cells of the periodontal structures subjected to static mechanical loading were considered eligible for inclusion for respectively the in-vivo or the in-vitro part. Risk of bias assessment was conducted according to the validated SYRCLE's RoB tool for animal studies and guideline for assessing quality of in-vitro studies for in-vitro studies. RESULTS: We retrieved 7583 articles in the initial electronic search, from which 79 and 51 were finally analyzed. From the 139 protein investigated, only the inflammatory proteins interleukin(IL)-1ß, cyclooxygenase(COX)-2 and prostaglandin(PG)-E2, osteoblast markers osteocalcin and runt-related transcription factor(RUNX)2, receptor activator of nuclear factor kappa-B ligand(RANKL) and osteoprotegerin(OPG) and extracellular signal-regulated kinases(ERK)1/2 are investigated in 10 or more studies. CONCLUSION: The investigated proteins were presented in a theoretical model of OTM. We can conclude that the cell activation and differentiation and recruitment of osteoclasts is mediated by osteocytes, osteoblasts and PDL cells, but that the osteogenic differentiation is only seen in stem cell present in the PDL. In addition, the recently discovered Ephrin/Ephs seem to play an role parallel with the thoroughly investigated RANKL/OPG system in mediating bone resorption during OTM.

A Comprehensive Craniofacial Study of 22q11.2 Deletion Syndrome.

The 22q11.2 deletion syndrome (22q11.2DS) is one of the most frequent microdeletion syndromes and presents with a highly variable phenotype. In most affected individuals, specific but subtle facial features can be seen. In this observational study, we aim to investigate the craniofacial and dental features of 20 children with a confirmed diagnosis of 22q11.2DS by analyzing 3-dimensional (3D) facial surface scans, 2-dimensional (2D) clinical photographs, panoramic and cephalometric radiographs, and dental casts. The 3D facial scans were compared to scans of a healthy control group and analyzed using a spatially dense geometric morphometric approach. Cephalometric radiographs were digitally traced, and measurements were compared to existing standards. Occlusal and dental features were studied on dental casts and panoramic radiographs. Interestingly, a general trend of facial hypoplasia in the lower part of the face could be evidenced with the 3D facial analysis in children with 22q11.2DS compared to controls. Cephalometric analysis confirmed a dorsal position of the mandible to the maxilla in 2D and showed an enlarged cranial base angle. Measurements for occlusion did not differ significantly from standards. Despite individual variability, we observed a retruded lower part of the face as a common feature, and we also found a significantly higher prevalence of tooth agenesis in our cohort of 20 children with 22q11.2DS (20%). Furthermore, 3D facial surface scanning proved to be an important noninvasive, diagnostic tool to investigate external features and the underlying skeletal pattern.

The effect of first and second premolar extractions on third molars: A retrospective longitudinal study.

OBJECTIVES: To analyse the effect of first and second premolar extractions on eruption space for upper and lower third molars and on third molar position and angulation during orthodontic treatment. METHODS: The sample consisted of 296 patients of which 218 patients were orthodontically treated without extraction and 78 patients with extraction of first or second premolars. The eruption space for third molars was measured on pre- and posttreatment lateral cephalograms, whereas the angulation, vertical position, the relation with the mandibular canal and the mineralization status of third molars were evaluated using pre- and posttreatment panoramic radiographs. All data were statistically analyzed. RESULTS: The increase in eruption space and the change in vertical position of upper and lower third molars significantly differed between patients treated with and without premolar extractions, whereas the change in angulation, relationship with the mandibular canal and mineralization status of the third molars did not significantly differ between patients treated with and without premolar extractions. CONCLUSIONS: The retromolar space and the position of third molars significantly change during orthodontic treatment in growing patients. Premolar extractions have a positive influence on the eruption space and vertical position of third molars, whereas they do not influence the angular changes of third molars. Due to the retrospective character of the study, these conclusions should be carefully considered. Further prospective research is necessary for better insights into this complex topic. CLINICAL SIGNIFICANCE: This study stresses the importance of considering the possible effects of orthodontic treatment on third molars during treatment planning.

Short-term hard and soft tissue changes after mandibular advancement surgery in Class II patients: a retrospective cephalometric study.

AIM: The aim of this study was to describe hard and soft tissue changes after mandibular advancement surgery and to investigate the possible differences between Class II facial patterns. MATERIALS AND METHODS: Lateral cephalograms of 109 patients who underwent combined orthodontic treatment and bilateral sagittal split osteotomy (BSSO) were studied. Radiographs were taken within 6 weeks before surgery (T0) and at least 6 months postoperatively (T1). Patients were classified into 3 groups according to the preoperative mandibular plane angle. Hard- and soft-tissue changes were analysed with an x-y cranial base coordinate system. Measurements were evaluated statistically. RESULTS: Soft and hard tissues of the chin moved forward and downward. The position of the upper lip remained unchanged, while the lower lip moved forward and upward and decreased in thickness. The soft tissue points of the chin follow their corresponding skeletal points almost completely, while the change of the lower lip was only 76 per cent of the movement of the underlying hard tissue. The increase of SNB was more evident in the low-angle group, as well as improvement of the facial convexity. Stomium superius moved more forward in the low- and medium-angle cases. Ratios of hard and soft tissue changes showed no differences for different facial patterns. LIMITATIONS: Limitations derived from the retrospective study design. Only short-term changes could be addressed. The distinction between surgical changes and changes due to skeletal relapse is difficult to assess. Also, the difficulty to reproduce a relaxed lip position during imaging may influence our results. CONCLUSION: Class II characteristics improved after mandibular advancement. Soft tissues of the chin follow their skeletal structures almost in a 1:1 relationship, while movement of the lower lip was less predictable. The facial pattern of Class II patients should be considered in treatment planning.

Dental development in patients with agenesis.

AIM: Recent research concerning tooth development and dental agenesis suggests that specific genes are associated with agenesis, and that these genetic factors could also cause delayed dental development of the remaining teeth. The objective of this study was to evaluate whether dental development of patients with agenesis is delayed, compared to a control group. SUBJECTS AND METHOD: Panoramic radiographs of 1145 patients with dental agenesis were collected (452 males, 693 females) aged 6.2 to 24.8 years. The control group included 2032 panoramic radiographs (977 males, 1055 females) aged 6.0 to 24.4 years. A total of 3177 orthopantomograms were staged according to Demirjian. All left permanent teeth present in the mandible (except third molars) were considered. In order to evaluate the difference between patients with and without agenesis, a developmental score (DS) was calculated. The association between the DS and the number of agenetic teeth was evaluated with a Spearman correlation. RESULTS: Based on the DS, patients with agenesis have a delayed development compared to patients in the control group (p < 0.0001). Within the agenesis group, there is a weak relation between the number of agenetic teeth and the DS: the higher the number of teeth with agenesis, the lower the DS (p < 0.0001 and p = 0.06 for females and males, respectively). CONCLUSION: The obtained results can be an important factor for treatment planning in patients with dental agenesis. Moreover, the presence of agenesis needs to be taken into account when using age estimation methods based on permanent tooth development.

Novel IRF6 Mutations Detected in Orofacial Cleft Patients by Targeted Massively Parallel Sequencing.

Common variants in interferon regulatory factor 6 ( IRF6) have been associated with nonsyndromic cleft lip with or without cleft palate (NSCL/P) as well as with tooth agenesis (TA). These variants contribute a small risk towards the 2 congenital conditions and explain only a small percentage of heritability. On the other hand, many IRF6 mutations are known to be a monogenic cause of disease for syndromic orofacial clefting (OFC). We hypothesize that IRF6 mutations in some rare instances could also cause nonsyndromic OFC. To find novel rare variants in IRF6 responsible for nonsyndromic OFC and TA, we performed targeted multiplex sequencing using molecular inversion probes (MIPs) in 1,072 OFC patients, 67 TA patients, and 706 controls. We identified 3 potentially pathogenic de novo mutations in OFC patients. In addition, 3 rare missense variants were identified, for which pathogenicity could not unequivocally be shown, as all variants were either inherited from an unaffected parent or the parental DNA was not available. Retrospective investigation of the patients with these variants revealed the presence of lip pits in one of the patients with a de novo mutation suggesting a Van der Woude syndrome (VWS) phenotype, whereas, in other patients, no lip pits were identified.

Dental care approach in patients with osteopetrosis.

AIM: To describe dental and dentofacial characteristics observed in patients diagnosed with osteopetrosis and to advise a dental care approach in these patients. METHODS: Four patients were clinically diagnosed with osteopetrosis, characterised by increased bone density, bone marrow failure, blindness and deafness due to compression of cranial nerves. All patients were dentally screened at different ages (2.5-31 years) and three of them were treated with a haematopoietic stem cell transplantation (HSCT) at the age of 6 months, 1 and 3.1 years. RESULTS: All patients showed similar dental characteristics but varying severity and extent. Dental pits, abnormalities in form, agenesis and enamel deformations are seen. The eruption of the permanent dentition occurs at a slow rate, primary teeth can persist, have no successor, and aberrant form of the primary/permanent teeth can delay eruption. Uneven surfaces and atypical dental crowns combined with visual impairment make brushing of the teeth and plaque removal more difficult to manage. CONCLUSION: Dental problems such as delay in tooth eruption, crown anomalies and agenesis are seen in the patients diagnosed with osteopetrosis, although the severity and extensiveness of the symptoms differ and possibly depend on the age of the patient at HSCT. Treatment management: Frequent dental follow-up examinations are necessary for guiding the eruption and professional dental cleanings. Aid in the eruption can be helpful. In the case of surgical interventions, an antibiotic prophylaxis is advised. A fluoride treatment can be added to prevent caries. The role of HSCT in dental findings needs further research.

Review: Facial endophenotypes in non-syndromic orofacial clefting.

Cleft lip and/or palate (CL/P) is one of the most frequent congenital malformations, with a frequency of 1 in 700 live births. Non-syndromic orofacial clefting is a multifactorial condition, with both a genetic and an environmental component. Although numerous studies have been published addressing the genetic etiology of CL/P, this factor remains incompletely understood. A promising approach to find candidate gene regions for CL/P is the investigation of endophenotypes, which are characteristics associated with a certain condition and that can be an expression of underlying susceptibility genes. This review focuses on the known facial endophenotypes in CL/P (such as distortion of the orbicularis oris muscle and facial features in non-affected relatives of patients with CL/P) and genes that could be associated with these characteristics. Possibilities for further endophenotype-related studies in the field of non-syndromic CL/P are discussed.

Surgery for velopharyngeal insufficiency: The outcomes of the University Hospitals Leuven.

OBJECTIVES: We reviewed the outcomes of patients who underwent a velopharyngoplasty and subsequent speech therapy for velopharyngeal insufficiency (VPI) to determine possible prognostic variables. METHODS: During the period 2002-2010, 91 patients with VPI underwent a velopharyngoplasty (either the Honig velopharyngoplasty, the modified Honig velopharyngoplasty or the Hynes pharyngoplasty). Of these, 62 had complete data for long-term evaluation of speech outcome and analysis of variables potentially influencing this outcome. Speech outcome was assessed using five criteria that were evaluated pre- and postoperatively: hypernasality, nasal emission, facial grimacing, retro-articulation and glottal stops. The former two variables were transformed into a semi-objective nasality index (NI), the latter three variables were assembled to form a subjective articulation index (AI). Prognostic variables for outcome that were studied included age at velopharyngoplasty, associated 22q11.2 deletion syndrome, intervention type, primary or secondary surgery and pre-intervention speech therapy. RESULTS: Before surgery, based on the NI, 15 patients had mild VPI and 44 patients had moderate to severe VPI. Postoperatively at 12 months, 46 patients had a good speech outcome (normal or mild VPI), 13 patients had moderate VPI and no more severe VPI was observed. The overall success rate of 78% after one year increased to 90% in the long-term (median 27 months) with further speech therapy. Patients without the diagnosis of 22q11.2 deletion syndrome had better speech outcomes than patients with the syndrome. No statistically significant effect of the age at velopharyngoplasty on speech outcome was found. No cases of sleep apnea syndrome were reported. CONCLUSIONS: Our protocol of patient tailored surgical interventions and further postoperative speech therapy results in good speech outcomes, with no or only mild remaining VPI for the majority of patients. The correction of VPI is more difficult for the subgroup of patients with 22q11.2 deletion syndrome.

Pierre Robin sequence: Management of respiratory and feeding complications during the first year of life in a tertiary referral centre.

OBJECTIVES: To review early clinical manifestations of Pierre Robin sequence (PRS) and their management during the first year of life in the University Hospitals Leuven. METHODS: Retrospective series of 48 patients with PRS born between 2001 and 2011 and treated at a tertiary referral hospital. Review of the current literature about management of respiratory and breathing difficulties in the early life of PRS patients. RESULTS: Of our cleft palate patients 15.3% presented with PRS. A syndrome was diagnosed in 14.6%, associated anomalies without a syndromic diagnosis in 56.3% and isolated PRS in 29.2% of the cases. Mortality rate directly related to PRS was 2.1%. Respiratory difficulties were observed in 83.3% and feeding difficulties in 95.6% of the patients. Respiratory problems were addressed in a conservative way in 75%, in a non-surgical invasive way in 42.5% and in a surgical way in 12.5%. A statistically significant relationship between the association of a syndrome or other anomalies, and a higher need for resuscitation and invasive treatment were found (chi-square test, p-values=0.019 and 0.034). Feeding difficulties were managed conservatively in 91.3%, invasively in 80.4% and surgically in 15.2%. CONCLUSIONS: PRS is frequently associated with other abnormalities or syndromes. Therefore routine screening for associated anomalies in neonates with PRS is recommendable. Respiratory and feeding complications are highly frequent and possibly severe, particularly in patients with associated anomalies or syndromes, and should be recognized and addressed appropriately in an early stage. There is a potential role for the nasopharyngeal airway in reducing the need for the more traditional surgical interventions for respiratory problems.

Evaluation of transverse maxillary expansion after a segmental posterior subapical maxillary osteotomy in cleft lip and palate patients with severe collapse of the lateral maxillary segments.

Objective : The purpose of this longitudinal retrospective study was to evaluate transverse maxillary expansion after a Schuchardt or segmental posterior subapical maxillary osteotomy (SPSMO) in patients with cleft lip and palate (CLP). A second aim was to compare these data with data for adult patients without CLP who were receiving a surgical assisted rapid palatal expansion (SARPE). Method : The study group comprised 19 patients with CLP and a severe transversally collapsed maxilla who were treated with SPSMO followed by hyrax expansion at the University Hospitals Leuven. Dental casts of the 19 patients were analyzed before treatment, at maximum expansion, during orthodontic treatment, at the completion of orthodontic treatment. and 2 years after orthodontic treatment and were measured at the canine, premolar, and molar levels. Adult patients without CLP who were enrolled in a prospective study served as the control group. Results : Maxillary expansion within the study group was significantly greater (P < .05) at all measured levels compared with the maxillary arch before treatment. No significant relapse was measured in the study group 2 years after orthodontic treatment. When comparing the study and control groups, the only statistical difference was that canine expansion was significantly greater in the study group. Conclusion : SPSMO followed by maxillary expansion and orthodontic treatment is an appropriate treatment option to correct a severe transversally collapsed maxilla in patients with CLP. The overall treatment effect of SPSMO expansion is comparable with the effects of SARPE, although canine expansion was greater in the SPSMO group.

Evaluation of Transverse Maxillary Expansion After a Segmental Posterior Subapical Maxillary Osteotomy in Cleft Lip and Palate Patients With Severe Collapse of the Lateral Maxillary Segments.

Objective : The purpose of this longitudinal retrospective study was to evaluate transverse maxillary expansion after a Schuchardt or segmental posterior subapical maxillary osteotomy (SPSMO) in patients with cleft lip and palate (CLP). A second aim was to compare these data with data for adult patients without CLP who were receiving a surgical assisted rapid palatal expansion (SARPE). Method : The study group comprised 19 patients with CLP and a severe transversally collapsed maxilla who were treated with SPSMO followed by hyrax expansion at the University Hospitals Leuven. Dental casts of the 19 patients were analyzed before treatment, at maximum expansion, during orthodontic treatment, at the completion of orthodontic treatment. and 2 years after orthodontic treatment and were measured at the canine, premolar, and molar levels. Adult patients without CLP who were enrolled in a prospective study served as the control group. Results : Maxillary expansion within the study group was significantly greater (P < .05) at all measured levels compared with the maxillary arch before treatment. No significant relapse was measured in the study group 2 years after orthodontic treatment. When comparing the study and control groups, the only statistical difference was that canine expansion was significantly greater in the study group. Conclusion : SPSMO followed by maxillary expansion and orthodontic treatment is an appropriate treatment option to correct a severe transversally collapsed maxilla in patients with CLP. The overall treatment effect of SPSMO expansion is comparable with the effects of SARPE, although canine expansion was greater in the SPSMO group.

Cranio-maxillofacial, orthodontic and dental treatment in three patients with Apert syndrome.

BACKGROUND: Apert syndrome is a severe developmental malformation, clinically characterised by craniosynostosis, midface hypoplasia, a cone-shaped calvarium, ocular manifestations, typical dental findings and syndactyly of the hands and feet. Early craniosynostosis of the coronal suture, the cranial base and agenesis of the sagittal suture are prodromal characteristics for the typical craniofacial appearance in patients with Apert syndrome. CASE REPORTS: The aim of this report was to describe the maxillofacial and orthodontic management of three patients with Apert syndrome who attended the Craniofacial, Maxillofacial and Orthodontic clinics of the University Hospitals of the KU Leuven (Belgium). The typical clinical features, the general orthognathic treatment approach as well as individual approaches of three patients with Apert syndrome are being highlighted. FOLLOW-UP: The three patients with Apert syndrome have been followed up very closely by all involved specialised departments as well as by multidisciplinary teams from birth. CONCLUSION: This report demonstrated that a combined orthodontic and orthognathic surgical treatment plan could significantly improve the occlusal function as well as the facial and occlusal aesthetics in patients with Apert syndrome.

Enamel defects on the maxillary premolars in patients with cleft lip and/or palate: a retrospective case-control study.

AIM: The purpose of this study was to investigate the presence of developmental defects of enamel on maxillary premolars in patients with cleft lip and/or palate. In addition, the relationship with the surgical technique of soft palate closure was studied. Such a relationship could be suspected since formation of enamel occurs around the same time period as soft palate closure. MATERIALS AND METHODS: The study consisted of three groups. Patients from the first group (n = 123) were recruited from the Cleft Lip and Palate Team of the University Hospitals Leuven (CLPT-UHL). The second group (n = 81) consisted of patients consulting the Cleft Lip and Palate Team of the Radboud University Medical Centre Nijmegen (CLPT-UMCN). Healthy non-cleft lip and/or palate patients (n = 100) recruited from a private orthodontic practice were enrolled in group 3. All maxillary premolars were examined. RESULTS: Out of the total sample, 43 patients showed developmental defects on one or more premolars. All defects occurred in patients of group 1 who received surgical closure by the CLPT-UHL. None of the patients from group 2 and 3 showed defects. CONCLUSIONS: It can be suggested that the surgical technique, used by the CLPT-UHL for soft palate closure, causes these defects. It is postulated that the technique used by the CLPT-UHL leads to interference with the blood supply of the developing premolar at a critical stage of tooth enamel development. More research is needed to confirm these findings.

Long-term changes in microbiology and clinical periodontal variables after completion of fixed orthodontic appliances.

OBJECTIVES: The aim of this prospective study was to monitor patients' microbiological and clinical periodontal parameters prior and up to 2 years after orthodontic treatment. MATERIAL AND METHODS: Twenty-four adolescents were treated with brackets. Fourteen of them received bands on upper first molars for extra-oral force application before bonding brackets to the remaining teeth. Microbiology, periodontal probing depth, bleeding on probing (BOP), and gingival crevicular fluid (GCF) flow were assessed at baseline (T1), bracket removal (T2), and 2 years post-treatment (T3). A statistical comparison was made over time and between bands and brackets. RESULTS: A significant increase from T1 to T2 and a decrease from T2 to T3 in pathogenicity of plaque were noted. No significant difference was observed concerning supragingival colony-forming units (CFU) ratio (aerobe/anaerobe) between T3 and T1. However, the subgingival CFU ratio (aerobe/anaerobe) at T3 did significantly differ from the ratio at T1. Periodontal probing depth, BOP and GCF flow showed a significant increase between T1 and T2 and a reduction between T2 and T3, resulting in the absence of significant differences between T3 and T1, except for BOP at banded sites. CONCLUSION: Placement of fixed appliances has an impact on periodontal parameters. The results showed that not all parameters were normalized at T3, indicating that the changes are only partially reversible.

[Heridity of non-syndromal orofacial clefts]. / Erfelijkheid van niet-syndromale orofaciale schisis.

Non-syndromal orofacial clefts are congenital anomalies with a high incidence of heterogeneous origin. The condition is usually multifactorially determined, caused by interaction between multiple genetic and environmental factors. So far, only a few causal factors have been identified. Even in genetically identical individuals like monozygotic twins, the orofacial cleft is usually not fully concordant. As an illustration of non-syndromal orofacial clefts, monozygotic twin brothers with orofacial clefts which are not fully concordant are presented.

[Dentomaxillary and -facial problems in cleidocranial dysplasia]. / Dentomaxillaire en -faciale problemen bij cleidocraniale dysplasie.

A 14-year old boy with cleidocranial dysplasia was referred because of an almost complete presence of the deciduous dentition. Diagnostics by means of a panoramic radiograph, lateral headfilm, CT scan and 3D computer images of the dentomaxillary complex showed 7 supernumerary permanent teeth: 13', 11', 21', 23', 28', 35' and 45'. A combined orthodontic-surgical treatment was started. The supernumary teeth were removed surgically and the impacted teeth are exposed. The closed eruption technique was used to extrude these teeth orthodontically.

The Leuven staged supraperiosteal retropositioning repair: long-term velopharyngeal function in non-syndromic cleft palate.

BACKGROUND: From 1989 to 1993, 113 previously untreated patients were admitted to the Multi-disciplinary Cleft Lip and Palate Team of the University Hospitals Leuven. Palate repair was performed in our centre by one surgeon (FO) in 88 patients. Our current surgical technique consists of a single-stage supraperiosteal retropositioning (modified Veau-Wardill-Kilner) for patients with a soft cleft palate only (SCP) or a soft cleft palate with up to 1 cm of the hard palate (HSCPpa). Patients with a larger or complete cleft of the secondary hard palate (HSCP) and patients with unilateral (UCLP) or bilateral (BCLP) cleft lip and palate undergo two surgical stages for palate closure: a supra-periosteal retropositioning is performed around 12 months of age, and a modified Langenbeck closure of the hard palate around 60 months of age. AIM: To assess velopharyngeal function with speech as outcome measure. PATIENTS AND METHODS: Velopharyngeal function was assessed in two ways. In one assessment, a "hard" outcome measure was the number of patients undergoing pharyngoplasty following palate repair in our centre (n = 88). In the other assessment, velopharyngeal function was evaluated in a homogeneous sub-population of 44 non-syndromic cleft patients with normal to slight impairment of the following functions: mental development, language development, and hearing. In this group, prospectively collected data about hypernasality and nasal emission were analysed retrospectively using a semi-objective nasality index (NI). Articulation was evaluated using a subjective articulation index (AI) representing articulation errors (retro-articulation, glottal stops and facial grimacing) associated with velopharyngeal insufficiency (VPI). Mean follow-up was 114 months. RESULTS: Despite rigid assessment by a phoniatrician and speech pathologist, only 1 patient out of 88 patients with soft palate surgery in our institution was thought to need pharyngoplasty. In the sub-cohort of 44 non-syndromic patients, nobody needed a pharyngoplasty. In the latter cohort, at the age of about eight years, 27 patients (61.5%) had undetectable nasality, 13 patients (29.5%) had an NI of 1 or "mild" nasality, and 4 patients (9%) had moderate nasality. At this point in time, articulation errors associated with VPI were noted in 14% of patients. CONCLUSION: In this subgroup of cleft palate patients treated following the Leuven protocol, there was no need for secondary pharyngoplasty. Ninety-one per cent of patients had no, or only mild, rhinolalia aperta by the age of eight years, and 84% did not display VPI-related articulation disorders. This suggests that velopharyngeal function in patients treated by this protocol is excellent compared to results in the literature.

Treatment of hemifacial microsomia in a growing child: the importance of co-operation between the orthodontist and the maxillofacial surgeon.

The treatment of patients with hemifacial microsomia (HM) always requires an interdisciplinary approach including at least maxillofacial surgery and orthodontics. Co-operation not only within the team, but also with the patients and their family is essential in order to achieve the best results. In the case history of the 10-(1/2) year old female patient reported here, three surgical interventions (two with costo-chondral bone grafts) and a 3-year orthodontic treatment have taken place. A harmonious facial and occlusal result was finally reached.