Orthodontic Treatment of a Kabuki Syndrome Patient.

Kabuki syndrome (KS) is a rare disorder characterized by somatic and psychological disturbances including special face morphology, skeletal anomalies, and other systemic disorders. Because of the diverse clinical manifestation, the management of a patient with KS may involve several medical and dental specialists, including orthodontics. The aim of the article is to present successful orthodontic treatment performed in a 14-year-old boy diagnosed with the KS. Dental relations and smile aesthetics were normalized after orthodontic treatment; however, problems with patient compliance and cooperation and an increased risk of root resorption may influence treatment outcomes. Interdisciplinary cooperation between medical and dental specialists is essential in patients with KS.

Infant and baby feeding and the development of the maxillofacial complex based on own observations and the literature.

The method and technique of feeding a young child affect the shape of the maxillofacial complex. Breastfeeding is the recommended method of feeding in the first six months of life. It is encouraged to continue natural feeding in later months, simultaneously developing other food extraction techniques. The correct formation of the stomatognathic system is a result of the correct organization of the motor patterns during feeding.

Craniofacial Morphology in Midline Cervical Cleft: Case Report and Review of Literature.

Congenital midline cervical cleft is a rare anomaly of the neck. This paper presents the case of a boy diagnosed with this disorder in which a preliminary orthodontic treatment was implemented. The craniofacial anomalies associated with this malformation produced a defect that could only be successfully treated through the implementation of orthodontic and surgical treatments. In this case, congenital midline cervical cleft was accompanied by certain disorders within the facial structures of the skull, primarily mandibular retrusion, flattening of the contour of the mandibular base, and a steep angle between the cranial base and the mandibular plane.

Evaluation of periodontal tissues in growing patients with bilateral cleft lip and palate. A pilot study.

AIM: To evaluate the periodontal status, mucogingival parameters and oral hygiene in growing patients with bilateral cleft lip and palate. MATERIAL AND METHODS: Assessment was performed in 15 patients aged 6 to 18 years with a bilateral cleft. Records included probing pocket depth, clinical attachment level, keratinized gingiva, recession, vestibule depth, biotype, type of fraena, dental plaque and bleeding. RESULTS: The mean scores of pocket depth were: 1.9 mm for central incisors, 1.6 mm for lateral incisors, 1.7 mm for canines, 2.0 mm for first premolars. There were only a few teeth with minimal attachment loss (1 mm). Gingival recessions were not recorded. High scores were recorded for the hygiene indicator, especially on the buccal, mesial and distal surfaces. Due to soft and hard tissue malformations, it was difficult to precisely assess the biotype and keratinized gingiva. However, keratinized gingiva was narrower near the teeth adjacent to the cleft. Similarly, the vestibule was shallower in this area. In 12 out of 15 children it was impossible to define the type of labial fraenum. CONCLUSIONS: Evaluation of the periodontal status is important for successful comprehensive rehabilitation in cleft patients. Specific features of hard (alveolar process) and soft tissue (scars, unusual fraena) malformations caused by the cleft and previous surgical procedures have functional and morphological implications. Narrower gingiva and a shallower vestibule in the presence of dental plaque and bleeding are unfavourable conditions to maintain a healthy periodontium. It is essential to include periodontal assessment and preventive treatment to a comprehensive approach as early as possible.

Impact of incorrect oral habits on mastication anomalies in children and adolescents - literature review and own observations.

Incorrect oral habits, such as dysfunctions and parafunctions, may lead to the development of mastication anomalies. The present analysis covered 15 publications from the years 2005-2015, examining the impact of harmful oral habits and dysfunctions on the development of mastication anomalies. Based on the literature review, it can be stated that malocclusion is common in populations worldwide, irrespective of the racial background. The malocclusion severity is related to harmful habits and dysfunctions co-occurring during individual development. The prevalence of malocclusion and dental anomalies related to parafunctions and dysfunctions has been observed to have a tendency to grow.

Occluso-facial manifestations in a child with the Rasmussen syndrome and intercurrent Lyme disease--a case report.

The Rasmussen Syndrome (RS) is a rare neurological disease, usually diagnosed in the 1st decade of life, leading to damage of the central nervous system. It is characterised by sudden seizures in previously healthy children. In this paper we present a case of a 14-year-old female patient with malocclusion who reported to the orthodontic clinic to start orthodontic treatment. The first symptoms of nervous system disorders manifested when the patient was 8 years old. Until then, the patient had been developing correctly. The interview revealed that at the age of 9 the patient was diagnosed with Lyme disease and a long-term antibiotics therapy was started. At the age of 10, the Rasmussen syndrome was diagnosed (the antibiotics therapy was discontinued) and confirmed by magnetic resonance imaging (MRI) and histopathological examination after brain biopsy. The study focuses in particular on the changes in the craniofacial skeleton and oral cavity, taking into account dynamically developing and increasing occlusion defects. The ethical aspect of the treatment has also been considered.

Evaluation of changes in the width of gingiva in children and youth. Review of literature.

Comprehensive health care in children and youth includes periodic oral examinations. The mucogingival complex undergoes significant changes during development. The factors that impact the width of attached and keratinized gingiva during this period of life are: tooth eruption phase, the position in the arch, the type of frena attachment and oral hygiene. Along with the child's development the width of attached and keratinized gingiva increases, except for the period of tooth replacement, when a temporary narrowing of attached gingiva of erupting permanent teeth is observed. The understanding of physiological processes of the mucogingival complex is prerequisite for diagnostics and treatment of gingival abnormalities in children and youth. Therefore close cooperation between paediatrician and dental specialists: paedodontist, orthodontist and periodontologist is essential.

[Fixed appliance therapy in patients with impaired short-circuit in the anterior part of the maxilla]. / Terapia aparatem stalym u pacjentów z zaburzeniami zwarcia w przednim odcinku szczeki.

UNLABELLED: Malocclusion in the anterior segment of maxilla and mandible are easily visible not only for dentists but also for the doctors of other specialties. Early diagnosis and appropriate therapy is important not only for occlusion but also for aesthetic reasons. AIM: The aim of the paper is to evaluate the anterior segment of maxilla and mandible in patients with malocclusion in this part and correct occlusion in the lateral segments. MATERIALS AND METHODS: Medical documentation, i.e. medical history, extra- and intraoral radiograms, diagnostic casts, panoramic and lateral cephalometric radiograms of patients aged 7-12 diagnosed with malocclusion in the anterior segment of maxilla and mandible and who were treated with a fixed sectional appliance and facemask was analyzed. Descriptive and cephalometric features were analyzed before (T1) and after (T2) the treatment in 25 children. The differences between the status before and after the treatment, and the extent of change between T1 and T2 were analyzed. RESULTS: Statistical analysis of mean values of selected metrical features before (at T1) and after (at T2) the treatment has revealed that all metrical features concerning soft, bony and dental tissues determining the facial profile, the shape of the bony and dental structures have changed and have reached values which are closer to the norm for the population for selected features. The changes were statistically significant (p<0.0001). CONCLUSIONS: Treatment with fixed appliances segment facemask resulted in statistically significant improvement in the parameters investigated, which demonstrates the applicability of this therapy in the treatment of anterior maxillary segment in patients with mixed dentition.

Malocclusions and craniofacial anomalies in a child with velo-cardio-facial syndrome.

Velo-Cardio-Facial syndrome (VCFS), also called 22q11.2 microdeletion syndrome, is a rare pathology. The syndrome is caused by 22q11.2 deletion, recognized as one of the most frequent pathogenic human microdeletions. The scope and severity of the phenotypic expression of 22q11.2 microdeletion is characterised by high variability, although cleft palate and congenital conotruncal malformations are among the clinical features often associated with that syndrome. In the presented case of a boy patient with submucous cleft palate and congenital cardiac defect, 22q11.2 microdeletion was identified at the age of 13 months. In the presented paper particular emphasis was placed on the issue of dental and orthodontic care in patients with changes in the oral cavity and the craniofacial area, as well as on the possibilities of treatment and prophylaxis. The necessity to perform a thorough examination of the oral cavity in infants was also underlined as a vital element of clinical assessment, in particular in the case of co-occurring structural defects of internal organs.

Microform cleft lip or congenital healed cleft lip?

This paper presents the case of a patient with a visible scar indicating the occurrence of a cleft lip in the past. Anamnestic examination ruled out any facial surgery having been performed on the child and the parents confirmed the existence of the scar from birth. Intraoral examination revealed hypodontia of a lateral incisor on the side opposite the scar as well as hyperdontia of lateral incisors on the same side as the scar. This seems to confirm the existence of a cleft in the primary palate during gestation. A microform cleft primary palate is extremely rare and there are only isolated reports in the international literature relating to the spontaneous healing of such defects in utero.

Orthodontic treatment of patients with a cleft lip and palate - standard procedures at the Warsaw Institute of Mother and Child.

Based on many years of multidisciplinary research relating to children and adolescents with a cleft lip and palate who had undergone one-step surgery at the Warsaw Institute of Mother and Child (IMC), orthodontic treatment standards have been developed for this group. IMC standard orthodontic assessment is conducted in children at the ages of 5, 10 and 15. This assessment involves a clinical examination, preparing diagnostic models, photographic documentation, as well as radiographic examination including a pantomogram, a lateral cephalometric radiograph and, where appropriate, also computed tomography. The treatment is conducted as part of the Programme of Orthodontic Care for Children with Congenital Craniofacial Disorders, and it lasts from a child's first appointment at the IMC till the completion of orthodontic treatment at the age of 18.

Long-term folow-up after distraction osteogenesis with face mask in cleft lip and palate patients - preliminary report.

INTRODUCTION: Patients with cleft lip and palate are often diagnosed with maxillary deficiency, i.e. the reduction of maxillary measurements in three dimensions. The method of distraction osteogenesis (DO) remedies the disorder caused by bone insufficiency by generating new bone tissue. Despite the fact that DO has long been considered an effective method to treat significant bone deficiencies, it is still not a standard treatment applied by clinicians to manage underdevelopment, especially in the maxillary area. There is only a small number of publications concerning the long-term follow-up results of the use of that method to treat patients with cleft lip and palate. THE OBJECTIVE: Long-term analysis of dentoskeletal changes in cleft lip and palate patients treated with distraction osteogenesis and the use of a facial mask. MATERIAL AND METHODS: In 2001-2004, 30 patients with cleft lip and palate and related maxillary deficiency were treated by means of distraction osteogenesis and a facial mask. However, due to incomplete documentation, only 15 persons, having full medical documentation and a set of cephalometric radiographs taken in three specified time periods, were qualified for the study. The cephalometric analysis was performed on the following lateral head radiographs: pre-distraction images (T0), images taken after active distraction (T1) and 5 years after the completion of the distraction process (T2). A group of 12 persons participated in the 5-year follow-up measurements (T2). RESULTS: Cephalometric assessment indicated that after the use of distraction osteogenesis, the maxilla advanced by the mean of 3.84 mm and the SNA angle increased by the mean of 2.76° (statistically significant data). The maxillary advancement was accompanied by downward mandibular rotation. Long-term assessment after 5 years indicated that the SNA angle was reduced by 0.64°. CONCLUSIONS: The use of distraction osteogenesis with a facial mask to manage maxillary deficiency deserves more attention. It requires a close cooperation of the orthodontic and surgical team, as well as high motivation on the part of the patient. In order to provide the final assessment of the long-term results, further studies need to be conducted on larger clinical material.

Complete unilateral cleft lip and palate operated on by means of the one-stage method - own experience.

The study presents the surgical protocol of unilateral cleft lip and palate (UCLP) treatment based on the one-stage method. It is exemplified by the treatment course of a patient suffering from UCLP. The method was worked out and implemented at the Institute of Mother and Child in Warsaw. Its main advantage is the early establishment of the anatomical structures of the naso-oral cave and the facial part of the skull, before the development of speech. The complete closure of each part of the cleft - the soft and hard palate and the lip before the end of the first year of life, enables the quick implementation of the next stages of treatment, such as alveolar osteoplasty, which facilitates both orthodontic treatment and speech therapy.

Eating habits of patients with cleft lip and palate terated with fixed appliances.

INTRODUCTION: Health education, built on the basis of various models as a lifelong process, has a considerable impact on eating habits. Correct nutritional patterns are particularly important during the period of intense growth and adolescence. The need to observe dietary recommendations concerns healthy, as well as sick persons, especially those with chronic diseases. To-date the data on the dietary habits of patients with cleft lip and palate during orthodontic treatment have been scarce. OBJECTIVE OF THE STUDY: The objective of the study was to determine the eating habits of patients with cleft lip and palate before and during orthodontic treatment with a fixed appliance. MATERIAL AND METHOD: The study covered 125 patients with cleft lip and palate, aged 14 to 31. The research tool was an own questionnaire assessing the dietary behaviour and oral hygiene habits during the treatment with a fixed orthodontic appliance. RESULTS: In the course of the orthodontic treatment with a fixed appliance, 79 patients (57.6%) did not change their eating patterns and 32 patients (24%) changed them to a moderate degree. The mean of 53 patients (42.7%) changed their hygienic habits considerably. The number of meals remained the same, however in the period when patients suffered pain after orthodontic adjustment appointments, the number of persons having 4-5 meals a day decreased by 32%. It is worth considering that no statistically significant changes were recorded as regards the habit of having sweet snacks and the insufficient consumption of fruit and vegetables. Difficulties with correct oral hygiene were reported after the consumption of e.g. spinach (59.8%), cucumber (57.6%), berries (54.4%), meat (58.2%). CONCLUSIONS: Eating habits of a considerable percentage of patients with a cleft defect, before or during the treatment with a fixed appliance, do not comply with healthy nutrition recommendations. The dietary education of patients undergoing orthodontic treatment is necessary to ensure proper oral health and prevent diet-related illnesses.

Pulp Revascularization After Autotransplantation of the Mandibular Canines With Partially Resected Roots: Report of 5 Cases With Follow-ups Between 26 and 80 Months.

INTRODUCTION: Transmigrated (migrated through the midline) mandibular canines constitute a treatment challenge. Advanced transmigration can be successfully treated by autotransplantation. In developing canines, pulp revascularization is typical after transplantation. On the contrary, the pulp of teeth with completed apex formation does not undergo revascularization. In this case, root canal treatment becomes compulsory and decreases the success of autotransplantation. The aim of this observational retrospective study was to evaluate whether partial resection of the root (performed during the autotransplantation of mature canines) would enable revascularization of the pulp after the surgery. METHODS: Five transmigrated mandibular canines with complete apex formation before the surgery were evaluated. During the autotransplantation surgery, the resection of 2 to 4 mm of the root apex was performed to open the path for revascularization of the pulp after surgery. The transplanted teeth were observed during healing after the surgery for the presence of pulp obliteration. Clinical and radiographic examinations were performed. RESULTS: All treated canines survived the minimum observation period of 2 years (ranging from 26 to 80 months, mean: 55 months) without pulp healing complications. The survival was 100%, and the success was 80%. In one canine, the external cervical root resorption was diagnosed and treated 1 year after the surgery. At the final examination, transplanted canines presented radiographic features of pulp obliteration, normal mobility, and healthy periodontal tissues. CONCLUSIONS: The surgical protocol proved to be successful in promoting revascularization to maintain pulp vitality, in all cases. The outcomes confirm that autotransplantation, combined with the resection of the root, constitutes a valid treatment for mature ectopic canines.

Periodontal Indices and Status in 34 Growing Patients with Unilateral Cleft Lip and Palate: A Split-Mouth Study.

Periodontal examination in growing patients with unilateral cleft lip and palate was performed in a split-mouth study design. Higher plaque acumulation and bleeding indices were recorded for teeth on the cleft side. Pocket probing depth (PPD) and clinical attachment level (CAL) values were higher at lateral incisors and canines adjacent to the cleft. Keratinized gingiva and depth of the vestibule were lower adjacent to the cleft. This study shows that growing patients with clefts present differences regarding periodontal parameters between the cleft and the control side. These alterations affected patients' ability to maintain proper oral hygiene and resulted in differences in PPD and CAL.

Rehabilitation of the orofacial complex by means of a stimulating plate in children with Down syndrome.

BACKGROUND: Disorders in the orofacial complex as well as muscle hypotension in children with Down syndrome can be corrected through orofacial therapy that makes use of a palatal plate. OBJECTIVES: The aim of this paper was to present how parents of children with DS assess the availability of treatment and the therapeutic effects of orthodontic palatal plate therapy on the tongue position and mimetic muscle tension in their children; and to determine whether implementing full Castillo-Morales therapy and using only a palatal plate lead to substantially different results. MATERIAL AND METHODS: The study was conducted on 100 children with DS between the ages of two months and two years (44 boys, 56 girls) who were treated by means of stimulating plate therapy. The study analyzed responses obtained from the parents to questions included in a questionnaire completed at every visit over a period of two years. RESULTS: The study found that in 50 patients with DS who underwent palatal plate rehabilitation there was a visible improvement of the mimetic muscles, tongue retraction and lip closure. CONCLUSIONS: Early orthodontic palatal plate therapy ought to be an integral part of the multidisciplinary rehabilitation of patients with DS.

Telomere shortening in Down syndrome patients--when does it start?

Down syndrome (DS) is one of the most common aneuploidy. In general population, its prevalence is 1:600-1:800 live births. It is caused by a trisomy of chromosome 21. DS is phenotypically manifested by premature aging, upward slant to the eyes, epicanthus, flattened face, and poor muscle tone. In addition to physical changes, this syndrome is characterized by early onset of diseases specific to old age, such as Alzheimer's disease, vision and hearing problems, and precocious menopause. Since DS symptoms include premature aging, the shortening of telomeres might be one of the markers of cellular aging. Consequently, the aim of the study was to determine the length of the telomeres in leukocytes from the blood of juvenile patients with DS (n=68) compared to an age-matched control group (n=56) and also to determine the diagnostic or predictive value for this parameter. We show that, for the first time, in juveniles, the average relative telomere length in studied subjects is significantly longer than in the control group (50.46 vs. 40.56, respectively arbitrary units [AU]; p=0.0026). The results provide interesting basis for further research to determine the causes and consequences of telomere maintaining and the dynamics of this process in patients with DS.

Smith-Lemli-Opitz syndrome - clinical consequences for dental care.

The Smith-Lemli-Opitz syndrome (SLOS) is a congenital, genetically conditioned, metabolic disorder with autosomal recessive inheritance. The syndrome is caused by high levels of cholesterol precursors, i.e. 7-dyhdrocholesterol (DHCR7) and 8-dehydrocholesterol (DHCR8), which results in cholesterol synthesis disorders. Cholesterol deficiency leads to a series of developmental disorders in the foetus and in extreme cases even death. The diagnosis of that congenital metabolic disorder is difficult due to a diversity of clinical symptoms (there are many mutations of the DHCR7 and DHCR8 genes), which can be exemplified by such cases as that of the 5-year-old boy with SLOS described below. In the presentation the authors have emphasized the problems related to hygienic procedures of the oral cavity and dental treatment, as well as the importance of the early diagnosis of the syndrome and of cooperation with parents. The publication aims at drawing the attention of clinicians to the need of taking SLOS into account in the differential diagnostics in children.

[Moebius syndrome with facial-dental impairments - rare or rather seldom diagnosed syndrome?]. / Zespól Moebiusa Z Zaburzeniamitwarzowo"zebowymi. Zespól Rzadkowystepujacy, Czy Rzadko Rozpoznawany?

As publications on craniofacial anomalies, malocclusions and dental complications recognised in patients suffering from Moebius syndrome are scarce, the authors of this paper decided to discuss the above aspects in broader terms along with the possibilities offered by orthodontic treatment. The etiology of Moebius syndrome has not hitherto been discovered, however the opinion prevails that it is brought on by multiple factors and conditions. In the analysed case, Moebius syndrome was diagnosed only when the patient was 6 years old. Based on the clinical examination, typical characteristics of the syndrome were observed: craniofacial dysmorphism as well as foot development disorder in the form of talipes equinovarus (club foot). Moreover, Type II Angle's classification of malocclusion was detected - crowded teeth in the mandible and maxilla and hypoplastic enamel. Cephalometric analysis identified retruded position of the mandible against the cranial base, protruded position of the maxilla, shortening of posterior face height, protrusion of incisors in the maxilla. The orthopantomogram showed the presence of all permanent teeth. At the beginning of the orthodontic treatment removable appliances were used, but despite good cooperation on the part of the patient, only a slight improvement was observed. Further orthodontic treatment envisaged extraction of permanent teeth and use of fixed appliances while waiting for the improvement of occlusion.