Surgical Treatment of Oromandibular Limb Hypogenesis Syndrome Type I A by Distraction Osteogenesis Combined With Orthodontic Rehabilitation.

ABSTRACT: Oromandibular limb hypogenesis syndrome is a rare developmental anomaly and only a few cases are reported with complete surgical and orthodontic rehabilitation. An adult male patient with isolated hypoglossia, micrognathism, hypodontia, (oromandibular limb hypogenesis syndrome type I A) was treated with a combination of distraction osteogenesis and orthodontic intervention. The patient was followed up for the duration of 6 years from his first visit to 4 years after the surgery. The combined procedure resulted in successful and satisfactory treatment of the patient by restoring facial aesthetics, occlusal balance, and functional harmony. However, there was not enough tongue enlargement due to late surgical intervention. The objective of this report is to describe the etiology of hypoglossia, the consequences for oral function, and to share our experience from the oral rehabilitation during the treatment procedure.

Orthognathic Hardware Complications in the Era of Patient-Specific Implants.

BACKGROUND: Patients undergoing orthognathic skeletal correction present with a variety of comorbidities that may affect surgical outcomes. The purpose of this study was to determine how patient risk factors and operative technique contribute to complication rates after orthognathic surgery in the era of patient-specific implants. METHODS: Retrospective cohort analysis was conducted of pediatric patients undergoing Le Fort I osteotomy, bilateral sagittal split osteotomy, and/or genioplasty from 2014 to 2018. Patient risk factors, operative characteristics, and postoperative outcomes were gathered and compared with appropriate statistics. RESULTS: Ninety-four patients met inclusion criteria, with an overall 1-year complication rate of 11.7 percent (11 of 94). Patient-specific mandibular plates are significantly associated with infection (p = 0.009; OR, 8.8), occurrence of any complication (p = 0.003; OR, 8.3), readmission (p < 0.001; OR, 11.1), and reoperation (p < 0.001; OR, 11.4). In patients with syndromes or history of cleft lip/palate, patient-specific mandibular plates are associated with infection (p = 0.006; OR, 10.3), readmission (p < 0.001; OR, 21.6), and reoperation (p < 0.001; OR, 22.9). In multivariate regression controlling for age, sex, syndrome status, and orofacial cleft history, use of patient-specific mandibular plates was associated with infection (p = 0.017; adjusted OR, 12.5), any complication (p = 0.007; adjusted OR, 11.8), readmission (p = 0.001; adjusted OR, 17.9), and reoperation (p = 0.001; adjusted OR, 18.9). CONCLUSIONS: In the era of patient-specific orthognathic surgery, syndromic status and use of patient-specific mandibular plates are associated with increased infection, readmission, and reoperation because of hardware-related complications. The authors' data support increased caution and counseling with use of patient-specific mandibular implants in patients with syndromic status, history of orofacial cleft, and history of previous maxillomandibular surgery given increased risk of hardware-related complications. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Radiographic review of anatomy and pathology of the masticator space: what the emergency radiologist needs to know.

The differential diagnosis of a masticator space (MS) lesion is broad, owing in part to the multiple structures contained within such a small region. It is also because the MS is adjacent to many of the other deep spaces within the head and neck, which can act as gateways for disease spread. Therefore, emergency radiologists must be familiar with anatomy of the MS, as well as adjacent spaces in order to provide an accurate diagnosis to the referring clinician. This article illustrates the anatomy and common pathologies within the MS using a case-based multimodality approach. Common masticator space pathologies can be categorized into inflammatory/infectious, neoplastic, and vasoformative lesions. Important imaging features of MS lesions and patterns of disease spread will be discussed, with the aim of making this complex deep space more approachable in the emergent setting.

Analysis of the stomatognathic system of children according orthodontic treatment needs.

PURPOSE: The present study evaluated electromyographic activity (EMG), masticatory performance, and tongue strength in children without and with orthodontic treatment needs. PATIENTS AND METHODS: A total of 90 children were screened and divided into the following groups: Group I (no treatment needed; mean age: 8.00 ± 0.43 years; n = 26), Group II (few malocclusions, treatment needed; mean age: 8.89 ± 0.43 years; n = 28), and Group III (slight-to-borderline treatment needed; mean age: 8.44 ± 0.22 years; n = 36). Orthodontic treatment need was classified on the basis of IOTN-DHC (Index of Orthodontic Treatment Need - Dental Health Component). The electromyographic Trigno EMG Systems was used for muscle analysis and the Iowa Oral Pressure Instrument (IOPI) was used to measure tongue strength. Data were analyzed using normality tests and one-way analysis of variance with a Bonferroni post hoc test (p ≤ 0.05). RESULTS: EMG in almost all mandibular movements was higher in Group III with statistically significant differences compared to position at rest: right masseter (p = 0.03); protrusion: left temporal (p = 0.02); saliva swallowing: left temporal (p = 0.05) and water swallowing: orbicularis oris mouth, right upper segment (p = 0.05). Lower masticatory performance was found in Group III, but the difference compared to Group I and II was not significant. There were no significant differences between the groups in terms of tongue strength. CONCLUSIONS: Children with borderline orthodontic treatment needs show functional disorders of the stomatognathic system.

Evaluation of stomatognathic problems in children with osteogenesis imperfecta (osteogenesis imperfecta - oi) - preliminary study.

According to epidemiological data, muscular dysfunctions of the masticatory system occur in 15-23% of the population. Preventive examinations of functional disorders of the stomatognathic system are, therefore, of particular importance. A distinct group of patients exposed to dysfunctions in the area of the masticatory organ locomotor apparatus comprises those with genetic diseases characterised by disorders in collagen formation. One of such diseases is osteogenesis imperfecta (OI) and dentinogenesis imperfecta that usually goes together with the former. AIM: The objective of this work was to evaluate the frequency with which particular disorders of the masticatory organ locomotor apparatus occur within the group of patients with osteogenesis imperfecta. MATERIAL AND METHODS: The study was performed on patients of the Orthopaedic Clinic of the Polish-American Paediatric Institute in Kraków. The mean age of the children was 7.9 years. In all the cases, a genetic diagnosis of OI has been confirmed. The research methods were based on an in-depth interview on family diseases, pregnancy, postnatal period, feeding, subjective assessment of dysfunctions in the stomatognathic system. An examination of the deformations in the stomatognathic system and the skeleton was conducted, as well as an examination of the trauma and tone of the jaw. The relationship between breastfeeding and swallowing and speech disorders was also evaluated. The impact of intubation on mandibular ranges was investigated. RESULTS: The results obtained were subjected to statistical analysis on the basis of which conclusions were drawn concerning disorders in the stomatognathic system which tend to occur in children with OI. The renunciation of breastfeeding significantly contributes to sucking and swallowing disorders, rumen disorders, as well as biomechanical disorders in the temporomandibular joint. A significant dependence between breastfeeding and swallowing problems was found, whereas there was no such dependence with respect to speech impediments. CONCLUSIONS: The results of the research conducted led to the following conclusions: 1. Among pediatric patients with OI there are disorders in the stomatognathic system. The most common dysfunctions are: abdominal, swallowing and sucking disorders, abnormal muscle structure of the rumen and biomechanical disorders in the temporomandibular joints. Breastfeeding significantly contributes to swallowing disorders. 2. The therapeutic process involving children with OI requires the cooperation of specialists in orthopedics, pediatrics, physiotherapy, orthodontics and neurologopedics to carry out comprehensive diagnostics and treatment tailored to the individual needs of the patient. 3. In order to draw final conclusions, there is a need for more research by means of objective tools, such as EMG and a condensate recorder.

Pfeiffer syndrome: oral healthcare management and description of new dental findings in a craniosynostosis.

Pfeiffer syndrome is a rare fibroblast growth factor receptor-related craniosynostosis with variable clinical presentations. We describe new dental findings of hypodontia, microdontia, dilacerations, and radicular dentin dysplasia in a 19-year-old girl, and discuss the oral health management.

Computational and clinical investigation on the role of mechanical vibration on orthodontic tooth movement.

The aim of this study is to investigate the biomechanics for orthodontic tooth movement (OTM) subjected to concurrent single-tooth vibration (50Hz) with conventional orthodontic force application, via a clinical study and computational simulation. Thirteen patients were recruited in the clinical study, which involved distal retraction of maxillary canines with 1.5N (150g) force for 12weeks. In a split mouth study, vibration and non-vibration sides were randomly assigned to each subject. Vibration of 50Hz, of approximately 0.2N (20g) of magnitude, was applied on the buccal surface of maxillary canine for the vibration group. A mode-based steady-state dynamic finite element analysis (FEA) was conducted based on an anatomically detailed model, complying with the clinical protocol. Both the amounts of space closure and canine distalization of the vibration group were significantly higher than those of the control group, as measured intra-orally or on models (p<0.05). Therefore it is indicated that a 50Hz and 20g single-tooth vibration can accelerate maxillary canine retraction. The volume-average hydrostatic stress (VHS) in the periodontal ligament (PDL) was computationally calculated to be higher with vibration compared with the control group for maxillary teeth and for both linguo-buccal and mesial-distal directions. An increase in vibratory frequency further amplified the PDL response before reaching a local natural frequency. An amplification of PDL response was also shown to be induced by vibration based on computational simulation. The vibration-enhanced OTM can be described by mild, vigorous and diminishing zones among which the mild zone is considered to be clinically beneficial.

Single stage correction of bilateral tessier 4 cleft.

Tessier facial clefts are among the rarest facial clefts reported in literature and many contradicting issues have always been rising over the management and surgical approaches involved during the craniofacial cleft repair. Among the craniofacial clefts Tessier no. 4 is an extremely rare facial anomaly, and there are very few evidence which clearly describe the surgical approaches and techniques. Often these type of craniofacial clefts yield very poor surgical results, and they require multidisciplinary sequential corrective surgeries. This article presents a rare case of an 18-month-old baby with bilateral Tessier no. 4 clefts and its successful rehabilitation.

Steinert syndrome and repercussions in dental medicine.

Steinert syndrome, also called myotonic dystrophy type 1, is a genetic disorder with autosomal dominant transmission characterized by myotonia and a multisystemic clinical picture that affects several tissues of the human body. The most common systemic phenotypes are: muscular, cardiac, respiratory, CNS, ocular, gynecological, digestive, orthopedical, as well as cognitive and psychological symptoms (cognitive decline). Muscles involved in voluntary movement are highly affected by myotonia especially distal muscles of upper limbs. These patients also show changes in face, chewing and pharynx muscles that can lead to swallowing and speech problems, dysphagia and in most cases to food aspiration and suffocation. Poor oral hygiene resulting from reduced motor mobility and reduced saliva flux can lead to gingival inflammation and periodontal disease. Other oral manifestations include disturbances at the temporomandibular articulation, dental occlusion changes and reduction in teeth number as a result of caries. Main causes of death are pneumonia and cardiac arrhythmias. The etiopathogeny of this syndrome is still not clear, conditioning the existence of a specific treatment for this disease. Nowadays, treatments consist on the release of the existing symptoms, in an attempt to give a better life quality to patients. It is very important to implement actions that can prevent complications and consequently decrease death. Treatments should be applied in an early stage of the disease. Bronchoscopy and artificial respiration should be used to prevent pneumonia, and regular electrocardiographic monitoring should be done to evaluate defects in the conductive system. Several approaches have been applied to rehabilitate swallowing dysfunction and avoid aspiration like videofluoroscopy, postural techniques and adjustment of diet type. It is the aim of this paper to clarify the ethiology, diagnosis, systemic and oral characteristics of the syndrome, as well as to discuss treatments to be applied according to patients affected organs.

Oral and craniofacial manifestations of Ellis-van Creveld syndrome: Case series.

PURPOSE: The objective of this case series was to determine the oral, dental and craniofacial features of patients with EvC syndrome. MATERIAL AND METHODS: Eight patients with EvC syndrome were enrolled. A complete family history, pedigree analysis, detailed medical history were collected. Findings of clinical examination, including craniofacial and orodental manifestations, and radiological investigations were thoroughly studied. RESULTS: All eight patients had characteristic face, hypertrophic frenulum, conical and peg-shaped teeth, hypodontia of deciduous and/or permanent teeth and also skeletal dysplasia, small chest, short stature and hypoplastic nails. Additionally dysmorphic filtrum, serrated appearance of gingiva, diastema, enamel hypoplasia, microdontia, taurodontism, single rooted permanent molar, delayed eruption and high caries rate were observed with varying degrees. Cephalometric evaluation revealed skeletal Class III growth pattern in four subjects and Class II growth pattern in one subject. CONCLUSION: Evaluation of craniofacial and orodental anomalies of EvC syndrome is required for accurate differential diagnosis from other congenital syndromes.

Stomatognathic evaluation at five years of age in children born premature and at term.

BACKGROUND: The high frequency of alterations of the stomatognathic system associated with premature birth may suggest that prematurity is an important risk factor in the development of this system. Prematurity has an incidence between 6-11% of births and is associated with factors such as genetic, maternal conditions (obstetric problems, nutritional status, infections) and antenatal care. In addition, undesirable situations, such as changes in enamel and the development of the skeletal structure, also appears to be associated with prematurity. This study aimed to look for changes in the stomatognathic system at five years of age associated with premature birth. METHODS: We estimated the prevalence of developmental disorders of the stomatognathic system in the primary dentition of preschool children at five years of age. Changes in preterm infants (n = 32) compared with term born (n = 381) were evaluated . Clinical examinations and questionnaire with sociodemographic and health of mothers and children information. Gestational age, birth weight, head circumference, Apgar score and mechanical ventilation, were collected from the medical records to birth records. The explanatory variable was preterm (<37 weeks gestational age). RESULTS: Prevalence of 7.7% of preterm infants was found. Of these, 40.6% had atresic palate, 56.2% malocclusion and 21.8% enamel hypoplasia. Forty (9.6%) children were not breastfed at the breast, and 26 (65.0%) had some type of malocclusion, showing association between not breastfeeding with an abnormal development of the stomatognathic system. The group of preterm infants showed five times more changes in head circumference and three times more mechanical ventilation use at birth. Change in head circumference at birth and mechanical ventilation has a significant association between groups of preterm and term infants. CONCLUSIONS: Mechanical ventilation at birth directly contributed to an increased risk of developmental disorders of the stomatognathic system in preterm infants, especially dental hypoplasia. Non-breastfed children had a higher risk of developing malocclusion. Alterations in head circumference were related effective on dental malocclusion. The results suggest that changes in the stomatognathic system are influenced by premature birth and points to the imperative need of using methods of preventive.

[APPLICATION OF THREE-DIMENSIONAL CEPHALOMETRIC ANALYSIS IN ORTHOGNATHIC SURGERY].

OBJECTIVE: To establish a model of three-dimensional (3-D) cephalometric analysis to study dentomaxillofacial deformities. METHODS: Between January 2012 and October 2013, 15 patients with dentomaxillofacial deformities were treated using 3-D cephalometric analysis in orthognathic surgery plan. There were 7 males and 8 females with an average age of 23.6 years (range, 17-37 years), including 4 cases of mandibular protrusion with maxillary deficiency, 4 cases of maxillary protrusion with mandibular deficiency, 2 cases of long face syndrome, and 5 cases of facial asymmetry. CT images were reconstructed by Mimics software. The anatomical landmarks were located, the reference planes and analysis planes were defined and the 3-D coordinate was established. The distance and degree between landmarks and analysis planes which defined in the measure project were measured. RESULTS: Based on the 3-D CT quantitative analysis methods, cephalometric analysis project was defined in the 3-D coordinate. 3-D cephalometric analysis provided a convenient and precise method for the clinical measurement of dentomaxillofacial morphology, and reduce the time in preoperation analysis. CONCLUSION: The model of 3-D CT cephalometric analysis can provide precise information in the diagnosis and treatment planning of orthognathic surgery.

Oromandibular and limb hypogenesis syndrome: treatment report.

The oromandibular and limb hypogenesis syndrome is characterized by aglossia or hypoglossia presenting with limb anomalies. In this case report, we describe congenital hypoglossia associated with glossopalatine ankylosis and middle finger hypomelia, a type III-D malformation in the Hall Classification. The orthodontic and surgical treatment consisted of 3 maxillary expansions, mandibular surgery, and dental alignment. This extensive treatment resulted in acceptable function and esthetic appearance; however, because of lingual hypoglossia, it did not effect perfect occlusion.

Evaluation of oral functions of the stomatognathic system according to the levels of asthma severity.

PURPOSE: To compare the orofacial functions (chewing, swallowing and speech) in children with asthma and healthy children. METHODS: A cross sectional study including 54 children of both genders with ages between 7 and 10 years was conducted. Twenty-seven of these subjects composed the experimental group, and were subdivided into two severity levels of asthma: Group I - mild intermittent and persistent asthma; Group II - persistent moderate to severe asthma. Twenty-seven healthy children were included in the control group (Group III). Speech-language pathology evaluation used the adapted Orofacial Myofunctional Assessment Protocol. Adaptation consisted in the exclusion of the structural part of the test, since this was not the aim of the study. The structural part was excluded because it was not the aim of this study. RESULTS: It was found alterations in oral functions, with significant differences between the three groups. These alterations showed no correlation with asthma severity, since the highest rate of alterations was found in Group I (mild asthma). CONCLUSION: Regardless of the severity level, children with asthma have altered patterns of chewing, swallowing and speech.

Oro-dental findings in Bardet-Biedl syndrome.

The Bardet-Biedl syndrome (BBS) is a human genetic disorder with an array of clinical features affecting many body systems. BBS is a pleiotropic disorder with mostly monogenic causes. It is also considered a primary ciliopathy syndrome. It is characterised by obesity, pigmentary retinopathy, polydactyly, mental deficiency and hypogonadism and recently a sixth feature, renal disease, has also been described. Since none of the diverse symptoms of BBS by itself is diagnostic of the disorder and many of the symptoms only become apparent over time, diagnosis of the BBS is often delayed until about 9 years of age when visual problems first appear.

Intraoral vertical ramus osteotomy improved the stomatognathic function in an elderly patient with mandibular protrusion: a case report.

This article reports the successful surgical-orthodontic treatment of an elderly patient with dentofacial deformity and signs and symptoms of temporomandibular disorder (TMD). The patient was a 63-year-old woman with a concave profile due to mandibular protrusion. To correct skeletal deformities, the mandible was posteriorly repositioned by employing intraoral vertical ramus osteotomy (IVRO) following presurgical orthodontic treatment. After active treatment for 31 months, the facial profile was significantly improved and satisfactory occlusion was achieved. In addition, TMD symptoms of clicking sounds on the left side and difficulty in mouth opening were resolved. Regarding the findings of magnetic resonance imaging, anterior disc displacement in the opening phase was improved in the temporomandibular joint on the left side. Furthermore, stomatognathic functions were also improved without any aggravation of age-related problems. In conclusion, surgical repositioning of the mandible using IVRO leads to both morphological and functional improvements even in elderly patients.