Combined Orthodontic-Surgical Sequential Treatment of Cleidocranial Dysplasia: A Case Report With 7-Year Follow-up and Review of the Literature.

Cleidocranial dysplasia (CCD) is a rare hereditary disorder characterized by skeletal malformations and dental abnormalities. Mutations of the transcription factor RUNX2 are responsible for the pathogenesis of CCD. We present a case of a 10-year-old boy with CCD, presenting with hypoplastic clavicles, delayed closure of the fontanelles, retarded exfoliation of the deciduous teeth, retarded eruption of the permanent teeth, and multiple impacted supernumerary teeth. Based on the clinical and radiographic examination results showing abnormalities of the bones and teeth, a diagnosis was reached easily, but it was difficult to achieve a complete curative effect. We carried out a highly organized schedule of treatment, including extraction of the deciduous and supernumerary teeth, partial resection of alveolar bone, distraction of impacted teeth, and orthodontic surgery. After 7-year follow-up, the patient has achieved acceptable occlusion and midfacial appearance. The main objectives of this study were to present the diagnosis and treatment of CCD and to emphasize the benefits of combined orthodontic-surgical sequential treatment.

Orthognathic Surgery Has a Significant Positive Effect on Perceived Personality Traits and Perceived Emotional Facial Expressions in Subjects With Primary Mandibular Deficiency.

This study tested the hypothesis that a layperson's social perceptions of a dentofacial deformity (DFD) patient with primary mandibular deficiency (PMD) are more positive after bimaxillary orthognathic surgery.A survey was implemented comparing layperson's social perceptions of emotional expressions and personality traits before and >6 months after orthognathic surgery when viewing standardized facial photographs. The study sample comprised 20 patients selected randomly from a larger primary mandibular deficiency database, treated by 1 surgeon after orthognathic surgery. The outcome variable was change in 6 perceived emotional expressions and 6 personality traits studied. Descriptive and bivariate statistics were computed (P < .05).Five hundred respondents (raters) completed the survey. The respondents were 52% male with 44% aging from 25 to 34. After bimaxillary and chin orthognathic surgery, primary mandibular deficiency patients were perceived to be significantly more dominant, trustworthy, friendly, intelligent, attractive, and less threatening (P < .05). They were also perceived as happier and less angry, surprised, sad, afraid, or disgusted than before surgery (P < .05).Laypeople consistently report improved social traits in primary mandibular deficiency patient's perceived emotional expressions and perceived personality traits after bimaxillary and chin orthognathic surgery.

A Morphological Classification Scheme for the Mandibular Hypoplasia in Treacher Collins Syndrome.

BACKGROUND: Mandibular hypoplasia is a hallmark of Treacher Collins syndrome (TCS), and its severity accounts for significant functional morbidity. The purpose of this study is to develop a mandibular classification scheme. METHODS: A classification scheme was designed based on three-dimensional computed tomography (3D-CT) scans to assess 3 characteristic features: degree of condylar hypoplasia, mandibular plane angle (condylion-gonion-menton), and degree of retrognathia (sella-nasion-B point angle). Each category was graded from I to IV and a composite mandible classification was determined by the median value among the 3 component grades. RESULTS: Twenty patients with TCS, aged 1 month to 20 years, with at least one 3D-CT prior to mandibular surgery were studied. Overall, 33 3D-CTs were evaluated and ordered from least to most severe phenotype with 10 (30%) Grade 1 (least severe), 14 (42%) Grade 2, 7 (21%) Grade 3, and 2 (7%) Grade 4 (most severe). Seven patients had at least 2 longitudinal scans encompassing an average 5.7 (range 5-11) years of growth. Despite increasing age, mandibular classification (both components and composite) remained stable in those patients over time (P = 0.2182). CONCLUSION: The authors present a classification scheme for the TCS mandible based on degree of condylar hypoplasia, mandibular plane angle (Co-Go-Me angle), and retrognathia (SNB angle). While there is a natural progression of the mandibular morphology with age, patients followed longitudinally demonstrate consistency in their classification. Further work is needed to determine the classification scheme's validity, generalizability, and overall utility.

Swinging Replantation: A Possible Protocol for the Management of Inverted Impacted Upper Central Incisor Tooth.

INTRODUCTION: Inverted tooth impaction is a rare occurrence. Third molar impaction is the most extensively studied, and only eight cases of inverted third molars have been reported in a literature search of 40 years from 1973 to 2013. In a study, 43.4% of the premaxillary supernumeraries were inverted and 21.1% were transversely oriented, while occasional inverted central incisors have been mentioned in the literature. Severe dilaceration makes the management of this case a big challenge. The case of an 8-year-old boy with an inverted, rotated, and impacted upper left central incisor obstructed by an odontome is presented here. Under local anesthesia, the odontome was excised; the inverted tooth was swung and repositioned. The patient experienced uneventful healing, and with 30 months follow-up, progressive tooth eruption has been observed. Vitality test is positive, while radiologically there was no sign of resorption. Swinging replantation is a protocol that has been used for the management of an inverted, rotated, and impacted central incisor. Open root apex is an important factor in the selection of the method. The minimal periodontal damage prevents inflammatory and replacement resorption. With some clear advantages over options like orthodontic and prosthetic replacement, it could be a method to be considered in the management of severely dilacerated central incisors. The clinical significance is that even though management of a severely malpositioned tooth has always been a big challenge in dental practice, requiring extraction and prosthodontic replacement, swinging replantation provides a possible protocol of tooth retention, i.e., less expensive and associated with fewer hospital visits.

Case of Inverted Supernumerary Tooth in Nasal Cavity.

The eruption of a tooth into the nasal cavity is a rare clinical entity. We report a case of an inverted supernumerary tooth in the nasal cavity. A 2-year-old boy was referred to our institution after examination at a local otorhinolaryngology department for otitis media. Radiological examination revealed a tooth-like structure in the right nasal cavity. The tooth was protruding from the floor of the nasal cavity along with granulation tissue. The diagnosis was an inverted tooth in the right nasal cavity. Forceps extraction was performed under general anesthesia.

A Mouthful of Anomalies.

Isolated bilateral macrodontia of the mandibular second premolars is a rare condition. We believe that the case reported here is the first in which isolated bilateral macrodontia of the mandibular second premolars presents with numerous dental anomalies affecting other teeth. A 14-year-old boy was referred to the Paediatric Dental Department of King's College Hospital with a partially erupted mandibular left second premolar. Clinical and radiographic examination subsequently revealed macrodontia of both mandibular second premolar teeth and multiple other dental anomalies. This report discusses the presentation and multidisciplinary management of this case. Clinical relevance: This case report describes an already rare condition made even more extraordinary owing to its presentation with multiple other dental anomalies.

Endodontic Treatment of Hypertaurodontic Mandibular Molar Using Reciprocating Single-file System: A Case Report.

Taurodontism is a developmental tooth disorder characterized by lack of constriction in the cementoenamel junction and consequent vertical stretch of the pulp chamber, accompanied by apical displacement of the pulpal floor. The endodontic treatment of teeth with this type of morpho-anatomical anomaly is challenging. The purpose of this article is to report the successful endodontic treatment of a hypertaurodontic mandibular molar using a reciprocating single-file system.

Importance of a multidisciplinary approach and monitoring in fetal warfarin syndrome.

Warfarin is a synthetic oral anticoagulant that crosses the placenta and can lead to a number of congenital abnormalities known as fetal warfarin syndrome. Our aim is to report on the follow-up from birth to age 8 years of a patient with fetal warfarin syndrome. He presented significant respiratory dysfunction, as well as dental and speech and language complications. The patient was the second child of a mother who took warfarin during pregnancy due to a metallic heart valve. The patient had respiratory dysfunction at birth. On physical examination, he had a hypoplastic nose, pectus excavatum, and clubbing of the fingers. Nasal fibrobronchoscopy showed upper airway obstruction due to narrowing of the nasal cavities. He underwent surgical correction with Max Pereira graft, zetaplasty, and osteotomies for the piriform aperture. At dental evaluation, he had caries and delayed eruption of the upper incisors. Speech and language assessment revealed high palate, mouth breathing, little nasal patency, and shortened upper lip. Auditory long latency and cognitive-related potential to auditory stimuli demonstrated functional changes in the cortical auditory pathways. We believe that the frequency of certain findings observed in our patient may be higher in fetal warfarin syndrome than is appreciated, since a significant number result in abortions, stillbirths, or children evaluated in the first year of life without a follow-up. Thus, a multidisciplinary approach and long-term monitoring of these patients may be necessary.

Talon cusp variations: 2 case reports.

Talon cusps in anterior teeth are relatively rare developmental anomalies. They are characterized by the presence of an accessory cusp-like structure projecting from the cingulum area or cementoenamel junction. Facial talons occur very rarely; <10 cases have been described in the literature. This article attempts to describe the variants of this anomaly with 2 case reports involving facial and bilateral palatal manifestation.

Dental problems in a patient with the classic type of Ehlers-Danlos syndrome--a case report.

The huge progress in diagnostic and therapeutic procedures in developmental medicine allowed not only to save lives of many children and adolescents, but also enforced the necessity of close cooperation between many specialists. Unfortunately dental treatment is still not an integral part of taking care of disabled children and youth with chronic diseases. The situation worsens, when we come across the so-called rare diseases. Lack of access to dental services, when it comes to disabled patients, results from financial situation, healthcare system, as well as parents' ignorance of necessity of more frequent prophylactic and treatment visits. Whereas the reluctance of dentists towards the care of disabled patients is caused by difficulties with enforcing the recommendations and lack of procedures for taking care of patients with rare diseases, which was repeatedly signalled by us. Such situation is ideally pictured by yet another described case of the 17 year old patient with Ehlers-Danlos syndrome. Despite many dental visits, no vital treatment decisions have been made. In our Department, in the procedures of 1-day surgery, tooth 48, being the cause of pain, has been extracted. Basing on all of the above, it can be deduced that preserving the continuation and consequence in spreading the knowledge of rare diseases among patients, as well as physicians and dentists, is a responsibility of everyone who even once came across this problem.

A case of familial syndactyly associated with eye and dental abnormalities.

Syndactyly occurs in 1 in 2,000 live births and is more common in white children. This article describes a patient with syndactyly and additional abnormalities indicating oculodentodigital dysplasia.

[Choice of local anesthetic volume at the infiltration anesthesia on the upper jaw].

The aim of this study was to investigate the effect of volume and pressure, at which the solution of local anesthetic is injected into the tissue, on the effectiveness of infiltration anesthesia of the periosteum on the upper jaw. We used the technique of tooth pain sensitivity thresholds. According to the rate of development and the duration of the maximum analgesia effect, the optimum volume for different levels of pressure was determined. The influence of the tissue density at the injection site on the effect criteria of analgesia was demonstrated.

Anesthetic considerations in Sheldon-Hall syndrome.

Arthrogryposis is characterized by multiple, nonprogressive joint contractures which may be caused by maternal disorders such as oligohydramnios as well as fetal akinesia resulting from primary disorders of muscle, connective tissue, or neurologic tissue. Its prevalence is about 1 : 3000. Distal arthrogryposis (DA) is a heterogenous group of genetic disorders with a characteristic flexion of the joints of the hands and feet divided into different types with additional features. Sheldon-Hall Syndrome (SHS), also known as distal arthrogryposis type 2A (DA2A), has some nonorthopedic features of specific importance to anesthetic care.

Dental findings in Hamamy syndrome.

This paper highlights features of dental rehabilitation of patients with Hamamy syndrome. A 10-year-old boy patient with Hamamy syndrome reported pain in the maxillary left central incisor, and all mandibular incisors. Intraoral clinical and radiographical examination showed enamel hypoplasia, severe dilacerated maxillary left central incisor and mandibular incisors, malocclusion, delayed eruption of teeth, taurodontism, and loss of lamina dura. Root canal treatment, strip crown and composite restorations were performed.

HATS syndrome: hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings.

Hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings (HATS syndrome) is a rare developmental disorder involving the first and second branchial arches. Physical manifestations may present at birth or during early childhood. Characteristic findings include unilateral abnormalities of the face involving the bones, teeth, gums, and skin. Among the characteristic cutaneous manifestations of HATS syndrome, Becker nevus is the most common. A variety of modalities have been utilized in the treatment of HATS syndrome, but no standardized therapy has been established. We report a case of this rare condition in a 14-year-old adolescent boy.

A 3-year prospective study of implant-supported, single-tooth restorations of all-ceramic and metal-ceramic materials in patients with tooth agenesis.

OBJECTIVES: The purpose of this clinical study was to describe outcome variables of all-ceramic and metal-ceramic implant-supported, single-tooth restorations. MATERIALS AND METHODS: A total of 59 patients (mean age: 27.9 years) with tooth agenesis and treated with 98 implant-supported single-tooth restorations were included in this study. Two patients did not attend baseline examination, but all patients were followed for 3 years. The implants supported 52 zirconia, 21 titanium and 25 gold alloy abutments, which retained 64 all-ceramic and 34 metal-ceramic crowns. At baseline and 3-year follow-up examinations, the biological outcome variables such as survival rate of implants, marginal bone level, modified Plaque Index (mPlI), modified Sulcus Bleeding Index (mBI) and biological complications were registered. The technical outcome variables included abutment and crown survival rate, marginal adaptation of crowns, cement excess and technical complications. The aesthetic outcome was assessed by using the Copenhagen Index Score, and the patient-reported outcomes were recorded using the OHIP-49 questionnaire. The statistical analyses were mainly performed by using mixed model of ANOVA for quantitative data and PROC NLMIXED for ordinal categorical data. RESULTS: The 3-year survival rate was 100% for implants and 97% for abutments and crowns. Significantly more marginal bone loss was registered at gold-alloy compared to zirconia abutments (P = 0.040). The mPlI and mBI were not significantly different at three abutment materials. The frequency of biological complications was higher at restorations with all-ceramic restorations than metal-ceramic crowns. Loss of retention, which was only observed at metal-ceramic crowns, was the most frequent technical complication, and the marginal adaptations of all-ceramic crowns were significantly less optimal than metal-ceramic crowns (P = 0.020). The professional-reported aesthetic outcome demonstrated significantly superior colour match of all-ceramic over metal-ceramic crowns (P = 0.015). However, no significant differences in the other aesthetic parameters at various restoration materials were registered. After 3 years, the patient-reported outcome variables at different restoration materials were not significantly different. CONCLUSION: The biological outcomes at the zirconia and metal abutments were comparable. All-ceramic crowns demonstrated better colour match, but higher frequency of marginal discrepancy compared to metal-ceramic crowns. Generally, the patients noticed no difference in aesthetic outcome of all-ceramic and metal-ceramic restorations.

Malformations of the zygomatic and maxillary regions.

The maxillofacial region develops during 3 to 8 weeks in an embryo. The process involves neural crest cell migration and proliferation as well as facial protrusion jointing and fusion. The maxillofacial region is one of the predilection sites of congenital malformations. We treated a 5-year-old Chinese boy with abnormal development of the left maxillofacial region. We describe in detail the patient's characteristics, diagnosis, and treatment processes and try to explain the possible causes of the disease.

Endodontic-periodontal management of a maxillary lateral incisor with an associated radicular lingual groove and severe periapical osseous destruction--a case report.

Radicular lingual grooves are morphological defects, which are found most frequently in maxillary anterior teeth and are a predisposing factor for periodontal disease. They are easily overlooked as aetiologic factors, as these grooves are covered by periodontal tissues. This case report presents a successful management of a case of a maxillary lateral incisor with an associated radicular lingual groove and severe periapical osseous destruction in a 30-year-old female patient. A combination of endodontic treatment, radiculoplasty to eliminate the radicular lingual groove, and periapical surgery to eliminate the periapical osseous defect was used. At two-year follow-up, the patient was comfortable and complete resolution of the periapical pathology was evident.

Advanced and prospective technologies for potential use in craniofacial tissues regeneration by stem cells and growth factors.

The processes of craniofacial tissues development and regeneration are largely dependent on sequential and reciprocal interactions between mesenchymal and epithelial components. These processes involve a series of inductive and permissive interactions that result in the determination, differentiation, and organization of craniofacial tissues. Stem cells and growth factors represent a very interesting research field for craniofacial tissues regeneration. They represent a potential key component in autologous graft for craniofacial tissues regeneration. An ideal goal of oral-craniofacial dental reconstructive therapy is to establish treatment modalities that predictably restore functional tissues. One major area of focus has been that of dental materials with marked improvements in the design of materials used to restore teeth/periodontium/bone lost as a consequence of disease or disorders. Interest in these technologies continues to increase in dental application as a substitute for traditional treatments and artificial components. Recent progress in the studies of molecular basis of tooth development, adult stem cell biology, and regeneration will provide fundamental knowledge for the realization of human craniofacial tissues regeneration in the near future.