Background: Arthrogryposis multiplex congenita is a rare condition that mainly involves the lower limbs, characterized by severe joint deformity and contracture, muscular atrophy, and functional impairment. Its clinical manifestations are heterogenous and may involve the maxillofacial district as well.Case presentation This case report describes a 20-year-old patient with arthrogryposis multiplex congenita with skeletal crossbite, facial asymmetry, reduced mouth opening and absence of lateral mandibular movement on the left side. After clinical evaluation, the following exams were required: postero-anterior cephalometric tracing, head and neck electromyography, computerized axiography, computed tomography scan, and maxillofacial magnetic resonance imaging. Orthognathodontic evaluation indicated skeletal asymmetry, reduced condylar movements on the left side and abnormally low electromyography activity of the masticatory muscles on the left side. Computed tomography and magnetic resonance imaging revealed unilateral left mandibular hypoplasia, hypotrophy, and fatty infiltration of masticatory muscles on the left side, as well as immobility of the left condyle during mouth opening, and hypoplasia of the left articular disk, which was however not displaced. Surgery was not indicated and conservative orthognathodontic treatment with function generating bite was suggested to balance the occlusal plane, as well as stretching exercises. Conclusions A rare case of arthrogryposis multiplex congenita with maxillofacial involvement illustrates that a patient-centred, multidisciplinary approach with accurate diagnosis is required to formulate the best treatment plan.Because of the considerable damage to the masticatory muscles, conservative orthognathodontic therapy may be the best treatment option.

The “surgery-first” approach, defined as a team approach between surgeons and orthodontists for orthognathic surgery without preoperative orthodontic treatment, is aimed at dental decompensation. A brief historical background and indications for the surgery-first approach are reviewed. Considering the complicated mechanism of postoperative orthodontic treatment, the proper selection of patients is a vital component of successful surgery-first approach.
Humans ; Orthodontics ; Orthodontists ; Orthognathic Surgery ; Surgeons

BACKGROUND: The aim of this study is to validate a new three-dimensional craniofacial stereophotogrammetry imaging system (3dMDface) through comparison with manual facial surface anthropometry. The null hypothesis was that there is no difference between craniofacial measurements using anthropometry vs. the 3dMDface system. METHODS: Facial images using the new 3dMDface system were taken from six randomly selected subjects, sitting in natural head position, on six separate occasions each 1 week apart, repeated twice at each sitting. Exclusion criteria were excess facial hair, facial piercings and undergoing current dentofacial treatment. 3dMDvultus software allowed facial landmarks to be marked and measurements recorded. The same measurements were taken using manual anthropometry, using soluble eyeliner to pinpoint landmarks, and sliding and spreading callipers and measuring tape to measure distances. The setting for the investigation was a dental teaching hospital and regional (secondary and tertiary care) cleft centre. The main outcome measure was comparison of the craniofacial measurements using the two aforementioned techniques. RESULTS: The results showed good agreement between craniofacial measurements using the 3dMDface system compared with manual anthropometry. For all measurements, except chin height and labial fissure width, there was a greater variability with the manual method compared to 3D assessment. Overall, there was a significantly greater variability in manual compared with 3D assessments (p < 0.02). CONCLUSIONS: The 3dMDface system is validated for craniofacial measurements.
Anthropometry* ; Chin ; Hair ; Head ; Hospitals, Teaching ; Imaging, Three-Dimensional* ; Methods ; Outcome Assessment (Health Care) ; Photogrammetry

After tooth extraction, dimensional changes affect the alveolar socket, leading to loss in alveolar bone height and width. Histological modifications also occur, with initial formation of a blood clot that is replaced with granulation tissue and subsequently with a provisional connective tissue matrix. Spontaneous healing ends with socket filling with woven bone, which is gradually replaced with lamellar bone and bone marrow. Adequate alveolar ridge dimensions and bone quality are required to assure optimal stability and osseointegration following dental implant placement. When a tooth is extracted, alveolar ridge preservation (ARP) procedures are an effective method to prevent collapse of the post-extraction socket. Heterologous bone is widely chosen by clinicians for ARP, and anorganic bone xenografts (ABXs) made bioinert by heat treatment represents the most used biomaterial in clinical applications. Collagen-preserving bone xenografts (CBXs) made of porcine or equine bone are fabricated by less invasive chemical or enzymatic treatments to remove xenogenic antigens, and these are also effective in preserving post-extraction sites. Clinical differences between anorganic bone substitutes and collagen-preserving materials are not well documented in the literature but understanding these differences could clarify how processing protocols influence biomaterial behavior in situ. This systematic review of the literature compares the dimensional changes and histological features of ABXs versus CBXs in ridge preservation procedures to promote awareness of different bone xenograft efficacies in stimulating the healing of post-extraction sockets.

BACKGROUND: In the absence of clear guidelines for facial aesthetic surgery, most surgeons rely on expert intuitive judgement when planning aesthetic and reconstructive surgery. One of the most famous theories regarding “ideal” facial proportions is that of the golden proportion. However, there are conflicting opinions as to whether it can be used to assess facial attractiveness. The aim of this investigation was to assess facial ratios of professional black models and to compare the ratios with the golden proportion. METHODS: Forty photographs of male and female professional black models were collected. Observers were asked to assign a score from 1 to 10 (1 = not very attractive, 10 = very attractive). A total of 287 responses were analysed for grading behaviour according to various demographic factors by two groups of observers. The best graded photographs were compared with the least well-graded photographs to identify any differences in their facial ratios. The models’ facial ratios were calculated and compared with the golden proportion. RESULTS: Differences in grading behaviour were observed amongst the two assessment groups. Only one out of the 12 facial ratios was not significantly different from the golden proportion. CONCLUSIONS: Only one facial ratio was observed to be similar to the golden proportion in professional model facial photographs. No correlation was found between facial ratios in professional black models with the golden proportion. It is proposed that an individualistic treatment for each ratio is a rather better method to guide future practice.
Beauty ; Demography ; Female ; Humans ; Male ; Methods ; Surgeons

Background: What we think we see consists of models constructed in our brains, which may be constrained, limited and perhaps modified at a cerebral level. Patients may view their mirror image differently to how others and the clini‑ cal team view them. Understanding potential variations in perception between real and mirror images is important in clinical practice. The aims were to assess differences in self-perception between frontal facial and mirrored photo‑ graphs, comparing the results with selfie photographs. Methods: Facial photographs were taken by one investigator under standardized conditions for preclinical and clini‑ cal students. Each student took a selfie photograph at rest and smiling using his/her smartphone. A mirror image was generated for each image. Each student was shown his/her original and mirror image, without being informed which was which. For each pair of images, students were asked to choose which photograph they perceived as more attractive. A set of photographs of a male volunteer was shown to all participants, to choose either the original or mir‑ ror image as the more attractive. Results: Most observers preferred the true image of the volunteer (P < 0.05), which may be evidence that most people prefer the true image of others, which is how they normally view them. Most observers preferred their own original photograph in frontal view at rest and smiling (P< 0.05), but preferred the mirror image of their selfie photo‑ graph at rest and smiling (P < 0.05). Conclusions Significant differences in perceptions of attractiveness between true and mirror-reversed frontal and selfie images were found. Observers preferred their image the way they view themselves in a mirror. The selfie is how other people view an individual. If a selfie is flipped horizontally, that is how an individual sees themselves in a mirror. Most observers preferred the mirror image of their selfie, which is how they would view themselves in a mirror.

BACKGROUND: This study is a quantitative evaluation of the influence of the mentolabial angle on perceived attractiveness and threshold values of desire for surgery. METHODS: The mentolabial angle of an idealized silhouette male Caucasian profile image was altered incrementally between 84° and 162°. Images were rated on a Likert scale by pretreatment orthognathic patients (n = 75), lay people (n = 75) and clinicians (n = 35). RESULTS: A mentolabial angle of approximately 107° to 118° was deemed the most attractive, with a range of up to 140° deemed acceptable. Angles above or below this range were perceived as unattractive, and anything outside the range of below 98° or above 162° was deemed very unattractive. A deep mentolabial angle (84°) or an almost flat angle (162°) was deemed the least attractive. In terms of threshold values of desire for surgery, for all groups, a threshold value of ≥162° and ≤84° indicated a preference for surgery, although clinicians were least likely to suggest surgery. The clinician group was the most consistent, and for many of the images, there was some variation in agreement between clinicians and lay people as to whether surgery is required. There was even more variability in the assessments for the patient group. CONCLUSIONS: It is recommended that in orthognathic and genioplasty planning, the range of normal variability of the mentolabial angle, in terms of observer acceptance, is taken into account as well as threshold values of desire for surgery. The importance of using patients as observers in attractiveness research is stressed.
Esthetics* ; Evaluation Studies as Topic ; Genioplasty ; Humans ; Male

BACKGROUND: Imatinib mesylate is an inhibitor of the tyrosine kinase Bcr–Abl and a first-line treatment for Philadelphia chromosome-positive chronic myeloid leukaemia (CML). Dermatological side effects include superficial oedema, pustular eruption, lichenoid reactions, erythroderma, and skin rash. Depigmentation of the skin and/or mucosa is uncommon, and hyperpigmentation is rare. CASE PRESENTATION: We present the case of a 63-year-old Caucasian male with widespread hyperpigmentation of the hard palate associated with a 9-year history of imatinib therapy to treat CML. He did not complain of any symptoms. Clinical examination did not reveal any abnormal pigmentation of the skin or other region of the oral mucosa. He did not smoke cigarettes or drink alcohol. His medication regimen was a proton pump inhibitor, a beta-blocker, cardioaspirin, atorvastatin, and imatinib 400 mg/day. Histopathologically, melanin and haemosiderin deposits were evident in the lamina propria. The lesion persisted, with no clinical change, through several follow-ups. We reviewed the literature to explore the possible relationship between oral hyperpigmentation and long-term imatinib mesylate treatment. CONCLUSIONS: We diagnosed oral pigmentation associated with imatinib intake based on the medical history and clinical features of the pigmented macules. Oral pigmentation may have a variety of causes, and differential diagnosis requires nodal analysis. Clinicians should be aware of possible oral mucosal hyperpigmentation in patients taking imatinib mesylate. Such pigmentation is benign and no treatment is needed, but surveillance is advisable.
Atorvastatin Calcium ; Dermatitis, Exfoliative ; Diagnosis, Differential ; Exanthema ; Follow-Up Studies ; Humans ; Hyperpigmentation* ; Imatinib Mesylate* ; Lichenoid Eruptions ; Male ; Melanins ; Middle Aged ; Mouth Mucosa ; Mucous Membrane* ; Palate, Hard* ; Pigmentation ; Protein-Tyrosine Kinases ; Proton Pumps ; Skin ; Smoke ; Tobacco Products

BACKGROUND: We reviewed the biological and mechanical properties of porous hydroxyapatite (HA) compared to other synthetic materials. Computer-aided design/computer-aided manufacturing (CAD/CAM) was also evaluated to estimate its efficacy with clinical and radiological assessments. METHOD: A systematic search of the electronic literature database of the National Library of Medicine (PubMed-MEDLINE) was performed for articles published in English between January 1985 and September 2013. The inclusion criteria were (1) histological evaluation of the biocompatibility and osteoconductivity of porous HA in vivo and in vitro, (2) evaluation of the mechanical properties of HA in relation to its porosity, (3) comparison of the biological and mechanical properties between several biomaterials, and (4) clinical and radiological evaluation of the precision of CAD/CAM techniques. RESULTS: HA had excellent osteoconductivity and biocompatibility in vitro and in vivo compared to other biomaterials. HA grafts are suitable for milling and finishing, depending on the design. In computed tomography, porous HA is a more resorbable and more osteoconductive material than dense HA; however, its strength decreases exponentially with an increase in porosity. CONCLUSIONS: Mechanical tests showed that HA scaffolds with pore diameters ranging from 400 to 1200 mum had compressive moduli and strength within the range of the human craniofacial trabecular bone. In conclusion, using CAD/CAM techniques for preparing HA scaffolds may increase graft stability and reduce surgical operating time.
Atrophy* ; Biocompatible Materials ; Durapatite* ; Humans ; In Vitro Techniques ; Methods ; National Library of Medicine (U.S.) ; Porosity ; Transplants

Background: Gorlin syndrome, also known as Gorlin-Goltz syndrome (GGS) or basal cell nevus syndrome (BCNS) or nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant familial cancer syndrome. It is characterized by the presence of numerous basal cell carcinomas (BCCs), along with skeletal, ophthalmic, and neurological abnormalities. It is essential to anticipate the diagnosis by identifying the pathology through the available diagnostic tests, clinical signs, and radiological manifestations, setting up an adequate treatment plan.Main bodyIn the first part, we searched recent databases including MEDLINE (PubMed), Embase, and the Cochrane Library by analyzing the etiopathogenesis of the disease, identifying the genetic alterations underlying them. Subsequently, we defined what are, to date, the major and minor clinical diagnostic criteria, the possible genetic tests to be performed, and the pathologies with which to perform differential diagnosis. The radiological investigations were reviewed based on the most recent literature, and in the second part, we performed a review regarding the existing jawbone protocols, treating simple enucleation, enucleation with bone curettage in association or not with topical use of cytotoxic chemicals, and “en bloc” resection followed by possible bone reconstruction, marsupialization, decompression, and cryotherapy. Conclusion To promote the most efficient and accurate management of GGS, this article summarizes the clinical features of the disease, pathogenesis, diagnostic criteria, differential diagnosis, and surgical protocols. To arrive at an early diagnosis of the syndrome, it would be advisable to perform radiographic and clinical examinations from the young age of the patient. The management of the patient with GGS requires a multidisciplinary approach ensuring an adequate quality of life and effective treatment of symptoms.