Klippel-Trenaunay-Weber syndrome: Oro-dental manifestations and management.

INTRODUCTION: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital systemic disease characterized by a classic typical triad: cutaneous haemangioma, arterio-venous fistulas or varicosities (or both) and unilateral hypertrophy of hard and soft tissue with different localizations. First described by the French physicians Marcel Klippel and Paul Trenaunay, in 1900, KTWS has a clinically incidence of 2-5/100000. The complete triad (port-wine stains, varicose veins, and soft tissue and/or bony hypertrophy) occurs in almost 2/3 of the patients. Moreover, these features may be present at birth or develops during growing becoming more evident with age. Typical orofacial manifestations include facial asymmetry, jaw enlargement and malocclusion. CASE PRESENTATION: A previously diagnosed 55-year-old male patient was referred to the Department of Cranio-Maxillofacial Surgery presenting with extended mandibular ramus exostosis on the left side and concomitant malocclusion. Surgical removal of the hypertrophic bone was performed due to progressive mouth opening restriction, limited oral hygiene ability and increased mandible deviation. Bleeding complications and wound healing disorders were not observed. Consistent mouth opening training resulted in an improvement in mouth opening. CLINICAL DISCUSSION AND CONCLUSION: It is suggested to consider KTWS as one of the differential diagnoses if vascular syndromes in the head and neck region occur. Special attention must be given to dental treatment due to eventual excessive haemorrhage that might occur after oral surgical procedures.

Distribution of bone thickness in the human mandibular ramus - a CBCT-based study.

BACKGROUND: The bone thickness of the human mandibular ramus is an important parameter in mandibular surgeries. The aim of this study was to systematically measure the bicortical bone thickness, the ramus dimensions and the position of the lingula. The measurements were tested on significant correlations to the patients' parameters. METHODS: Based on CBCT scans 150 rami were reconstructed as 3D polygon surfaces. An anatomical grid was adapted to the ramus surface to mark the bone thickness measurement points and to achieve comparability between the measurements on different mandibles. The bone thickness, ramus height, ramus width and the gonion angle were measured. A cluster analysis was performed with these parameters to identify clinically relevant groups with anatomical similarities. RESULTS: The median distribution of the bone thickness was calculated and visualized in a pseudo-colour map. The mean ramus height was 44.78 mm, the mean width was 31.31 mm and the mean gonion angle was 124.8°. The average distance from the lingula to the dorsal tangent was 53% of the total width and its distance to the caudal tangent was 65% of the total height. Significant correlations between the bone thickness and the ramus proportions could be identified. Age and sex had no significant influence on the mean bone thickness. The measured rami could be divided into two groups by cluster analysis. CONCLUSION: The dimensions of the human mandibular ramus can be determined from 3D reconstructed surface models from CBCT scans. Measurements could be made comparable by applying an anatomically oriented grid. A cluster analysis allowed the differentiation of two groups with different bone thickness distributions and geometries, which can be used for the optimization of osteosynthesis systems and their precision of adaptation to different ramus morphologies.

Dental implants in growing patients: a systematic review.

The aim of this systematic review (for which we followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines) was to provide an overview of the protocols and clinical outcomes of dental implants placed in growing jaws. We searched the MEDLINE/PubMed, Embase, Scopus, and Science Direct databases in October and November, 2017. A total of 3492 studies were identified, and all the studies reporting the outcomes of dental implants placed during the growth phase were included in the study. After duplicates had been removed, 2133 studies were screened based on their titles and abstracts, and 162 were selected for reading. Finally, 28 studies were included in the review. Overall, 493 dental implants were placed in 147 patients aged from 3-18 years old with follow-up being from 1-20 years. The most common disorders seen that were associated with missing teeth were ectodermal dysplasia and dental trauma. The main complications reported were the infraocclusion positioning of dental implants in the maxillary arch and the rotation of dental implants in the mandibular arch. Dental implants were indicated for the anterior regions of the maxilla and mandible in patients over 10 years old, and placement of maxillary implants in a more coronal position was recommended. Consultations and adjustments to prostheses were required until growth had ceased. In growing jaws, dental implants require positional modifications, and they should be considered only under special circumstances.

[Alternative therapy option for plastic-aesthetic treatment of defects after resection in the upper third of the facial region in the sense of subtotal scalping instead of local flap plastics]. / Alternative Therapieoption zur plastisch-ästhetischen Defektversorgung nach Resektion im oberen Gesichtsdrittel im Sinne einer Teilskalpierung anstelle lokaler Lappenplastiken.

In the presented case, the resulting defect size after resection of a dermatofibrosarcoma protuberans exceeded the treatment capability with local flaps in the region of the exposed facial skin. Through the use of conventional wound healing in combination with a meshed split-thickness skin graft, plastic aesthetic soft tissue treatment with an aesthetically satisfactory result was possible.

Sclerosing odontogenic carcinoma: current diagnostic and management considerations concerning a most unusual neoplasm.

Sclerosing odontogenic carcinoma (SOC) is a primary intraosseous carcinoma of the jaws that has been listed as a separate entity for the first time in the latest version of the World Health Organization classification of Head and Neck Tumours (2017). The aim of this study was to analyse and interpret the existing literature on SOC in the context of a clinical case treated in the authors' department. A systematic search of the PubMed database was performed in accordance with the PRISMA guidelines, yielding nine cases of SOC reported so far. In summary, characteristic clinical and radiological features of SOC include asymptomatic swelling, location predominantly in the mandible, tumour primarily lytic in appearance, presence of cortical bone destruction, and lack of metastatic spread. Due to the rarity of the disease, close collaboration between oral/maxillofacial surgeons and pathologists is crucial to avoid misdiagnosis. With complete excision, no recurrence of SOC should be expected.

Development of a database to record orofacial manifestations in patients with rare diseases: a status report from the ROMSE (recording of orofacial manifestations in people with rare diseases) database.

The aim of this working group was to establish a ROMSE (recording of orofacial manifestations in people with rare diseases) database to provide clinicians, patients, and their families with better information about these diseases. In 2011, we began to search the databases Orphanet, OMIM® (Online Mendelian Inheritance in Man®), and PubMed, for rare diseases with orofacial symptoms, and since 2013, the collected information has been incorporated into a web-based, freely accessible database. To date, 471 rare diseases with orofacial signs have been listed on ROMSE, and 10 main categories with 99 subcategories of signs such as different types of dental anomalies, changes in the oral mucosa, dysgnathia, and orofacial clefts, have been defined. The database provides a platform for general clinicians, orthodontists, and oral and maxillofacial surgeons to work on the best treatments.

Orthognathic surgery in Melnick-Needles-Syndrome. Case report and review of the literature.

Melnick-Needles Syndrome (MNS) is a congenital syndrome associated with severe architectural disorder of the skeletal system that can cause significant effects on the craniofacial skeleton including poor aesthetics, impaired speech and masticatory problems. The authors report a case of a female patient who experienced masticatory constraint, hindered speech and functional problems related to a severe bite dysfunction due to the mandibular hypoplasia and the underlying bony architectural disorder associated with MNS. The patient underwent bilateral sagittal split osteotomy (BSSO) to correct her skeletal malocclusion and improve the characteristic aesthetics and speech. The inherent bony abnormalities with their altered collagen structure presented unique challenges to orthognathic surgery in this patient with an unpredictable bone healing process. In MNS patients a fragile trabecular bone structure and an increased bleeding tendency is thought to delay or forestall wound healing. The patient was treated successfully with BSSO and monocortical plate fixation following a well-established algorithm from orthodontic preparation to surgical protocol. A very satisfying outcome has been achieved, concerning functional rehabilitation and aesthetic improvement. A review of the literature revealed that little is known about bone regeneration and fracture healing in cases with MNS.

Prediction model for early percutaneous endoscopic gastrostomy (PEG) in head and neck cancer treatment.

Head and neck cancer patients are prone to nutritional problems, partly due to the location and size of the disease, due to significant comorbidity and also often therapeutic intervention. Excessive weight loss after surgery reduces further the patients' physical resistibility and increases the complication rate for adjuvant radiation and or chemotherapy. Possible effective interventions are dietary counseling, nutritional supplements or drug interventions. Aim of our research was to reveal reliable clinical predictive parameters, which calculate risks as the reduced nutritional state against the possible complications of PEG insertion and finally define an algorithm for pre-therapeutic PEG insertion to optimize the general treatment conditions by sufficient nutrition. We explored the data of 152 patients in a time period from 2005 to 2010 considering age, gender, body mass index, staging, size and localization of the tumor or need for a neck dissection. The decisive predictive parameters are: body mass index, size and localization of tumor, lymph node affection, resection of the root of the tongue or the oropharynx region and performance of a neck dissection. In this retrospective study we established a prediction model that allows a substantiated evaluation of post-therapeutic dysphagia considering relevant clinical features as well as the specific surgical therapy. From this assessment derives the indication of a safe pre-therapeutic application of a percutaneous endoscopic gastrostomy (PEG).

Free vascularized fibular grafting for reconstruction after tumor resection in the upper extremity.

BACKGROUND: Limb salvage is viable in the majority of patients with malignant bone tumors, but especially in case of extensive tumors and bad soft tissue conditions, it is challenging in upper extremity. OBJECTIVES/METHOD: The clinical and radiological results of 21 patients, who had free vascularized fibular grafts (VFG), for diaphyseal (14), and epipyseal (7) defect reconstruction of the upper extremity, are presented. The indications for VFG were resection after osteosarcoma (9 cases), Ewings sarcoma (9 cases), chondrosarcoma (1 case), rhabdomyosarcoma (1 case), and 1 case of fibrous dysplasia. The 20 malignant tumors were staged as follows: 2a (1), 2b (18), 3 (1). The mean follow-up was 43.6 months (min 6.0-max 131.9). Functional results were described and graded quantitatively according to the MSTS-score. RESULTS: Results were satisfactory with regard to pain, emotional acceptance, manual dexterity, and function. Lifting ability was decreased in two patients. Hypertrophy index was 31% (min 13%-max 71%). Main complications were fracture (5), pseudoarthrosis (4), prolonged wound healing (4), temporary nerve irritation (2), and deep infection (1). Re-operation was required in eight patients (12 operations). CONCLUSION: VFG offers a good possibility for biological reconstruction of large skeletal defects, with an acceptable complication and re-operation rate. When conservative treatment of complications was not successful, further surgery led to recovery in the majority of cases.

[Aggressive fibromatosis in the jaw and facial region with bone involvement. A review]. / Aggressive Fibromatose im Kiefer-Gesichts-Bereich mit ossärer Beteiligung. Eine Ubersicht.

BACKGROUND: Aggressive fibromatosis (AF) involving bones of the head is rare and surgery is often complicated by a high recurrence rate. Interdisciplinary treatment is of the utmost importance to avoid extensive, mutilating resection. Two cases emphasize the difficulties in the management. CASE REPORTS: A 67-year-old woman was referred to our unit with a blepharochalasis of the left upper palpebra and a palsy of the face on the left side. Her medical history included 12 operations over the previous 4 years for an extensive AF. MATERIAL AND METHODS: Our review includes all case reports of AF involving bones of the head published between 1960 and 2004. Additionally, our two cases are presented. Signs, symptoms and outcome were analyzed in relation to different treatment options. CONCLUSION: According to the literature, surgery is the most common treatment for AF in the head and neck region. Alternative modes of therapy must be considered because of the high recurrence rate and to avoid mutilating operations.

Score system for elective tracheotomy in major head and neck tumour surgery.

BACKGROUND: This study was designed to evaluate prognostic parameters for respiratory failure after major oropharyngeal resections in head and neck cancer surgery, focusing on a score system to identify patients requiring an elective tracheotomy and to avoid tracheotomy under emergency conditions. METHODS: One hundred and fifty-two out of 928 patients with oropharyngeal cancers, treated between January 1993 and June 2000 at our hospital, fulfilled the inclusion criteria for a retrospective analysis. This collective underwent tumour resection in different regions of the oropharynx combined with bony resection of the mandible and neck dissection without primary tracheotomy. The reconstruction was accomplished using radial forearm flaps (n1 = 59) or local flaps (n2 = 93). These two groups were subdivided into patients treated post-operatively by tracheotomy due to respiratory failure (n1 = 26; n2 = 12) and those without such treatment (n1 = 33; n2 = 81). The database comprising tumour localization and size, staging, general medical condition, smoking and alcohol consumption was evaluated by logistic regression. RESULTS: We developed a score system which predicts the likelihood of post-operative respiratory failure. For indication of tracheotomy, tumour size and localization, multimorbidity, alcohol consumption and pathologic chest X-ray findings were identified as significant parameters with different weightings. The predictive value for tracheotomy (yes/no) using the score system was 96.7% for the total collective. CONCLUSION: The decision on whether or not an elective tracheotomy in major head and neck tumour surgery is necessary can be facilitated using this score system which is based on objective facts. It may reduce post-operative complications and contribute to safer treatment.

[Peri-implant bone formation around cylindrical and conical implant systems]. / Tierexperimentelle Evaluation des periimplantären Knochens bei zylindrischen gegenüber konischen Implantattypen.

BACKGROUND: The treatment of patients with early or immediately loaded dental implants has renewed interest in the behavior of osseointegration at the implant surface. Whereas it is generally accepted that peri-implant tissue formation and mineralization are dependent on the local mechanical environment in the interface zone, controversies exist concerning the impact of implant design on peri-implant bone formation. The aim of the present study was the in vivo evaluation of peri-implant bone formation by two different implant systems: cylindrical (ITI) versus conical (ILI). MATERIAL AND METHOD: A total of 60 implants (30 ITI and 30 ILI) were placed in the cranial and caudal part of the tibia of eight Göttinger minipigs. Half of the minipigs were sacrificed at 7 days and 28 days of osseointegration. Implant-containing bone specimens were prepared for histological and ultrastructural investigations. RESULTS: Histological and scanning electron-microscopic investigations showed a direct contact of bone-like minerals over the whole implant surface from day 7 of implant/bone interaction. Whereas the ILI implant showed direct contact up to the top of the crestal bone, ITI implants demonstrated a crestally located narrow gap without ossification over the whole experimental period. CONCLUSION: Our investigations support the hypothesis of an implant design-inherent emergence and maintenance of crestal bone.

[Odontogenic foci--possible etiology of urticaria?]. / Odontogene Foci-mögliche Ursache einer Urtikaria?

Recent studies have shown that chronic odontogenic infections could pose a risk for myocardial infarction, cerebral ischemia, and arteriosclerosis. However, the correlation between urticaria and dental infections has rarely been examined so far. Therefore, we performed a case-control study using a standardized questionnaire and examination. We investigated 66 patients suffering from an acute or chronic urticaria and 65 age- and sex-matched healthy patients as a control group. Dental status was determined by a so-called total dental index (TDI) which primarily reflects caries, periodontitis, periapical lesions, and nonvital and missing teeth. All 66 patients were referred from the department of dermatology. After their treatment in hospital, all patients received a questionnaire with questions on intensity and localization of the urticaria. The TDI of the urticaria patients was slightly better ( n=66; 2.6+/-1.98) than that of the control group ( n=65, TDI=3.3+/-1.86). Subsequently, it was determined if the urticaria had receded after dental treatment. In conclusion, chronic dental infections do not seem to correlate with an increased risk for urticaria.

[Primary implant stability with different bone surgery techniques. An in vitro study of the mandible of the minipig]. / Primäre Implantatstabilität bei unterschiedlichen Knochenaufbereitungstechniken. Eine In-vitro-Untersuchung am Unterkiefer des Minischweins.

The treatment concept of osseointegration is based on a stable embodiment of implants in bone and the maintenance of stability during functional load. A goal of the surgical preparation technique is therefore to obtain a stable and firm implant anchorage. The aim of this study was to evaluate implant stability after different surgical treatment of the bony implantation bed. Thirty cylindrical solid-screw-shaped implants with standard SLA ITI configuration were implanted into the explanted mandibles of five minipigs. The implant sites were prepared either by a conventional burr technique (group A), by burr technique with additional thread cutting (group B), or by the osteotome technique (group C). Primary implant stability was evaluated by resonance frequency analysis and removal torque test. The average value of the resonance frequency analysis (RFA) was 6000+/-469 cycles/s in group A, 5700+/-557 cycles/s in group B, and 5540+/-527 cycles/s in group C. Removal torque values of group A (507+/-57 Nmm) were significantly higher than those of group B (466+/-45 Nmm) and group C (240+/-31 Nmm) (between group A and C p<0.05, group A to B p=0.39, and B to C p<0.05). It can be concluded from this study that the conventional burr technique achieves a statistically significantly better primary bone anchorage than the osteotome technique.

[Evaluating angiogenesis and osteogenesis modified by vascular endothelial growth factor (VEGF)]. / Beurteilung der Angiogenese und Osteogenese unter dem Einfluss von Vascular endothelial growth factor (VEGF).

PURPOSE: This intention of this study was to investigate the influence of controlled release of vascular endothelial growth factor (VEGF) on angiogenesis and osteogenesis in a mandibular defect model. METHODS: A total of 56 rabbits were operated and bicortical holes were placed in the mandible. The defects were filled with collagen type I implants, collagen implants complexed with 0.8-microgram VEGF165, or left without any filling. After 3, 7, 14, and 28 days specimens were taken and histologic, histomorphometric, and immunohistologic analyses were carried out concerning density of vessels, total surface of vessels, bone surface, and bone density. RESULTS: The number of vessels was increased in all groups up to 14 days, followed by physiologic regression in the control groups, whereas the study group showed persistently high numbers. The density of regenerated bone was significantly higher in the study group. CONCLUSION: The activation of angiogenesis using VEGF165 leads to more intensive angiogenesis and bone regeneration.

Salivary levels of vascular endothelial growth factor (VEGF) in recurrent aphthous ulceration.

BACKGROUND: Vascular endothelial growth factor (VEGF) is a multifunctional angiogenic cytokine involved in angiogenesis and wound healing. Its presence in recurrent aphthous ulceration has not been reported to date. The aim of this study was to investigate the association of salivary levels of VEGF with various stages of recurrent aphthous ulceration (RAU). METHODS: VEGF levels were determined in a group of 27 age and sex-matched healthy controls and in 30 patients with minor and major RAU grouped into the three stages: (I) early active stage, (II) active stage, and (III) remission period. VEGF levels (pg/ml; mean +/- SD) in unstimulated whole saliva were determined by enzyme immunoassay. RESULTS: Patients with major RAU - stages I and II - had decreased VEGF values (765 +/- 458 and 341 +/- 109, respectively) when compared both to healthy controls (1652 +/- 567; P < 0.01) and to stage III major RAU (1524 +/- 784; P < 0.005). CONCLUSION: Salivary VEGF levels seemed to be associated with ulcer development in major RAU, showing stage-dependent alterations during the course of this disorder.

Imaging of cartilage and mimic muscles with MRI: anatomic study in healthy volunteers and patients with unilateral cleft lip and palate.

OBJECTIVE: The aim of the study was to visualize different soft tissues in the perioral, nasal, and paranasal region by means of magnetic resonance imaging (MRI) in patients with unilateral cleft lip and palate (UCLP). DESIGN: In this descriptive study, images of different MRI systems were assessed and compared. METHOD: MRI was applied in five consecutive patients operated on for UCLP who underwent secondary lip and nasal correction, two patients who had not had UCLP operations, and five healthy volunteers as controls. The mimic muscles, vessels, and nasal cartilages were evaluated. RESULTS: It was possible to visualize different parts of the paranasal and perioral mimic muscles and their interlacement in the upper lip. The nasal cartilages were also visible, and the changes after operation were demonstrated. CONCLUSIONS: MRI shows differentiated visualization of soft tissues in the cleft region and their changes after surgery. It is a valuable tool in the preoperative planning and postoperative follow-up in patients with UCLP.

Natural immunity in recurrent aphthous ulceration.

In the present study we investigated immunophenotypes of peripheral blood lymphocytes, natural killer (NK) cell activity as well as spontaneous migration, ingestion, digestion and antibody-dependent cell-mediated cytotoxicity (ADCC) as effector functions of polymorphonuclear neutrophil granulocytes (PMNs) in 51 patients with acute stage recurrent aphthous ulceration (RAU), during remission, and 47 age-matched healthy individuals. Statistically significant lower B-lymphocyte (CD19) values were found between patients with acute RAU, and those during the remission period (P<0.001), when compared with those of the controls. Total T-lymphocyte (CD3) percentages were lower in patients with RAU, and also during the remission period, when compared with the controls (P<0.001). The percentages of CD4 lymphocytes were significantly lower in patients with RAU in comparison with those of the controls (P<0.001). T-suppressor cells (CD8) were unchanged in all three groups of participants. Significantly lower spontaneous migration and ingestion values were found in patients with acute RAU, when compared with those of the controls, and during the remission period (P<0.001). Digestion values differed insignificantly between the patients with acute RAU and during the remission period. During the remission period, digestion values were significantly elevated when compared with those of the controls (P<0.05). ADCC values were lower during the remission period (P<0.001), when compared with the values during acute RAU and with those of the controls. Significantly depressed NK activity (P<0.001) was observed in patients with acute RAU, when compared with that of the controls. During the remission period, values of NK activity were also lower (P<0.001) when compared with those of the controls. These results suggest either a specific or nonspecific immunological disorder in patients with RAU.

[Prospective cephalometric evaluation of a new 3-dimensional adjustable osteosynthesis system for sagittal ramus osteotomy]. / Prospektive kephalometrische Evaluation eines neuen dreidimensional justierbaren Osteosynthesesystems für die sagittale Ramusosteotomie.

The aim of the study was the prospective cephalometric analysis of 58 patients who underwent sagittal split ramus osteotomy (SSRO). Stabilisation of the fragments was achieved with an adjustable monocortical bone fixation system. Lateral cephalograms were taken preoperatively, postoperatively, 6 months postoperatively, and 12 months postoperatively. The radiographs were digitised, and a computerised analysis was performed. The mean mandibular advancement was 8.5 mm with a mean relapse of 0.8 mm after 12 months (P < 0.05). The mean mandibular setback was 7.8 mm with a mean relapse of 1.2 mm after 12 months (P < 0.05). The results of the present study show a minimal relapse after mandibular advancement and setback for the SSRO and the new adjustable fixation system. Postoperative analysis reveals a safe intraoperative positioning of the condyles with subsequent prevention of an immediate relapse. The results after 12 months indicate sufficient long-term stability, although additional evaluations with results after 5 and 10 years are necessary for a final report.