Variation of resection margins in oral cancer in dependence of tumor stage and subsite - a retrospective cohort study.

OBJECTIVES: Surgical resection is a key component of the treatment of head and neck cancer and the achievement of free surgical margins are essential for the patients' outcome in terms of survival. While there is a general recommendation for a free resection range of 5 mm, up to date, there is a lack of investigations on the quality of tumor resection in dependence of affected subsite and tumor stage. In the presented study, predictors for the achieved resection margins in surgically treated oral squamous cell carcinomas were analyzed. MATERIALS AND METHODS: A cohort of 567 patients was included in a retrospective analysis and resection status with exact margin ranges were analysed. Tumor stage, affected subsite and the results of the intraoperative frozen section analysis were assessed. Primary endpoint was the achieved resection margin in mm, secondary endpoints were overall and progression-free survival. RESULTS: The observed mean values of minimal resection margins differed significantly between the investigated subsites (p = 0.042),pathological tumor stages (p < 0.001) and in tumors which demonstrated perineural infiltration (Pn1, p = 0.002). Furthermore, there was a significant impact of the results of the intraoperative frozen section analysis on progression-free and overall survival (p < 0.001). CONCLUSIONS: Our data clearly indicate that resection status differs between tumors of different subsites and tumor stages. CLINICAL RELEVANCE: Clinical procedures should be adapted in order to achieve similar certainty in all resections, and, thus to improve patients' outcome.

Second primary squamous cell carcinoma of the oral cavity - a retrospective cohort study of therapeutic procedures and oncological outcome.

OBJECTIVES: In the presented study, the occurrence rates of second primary oral carcinomas and their prognostic relevance were analyzed. MATERIALS AND METHODS: All patients with surgically treated oral squamous cell carcinomas within the years 2010 and 2022 in our department were included in this retrospective cohort study. Two groups were designed including patients with second primary carcinomas and patients with local tumor recurrences. Occurrence rates, tumor stages and applied therapies were assessed. Primary outcome was overall survival in dependence of the index tumor. Secondary outcomes were overall survival in dependence of local recurrences or second primary tumors. RESULTS: An overall number of 908 patients was included in the analysis. 98 patients (10.8%) developed a second primary oral squamous cell carcinoma. Patients with second primary tumors presented significantly (p < 0.001) better overall survival in dependence of the index tumor compared to patients suffering from local recurrences. There was no significant difference in overall survival (p = 0.4) in dependence of the date of second primary tumor or local recurrence. Patients with second primary tumors were more likely to receive surgery-based therapy compared to patients with local recurrences who more frequently received definitive radiotherapy. CONCLUSION: Our data indicates different clinical courses in terms of therapy and survival of patients suffering from second primary tumors compared to patients with local tumor recurrences. This may be due to a more aggressive biology of local recurrences and earlier detection of second primaries due to oncological follow-up of the index tumor. CLINICAL RELEVANCE: The differentiation of local tumor recurrences and second primary tumors is of clinical relevance, as applicable therapies and resulting prognosis may differ significantly.

Diagnostic accuracy comparing OPT and CBCT in the detection of non-vital bone changes before tooth extractions in patients with antiresorptive intake.

OBJECTIVES: To compare the diagnostic accuracy in detecting early non-vital bone changes between orthopantomography (OPT) and cone-beam computed tomography (CBCT) in correlation with histopathological findings before tooth extractions in patients with antiresorptive (AR) intake. SUBJECTS: Patients with an indication tooth extraction who had received OPT and CBCT preoperatively while or after undergoing AR treatment were prospectively enrolled over a 24-month period in the progesterone in spontaneous miscarriage (PRISM) trial. Imaging studies were randomly analyzed by three examiners for early non-vital bone changes using specific predefined characteristics and a 5-level scale (1 definite absence of criteria to 5 definite presence of criteria). Sensitivity and specificity values were calculated in correlation with the histopathologically evaluated bone samples at the time point of tooth extraction. RESULTS: One hundred thirty patients with 237 treated extraction sites met the inclusion criteria. For all images evaluated by all examiners, CBCT (430/492; 87.4%; receiver operating characteristic [ROC]: area under the curve [AUC] = 0.88; p < 0.001) was more likely to detect histopathologically confirmed non-vital bone than the OPT (132/492; 26.8%; ROC: AUC = 0.562; p = 0.115). CONCLUSIONS: In the detection of non-vital bone changes, CBCT is superior to OPT in both sensitivity and specificity. Specific imaging characteristics allow for the prediction of early non-vital bone changes already at the time before tooth extractions.

Infants With Trigonocephaly Treated With Fronto-Orbital Remodelling and Endocortical Positioning of Resorbable Plates-A Retrospective Cohort Study Including 101 Patients.

PURPOSE: Fronto-orbital remodeling is the gold standard for surgical treatment of metopic synostosis in many craniofacial centers. For fixation, resorbable materials are widely used. Because of swelling during resorption, the positioning of the plates can be relevant. Therefore, the goal of this study was to investigate whether there are limitations to endocortical positioning of resorbable plates. METHODS: In this retrospective study, all patients with metopic craniosynostosis resulting in trigonocephaly, who were treated with a standardized fronto-orbital remodeling and endocortical positioning of resorbable plates, between February 2012 and December 2019 were included. The endpoint of this study was the postoperative complications that can be attributed to the material used. RESULTS: A total of 101 patients, who were treated at our unit, could be included with a male predominance, 81 (80.2%) boys versus 20 (19.8%) girls. Surgery was performed at a median age of 9 months (±2.56). No complications were seen related to resorbable plates. All patients of our study population were categorized as Whitaker classification I and II. CONCLUSION: This study shows that the risk level in using endocortical resorbable plates positioned on the inner surface of the bone is low. From an aesthetic point of view, we could not detect any disadvantage.

Dynamic Up-Regulation of PD-L1 in the Progression of Oral Squamous Cell Carcinoma.

The introduction of immune checkpoint inhibition for recurrent and metastatic head and neck cancer has brought a new treatment option for patients suffering from advanced oral cancers without a chance for curation using surgery or radiotherapy. The application of immune checkpoint inhibitors in most cases is based on the expression levels of PD-L1 in the tumor tissue. To date, there is a lack of data on the dynamic regulation of PD-L1 during disease progression. Therefore, this study aimed to evaluate the expression levels of PD-L1 in a large cohort of patients (n = 222) with oral squamous cell carcinoma including primary and recurrent tumors. Semiautomatic digital pathology scoring was used for the assessment of PD-L1 expression levels in primary and recurrent oral squamous cell carcinoma. Survival analysis was performed to evaluate the prognostic significance of the protein expression at different stages of the disease. We found a significant up-regulation of PD-L1 expression from primary disease to recurrent tumors (mean PD-L1 H-scores: primary tumors: 47.1 ± 31.4; recurrent tumors: 103.5 ± 62.8, p < 0.001). In several cases, a shift from low PD-L1 expression in primary tumors to high PD-L1 expression in recurrent tumors was identified. Multivariate Cox regression analysis did not reveal a significantly higher risk of death (p = 0.078) or recurrence (p = 0.926) in patients with higher PD-L1 expression. Our findings indicate that the exclusive analysis of primary tumor tissue prior to the application of checkpoint blockade may lead to the misjudgment of PD-L1 expression in recurrent tumors.

HLA-E and Its Soluble Form as Indicators of a Sex-Specific Immune Response in Patients with Oral Squamous Cell Carcinoma.

The human leukocyte antigene E (HLA-E) is associated with tumorigenesis in various cancers. Immunoncology along with sex-specific aspects in cancer therapy are now in scientific focus. Therefore, immunohistochemical HLA-E expression was retrospectively analysed in a cohort of oral squamous cell carcinomas (OSCC) after surgical therapy. Then, serum concentration of HLA-E (sHLA-E) was quantified in a prospective cohort by enzyme-linked immunosorbent assay. High HLA-E expression was associated with advanced UICC stage (Spearman's correlation: p = 0.002) and worse survival (Cox-regression: progression-free survival: hazard ratio (HR) 3.129, confidence range (CI) 1.443-6.787, p = 0.004; overall survival: HR 2.328, CI 1.071-5.060, p = 0.033). The sHLA-E concentration was significantly higher in the control group than in tumor group (Mann-Whitney U-test (MW-U): p = 0.021). Within the tumor group, women showed significantly higher sHLA-E levels than men (MW-U: p = 0.049). A closer look at the tumor group and the control group showed that gender-specific differences exist: while no differences in sHLA-E concentration were detectable between female subjects of tumor group and control group (MW-U: p = 0.916), male subjects of tumor group had a significantly lower sHLA-E concentration compared to those of control group (MW-U: p = 0.001). In summary, our results provide evidence for sex-specific differences in immune responses in OSCC. This fact should be considered regarding future immunotherapy regimens.

Clinical outcome and prognostic factors in recurrent oral squamous cell carcinoma after primary surgical treatment: a retrospective study.

OBJECTIVES: Survival for patients with recurrent oral squamous cell carcinoma is usually poor, and the most effective treatment has not yet been clearly defined. The present study evaluates the outcome in radiotherapy-naïve patients after recurrence of oral squamous cell carcinoma with respect to different treatment modalities including surgery, radiation, chemoradiation, and palliative treatment. PATIENTS AND METHODS: In this retrospective study, we included all patients with primary oral squamous cell carcinoma who received exclusively surgical therapy between 2010 and 2020 and who suffered from locoregional recurrence in their follow-up. Patients with previous adjuvant therapy were excluded from this protocol. Clinical and pathological parameters were collected and statistically evaluated. Survival analysis was performed according to Kaplan-Meier. The primary endpoints were overall and progression-free survival in dependance of treatment strategy for recurrent tumors. RESULTS: Out of a total of 538 patients with surgically treated primary oral squamous cell carcinoma, 76 patients met the inclusion criteria. The mean follow-up was 38 ± 32 months. Patients who received surgically based therapy had a significantly better outcome in terms of disease-free survival (DFS) and overall survival (OS) (DFS p < 0.001; OS p < 0.001). The presence of regional metastases and a short disease-free interval (DFI) between primary and recurrent cancer were significant predictors for adverse outcomes (DFI p < 0.001). CONCLUSION: We recommend primary surgical therapy for radiotherapy-naïve patients with recurrent oral squamous cell carcinoma, supplemented by risk-adapted adjuvant therapy. CLINICAL RELEVANCE: Surgical therapy continues to play a central role in the treatment of radiotherapy-naïve patients with recurrent oral squamous cell carcinoma.

Wound closure and alveoplasty after preventive tooth extractions in patients with antiresorptive intake-A randomized pilot trial.

OBJECTIVES: To compare success rates between the sub-periosteal prepared (SPP) muco-periosteal flap and the epi-periosteal prepared (EPP) mucosa flap and the feasibility of alveoplasty after surgical tooth extractions in patients undergoing/after antiresorptive treatment. SUBJECTS: Patients with an indication for preventive tooth extraction undergoing/after antiresorptive treatment were enrolled over a 24-month period in a parallel-group randomized clinical pilot trial and randomly assigned for primary wound closure to either the SPP or the EPP group. The primary outcome was treatment failure 8 weeks after surgery. To assess the feasibility of alveoplasty, necrotic bone changes at the time point of tooth extraction were evaluated. RESULTS: One hundred and sixty patients were randomized to the SSP (n = 82) or the EPP (n = 78) group. One hundred and fifty-seven patients met the primary endpoint 8 weeks after surgery with five treatment failures for the SPP group (6.3%) and 18 (23.4%) for the EPP group (p = .004). A significant relationship (p < .0001) was observed between symptomatic teeth and non-vital bone found in 54.8% of all biopsies. CONCLUSIONS: The strong superiority of the muco-periosteal flap as primary wound closure revealed the feasibility and effectiveness of the study. The large number of necrotic biopsies emphasizes the importance of alveoplasty as a preventive measure.

A six-gene expression signature related to angiolymphatic invasion is associated with poor survival in laryngeal squamous cell carcinoma.

PURPOSE: Angiolymphatic invasion serves as a histopathological risk factor for unfavorable survival in head and neck squamous cell carinoma. The aim of the study was to explore  the molecular mechanisms characterizing angiolymphatic invasion and therefore identify a gene expression signature related to angiolymphatic invasion. METHODS: Gene expression analysis of head and neck squamous cell carcinoma was carried out based on clinical and whole genome expression data provided by The Cancer Genome Atlas. Results were validated in an independent cohort of laryngeal squamous cell carcinoma and confirmed by immunohistochemistry staining. RESULTS: A gene expression signature consisting of six genes (SHH, SLC18A3, LCE3E, LCE2B, LCE3D and DSG-1) related to angiolymphatic invasion was identified. The gene expression profile identified a subset of patients with decreased overall survival (p = 0.02, log rank test), which was most prominent for patients with laryngeal squamous cell carcinoma (p = 0.004, log rank test). Furthermore, these patients showed a significant shorter progression-free survival (p = 0.002, log rank test). By use of this gene expression signature, patients at high risk of recurrence could be identified even if morphological changes were not yet recognizable. CONCLUSION: Angiolymphatic invasion is characterized by a distinct histopathological phenotype and specific gene expression signature. The newly identified signature might serve as a reliable predictor of outcome in laryngeal cancer and add additional benefit to histopathological evaluation.

Development of osteomyelitis following dental abscesses-influence of therapy and comorbidities.

OBJECTIVES: Bacterial osteomyelitis of the jaw is a severe disease potentially requiring extensive surgical treatment. We have evaluated the incidence rates of bacterial osteomyelitis following dental abscessation associated with primary or secondary tooth extraction. MATERIALS AND METHODS: A retrospective cohort study was designed and included patients with dental abscesses and surgical treatment including the extraction of focus teeth. Patients were either treated with primary removal during acute infection or secondary delayed extraction within an infection-free interval. The primary outcome variable was the occurrence of bacterial osteomyelitis following the abscess. Secondary outcomes were the influence of general disease, antibiotic therapy, and surgical technique. RESULTS: One hundred nine patients were enrolled in the study; 4 patients (3.7%) developed osteomyelitis. Primary tooth extraction was performed on all these patients (p = 0.017). Significant associations of diabetes (p = 0.001), the use of clindamycin (p = 0.025), and transcutaneous incision (p = 0.017) with the incidence of osteomyelitis were detected. CONCLUSIONS: More severe infections may be associated with a higher risk for the development of osteomyelitis following dental abscesses. A history of diabetes and clindamycin therapy might form further predisposing risk factors. Because of the low incidence and the small case number, our data need to be interpreted carefully. CLINICAL RELEVANCE: Osteomyelitis of the jaw is a rare but severe disease that may require extensive therapy and that impairs the quality of life of affected patients. The evaluation of risk factors to enable further reduction of incidence is therefore urgently required.

In vivo comparison of MRI- and CBCT-based 3D cephalometric analysis: beginning of a non-ionizing diagnostic era in craniomaxillofacial imaging?

OBJECTIVES: To evaluate whether magnetic resonance imaging (MRI) can serve as an alternative diagnostic tool to the "gold standard" cone-beam computed tomography (CBCT) in 3D cephalometric analysis. METHODS: In this prospective feasibility study, 12 patients (8 males, 4 females; mean age ± SD, 26.1 years ± 6.6) underwent 3D MRI and CBCT before orthognathic surgery. 3D cephalometric analysis was performed twice by two independent observers on both modalities. For each dataset, 27 cephalometric landmarks were defined from which 35 measurements (17 angles, 18 distances) were calculated. Statistical analyses included the calculation of Euclidean distances, intraclass correlation coefficients (ICCs), Bland-Altman analysis, and equivalence testing (linear mixed effects model) with a predefined equivalence margin of ± 1°/1 mm. RESULTS: Analysis of reliability for CBCT vs. MRI (intra-rater I/intra-rater II/inter-rater) revealed Euclidean distances of 0.86/0.86/0.98 mm vs. 0.93/0.99/1.10 mm for landmarks, ICCs of 0.990/0.980/0.986 vs. 0.982/0.978/0.980 for angles, and ICCs of 0.992/0.988/0.989 vs. 0.991/0.985/0.988 for distances. Bland-Altman analysis showed high levels of agreement between CBCT and MRI with bias values (95% levels of agreement) of 0.03° (- 1.49; 1.54) for angles and 0.02 mm (- 1.44; 1.47) for distances. In the linear mixed effects model, the mean values of CBCT and MRI measurements were equivalent. CONCLUSION: This feasibility study indicates that MRI enables reliable 3D cephalometric analysis with excellent agreement to corresponding measurements on CBCT. Thus, MRI could serve as a non-ionizing alternative to CBCT for treatment planning and monitoring in orthodontics as well as oral and maxillofacial surgery. KEY POINTS: • Clinically established 3D cephalometric measurements performed on MRI are highly reliable and show an excellent agreement with CBCT (gold standard). • The MRI technique applied in this study could be used as a non-ionizing diagnostic tool in orthodontics as well as oral and maxillofacial surgery. • Since most patients benefiting from 3D cephalometry are young in age, the use of MRI could substantially contribute to radiation protection and open up new possibilities for treatment monitoring.

Differences between auto-fluorescence and tetracycline-fluorescence in medication-related osteonecrosis of the jaw-a preclinical proof of concept study in the mini-pig.

OBJECTIVES: Fluorescence-guided bone surgery is a well-established technique in the treatment of medication-related osteonecrosis of the jaw. No histopathological evidence for bone auto-fluorescence is currently available, and thus, any differences from tetracycline-fluorescence remain unclear. Therefore, the goals of this study were to find out if macroscopic and histological differences occur between the auto- and tetracycline-fluorescence in the delineation of viable and necrotic jawbone in the mini-pig. MATERIALS AND METHODS: According to the proof of concept, osteonecrosis was provoked in eight Göttingen minipigs. Pigs were divided into two groups (AF group: no fluorochrome label; TF group: tetracycline label). Delineation of necrosis and viable bone was evaluated in vivo and in vitro macro-/microscopically, correlated to fluorescence properties and compared between the two study groups. RESULTS: No macroscopic and microscopic clinical differences were seen in fluorescence between the AF and TF groups. Macroscopic and microscopic viable bone fluoresced green, whereas necrotic bone showed no or only pale fluorescence in both groups. The auto-fluorescence was attributable to the arrangements and structure of collagen and the cell-filled bone lacunae. CONCLUSION: Neither in vivo nor in vitro macroscopically differences are apparent between the auto-fluorescence and the tetracycline-fluorescence of bone. The auto-fluorescence is attributable to the arrangements and structure of collagen and the cell-filled bone lacunae. Tetracycline-fluorescence is a mixture of tetracycline (at the bone edges with increased bone formation) and large components of auto-fluorescence. CLINICAL RELEVANCE: Because auto-fluorescence is easy to apply, reproducible, and does not rely on the subjective impression of the surgeon, it promises to be an important standardized alternative to tetracycline-labeled MRONJ therapy.

In vivo reliability of 3D cephalometric landmark determination on magnetic resonance imaging: a feasibility study.

OBJECTIVE: 3D cephalometric analysis performed on cone-beam or multi-slice computed tomography (CBCT, MSCT) has superior diagnostic value compared to 2D cephalometry based on radiographs. However, this comes at the expense of increased radiation risks for the predominantly young patients. Magnetic resonance imaging (MRI) has the potential to overcome this diagnostic dilemma but has not been established for 3D cephalometry so far. Since landmark reliability forms the basis for 3D cephalometry, we evaluated the in vivo reliability of established 3D landmarks using MRI. MATERIALS AND METHODS: Sixteen orthodontic patients underwent MRI at 3 Tesla using a 0.5 mm 3D sequence. On each MRI scan, 44 cephalometric landmarks were determined. Image analysis was performed twice by two independent observers. Intra- and inter-rater agreement was assessed by mean measurement errors and intraclass correlation coefficients (ICCs). Measurement errors were calculated as Euclidean distances and distances for x-, y-, and z-coordinates. RESULTS: Overall, MRI-based 3D cephalometric landmarks revealed a high reliability comparable to prior in vivo studies using CBCT/MSCT. Intra- and inter-rater ICCs were consistently higher than 0.9. Intra-rater comparisons showed mean measurement differences (ranges) of 0.87 mm (0.41-1.63) for rater I and 0.94 mm (0.49-1.28) for rater II. Average inter-rater difference was 1.10 mm (0.52-2.29). Distinct differences in reliability between the various landmarks were observed, corresponding well with the landmarks' specific shapes. CONCLUSIONS: The present study demonstrates that MRI enables reliable determination of 3D cephalometric landmarks in vivo. CLINICAL RELEVANCE: This study emphasizes the potential of MRI to perform treatment planning and monitoring in orthodontics as well as oral and maxillofacial surgery without radiation exposure.

Traumatologie des Gesichtsschädels im Kindesalter.

Associated injuries are a common feature in pediatric facial fractures, which reinforce the importance of a complete initial assessment of a child with facial trauma.Particular kinds of fracture tend to occur at specific anatomic sites in children, with the severity and extent of the fracture varying according to the patient's age and the stage of skeletal development. The frequency of severe fractures increases with age, as does the frequency of surgically treated fractures. Maxillofacial surgical intervention is indicated only for the repair of severely displaced fractures that are likely to cause functional impairment, esthetic deformity, or both. The effect of treatment on long-term growth and development must be the cornerstone when choosing the optimal therapeutic option. While internal fixation is routinely used for adult facial trauma patients, the application of these surgical techniques in children is controversial as subperiosteal dissection might disturb the growth of the bone.

Impact of Le-Fort I osteotomy on anatomical and functional aspects of the nasal airway and on quality of life.

OBJECTIVES: Orthognathic surgery is a well-established procedure for skeletal deformities. Beneficial influences to the posterior airway space (PAS) have been described, but little is known about the subjective aesthetical and functional nasal aspects after orthognathic surgery. The aim of this study was to evaluate nasal airflow by anterior rhinomanometry and volumetric changes in the nasal airway space after mono- or bimaxillary surgery using cone-beam computed tomography (CBCT) and a new segmentation software. Furthermore, changes of patient's quality of life (QoL) should be assessed. METHODS: Ten patients (9 skeletal class malformation III, 1 skeletal class malformation I) were included. CBCT images, rhinological inspections and anterior rhinomanometries were performed before (T0) and after surgery (T1). All patients completed the FROI-17, the ROE and the SF-36 questionnaires. RESULTS: A significant postoperative gain for nasal airway volume compared with the baseline was shown (p < 0.014). No statistically significant differences between pre- and postoperative flow rates were found (p = 0.114). Pre- and postoperative cohorts did not differ in responses of disease-specific (ROE and FROI-17) and generic QoL questionnaires (SF-36). CONCLUSION: Maxillary relocation surgery leads to a significant increase in nasal airway space. Subjectively, orthognathic patients did not experience any functional but psychosocial aspects after bimaxillary surgery.

Reconstruction of the severely atrophic edentulous maxillae with calvarial bone grafts.

OBJECTIVES: Treatment of the severely atrophic maxilla with dental implants is challenging due to the insufficient horizontal and vertical bone volume and centripetal resorption pattern of the maxilla. Bone-augmentation procedures are often necessary prior to implant placement. The objective of this study was to assess the suitability of using calvarial bone grafts to enable implant placement in severely atrophic maxillae. MATERIAL AND METHODS: Seventeen patients with severe atrophic edentulous maxillae were reconstructed with autogenous calvarial bone grafts. After a 4-month healing period, the patients received a total of 134 dental implants, which were left to heal in submerged positions for 3 months before prosthetic rehabilitation was performed. Patients were followed clinically and radiographically for an average observation period of 53.94 months. RESULTS: At the intraoral recipient sites, two infections developed, causing partial loss of the respective bone grafts. Implant placement, however, was possible at all sites. No donor-site complications occurred. Two of 134 implants were lost in two patients prior to prosthetic loading. The implant survival rate was 98.51%. The implant success rate was 87.6%, and a mean marginal bone loss of 0.62 mm (SD 0.77 mm) was documented. CONCLUSIONS: Patients with severe bone atrophy of the edentulous maxilla can be successfully reconstructed with calvarial bone grafts and dental implants and show a stable clinical and radiographic situation after a mean observation period of 53 months.

Free Vastus Intermedius Muscle Flap: A Successful Alternative for Complex Reconstruction of the Neurocranium in Preoperated Patients.

The reconstruction of large cranial and scalp defects is a surgical and esthetic challenge. Single autologous tissue transfer can be insufficient due to the defect size and the anatomic complexity of the recipient site. Alloplastic patient-specific preformed implants can be used to recover hard tissue defects of the neurocranium. Nevertheless, for long-term success adequate soft tissue support is required. In this brief clinical study, the authors describe calvarian reconstruction in a 33-year-old patient with wound healing disorder after an initial resection of ependymoma. The patient suffered from osteonecrosis and wound breakdown in the fronto-parietal region. An alloplastic polymethylmethacrylate implant for hard tissue support was manufactured based on 3-dimensional visualization of a computed tomography scan. After the resection of remaining pathologic bone from earlier surgical procedures, the alloplastic implant was inserted to achieve functional coverage of the brain. Due to anatomic variation of donor site vessels during anterolateral thigh flap preparation, the authors performed a vastus intermedius free flap as a new muscular flap for craniofacial reconstruction. The authors achieved excellent functional and esthetic results. The muscular vastus intermedius free flap in combination with a split skin graft proves to be a new alternative to the anterolateral thigh flap for soft tissue reconstruction of the neurocranium.

A Patient With Pansynostosis and Williams-Beuren Syndrome.

BACKGROUND: Williams-Beuren syndrome (WBS) is a multisystemic genetic disorder caused by a gene deletion at gene locus 7q11.23. This article presents the first described case of a patient with WBS and simultaneous pansynostosis. CASE PRESENTATION: This article presents the management of this young Caucasian boy from birth until the age of 12 years and provides an overview of previously described manifestations of WBS in the craniofacial region. CONCLUSIONS: This case demonstrates the surgical treatment of pansynostosis in a child with WBS and might provide interesting aspects in the diagnostics and management of this rare malformation.