Clear Cell Odontogenic Carcinoma: First Report of Novel EWSR1-CREM Fusion Gene in Case of Long-Term Misdiagnosis.

Clear Cell odontogenic Carcinomas (CCOC) are rare, aggressive malignant odontogenic tumours which are often misdiagnosed as benign odontogenic tumours due to the non-specific histologic appearance, and benign early clinical presentation. However, due to their propensity to metastasize, the best outcomes are experienced with they are diagnosed early and treated aggressively. In this paper, we present a case of a CCOC misdiagnosed as a clear cell calcifying epithelial odontogenic tumour which was only found to be a CCOC after cervical node metastasis. The original diagnosis was questioned and confirmed to be a CCOC by identification of the chromosomal translocation EWSR1 on fluorescence in situ hybridization. This has recently been described in CCOC and a wide variety of other mesenchymal and epithelial neoplasms. Previous reports have demonstrated EWSR1-ATF1 and EWSR1-CREB1 fusions in CCOC. Next generation sequencing of this case demonstrated the EWSR1-CREM fusion gene which has not been previously reported for CCOC. CREM fusion proteins have only recently been found in several tumour types including the closely associated hyalinizing clear cell carcinoma of salivary glands. This is discussed in this paper, and the role of the discovery of the CREM fusion protein in CCOC adds to your understating of the role of CREM in oncogenesis, and the possible link between CCOCs and hyalinizing clear cell carcinomas.

Factors affecting prognosis in locoregional recurrence of oral squamous cell carcinoma.

BACKGROUND: Recurrence of oral squamous cell carcinoma (rOSCC) after primary treatment is associated with poor survival outcomes. Salvage treatment with further surgery, radiotherapy and chemotherapy has high morbidity, making patient selection crucial. However, in the recurrence setting, reliable stratification data are scarce. Decision-making in this context is consequently complex. We investigated factors influencing overall survival after rOSCC. METHODS: Retrospective cohort study of patients with rOSCC (n = 83) at the Queen Elizabeth Hospital Birmingham, UK between 2006 and 2016. Associations with overall survival were analysed using univariate and multivariate analyses to identify important clinical prognostic indicators. RESULTS: Overall survival at 1 year was 32.5% and at 5 years was 18.1% after a median follow-up of 7.4 months. Multivariate analysis identified four independent predictors of overall survival following rOSCC: size of primary tumour (HR 2.077; 95% CI 1.034-4.172), extent of recurrent disease (HR 3.286; 95% CI 1.545-6.991), history of moderate alcohol consumption (HR 0.351; 95% CI 0.162-0.763), and close or positive margins at primary resection (HR 1.955; 95% CI 1.063-3.595). CONCLUSIONS: We identified four key factors that help prognostication and risk stratification of rOSCC. Given the high morbidity associated with salvage treatment, we recommend that the multidisciplinary team (MDT) and the patient weigh these factors carefully when considering further treatment. Further investigation of the biology underlying these oncophenotypes may contribute to better patient stratification.

Free Bone Grafts for Mandibular Reconstruction in Patients Who Have Not Received Radiotherapy: The 6-cm Rule-Myth or Reality?

Bony reconstruction of the mandible after surgical resection results in improved rehabilitation and aesthetics. Composite tissue transfer has transformed reconstruction, particularly in patients who have received radiotherapy. However, there is morbidity related to free tissue transfer. Free nonvascularized bone grafts have much lower morbidity. Surgeons believe that free bone grafts greater than 6.0 cm are prone to failure. The aims of this study was to assess whether bone grafts greater than 6.0 cm in length have a high risk of failure. A retrospective study was performed on all patients who had free bone grafts greater than 6.0 cm in length at Birmingham, UK, and Florida, the United States. None of the patients received radiotherapy. A total of 14 patients had undergone bone grafts for mandibular defects greater than 6.0 cm in length; 13 of the bone grafts were successful. Of these 13, none were infected and there was radiographic evidence of bony union. Some of the patients have been dentally rehabilitated with implants. Contrary to much of the literature and many surgeons belief, our study has shown that long mandibular defects (>6.0 cm) are not a contraindication to the use of free bone grafts. Key principles to achieve success are discussed in this article.

Jejunal free flap for reconstruction of pharyngeal defects in patients with head and neck cancer-the Birmingham experience.

We retrospectively audited operative complications, success of flaps, and speech and swallowing outcomes in patients with head and neck cancer who had reconstruction with jejunal free tissue transfer to the pharynx. A total of 104 patients had jejunal free flaps between 1987 and 2009 at University Hospital, Birmingham. Management was by a multidisciplinary team, and the same vascular surgeon did all the anastomoses. We investigated the relations between patients, operative factors, and postoperative complications, and noted the ischaemic time of the flaps and coexisting conditions of the patients. Outcomes measured included initial and final survival rates of flaps, donor and recipient site complications, and speech and swallowing outcomes on discharge and up to 2 years postoperatively. Of the 104 patients, 14 (13%) had initial flap complications but overall flap survival was 97%. A total of 11 (11%) patients developed a fistula at a mean of 15 days postoperatively and 11 (11%) had minor donor site complications. A total of 95 (91%) were able to resume oral diet on discharge. Of the 44 who were followed up on discharge, 32 (73%) were able to maintain oral intake at 2 years and 31 (70%) could use their voice in everyday situations. The jejunal free flap enables the tumour to be removed, and reconstruction and restoration of function to be done in a single operation using tissue that is versatile. The operation is associated with low morbidity at the donor and recipient sites, and results in good speech and swallowing outcomes. The flap can also be used to reconstruct pharyngolaryngeal defects.

Use of a titanium cutting guide to assist with raising and inset of a DCIA free flap.

Reconstructing maxillary defects can be challenging. In particular, Class 3 and 4 defects require careful planning. Bone for reconstruction must be placed in the correct three dimensional (3D) position in order to achieve a good cosmetic and functional result. Correct size and positioning of the harvested bone also enables placement of dental implants and allows rehabilitation. We describe the use of a Deep Circumflex Iliac Artery Flap (DCIA) cutting guide to aid safe harvest and positioning of the correct quality and quantity of bone. We illustrate the benefits of using a guide based upon stereolithographic planning models of the pelvis by presenting 4 cases where planning to this level has been used. Our experiences clearly demonstrate the potential benefits that preformed cutting guides may have on outcomes when reconstructing complex maxillary defects. In particular, we note a reduction in operative time and improved functional and aesthetic results. The use of such a guide has not previously been described.

Interobserver agreement of the German translation of the Motivational Interviewing Sequential Code for Observing Process Exchanges (MI-SCOPE;D).

The aim of this study is to assess the interobserver agreement of the German translation of the MI-SCOPE. We applied it to transcribed counseling sessions on smoking cessation and relapse prevention with women postpartum. The MI-SCOPE is an instrument to assess and quantify MI-specific processes. Twenty percent random samples for parsing and coding each were drawn from 162 transcripts of MI sessions from the treatment arm of an RCT to assess interobserver agreement. Whole transcripts were coded. Each transcript was randomly assigned to two of three raters who parsed and coded the transcripts separately. Cohen's κ was computed to assess interobserver agreement. Concordance about parses ranged between κ = .702 and κ = .955 (25th percentile-median-75th percentile: .896-.918-.936). The kappas did not differ significantly between the three combinations of raters (H = 2.648; df = 2, p = .266). Concordance about codes for all 46 categories ranged between κ = .590 and κ = .822 (25th percentile-median-75th percentile: .680-.718-.748). The kappas did not differ significantly between the three combinations of raters (H = 4.095; df = 2, p = .129). The German translation of the MI-SCOPE yielded good to excellent κ for parsing as well as for coding. This indicates that MI as an intervention can be taught, learned, and adherence to MI as an intervention may be objectively observed and assessed.

Maxillofacial injuries in the workplace.

Over a 2-year period we reviewed patients who presented to a UK maxillofacial unit with facial injuries sustained at work. We looked at links between the mechanism, injury, and characteristics of such injuries.

Case report: atypical presentation of jugular foramen mass.

INTRODUCTION: Jugular foramen lesions are often associated with pathology of adjacent structures due to either compression or direct invasion. Common presenting symptoms include pulsatile tinnitus, a neck mass, hearing loss, and cranial nerve palsies, leading to changes in taste, vocal cord paralysis, dysphagia, and sternocleidomastoid/trapezius weakness (A. Hakuba, K. Hashi, K. Fujitani, et al., Jugular foramen neurinomas. Surg Neurol 1979; 11:83-94). This patient was found to have a jugular foramen mass after presenting with the unusual constellation of visual changes and headache. CASE PRESENTATION: A jugular foramen mass in a young woman was discovered after presenting with visual changes and headache; the patient was found to have papilledema on initial examination. Otologic and head and neck examination were normal. Subsequent imaging demonstrated a mass at the right jugular foramen with compression of this structure; a contralateral transverse sinus stenosis was also seen. This latter abnormality (along with obstruction of the jugular foramen) impeded venous drainage leading to papilledema and visual changes. DISCUSSION: In a patient presenting with papilledema and severe headache with an associated jugular foramen mass, a multidisciplinary approach benefits the patient with input from interventional neuroradiology, neurosurgery, and neuro-ophthalmology. Venous outflow was compromised through the left stenotic transverse sinus, and the normal outflow on the right side through the jugular bulb was impeded by the tumor; obstructions of both led to symptomatic impeded venous outflow. This compromise in venous outflow led to an increase in superior sagittal sinus pressure, with subsequent increase in intracranial pressure and resultant papilledema. In an attempt to increase blood flow, an angioplasty was performed on the patient's affected transverse sinus. In addition, symptomatology consistent with pseudotumor cerebri prompted the use of acetazolamide for medical management. After both therapies, the patient's symptoms dramatically improved and were stable. The tumor has also remained stable, with no immediate need for surgical resection, stereotactic radiation, or consideration of an intraluminal transverse sinus stent placement or shunting. CONCLUSION: The unique presentation of a jugular foramen mass in a young woman leading to papilledema highlights the need for high clinical suspicion of potential etiologies necessary for diagnosis. Despite the benign nature of her disease process, an unusual constellation of anatomical factors lead to the need for acute intervention.

The use of a T-shaped contoured unilocking titanium radial plate for prophylactic internal fixation of the radial osteocutaneous donor site.

The radial osteocutaneous flap retains a limited role in reconstructive maxillofacial surgery The application of prophylactic internal fixation, using straight 3.5 mm plates, has become established to substantially reduce the incidence of fracture at the radial donor site. New lower profile T-shaped 2.4 mm plates and anatomically contoured 3.5 mm plates are now available, both with unilocking screw fixation systems. These plates are easy to apply and allow the removal of up to 50% of the circumference of the radial bone, including the maximum amount of good quality bone from the distal radius. Although there have been no reports of complications as a result of a stress shielding effect with larger plates these refinements in plate design should lessen any remaining concerns.

Correlation of aberrometry, contrast sensitivity, and subjective symptoms with quality of vision after LASIK.

PURPOSE: To compare which parameter category (wavefront data, psychophysical data, or subjective symptoms) predicts best subjective quality of vision after LASIK. METHODS: Twenty-eight eyes (15 patients) were included. Twenty-three eyes (12 patients) underwent uneventful LASIK; 5 eyes (3 patients) were symptomatic eyes treated with myopic LASIK elsewhere. Mean preoperative spherical equivalent refraction was -4.79+/-1.92 diopters (D) (range: -1.63 to -7.13 D); mean patient age was 36.6+/-7.4 years (range: 18 to 48 years). All examinations were performed 1 month postoperatively. The wavefront error was described with Zernike polynomials (6-mm pupil). Psychophysical tests included high-contrast visual acuity and contrast sensitivity with and without glare at 167 cd/m(-2), 1.67 cd/m(-2), and 0.167 cd/m(-2) with best spectacle correction. Correspondingly, overall subjective quality of vision and frequency of visual symptoms (glare, halos, starbursts, ghosting, blur) were assessed for three lighting conditions (photopic, high-mesopic, and low-mesopic) using a questionnaire with a visual analog scale. For each parameter category and each lighting condition, a multiple stepwise backwards regression model with the overall quality of vision item value as dependent was applied. RESULTS: Under all lighting conditions, subjective symptom scores predicted subjective quality of vision best (adjusted R2=0.83-0.92) with blur as the main predictor throughout all conditions. Psychophysical tests did not significantly predict postoperative subjective quality of vision. The adjusted R2 for the Zernike coefficients was highest for low-mesopic (0.56) and lowest for photopic conditions (0.31). CONCLUSIONS: Different parameter categories for the description of optical quality did not predict subjective quality of vision after LASIK equally. Subjective symptom scores had the highest predictability, whereas psychophysical tests with spectacle correction had no predictability. The latter probably do not reflect all dimensions of subjective quality of vision.

Comparison of optical quality metrics to predict subjective quality of vision after laser in situ keratomileusis.

PURPOSE: To compare wavefront-derived metrics to predict subjective quality of vision after laser in situ keratomileusis (LASIK) for myopia. SETTING: Department of Ophthalmology, Goethe University, Frankfurt am Main, Germany. METHODS: One month postoperatively, wavefront sensing was performed and overall subjective quality of vision assessed under 3 lighting conditions (photopic, high mesopic, low mesopic) with a questionnaire. Four wavefront-error representations were computed for a pupil diameter of 6.0 mm and individual physiological pupil diameters at 0.4 lux: (1) the visual Strehl ratio based on optical transfer function (VSOTF), (2) the root-mean-square (RMS) value of Zernike orders 2 to 5 (total RMS), (3) higher-order aberration (HOA) RMS, and (4) a wavefront-error breakdown into the RMS of lower-order aberrations, coma, spherical aberration, and remaining HOA. The impact of the postoperative wavefront error on subjective quality of vision was calculated using linear regression analysis. RESULTS: Fifty-six eyes (29 patients) were included. The ability of wavefront error-derived metrics to predict subjective quality of vision was limited. The VSOTF, calculated for the best-corrected eye, showed the highest predictability. Calculation of wavefront error for individual physiological pupil diameters did not improve predictive ability of the metrics. Eyes with a high theoretical retinal-image quality had a high subjective quality of vision, and eyes with a low subjective quality of vision had a low theoretical image quality. CONCLUSIONS: Postoperative wavefront error had limited influence on the subjective quality of vision. Postoperative retinal image quality should be kept as high as possible to provide good subjective quality of vision.