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Introduction The authors present the guidelines of the French Society of Otorhinolaryngology (SFORL) for clinical and radiological assessment of cystic neck lymphadenopathy of unknown primary in adults. Most cases concern head and neck carcinoma metastasis, often in the oropharyngeal area, or less frequently differentiated thyroid carcinoma or non-keratinizing nasopharyngeal carcinoma. Methods A multidisciplinary task force was commissioned to carry out a review of the literature on the etiological work-up in cystic neck lymphadenopathy in adults: clinical examination, conventional imaging (ultrasound, CT, MRI) and metabolic imaging. Guidelines were drafted based on the articles retrieved, and graded A, B, C or expert opinion according to decreasing level of evidence. Results Oriented clinical examination, cervical and thyroid ultrasound scan and contrast-enhanced neck and chest CT scan are recommended in the assessment of cystic neck lymphadenopathy of unknown primary in adult patients. PET-CT is recommended prior to panendoscopy, to identify the primary tumor. Conclusion Clinical and radiological assessment is fundamental for etiologic diagnosis of cystic neck lymphadenopathy in adult patients, and should be completed by cytological examination before in initiating treatment.

Background The aim of this study was to evaluate the impact of the level of neck transection on clinically relevant postoperative pancreatic fistula (CR-POPF) after standard pancreaticoduodenectomy (PD) with pancreaticojejunostomy. Method A total of 195 patients with an early postoperative CT scan were retrospectively analyzed and divided into 2 groups (CR-POPF and No CR-POPF) in order to seek potential risk factors for CR-POPF. We focused our analysis on the relationship between CR-POPF and the level of neck transection, defined by measuring the distance between the left side of the portal vein and the remnant pancreatic stump on the postoperative CT scan. Result CR-POPF occurred in 58 out of 195 PD (29.7%); grade B (17%) and grade C (12.7%). The Clavien-Dindo ≥ 3 morbidity rate was 33% (65/195) and the mortality rate was 2.5% (5/195). Multivariate analysis indicated that a ‘right-sided’ level of neck transection (P = 0.007), a firm pancreatic texture (P = 0.001), and a PD for non-pancreatic ductal adenocarcinoma histology (P = 0.032) were independent risk factors for CR-POPF. A full neck resection with systematic transection ≥7 mm at the left side of the portal vein seems to prevent CR-POPF harboring a protective effect (OR 0.056; 95% CI 0.003 to 0.978; P = 0.039). Conclusion Here we further consolidate the concept describing the pancreatic neck as a vascular watershed, showing that a long remnant pancreatic neck could be an independent risk factor for CR-POPF after PD (NCT03850236). Trial registration number and agency The present study was approved by our local ethics committee and was declared on ClinicalTrials.gov (ID: NCT03850236).

International audience; Variants affecting the function of different subunits of the BAF chromatin-remodelling complex lead to various neurodevelopmental syndromes, including Coffin-Siris syndrome. Furthermore, variants in proteins containing PHD fingers, motifs recognizing specific histone tail modifications, have been associated with several neurological and developmental-delay disorders. Here, we report eight heterozygous de novo variants (one frameshift, two splice site, and five missense) in the gene encoding the BAF complex subunit double plant homeodomain finger 2 (DPF2). Affected individuals share common clinical features described in individuals with Coffin-Siris syndrome, including coarse facial features, global developmental delay, intellectual disability, speech impairment, and hypoplasia of fingernails and toenails. All variants occur within the highly conserved PHD1 and PHD2 motifs. Moreover, missense variants are situated close to zinc binding sites and are predicted to disrupt these sites. Pull-down assays of recombinant proteins and histone peptides revealed that a subset of the identified missense variants abolish or impaire DPF2 binding to unmodified and modified H3 histone tails. These results suggest an impairment of PHD finger structural integrity and cohesion and most likely an aberrant recognition of histone modifications. Furthermore, the overexpression of these variants in HEK293 and COS7 cell lines was associated with the formation of nuclear aggregates and the recruitment of both wild-type DPF2 and BRG1 to these aggregates. Expression analysis of truncating variants found in the affected individuals indicated that the aberrant transcripts escape nonsense-mediated decay. Altogether, we provide compelling evidence that de novo variants in DPF2 cause Coffin-Siris syndrome and propose a dominant-negative mechanism of pathogenicity.

International audience; BACKGROUND: Using the intubation difficulty scale (IDS), we sought to confirm that obese patients are more difficult to intubate than lean patients. We assessed classical bedside tests and included neck circumference. METHODS: We prospectively compared the incidence of difficult tracheal intubation in 70 obese [body mass index (BMI) > or = 30 kg/m(2)] and 61 lean patients (BMI < 30 kg/m(2)). The IDS scores, categorized as difficult intubation (IDS > 5) or not (IDS < or = 5), and the patient data, were compared between lean and obese patients. Preoperative measurements [BMI, neck circumference (at the level of the thyroid cartilage), width of mouth opening, sternomental distance, and thyromental distance], medical history of obstructive sleep apnea syndrome, and several scores (Mallampati, Wilson, El Ganzouri) were recorded. The view during direct laryngoscopy was graded, and the IDS was recorded. We then compared patients with IDS < or = 5 and > 5, concerning each item. RESULTS: The results indicate that difficult tracheal intubation is more frequent in obese than in lean patients (14.3% vs 3%; P = 0.03). In the patients with IDS > 5, thyromental distance, BMI, large neck circumference, and higher Mallampati score were the only predictors of potential intubation problems. CONCLUSION: We found that problematic intubation was associated with thyromental distance, increasing neck circumference, BMI, and a Mallampati score of > or = 3. Neck circumference should be assessed preoperatively to predict difficult intubation.

International audience; Radiotherapy planning requires accurate delineations of the critical structures. To avoid manual contouring, atlas-based segmentation can be used to get automatic delineations. However, the results strongly depend on the chosen atlas, especially for the head and neck region where the anatomical variability is high. To address this problem, atlases adapted to the patient's anatomy may allow for a better registration, and already showed an improvement in segmentation accuracy. However, building such atlases requires the definition of a criterion to select among a database the images that are the most similar to the patient. Moreover, the inter-expert variability of manual contouring may be high, and therefore bias the segmentation if selecting only one image for each region. To tackle these issues, we present an original method to design a piecewise most similar atlas. Given a query image, we propose an efficient criterion to select for each anatomical region the K most similar images among a database by considering local volume variations possibly induced by the tumor. Then, we present a new approach to combine the K images selected for each region into a piecewise most similar template. Our results obtained with 105 CT images of the head and neck show that our method reduces the over-segmentation seen with an average atlas while being robust to inter-expert manual segmentation variability.

International audience; A novel mechanism of MRI contrast enhancement, based on the detection by a balanced steady-state free precession (SSFP) sequence of the proton resonance frequency shift induced by bulk magnetic susceptibility (BMS) contrast agents, was investigated. The potential for this contrast mechanism to image blood vessels was explored. The relaxation time and the frequency shift effects of gadolinium- and dysprosium-DOTA on SSFP signal was first simulated and evaluated on a water phantom at 1.5 T. In vitro, a 5-mM concentration in contrast agent induced a 20-Hz frequency shift, leading to a signal increase of 92% for Dy-DOTA, and a 10-Hz frequency shift, leading to a signal increase of 58% for Gd-DOTA at the reference frequency, taking into account the nonlinear SSFP signal response on frequency offset. The concept was then evaluated in vivo on anesthetized rabbits. Low doses of dysprosium-DOTA were injected in their vascular system, and imaging was performed at the level of neck vessels. Following a bolus injection, mean signal changes of 31%, 20% and 14% were observed in the carotid arteries, the vertebral veins and the jugular veins, respectively. The bolus peak times in arteries and veins were consistent with the rabbit vascular circulation. This frequency-shift based contrast mechanism presents interesting potential for contrast-enhanced MR angiography (CE-MRA) compared to usual relaxation-based contrast, but further investigations on reproducibility will be necessary.

The purpose of the present experiment was to investigate the extent to which subjects can perceive, at very slow velocities, an angular rotation of the support surface about the medio-lateral axis of the ankle, knee, hip, or neck joint when visual cues are not available. Subjects were passively displaced on a slowly rotating platform at .01, .03, and .05 deg/sec. The subjects' task was to detect movements of the platform in four different postural conditions allowing body oscillations about the ankle, knee, hip, or neck joint. In Experiment 1, subjects had to detect backward and forward rotation (pitching). In Experiment 2, they had to detect left and right rotations of the platform (rolling). In Experiment 3, subjects had to detect both backward/forward and left/right rotations of the platform, with the body fixed and the head either fixed or free to move. Overall, when the body was free to oscillate about the ankle, knee, or hip joints, a similar threshold for movement perception was observed. This threshold was lower for rolling than for pitching. Interestingly, in these postural conditions, an unconscious compensation in the direction opposite to the platform rotation was observed on most trials. The threshold for movement perception was much higher when the head was the only segment free to oscillate about the neck joint. These results suggest that, in static conditions, the otoliths are poor detectors of the direction of gravity forces. They also suggest that accurate perception of body orientation is improved when proprioceptive information can be dynamically integrated.

International audience; BACKGROUND: To lower the risk of complications, carotid angioplasty and stenting (CAS) has been proposed as an alternative to open surgery for carotid artery stenosis after neck irradiation. However, there are little postoperative data to support the benefits of this strategy. This study evaluated the outcome of CAS in patients who had undergone neck irradiation. METHODS: This retrospective study was conducted at 15 vascular surgery or interventional radiology centers in France between January 1998 and July 2006. A total of 135 patients (115 men) with a mean age of 67 +/- 8 years (range, 43-88) underwent CAS for 149 irradiation-induced lesions. The interval between irradiation and discovery of the lesions was 12 +/- 8 years. Mean diameter reduction was 81% (range, 50%-95%), and stenosis was symptomatic in 34%. Contralateral carotid lesions were observed in 48% of patients, including thrombosis in 18 and stenosis >50% in 53. RESULTS: Technical failure occurred during CAS in three cases. The overall technical success rate was 98%. A cerebral protection device was used in 59%. No death, one transient ischemic attack, and two strokes occurred during the first postoperative month. Mean follow-up was 30 months. Six patients were lost to follow-up. Survival rates were 93.9% at 1 year and 75.3% at 3 years. Complications after the first postoperative month included neurologic events in six, carotid thrombosis in nine, and restenosis in 18. The rates of freedom from neurologic and anatomic events were, respectively, 96.2% and 93.2% at 1 year and 93.1% and 85.9% at 3 years. CONCLUSION: The immediate outcome of CAS for irradiation-induced carotid artery stenosis was satisfactory. Medium-term neurologic outcome was acceptable, but the incidence of anatomic events such as thrombosis and restenosis was high. A randomized study is needed to confirm that the outcome of the endovascular and surgical therapy is comparable in this indication.