Dangers and therapeutic difficulties of intracranial hemangioma in infants: A CARE case report.

INTRODUCTION: Description of neurological complications induced by intracranial hemangioma in infants and by the initiation of beta-blocker treatment (propranolol). OBSERVATION: A 2-month-old infant was referred for grade 5 non-congenital unilateral peripheral facial palsy. Work-up revealed ipsilateral profound hearing loss and two intracranial hemangiomas: one in the ipsilateral internal auditory canal (IAC), the other in the cerebellum opposite the nodule of vermis. Initial treatment with a beta-blocker (propranolol 1mg/kg/day for 1month, then 3mg/kg/day) resulted in disappearance of symptoms and regression of lesions within 8weeks. At 20months after introduction of maintenance therapy (propranolol 3mg/kg/day), two asthma attacks occurred, leading to initiation of fluticasone and continuation of the beta-blocker. Thirty months after discontinuation of treatment, no further progression was noted. DISCUSSION: Unilateral facial palsy in an infant suggests a number of diagnoses. MRI revealed IAC hemangioma. The choice of dosage and duration of treatment was based on a review of the literature and a strategy defined in multidisciplinary consultation.

Carcinomas of the external auditory canal: Management and results: A multicenter REFCOR propensity score matching study.

OBJECTIVES: To analyse prognostic factors and survival outcomes of malignant tumors of the external auditory canal, to investigate the role of regional surgery, and adjuvant radiotherapy in early stages and to investigate the role of surgery in operable T4 stage. SETTING: A retrospective analysis was conducted on all patients prospectively included in the national database of the French Expertize Network for Rare ENT Cancers (REFCOR) from January 2000 to December 2016. PARTICIPANTS: 103 patients from 19 reference centers were included. A propensity score matching analysis was applied to enable comparisons between treatments. MAIN OUTCOMES AND MEASURES: Event-free survival, overall survival and factors of poor prognosis of the cohort were described. The interest of local and regional surgery and postoperative radiotherapy were evaluated. RESULTS: The factors of poor prognosis on event-free survival were immunosuppression (p = 0.002), Karnofsky status less than 90% (p = 0.02), body mass index less than 19 Kg / m2 (p = 0.0009), peripheric facial palsy (p = 0.0016), and positive margin (p = 0.0006). In early stages, locoregional surgery was associated with an increase in event-free survival (p = 0.003, HR = 0.21) versus local surgery alone, while postoperative radiotherapy was not associated with an increase in event-free survival (p = 0.86, HR = 0.91) or overall (p = 0.86, HR = 0.91). In locally advanced stages, locoregional surgery followed by radiotherapy was associated with an increase in event-free survival (p = 0.03, HR = 0.39) and overall (p = 0.02, HR = 0.34) versus chemoradiotherapy alone. CONCLUSION AND RELEVANCE: Regional surgery is recommended for early stages of cancers of the external auditory canal. In operable cases, locoregional surgery followed by radiotherapy is recommended.

Double jeopardy, glomangiopericytoma and Glanzmann thrombasthenia resulting in recurrent epistaxis: a case report.

Glanzmann thrombasthenia is a rare bleeding disorder induced by inherited defects of the platelet membrane αIIbß3 glycoprotein. Glomangiopericytoma, on the other hand, is a very rare sinonasal tumor demonstrating a perivascular myoid phenotype. We herein report the first described case in the literature of Glanzmann thrombasthenia and glomangiopericytoma. The patient is a 40-year-old man diagnosed with type 1 Glanzmann thrombasthenia who presented with repetitive and profuse posterior epistaxis initially managed with platelet transfusions and recombinant activated factor VII (rFVIIa). Due to the unresolved epistaxis, nasal endoscopy was performed revealing a vascularized tumor. Subsequently, a sphenopalatine artery embolization followed by a surgical excision of the tumor was performed. The pathology report diagnosis of the tumor was glomangiopericytoma. This case sheds the lights on a very rare cause of epistaxis in a patient with Glanzmann thrombasthenia, with a challenging multidisciplinary management. A local cause of epistaxis should always be considered even in case of a diagnosed bleeding disorder, especially when the bleeding is recurrent.

Definition of severity and treatment response in chronic rhinosinusitis with nasal polyps: a Delphi study among French experts.

INTRODUCTION: The introduction of biotherapies has significantly changed the management of patients with chronic rhinosinusitis with nasal polyps (CRSwNP). These drugs are generally reserved for severe or recurrent CRSwNP. Thus, the concepts of severity of the disease and treatment response must be mastered by otorhinolaryngologists. However, a clear definition of these concepts in CRSwNP is missing. METHODS: This article focuses on definitions of severity and treatment response in CRSwNP by providing an expert consensus among French rhinologists, using a Delphi study. RESULTS: The severity assessment should seek the presence of uncontrolled asthma, olfactory disorders, nasal blockage, impaired quality of life (QOL) and cumulative annual dose of systemic corticosteroids.The treatment response should assess the presence of olfactory disorders, nasal blockage, QOL impairment, response to background therapy, resistance and/or dependence to oral corticosteroids, cumulative annual dose of systemic corticosteroids, response to surgery and to biologics.A failure after polypectomy should not be considered as a failure of surgical management of CRSwNP and must discuss the realization of an extended sinus surgery procedure before the prescription of biologics. CONCLUSION: Definitions of severity, control of CRSwNP, as well as therapeutic strategies to improve patients' QOL achieved high level of consensus.

Biomimetic Bilayered Scaffolds for Tissue Engineering: From Current Design Strategies to Medical Applications.

Tissue damage due to cancer, congenital anomalies, and injuries needs new efficient treatments that allow tissue regeneration. In this context, tissue engineering shows a great potential to restore the native architecture and function of damaged tissues, by combining cells with specific scaffolds. Scaffolds made of natural and/or synthetic polymers and sometimes ceramics play a key role in guiding cell growth and formation of the new tissues. Monolayered scaffolds, which consist of uniform material structure, are reported as not being sufficient to mimic complex biological environment of the tissues. Osteochondral, cutaneous, vascular, and many other tissues all have multilayered structures, therefore multilayered scaffolds seem more advantageous to regenerate these tissues. In this review, recent advances in bilayered scaffolds design applied to regeneration of vascular, bone, cartilage, skin, periodontal, urinary bladder, and tracheal tissues are focused on. After a short introduction on tissue anatomy, composition and fabrication techniques of bilayered scaffolds are explained. Then, experimental results obtained in vitro and in vivo are described, and their limitations are given. Finally, difficulties in scaling up production of bilayer scaffolds and reaching the stage of clinical studies are discussed when multiple scaffold components are used.

Diffusion-weighted and gadolinium-enhanced dynamic MRI in parotid gland tumors.

PURPOSE: To evaluate the value of diffusion-weighted imaging and dynamic contrast-enhanced MRI for the diagnosis of parotid gland tumors. METHODS: Retrospective review of patients with surgically treated parotid tumors between January 2009 and June 2020, who underwent a preoperative parotid gland MRI including standard morphological sequences, diffusion-weighted echoplanar imaging with apparent diffusion coefficient measurement and T1-weighted gadolinium-enhanced dynamic MRI sequences with Fat Saturation. The lesion was classified between malignant vs benign and precisions regarding its histological type were given when possible. Imaging findings were compared with pathology results. RESULTS: Inclusion of 133 patients (mean age: 53 years). Multiparametric MRI had a sensitivity of 90.3%, a specificity of 77.5%, an overall accuracy of 80.5%, a positive predictive value of 54.9% and a negative predictive value of 96.3% to differentiate benign parotid tumor from malignant ones. Specificity (85.5%) and positive predictive value (67.6%) were improved for cases, where anatomical and functional MRI characteristics were conclusive and consistent with clinical findings. CONCLUSIONS: Combining diffusion-weighted and gadolinium-enhanced dynamic sequences, in addition to morphological ones enables high (> 90%) sensitivity to detect malignant parotid gland tumors. It also gives the possibility to characterize pleomorphic adenomas and Warthin tumors and to avoid fine-needle aspiration in cases of typical imaging presentation and reassuring clinical findings.

Airway replacement using stented aortic matrices: Long-term follow-up and results of the TRITON-01 study in 35 adult patients.

Over the past 25 years, we have demonstrated the feasibility of airway bioengineering using stented aortic matrices experimentally then in a first-in-human trial (n = 13). The present TRITON-01 study analyzed all the patients who had airway replacement at our center to confirm that this innovative approach can be now used as usual care. For each patient, the following data were prospectively collected: postoperative mortality and morbidity, late airway complications, stent removal and status at last follow-up on November 2, 2021. From October 2009 to October 2021, 35 patients had airway replacement for malignant (n = 29) or benign (n = 6) lesions. The 30-day postoperative mortality and morbidity rates were 2.9% (n = 1/35) and 22.9% (n = 8/35) respectively. At a median follow-up of 29.5 months (range 1-133 months), 27 patients were alive. There have been no deaths directly related to the implanted bioprosthesis. Eighteen patients (52.9%) had stent-related granulomas requiring a bronchoscopic treatment. Ten among 35 patients (28.6%) achieved a stent free survival. The actuarial 2- and 5-year survival rates (Kaplan-Meier estimates) were respectively 88% and 75%. The TRITON-01 study confirmed that airway replacement using stented aortic matrices can be proposed as usual care at our center. Clinicaltrials.gov Identifier: NCT04263129.

Skull Vibration-Induced Nystagmus Test in a Human Model of Horizontal Canal Plugging.

BACKGROUND/AIM: the aim of this study was to assess the skull vibration-induced nystagmus test (SVINT) results and vestibular residual function after horizontal semicircular canal (HSCC) plugging. METHODS: In this retrospective chart review performed in a tertiary referral center, 11 patients who underwent unilateral horizontal semicircular canal plugging (uHSCCP) for disabling Menière's disease (MD) were included. The skull vibration-induced nystagmus (SVIN) slow-phase velocity (SPV) was compared with the results of the caloric test (CaT), video head impulse test (VHIT), and cervical vestibular-evoked myogenic potentials (cVEMP) performed on the same day. RESULTS: Overall, 10 patients had a strong SVIN beating toward the intact side (Horizontal SVIN-SPV: 8.8°/s ± 5.6°/s), 10 had a significant or severe ipsilateral CaT hypofunction, 10 had an ipsilateral horizontal VHIT gain impairment, and 3 had altered cVEMP on the operated side. Five had sensorineural hearing worsening. SVIN-positive results were correlated with CaT and horizontal VHIT (HVHIT) results (p < 0.05) but not with cVEMP. SVIN-SPV was correlated with CaT hypofunction in % (p < 0.05). Comparison of pre- and postoperative CaT % hypofunction showed a significant worsening (p = 0.028). CONCLUSION: SVINT results in a human model of horizontal canal plugging are well correlated with vestibular tests exploring horizontal canal function, but not with cVEMP. SVINT always showed a strong lesional nystagmus beating away from the lesion side. SVIN acts as a good marker of HSCC function. This surgical technique showed invasiveness regarding horizontal canal vestibular function.

An image-guided (CT) assessment of a new asymmetric balloon for the treatment of epistaxis.

PURPOSE: The main objective was to perform an image-guided (CT) assessment of the efficacy of the CAVI-T™ balloon to compress the sphenopalatine artery (SPA) on cadaver heads, for the management of epistaxis. The secondary objectives were to analyse the deployment and stability of this balloon according to the volume injected into the nasal cavity, to optimise its use. METHODS: A descriptive anatomical study was performed. The catheterization of the SPA was performed on four fresh-frozen heads with a SPA approach through the maxillary sinus, leaving the nasal cavity unscathed. Computed Tomography images were acquired without and with the balloon, inflated by injections of progressive volumes of diluted iodine, for optimal contrast with the surrounding tissues. We evaluated the positioning of the balloon according to two predetermined markers on the device. RESULTS: Out of 68 image-guided acquisitions, the CAVI-T™ balloon compressed the SPA in 88% of cases. The other nasal cavity structures were compressed in 86% to 100% of the cases, depending on the positioning of the CAVI-T™ balloon, therefore allowing a complete obstruction of the nasal cavity. The device remained stable upon inflation and did not obstruct the nasopharynx. CONCLUSION: The CAVI-T™ balloon provided effective compression of the SPA and the different structures of the nasal cavity.

The dynamics of endolymphatic hydrops and vestibular disorders.

Endolymphatic hydrops is defined as an accumulation of endolymph in the inner ear leading to a buildup of pressure and distortion of intralabyrinthine structures. The pressure variation is neither obvious nor easy to measure and remains not clearly confirmed. The distortion of endolymphatic structures has been the main described phenomenon since Hallpike, Cairns and Yamakawa in 1938. However, some clinical symptoms associated with endolymphatic hydrops are in addition to the typical triad of symptoms of Meniere's disease. This introduction to the state of the art is an analysis of the relationship between hydrops and clinical vestibular disorders, with a focus on the dynamics of endolymphatic hydrops. The distortion of endolabyrinthine structures can be considered as a dynamic process modeled with mechanical elastic behavior.

Predictive Value of Early Post-Treatment Diffusion-Weighted MRI for Recurrence or Tumor Progression of Head and Neck Squamous Cell Carcinoma Treated with Chemo-Radiotherapy.

AIMS: To investigate the predictive capacity of early post-treatment diffusion-weighted magnetic resonance imaging (MRI) for recurrence or tumor progression in patients with no tumor residue after chemo-radiotherapy (CRT) for head and neck squamous cell carcinoma, and, to assess the predictive capacity of pre-treatment diffusion-weighted MRI for persistent tumor residue post-CRT. MATERIALS AND METHOD: A single center cohort study was performed in one French hospital. All patients with squamous cell carcinoma receiving CRT (no surgical indication) were included. Two diffusion-weighted MRI were performed: one within 8 days before CRT and one 3 months after completing CRT with determination of median tumor apparent diffusion coefficient (ADC). MAIN OUTCOME: The primary endpoint was progression-free survival. RESULTS: 59 patients were included prior to CRT and 46 (78.0%) completed CRT. A post-CRT tumor residue was found in 19/46 (41.3%) patients. In univariate analysis, initial ADC was significantly lower in patients with residue post CRT (0.56 ± 0.11 versus 0.79 ± 0.13; p < 0.001). When initial ADC was dichotomized at the median, initial ADC lower than 0.7 was significantly more frequent in patients with residue post CRT (73.7% versus 11.1%, p < 0.0001). In multivariate analysis, only initial ADC lower than 0.7 was significantly associated with tumor residue (OR = 22.6; IC [4.9-103.6], p < 0.0001). Among 26 patients without tumor residue after CRT and followed up until 12 months, 6 (23.1%) presented recurrence or progression. Only univariate analysis was performed due to a small number of events. The only factor significantly associated with disease progression or early recurrence was the delta ADC (p = 0.0009). When ADC variation was dichotomized at the median, patients with ADC variation greater than 0.7 had time of disease-free survival significantly longer than patients with ADC variation lower than 0.7 (377.5 [286-402] days versus 253 [198-370], p < 0.0001). Conclusion and relevance: Diffusion-weighted MRI could be a technique that enables differentiation of patients with high potential for early recurrence for whom intensive post-CRT monitoring is mandatory. Prospective studies with more inclusions would be necessary to validate our results.

Unusual Locations of Benign Paroxysmal Positional Vertigo: Rare Entity or Regular Occurrence?

INTRODUCTION: According to the literature and our recent experience, even if patients present with symptoms strongly suggestive of benign paroxysmal positional vertigo (BPPV), the observed positional nystagmus does not always correspond to any previously described typical location. The aim of this multicentric study was to evaluate the frequency of both typical and less common forms of BPPV. MATERIAL AND METHODS: All consecutive patients presenting with BPPV in two hospitals between November 2016 and October 2017 were included. For each subject, answers to a standardized evaluation form and a recorded videonystagmoscopy were obtained by two otorhinolaryngologists. Appropriate diagnostic and therapeutic maneuvers were performed. RESULTS: A total of 532 patients were studied and 491 cases of typical BPPV were found: 370 cases of canalolithiasis of the posterior semicircular canal (SCC), 3 cases of canalolithiasis of the anterior SCC, 107 cases of canalolithiasis of the lateral SCC, and 11 cases of cupulolithiasis of the lateral SCC. Fourty one patients reported symptoms suggestive of BPPV with positional nystagmus that could correspond to unusual locations of otoconia in the SCC: 18 cases of canalolithiasis in the anterior ampulla of the lateral SCC, 16 cases of posterior short arm canalolithiasis, four cases of pseudo-spontaneous nystagmus in canalolithiasis of the lateral SCC, and three cases of canalolithiasis of the posterior third of SCC. DISCUSSION: Unusual BPPV accounted for almost 8% of BPPV treated in our clinics; it is paramount to know how to diagnose them, to carry out the appropriate therapeutic maneuvers and relieve these patients.