Association of bifid epiglottis and laryngeal web with Bardet-Biedl syndrome: A case report.

Bardet-Biedl syndrome (BBS) is a rare autosomal-recessive disease characterized by rod-cone dystrophy, obesity, postaxial polydactyly, cognitive impairment, hypogonadism and renal abnormalities. Bifid epiglottis and anterior laryngeal web are rare congenital anomalies and are often constituent of polymalformation syndromes. We report a case of a 9-month-old patient initially referred in otolaryngology (ENT) for dysphonia and recurrent respiratory infections. Physical exam and fiberoptic nasopharyngolaryngoscopy showed bifid epiglottis and laryngeal web associated with BBS. Those laryngeals anomalies may be underdiagnosed in BBS and this case supports the importance of upper airway evaluation by an ENT team, especially with respiratory symptoms or dysphagia.

Desmoid-type fibromatosis of the head and neck in children: A changing situation.

INTRODUCTION: Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies. METHODS: This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. All medical files were reviewed and their outcomes analyzed according to the initial therapies provided. RESULTS: Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XIth cranial nerve sacrifice or sensory impairment. CONCLUSION: HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

Information quality and dynamics of patients' interactions on tonsillectomy web resources.

Information technologies have drastically altered the way patients gather health-related information. By analysing web resources on tonsillectomy, we expose information quality and dynamics of patients' interactions in the online continuum. Readability was assessed using Flesch Reading Ease (FRE), Flesch Kincaid Grade Level (FKGL), Simple Measure of Gobbledygook (SMOG), and Gunning Fog Index (GFI). Comprehensibility and actionability were assessed using the Patient Education Materials Assessment Tool (PEMAT). Metrics of forums included author characteristics (level of disclosure, gender, age, avatar image, etc.), posts' motive (community support vs. medical information) and content (word count, emoticon use, number of replies, etc.). Analysis of 6 professional medical websites, of 10 health information portals, and of 3 discussion forums totalizing 1369 posts on 358 threads, from January 1, 2007 to December 31, 2014, reveals that online resources exceed understandability recommendations. Women were more present on online health forums (68.2% of authors disclosing their gender) and invested themselves more in their avatar. Authors replying were significantly older than authors of original posts (39.7 ± 0.8 years vs. 29.2 ± 0.9 years, p < 0.001). The degree of self-disclosure was inversely proportional to the requests for medical information (p < 0.001). Men and women were equally seeking medical information (men: 74.0%, women: 77.0%) and community support (men: 65.7%, women: 70.4%), however women responded more supportively (women 86.2%, men 59.1%, p < 0.001). The dynamics of patients' interactions used to overcome accessibility difficulties encountered is complex. This work outlines the necessity for comprehensible medical information to adequately answer patients' needs.

Value of radiofrequency ablation in the management of retropharyngeal lymphatic malformation.

Lymphatic malformations are benign malformations frequently occurring in the head and neck. Retropharyngeal location is rare, can be life threating and its management is particularly challenging. Over a three-year period, three patients presented with symptomatic (dyspnea and/or dysphagia) retropharyngeal lymphatic malformation. All were treated using a radiofrequency ablation of lymphatic malformation through a trans-oral approach. No major complications occurred following the surgery. During the follow-up, no recurrence was noted and all patients were asymptomatic. Radiofrequency ablation in the management of retropharyngeal lymphatic malformations is a simple technique with very good results and allows a fast recovery with minimum morbidity and a short hospital stay.

Recurrent dacryostenosis as initial presentation of sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease with an unknown etiology. It most commonly affects young and middle-aged females. It can affect any organ, but mostly lung, skin, and eyes. Up to half of patients are asymptomatic and the disease is often detected incidentally on abnormal chest radiography. We report the case of a 31-year-old male with bilateral recurrent dacryostenosis. The nasolacrimal obstruction was the initial manifestation of systemic sarcoidosis.