Πέμπτη 25 Μαρτίου 2021

Hereditäre hämorrhagische Teleangiektasie: Symptome und diagnostische Latenz

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Laryngorhinootologie
DOI: 10.1055/a-1408-5160

Hintergrund Patienten mit hereditärer hämorrhagischer Teleangiektasie (HHT) leiden unter einer systemischen Erkrankung des Gefäßbindegewebes, bei der eine Vielzahl verschiedener Symptome auftritt. Material und Methoden Die Daten aller Patienten, die sich von April 2014 bis August 2019 im Westdeutschen Morbus-Osler-Zentrum vorstellten, wurden in einer retrospektiven Studie analysiert. Ergebnisse Bei 235 Patienten konnte die Diagnose HHT als definitiv (235/282; 83 %) und bei 26 als möglich gestellt werden (26/282; 9 %). Die mittlere diagnostische Latenz zwischen Erstsymptomen und Diagnose betrug 18 Jahre. Direkte oder indirekte Blutungszeichen wurden oft als erste Symptome der Erkrankung HHT genannt (224/241; 93 %). 83 % der Patienten mit einem Grad der Behinderung gaben HHT als Hauptursache an. Insbesondere ältere, weibliche Patienten bzw. Patienten mit starker Epistaxis litten an einer chronischen Eisenmangelanämie (Eisensubstitution:148/261; 57 %; Erythrozytenkonzentrate: Mittelwert: 9 ± Standardabweichung: 41, Minimum – Maximum: 0–400, Anzahl der Patienten: 218). 10 % erhielten eine Thrombozytenaggregationshemmung oder Antikoagulation und tolerierten diese. 74 % der Patienten mit HHT pflegten ihre Nasenschleimhaut (177/238) und zeigten weniger Blutungen als Patienten ohne Nasenpflege (ESS: t-Test: 3,193; p = 0,003; Anämi e: Chi-Quadrat: 5,173; p = 0,023). Schlussfolgerungen Die Diagnoselatenz der Erkrankung HHT betrug knapp 2 Jahrzehnte. Patienten mit HHT leiden insbesondere an rezidivierenden Blutungen, die dabei meistindizierte Behandlung der ersten Wahl ist eine konsequente Nasenpflege und verschiedene koagulative Therapieoptionen. Bei Begleiterkrankungen mit Indikation zur Gerinnungshemmung lohnt es sich meist, deren Einsatz zu versuchen.
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Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

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Entwicklung der Publikationsleistung der Universitätskliniken für Hals-Nasen-Ohrenheilkunde, Kopf- und Hals-Chirurgie während der SARS-CoV-2-Pandemie im Jahr 2020 in Deutschland

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Laryngorhinootologie
DOI: 10.1055/a-1430-7735

Einleitung Die SARS-CoV-2-Pandemie hat an den Universitätskliniken für Hals-Nasen-Ohrenheilkunde (HNO) zu tiefgreifenden Einschränkungen im Bereich der Krankenversorgung und der studentischen Lehre geführt. Die Auswirkungen auf den Bereich Forschung sind dagegen uneinheitlich. Zur Einordnung der pandemiebedingten Effekte auf die Forschung wurde die Entwicklung der Anzahl der wissenschaftlichen Publikationen der HNO-Universitätskliniken in Deutschland vor und während der Pandemie analysiert. Material und Methoden Es wurde die Publikationsleistung aus den Jahren 2015–2020 der derzeitigen 39 Klinikdirektoren mithilfe einer Literaturrecherche (PubMed) erhoben. Eingeschlossen wurden alle Nennungen der Klinikdirektoren als Erst-, Letzt- oder Co-Autor einer Arbeit. Es wurde die absolute und relative Entwicklung der Publikationsleistung jedes Autors ermittelt und statistisch ausgewertet. Ergebnisse Die Literaturrecherche ergab 2420 Publikationen. Zwischen 2015 und 2019 wurden pro Jahr durchschnittlich 368 Publikationen von allen Autoren veröffentlicht. 2020 stieg diese Zahl um 57,9 % auf 581 Publikationen an. Während zwischen 2015 und 2019 die Anzahl der monatlichen Publikationen konstant blieb, zeigte sich ab Mai 2020 ein deutlicher Anstieg bis zu einem Maximum von 74 Veröffentlichungen im September 2020. 34 Arbeiten (5,9 %) aus dem Jahr 2020 wiesen einen thematischen Bezug zur SARS-CoV-2-Pandemie auf, wobei 7 dieser Arbeiten (20,6 %) durch standortübergreifende Veröffentlichungen entstanden sind. Schlussfolgerung Im Jahr 2020 konnte die Zahl der wissenschaftlichen Publikationen auf mehr als das 1,5-fache der Veröffentlichungen der Vorjahre gesteigert werden. Diese Steigerung stand in einem deutlichen zeitlichen Zusammenhang zur Reduktion der elektiven Krankenversorgung während der SARS-CoV-2-Pandemie ab Mitte März 2020. Wahrscheinlich haben freie zeitliche Kapazitäten diese gesteigerte Publikationsleistung ermöglicht. Unsere Ergebnisse belegen das große wissenschaftliche Potenzial der universitären HNO-Kliniken, das trotz der Pandemie erfolgreich umgesetzt werden konnte.
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Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

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Implications of limited exolaryngeal disease and cricoarytenoid joint involvement in organ conservation protocols for laryngopharyngeal cancers: Results from a prospective study

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Abstract

Background

To identify clinicoradiological factors that determine functional outcomes in laryngopharyngeal cancers treated with chemoradiotherapy.

Methods

One hundred patients of locally advanced laryngopharyngeal cancers who were treated with chemoradiotherapy were accrued in this prospective study. The coprimary endpoint of the study was local control (LC) and functional larynx preservation survival (FLPS).

Results

The median follow‐up was 39 months. Thirty‐nine patients had a local failure of which 17 underwent a salvage laryngectomy. A dysfunctional larynx with clinic‐radiologically disease was seen in only 1 patient. Factors significant for LC were thyroid cartilage erosion/lysis and cricoarytenoid joint involvement. Within the T4a subset, patients with exolaryngeal disease through the soft tissue framework had significantly better LC and FLPS than those with cartilage erosion/lysis.

Conclusions

Patients with limited exolaryngeal disease through the soft‐tissue framework can be considered for functional organ preservation, while those with thyroid cartilage involvement and cricoarytenoid joint involvement are not suitable.

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Selective embolization of lingual artery in transoral robotic surgery for the management of recurrent base of tongue carcinomas

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Abstract

Background

The incidence of oropharyngeal carcinoma has been on the rise in recent decades. About 30% of patients who undergo definitive chemoradiation as the initial treatment present with residual/recurrent disease. In such a situation, surgical salvage either in the form of traditional open surgery or transoral robotic surgery (TORS) remains a viable treatment option. However, the extensive vascular supply of the posterior tongue and tonsillar bed increases the risk of perioperative bleeding, which is a key concern. The article describes the technique of selective pre‐operative embolization to reduce the risk of perioperative bleeding and enumerate its advantages in providing a bloodless field during surgery.

Methods

Prospective study of 5 patients with recurrent or residual midline BOT tumours who underwent TORS after selective lingual artery embolization at our centre.

Results and Conclusions

None of the patients had any major perioperative bleeding or post procedural complications. All the patients after TORS had their tongue vascularity preserved with adequate recovery of tongue functions. Selective embolization of the feeder vessels provides a favorable bloodless surgical field without affecting the vascular integrity of the remnant tongue. This added advantage helps restoring the normal oral phase of swallowing.

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Surgical rehabilitation of swallowing with polydimethylsiloxane injections after open partial horizontal laryngectomy: Long‐term functional results and quality of life

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Abstract

Background

Swallowing disorders are common problems after partial laryngectomy. The aim of this study is to illustrate the long‐term functional results of rehabilitation of swallowing by polydimethylsiloxane (PDMS) injection.

Methods

Twenty‐eight patients with dysphagia after partial laryngectomy who underwent injection of PDMS for rehabilitation of swallowing were included in the study. Impairment of swallowing and quality of life (QoL) were investigated with questionnaires: M. D. Anderson Dysphagia Inventory (MDADI) and Performance Status Scale for Head and Neck Cancer (PSS‐HNC). Functional results at fiberoptic endoscopic evaluation of swallowing (FEES) were analyzed using a modified penetration‐aspiration scale.

Results

Mean follow‐up was 8.5 years. Twenty‐six patients showed an improvement at questionnaires (p < 0.001). Median improvement was 6 (p < 0.001) in the modified penetration‐aspiration scale. Total laryngectomy was required in one patient.

Conclusions

PDMS injection is a good option for rehabilitation of swallowing in case of dysphagia after partial laryngectomy. It improves QoL and the results persist after a long follow‐up period.

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Muscle invasion in oropharyngeal carcinoma undergoing transoral robotic surgery

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Abstract

Backgrounds

Pathologic features of oropharyngeal squamous cell carcinoma (OPSCC) treated with trans‐oral robotic surgery predict prognosis and adjuvant therapy. We hypothesized that pathologic muscle invasion (pMI) is associated with poor pathological markers.

Methods

Retrospective review of surgically treated OPSCC to identify pMI and its association with poor pathologic markers.

Results

pMI was present in 12/37 patients, and compared to non‐pMI, was associated with higher rates of lymphovascular invasion (75% vs. 36%, p = 0.03), perineural invasion (16.7% vs. 0%, p = 0.04), extranodal extension (66.7% vs. 20%, p < 0.01), and tumor stage (8.3% vs. 48% pT1, 75% vs. 52% pT2 and 16.7% vs. 0% pT3). pMI was associated with having a positive margin on main specimen (41.7% vs. 12%, p = 0.04) but not after considering additional margins.

Conclusions

Muscle invasion was associated with higher pathologic tumor staging, poor pathologic factors, and higher rates of positive margin on main specimen.

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Increased rate of recurrence and high rate of salvage in patients with human papillomavirus–associated oropharyngeal squamous cell carcinoma with adverse features treated with primary surgery without recommended adjuvant therapy

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Abstract

Background

Some patients with human papillomavirus (HPV)–associated oropharyngeal squamous cell carcinoma (OPSCC) do not receive guideline‐recommended postoperative radiation therapy (PORT) following primary transoral robotic surgery (TORS).

Methods

Three‐hundred and sixty‐four patients with treatment‐naïve, HPV‐associated OPSCC were recommended to receive PORT based on clinicopathological features following TORS. Patients were stratified based on if they received PORT. Oncologic outcomes were compared.

Results

The 3‐year locoregional failure (LRF) was 32% in patients who did not receive PORT and 4% in patients who received PORT (P < .001). Despite increased LRF, avoiding PORT was not associated with increased 3‐year distant metastasis rates (8% vs 4%, P = .56) or worse 3‐year survival (95% vs 98%, P = .34). Recurrences in the surgery alone cohort varied between local and regional sites and were often successfully salvaged.

Conclusions

Patients with HPV‐associated OPSCC who do not receive indicated PORT have an increased risk of LRF but similar survival due to high salvage rates.

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Response Evaluation Criteria in Solid Tumors (RECIST) and PET Response Criteria in Solid Tumors (PERCIST) for response evaluation of the neck after chemoradiotherapy in head and neck squamous cell carcinoma

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Abstract

Background

An optimal approach to imaging assessment of neck after chemoradiotherapy must be established to avoid unnecessary neck dissection.

Methods

We retrospectively examined 101 patients and compared between Response evaluation criteria in solid tumors (RECIST), PET response criteria in solid tumors (PERCIST), and positron emission tomography/computed tomography (PET/CT) qualitative assessment.

Results

PERCIST was superior to RECIST in positive predictive value (PPV; 47% vs. 36%), with equivalent negative predictive value (NPV; 78%). Only 3 of 15 patients with incomplete responses on either RECIST or PERCIST alone had regional treatment failure, and the combination of RECIST and PERCIST improved PPV (55%) without reducing NPV. This combination yielded the highest hazard ratio of regional treatment failure. The combination of RECIST and PET/CT qualitative assessment also improved PPV (50%). In human papillomavirus (HPV)–related oropharyngeal cancer, NPV was 100% across the imaging assessments, while PPV was poor (14%–33%).

Conclusions

Combining RECIST and PERCIST might optimize decision making in neck management after chemoradiotherapy. HPV status would affect the accuracy of response evaluation.

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Benign connective and soft‐tissue neoplasms of the oral and maxillofacial region: Cross‐sectional study of 1066 histopathological specimens

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Abstract

Background

Benign connective and soft‐tissue neoplasms (CSTNs) are unusual in the head and neck region. The aim of the present study was to evaluate the demographic and clinicopathological features of these neoplasms.

Methods

A cross‐sectional study was conducted of cases diagnosed as benign CSTNs, with data collected from biopsy records. The chi‐square test was used. A p‐value <0.05 was considered indicative of statistical significance.

Results

Among the 38 119 specimens, 1066 (2.79%) were benign CSTNs: 369 fibroblastic/myofibroblastic, 250 adipocytic, 179 vascular, 130 neural, 94 osseous/cartilaginous, 19 muscular, and two fibrohistiocytic. Most patients were female (62.8%) and white‐skinned (45.8%). Mean age was 42 years. The tongue (25.2%) was the most affected site for extraosseous neoplasms.

Conclusion

This study had the largest sample of benign oral and maxillofacial CSTNs. Although these tumors have similar clinical features, the characterization and differentiation detailed here may help clinicians with regards to the correct diagnosis.

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Speech and language characteristics in Smith–Magenis syndrome: Case report

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Message:

Smith-Magenis syndrome (SMS) is a developmental disorder that affects many parts of the body. The major features of this condition include mild to moderate intellectual disability , delayed speech and language skills, distinctive facial features, sleep disturbances, and behavioral problems.

Smith-Magenis syndrome | Genetic and Rare Diseases ...

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Publication date: Available online 24 March 2021

Source: Revista de Logopedia, Foniatría y Audiología

Author(s): Elena Garayzábal Heinze, Irene Hidalgo de la Guía, Kriscia Gobi Rosa, Célia Maria Giacheti, Natalia Freitas Rossi

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Incorporating Virtual Reality to Improve Otolaryngology Resident Wellness: One Institution's Experience

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Objective

Burnout is defined as work‐related emotional exhaustion, depersonalization, and decreased sense of accomplishment. Virtual reality (VR) has emerged as an effective treatment modality for various conditions related to anxiety, however, few studies have assessed its role for stress management in residents. We hypothesize that VR‐based mindfulness meditation can reduce resident burnout in real‐world settings.

Study Design

Prospective randomized crossover trial.

Methods

Resident participants completed the validated Maslach Burnout Inventory (MBI). One group used a VR‐based meditation app; the second group received no intervention. After a 2‐month rotation, all subjects completed an MBI and crossed over to the other arm. Wilcoxon rank‐sum tests were used to compare MBI scores before and after intervention, and to compare results by gender and postgraduate year. Mann‐Whitney U tests were used to assess qualitative differences between participants.

Results

Eighteen residents completed the study. Five participants were female and 13 were male. Weekly use of VR‐guided meditation and paced breathing was associated with a significant decrease in emotional exhaustion (P = .009), and on subgroup analysis, male gender specifically was associated with a decrease in emotional exhaustion (P = .027). In the post‐intervention survey, 42.9% subjects reported that VR encouraged them to employ paced breathing techniques, 71.4% reported that they would use the technology if regularly available, and 21.4% reported they would use paced breathing in the future.

Conclusion

VR‐based therapy may serve as a successful tool in stress management and reduce the rate of burnout among otolaryngology residents.

Level of Evidence

N/A Laryngoscope, 2021

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Obstructive Sleep Apnea in Children Under 3 Years of Age

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Objective

To identify predictors of severe obstructive sleep apnea (OSA) in children under 3 years of age and to compare demographics, comorbidities, and polysomnographic characteristics of infants and toddlers with OSA.

Study Design

Retrospective case series

Methods

We examined children under 3 years of age who had polysomnogram between August 2012 and March 2020. Demographics, clinical, and polysomnographic parameters were compared in children age 0–1 versus 1–3 years and 0–2 versus 2–3 years and severe versus mild–moderate OSA. Univariate analysis was used to compare age groups; multiple logistic regression for predictors of severe OSA. Significance was set at P < .05.

Results

Of the 413 children, 267 (65%) were male and 131 (32%) obese. The population included Hispanic (41%), African American (28%), and Caucasian (25%) children. A total of 98.5% had OSA and 35% had severe OSA. Children under 1 year of age more commonly had gastroesophageal reflux disease (GERD) (38% vs. 23%; P = .014); tonsillar hypertrophy was more common in children over 2 years of age (56% vs. 34%, P = .001). Down syndrome (odds ratio (OR): 3.16, 95% confidence interval (CI) = 1.14–8.68, P = .026) and tonsillar hypertrophy (OR: 1.97, 95% CI = 1.28–3.02, P = .002) were predictors of severe OSA.

Conclusion

Children under 3 years of age with OSA are more likely to be male and have GERD. Down syndrome and tonsillar hypertrophy are predictors of severe OSA, and children with these conditions should be prioritized for polysomnography.

Level of Evidence

4. Laryngoscope, 2021

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