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Introduction: The recent POLDER trial investigated the effects of external beam radiotherapy (EBRT) on dysphagia caused by incurable oesophageal cancer. An estimated life expectancy of minimally three months was required for inclusion. However, nearly one-third of the included patients died within three months. The aim of this study was to investigate if the use of prediction models could have improved the physician's estimation of the patient's survival., Methods: Data from the POLDER trial ( N  = 110) were linked to the Netherlands Cancer Registry to retrieve patient, tumour, and treatment characteristics. Two published prediction models (the SOURCE model and Steyerberg model) were used to predict three-month survival for all patients included in the POLDER trial. Predicted survival probabilities were dichotomised and the accuracy, sensitivity, specificity, and the area under the curve (AUC) were used to evaluate the predictive performance., Results: The SOURCE and Steyerberg model had an accuracy of 79% and 64%, and an AUC of 0.76 and 0.60 ( p = .017), respectively. The SOURCE model had higher specificity across survival cut-off probabilities, the Steyerberg model had a higher sensitivity beyond the survival probability cut-off of 0.7. Using optimal cut-off probabilities, SOURCE would have wrongfully included 16/110 patients into the POLDER and Steyerberg 34/110., Conclusion: The SOURCE model was found to be a more useful decision aid than the Steyerberg model. Results showed that the SOURCE model could be used for three-month survival predictions for patients that are considered for palliative treatment of dysphagia caused by oesophageal cancer in addition to clinicians' judgement.

Patients with squamous cell carcinoma of the oropharynx are generally treated with (chemo) radiation. Patients with oropharyngeal cancer have better survival than patients with squamous cell carcinoma of other head and neck subsites, especially when related to human papillomavirus. However, radiotherapy results in a substantial percentage of survivors suffering from significant treatment-related side-effects. Late radiation-induced side-effects are mostly irreversible and may even be progressive, and particularly xerostomia and dysphagia affect health-related quality of life. As the risk of radiation-induced side-effects highly depends on dose to healthy normal tissues, prevention of radiation-induced xerostomia and dysphagia and subsequent improvement of health-relatedquality of life can be obtained by applying proton therapy, which offers the opportunity to reduce the dose to both the salivary glands and anatomic structures involved in swallowing.This review describes the results of the first cohort studies demonstrating that proton therapy results in lower dose levels in multiple organs at risk, which translates into reduced acute toxicity ( i.e. up to 3 months after radiotherapy), while preserving tumour control. Next to reducing mucositis, tube feeding, xerostomia and distortion of the sense of taste, protons can improve general well-being by decreasing fatigue and nausea. Proton therapy results in decreased rates of tube feeding dependency and severe weight loss up to 1 year after radiotherapy, and may decrease the risk of radionecrosis of the mandible. Also, the model-based approach for selecting patients for proton therapy in the Netherlands is described in this review and future perspectives are discussed.

Background: Dysphagia and dysarthria are frequently described in pediatric neuromuscular diseases (pNMD). The consequences can be substantial: failure to thrive, malnutrition, aspiration pneumonia, or communication problems. Early detection and identification of risk factors and etiology support preventing complications and morbidity, including impact on quality of life. Information about the prevalence of dysphagia and dysarthria in pNMD is scarce., Objective: To describe the pooled prevalence of dysphagia and dysarthria in pNMD in the Netherlands. In addition, we describe the prevalence of dysphagia and dysarthria each, and the prevalence of chewing (oral) and swallowing problems per diagnostic group, based on their anatomic origin., Methods: Data were collected from 295 children (mean age 11;0 years, range 2;6-18;0) with pNMD in 12 hospitals and rehabilitation centers in the Netherlands. A speech language therapist established whether dysphagia and dysarthria were present or not., Results: In almost all the 14 diagnostic groups of pNMD, dysphagia and dysarthria were present. Pooled overall prevalence of dysphagia and dysarthria was 47.2% and 31.5%, respectively. Of 114 children with dysphagia, 90.0% had chewing problems, 43.0% showed swallowing problems and 33.3% showed both chewing and swallowing problems., Conclusions: The overall pooled prevalence of dysphagia and dysarthria was high in the population of pNMD. It can be argued that periodic monitoring of dysphagia and dysarthria and early referral to a speech language therapist should be a necessity from the start of the diagnosis in the whole pNMD population.

Objective: The impact of difficulties with eating and drinking in adolescents and young adults with cerebral palsy is unknown. The purpose of this study is to find out which difficulties adolescents and young adults with cerebral palsy experience with eating and drinking in daily life and how they deal with these difficulties. The study also explores how they think about themselves with respect to eating and what does or does not help regarding social participation. Method: We collected the data from ten participants with spastic cerebral palsy (aged 15-23 years) living in the Netherlands. We used a qualitative study design with a conventional content analysis. Semi-structured in-depth interviews were used to identify meaningful factors related to eating and drinking difficulties. We coded relevant phrases from each interview and clustered and synthesized them into categories. Result: We derived four categories from the transcripts: (I) perceived eating and drinking difficulties (e.g., not managing to eat all food textures and/or choking); (II) challenges in physical and social context (e.g., accessibility of restaurants, menu supply, and/or needing assistance or not); (III) dealing with eating and drinking difficulties (e.g., adaptation, food avoidance, and/or giving up); (IV) Negative feelings about their eating and drinking (e.g., shame, frustration, fear for choking, and/or concerns about the future). One striking finding was that most participants had not recently received either monitoring or intervention for their feeding skills. Conclusion: This study shows that adolescents and young adults with cerebral palsy experience many restrictions in eating and drinking situations leading to negative feelings and lower participation levels, while little attention is directed towards these difficulties. Regular multidisciplinary rehabilitation programs should include evaluation, advice, and intervention regarding eating and drinking ability in order to increase social participation and self-management. Implications for Rehabilitation Adolescents and young adults with cerebral palsy experience difficulties with eating, drinking, and swallowing, and they encounter difficulties in participating in mealtimes with family and friends. Although adolescents and young adults with cerebral palsy rated their eating and drinking abilities as reasonable, they reported feelings of shame, frustration, fear, distress, and concerns for the future or unknown environments. Regular multidisciplinary involvement with eating and drinking is needed for purposes of evaluation, advice, and intervention throughout the life course, adjusted to living conditions and the latest evidence. Adolescents and young adults with cerebral palsy showed limited initiative in asking for personal assistance in eating and drinking activities.

Purpose: Although office-based transnasal esophagoscopy has been investigated extensively, a cost analysis is still lacking. We performed a cost analysis combined with feasibility study for two diagnostic processes: patients with globus pharyngeus and/or dysphagia, and hypopharyngeal carcinoma., Methods: Prospective cohort study., Results: Forty-one procedures were performed, of which 35 were fully completed. The procedure was well tolerated with mild complaints such as nasal or pharyngeal pain and burping. Four complications occurred: two minor epistaxis and two vasovagal reactions. In patients with globus pharyngeus and/or dysphagia, transnasal esophagoscopy resulted in a cost saving of €94.43 (p 0.026) per procedure, compared to our regular diagnostic process. In patients with suspicion of hypopharyngeal carcinoma, cost savings were €831.41 (p 0.000) per case., Conclusions: Cost analysis showed that office-based transnasal esophagoscopy can provide significant cost savings for the current standard of care. Furthermore, this procedure resulted in good patient acceptability and few complications.

Background: Self-expandable metal stents (SEMSs) are effective for improving dysphagia in patients with incurable esophageal cancer but are also associated with recurrent dysphagia and adverse events. In the past decades, new SEMSs have been introduced, but also patients' risk profiles have altered. It is unknown if these changes have affected SEMS outcomes., Methods: This retrospective cohort study was conducted in a tertiary referral center in the Netherlands. Patients who underwent palliative esophageal SEMS placement for malignant dysphagia between 1994 and 2017 were included. The primary outcome was to assess shifts over time with respect to recurrent dysphagia and adverse events after SEMS placement., Results: 997 patients who underwent SEMS placement were included. Recurrent dysphagia occurred in 309 patients (31 %) and remained stable, although with a trend towards an increase over time (hazard ratio [HR] 1.02 per 1-year increase; P  = 0.05). Migration rate significantly increased over time (HR 1.04 per 1-year increase; P  = 0.01). SEMS-related complications occurred in 461 patients (46.2 %), with 207 (20.7 %) major and 336 (33.7 %) minor complications. Prior chemoradiotherapy was significantly associated with major complications (HR 1.69; P  < 0.001). Pain was the most common adverse event and showed a significant increase over time ( P  < 0.01). Factors associated with pain were prior chemoradiotherapy, absence of a fistula, axial and radial forces, and squamous cell carcinoma., Conclusions: Despite the introduction of novel esophageal SEMS designs, recurrent dysphagia has not declined over the years. Stent-related complications have increased in recent years, which seems to be mainly associated with more frequent use of chemoradiotherapy prior to SEMS placement., Competing Interests: Prof. Bruno has received personal fees as lecturer and consultant. Reports having received institutional financial support for industry and investigator initiated studies from Boston Scientific, Cook Medical, Pentax Medical and 3M. Prof. Siersema has received research support from Ella-CS, Boston Scientific USA and Cook Medical Ireland. Dr. Spaander has received institutional financial support for investigator initiated studies from Boston Scientific. The remaining authors have no competing interests to report., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Eosinophilic esophagitis (EoE) is a chronic esophageal inflammation that may lead to stricture formation. This narrowing can cause major complications including food impactions. Despite increasing interest in EoE accurate data on its natural course is scarce. Therefore, we aimed to investigate the natural course of EoE and to evaluate the association between undiagnosed disease and the occurrence of complications over two decades in a large cohort., Methods: We retrospectively analyzed charts of patients diagnosed with EoE between 1996 and 2015, collected from 15 hospitals throughout the Netherlands. Histologic, clinical, and endoscopic characteristics were identified and stratified by age and diagnostic delay., Results: We included 721 patients (524 males, 117 children (≤18 years)). Dysphagia and food impactions were more common in adults whereas children more often presented with vomiting and abdominal pain (all p < 0.001). The prevalence of fibrotic endoscopic features was higher in adults (76%) than in children (39%) (p < 0.001). As time with undiagnosed disease progressed the percentage of patients with strictures and food impactions increased from 19% and 24% (diagnostic delay ≤ 2 years) to 52% and 57% (diagnostic delay ≥ 21 years) (p < 0.001), respectively. In a multivariate logistic regression model, diagnostic delay (odds ratio (OR) = 1.09; 95% confidence interval (CI) = 1.05-1.13) and male gender (OR = 2.69, 95% CI = 1.61-4.50) were the major risk factors for stricture presence., Conclusion: During the natural course of EoE, progression from an inflammatory to a fibrostenotic phenotype occurs. With each additional year of undiagnosed EoE the risk of stricture presence increases with 9%.

Purpose: To assess the interrater reliability, construct validity and usability of the Eating and Drinking Ability Classification System (EDACS) among Dutch children with Cerebral Palsy (CP) when used by speech and language therapists (SLTs) familiar and unfamiliar with the child's eating and drinking performance and parents., Methods: Translation was undertaken using the method of Eremenco. Agreement between SLTs and parents when using EDACS was determined by intraclass correlation coefficient (ICC) and linear weighted Kappa (κW). Associations with other functional classification systems including the Dysphagia Management Staging Scale (DMSS) were investigated to determine construct validity by Kendall's tau-b., Results: Thirty-one SLTs classified 149 children (67 girls; mean 10 y, SD 4 y, range 3-21 y) with EDACS. Pairs of SLTs showed good agreement ([ICC] = 0.84; 95% confidence interval [CI] 0.79-0.88; [κW] = 0.71). Eighty-one parents showed good agreement with SLTs (n= 31) as well (ICC = 0.80; 95% CI 0.71-0.87; κW= 0.61). There was a significant and strong positive correlation of EDACS with DMSS (Kendall's tau-b 0.81) supporting its construct validity. Usability of EDACS was generally good., Conclusion: The Dutch version of EDACS is reliable and valid, and can be used easily by (familiar and unfamiliar) SLTs and parents of children with CP. Parents and professionals showed a high level of consistency when classifying eating and drinking abilities. EDACS enables uniform and efficient communication about safety and efficiency of functional eating and drinking ability in clinical and research contexts.

Objective: In total laryngectomy, the neopharynx can be closed in several ways. It is suggested that a pseudo-diverticulum is seen more frequently in patients closed with vertical closure than with "T"-shaped closure, causing postoperative dysphagia. We report the results of patients treated with vertical closure and "T"-shaped closure with regard to the formation of a pseudo-diverticulum and postoperative dysphagia., Methods: In our retrospective cohort study, we identified 117 consecutive laryngectomized patients treated in the VU University Medical Center of Amsterdam between March 2009 and December 2013. Evaluations with statistical analysis of postoperative outcome measures (the formation of a pseudo-diverticulum and dysphagia), qualitative and quantitative variables were conducted., Results: Patient demographics were similar between the vertical-shaped closure and the "T"-shaped closure groups. In 84.6% of patients with vertical closure, a pseudo-diverticulum was seen compared to 18.5% with "T"-shaped closure (p < 0.001). Dysphagia was increasingly seen in patients with a pseudo-diverticulum (60.5%) compared to patients without a pseudo-diverticulum (39.5%) (p = 0.090)., Conclusion: Formation of a pseudo-diverticulum is more frequently seen in laryngectomy patients closed with vertical closure than in patients closed with "T"-shaped closure of the neopharynx. It is favorable to implement "T"-shaped closure in laryngectomy.

Purpose: Patients with a neuromuscular disease (NMD) can present with dysarthria and/or dysphagia. Literature regarding prevalence rates of dysarthria and dysphagia is scarce. The purpose of this study was to determine prevalence rates, severity and co-presence of dysarthria and dysphagia in adult patients with NMD., Method: Two groups of adult patients with NMD were included: 102 consecutive outpatients (the "unselected cohort") and 118 consecutive patients who were referred for multidisciplinary assessment (the "selected cohort"). An experienced speech-language pathologist examined each patient in detail., Results: The pooled prevalence of dysarthria was 46% (95% CI: 36.5-55.9) and 62% (95% CI: 53.3-70.8) in the unselected and selected cohorts, respectively. The pooled prevalence of dysphagia was 36% (95% CI: 27.1-45.7) and 58% (95% CI: 49.4-67.2) in the unselected and selected cohorts, respectively. There was a modest but significant association between the presence of dysarthria and dysphagia (rs = 0.40; p < 0.01). Although the dysphagia was generally mild, dysarthria was moderate to severe in 15% of the dysarthric patients., Conclusion: The prevalence rates of dysarthria and dysphagia among patients with various types of NMD are high. Physicians should therefore be aware of this prevalence and consider referring NMD patients to a speech-language pathologist. IMPLICATONS OF REHABILITATION: Both dysarthria and dysphagia are highly prevalent among patients with neuromuscular diseases; moreover, although often mild, these disorders can occur relatively early in the course of the disease. Clinicians should routinely check for signs and symptoms related to dysarthria and/or dysphagia in patients who present with a neuromuscular disease, preferably using standardised instruments.

Background: In anterior cervical spine surgery a retractor is obligatory to approach the spine. Previous studies showed an increase of endotracheal tube cuff pressure after placement of a retractor. It is known that high endotracheal tube cuff pressure increases the incidence of postoperative dysphagia, hoarseness, and sore throat. However, until now no evidence supports the fact whether adjusting the endotracheal tube cuff pressure during anterior cervical spine surgery will prevent this comorbidity. We present the design of a randomized controlled trial to determine whether adjusting endotracheal tube cuff pressure after placement of a retractor during anterior cervical spine surgery will prevent postoperative dysphagia., Methods/design: 177 patients (aged 18-90 years) scheduled for anterior cervical spine surgery on 1 or more levels will be included. After intubation, endotracheal tube cuff pressure is manually inflated to 20 mm Hg in all patients. Patients will be randomized into two groups. In the control group endotracheal tube cuff pressure is not adjusted after retractor placement. In the intervention group endotracheal tube cuff pressure after retractor placement is maintained at 20 mm Hg and air is withdrawn when cuff pressure exceeds 20 mm Hg. Endotracheal tube cuff pressure is measured after intubation, before and after placement and removal of the retractor. Again air is inflated if cuff pressure sets below 20 mmHg after removal of the retractor. The primary outcome measure is postoperative dysphagia. Other outcome measures are postoperative hoarseness, postoperative sore throat, degree of dysphagia, length of hospital stay, and pneumonia. The study is a single centre double blind randomized trial in which patients and research nurses will be kept blinded for the allocated treatment during the follow-up period of 2 months., Discussion: Postoperative dysphagia occurs frequently after anterior cervical spine surgery. This may be related to high endotracheal tube cuff pressure. Whether adaptation and maintaining the pressure after placement of the retractor will decrease the incidence of dysphagia, has to be determined by this trial., Trial Registration: Netherlands Trial Register (NTR) 3542: http://www.trialregister.nl.

Purpose: To report the acute toxicity profile and compliance from a randomized Phase III trial comparing accelerated radiotherapy (AR) with accelerated radiotherapy plus carbogen and nicotinamide (ARCON) in laryngeal cancer., Methods and Materials: From April 2001 to February 2008, 345 patients with cT2-4 squamous cell laryngeal cancer were randomized to AR (n = 174) and ARCON (n = 171). Acute toxicity was scored weekly until Week 8 and every 2-4 weeks thereafter. Compliance to carbogen and nicotinamide was reported., Results: Between both treatment arms (AR vs. ARCON) no statistically significant difference was observed for incidence of acute skin reactions (moist desquamation: 56% vs. 58%, p = 0.80), acute mucosal reactions (confluent mucositis: 79% vs. 85%, p = 0.14), and symptoms related to acute mucositis (severe pain on swallowing: 53% vs. 58%, p = 0.37; nasogastric tube feeding: 28% vs. 28%, p = 0.98; narcotic medicines required: 58% vs. 58%, p = 0.97). There was a statistically significant difference in median duration of confluent mucositis in favor of AR (2.0 vs 3.0 weeks, p = 0.01). There was full compliance with carbogen breathing and nicotinamide in 86% and 80% of the patients, with discontinuation in 6% and 12%, respectively. Adjustment of antiemesis prophylaxis was needed in 42% of patients., Conclusion: With the exception of a slight increase in median duration of acute confluent mucositis, the present data reveal a similar acute toxicity profile between both regimens and a good compliance with ARCON for clinical stage T2-4 laryngeal cancers. Treatment outcome and late morbidity will determine the real therapeutic benefit., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Quality of life is an important outcome measurement in objectifying the current health status or therapy effects in patients with oropharyngeal dysphagia. In this study, the validity and reliability of the Dutch version of the Deglutition Handicap Index (DHI) and the MD Anderson Dysphagia Inventory (MDADI) have been determined for oncological patients with oropharyngeal dysphagia. At Maastricht University Medical Center, 76 consecutive patients were selected and asked to fill in three questionnaires on quality of life related to oropharyngeal dysphagia (the SWAL-QOL, the MDADI, and the DHI) as well as a simple one-item visual analog Dysphagia Severity Scale. None of the quality-of-life questionnaires showed any floor or ceiling effect. The test-retest reliability of the MDADI and the Dysphagia Severity Scale proved to be good. The test-retest reliability of the DHI could not be determined because of insufficient data, but the intraclass correlation coefficients were rather high. The internal consistency proved to be good. However, confirmatory factor analysis could not distinguish the underlying constructs as defined by the subscales per questionnaire. When assessing criterion validity, both the MDADI and the DHI showed satisfactory associations with the SWAL-QOL (reference or gold standard) after having removed the less relevant subscales of the SWAL-QOL. In conclusion, when assessing the validity and reliability of the Dutch version of the DHI or the MDADI, not all psychometric properties have been adequately met. In general, because of difficulties in the interpretation of study results when using questionnaires lacking sufficient psychometric quality, it is recommended that researchers strive to use questionnaires with the most optimal psychometric properties.

The phenotype of DM2 shows similarities as well as differences to that of Myotonic Dystrophy type 1 (DM1). Gastrointestinal dysfunction is common in DM1 and 25% of the patients consider this to be the most disabling consequence of the disease. Little is known about gastrointestinal involvement in Myotonic Dystrophy type 2 (DM2). The aim of the study was to explore the occurrence and characteristics of gastrointestinal symptoms in patients with DM2. This was compared to symptoms in adult-onset DM1 patients, and to age- and sex-matched healthy controls. Twenty-nine genetically proven DM2 patients filled out two standardized questionnaires about gastrointestinal symptoms; most important outcome measures were answers to questions about dysphagia, abdominal pain, and constipation. The results were compared to those of 29 adult-onset DM1 patients, and to 87 age- and sex-matched healthy controls. Radiological measurement of colon transit time was investigated in 18 DM2 patients. Dysphagia for liquids (38%) and solid food (41%), abdominal pain (62%), and constipation (62%) were all significantly more common among DM2 patients than among healthy controls, and comparable to their occurrence in DM1. Colon transit time was increased in 24% of the DM2 patients. Our results show that gastrointestinal symptoms are highly prevalent in DM2 patients. Gastrointestinal dysfunction may be attributed to any part of the gastrointestinal tract. The results provide new insight into the clinical picture of DM2.

Parkinson's disease is a slowly progressive and irreversible disorder of the nervous system. Drooling is listed as a secondary symptom of Parkinson's disease. Its cause is insufficiently clear. In the literature 2 possible causes are described: hypersalivation and swallowing abnormalities. These parameters have not been measured in a single study before. This article presents a review of the literature on the subject and describes the design for a future clinical study. The aim of this clinical study is to gain an insight into the cause and the prevalence of drooling in Parkinson's patients. A group of 50 Parkinson's patients will be compared to a group of 50 controls. The objectives of this comparative study comprise the assessment of salivary flow volumes, swallowing capacity, subjective experiences with drooling and an objective observation of drooling, the drooling quotient. Finally, the possibilities for treatment will be described.

Dysphagia is a common problem in older patients and is becoming a larger health care problem as the populations of the United States and other developed countries rapidly age. Changes in physiology with aging are seen in the upper esophageal sphincter and pharyngeal region in both symptomatic and asymptomatic older individuals. Age related changes in the esophageal body and lower esophageal sphincter are more difficult to identify, while esophageal sensation certainly is blunted with age. Stroke, Parkinson's disease, amyotrophic lateral sclerosis, Zenker's diverticula, and several other motility and structural disorders may cause oropharyngeal dysphagia in an older patient. Esophageal dysphagia can also be caused by both disorders of motility (achalasia, diffuse esophageal spasm, scleroderma and others) and structure (malignancy, strictures, rings, external compression, and others). Many of these disorders have an increased prevalence in older patients and should be sought with an appropriate diagnostic evaluation in older patients. The treatment of dysphagia in older patients is similar to that in younger patients, but more invasive therapies such as surgery may not be possible in some older patients making less aggressive medical and endoscopic therapy more attractive.

Quality of Life (QL) is hard to assess and seldom measured in patients having carcinomas with an unfavourable prognosis. Oesophageal cancer is one of the malignancies with a low 5-year survival rate. Dysphagia (problems in swallowing food) is considered to be the most important indicator of QL in patients with oesophageal carcinoma. Moreover, the psycho-social aspects and subjective QL in cancer have recently gained importance. The present study investigated QL in a 132 patients with oesophageal cancer. Eighty-three of them had a surgical operation (removal of part of the oesophagus and part of the stomach, followed by a reconstruction of the digestive tract). Sixty-seven patients filled in questionnaires before and after the operation. Complete sets of data were obtained from 62 patients. Time interval between operation and postoperative assessment varied from 3 to 7 months. Indicators of QL were: Psychological Distress, Physical Symptoms, Global Evaluations, Activity Level, Swallowing Problems and Food Intake. Swallowing Problems showed moderate correlations with the other QL indicators. Physical Symptoms increased, whereas the Activity Level, Psychological Distress, and Swallowing Problems decreased; Global Evaluations remained unaltered.

An outbreak of botulism in four yearlings of a single herd is reported. One animal recovered spontaneously. The other animals underwent euthanasia (animal A) or were slaughtered (animals B and C). Cl. botulinum toxin type C was detected in the sera of animals A and B as well as in the liver and abomasal contents of animal A. In addition, Cl. botulinum was isolated from the liver of animal A. The symptoms are described. Total muscular weakness, also resulting in lingual paralysis and inability to swallow, was the most important clinical feature. The mechanism of action of Cl. botulinum toxin, possible methods of treatment and aspects of meat inspection are discussed.