Global State of the Art and Science of Childhood Dysphagia: Similarities and Disparities in Burden.

Feeding/swallowing and airway protection are complex functions, essential for survival, and continue to evolve throughout the lifetime. Medical and surgical advances across the globe have improved the long-term survival of medically complex children at the cost of increasing comorbidities, including dysfunctional swallowing (dysphagia). Dysphagia is prominent in children with histories of preterm birth, neurologic and neuromuscular diagnoses, developmental delays, and aerodigestive disorders; and is associated with medical, health, and neurodevelopmental problems; and long-term socioeconomic, caregiver, health system, and social burdens. Despite these survival and population trends, data on global prevalence of childhood dysphagia and associated burdens are limited, and practice variations are common. This article reviews current global population and resource-dependent influences on current trends for children with dysphagia, disparities in the availability and access to specialized multidisciplinary care, and potential impacts on burdens. A patient example will illustrate some questions to be considered and decision-making options in relation to age and development, availability and accessibility to resources, as well as diverse cultures and family values. Precise recognition of feeding/swallowing disorders and follow-up intervention are enhanced by awareness and knowledge of global disparities in resources. Initiatives are needed, which address geographic and economic barriers to providing optimal care to children with dysphagia.

Toward Better Understanding of Pediatric Feeding Disorder: A Proposed Framework for Patient Characterization.

To establish a foundation for methodologically sound research on the epidemiology, assessment, and treatment of pediatric feeding disorder (PFD), a 28-member multidisciplinary panel with equal representation from medicine, nutrition, feeding skill, and psychology from seven national feeding programs convened to develop a case report form (CRF). This process relied upon recent advances in defining PFD, a review of the extant literature, expert consensus regarding best practices, and review of current patient characterization templates at participating institutions. The resultant PFD CRF involves patient characterization in four domains (ie, medical, nutrition, feeding skill, and psychosocial) and identifies the primary features of a feeding disorder based on PFD diagnostic criteria. A corresponding protocol provides guidance for completing the assessment process across the four domains. The PFD CRF promotes a standard procedure to support patient characterization, enhance methodological rigor, and provide a useful clinical tool for providers and researchers working with these disorders.

Assessment Across the Life Span: The Clinical Swallow Evaluation.

Purpose This clinical focus article considers the roles of the clinical swallow examination (CSE) as a clinically meaningful assessment method used in both adult and pediatric populations. Method This clinical focus article explores the utility of the CSE across the life span. Specifically, components, reliability, standardized assessments, and limitations of the CSE within the adult and pediatric populations are highlighted. Conclusions The CSE remains a crucial assessment tool for the speech-language pathologist. The experienced clinician can make important judgments regarding patient safety and function. If the CSE is conducted mindfully and methodically, findings can help chart the course of care for individuals needing additional assessment and possibly intervention.

The Pediatric Subjective Global Nutrition Assessment Classifies More Children With Cerebral Palsy as Malnourished Compared With Anthropometry.

BACKGROUND: Nutrition assessment is multidimensional; however, much of the literature examining the nutritional status of children with cerebral palsy (CP) focuses on a single dimension. OBJECTIVE: The aim of the study was to evaluate nutritional status in children and adolescents with CP by comparing results from the Pediatric Subjective Global Nutrition Assessment (SGNA) with results from traditional anthropometric measures. DESIGN: This study was a cross-sectional observational study. PARTICIPANTS/SETTING: This study was conducted in a tertiary hospital outpatient setting in Brisbane, Australia, from February 2017 to March 2018. A total of 89 children (63 boys) with CP aged between 2 and 18 years of age were included. All Gross Motor Function Classification System levels were observed. The majority of children were in Gross Motor Function Classification System I and II (57, 64%) compared with Gross Motor Function Classification System III to V (32, 36%). Children with feeding tubes and those acutely unwell or hospitalized were excluded. MAIN OUTCOME MEASURES: Children were classified as well nourished, moderately malnourished, or severely malnourished by dietitians using the SGNA. Weight, height, body mass index (BMI), triceps skinfold thickness, subscapular skinfold thickness, and mid upper arm circumference were measured and converted to z scores to account for age and sex differences. Moderate malnutrition was defined by z scores -2.00 to -2.99 and severe malnutrition as ≤-3.00 z scores. STATISTICAL ANALYSIS PERFORMED: Multinomial logistic analyses were used to compare results from the SGNA and each single measurement. Continuous outcomes were transformed into z scores. Agreement was assessed with 2 categories: not malnourished and malnourished. Comparison statistics included percent agreement, sensitivity, and specificity. RESULTS: More children were classified as moderately or severely malnourished by SGNA than any of the anthropometric z score cutoffs. The majority of children were well nourished (n = 63) with 20 (22%) moderately malnourished and 6 (7%) severely malnourished by SGNA. The SGNA classified 11 children as malnourished that were not classified as malnourished by BMI. Children with moderate or severe malnutrition by SGNA had lower weight (P < .001, P < .001), BMI (P < .001, P < .001), mid upper arm circumference (P < .001, P < .001), triceps skinfold thickness (P = .01, P = .007), and subscapular skinfold thickness (P = .005, P = .02) z scores than well-nourished children. CONCLUSION: The SGNA identified more potentially malnourished children including children classified as well nourished by the single measurements such as BMI, height, and weight. The SGNA provided a clinically useful multidimensional approach to nutrition assessment for children with CP.

Development and validation of a screening tool for feeding/swallowing difficulties and undernutrition in children with cerebral palsy.

AIM: To develop and validate a screening tool for feeding/swallowing difficulties and/or undernutrition in children with cerebral palsy (CP). METHOD: This cross-sectional, observational study included 89 children with CP (63 males, 26 females; median age 6y 0mo; interquartile range 4y 0mo-8y 11mo), across all Gross Motor Function Classification System levels. Children with feeding tubes were excluded. Children were classified as well-nourished or moderately to severely undernourished, using the paediatric Subjective Global Nutrition Assessment. Eating and drinking abilities were classified using the Eating and Drinking Ability Classification System (EDACS) from mealtime observation and videofluoroscopic swallow studies when indicated. Parents/caregivers answered 33 screening questions regarding their child's feeding/swallowing abilities and nutritional status. The diagnostic ability of each question for identifying children with feeding/swallowing difficulties and undernutrition was calculated and the combination of questions with the highest sensitivity and specificity identified. RESULTS: Feeding difficulties impacted on swallow safety in 26 children (29%) and 26 children (29%) were moderately or severely undernourished. The 4-item final tool had high sensitivity and specificity for identifying children with feeding/swallowing difficulties (81% and 79% respectively) and undernutrition (72% and 75% respectively). The tool successfully identified 100 per cent of children with severe undernutrition and 100 per cent of those classified as EDACS level IV or V. INTERPRETATION: Screening for feeding/swallowing difficulties and undernutrition will enable early identification, assessment, and management for those children in need. WHAT THIS PAPER ADDS: A screening tool with high sensitivities and specificities for identifying children with feeding/swallowing difficulties and undernutrition. The tool identified 100 per cent of children with severe undernutrition. The tool identified 100 per cent of children in Eating and Drinking Ability Classification System levels IV or V.

Parent-reported indicators for detecting feeding and swallowing difficulties and undernutrition in preschool-aged children with cerebral palsy.

AIM: To determine the most accurate parent-reported indicators for detecting (1) feeding/swallowing difficulties and (2) undernutrition in preschool-aged children with cerebral palsy (CP). METHOD: This was a longitudinal, population-based study, involving 179 children with CP, aged 18 to 60 months (mean 34.1mo [SD 11.9] at entry, 111 males, 68 females [Gross Motor Function Classification System level I, 84; II, 23; III, 28; IV, 18; V, 26], 423 data points). Feeding/swallowing difficulties were determined by the Dysphagia Disorders Survey and 16 signs suggestive of pharyngeal phase impairment. Undernutrition was indicated by height-weight and skinfold composite z-scores less than -2. Primary parent-reported indicators included mealtime duration, mealtime stress, concern about growth, and respiratory problems. Other indicators were derived from a parent feeding questionnaire, including 'significant difficulty eating and drinking'. Data were analysed using multilevel mixed-effects regression and diagnostic statistics. RESULTS: Primary parent-reported indicators associated with feeding/swallowing were 'moderate-severe parent stress' (odds ratio [OR]=3.2 [95% confidence interval {CI} 1.3-7.8]; p<0.01), 'moderate-severe concern regarding growth' (OR=4.5 [95% CI 1.7-11.9]; p<0.01), and 'any respiratory condition' (OR=1.8 [95% CI 1.4-5.8]; p<0.01). The indicator associated with undernutrition was 'moderate-severe concern regarding growth' (height-weight OR=13.5 [95% CI 3.0-61.3]; p<0.01; skinfold OR=19.1 [95% CI 3.7-98.9]; p<0.01). 'Significant difficulty eating and drinking' was most sensitive/specific for feeding outcome (sensitivity=58.6%, specificity=100.0%), and 'parent concern regarding growth' for undernutrition (sensitivity=77.8%, specificity=77.0%). INTERPRETATION: Parent-reported indicators are feasible for detecting feeding and swallowing difficulties and undernutrition in children with CP, but need formal validation. WHAT THIS PAPER ADDS: Parent-reported indicators can detect feeding/swallowing difficulties and undernutrition in children with cerebral palsy. Most accurate screening questions were 0-10 scales for 'difficulty eating' and 'difficulty drinking'. Supplementation of these scales with additional indicators would improve detection.

Instrumental Assessment of Pediatric Dysphagia.

Speech-language pathologists (SLPs) have fulfilled primary roles in the evaluation and management of children with feeding/swallowing disorders for more than five decades. The increased incidence and prevalence of newborns, infants, and children with feeding and swallowing disorders has resulted in increased use of instrumental swallowing evaluations. The videofluoroscopic swallow study and fiberoptic endoscopic evaluation of swallowing are the two most commonly used swallowing assessments by SLPs, with ultrasound used less frequently. This article focuses on updates over the past decade in the procedures and utility of instrumental assessments of swallowing function, and identifies future directions that may enable us to meet the needs of the children who are in our care to attain functional outcomes.

Pediatric Feeding/Swallowing: Yesterday, Today, and Tomorrow.

Speech-language pathologists (SLPs) have played primary roles in the evaluation and management of children with feeding/swallowing disorders for more than five decades. Medical, surgical, and technological advances have improved the survival of young fragile infants and children, many of whom will present with feeding/swallowing problems. Regardless of their underlying etiologies, many of these children are at risk for aspiration-induced lung disease, undernutrition or malnutrition, developmental deficits, and stressful interactions with their caregivers. Unfortunately, our understanding of the physiology/pathophysiology of swallowing and its maturation, the development of standardized and efficacious evaluation and therapy tools, and identification of functional outcomes have not kept pace with our ability to identify children who are at increased risk for dysphagia and the associated sequelae. Given this paucity of evidence to guide practice in pediatrics, clinicians rely upon a combination of data extrapolated from adults with dysphagia, anecdotal reports, and institution-specific guidelines. This article focuses on updates in population demographics and advances in evaluation and treatment over the past decade and identifies future directions that may enable us to meet the needs of the children who are in our care to attain functional outcomes.

Risk of dysphagia and speech and language delay in PHACE syndrome.

PHACE (posterior fossa, hemangioma, arterial lesions, cardiac, and eye) syndrome consists of infantile hemangiomas of the head and neck along with a spectrum of noncutaneous anomalies. Neurodevelopmental abnormalities have also been noted. Here we describe the association between PHACE syndrome and abnormalities in oropharyngeal development and coordination manifesting as dysphagia or speech and language delay. A retrospective chart review was conducted of 34 patients with PHACE syndrome. Data were collected from prior clinical notes and radiographic studies and the results of a comprehensive questionnaire that those who attended the July 2012 PHACE Syndrome Family Conference completed. Seventeen of 34 patients with PHACE syndrome and signs or symptoms of dysphagia or speech or language problems were included for analysis. Nine had dysphagia, seven had a history of cardiac surgery, four had a posterior fossa malformation, and seven had lip or oropharynx hemangiomas. Speech or language delay was noted in 16; posterior fossa abnormalities and lip or oropharynx hemangiomas were the most commonly seen associated finding in this group. There was considerable overlap between subset populations with dysphagia, speech delay, and language delay. A subset of individuals with PHACE syndrome experience dysphagia, speech delay, or language delay. This risk seems to be greater in certain subsets of patients, including those with posterior fossa malformations or lip or oropharynx hemangiomas and those with a history of cardiac surgery. Although this descriptive study was not comprehensive enough to examine prevalence, the high incidence of dysphagia and speech and language delay seen in our cohort warrants future prospective studies to further investigate the association.

Transient swallowing dysfunction in typically developing children following supraglottoplasty for laryngomalacia.

OBJECTIVE: To evaluate early postoperative swallowing function in otherwise typically-developing children following supraglottoplasty. METHODS: Retrospective chart review case series. RESULTS: Of 37 children identified as having undergone supraglottoplasty for severe laryngomalacia at our institution between January 2007 and October 2011, 24 were identified as otherwise typically developing with no indications/signs of swallowing problems pre-operatively and eligible for inclusion in this study. Twenty-two children underwent bilateral supraglottoplasty and 2 children underwent unilateral supraglottoplasty using the CO2 laser or the laryngeal skimmer microdebrider in combination with cold steel technique, based on the discretion of four Pediatric Otolaryngologists. Seventeen children were seen post-operatively for a clinical swallow evaluation. Six children were found to have swallowing dysfunction. Four of the six children showed variable symptoms, signs, or findings concerning for aspiration with oral feeding. Three of six underwent video fluoroscopic swallow study (VFSS). All 6 children responded to dietary modifications, positioning alterations, and anti-reflux medications. All showed improvement by their 4-week post-operative follow-up visit. The median age of the 24 patients at the time of surgery was 3 months, with a range from 2 weeks to 4 years. The median age of the patients found to have transient post-operative swallowing dysfunction was 1.5 months, with a range of 2 weeks to 4 months. There was no association between post-operative swallowing dysfunction and the surgical technique employed. CONCLUSIONS: Supraglottoplasty in otherwise typically developing children carries a risk of transient swallowing dysfunction with a low risk of persistent dysfunction. Most patients can be assessed post-operatively via clinical swallow evaluation without requiring an instrumental swallow study.

Children with central and peripheral neurologic disorders have distinguishable patterns of dysphagia on videofluoroscopic swallow study.

To determine whether findings on videofluoroscopic swallow studies reveal different patterns of dysphagia between children with central and peripheral neurologic disorders, a retrospective study of 118 videofluoroscopic swallow studies was completed. There were 3 groups: cerebral palsy with only spastic features (n = 53), cerebral palsy with dyskinetic features (n = 34), and neuromuscular disorders (myotonic dystrophy I, n = 5; spinal muscular atrophy I-II, n = 8; Duchenne muscular dystrophy, n = 8; other neuromuscular disorder, n = 10). Interpretation of the videofluoroscopic swallow studies was not blinded. The video fluoroscopic swallow study findings were compared dichotomously between the groups. Children with cerebral palsy demonstrated dysphagia in 1 or all phases of swallowing. In neuromuscular disorder, muscle weakness results in pharyngeal residue after swallow. The underlying swallowing problem in neuromuscular disorder is muscle weakness whereas that in cerebral palsy is more complex, having to do with abnormal control of swallowing. This study serves as a first exploration on specific characteristics of swallowing in different neurologic conditions and will help clinicians anticipate what they might expect.

Endoscopic cricopharyngeal myotomy for management of cricopharyngeal achalasia (CA) in an 18-month-old child.

A 6-month-old patient presented with dysphagia and failure to thrive. Video fluoroscopic swallow study (VFSS), esophagogastroduodenoscopy, and manometry were diagnostic for CA. A gastrostomy tube was placed at 8 months. Botulinum toxin injection improved symptoms, but within 10 weeks symptoms returned. At 18 months, an uncomplicated endoscopic CPM was performed. A postoperative VFSS demonstrated cricopharyngeal bar resolution. Within 3 months, patient was feeding orally without a G tube. Pediatric CPA treatment options consist of dilation, botox, and transcervical CPM. To our knowledge, this is the youngest patient treated with endoscopic CPM. Intraoperative video and photographs are presented.

Fiberoptic Endoscopic Evaluation of Swallowing in children: feeding outcomes related to diagnostic groups and endoscopic findings.

OBJECTIVE: Fiberoptic Endoscopic Evaluation of Swallowing (FEES) is used as an adjunct to assess swallowing function in children with complex feeding disorders. We report the feeding outcomes of patients who underwent FEES to determine whether associations exist between clinical diagnoses or FEES findings and feeding outcomes. METHODS: Retrospective review of children who underwent FEES for dysphagia or aspiration from 2003 to 2009. The clinical diagnoses and initial FEES findings were compared to follow up feeding status for associations. RESULTS: 79 patients were included (44 males and 35 females). The change from initial to final status: total oral feeding (42-67%), NPO ± minimal tastes (39-21%) and oral feeding with tube feeding (19-12%). Of the clinical diagnoses, tonsillar hypertrophy was associated with ultimately obtaining total oral feeding status (p = 0.046) while the inability to obtain total oral feeding status was associated with neurologic (p < 0.001). The initial FEES findings showed no significant associations with long-term feeding status. CONCLUSION: Many children overcome their dysphagia but those with neurologic disorders are less likely to achieve total oral feeding status. In children with dysphagia evaluated by FEES, the long-term feeding status is not significantly associated with the initial FEES findings.

Preliminary temporal measurement analysis of normal oropharyngeal swallowing in infants and young children.

Temporal measures of normal pediatric oropharyngeal deglutition have not been studied. Knowledge of range and variation of normative temporal measures could define abnormal deglutition and assist in design of appropriate compensatory and rehabilitative treatment techniques. The purpose of this retrospective study was to determine temporal measurements for oral filling, oral transit, onset of laryngeal closure, time of bolus arrival at the valleculae, pharyngeal delay, pharyngeal transit, and UES opening. Videofluoroscopic swallow studies of 15 normally swallowing pediatric subjects were divided into three age groups and method of liquid delivery. Mean, standard deviation, percentages, and extension of the median were utilized to determine relationships of temporal measures. Mean temporal duration increased with age for oral filling, oral transit time, time of laryngeal closure, UES opening, and pharyngeal delay time. However, no significant differences were found between age groups indicating a deglutitive biomechanical adaptation to growth of the oral and pharyngeal cavity. Feeding method for bottle versus cup mean duration increased for oral transit time, laryngeal closure time, UES opening, and pharyngeal delay time. Bolus head location relative to onset of laryngeal vestibule closure changed with increased age and method of feeding. Temporal measures were not significantly different for age groups or feeding methods. Bolus location was at or fully contained in the valleculae at the onset of laryngeal closure and appeared to be a normal finding in functional pediatric swallows and is not indicative of a delay or disorder.

Evidence-based systematic review: effects of oral motor interventions on feeding and swallowing in preterm infants.

PURPOSE: To conduct an evidence-based systematic review and provide an estimate of the effects of oral motor interventions (OMIs) on feeding/swallowing outcomes (both physiological and functional) and pulmonary health in preterm infants. METHOD: A systematic search of the literature published from 1960 to 2007 was conducted. Articles meeting the selection criteria were appraised by 2 reviewers and vetted by a 3rd for methodological quality. RESULTS: Twelve studies were included and focused on 3 OMIs-nonnutritive sucking (NNS), oral/perioral stimulation, and NNS plus oral/perioral stimulation. Six studies addressed the effects of OMI on the feeding/swallowing physiology outcomes of feeding efficiency or sucking pressures. Ten studies addressed the functional feeding/swallowing outcomes of oral feeding or weight gain/growth. No studies reported data on pulmonary health. Methodological quality varied greatly. NNS alone and with oral/perioral stimulation showed strong positive findings for improvement in some feeding/swallowing physiology variables and for reducing transition time to oral feeding. Prefeeding stimulation showed equivocal results across the targeted outcomes. None of the OMIs provided consistent positive results on weight gain/growth. CONCLUSIONS: Although some OMIs show promise for enhancing feeding/swallowing in preterm infants, methodological limitations and variations in results across studies warrant careful consideration of their clinical use.

The effects of oral-motor exercises on swallowing in children: an evidence-based systematic review.

AIM: The aim of this unregistered evidence-based systematic review was to determine the state and quality of evidence on the effects of oral motor exercises (OME) on swallowing physiology, pulmonary health, functional swallowing outcomes, and drooling management in children with swallowing disorders. METHOD: A systematic search of 20 electronic databases was completed to identify relevant peer-reviewed literature published in English between 1960 and 2007. Experimental or quasi-experimental design studies examining OME as a treatment for children with swallowing disorders were appraised for methodological quality by two assessors and reviewed by a third. RESULTS: Sixteen studies of varying methodological quality were included. No study examining the effects of OME on pulmonary health in children was identified. The included studies incorporated a wide variety of OME, and mixed findings were noted across all of the outcomes targeted in this review. INTERPRETATION: Based on the results of this evidence-based systematic review, there is insufficient evidence to determine the effects of OME on children with oral sensorimotor deficits and swallowing problems. Well-designed studies are needed to provide clinicians with evidence that can be incorporated into the preferences of the client and the clinicians' knowledge of anatomy, physiology, and neurodevelopment in the management of this group of children.

Evidence-based systematic review: effects of neuromuscular electrical stimulation on swallowing and neural activation.

PURPOSE: To systematically review the literature examining the effects of neuromuscular electrical stimulation (NMES) on swallowing and neural activation. The review was conducted as part of a series examining the effects of oral motor exercises (OMEs) on speech, swallowing, and neural activation. METHOD: A systematic search was conducted to identify relevant studies published in peer-reviewed journals from 1960 to 2007. All studies meeting the exclusion/inclusion criteria were appraised for quality and categorized as efficacy or exploratory research based on predetermined criteria. RESULTS: Out of 899 citations initially identified for the broad review of OMEs, 14 articles relating to NMES qualified for inclusion. Most of the studies (10/14) were considered exploratory research, and many had significant methodological limitations. CONCLUSIONS: This systematic review reveals that surface NMES to the neck has been most extensively studied with promising findings, yet high-quality controlled trials are needed to provide evidence of efficacy. Surface NMES to the palate, faucial pillars, and pharynx has been explored in Phase I research, but no evidence of efficacy is currently available. Intramuscular NMES has been investigated in a single Phase I exploratory study. Additional research is needed to document the effects of such protocols on swallowing performance.