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Infant Dysphagia: Contributions from Atypical Anatomy KYLA O’BOYLE, MS, CCC-SLP MERCY ONE DES MOINES MEDICAL CENTER MARCH 28,2019 Introduction & Presentation Overview Anatomy and Physiology Severity and interventions Clinical Presentations Case Studies QUESIONS Babies are Just Different }Normal Swallow Aspiration in Infants Infants that aspirate do so SILENTLY Aspiration not typically correlated with physiologic instability Bolus flow in aspiration in infants vs adults Response to aspiration Laryngomalacia Overview Congenital condition Present in 45-75% of pediatric patients with stridor Hallmark is inhalator stridor Onset Shortly after birth with average age presentation 2 weeks (Olney et al 1999) Progression Typically worsens over first 6 months with gradual improvement Resolution Most cases are symptom free by 18-24 months (Thompson et al 2007; Holinger et al 1967) Diagnosis via Flexible Fiberoptic Laryngoscopy Common Findings Inspiratory prolapse of the arytenoids Redundant arytenoid mucosa Shortened aryepiglottic folds Omega shaped and/or elongated/tubular epiglottis

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3/26/2019

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Infant Dysphagia: Contributions from Atypical Anatomy

KYLA O’BOYLE, MS, CCC-SLP

MERCY ONE DES MOINES MEDICAL CENTER MARCH 28,2019

Introduction & Presentation Overview

Anatomy and Physiology

Severity and interventions

Clinical Presentations

Case Studies

QUESIONS

Babies are Just Different

}Normal Swallow

Aspiration in InfantsInfants that aspirate do so SILENTLY

Aspiration not typically correlated with physiologic instability

Bolus flow in aspiration in infants vs adults

Response to aspiration

Laryngomalacia OverviewCongenital condition

Present in 45-75% of pediatric patients with stridor

Hallmark is inhalator stridor

Onset◦ Shortly after birth with average age presentation 2 weeks (Olney et al 1999)

Progression◦ Typically worsens over first 6 months with gradual improvement

Resolution◦ Most cases are symptom free by 18-24 months (Thompson et al 2007; Holinger et al 1967)

Diagnosis via Flexible Fiberoptic LaryngoscopyCommon Findings

◦ Inspiratory prolapse of the arytenoids◦ Redundant arytenoid mucosa◦ Shortened aryepiglottic folds ◦ Omega shaped and/or elongated/tubular epiglottis

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LarynogmalaciaLaryngomalacia Video

EtiologyMultifactorial◦Anatomic ◦Inflammatory ◦Neurological

Typing Laryngomalacia Olney et al 1999

Image Source: https://sites.uclouvain.be/anesthweekly/MRP_ENG/index.html?Laryngomalacie

Severity Ratings Per Thompson et al 2017Mild Laryngomalacia

◦ Inspiratory stridor◦ With or without cough during feeding

Moderate Laryngomalacia◦ Inspiratory stridor◦ Choking or gasping during feeds, frequent regurgitation, brief apneas or cyanotic episodes OR

intermittent dyspnea with retractions (did not require interventions)

Severe Laryngomalacia◦ Stridor◦ Life threatening issues: failure to thrive, apneas, cyanosis or dyspnea requiring interventions, pectus

excavatum, pulmonary HTN, or cor pulmonale

Surgical InterventionsOnly indicated in about 10-15% of cases with average age at surgery 3-5 months

Trachestomy

Suprglotoplasty based on type of laryngomalacia Supragolotoplasty Video◦ CO2 Laser vs cold instrumentation ◦ Epiglottopexy◦ Generally successful

◦ 10% occurrence of complications including aspiration

Bedwell 2016

Dysphagia Incidence: Simons et al 2016Retrospective cohort study with 324 patients with laryngomalaciaAbnormal swallow function confirmed with CSE/MBSS and/or FEES in 80% of patients with dysphagia symptomsAbnormal swallow function found in two-thirds of laryngomalacia patients with no subjective dysphagia

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Aspiration in InfantsInfants that aspirate do so SILENTLY

Aspiration not typically correlated with physiologic instability

Bolus flow in aspiration in infants vs adults

Response to aspiration

Case Studies in LaryngomalaciaBirth history

Clinical Swallow Evaluation

Complicating factors

MBSS Findings

MBSS8 MBSS1

Outcomes

Feeding InterventionsP R E - F E ED I N G S K I L L D E VE LO P M EN T

I N I T I AT I N G A ND A D VA N C I N G P O I N TA K E

N I P P L E S EL EC T I O N

P O S I T I O N I N G

E X T ER N A L PA C I N G

T HI C K E NI NG

Standard InterventionsProactive Interventions:

◦ Pre-feeding skill development◦ Gentle initiation of oral feeds

Reactive Interventions◦ Positioning◦ Flow Rate Selection or modification◦ Feeding strategies/External Pacing◦ Thickening

Thickening for InfantsDysphagia and/or Reflux

AAP and FDA positions◦ Digestive immaturity, malabsorption, NEC

No standards◦ Thickener used, Recipes, Viscosities Used, Measuring, Mixing, dwell time and warming thickened feeds

◦ Starch-based Thickeners◦ Commercial Thickeners◦ Rice cereal

Madhoun et al. (2015) Neonatal Nursing Reviews

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Rice CerealMacros shift from protein and fat to carbs which may lead to excessive weight gain (Horvath et at., 2008)

Introduction of solids prior to 4 months◦ Associated with obesity (six fold increase) at age 3, (Grummer-Strawn et al, 2008)◦ Decreased duration of breastfeeding (Huh et al., 2011)◦ Increased risk of diabetes, eczema, and celiac disease (Norris et al., 2005; Tarini et al., 2006)

Constipation (Mascarenhas et al., 2005)

Additional iron and inorganic Arsenic (Juskelis et al., 2013; McCallum, 2011)

Unreliable for thickening breastmilk

International Dysphagia Diet Standardization Initiative (IDDSI)Goals of IDDSI

◦ Establish and international standard for fluids and modified foods

Development of Framework

ASHA Endorsement◦ Recently announced◦ 2 year transition plan

Transitional Key

4 Extremely Thick: Pudding or Spoon Thick

3 Moderately Thick: Honey Thick Liquids

2 Mildly Thick: Nectar Thick Liquids

1 Slightly Thick: “half strength nectar”

0 Thin Liquids Thin Liquids

IDDSI Flow Test

https://youtu.be/BhfJWu1ybbs (procedure instructions)

Reflux Formulas for Dysphagia?Line Spread Study

◦ Enfamil AR approached statistically significant differences in viscosity vs standard formulas other reflux formulas (Mayfield et al 2014)

Sheer rates are dramatically different for common formulas ◦ Enfamil AR 20 kcal at 50 mPa s: 76◦ Similac Sensitive for Fussiness and Gas (20 kcal) at 50 mPa s: 6 (Frazier et al 2016)

Clinical Findings at MMCUse of Enfamil AR 24 kcal formula

IDDSI Syringe Testing ◦ Warmed 3-4 ◦ Cold 7

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Changes in MBSS Practices

Viscosity Matched Barium◦ Slightly thick formulas◦ Patients with added rice cereal for reflux◦ Use for pediatric MBSImP?

Possible Implications for Adult Patients◦ Health drinks◦ Patient and family education/empowerment

Barium and Infant FormulaLiquid E-Z-Paque (60% concentration) - NECTAR

E-Z-Paque Powder (60% concentration) - Thin

E-Z Paque Powder (20% concentration) - Thin

Enfamil AR 20 kcal - NECTAR

Enfamil AR 20 kcal + E-Z-Paque Powder (20%) - Thin!

Enfamil AR 24 kcal - Thin

Enfamil AR 24 kcal + E-Z-Paque Powder (20%) - NECTAR!

Similac Sensitive for Gass and Fussiness - Thin

Frazier et al (2016) Dysphgia

Conclusions45-75% of infants with stridor have laryngomalacia

Most infants with identified laryngomalacia have some abnormal swallow function

Current typing or severity ratings are not predictive of the presence or absence of dysphagia or aspiration

Highlights need for CSL as well as instrumental evaluations for children with suspected or confirmed laryngomalacia

ReferencesAls H, Duffy FH, McNulty GB, Rivkin RJ, Vajapeyam S, Mulkern RV, et al. Early experience alters brain function and structure. Pediatrics. 2004;113:846-857.

Al-Sayed LE, Schrank WI, Thach BT. Ventilatory sparing strategies and swallowing pattern during bottle feeding in human infants. J Appl. Physiol. 77(1): 78-83, 1994.

American Academy of Pediatrics Committee on Fetus and Newborn. Hospital discharge of the high risk neonate. Pediatrics. 2008;122:1119-1126.

Arvedson J, Rogers B, Buck G, Smart P, Msall M. Silent aspiration prominent in children with dysphagia. International Journal of Pediatric Otorhinolaryngology. 1994; 28: 173-181.

Bedwell J, Zalzal, G. Laryngomalacia. Seminars in Pediatric Surgery. 2016; 25: 119-122.

Bullock K, Mayfield E, Gould C, Woods L, Walters J. (2013) Viscosity Measurements of Fortified Breast Milk. 2013 ASHA Convention.

Frazier J, Chestnut AH, Jackson A, Barbon CEA, Steele CM, Pickler L. Understanding the viscosity of liquids used in infant dysphagia management. Dysphagia 2016; 31:672-679.

Goldfield EC, Smith V, Buonomo C, Perez J, Larson K. Preterm infant swallowing of thin and nectar thick liquids: Changes in lingual-palatal coordination and relation to bolus transit. Dysphagia. 2013; 28:234-244.

Grummer-Strawn LM, Scanlon KS, Fein SB. Infant feeding and feeding transition during the first year of life. Pediatrics. 2008; 122(Suppl, 2):S36-S42.

Holinger L. Etiology of Stridor in the neonate, infant and child. Ann Otol Rhinol Laryngol. 1980 98:397-400.

References cont.Horvath A, Dziechciraz P, Szajewska H. The effect of thickened-feed interventions of garstroesophageal reflux in infants; systematic review and meta-analysis of randomized, controlled trials. Pediatrics. 2008; 122:e1268-e1277.

Huh SY, Rifas-Shiman SL, Taveras EM, Oken E, Gillman MW. Timing of solid food introduction and risk of obesity in preschool-aged children. Pediatrics. 2011; 127:e544-e511.

Juskelis R, Li W, Nelson J, Cappozzo JC. Arsenic speciation in rice cereals for infants. J. Agric. Food Chem. 2013; 61:10670-10676.

Madhoun LL, Siler-Wurst KK, Sitaram S, Jadcherla SR. Feed-thickening practices in NICUs in the current era: Variability in prescription and implementation patterns. Journal of Neonatal Nursing, 2015 December 1; 21(6): 255-262.

MascarenhasR, Landry L, Khoshoo V. Difficulty in defecation in infants with gastroesophageal reflux treated with smaller volume feeds thickened with rice cereal. Clinical Pediatrics, 2005; 44:671-673.

Mayfield E, Woods L, Gould C, Walters J, Bullock K. (2014) Viscosity Measurements of Fortified Infant Formulas. 2014 ASHA Convention.

McCallum S. Addressing nutrient density in the context of the use of thickened liquids in dysphagia treatment. ICAN: Infant Child Adolesc. Nutr. 2011; 3:351-360.

Munson PD, Saad AG, El=Jamal SM, et al. Submucosal nerve hypertrophy in congenital laryngomalacia. Laryngoscope. 2011:121;627-629.

Norris JM, Barriga K, Hoffenberg EJ, Taki I, Miao DM, Haas JE, Emery LM, Sokol RJ, Erlich HA, Eisenbarth GS, Rewers M. Risk of celiac disease autoimmunity and timing of gluten introduction in the diet of infants at increased risk of disease. JAMA. 2005; 293:1713-1720.

Olney DR, Greinwald JH Jr. Smith RJ. Et al. Laryngomalacia and its treatment. Laryngoscope. 1999;109:1770-1775.

Orenstein SR, Maghill HL, Brooks P. Thickening of infant feedings for therapy of gstroesophageal reflux. J. Pediatr. 1987; 110:181-186.

Simons JP, Greenberg LL, Mehta DK, et al. Laryngomalacia and swallowing function in children. Laryngoscope. 2015; 7:478-484.

Tarini BA, Carroll AE, Sox CM, Christakis DA. Systematic review of the relationship between early introduction of solid foods to infants and the development of allergic disease. Arch. Pediat. Adol. Med. 2006; 160:502-507.

Questions?

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