Pediatric Dysphagia: Complex Case Studies of Patients with ...

Pediatric Dysphagia: Pediatric Dysphagia: Complex Case Studies of Complex Case Studies of

Patients with Patients with Aerodigestive Disorders Aerodigestive Disorders

(2421)(2421)Jennifer Perez, M.S., CCC-SLPJennifer Perez, M.S., CCC-SLP

Children’s Hospital Boston, Boston, MAChildren’s Hospital Boston, Boston, MAAlana Lowry, M.S., CCC-SLPAlana Lowry, M.S., CCC-SLP

Fletcher Allen Healthcare, Burlington, VTFletcher Allen Healthcare, Burlington, VTKara Fletcher Larson, M.S., CCC-SLPKara Fletcher Larson, M.S., CCC-SLPSouth Shore Hospital, Weymouth, MASouth Shore Hospital, Weymouth, MA

Perez, Lowry, Fletcher Larson ASHA 2008

3 SLP’s = 1 Vision3 SLP’s = 1 Vision

All have worked and trained extensively All have worked and trained extensively at Children’s Hospital Bostonat Children’s Hospital Boston

Clinical specialty in pediatric dysphagiaClinical specialty in pediatric dysphagia High volume of pediatric High volume of pediatric

videofluoroscopyvideofluoroscopy Level 3 NICU Level 3 NICU Exposure to wide range of Exposure to wide range of

medical/surgical pediatric diagnosesmedical/surgical pediatric diagnoses Working within the division of ORLWorking within the division of ORL


Children’s Hospital BostonChildren’s Hospital Boston

Acute, urban pediatric Acute, urban pediatric hospitalhospital

Associated with Harvard Associated with Harvard Medical SchoolMedical School

397 bed comprehensive 397 bed comprehensive pediatric healthcare centerpediatric healthcare center Medical/Surgical ICUMedical/Surgical ICU Cardiac ICUCardiac ICU Neonatal ICU (Level III)Neonatal ICU (Level III)

204 specialized clinical 204 specialized clinical programsprograms


Aero-digestive DisordersAero-digestive Disorders

Congenital Anomalies of LarynxCongenital Anomalies of Larynx

Upper Airway Obstruction Upper Airway Obstruction

Esophageal Obstructive DisordersEsophageal Obstructive Disorders

Center for Center for Aero-Digestive Aero-Digestive

DisordersDisorders(CADD)(CADD)


Center for Aero-Digestive DisordersCenter for Aero-Digestive Disorders Multi-disciplinary clinic including pediatric specialists from:Multi-disciplinary clinic including pediatric specialists from:

Otolaryngology (ORL)Otolaryngology (ORL) Gastroenterology Gastroenterology PulmonaryPulmonary SurgerySurgery RadiologyRadiology Speech-Language PathologySpeech-Language Pathology

Pediatric Dysphagia specialistsPediatric Dysphagia specialists Voice specialistsVoice specialists

NeurologyNeurology GeneticsGenetics


Center for Aero-Digestive DisordersCenter for Aero-Digestive Disorders

Providing comprehensive services to infants and Providing comprehensive services to infants and children with complex problems involving the:children with complex problems involving the:

AirwayAirway Pulmonary tractPulmonary tract Upper digestive tract Upper digestive tract



Frequently treated medical conditions:Frequently treated medical conditions: AsthmaAsthma Congenital anomalies of the esophagus and tracheaCongenital anomalies of the esophagus and trachea CroupCroup Gastroesophageal refluxGastroesophageal reflux Iatrogenic or acquired tracheal stenosisIatrogenic or acquired tracheal stenosis Laryngeal cleftLaryngeal cleft LaryngomalaciaLaryngomalacia Lymphatic malformationLymphatic malformation PneumoniaPneumonia StridorStridor Subglottic stenosisSubglottic stenosis Tracheoesophageal fistula/esophageal atresiaTracheoesophageal fistula/esophageal atresia



Logistics—How the CADD works:Logistics—How the CADD works: Once per week, patients see ORL, GI, Pulmonary in clinicOnce per week, patients see ORL, GI, Pulmonary in clinic

Speech-Pathology available for consultation Speech-Pathology available for consultation

Following clinic, team members meet to discuss and Following clinic, team members meet to discuss and collaborate on findings and determine follow-up plancollaborate on findings and determine follow-up plan

Once per month, CADD conference of various disciplines Once per month, CADD conference of various disciplines for review of most complex patients/casesfor review of most complex patients/cases


Importance of the speech-language pathologist Importance of the speech-language pathologist on the Aero-Digestive teamon the Aero-Digestive team

Clinical screening for feeding & swallowing safetyClinical screening for feeding & swallowing safety

Objective assessment of swallow function pre- Objective assessment of swallow function pre- and/or post-surgical interventionand/or post-surgical intervention

Comprehensive feeding evaluation/therapy for Comprehensive feeding evaluation/therapy for sensory-based feeding issues, oral motor delays sensory-based feeding issues, oral motor delays secondary to complex medical/surgical historysecondary to complex medical/surgical history


Center for Aero-Digestive DisordersCenter for Aero-Digestive Disorders Criteria for referral for objective swallowing evaluation:Criteria for referral for objective swallowing evaluation:

Coughing, choking or sputtering (during or after feeds)Coughing, choking or sputtering (during or after feeds)

Wet/junky/congested breath sounds (during or after feeds)Wet/junky/congested breath sounds (during or after feeds)

Chronic wheezing or stridorChronic wheezing or stridor

Chronic pneumonia, croup, bronchitisChronic pneumonia, croup, bronchitis

Oxygen desaturation/cyanosis (during or after feeds)Oxygen desaturation/cyanosis (during or after feeds)

Apnea/bradycardia, etc. with feedsApnea/bradycardia, etc. with feeds

Color change with feedsColor change with feeds

Chronic fever of unclear etiologyChronic fever of unclear etiology


Objective Assessment of Swallow FunctionObjective Assessment of Swallow FunctionFEES vs. VFSSFEES vs. VFSS

FEES FEES Fiberoptic Endoscopic Fiberoptic Endoscopic

Evaluation of SwallowingEvaluation of Swallowing

No radiation exposureNo radiation exposure Invasive-Difficulty maintaining Invasive-Difficulty maintaining

cooperation in children cooperation in children 12 mo - 4 yrs 12 mo - 4 yrs

Does not provide information Does not provide information regarding interaction of oral, regarding interaction of oral, pharyngeal and esophageal phases of pharyngeal and esophageal phases of swallowing swallowing

Can assess breastfeedingCan assess breastfeeding Can view secretionsCan view secretions Equipment is portableEquipment is portable Can assess VPI and vocal cord issuesCan assess VPI and vocal cord issues

VFSSVFSSVideofluoroscopic Swallow StudyVideofluoroscopic Swallow Study(Modified Barium Swallow Study)(Modified Barium Swallow Study)

Exposure to radiation Exposure to radiation Non-invasiveNon-invasive Dynamic assessment of oral, Dynamic assessment of oral,

pharyngeal, and esophageal phases of pharyngeal, and esophageal phases of swallowingswallowing

Cannot assess breastfeedingCannot assess breastfeeding Readily available in most medical Readily available in most medical

centerscenters Gold standard assessment of Gold standard assessment of

swallowing physiologyswallowing physiology

Adapted from: Arvedson & Lefton-Greif, 1998


Management of Dysphagia Management of Dysphagia Establish safe oral feeding plan :Establish safe oral feeding plan :

? Need for non-oral supplementation? Need for non-oral supplementation

Diet modificationDiet modification

Modify delivery methodModify delivery method

Limit volume/length of timeLimit volume/length of time

Determine safest positioningDetermine safest positioning

Counseling familiesCounseling families

**Collaboration with other medical providers in CADD is essential for decision-making and **Collaboration with other medical providers in CADD is essential for decision-making and establishing these dietary changes**establishing these dietary changes**


Congenital Anomalies of the Larynx:Congenital Anomalies of the Larynx:

Laryngeal CleftLaryngeal Cleft


Laryngeal CleftLaryngeal Cleft

Definition:Definition:

Communication between the posterior larynx and Communication between the posterior larynx and esophagusesophagus

Failure of the tracheoesophageal septum to Failure of the tracheoesophageal septum to developdevelop


Case Study # 1:Case Study # 1:

-5 week old baby boy-5 week old baby boy-Clinical symptoms: -Clinical symptoms:

-coughing while drinking thin liquids-coughing while drinking thin liquids-pneumonia-pneumonia-losing weight due to poor feeding-losing weight due to poor feeding

-Admitted to pulmonary service at CHB-Admitted to pulmonary service at CHB-NG-tube placed -NG-tube placed

-Referred for VFSS-Referred for VFSS


Case Study # 1 (cont):Case Study # 1 (cont):

--VFSS ResultsVFSS Results::

-silent aspiration with thin liquids-silent aspiration with thin liquids

-consistent deep laryngeal penetration with nectar--consistent deep laryngeal penetration with nectar-thick liquidsthick liquids

-nasopharyngeal reflux-nasopharyngeal reflux--assumed dysphagia due to discoordinated suck-swallow-breathe patternassumed dysphagia due to discoordinated suck-swallow-breathe pattern

-VFSS repeated no improvement x 2 -VFSS repeated no improvement x 2

-G-tube placed at 4 months old-G-tube placed at 4 months old



-At 11 months of age:-At 11 months of age:

-Baby assessed for laryngeal cleft (DL/B)-Baby assessed for laryngeal cleft (DL/B)

-found to have Type 1 laryngeal cleft-found to have Type 1 laryngeal cleft

-Surgically repaired-Surgically repaired

-Repeat VFSS 4 weeks post surgery -Repeat VFSS 4 weeks post surgery

-continued aspiration of thin liquids-continued aspiration of thin liquids



VFSS Clip # 2VFSS Clip # 2Post-SurgeryPost-Surgery




-no aspiration with thin liquids-no aspiration with thin liquids

-Now: Child 3 years old-Now: Child 3 years old

-G-tube removed -G-tube removed

-stable respiratory status-stable respiratory status


Classification of Laryngeal CleftsClassification of Laryngeal Clefts

According to length:According to length:

Type I: interarytenoid onlyType I: interarytenoid only Type II: partial cricoidType II: partial cricoid Type III: complete cricoidType III: complete cricoid Type IV: extending into thoracic tracheaType IV: extending into thoracic trachea


Types of Laryngeal CleftsTypes of Laryngeal Clefts

Types I and IITypes I and II Diagnosis may take months to yearsDiagnosis may take months to years

Types III and IVTypes III and IV Diagnosed on the first day of life due to severityDiagnosed on the first day of life due to severity High morbidity associated with Type IVHigh morbidity associated with Type IV


Classification of Laryngeal cleftsClassification of Laryngeal clefts

Benjamin and Inglis, 1989


Type IType I

PettersonPetterson ArmitageArmitage BenjaminBenjamin

Partial cricoid cleft Partial cricoid cleft extendingextendingbelow the level of below the level of the vocal cordsthe vocal cords

Complete cricoid cleftComplete cricoid cleft

Cleft extending to Cleft extending to cervical tracheacervical trachea

Cleft extending to Cleft extending to thoracic tracheathoracic trachea

Interarytenoid and Interarytenoid and supra glottic cleftsupra glottic cleft Type I AType I A Type IType I

Type I BType I B Type IIType II

Type IType I Type I CType I C Type IIIType III

Type IIType II Type IIType II Type IIIType III

Type IIIType III Type IIIType III Type IVType IV

Classification

Perez, Lowry, Fletcher Larson ASHA 2008 Photos courtesy of Dr. Reza Rahbar, ORL/CHB


Associated AnomaliesAssociated Anomalies

Aero-Digestive:Aero-Digestive: Tracheoesophageal fistulas: 20-37%Tracheoesophageal fistulas: 20-37% Tracheomalacia, laryngomalacia, subglottic Tracheomalacia, laryngomalacia, subglottic

stenosisstenosis Hypoplastic lungs, transposition of the great Hypoplastic lungs, transposition of the great

arteriesarteries Bifid uvula, cleft lip, cleft palateBifid uvula, cleft lip, cleft palate


Associated SyndromesAssociated Syndromes

Pallister Hall syndrome:Pallister Hall syndrome: Hypothalamic hamartoblastoma , Hypopituitarism, Hypothalamic hamartoblastoma , Hypopituitarism,

Polydactyly, Imperforate anusPolydactyly, Imperforate anus

Opitz-Frias syndrome:Opitz-Frias syndrome:

Cleft lip palate, Hypertelorism, Airway cleft, HypospadiasCleft lip palate, Hypertelorism, Airway cleft, Hypospadias

VACTERL VACTERL Heart and vertebral anomalies , Esophageal atresia , Heart and vertebral anomalies , Esophageal atresia ,

Polydactylie, Absent left radius, Anorectal anomaliesPolydactylie, Absent left radius, Anorectal anomalies


Associated SyndromesAssociated SyndromesAt Children’s Hospital Boston, of 81 patients with At Children’s Hospital Boston, of 81 patients with

diagnosis of laryngeal cleft (2003-2008):diagnosis of laryngeal cleft (2003-2008): TEF (s/p repair) (N=6)TEF (s/p repair) (N=6) Multiple Congenital Anomalies (N=13)Multiple Congenital Anomalies (N=13)

Trisomy 21 (N=3) Trisomy 21 (N=3) Fetal Alcohol syndrome (N=1) Fetal Alcohol syndrome (N=1) Tetralogy of Fallot (N=2) Tetralogy of Fallot (N=2) Waardenburg (N=1) Waardenburg (N=1) Scimitar (N=1) Scimitar (N=1) CHARGE association (N=2)CHARGE association (N=2) Opitz (N=1)Opitz (N=1) VACTERL (N=2)VACTERL (N=2)


Incidence of Laryngeal Clefts Incidence of Laryngeal Clefts (all types)(all types)

Live birth: 1 in 10,000-1 in 20,000Live birth: 1 in 10,000-1 in 20,000

Incidence of laryngeal cleft increases to 0.6% Incidence of laryngeal cleft increases to 0.6% in patients with co-existing TEFin patients with co-existing TEF

Strong association with other anomalies, but Strong association with other anomalies, but also can exist in isolationalso can exist in isolation


Symptoms of Laryngeal CleftSymptoms of Laryngeal Cleft

Cough*Cough* Wheezing*Wheezing* StridorStridor Feeding DifficultyFeeding Difficulty AspirationAspiration Respiratory DistressRespiratory Distress

*Most common referral symptoms*Most common referral symptoms* Symptoms are non-specific* Symptoms are non-specific


Diagnosis of Laryngeal CleftDiagnosis of Laryngeal Cleft

High index of suspicion based on:High index of suspicion based on: Clinical presentation Clinical presentation

Medical history/Systematic chart reviewMedical history/Systematic chart review

Interpretation of preoperative studiesInterpretation of preoperative studies Chest radiograph, VFSS/FEES, Upper GI seriesChest radiograph, VFSS/FEES, Upper GI series

Thorough endoscopic airway evaluation under general Thorough endoscopic airway evaluation under general anesthesiaanesthesia

Direct laryngoscopy, bronchoscopy, esophagoscopyDirect laryngoscopy, bronchoscopy, esophagoscopy Endoscopist MUST perform diligent interarytenoid palpationEndoscopist MUST perform diligent interarytenoid palpation


Diagnosis of Laryngeal CleftDiagnosis of Laryngeal Cleft

Airway endoscopy Airway endoscopy (in which laryngeal cleft is (in which laryngeal cleft is suspected)suspected) is needed for definitive diagnosis is needed for definitive diagnosis

VFSS does not identify laryngeal cleft, but can VFSS does not identify laryngeal cleft, but can confirm symptoms of aspiration due to overall confirm symptoms of aspiration due to overall neuromuscular discoordination or isolated neuromuscular discoordination or isolated pharyngeal dysphagiapharyngeal dysphagia


Increasing Incidence: Why?Increasing Incidence: Why? Literature review documents incidence of Type I laryngeal Literature review documents incidence of Type I laryngeal

cleft is higher than in the pastcleft is higher than in the past

7.6 % (Chien et al, 2006)7.6 % (Chien et al, 2006) 6.2 % (Parsons et al, 1998)6.2 % (Parsons et al, 1998) 7.1 % (Watters & Russell, 2003)7.1 % (Watters & Russell, 2003)

-Rising incidence may be due to:-Rising incidence may be due to:-type of patients referred to aerodigestive clinics-type of patients referred to aerodigestive clinics-higher index of suspicion for presence of laryngeal -higher index of suspicion for presence of laryngeal

cleft cleft at time of airway endoscopy (ORL now looking more at time of airway endoscopy (ORL now looking more specifically for laryngeal cleft)specifically for laryngeal cleft)


Surgical Repair of Laryngeal CleftSurgical Repair of Laryngeal Cleft

Conventional Treatment:Conventional Treatment: Invasive surgery requiring incision in the neck and opening Invasive surgery requiring incision in the neck and opening

of the larynxof the larynx Endoscopic Repair: Endoscopic Repair:

Laser and suture Laser and suture Robotic Repair:Robotic Repair:

Work through the oral cavity and in the restricted confines Work through the oral cavity and in the restricted confines of the airway without impeding breathingof the airway without impeding breathing

First performed by Dr. Reza Rahbar at Children’s Hospital First performed by Dr. Reza Rahbar at Children’s Hospital BostonBoston


Photos courtesy of Dr. Reza Rahbar, Children’s Hospital Boston

Endoscopic Repair of Laryngeal Cleft


SLP Perspective:SLP Perspective:Typical course for patientTypical course for patient

1.1. PCP or other specialist refers for MBS due to PCP or other specialist refers for MBS due to chronic pulmonary symptoms of unclear etiology chronic pulmonary symptoms of unclear etiology associated with feedingassociated with feeding

2.2. VFSS: Documentation of aspiration with liquidsVFSS: Documentation of aspiration with liquids

3.3. SLP modifies diet (i.e,. Nectar-thick liquids) to SLP modifies diet (i.e,. Nectar-thick liquids) to promote swallowing safety, minimize aspiration promote swallowing safety, minimize aspiration

-(patient may need NG-tube)-(patient may need NG-tube)

4.4. SLP contact PCP to consider referral to CADDSLP contact PCP to consider referral to CADD

5.5. Patient seen in CADD by ORL, GI, PulmonaryPatient seen in CADD by ORL, GI, Pulmonary


SLP Perspective:SLP Perspective:Typical course for patient (cont.)Typical course for patient (cont.)

6. 6. CADD team completes direct CADD team completes direct laryngoscopy/bronchoscopy to confirm diagnosis of laryngoscopy/bronchoscopy to confirm diagnosis of laryngeal cleftlaryngeal cleft

7.7. Team decides: Team decides: -conservative management or surgical repair??-conservative management or surgical repair??

IF SURGICAL REPAIR:IF SURGICAL REPAIR:8. 8. Maintain diet of thickened liquids for 6-8 weeks Maintain diet of thickened liquids for 6-8 weeks

after surgeryafter surgery9.9. Repeat VFSS at 8-12 weeks after surgery, and Repeat VFSS at 8-12 weeks after surgery, and

repeat every 3 months as needed repeat every 3 months as needed 10.10. If no aspiration, child resumes full oral dietIf no aspiration, child resumes full oral diet


Who to consider for laryngeal cleft:Who to consider for laryngeal cleft:

Any infant or child with normal development (i.e., no Any infant or child with normal development (i.e., no neurogenic, medical and genetic etiology) who neurogenic, medical and genetic etiology) who presents with “isolated swallowing dysfunction”presents with “isolated swallowing dysfunction”

Any infant or child with history of Any infant or child with history of Tracheoesophageal fistula (TEF) and aspiration on Tracheoesophageal fistula (TEF) and aspiration on VFSSVFSS

Any infant or child with a history of chronic Any infant or child with a history of chronic aspiration that does not improve over time or with aspiration that does not improve over time or with traditional interventiontraditional intervention


Conservative Monitoring Conservative Monitoring

If no surgical intervention, conservative If no surgical intervention, conservative management of dysphagia:management of dysphagia:

Diagnose and treat symptoms of GERDiagnose and treat symptoms of GER SLP works with family to modify dietSLP works with family to modify diet Close follow-up with CADD Close follow-up with CADD


Our experience at CHBOur experience at CHB

2003-20082003-2008 81 patients referred for evaluation of cough, 81 patients referred for evaluation of cough,

feeding difficulty, and aspiration feeding difficulty, and aspiration 74 of 81 patients found to have significant 74 of 81 patients found to have significant

laryngeal cleft (Type I or Type II)laryngeal cleft (Type I or Type II) 25 of 74 = conservative monitoring with medical 25 of 74 = conservative monitoring with medical

therapytherapy 49 of 74 = endoscopic CO2 laser repair of Type I 49 of 74 = endoscopic CO2 laser repair of Type I

and Type II laryngeal cleft and Type II laryngeal cleft (age range: 8 mo-15 years)(age range: 8 mo-15 years)


Our experience at CHBOur experience at CHB

--Type I cleft: 100 % reported feeding difficulty with Type I cleft: 100 % reported feeding difficulty with thin liquidsthin liquids

-Wheezing and chronic cough most common respiratory -Wheezing and chronic cough most common respiratory symptomssymptoms

-86% patients with aspiration on pre-surgical VFSS-86% patients with aspiration on pre-surgical VFSS

--71% patients with Type I =no aspiration on VFSS post-repair71% patients with Type I =no aspiration on VFSS post-repair

--82% patients with Type II =no aspiration on VFSS post repair82% patients with Type II =no aspiration on VFSS post repair

-Overall improvement noted in decreasing cough, normal -Overall improvement noted in decreasing cough, normal VFSS, decreasing need for hospitalizationVFSS, decreasing need for hospitalization


Case Study # 2:Case Study # 2:

-5 month old baby girl-5 month old baby girl

-Clinical symptoms: -Clinical symptoms:

-coughing while drinking thin liquids-coughing while drinking thin liquids

-wheezing (worsens with feeding)-wheezing (worsens with feeding)

-frequent spit ups with feeds-frequent spit ups with feeds

-Referred for VFSS by PCP-Referred for VFSS by PCP



--VFSS ResultsVFSS Results::-silent aspiration with thin and nectar-thick liquids-silent aspiration with thin and nectar-thick liquids-no aspiration with honey-thick liquids-no aspiration with honey-thick liquids-no aspiration with purees-no aspiration with purees

-Discuss results with PCP who refers to CADD-Discuss results with PCP who refers to CADD--Nutrition assessmentNutrition assessment

-Airway endoscopy reveals Type 1 laryngeal -Airway endoscopy reveals Type 1 laryngeal cleftcleft-Patient undergoes surgical repair-Patient undergoes surgical repair

-Repeat VFSS 3 months after laryngeal cleft -Repeat VFSS 3 months after laryngeal cleft repairrepair


VFSS Clip # 2VFSS Clip # 2 Post-op Post-op


What the SLP has learned…What the SLP has learned…

*Child with aspiration on VFSS who has otherwise normal *Child with aspiration on VFSS who has otherwise normal development, who presents with any chronic respiratory development, who presents with any chronic respiratory symptoms is a candidate for CADD work-upsymptoms is a candidate for CADD work-up

*Maintain pre-surgical diet at least 6-8 weeks after surgery*Maintain pre-surgical diet at least 6-8 weeks after surgery

*Do not repeat VFSS too soon after surgery-typically wait 8-12 *Do not repeat VFSS too soon after surgery-typically wait 8-12 weeks (minimize radiation exposure)weeks (minimize radiation exposure)

*Collaboration with Aero-digestive team is essential!*Collaboration with Aero-digestive team is essential!


Fletcher Allen Health Fletcher Allen Health Care Care

Rural, community Rural, community hospital hospital

Level I Trauma CenterLevel I Trauma Center Level III 20-bed NICULevel III 20-bed NICU 10-bed NTU10-bed NTU 30-bed Pediatric Floor30-bed Pediatric Floor PICUPICU


Vermont’s Academic Vermont’s Academic Medical CenterMedical Center

In alliance with In alliance with UVMUVM

Teaching hospitalTeaching hospital Vermont Oncology Vermont Oncology

CenterCenter Innovative careInnovative care Acute Acute

Rehabilitation Rehabilitation Center-Fanny Center-Fanny AllenAllen


What is Upper Airway What is Upper Airway Obstruction (UAO)?Obstruction (UAO)?

An obstruction present in the upper An obstruction present in the upper airway due to anatomic or airway due to anatomic or physiologic anomalies disrupting physiologic anomalies disrupting respiration and the oral feeding respiration and the oral feeding processprocess

Can be tracheal, laryngeal or Can be tracheal, laryngeal or pharyngeal in naturepharyngeal in nature

Miller & Willging, 2007Miller & Willging, 2007


Upper vs. Lower Airway Upper vs. Lower Airway ObstructionObstruction

Pattern of stridor is key for Pattern of stridor is key for differential diagnosis of UAOdifferential diagnosis of UAO

Inspiratory stridor Inspiratory stridor Expiratory stridorExpiratory stridor Biphasic stridorBiphasic stridor

Koufman, J & Block, C. American Journal of Speech-Language Pathology Vol.17 327-334 November 2008.


Upper Airway Upper Airway Obstruction: Obstruction:

ClassificationsClassificationsCongenital:Congenital: Choanal atresiaChoanal atresia Cleft lipCleft lip MicrognathiaMicrognathia LaryngomalaciaLaryngomalacia NeoplasmNeoplasm Laryngeal cleftLaryngeal cleft

Acquired:Acquired: CroupCroup EpiglottitisEpiglottitis TracheitisTracheitis Foreign body Foreign body

ingestioningestion Inhalation injuryInhalation injury

The following four slides adapted from Andrews, T. Airway obstruction in craniofacial anomalies: In: Myer, C, Cotton, R, Shott, S. eds. The Pediatric Airway: An Interdisciplinary Approach, Philadelphia, PA: JB Lippencott; 1995:249.Most recently published in Miller & Willging, 2007.


Upper Airway Upper Airway Obstruction: Obstruction:

ClassificationsClassificationsAcute:Acute: Cases are seen in Cases are seen in

emergency emergency departmentdepartment

Cases of UAO are Cases of UAO are acquiredacquired

Infection and Infection and croupcroup

Chronic:Chronic: Pierre Robin Pierre Robin

sequencesequence Treacher-Collins Treacher-Collins

syndromesyndrome Apert syndromeApert syndrome Beckwith-Beckwith-

Wiedemann Wiedemann syndromesyndrome


Etiologies of UAOEtiologies of UAO

Infection/Infection/InflammationInflammation

Accounts for 90% of Accounts for 90% of UAOUAO

CroupCroup EpiglottitisEpiglottitis TracheitisTracheitis Retropharyngeal Retropharyngeal

abscessabscess Peritonsillar abscessPeritonsillar abscess

Accident/Trauma:Accident/Trauma: Foreign body Foreign body

ingestioningestion Inhalation injuryInhalation injury BurnsBurns External trauma to External trauma to

neckneck Post-intubationPost-intubation


Types of UAOTypes of UAO

Nasopharyngeal:Nasopharyngeal:• Adenoid hypertrophyAdenoid hypertrophy• Choanal atresiaChoanal atresia• Midface hypoplasiaMidface hypoplasia

Oropharyngeal:Oropharyngeal: MacroglossiaMacroglossia Pharyngeal hypotoniaPharyngeal hypotonia Cleft Lip/PalateCleft Lip/Palate

Laryngeal typeLaryngeal type

anomalies:anomalies: Laryngeal cleftLaryngeal cleft Vocal fold Vocal fold

paralysisparalysis NeoplasmNeoplasm Laryngeal WebLaryngeal Web LaryngomalaciaLaryngomalacia


Role of Laryngeal-Role of Laryngeal-Pharyngeal Reflux and Pharyngeal Reflux and

GERD in UAOGERD in UAO ErythemaErythema Post-glottic edemaPost-glottic edema Vocal fold edemaVocal fold edema Decreased sensationDecreased sensation Do protonics reverse sensory Do protonics reverse sensory

deficits?deficits? Kirby & Noel, 2007Kirby & Noel, 2007

Case Study 1Case Study 1

Pediatric Patient with Pediatric Patient with Congenital UAO: Congenital UAO:

oropharyngeal and laryngeal oropharyngeal and laryngeal typestypes


Case 1 (timeline:13 Case 1 (timeline:13 months) months)

Baby born at 41 6/7 weeks gestational ageBaby born at 41 6/7 weeks gestational age 3232 grams3232 grams Rapid progression of Persistent Rapid progression of Persistent

Pulmonary Hypertension (PPHN)Pulmonary Hypertension (PPHN) Dx Pneumothorax and underwent Dx Pneumothorax and underwent

ThoracentisisThoracentisis Intubated and on high frequency Intubated and on high frequency

oscillator at FAHCoscillator at FAHC Transferred to CHB on Day of life (DOL) Transferred to CHB on Day of life (DOL)

#2 for ECMO#2 for ECMO


A baby on ECMOA baby on ECMO


Case 1 TimelineCase 1 Timeline

DOL 0-2 at FAHCDOL 0-2 at FAHC DOL 2-16 at CHBDOL 2-16 at CHB DOL 16 through 10/08/07 at FAHCDOL 16 through 10/08/07 at FAHC 10/08/07-11/20/07 at CHB10/08/07-11/20/07 at CHB 11/20/07 readmitted to FAHC 11/20/07 readmitted to FAHC


Case 1 at FAHCCase 1 at FAHC

First Feeding Team consult on DOL First Feeding Team consult on DOL 19 19

Baby with stridor at rest and feedingBaby with stridor at rest and feeding Bronchoscopy revealing significant Bronchoscopy revealing significant

airway prolapse, laryngomalacia and airway prolapse, laryngomalacia and vocal fold immobilityvocal fold immobility

Baby on O2 nasal cannulaBaby on O2 nasal cannula Weight loss Weight loss Breastfeeding attempts were madeBreastfeeding attempts were made


Case 1 at CHBCase 1 at CHB

Baby re-intubated after trial CPAP, Baby re-intubated after trial CPAP, but worsening respiratory distressbut worsening respiratory distress

Undergoes supraglottoplasty for Undergoes supraglottoplasty for severe laryngomalaciasevere laryngomalacia

Dx subglottic stenosis Dx subglottic stenosis Fails extubationFails extubation Tracheostomy (Shiley 3.5 uncuffed)Tracheostomy (Shiley 3.5 uncuffed) Ng tube for poor feeding and Ng tube for poor feeding and

aspirationaspiration


Case 1 at FAHCCase 1 at FAHC

Returns to Vermont for trach Returns to Vermont for trach teachingteaching

Does baby need G tube?Does baby need G tube? Cultural differences: Somalian family Cultural differences: Somalian family

with 6 kidswith 6 kids Mother breast fed all childrenMother breast fed all children Baby now showing signs of oral Baby now showing signs of oral

defensivenessdefensiveness


VFSS results revealVFSS results reveal Discoordination of sucking skillDiscoordination of sucking skill Trach= “open system”Trach= “open system” Laryngeal penetration with thin and Laryngeal penetration with thin and

nectar thick liquid-what are the nectar thick liquid-what are the implications?implications?

MicroaspirationMicroaspiration Silent in natureSilent in nature Oral defensiveness Oral defensiveness GERDGERD


VFSS: Looking at the VFSS: Looking at the whole picturewhole picture

Oral defensivenessOral defensiveness Poor growthPoor growth Recurrent clinical signs aspiration with Recurrent clinical signs aspiration with

breast milk exiting trach site during breast milk exiting trach site during nipplingnippling

Baby undergoes G tube placement for Baby undergoes G tube placement for supplemental feedingssupplemental feedings


Case 1 VFSS 2 Results Case 1 VFSS 2 Results RevealReveal

5 weeks later5 weeks later TrachTrach Passy Muir Speaking Valve in placePassy Muir Speaking Valve in place No documentation of aspiration with No documentation of aspiration with

thin liquidthin liquid Balance PO (per mouth) and PG (per Balance PO (per mouth) and PG (per

gavage) feedingsgavage) feedings


Case 1 Re-admitted to Case 1 Re-admitted to FAHCFAHC

5 weeks later5 weeks later Recurrent upper respiratory Recurrent upper respiratory

infectionsinfections Post-tussive emesisPost-tussive emesis Bilateral Infiltrates on CXRBilateral Infiltrates on CXR Cultural differences: everyone eats Cultural differences: everyone eats

out of same bowl with hands (and out of same bowl with hands (and germs)germs)


Case 1 Treatment Plan: Case 1 Treatment Plan: “Interim Feeding Plan”“Interim Feeding Plan”

When baby is healthy with no URI, When baby is healthy with no URI, emesis, PO feed thin liquids via bottleemesis, PO feed thin liquids via bottle

If baby is ill, with URI, emesis, make If baby is ill, with URI, emesis, make temporary, alternate modifications to temporary, alternate modifications to feeding. This could include NPOfeeding. This could include NPO

Less conservative, would be nectar thick Less conservative, would be nectar thick liquids and/or begin spoon feeding at 6 liquids and/or begin spoon feeding at 6 mo.mo.

Rely on G tube as primary means of Rely on G tube as primary means of nutrition and hydrationnutrition and hydration


Case 1: Happy OutcomesCase 1: Happy Outcomes

Vocal fold mobility returnsVocal fold mobility returns Baby decannulatedBaby decannulated Full PO feeder of thin liquids via Full PO feeder of thin liquids via

sippy cup and bottle and soft solidssippy cup and bottle and soft solids Will keep G tube through winter Will keep G tube through winter

months as back-up for “Interim months as back-up for “Interim Feeding Plan”Feeding Plan”

Achieving developing milestones in Achieving developing milestones in timely age-appropriate mannertimely age-appropriate manner


What we learn What we learn (and questions to ask)(and questions to ask)

Should breastfeeding be allowed if baby Should breastfeeding be allowed if baby with s/s aspiration?with s/s aspiration?

Is any aspiration allowed?Is any aspiration allowed? The importance of PMV to restore healthy The importance of PMV to restore healthy

upper airway and “closed system”upper airway and “closed system” VFSS is only ONE window in time and VFSS is only ONE window in time and

only a small piece to our overall only a small piece to our overall assessmentassessment

How to embrace and manage cultural How to embrace and manage cultural differencesdifferences


Pediatric Patient with Pediatric Patient with Congenital UOA: acute, Congenital UOA: acute,

oropharyngeal typeoropharyngeal type


Case 2 (timeline: 9 Case 2 (timeline: 9 months)months)

10 month old admitted to FAHC with 10 month old admitted to FAHC with respiratory distress and neck edemarespiratory distress and neck edema

Tracheostomy Tracheostomy Biopsied revealed ganglioneuroblstomaBiopsied revealed ganglioneuroblstoma Baby received cycle of carboplatin and Baby received cycle of carboplatin and

etoposide, which decreased size of tumor etoposide, which decreased size of tumor temporarilytemporarily

Mass increased in size, compromising her Mass increased in size, compromising her airway and necessitating intubationairway and necessitating intubation


Case 2 at CHBCase 2 at CHB

Transferred to CHB for debulking of the Transferred to CHB for debulking of the massmass

Subsequent pathology reveals a benign Subsequent pathology reveals a benign cervical right neck neuroectodermal cervical right neck neuroectodermal lesionlesion

Most notable complication after debulking Most notable complication after debulking is poor feedingis poor feeding

VFSS in Boston reveals silent aspiration VFSS in Boston reveals silent aspiration of puree and all liquid consistenciesof puree and all liquid consistencies


Case 2 Case 2 MRI reveals residual tumor in posterior MRI reveals residual tumor in posterior

fossa and neck after debulkingfossa and neck after debulking Large enhancing mask in the right Large enhancing mask in the right

parapharyngeal spaceparapharyngeal space Neurosurgery deferred further resection Neurosurgery deferred further resection

due to low growth potential and stable due to low growth potential and stable size on MRIsize on MRI

Sub total resection: implications for long Sub total resection: implications for long term and feeding?term and feeding?


Case 2 VFSS results Case 2 VFSS results revealreveal

Silent aspiration with purees and Silent aspiration with purees and liquidsliquids

Delayed initiation of swallowDelayed initiation of swallow Significant pharyngeal weakness Significant pharyngeal weakness

and poor pharyngeal constrictionand poor pharyngeal constriction Post-swallow residuePost-swallow residue


Case 2: Treatment planCase 2: Treatment plan

NPO recommended to PCPNPO recommended to PCP Baby underwent G tube placement given Baby underwent G tube placement given

likely long-term nature of impairmentlikely long-term nature of impairment Parents extremely frustrated by NPO Parents extremely frustrated by NPO

statusstatus Oral Stimulation program and tactile play Oral Stimulation program and tactile play

with textures in absence of PO intakewith textures in absence of PO intake


What we learn What we learn (and questions to ask)(and questions to ask)

Understanding prognosis is essential to Understanding prognosis is essential to make recommendationsmake recommendations

Is any aspiration allowed?Is any aspiration allowed? How do we balance the need for NPO How do we balance the need for NPO

status with developmental feeding needs status with developmental feeding needs and family desires to feed the child?and family desires to feed the child?

Is NPO a feasible and reasonable Is NPO a feasible and reasonable recommendation?recommendation?


Pediatric patient with Pediatric patient with Congenital UAO: laryngeal Congenital UAO: laryngeal

anomaly typeanomaly type


Case 3 (timeline: 3 Case 3 (timeline: 3 months) months)

Born at 41 weeks gestational ageBorn at 41 weeks gestational age 4095 grams; LGA4095 grams; LGA C-section due to fetal distressC-section due to fetal distress Respiratory distress at birthRespiratory distress at birth Consulted Feeding Team at DOL 11 Consulted Feeding Team at DOL 11

(recommended NPO)(recommended NPO) StridorStridor IntubatedIntubated


Case 3Case 3 Feeding Team re-consulted 3 weeks later Feeding Team re-consulted 3 weeks later

after successful extubationafter successful extubation s/p epiglottoplasty and supraglottoplasty s/p epiglottoplasty and supraglottoplasty

for redundant tissuefor redundant tissue Baby with weak cry, aphonic at timesBaby with weak cry, aphonic at times S/s aspiration during nipplingS/s aspiration during nippling Inspiratory stridor continuesInspiratory stridor continues MRI was normalMRI was normal


Laryngoscopy results Laryngoscopy results revealreveal

Larynx remarkable for enlarged Larynx remarkable for enlarged arytenoid cartilagesarytenoid cartilages

Paradoxical VF movement with each Paradoxical VF movement with each inspirationinspiration

A-P collapse of trachea was A-P collapse of trachea was observed dynamicallyobserved dynamically

No clear pulsatile compression of No clear pulsatile compression of trachea was seentrachea was seen


VFSS results revealVFSS results reveal

Day after laryngoscopyDay after laryngoscopy Aspiration with thin liquidsAspiration with thin liquids No evidence of aspiration with No evidence of aspiration with

nectar thick liquidsnectar thick liquids Ng tube in placeNg tube in place


Case 3: Treatment PlanCase 3: Treatment Plan

Recommendations for baby to nipple Recommendations for baby to nipple nectar thick liquids via slow flow nipplenectar thick liquids via slow flow nipple

PO ad lib when baby showing interest in PO ad lib when baby showing interest in nipplingnippling

Gavage feedings continue to supplement Gavage feedings continue to supplement until adequate volumes consumed POuntil adequate volumes consumed PO

Repeat VFSS in 6-8 weeksRepeat VFSS in 6-8 weeks


Case 3: Differential Case 3: Differential DiagnosisDiagnosis

Paradoxical Vocal Fold Movement Paradoxical Vocal Fold Movement (PVFM)(PVFM)

Known an a functional or non-Known an a functional or non-organic disorderorganic disorder

Etiology suggests psychopathologyEtiology suggests psychopathology Laryngeal dystonia?Laryngeal dystonia? Neurogenic etiology?Neurogenic etiology?


Case 3: Literature review Case 3: Literature review

Heatley & Swift, 1996 report a case Heatley & Swift, 1996 report a case of 4 month old with PVCD related to of 4 month old with PVCD related to underlying GERDunderlying GERD

Omland & Brondbo, 2008 report 4 Omland & Brondbo, 2008 report 4 cases infant of BVCP vs PVCMcases infant of BVCP vs PVCM

EMG can provide further diagnostic EMG can provide further diagnostic info if the laryngeal muscles have info if the laryngeal muscles have normal electrical activitynormal electrical activity


Case 3Case 3

PVFM classified as Cortical or Upper PVFM classified as Cortical or Upper Motor Neuron OR Nuclear and Lower Motor Neuron OR Nuclear and Lower Motor NeuronMotor Neuron

In infant with no clear organic In infant with no clear organic etiology, can’t be classified under etiology, can’t be classified under aboveabove

GERDGERD Laryngeal Dyskinesia (lack of Laryngeal Dyskinesia (lack of

abductory movement with inhalation)abductory movement with inhalation)


Final Thoughts on what Final Thoughts on what we learn…we learn…

How do we best manage aspiration in How do we best manage aspiration in pediatrics?pediatrics?

Least restrictive, safest dietLeast restrictive, safest diet ““Do no Harm” principleDo no Harm” principle Is any aspiration allowed?Is any aspiration allowed? Colin Wallis, MD, Pediatric Pulmonologist, Colin Wallis, MD, Pediatric Pulmonologist,

of Great Ormond Street Hospital asks “Is of Great Ormond Street Hospital asks “Is there damage from aspiration?” not is it there damage from aspiration?” not is it occurringoccurring


Final ThoughtsFinal Thoughts

Upper airway obstruction is an area Upper airway obstruction is an area where the medical SLP needs where the medical SLP needs expertiseexpertise

Teaming with ENT, Pulmonary, GI, Teaming with ENT, Pulmonary, GI, Radiology is essentialRadiology is essential

Continuing Education for SLP and Continuing Education for SLP and always asking questions is essential always asking questions is essential

Esophageal Esophageal Etiologies of Etiologies of

Pediatric Pediatric DysphagiaDysphagia


South Shore HospitalSouth Shore Hospital Regional Medical Regional Medical

Center SE MACenter SE MA 4,800 births/year4,800 births/year 380 inpatient beds380 inpatient beds Level 2 Trauma Level 2 Trauma

CenterCenter 10 Level 3 NICU10 Level 3 NICU 20 Level 2 NICU20 Level 2 NICU Pediatric FloorPediatric Floor Outpatient Pediatric Outpatient Pediatric

Feeding ClinicFeeding Clinic Clinical AffiliationsClinical Affiliations


Anatomy of the Anatomy of the EsophagusEsophagus

Refer to the esophagus as the Refer to the esophagus as the “proximal” and “distal” portions.“proximal” and “distal” portions.

Proximal third of the esophagus- Proximal third of the esophagus- striated muscle striated muscle

Distal third of the esophagus (and Distal third of the esophagus (and remainder of the gut)-smooth muscle remainder of the gut)-smooth muscle


Anatomy & Physiology of Anatomy & Physiology of the Esophagusthe Esophagus

After the upper esophageal sphincter After the upper esophageal sphincter relaxes, a reflexive, peristaltic relaxes, a reflexive, peristaltic “primary stripping wave” is initiated.“primary stripping wave” is initiated.

This propels the bolus distallyThis propels the bolus distally Amplitude of this wave is higher in Amplitude of this wave is higher in

the distal esophagusthe distal esophagus Traveling at 3-5 cm per second Traveling at 3-5 cm per second

(Rubenstein, J.)(Rubenstein, J.)


Anatomy & Physiology of Anatomy & Physiology of the Esophagusthe Esophagus

Stripping wave initiated by the Stripping wave initiated by the oropharyngeal swallow causing the oropharyngeal swallow causing the LES to relaxLES to relax

Remains open until the stripping Remains open until the stripping wave has arrived at the LES wave has arrived at the LES

Secondary stripping waves originate Secondary stripping waves originate from within the proximal esophagus from within the proximal esophagus and propel any residual bolus into and propel any residual bolus into the stomachthe stomach


Esophageal Dysphagia Esophageal Dysphagia Occurs When:Occurs When:

Amplitude of the stripping wave is too lowAmplitude of the stripping wave is too low Velocity of the wave is too slow or too fastVelocity of the wave is too slow or too fast The wave is so fast the UES and LES The wave is so fast the UES and LES

contract simultaneouslycontract simultaneously The UES or LES fail to relaxThe UES or LES fail to relax There is mechanical obstruction that There is mechanical obstruction that

cannot be overcome by the stripping wavecannot be overcome by the stripping wave


Etiologies of Esophageal Etiologies of Esophageal DysphagiaDysphagia

Differential diagnosis from Differential diagnosis from oropharyngeal etiologiesoropharyngeal etiologies

Co-occurring oropharyngeal and Co-occurring oropharyngeal and esophageal etiologyesophageal etiology

Careful history is importantCareful history is important Child should be asked to “point” Child should be asked to “point”

with one finger to the area they feel with one finger to the area they feel there is “hang up” of the bolusthere is “hang up” of the bolus



2 Main Etiologies 2 Main Etiologies of Esophageal of Esophageal DysphagiaDysphagia

Mechanical CausesMechanical Causes

Motility Motility DisturbancesDisturbances

Food impaction is Food impaction is more frequent with more frequent with mechanical causesmechanical causes

Patients with Patients with mechanical mechanical obstruction can obstruction can typically still typically still swallow liquids swallow liquids

More pronounced More pronounced solid food solid food dysphagiadysphagia



Mechanical disturbance- usually localize Mechanical disturbance- usually localize the “hang up” of the food distal to the the “hang up” of the food distal to the larynx larynx

Oropharyngeal etiology localize the Oropharyngeal etiology localize the “hang up” proximal to the larynx“hang up” proximal to the larynx

Often not possible to determine etiology Often not possible to determine etiology and hence diagnostic testing is indicatedand hence diagnostic testing is indicated

If SLP finds evidence of an esophageal If SLP finds evidence of an esophageal disorder a recommendation is made for disorder a recommendation is made for appropriate referral to a specialistappropriate referral to a specialist

Case Study of a Case Study of a Pediatric Patient Pediatric Patient with Mechanical with Mechanical

ObstructionObstruction


VACTERL or VATER VACTERL or VATER AssociationAssociation

Acronym used to describe a series of Acronym used to describe a series of characteristics which have been found to occur characteristics which have been found to occur togethertogether

V =vertebrae, which are abnormal (70% patients)V =vertebrae, which are abnormal (70% patients) A =anal atresia/ imperforate anus (55% patients)A =anal atresia/ imperforate anus (55% patients) C = cardiac defects (75% patients)C = cardiac defects (75% patients) T= tracheoesophageal fistula (TEF), an abnormal T= tracheoesophageal fistula (TEF), an abnormal

connection between the trachea and the esophagusconnection between the trachea and the esophagus E= esophageal atresia, (EA) the esophagus does E= esophageal atresia, (EA) the esophagus does

not connect to the stomachnot connect to the stomach R= renal defectsR= renal defects L= limb (arm) defectsL= limb (arm) defects


VACTERL AssociationVACTERL Association

Babies diagnosed Babies diagnosed with VACTERL with VACTERL have at least 3 or have at least 3 or more of the more of the anomalies.anomalies.

Wide range of Wide range of manifestationsmanifestations

EA with TEF EA with TEF occurs in 70% occurs in 70% patientspatients

EA/TEF can also EA/TEF can also occur as an isolated occur as an isolated defectdefect EA/TEF as an isolated EA/TEF as an isolated

condition occurs in 1 condition occurs in 1 in 3,000-5,000 birthsin 3,000-5,000 births

Typically have Typically have normal development normal development and intelligenceand intelligence


Esophageal Atresia (EA) & Esophageal Atresia (EA) & Tracheoesophageal Fistula Tracheoesophageal Fistula

(TEF)(TEF) Presence of EA suspected in the post Presence of EA suspected in the post

natal periodnatal period Newborn presents with excessive Newborn presents with excessive

salivationsalivation Coughing, choking, sneezing.Coughing, choking, sneezing. Upon oral feeding the infant Upon oral feeding the infant

struggles and may become cyanoticstruggles and may become cyanotic Respiratory distressRespiratory distress


EA & TEFEA & TEF

Congenital EA represents failure of the Congenital EA represents failure of the esophagus to develop as a continuous esophagus to develop as a continuous passage.passage.

TEF represents an abnormal opening TEF represents an abnormal opening between the trachea and the esophagusbetween the trachea and the esophagus

DX is made, all oral feedings are DX is made, all oral feedings are stoppedstopped

Surgical repair within the first days of Surgical repair within the first days of lifelife


Case StudyCase Study

4 year 11 month old male with VACTERL 4 year 11 month old male with VACTERL AssociationAssociation

Followed since infancy through Followed since infancy through Swallowing Disorders ProgramSwallowing Disorders Program

Post natal diagnosis of EA/TEF s/p Post natal diagnosis of EA/TEF s/p surgical repair on DOL#3surgical repair on DOL#3

Cardiac diagnosis of Tetrology of Fallot Cardiac diagnosis of Tetrology of Fallot s/p repair (multiple staged repairs)s/p repair (multiple staged repairs)

G-tube placed in infancy s/p EA/TEF G-tube placed in infancy s/p EA/TEF repairrepair



History of aspiration with thin and nectar History of aspiration with thin and nectar thick liquids on VFSS first identified at 6 thick liquids on VFSS first identified at 6 months of age.months of age.

Cleared to orally feed purees only.Cleared to orally feed purees only. G-tube to meet hydration, nutritional G-tube to meet hydration, nutritional

needsneeds No improvement with swallow function No improvement with swallow function

over the yearsover the years Referred to CADD clinic at 3 years of ageReferred to CADD clinic at 3 years of age



Dx with type 1 laryngeal cleft (LC)Dx with type 1 laryngeal cleft (LC) S/P endoscopic repair of LC at 3 S/P endoscopic repair of LC at 3

years 6 months oldyears 6 months old Pre-surgically patient eating diet of Pre-surgically patient eating diet of

purees, soft dissolvables and solids purees, soft dissolvables and solids (hamburger, pasta, grilled chicken, (hamburger, pasta, grilled chicken, green beans) in limited volume.green beans) in limited volume.

All liquids via g-tubeAll liquids via g-tube


Case StudyCase Study 6 months s/p LC 6 months s/p LC

repair patients VFSS repair patients VFSS revealed improvement revealed improvement in underlying swallow in underlying swallow functionfunction

Patient cleared to Patient cleared to take thin and nectar take thin and nectar thick liquidsthick liquids

Patient co-existing Patient co-existing sensory based feeding sensory based feeding disorderdisorder

Volume of solids Volume of solids limited due to texture limited due to texture sensitivity, hyper-sensitivity, hyper-sensitive gag reflexsensitive gag reflex

Reduced efficiency Reduced efficiency with chewingwith chewing

Receiving Receiving motor/sensory based motor/sensory based feeding therapyfeeding therapy



Patient returns for repeat VFSS at 4 years Patient returns for repeat VFSS at 4 years 11 months due to complaints “food is 11 months due to complaints “food is getting stuck”getting stuck”

Patient points to area below the Patient points to area below the larynx/chest arealarynx/chest area

Mother describes patient as “forceful Mother describes patient as “forceful swallow” with facial grimacing, decreased swallow” with facial grimacing, decreased interest in solids, decreased volume of interest in solids, decreased volume of solidssolids

Asymptomatic with liquidsAsymptomatic with liquids


Videofluoroscopic Results Videofluoroscopic Results Reveal:Reveal:

No evidence of aspiration with thin No evidence of aspiration with thin liquidsliquids

No evidence of aspiration with solids No evidence of aspiration with solids which pass from the pharynx through which pass from the pharynx through the UESthe UES

Normal pharyngeal phase of swallowingNormal pharyngeal phase of swallowing (confirm this on video clip)(confirm this on video clip)


Videofluoroscopic Results Videofluoroscopic Results Reveal:Reveal:

Esophageal phase reveals moderate Esophageal phase reveals moderate anastomotic narrowing at the site of anastomotic narrowing at the site of the EA repairthe EA repair

Piece of chicken impacted at the site Piece of chicken impacted at the site of the anastomosisof the anastomosis

Liquid wash downs required to clear Liquid wash downs required to clear the solidthe solid

Continued ingestion of solids can Continued ingestion of solids can lead to “back up”lead to “back up”


Treatment PlanTreatment Plan Attending radiologist Attending radiologist

at VFSS paged the GI at VFSS paged the GI physician to discuss physician to discuss dx of a stricturedx of a stricture

Patient placed on Patient placed on diet of liquids/purees diet of liquids/purees only until surgical only until surgical decisiondecision

Patient underwent Patient underwent dilatation of the dilatation of the esophagus esophagus

1 week s/p dilatation 1 week s/p dilatation patient resumed and patient resumed and tolerated solid foods.tolerated solid foods.

Avoid hard to chew Avoid hard to chew solids & high risk solids & high risk choking foodschoking foods

Avoid “gummy” Avoid “gummy” foodsfoods

Smaller, more Smaller, more frequent mealsfrequent meals


What we learn……..What we learn……..

We must look beyond the pharyngeal We must look beyond the pharyngeal swallowswallow

Most neonates with EA/TEF have Most neonates with EA/TEF have some degree of esophageal some degree of esophageal dysmotilitydysmotility

Strictures at the site of the Strictures at the site of the anastomosis are common and may anastomosis are common and may require mulitple dilatationsrequire mulitple dilatations



Solid food dysphagia often the first Solid food dysphagia often the first sign the stricture has re-occurredsign the stricture has re-occurred

As children mature they are “in As children mature they are “in tune” to their esophagus and can tune” to their esophagus and can describe symptomsdescribe symptoms

Many older children will self-restrict Many older children will self-restrict their diettheir diet

VFSS is an effective procedure for VFSS is an effective procedure for identifying the strictureidentifying the stricture



VFSS is only one instrumental VFSS is only one instrumental evaluation that may give rise to evaluation that may give rise to esophageal dysphagiaesophageal dysphagia

Further diagnostic testing may Further diagnostic testing may include: include: Upper GI seriesUpper GI series Esophagoduodenscopy (EGD)Esophagoduodenscopy (EGD)

Case Study of a Case Study of a Pediatric Patient Pediatric Patient

with Motility with Motility DisturbanceDisturbance



3 month old infant referred for VFSS 3 month old infant referred for VFSS by pediatric otolaryngologist (ORL)by pediatric otolaryngologist (ORL)

Presenting symptoms coughing and Presenting symptoms coughing and choking during feeding choking during feeding

Mother reports “gasping” during Mother reports “gasping” during bottle feedsbottle feeds

PCP placed infant on Zantac with no PCP placed infant on Zantac with no improvement in symptomsimprovement in symptoms


VFSS ResultsVFSS Results

Documentation of silent aspiration Documentation of silent aspiration with thin, nectar thick and honey with thin, nectar thick and honey thick liquidthick liquid

Build up of residue above the upper Build up of residue above the upper esophageal sphincteresophageal sphincter

Failure of the UES to relaxFailure of the UES to relax Aspiration results from a build-up/ Aspiration results from a build-up/

spill over of liquid due to failure of spill over of liquid due to failure of the UES to relaxthe UES to relax


VFSS ResultsVFSS Results

Coordination of suck swallow Coordination of suck swallow breathe alteredbreathe altered

Airway protection is compromisedAirway protection is compromised Co-occurrence of oropharyngeal Co-occurrence of oropharyngeal

dysphagia along with an esophageal dysphagia along with an esophageal phase dysphagiaphase dysphagia


Treatment PlanTreatment Plan

Attending radiologist at VFSS paged Attending radiologist at VFSS paged referring ORL physicianreferring ORL physician

Patient made NPO by referring Patient made NPO by referring physicianphysician

Patient admitted to the hospital Patient admitted to the hospital GI service consultedGI service consulted


Cricopharyngeal Cricopharyngeal DysfunctionDysfunction

Cricopharyngeal dysfunction as a cause Cricopharyngeal dysfunction as a cause of oropharyngeal dysphagia in infancyof oropharyngeal dysphagia in infancy

Multi-phase dysphagiaMulti-phase dysphagia Results from 2 conditions:Results from 2 conditions: Failure of relaxation of the upper Failure of relaxation of the upper

esophageal sphincter (UES) and/oresophageal sphincter (UES) and/or Cricopharyngeal incoordinationCricopharyngeal incoordination (Mihailocivc, T, & Perisic, V.N., 1992)(Mihailocivc, T, & Perisic, V.N., 1992)


Cricopharyngeal Cricopharyngeal dysfunctiondysfunction

Manifested by Manifested by swallowing swallowing difficultiesdifficulties

Nasopharyngeal Nasopharyngeal regurgitationregurgitation

Coughing, chokingCoughing, choking Aspiration Aspiration

pneumoniapneumonia

Diagnosis typically Diagnosis typically made by made by radiography and radiography and motility studiesmotility studies

Cricopharyngeal Cricopharyngeal dysfunction caused dysfunction caused by either primary by either primary UES motility UES motility disorder ordisorder or

May be associated May be associated with central nervous with central nervous system diseasessystem diseases


Case Study Con’t…Case Study Con’t…

Patient admitted to the hospital for Patient admitted to the hospital for nasogastric tube feedingsnasogastric tube feedings

GI work up revealed cricopharyngeal GI work up revealed cricopharyngeal achalasiaachalasia

Patient underwent esophageal Patient underwent esophageal dilatation during admissiondilatation during admission

D/C home full oral feeder with D/C home full oral feeder with resolution of symptomsresolution of symptoms

f/u with GI physicianf/u with GI physician


Repeat VFSS Repeat VFSS 8 weeks s/p 8 weeks s/p

dilatation patient dilatation patient returned for repeat returned for repeat VFSSVFSS

Full PO feeding of Full PO feeding of thin liquid via bottle thin liquid via bottle in the interimin the interim

Spoon feeding had Spoon feeding had been initiatedbeen initiated

No intermittent No intermittent respiratory respiratory symptomssymptoms

VFSS reveals VFSS reveals qualitative qualitative improvement but improvement but continued pooling continued pooling at the UESat the UES

Aspiration with Aspiration with thin liquids thin liquids documenteddocumented

No aspiration with No aspiration with spoon feeding of spoon feeding of purees but poolingpurees but pooling


Treatment PlanTreatment Plan Medical team met Medical team met

to discuss the caseto discuss the case Cleared to continue Cleared to continue

on thin liquids with on thin liquids with pacing strategy in pacing strategy in placeplace

Alter pacifier for Alter pacifier for non-nutritive non-nutritive sucking to help sucking to help clear the residue clear the residue with subsequent with subsequent swallowsswallows

Nectar thick Nectar thick liquids not liquids not indicated due to indicated due to increased build up increased build up

Return to GI to Return to GI to discuss need for discuss need for re-dilatationre-dilatation


Treatment Options for Treatment Options for Cricopharyngeal AchalasiaCricopharyngeal Achalasia

Cricopharyngeal Cricopharyngeal myotomymyotomy Surgical division of the Surgical division of the

musclemuscle Renders the muscle inactiveRenders the muscle inactive Often as “last option”Often as “last option”

Dilatation Dilatation Single balloon catheter Single balloon catheter

dilatation proven to be dilatation proven to be successfulsuccessful

Less invasive than myotomyLess invasive than myotomy Results apparent 24-48 Results apparent 24-48

after procedureafter procedure Patient may require repeat Patient may require repeat

dilatationsdilatations

Botox injection to the UESBotox injection to the UES More commonly used in More commonly used in

the adult populationthe adult population Efficacy of botox use to Efficacy of botox use to

LES in pediatricsLES in pediatrics Botox effect may wear off Botox effect may wear off

over timeover time Need to re-injectNeed to re-inject Risk of injecting the Risk of injecting the

wrong site/musclewrong site/muscle


Case study Con’t….Case study Con’t….

Different treatment modality trialedDifferent treatment modality trialed Patient received Botox injection to the Patient received Botox injection to the

UESUES Due to complications from Botox Due to complications from Botox

injection patient required hospital injection patient required hospital admission for NG tube placementadmission for NG tube placement

Decision was made for g-tube Decision was made for g-tube placement and repeat dilatation to the placement and repeat dilatation to the UES due to on-going dysphagia and UES due to on-going dysphagia and aspirationaspiration


Case Study Con’t….Case Study Con’t….

Improvement in underlying swallow Improvement in underlying swallow function s/p second dilatationfunction s/p second dilatation

Aspiration resolvedAspiration resolved Patient progressed to mashed and soft Patient progressed to mashed and soft

table foods (1 year of age)table foods (1 year of age) Soft solids pass through site of Soft solids pass through site of

dilatation without hold updilatation without hold up Cricopharyngeal muscle still Cricopharyngeal muscle still

prominent but with improved prominent but with improved coordinationcoordination


What we learnWhat we learn

ASHA Guidelines for Speech Language ASHA Guidelines for Speech Language Pathologists Performing Videofluoroscopic Pathologists Performing Videofluoroscopic Swallow Studies (2004) statesSwallow Studies (2004) states

““Clinicians should be aware that Clinicians should be aware that oropharyngeal swallow function is often oropharyngeal swallow function is often altered in patients with esophageal altered in patients with esophageal motility disorders and dysphagia.”motility disorders and dysphagia.”

““SLP’s have knowledge and skills to SLP’s have knowledge and skills to recognize patient signs and symptoms recognize patient signs and symptoms associated with esophageal dysphagiaassociated with esophageal dysphagia


What we learnWhat we learn Results of the instrumental oropharyngeal Results of the instrumental oropharyngeal

swallow evaluation should include an swallow evaluation should include an esophageal screening.esophageal screening.

Results of the esophageal screening may Results of the esophageal screening may reveal a structural or motor abnormality reveal a structural or motor abnormality suggesting that a referral to a suggesting that a referral to a Gastroenterologist may be indicated. Gastroenterologist may be indicated. (Easterling, 2007, Perspectives)(Easterling, 2007, Perspectives)

““SLP’s should have sufficient knowledge of SLP’s should have sufficient knowledge of esophageal structure and function to make esophageal structure and function to make an appropriate referral and plan an appropriate referral and plan cooperative managementcooperative management


Seeking ResourcesSeeking Resources

Accessibility to a pediatric medical center that Accessibility to a pediatric medical center that offers multidisciplinary team approach such offers multidisciplinary team approach such as The Center for Aerodigestive Disorders.as The Center for Aerodigestive Disorders.

Underscores the need for graduate training in Underscores the need for graduate training in pediatric dysphagiapediatric dysphagia

Course work and clinical practicumCourse work and clinical practicum Collaboration among hospital and community Collaboration among hospital and community

based SLP’s for carry over of safe and based SLP’s for carry over of safe and successful feeding plan in the home setting. successful feeding plan in the home setting.

Questions?Questions?


How to contact us:How to contact us:

[email protected]@childrens.harvard.eduu

[email protected]@vtmednet.org

[email protected][email protected]


ReferencesReferences ASHA 2008 Upper Airway Obstruction ReferencesASHA 2008 Upper Airway Obstruction References Andrews, T. Airway obstruction in craniofacial anomalies: In: Myer, C, Cotton, R, Shott, S. Andrews, T. Airway obstruction in craniofacial anomalies: In: Myer, C, Cotton, R, Shott, S.

eds. The Pediatric Airway: An Interdisciplinary Approach, Philadelphia, PA: JB Lippencott; eds. The Pediatric Airway: An Interdisciplinary Approach, Philadelphia, PA: JB Lippencott; 1995:249. (Most recently published in Miller & Willging, 2007.)1995:249. (Most recently published in Miller & Willging, 2007.)

Koufman, J & Block, C. American Journal of Speech-Language PathologyKoufman, J & Block, C. American Journal of Speech-Language Pathology Vol.17 327- Vol.17 327-334 November 2008334 November 2008

Omland, T, Brondbo, K (2008) Paradoxical vocal cord movement in newborn and congenital Omland, T, Brondbo, K (2008) Paradoxical vocal cord movement in newborn and congenital idiopathic vocal cord paralysis: two of a kind? European Archives of Otorhinolaryngology idiopathic vocal cord paralysis: two of a kind? European Archives of Otorhinolaryngology 265:803-807.265:803-807.

Mathers-Schmidt, B (2001) Paradoxical Vocal Fold Motion: A Tutorial on a Complex Disorder Mathers-Schmidt, B (2001) Paradoxical Vocal Fold Motion: A Tutorial on a Complex Disorder and the Speech-Language Pathologist’s Role. American Journal of Speech-language Pathology and the Speech-Language Pathologist’s Role. American Journal of Speech-language Pathology 10: 111-125.10: 111-125.

Heatley, D, Swift, E (1996) Paradoxical vocal cord dysfunction in an infant with stridor and Heatley, D, Swift, E (1996) Paradoxical vocal cord dysfunction in an infant with stridor and Gastroesophageal reflux. International Journal of Pediatric Otorhinolaryngology 34: 149-151.Gastroesophageal reflux. International Journal of Pediatric Otorhinolaryngology 34: 149-151.

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