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Title: Pediatric Dysphagia: Complex Case Studies of Patients with Aerodigestive Disorders 2421


1
Pediatric Dysphagia Complex Case Studies of
Patients with Aerodigestive Disorders (2421)
  • Jennifer Perez, M.S., CCC-SLP
  • Childrens Hospital Boston, Boston, MA
  • Alana Lowry, M.S., CCC-SLP
  • Fletcher Allen Healthcare, Burlington, VT
  • Kara Fletcher Larson, M.S., CCC-SLP
  • South Shore Hospital, Weymouth, MA

2
3 SLPs 1 Vision
  • All have worked and trained extensively at
    Childrens Hospital Boston
  • Clinical specialty in pediatric dysphagia
  • High volume of pediatric videofluoroscopy
  • Level 3 NICU
  • Exposure to wide range of medical/surgical
    pediatric diagnoses
  • Working within the division of ORL

3
Childrens Hospital Boston
  • Acute, urban pediatric hospital
  • Associated with Harvard Medical School
  • 397 bed comprehensive pediatric healthcare center
  • Medical/Surgical ICU
  • Cardiac ICU
  • Neonatal ICU (Level III)
  • 204 specialized clinical programs

4
Aero-digestive Disorders
  • Congenital Anomalies of Larynx
  • Upper Airway Obstruction
  • Esophageal Obstructive Disorders

5
Center for Aero-Digestive Disorders
  • (CADD)

6
Center for Aero-Digestive Disorders
  • Multi-disciplinary clinic including pediatric
    specialists from
  • Otolaryngology (ORL)
  • Gastroenterology
  • Pulmonary
  • Surgery
  • Radiology
  • Speech-Language Pathology
  • Pediatric Dysphagia specialists
  • Voice specialists
  • Neurology
  • Genetics

7
Center for Aero-Digestive Disorders
  • Providing comprehensive services to infants and
    children with complex problems involving the
  • Airway
  • Pulmonary tract
  • Upper digestive tract

8
Center for Aero-Digestive Disorders
  • Frequently treated medical conditions
  • Asthma
  • Congenital anomalies of the esophagus and trachea
  • Croup
  • Gastroesophageal reflux
  • Iatrogenic or acquired tracheal stenosis
  • Laryngeal cleft
  • Laryngomalacia
  • Lymphatic malformation
  • Pneumonia
  • Stridor
  • Subglottic stenosis
  • Tracheoesophageal fistula/esophageal atresia

9
Center for Aero-Digestive Disorders
  • LogisticsHow the CADD works
  • Once per week, patients see ORL, GI, Pulmonary in
    clinic
  • Speech-Pathology available for consultation
  • Following clinic, team members meet to discuss
    and collaborate on findings and determine
    follow-up plan
  • Once per month, CADD conference of various
    disciplines for review of most complex
    patients/cases

10
Importance of the speech-language pathologist on
the Aero-Digestive team
  • Clinical screening for feeding swallowing
    safety
  • Objective assessment of swallow function pre-
    and/or post-surgical intervention
  • Comprehensive feeding evaluation/therapy for
    sensory-based feeding issues, oral motor delays
    secondary to complex medical/surgical history

11
Center for Aero-Digestive Disorders
  • Criteria for referral for objective swallowing
    evaluation
  • Coughing, choking or sputtering (during or after
    feeds)
  • Wet/junky/congested breath sounds (during or
    after feeds)
  • Chronic wheezing or stridor
  • Chronic pneumonia, croup, bronchitis
  • Oxygen desaturation/cyanosis (during or after
    feeds)
  • Apnea/bradycardia, etc. with feeds
  • Color change with feeds
  • Chronic fever of unclear etiology

12
Objective Assessment of Swallow FunctionFEES vs.
VFSS
  • FEES
  • Fiberoptic Endoscopic
  • Evaluation of Swallowing
  • No radiation exposure
  • Invasive-Difficulty maintaining cooperation in
    children
  • 12 mo - 4 yrs
  • Does not provide information regarding
    interaction of oral, pharyngeal and esophageal
    phases of swallowing
  • Can assess breastfeeding
  • Can view secretions
  • Equipment is portable
  • Can assess VPI and vocal cord issues
  • VFSS
  • Videofluoroscopic Swallow Study
  • (Modified Barium Swallow Study)
  • Exposure to radiation
  • Non-invasive
  • Dynamic assessment of oral, pharyngeal, and
    esophageal phases of swallowing
  • Cannot assess breastfeeding
  • Readily available in most medical centers
  • Gold standard assessment of swallowing physiology

Adapted from Arvedson Lefton-Greif, 1998
13
Management of Dysphagia
  • Establish safe oral feeding plan
  • ? Need for non-oral supplementation
  • Diet modification
  • Modify delivery method
  • Limit volume/length of time
  • Determine safest positioning
  • Counseling families
  • Collaboration with other medical providers in
    CADD is essential for decision-making and
    establishing these dietary changes

14
Congenital Anomalies of the LarynxLaryngeal
Cleft
15
Laryngeal Cleft
  • Definition
  • Communication between the posterior larynx and
    esophagus
  • Failure of the tracheoesophageal septum to
    develop

16
Case Study 1
  • -5 week old baby boy
  • -Clinical symptoms
  • -coughing while drinking thin liquids
  • -pneumonia
  • -losing weight due to poor feeding
  • -Admitted to pulmonary service at CHB
  • -NG-tube placed
  • -Referred for VFSS

17
Case Study 1 (cont)
  • -VFSS Results
  • -silent aspiration with thin liquids
  • -consistent deep laryngeal penetration with
    nectar-thick liquids
  • -nasopharyngeal reflux
  • -assumed dysphagia due to discoordinated
    suck-swallow-breathe pattern
  • -VFSS repeated no improvement x 2
  • -G-tube placed at 4 months old

18
Case Study 1 (cont)
  • -At 11 months of age
  • -Baby assessed for laryngeal cleft (DL/B)
  • -found to have Type 1 laryngeal cleft
  • -Surgically repaired
  • -Repeat VFSS 4 weeks post surgery
  • -continued aspiration of thin liquids
  • -Repeat VFSS 12 weeks post surgery

19
VFSS Clip 2Post-Surgery
20
Case Study 1 (cont)
  • -Repeat VFSS 12 weeks post surgery
  • -no aspiration with thin liquids
  • -Now Child 3 years old
  • -G-tube removed
  • -stable respiratory status

21
Classification of Laryngeal Clefts
  • According to length
  • Type I interarytenoid only
  • Type II partial cricoid
  • Type III complete cricoid
  • Type IV extending into thoracic trachea

22
Types of Laryngeal Clefts
  • Types I and II
  • Diagnosis may take months to years
  • Types III and IV
  • Diagnosed on the first day of life due to
    severity
  • High morbidity associated with Type IV

23
Classification of Laryngeal clefts
Benjamin and Inglis, 1989
24
Classification
Interarytenoid and supra glottic cleft
Type I
Type I A
  • Type I

Type II
Type I B
Complete cricoid cleft
Type I
Type III
Type I C
Type II
Type II
Type III
Cleft extending to thoracic trachea
Type III
Type IV
Type III
25
Photos courtesy of Dr. Reza Rahbar, ORL/CHB
26
Associated Anomalies
  • Aero-Digestive
  • Tracheoesophageal fistulas 20-37
  • Tracheomalacia, laryngomalacia, subglottic
    stenosis
  • Hypoplastic lungs, transposition of the great
    arteries
  • Bifid uvula, cleft lip, cleft palate

27
Associated Syndromes
  • Pallister Hall syndrome
  • Hypothalamic hamartoblastoma , Hypopituitarism,
    Polydactyly, Imperforate anus
  • Opitz-Frias syndrome
  • Cleft lip palate, Hypertelorism, Airway cleft,
    Hypospadias
  • VACTERL
  • Heart and vertebral anomalies , Esophageal
    atresia , Polydactylie, Absent left radius,
    Anorectal anomalies

28
Associated Syndromes
  • At Childrens Hospital Boston, of 81 patients
    with diagnosis of laryngeal cleft (2003-2008)
  • TEF (s/p repair) (N6)
  • Multiple Congenital Anomalies (N13)
  • Trisomy 21 (N3)
  • Fetal Alcohol syndrome (N1)
  • Tetralogy of Fallot (N2)
  • Waardenburg (N1)
  • Scimitar (N1)
  • CHARGE association (N2)
  • Opitz (N1)
  • VACTERL (N2)

29
Incidence of Laryngeal Clefts (all types)
  • Live birth 1 in 10,000-1 in 20,000
  • Incidence of laryngeal cleft increases to 0.6 in
    patients with co-existing TEF
  • Strong association with other anomalies, but also
    can exist in isolation

30
Symptoms of Laryngeal Cleft
  • Cough
  • Wheezing
  • Stridor
  • Feeding Difficulty
  • Aspiration
  • Respiratory Distress
  • Most common referral symptoms
  • Symptoms are non-specific

31
Diagnosis of Laryngeal Cleft
  • High index of suspicion based on
  • Clinical presentation
  • Medical history/Systematic chart review
  • Interpretation of preoperative studies
  • Chest radiograph, VFSS/FEES, Upper GI series
  • Thorough endoscopic airway evaluation under
    general anesthesia
  • Direct laryngoscopy, bronchoscopy, esophagoscopy
  • Endoscopist MUST perform diligent interarytenoid
    palpation

32
Diagnosis of Laryngeal Cleft
  • Airway endoscopy (in which laryngeal cleft is
    suspected) is needed for definitive diagnosis
  • VFSS does not identify laryngeal cleft, but can
    confirm symptoms of aspiration due to overall
    neuromuscular discoordination or isolated
    pharyngeal dysphagia

33
Increasing Incidence Why?
  • Literature review documents incidence of Type I
    laryngeal cleft is higher than in the past
  • 7.6 (Chien et al, 2006)
  • 6.2 (Parsons et al, 1998)
  • 7.1 (Watters Russell, 2003)
  • -Rising incidence may be due to
  • -type of patients referred to aerodigestive
    clinics
  • -higher index of suspicion for presence of
    laryngeal cleft at time of airway endoscopy (ORL
    now looking more specifically for laryngeal
    cleft)

34
Surgical Repair of Laryngeal Cleft
  • Conventional Treatment
  • Invasive surgery requiring incision in the neck
    and opening of the larynx
  • Endoscopic Repair
  • Laser and suture
  • Robotic Repair
  • Work through the oral cavity and in the
    restricted confines of the airway without
    impeding breathing
  • First performed by Dr. Reza Rahbar at Childrens
    Hospital Boston

35
Endoscopic Repair of Laryngeal Cleft
Photos courtesy of Dr. Reza Rahbar, Childrens
Hospital Boston
36
SLP PerspectiveTypical course for patient
  • PCP or other specialist refers for MBS due to
    chronic pulmonary symptoms of unclear etiology
    associated with feeding
  • VFSS Documentation of aspiration with liquids
  • SLP modifies diet (i.e,. Nectar-thick liquids) to
    promote swallowing safety, minimize aspiration
  • -(patient may need NG-tube)
  • SLP contact PCP to consider referral to CADD
  • Patient seen in CADD by ORL, GI, Pulmonary

37
SLP PerspectiveTypical course for patient
(cont.)
  • 6. CADD team completes direct laryngoscopy/bronch
    oscopy to confirm diagnosis of laryngeal cleft
  • Team decides
  • -conservative management or surgical repair??
  • IF SURGICAL REPAIR
  • 8. Maintain diet of thickened liquids for 6-8
    weeks after surgery
  • Repeat VFSS at 8-12 weeks after surgery, and
    repeat every 3 months as needed
  • If no aspiration, child resumes full oral diet

38
Who to consider for laryngeal cleft
  • Any infant or child with normal development
    (i.e., no neurogenic, medical and genetic
    etiology) who presents with isolated swallowing
    dysfunction
  • Any infant or child with history of
    Tracheoesophageal fistula (TEF) and aspiration on
    VFSS
  • Any infant or child with a history of chronic
    aspiration that does not improve over time or
    with traditional intervention

39
Conservative Monitoring
  • If no surgical intervention, conservative
    management of dysphagia
  • Diagnose and treat symptoms of GER
  • SLP works with family to modify diet
  • Close follow-up with CADD

40
Our experience at CHB
  • 2003-2008
  • 81 patients referred for evaluation of cough,
    feeding difficulty, and aspiration
  • 74 of 81 patients found to have significant
    laryngeal cleft (Type I or Type II)
  • 25 of 74 conservative monitoring with medical
    therapy
  • 49 of 74 endoscopic CO2 laser repair of Type I
    and Type II laryngeal cleft
  • (age range 8 mo-15 years)

41
Our experience at CHB
  • -Type I cleft 100 reported feeding difficulty
    with thin liquids
  • -Wheezing and chronic cough most common
    respiratory symptoms
  • -86 patients with aspiration on pre-surgical
    VFSS
  • -71 patients with Type I no aspiration on VFSS
    post-repair
  • -82 patients with Type II no aspiration on VFSS
    post repair
  • -Overall improvement noted in decreasing cough,
    normal VFSS, decreasing need for hospitalization

42
Case Study 2
  • -5 month old baby girl
  • -Clinical symptoms
  • -coughing while drinking thin liquids
  • -wheezing (worsens with feeding)
  • -frequent spit ups with feeds
  • -Referred for VFSS by PCP

43
Case Study 2 (cont)
  • -VFSS Results
  • -silent aspiration with thin and nectar-thick
    liquids
  • -no aspiration with honey-thick liquids
  • -no aspiration with purees
  • -Discuss results with PCP who refers to CADD
  • -Nutrition assessment
  • -Airway endoscopy reveals Type 1 laryngeal cleft
  • -Patient undergoes surgical repair
  • -Repeat VFSS 3 months after laryngeal cleft repair

44
VFSS Clip 2 Post-op
45
What the SLP has learned
  • Child with aspiration on VFSS who has otherwise
    normal development, who presents with any chronic
    respiratory symptoms is a candidate for CADD
    work-up
  • Maintain pre-surgical diet at least 6-8 weeks
    after surgery
  • Do not repeat VFSS too soon after
    surgery-typically wait 8-12 weeks (minimize
    radiation exposure)
  • Collaboration with Aero-digestive team is
    essential!

46
Fletcher Allen Health Care
  • Rural, community hospital
  • Level I Trauma Center
  • Level III 20-bed NICU
  • 10-bed NTU
  • 30-bed Pediatric Floor
  • PICU

47
Vermonts Academic Medical Center
  • In alliance with UVM
  • Teaching hospital
  • Vermont Oncology Center
  • Innovative care
  • Acute Rehabilitation Center-Fanny Allen

48
What is Upper Airway Obstruction (UAO)?
  • An obstruction present in the upper airway due to
    anatomic or physiologic anomalies disrupting
    respiration and the oral feeding process
  • Can be tracheal, laryngeal or pharyngeal in
    nature
  • Miller Willging, 2007

49
Upper vs. Lower Airway Obstruction
  • Pattern of stridor is key for differential
    diagnosis of UAO
  • Inspiratory stridor
  • Expiratory stridor
  • Biphasic stridor

Koufman, J Block, C. American
Journal of Speech-Language Pathology Vol.17
327-334 November 2008.
50
Upper Airway Obstruction Classifications
  • Congenital
  • Choanal atresia
  • Cleft lip
  • Micrognathia
  • Laryngomalacia
  • Neoplasm
  • Laryngeal cleft
  • Acquired
  • Croup
  • Epiglottitis
  • Tracheitis
  • Foreign body ingestion
  • Inhalation 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 1995249. Most
recently published in Miller Willging, 2007.
51
Upper Airway Obstruction Classifications
  • Acute
  • Cases are seen in emergency department
  • Cases of UAO are acquired
  • Infection and croup
  • Chronic
  • Pierre Robin sequence
  • Treacher-Collins syndrome
  • Apert syndrome
  • Beckwith-Wiedemann syndrome

52
Etiologies of UAO
  • Infection/Inflammation
  • Accounts for 90 of UAO
  • Croup
  • Epiglottitis
  • Tracheitis
  • Retropharyngeal abscess
  • Peritonsillar abscess
  • Accident/Trauma
  • Foreign body ingestion
  • Inhalation injury
  • Burns
  • External trauma to neck
  • Post-intubation

53
Types of UAO
  • Nasopharyngeal
  • Adenoid hypertrophy
  • Choanal atresia
  • Midface hypoplasia
  • Oropharyngeal
  • Macroglossia
  • Pharyngeal hypotonia
  • Cleft Lip/Palate
  • Laryngeal type
  • anomalies
  • Laryngeal cleft
  • Vocal fold paralysis
  • Neoplasm
  • Laryngeal Web
  • Laryngomalacia

54
Role of Laryngeal-Pharyngeal Reflux and GERD in
UAO
  • Erythema
  • Post-glottic edema
  • Vocal fold edema
  • Decreased sensation
  • Do protonics reverse sensory deficits?
  • Kirby Noel, 2007

55
Case Study 1
  • Pediatric Patient with Congenital UAO
    oropharyngeal and laryngeal types

56
Case 1 (timeline13 months)
  • Baby born at 41 6/7 weeks gestational age
  • 3232 grams
  • Rapid progression of Persistent Pulmonary
    Hypertension (PPHN)
  • Dx Pneumothorax and underwent Thoracentisis
  • Intubated and on high frequency oscillator at
    FAHC
  • Transferred to CHB on Day of life (DOL) 2 for
    ECMO

57
A baby on ECMO
58
Case 1 Timeline
  • DOL 0-2 at FAHC
  • DOL 2-16 at CHB
  • DOL 16 through 10/08/07 at FAHC
  • 10/08/07-11/20/07 at CHB
  • 11/20/07 readmitted to FAHC

59
Case 1 at FAHC
  • First Feeding Team consult on DOL 19
  • Baby with stridor at rest and feeding
  • Bronchoscopy revealing significant airway
    prolapse, laryngomalacia and vocal fold
    immobility
  • Baby on O2 nasal cannula
  • Weight loss
  • Breastfeeding attempts were made

60
Case 1 at CHB
  • Baby re-intubated after trial CPAP, but worsening
    respiratory distress
  • Undergoes supraglottoplasty for severe
    laryngomalacia
  • Dx subglottic stenosis
  • Fails extubation
  • Tracheostomy (Shiley 3.5 uncuffed)
  • Ng tube for poor feeding and aspiration

61
Case 1 at FAHC
  • Returns to Vermont for trach teaching
  • Does baby need G tube?
  • Cultural differences Somalian family with 6 kids
  • Mother breast fed all children
  • Baby now showing signs of oral defensiveness

62
VFSS results reveal
  • Discoordination of sucking skill
  • Trach open system
  • Laryngeal penetration with thin and nectar thick
    liquid-what are the implications?
  • Microaspiration
  • Silent in nature
  • Oral defensiveness
  • GERD

63
VFSS Looking at the whole picture
  • Oral defensiveness
  • Poor growth
  • Recurrent clinical signs aspiration with breast
    milk exiting trach site during nippling
  • Baby undergoes G tube placement for supplemental
    feedings

64
Case 1 VFSS 2 Results Reveal
  • 5 weeks later
  • Trach
  • Passy Muir Speaking Valve in place
  • No documentation of aspiration with thin liquid
  • Balance PO (per mouth) and PG (per gavage)
    feedings

65
Case 1 Re-admitted to FAHC
  • 5 weeks later
  • Recurrent upper respiratory infections
  • Post-tussive emesis
  • Bilateral Infiltrates on CXR
  • Cultural differences everyone eats out of same
    bowl with hands (and germs)

66
Case 1 Treatment Plan Interim Feeding Plan
  • When baby is healthy with no URI, emesis, PO feed
    thin liquids via bottle
  • If baby is ill, with URI, emesis, make temporary,
    alternate modifications to feeding. This could
    include NPO
  • Less conservative, would be nectar thick liquids
    and/or begin spoon feeding at 6 mo.
  • Rely on G tube as primary means of nutrition and
    hydration

67
Case 1 Happy Outcomes
  • Vocal fold mobility returns
  • Baby decannulated
  • Full PO feeder of thin liquids via sippy cup and
    bottle and soft solids
  • Will keep G tube through winter months as back-up
    for Interim Feeding Plan
  • Achieving developing milestones in timely
    age-appropriate manner

68
What we learn (and questions to ask)
  • Should breastfeeding be allowed if baby with s/s
    aspiration?
  • Is any aspiration allowed?
  • The importance of PMV to restore healthy upper
    airway and closed system
  • VFSS is only ONE window in time and only a small
    piece to our overall assessment
  • How to embrace and manage cultural differences

69
Case Study 2
  • Pediatric Patient with Congenital UOA acute,
    oropharyngeal type

70
Case 2 (timeline 9 months)
  • 10 month old admitted to FAHC with respiratory
    distress and neck edema
  • Tracheostomy
  • Biopsied revealed ganglioneuroblstoma
  • Baby received cycle of carboplatin and etoposide,
    which decreased size of tumor temporarily
  • Mass increased in size, compromising her airway
    and necessitating intubation

71
Case 2 at CHB
  • Transferred to CHB for debulking of the mass
  • Subsequent pathology reveals a benign cervical
    right neck neuroectodermal lesion
  • Most notable complication after debulking is poor
    feeding
  • VFSS in Boston reveals silent aspiration of puree
    and all liquid consistencies

72
Case 2
  • MRI reveals residual tumor in posterior fossa and
    neck after debulking
  • Large enhancing mask in the right parapharyngeal
    space
  • Neurosurgery deferred further resection due to
    low growth potential and stable size on MRI
  • Sub total resection implications for long term
    and feeding?

73
Case 2 VFSS results reveal
  • Silent aspiration with purees and liquids
  • Delayed initiation of swallow
  • Significant pharyngeal weakness and poor
    pharyngeal constriction
  • Post-swallow residue

74
Case 2 Treatment plan
  • NPO recommended to PCP
  • Baby underwent G tube placement given likely
    long-term nature of impairment
  • Parents extremely frustrated by NPO status
  • Oral Stimulation program and tactile play with
    textures in absence of PO intake

75
What we learn (and questions to ask)
  • Understanding prognosis is essential to make
    recommendations
  • Is any aspiration allowed?
  • How do we balance the need for NPO status with
    developmental feeding needs and family desires to
    feed the child?
  • Is NPO a feasible and reasonable recommendation?

76
Case Study 3
  • Pediatric patient with Congenital UAO laryngeal
    anomaly type

77
Case 3 (timeline 3 months)
  • Born at 41 weeks gestational age
  • 4095 grams LGA
  • C-section due to fetal distress
  • Respiratory distress at birth
  • Consulted Feeding Team at DOL 11 (recommended
    NPO)
  • Stridor
  • Intubated

78
Case 3
  • Feeding Team re-consulted 3 weeks later after
    successful extubation
  • s/p epiglottoplasty and supraglottoplasty for
    redundant tissue
  • Baby with weak cry, aphonic at times
  • S/s aspiration during nippling
  • Inspiratory stridor continues
  • MRI was normal

79
Laryngoscopy results reveal
  • Larynx remarkable for enlarged arytenoid
    cartilages
  • Paradoxical VF movement with each inspiration
  • A-P collapse of trachea was observed dynamically
  • No clear pulsatile compression of trachea was seen

80
VFSS results reveal
  • Day after laryngoscopy
  • Aspiration with thin liquids
  • No evidence of aspiration with nectar thick
    liquids
  • Ng tube in place

81
Case 3 Treatment Plan
  • Recommendations for baby to nipple nectar thick
    liquids via slow flow nipple
  • PO ad lib when baby showing interest in nippling
  • Gavage feedings continue to supplement until
    adequate volumes consumed PO
  • Repeat VFSS in 6-8 weeks

82
Case 3 Differential Diagnosis
  • Paradoxical Vocal Fold Movement (PVFM)
  • Known an a functional or non-organic disorder
  • Etiology suggests psychopathology
  • Laryngeal dystonia?
  • Neurogenic etiology?

83
Case 3 Literature review
  • Heatley Swift, 1996 report a case of 4 month
    old with PVCD related to underlying GERD
  • Omland Brondbo, 2008 report 4 cases infant of
    BVCP vs PVCM
  • EMG can provide further diagnostic info if the
    laryngeal muscles have normal electrical activity

84
Case 3
  • PVFM classified as Cortical or Upper Motor Neuron
    OR Nuclear and Lower Motor Neuron
  • In infant with no clear organic etiology, cant
    be classified under above
  • GERD
  • Laryngeal Dyskinesia (lack of abductory movement
    with inhalation)

85
Final Thoughts on what we learn
  • How do we best manage aspiration in pediatrics?
  • Least restrictive, safest diet
  • Do no Harm principle
  • Is any aspiration allowed?
  • Colin Wallis, MD, Pediatric Pulmonologist, of
    Great Ormond Street Hospital asks Is there
    damage from aspiration? not is it occurring

86
Final Thoughts
  • Upper airway obstruction is an area where the
    medical SLP needs expertise
  • Teaming with ENT, Pulmonary, GI, Radiology is
    essential
  • Continuing Education for SLP and always asking
    questions is essential

87
Esophageal Etiologies of Pediatric Dysphagia
88
South Shore Hospital
  • Regional Medical Center SE MA
  • 4,800 births/year
  • 380 inpatient beds
  • Level 2 Trauma Center
  • 10 Level 3 NICU
  • 20 Level 2 NICU
  • Pediatric Floor
  • Outpatient Pediatric Feeding Clinic
  • Clinical Affiliations

89
Anatomy of the Esophagus
  • Refer to the esophagus as the proximal and
    distal portions.
  • Proximal third of the esophagus- striated muscle
  • Distal third of the esophagus (and remainder of
    the gut)-smooth muscle

90
Anatomy Physiology of the Esophagus
  • After the upper esophageal sphincter relaxes, a
    reflexive, peristaltic primary stripping wave
    is initiated.
  • This propels the bolus distally
  • Amplitude of this wave is higher in the distal
    esophagus
  • Traveling at 3-5 cm per second (Rubenstein, J.)

91
Anatomy Physiology of the Esophagus
  • Stripping wave initiated by the oropharyngeal
    swallow causing the LES to relax
  • Remains open until the stripping wave has arrived
    at the LES
  • Secondary stripping waves originate from within
    the proximal esophagus and propel any residual
    bolus into the stomach

92
Esophageal Dysphagia Occurs When
  • Amplitude of the stripping wave is too low
  • Velocity of the wave is too slow or too fast
  • The wave is so fast the UES and LES contract
    simultaneously
  • The UES or LES fail to relax
  • There is mechanical obstruction that cannot be
    overcome by the stripping wave

93
Etiologies of Esophageal Dysphagia
  • Differential diagnosis from oropharyngeal
    etiologies
  • Co-occurring oropharyngeal and esophageal
    etiology
  • Careful history is important
  • Child should be asked to point with one finger
    to the area they feel there is hang up of the
    bolus

94
Etiologies of Esophageal Dysphagia
  • 2 Main Etiologies of Esophageal Dysphagia
  • Mechanical Causes
  • Motility Disturbances
  • Food impaction is more frequent with mechanical
    causes
  • Patients with mechanical obstruction can
    typically still swallow liquids
  • More pronounced solid food dysphagia

95
Etiologies of Esophageal Dysphagia
  • Mechanical disturbance- usually localize the
    hang up of the food distal to the larynx
  • Oropharyngeal etiology localize the hang up
    proximal to the larynx
  • Often not possible to determine etiology and
    hence diagnostic testing is indicated
  • If SLP finds evidence of an esophageal disorder a
    recommendation is made for appropriate referral
    to a specialist

96
Case Study of a Pediatric Patient with Mechanical
Obstruction
97
VACTERL or VATER Association
  • Acronym used to describe a series of
    characteristics which have been found to occur
    together
  • V vertebrae, which are abnormal (70 patients)
  • A anal atresia/ imperforate anus (55 patients)
  • C cardiac defects (75 patients)
  • T tracheoesophageal fistula (TEF), an abnormal
    connection between the trachea and the esophagus
  • E esophageal atresia, (EA) the esophagus does
    not connect to the stomach
  • R renal defects
  • L limb (arm) defects

98
VACTERL Association
  • Babies diagnosed with VACTERL have at least 3 or
    more of the anomalies.
  • Wide range of manifestations
  • EA with TEF occurs in 70 patients
  • EA/TEF can also occur as an isolated defect
  • EA/TEF as an isolated condition occurs in 1 in
    3,000-5,000 births
  • Typically have normal development and intelligence

99
Esophageal Atresia (EA) Tracheoesophageal
Fistula (TEF)
  • Presence of EA suspected in the post natal period
  • Newborn presents with excessive salivation
  • Coughing, choking, sneezing.
  • Upon oral feeding the infant struggles and may
    become cyanotic
  • Respiratory distress

100
EA TEF
  • Congenital EA represents failure of the esophagus
    to develop as a continuous passage.
  • TEF represents an abnormal opening between the
    trachea and the esophagus
  • DX is made, all oral feedings are stopped
  • Surgical repair within the first days of life

101
Case Study
  • 4 year 11 month old male with VACTERL Association
  • Followed since infancy through Swallowing
    Disorders Program
  • Post natal diagnosis of EA/TEF s/p surgical
    repair on DOL3
  • Cardiac diagnosis of Tetrology of Fallot s/p
    repair (multiple staged repairs)
  • G-tube placed in infancy s/p EA/TEF repair

102
Case Study
  • History of aspiration with thin and nectar thick
    liquids on VFSS first identified at 6 months of
    age.
  • Cleared to orally feed purees only.
  • G-tube to meet hydration, nutritional needs
  • No improvement with swallow function over the
    years
  • Referred to CADD clinic at 3 years of age

103
Case Study
  • Dx with type 1 laryngeal cleft (LC)
  • S/P endoscopic repair of LC at 3 years 6 months
    old
  • Pre-surgically patient eating diet of purees,
    soft dissolvables and solids (hamburger, pasta,
    grilled chicken, green beans) in limited volume.
  • All liquids via g-tube

104
Case Study
  • 6 months s/p LC repair patients VFSS revealed
    improvement in underlying swallow function
  • Patient cleared to take thin and nectar thick
    liquids
  • Patient co-existing sensory based feeding disorder
  • Volume of solids limited due to texture
    sensitivity, hyper-sensitive gag reflex
  • Reduced efficiency with chewing
  • Receiving motor/sensory based feeding therapy

105
Case Study
  • Patient returns for repeat VFSS at 4 years 11
    months due to complaints food is getting stuck
  • Patient points to area below the larynx/chest
    area
  • Mother describes patient as forceful swallow
    with facial grimacing, decreased interest in
    solids, decreased volume of solids
  • Asymptomatic with liquids

106
Videofluoroscopic Results Reveal
  • No evidence of aspiration with thin liquids
  • No evidence of aspiration with solids which pass
    from the pharynx through the UES
  • Normal pharyngeal phase of swallowing
  • (confirm this on video clip)

107
Videofluoroscopic Results Reveal
  • Esophageal phase reveals moderate anastomotic
    narrowing at the site of the EA repair
  • Piece of chicken impacted at the site of the
    anastomosis
  • Liquid wash downs required to clear the solid
  • Continued ingestion of solids can lead to back
    up

108
Treatment Plan
  • Attending radiologist at VFSS paged the GI
    physician to discuss dx of a stricture
  • Patient placed on diet of liquids/purees only
    until surgical decision
  • Patient underwent dilatation of the esophagus
  • 1 week s/p dilatation patient resumed and
    tolerated solid foods.
  • Avoid hard to chew solids high risk choking
    foods
  • Avoid gummy foods
  • Smaller, more frequent meals

109
What we learn..
  • We must look beyond the pharyngeal swallow
  • Most neonates with EA/TEF have some degree of
    esophageal dysmotility
  • Strictures at the site of the anastomosis are
    common and may require mulitple dilatations

110
What we learn..
  • Solid food dysphagia often the first sign the
    stricture has re-occurred
  • As children mature they are in tune to their
    esophagus and can describe symptoms
  • Many older children will self-restrict their diet
  • VFSS is an effective procedure for identifying
    the stricture

111
What we learn..
  • VFSS is only one instrumental evaluation that may
    give rise to esophageal dysphagia
  • Further diagnostic testing may include
  • Upper GI series
  • Esophagoduodenscopy (EGD)

112
Case Study of a Pediatric Patient with Motility
Disturbance
113
Case Study
  • 3 month old infant referred for VFSS by pediatric
    otolaryngologist (ORL)
  • Presenting symptoms coughing and choking during
    feeding
  • Mother reports gasping during bottle feeds
  • PCP placed infant on Zantac with no improvement
    in symptoms

114
VFSS Results
  • Documentation of silent aspiration with thin,
    nectar thick and honey thick liquid
  • Build up of residue above the upper esophageal
    sphincter
  • Failure of the UES to relax
  • Aspiration results from a build-up/ spill over of
    liquid due to failure of the UES to relax

115
VFSS Results
  • Coordination of suck swallow breathe altered
  • Airway protection is compromised
  • Co-occurrence of oropharyngeal dysphagia along
    with an esophageal phase dysphagia

116
Treatment Plan
  • Attending radiologist at VFSS paged referring ORL
    physician
  • Patient made NPO by referring physician
  • Patient admitted to the hospital
  • GI service consulted

117
Cricopharyngeal Dysfunction
  • Cricopharyngeal dysfunction as a cause of
    oropharyngeal dysphagia in infancy
  • Multi-phase dysphagia
  • Results from 2 conditions
  • Failure of relaxation of the upper esophageal
    sphincter (UES) and/or
  • Cricopharyngeal incoordination
  • (Mihailocivc, T, Perisic, V.N., 1992)

118
Cricopharyngeal dysfunction
  • Manifested by swallowing difficulties
  • Nasopharyngeal regurgitation
  • Coughing, choking
  • Aspiration pneumonia
  • Diagnosis typically made by radiography and
    motility studies
  • Cricopharyngeal dysfunction caused by either
    primary UES motility disorder or
  • May be associated with central nervous system
    diseases

119
Case Study Cont
  • Patient admitted to the hospital for nasogastric
    tube feedings
  • GI work up revealed cricopharyngeal achalasia
  • Patient underwent esophageal dilatation during
    admission
  • D/C home full oral feeder with resolution of
    symptoms
  • f/u with GI physician

120
Repeat VFSS
  • 8 weeks s/p dilatation patient returned for
    repeat VFSS
  • Full PO feeding of thin liquid via bottle in the
    interim
  • Spoon feeding had been initiated
  • No intermittent respiratory symptoms
  • VFSS reveals qualitative improvement but
    continued pooling at the UES
  • Aspiration with thin liquids documented
  • No aspiration with spoon feeding of purees but
    pooling

121
Treatment Plan
  • Medical team met to discuss the case
  • Cleared to continue on thin liquids with pacing
    strategy in place
  • Alter pacifier for non-nutritive sucking to help
    clear the residue with subsequent swallows
  • Nectar thick liquids not indicated due to
    increased build up
  • Return to GI to discuss need for re-dilatation

122
Treatment Options for Cricopharyngeal Achalasia
  • Cricopharyngeal myotomy
  • Surgical division of the muscle
  • Renders the muscle inactive
  • Often as last option
  • Dilatation
  • Single balloon catheter dilatation proven to be
    successful
  • Less invasive than myotomy
  • Results apparent 24-48 after procedure
  • Patient may require repeat dilatations
  • Botox injection to the UES
  • More commonly used in the adult population
  • Efficacy of botox use to LES in pediatrics
  • Botox effect may wear off over time
  • Need to re-inject
  • Risk of injecting the wrong site/muscle

123
Case study Cont.
  • Different treatment modality trialed
  • Patient received Botox injection to the UES
  • Due to complications from Botox injection patient
    required hospital admission for NG tube placement
  • Decision was made for g-tube placement and repeat
    dilatation to the UES due to on-going dysphagia
    and aspiration

124
Case Study Cont.
  • Improvement in underlying swallow function s/p
    second dilatation
  • Aspiration resolved
  • Patient progressed to mashed and soft table foods
    (1 year of age)
  • Soft solids pass through site of dilatation
    without hold up
  • Cricopharyngeal muscle still prominent but with
    improved coordination

125
What we learn
  • ASHA Guidelines for Speech Language Pathologists
    Performing Videofluoroscopic Swallow Studies
    (2004) states
  • Clinicians should be aware that oropharyngeal
    swallow function is often altered in patients
    with esophageal motility disorders and
    dysphagia.
  • SLPs have knowledge and skills to recognize
    patient signs and symptoms associated with
    esophageal dysphagia

126
What we learn
  • Results of the instrumental oropharyngeal swallow
    evaluation should include an esophageal
    screening.
  • Results of the esophageal screening may reveal a
    structural or motor abnormality suggesting that a
    referral to a Gastroenterologist may be
    indicated. (Easterling, 2007, Perspectives)
  • SLPs should have sufficient knowledge of
    esophageal structure and function to make an
    appropriate referral and plan cooperative
    management

127
Seeking Resources
  • Accessibility to a pediatric medical center that
    offers multidisciplinary team approach such as
    The Center for Aerodigestive Disorders.
  • Underscores the need for graduate training in
    pediatric dysphagia
  • Course work and clinical practicum
  • Collaboration among hospital and community based
    SLPs for carry over of safe and successful
    feeding plan in the home setting.

128
Questions?
129
How to contact us
  • Jennifer.perez_at_childrens.harvard.edu
  • Alanalowry_at_vtmednet.org
  • Kara_Larson_at_sshosp.org

130
References
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