Title: Pediatric Dysphagia: Complex Case Studies of Patients with Aerodigestive Disorders 2421
1Pediatric 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
23 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
3Childrens 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
4Aero-digestive Disorders
- Congenital Anomalies of Larynx
- Upper Airway Obstruction
- Esophageal Obstructive Disorders
5Center for Aero-Digestive Disorders
6Center 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
7Center for Aero-Digestive Disorders
- Providing comprehensive services to infants and
children with complex problems involving the - Airway
- Pulmonary tract
- Upper digestive tract
8Center 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
9Center 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
10Importance 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
11Center 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
12Objective 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
13Management 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
14Congenital Anomalies of the LarynxLaryngeal
Cleft
15Laryngeal Cleft
- Definition
- Communication between the posterior larynx and
esophagus - Failure of the tracheoesophageal septum to
develop
16Case 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
17Case 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
18Case 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
-
19VFSS Clip 2Post-Surgery
20Case 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
21Classification of Laryngeal Clefts
- According to length
- Type I interarytenoid only
- Type II partial cricoid
- Type III complete cricoid
- Type IV extending into thoracic trachea
22Types 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
23Classification of Laryngeal clefts
Benjamin and Inglis, 1989
24Classification
Interarytenoid and supra glottic cleft
Type I
Type I A
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
25Photos courtesy of Dr. Reza Rahbar, ORL/CHB
26Associated Anomalies
- Aero-Digestive
- Tracheoesophageal fistulas 20-37
- Tracheomalacia, laryngomalacia, subglottic
stenosis - Hypoplastic lungs, transposition of the great
arteries - Bifid uvula, cleft lip, cleft palate
27Associated 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
28Associated 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)
29Incidence 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
30Symptoms of Laryngeal Cleft
- Cough
- Wheezing
- Stridor
- Feeding Difficulty
- Aspiration
- Respiratory Distress
- Most common referral symptoms
- Symptoms are non-specific
31Diagnosis 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
32Diagnosis 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
33Increasing 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)
34Surgical 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
35Endoscopic Repair of Laryngeal Cleft
Photos courtesy of Dr. Reza Rahbar, Childrens
Hospital Boston
36SLP 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
37SLP 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
38Who 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
39Conservative 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
40Our 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)
41Our 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
42Case 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
43Case 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
44VFSS Clip 2 Post-op
45What 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!
46Fletcher Allen Health Care
- Rural, community hospital
- Level I Trauma Center
- Level III 20-bed NICU
- 10-bed NTU
- 30-bed Pediatric Floor
- PICU
47Vermonts Academic Medical Center
- In alliance with UVM
- Teaching hospital
- Vermont Oncology Center
- Innovative care
- Acute Rehabilitation Center-Fanny Allen
48What 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
49Upper 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.
50Upper 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.
51Upper 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
52Etiologies 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
53Types 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
54Role 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
55Case Study 1
- Pediatric Patient with Congenital UAO
oropharyngeal and laryngeal types
56Case 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
57A baby on ECMO
58Case 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
59Case 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
60Case 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
61Case 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
62VFSS 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
63VFSS 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
64Case 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
65Case 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)
66Case 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
67Case 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
68What 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
69Case Study 2
- Pediatric Patient with Congenital UOA acute,
oropharyngeal type
70Case 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
71Case 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
72Case 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?
73Case 2 VFSS results reveal
- Silent aspiration with purees and liquids
- Delayed initiation of swallow
- Significant pharyngeal weakness and poor
pharyngeal constriction - Post-swallow residue
74Case 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
75What 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?
76Case Study 3
- Pediatric patient with Congenital UAO laryngeal
anomaly type
77Case 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
78Case 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
79Laryngoscopy 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
80VFSS results reveal
- Day after laryngoscopy
- Aspiration with thin liquids
- No evidence of aspiration with nectar thick
liquids - Ng tube in place
81Case 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
82Case 3 Differential Diagnosis
- Paradoxical Vocal Fold Movement (PVFM)
- Known an a functional or non-organic disorder
- Etiology suggests psychopathology
- Laryngeal dystonia?
- Neurogenic etiology?
83Case 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
84Case 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)
85Final 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
86Final 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
87Esophageal Etiologies of Pediatric Dysphagia
88South 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
89Anatomy 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
90Anatomy 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.)
91Anatomy 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
92Esophageal 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
93Etiologies 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
94Etiologies 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
95Etiologies 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
96Case Study of a Pediatric Patient with Mechanical
Obstruction
97VACTERL 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
98VACTERL 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
99Esophageal 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
100EA 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
101Case 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
102Case 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
103Case 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
104Case 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
105Case 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
106Videofluoroscopic 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)
107Videofluoroscopic 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
108Treatment 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
109What 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
110What 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
111What 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)
112Case Study of a Pediatric Patient with Motility
Disturbance
113Case 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
114VFSS 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
115VFSS Results
- Coordination of suck swallow breathe altered
- Airway protection is compromised
- Co-occurrence of oropharyngeal dysphagia along
with an esophageal phase dysphagia
116Treatment Plan
- Attending radiologist at VFSS paged referring ORL
physician - Patient made NPO by referring physician
- Patient admitted to the hospital
- GI service consulted
117Cricopharyngeal 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)
118Cricopharyngeal 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
119Case 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
120Repeat 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
121Treatment 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
122Treatment 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
123Case 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
124Case 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
125What 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
126What 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
127Seeking 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.
128Questions?
129How to contact us
- Jennifer.perez_at_childrens.harvard.edu
- Alanalowry_at_vtmednet.org
- Kara_Larson_at_sshosp.org
130References
- ASHA 2008 Upper Airway Obstruction References
- 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.) - Koufman, J Block, C. American Journal of
Speech-Language Pathology Vol.17 327-334 November
2008 - 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
265803-807. - Mathers-Schmidt, B (2001) Paradoxical Vocal Fold
Motion A Tutorial on a Complex Disorder and the
Speech-Language Pathologists Role. American
Journal of Speech-language Pathology 10 111-125. - 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. - Ibrahim, W, Gheriani H, Almohamed, A, Raza, T
(2007) Paradoxical vocal cord motion disorder
past, present and future. 83 164-172 - Haibeck, L, Mandell, D (2008) The Aerodigestive
Clinic Multidisciplinary Management of Pediatric
Dysphagia. ASHA Division 13 Perspectives on
Swallowing and Swallowing Disorders 17 101-109. - Arvedson, J (2008) Food for Thought on Pediatric
Feeding and Swallowing. ASHA Division 13
Perspectives on Swallowing and Swallowing
Disorders 17 110-118. - Miller, C, Willging, JP (2007) The Implications
of Upper-Airway Obstruction on Successful Infant
Feeding. Seminars in Speech and Language
28190-203. - Zaichkin, J (2002) Newborn Intensive Care What
every parent needs to know. 2nd edition. NICU
INK Book Publishers, Santa Rosa, CA. - Kirby, M, Noel, R. (2007) Nutrition and
Gastrointestinal Tract Assessment and Management
of Children with Dysphagia. Seminars In Speech
and Language 28 180-189.
131References
- Arvedson, J. Lefton-Greif, M. 1998. Pediatric
Videofluoroscopic Swallow Studies A
Professional Manual with Caregiver Guidelines.
San Antonio, TX Communication Skill Builders - Rahbar, R. et al. The presentation and
management of laryngeal cleft A 10-year
experience. Arch Otolaryngol Head Neck Surg.
2006 132 1336-1341. - Parsons, DS. et al. Type I posterior laryngeal
clefts. Laryngoscope. 1998 108 403-410. - Watters K., J. Russel. Diagnosis and management
of type I laryngeal cleft. International Journal
of Pediatric Otorhinolaryngology. 2003 67
591-596. - Chien, W. et al. Type I laryngeal cleft
Establishing a functional diagnostic and
management algorithm. International Journal of
Pediatric Otorhinolaryngology. 2006, 70
2073-2079. - Benjamin B. A Inglis. Minor congenital laryngeal
clefts diagnosis and classification. Ann Otol
Rhinol Laryngol 1989 98 417-420. - Bosely, M. et al. The utility of fiberoptic
endoscopic evaluation of swallowing (FEES) in
diagnosing and treating children with Type I
laryngeal clefts. International Journal of
Pediatric Otorhinolaryngology. 2006 70 339-343.
132References
- Walton, J.M. Tougas, G. Botulinum toxin use in
pediatric esophageal achalasia A case report.
Journal of Pediatric Surgery, 1997, 801-920. - Massey, Benson, T. Treatment of Dysphagic
Symptoms Resulting from Esophageal Disorders,
Perspctives on Swallowing and Swallowing
Disorders (Dysphagia) Volume 16, Number 4, Dec.
2007, 14-18. - Rubenstein, Joel, H. Esophageal Etiologies of
Dysphagia A Guide for SLPs. Perspctives on
Swallowing and Swallowing Disorders (Dysphagia)
Volume 16, Number 4, Dec. 2007, 1-6. - Easterling, Caryn. Food for Thought What happens
in the esophagus, doesnt stay in the
esophagus..So who cares? A Guide for SLPs.
Perspctives on Swallowing and Swallowing
Disorders (Dysphagia) Volume 16, Number 4, Dec.
2007, 19-20 - Mihailovic, T., Perisic, V.N. Balloon dilatation
of cricopharyngeal achalasia. Pediatric Radiology
(1992), 22 522-524. - Kalincinski, P., Dluski, E., Drewniak, T and
Kaminski, W. Esophageal manometric studies in
children with achalasia before and after
operative treatment. Pediatric Surgery (1997), 12
, 571-575. - Lopez-Alonso, M, Moya, Maria Jose, Cabo, J.A.,
Ribas, J, Macias, Maria del, Silny, J, Sifrim, D.
Twenty-four Hour Esophageal Impedence- pH
Monitoring in Healthy Preterm Neonates Rate and
Characteristics of Acid, Weakly Acidic, and
Weakly Alkaline Gastroesophageal Reflux.
Pedaitrics, Vol. 118, No. 2 August 2006,
e299-e308. - Henry, Shawna M, RNC, MSN, RN. Discerning
Differences Gastroesophageal Reflux and
Gastroesophageal Reflux Disease in Infants. The
Art and Science of Caring Focus on the Family
The Long Road Home. (2004) Volume 4 (4) p.
235-247.