dysphagia in children - Peyton Manning Children`s Hospital

10/5/2010
DYSPHAGIA IN CHILDREN
Hamaker, Sufi, Akanli, Escobar, Wilner, Maisel, Schaefer, Hannah
10/5/2010
Objectives
• Learn different ways to evaluate swallowing
• Common and uncommon causes of dysphagia in children
• Advantages of Multidisciplinary Approach
10/5/2010
Dysphagia
• Involves both feeding and swallowing
• Can be multisystem disorder: motor and sensory functions; neurologic, cardiac, respiratory and gastrointestinal systems
• Feeding involves oral stage function (suck, chew, cup drink, sensory averse responses)
• Swallowing is primarily pharyngeal and involves risk of aspiration.
10/5/2010
What is Dysphagia?
3 types of dysphagia
Oral Phase
Pharyngeal Phase
Esophageal Phase
10/5/2010
What is Dysphagia?
Oral phase‐ difficulty sucking, chewing, moving food or liquid into the throat, and/or controlling bolus in mouth (a‐b)
10/5/2010
What is Dysphagia?
Pharyngeal phase‐ difficulty triggering the swallow, squeezing the food down the throat, closing off the airway to prevent penetration and aspiration and closure of nasopharynx. (c‐d)
10/5/2010
What is Dysphagia?
Esophageal phase‐ difficulty relaxing and tightening the UES and LES and/or reduced esophageal motility (e‐f)
10/5/2010
What causes dysphagia?
• Structural Etiologies
• Neurologic Etiologies
• Respiratory Compromises (cardiac, pulmonary, or airway anomalies)
• GI : mucosal , anatomical, functional
• Maladaptive Feeding Behaviors
NOT mutually exclusive with interacting characteristics
10/5/2010
STRUCTURAL ETIOLOGIES
• Choanal atresia
• Craniofacial anomalies (cleft lip and palate, micrognathia, macroglossia)
• Laryngeal Cleft
• Laryngomalacia, Tracheomalacia, Pharyngo‐
malacia
• Glottic web/Subglottic stenosis
• Vocal fold paralysis (risk after cardiac repair)
* Multiple other causes
10/5/2010
NEUROLOGIC ETIOLOGIES
• Prematurity
• Cerebral Palsy
• Chiari Malformation
• Brain Tumor
• Stroke
• Muscular Dystrophy
• Congenital Disorders and Myopathies
* Multiple Other Causes
10/5/2010
RESPIRATORY EFFORT AND DYSPHAGIA
• Swallowing and breathing are mutually exclusive 10/5/2010
Swallow and breathing are mutually exclusive:
• One requires an open glottis and the other closed glottis and elevation of the larynx
• Increases in respiratory rate correlates to decreased time for oral phase of swallowing
• Lung compliance reduces as fibrosis and pneumonitis associated interstitial edema and surface tension increase
10/5/2010
Swallow and breathing are mutually exclusive:
• Lower lung compliance leads to increased work of breathing to maintain same tidal volume and homeostasis
• Increased respiratory correlates to greater intra‐abdominal pressure fluxuations and so increased gastro‐esophegeal reflux episodes (based on intubated Doberman studies)
• Airway obstruction acts in a similar fashion as fibrosis and is overcome by increased work of breathing causes prolonged exhalation times.
10/5/2010
Summary
Increased airway edema, gastroesophageal
reflux disease, and decreased swallow time:
• All lead to increase in aspiration risk and a cycle of worsening lung disease that leads to chronic lung destruction and ultimate respiratory failure
• So treating just one aspect of the problem will not break the cycle
• A combined approach is the only way to change the disease course
10/5/2010
GI ETIOLOGIES
• Mucosal
– Allergic
– Bacterial/Fungal
– Caustic
• Anatomic
– Web
– Malrotation
– TEF
– Stricture/Atresia
• Functional
– Gastroparesis
– Esophageal dysmotility
– Ileus
– Foreign Body
10/5/2010
Prematurity Feeding
•
•
•
•
•
Suck = 15 ‐18 wks gestation
Swallow= 12‐14 wks gestation
Breathe = 26‐30 wks gestation
Coordination = 34‐40 wks gestation
Compensatory Strategies =Often necessary to successfully feed
10/5/2010
Maladaptive Behaviors
•
•
•
•
•
•
•
•
Refusal to be in highchair
Batting at Spoon
Crying thru meal
Spitting food out/severly limited diet
Food pocketing
Throwing food
Gagging/Vomitting
Lack of attention to food
10/5/2010
How to assess dysphagia?
• Clinical Evaluation
• Bedside swallow • Cervical Auscultation
• Limitations: CANNOT rule out aspiration
• N= 14, 8 of 9 aspirations noted were silent
(Newman, et al. Pediatrics Dec 2001. 108(6):e106‐109)
• Videofluoroscopy Swallow Study (VFSS)
• Fiberoptic Endoscopic Evaluation of Swallowing (FEES)
10/5/2010
Clinical Observation of Skills
(aka Bedside Evaluation)
•
•
•
•
•
•
Medical History
Feeding History (parent report)
Current diet
Basic Cranial Nerve Assessment
Reflexive Assessment (root, gag, bite)
Observation of feeding (includes primarily oral but can make inferences regarding the pharyngeal as silent aspiration cannot be ruled out during bedside evaluation)
10/5/2010
VFSS
•
•
•
•
Completed in Radiology with Speech‐
Language Pathologist, Radiology Tech, and Radiologist or Radiology Assistant at Women’s and 86th Street
Barium mixed into liquids, purees, and solids to observe the oral, pharyngeal, and cervical esophageal stages of the swallow
Clear evaluation of airway protection before, during and after the swallow
Visualization of upper esophageal structure and function
10/5/2010
VFSS: PROS AND CONS
•
•
•
•
PROS
View ALL phases of swallow
No discomfort to child
May try compensatory techniques
Able to easily note penetration or aspiration in rapid chain swallowing
•
•
•
•
•
CONS
Radiation exposure
Taste of barium/Viscosity may change with barium
Limited time without being able to view entire meal in realistic setting
Unable to visualize the swallow during breast feeding
Staffing/Time determined by Radiology Department
10/5/2010
FEES
• Completed with Speech‐Language Pathologist and ENT in Outpatient Services at 86th Street
• Endoscope transnasally passed to view pharyngeal and laryngeal structures at rest, before, and after the swallow
10/5/2010
FEES: PROS AND CONS
•
•
•
•
•
•
•
•
PROS
Assessment of secretion management
Clear view of structures
Portability
Assess sensory function
Detect signs of reflux irritation
Able to assess swallow during breast feeding
No time limits
Does not require alteration of food or liquid with Barium
•
•
•
•
•
•
•
CONS
Discomfort with scope
Presence of scope may impact swallow and/or trigger gagging
Focus limited to pharyngeal phase of swallow
Risk for laryngospasm or vasovagal response
WHITE OUT: view disappears during moment of swallow –
frequent white out with chain swallowing in infants
Not as many trained therapists
Children require ORL because anomalies
10/5/2010
FIELD OF VIEW
VFSS
•
•
•
•
Oral Cavity
Oronasal cavity
Pharynx
Upper Esophagus
FEES
•
•
•
•
•
Nasal cavity
Nasopharynx
Hypopharynx
Endolarynx
Anterior wall of trachea
10/5/2010
Interpretation Comparisons
PARAMETERS
VSS
FEES
Velopharyngeal Close
Good
Excellent
VC Mobility/ Close
Good/fair
Excellent
Laryngeal Elevation
Excellent
Good
Oral phase
Excellent
Fair
Pharyngeal Squeeze
Good
Excellent
Pooling of Secretions
Poor
Excellent
Premature Spillage
Excellent
Excellent
Penetration
Good
Excellent
Aspiration
Excellent
Good (loss of view)
Residue
Excellent
Excellent
10/5/2010
WHICH STUDY IS BETTER?
• Both are valuable tools in assessment of dysphagia • One should be used vs. the other given the patient’s history, diagnoses, and goals
• Consult Speech Pathology for BSE
– SLP will determine which test will be more effective
10/5/2010
Aerodigestive Clinic
• Various versions throughout the country, ours at PMCH includes:
• ENT
• Pulmonary
• GI
• Genetics/Newborn Follow‐Up
• Physiatry
• Speech Therapy
10/5/2010
Other Centers Across Country • May or may not include same as ours or others such as:
– Pediatric Surgery
– Nutrition
– Dietary
– Interdisciplinary Feeding Clinic
– Psychology
10/5/2010
Multidisciplinary Goals
• Allows cross‐fertilization of thoughts and ideas
• Coordination of care
• Saves multiple anesthetics • Varied approaches to same problem
• Family “hears” the same plan…all on same page with education and on going evaluation
10/5/2010
Case Study 1
• 19 month old boy with recurrent croup, cough, intermittent vomiting & dysphagia
• PCP notes he ate egg for the first time and developed choking, then wheezing and was seen in the ER and was treated for asthma
• Cough and choking on food persisted
• PMH: Pneumonia x 2 in 3 months
• Referred to ENT and GI
10/5/2010
Case Study 1
•
•
•
•
•
•
ENT Consultation
Recurrent croup, chronic cough
Meds: Albuterol PRN
PE: 1+ tonsils, normal exam
Suspected reflux, agreed with GI consult
Reflux precautions reviewed
10/5/2010
Case Study 1
• GI Consultation
• Chronic coughing, choking with feeding, recurrent vomiting and sluggish weight gain
• He chokes, sputters and coughs with liquid
• Daily emesis for months
• PMH: infantile reflux
• UGI barium: Normal
10/5/2010
Case Study 1
• Speech Therapy Evaluation
• Moderate oral, moderate‐severe pharyngeal dysphagia
• Pyriform sinus pooling before swallow, decreased laryngeal closure resulting in penetration/silent aspiration with thin liquids
• Rec: soft toddler diet with nectar thick liquids
• Feeding team evaluation
• First steps therapy
10/5/2010
EGD
10/5/2010
Follow up 5 weeks later:
• After starting swallowed fluticasone and oral lansoprazole, symptoms resolved
• Biopsies normalized
10/5/2010
Results Case Study 1
• Peripheral eosinophilia (12%)
• Egg white IgE and Milk IgE Class 2
• Esophagus:
– Marked eosinophilia, over 80 eos/hpf in – Basal cell hyperplasia
• Diagnosis: Eosinophilic Esophagitis
10/5/2010
Pediatric Eosinophilic Esophagitis
• Reflux symptoms most common presentation
– Vomiting
– Regurgitation
– Abdominal pain
– Dysphagia
– Food refusal/poor wt. gain
• Unresponsive to PPIs for GERD
• Strictures less common
10/5/2010
Diagnosis:
Clinical symptoms in EE
Symptom
Median Age of Presentation (years)
Feeding disorders
2
Vomiting/reflux
8
Abdominal pain
12
Dysphagia
13.4 and adults
Food impaction
16 and adults
Noel NEJM;351, 2004
10/5/2010
Diagnosis:
Endoscopic Features of EE
Vertical Lines
Rings
White Specks
10/5/2010
Normal
esophagus
GER
Eosinophilic
esophagitis
10/5/2010
EE vs. GERD
Characteristic
EE
GERD
Atopy
High
Nml
Food sensitization
High
Nml
Histology
>15-20 eos/hpf
0-7 eos/hpf
Peripheral eosinophilia
~50%
rare
Esophageal pH
Normal
Abnormal
PPI
Usually not helpful
Helpful
Steroids
Helpful
Not helpful
Food allergen elimination
Sometimes helpful
Not helpful
10/5/2010
Treatment Options
Anti‐inflammatory medication
Acid suppression
Elimination diet
Antihistamines and mast cell stabilizers (cromolyn)
• Leukotriene inhibitors (montelukast)
• Mepolizumab (anti IL‐5 antibody)
• Immunomodulators
•
•
•
•
10/5/2010
http://www.cghjournal.org/home
10/5/2010
Results: Demographics & Symptoms
Prednisone Group
N=40
Mean age (years)
a
7.0
Fluticasone Group
N=40
7.2
p-value
nsa
Age range (years)
1.1 – 15.8
1.1 – 16.1
Male
31 (78%)
28 (70%)
Presenting Symptomsb
Prednisone Group
N=40
Vomiting/Regurgitation
23 (58%)
26 (65%)
ns
Abdominal pain
11 (28%)
12 (30%)
ns
Epigastric pain/Heartburn
4 (10%)
4 (10%)
ns
Dysphagia/feeding problem
9 (23%)
6 (15%)
ns
Food/foreign body impaction
3 (8%)
1 (3%)
ns
Poor weight gain
1 (3%)
3 (8%)
ns
Cough
0 (0%)
2 (5%)
ns
Sore throat
1 (3%)
0 (0%)
ns
Belching
1 (3%)
0 (0%)
ns
Fluticasone Group
N=40
ns = non-significant, p-value >0.05; bPatients may have had more than one symptom
ns
p-value
10/5/2010
10/5/2010
Conclusions from EE study
• Systemic and topical corticosteroid therapy is effective in achieving initial histological and clinical remission of AEE
• Pred resulted in a greater degree of histological improvement but no evidence for an associated short or long‐term clinical advantage over Flutic
• No difference found symptom resolution, relapse rates or time relapse were noted between P and F
• Symptom relapse common to both groups upon discontinuation of therapy
• Systemic adverse effects were commonly associated with P
10/5/2010
Case Study 2
• 10‐1/2 year old girl with SMA type 2. • 1‐1/2 Post scoliosis surgery, L adductor and R hamstring lengthening, post‐op dysphagia with aspiration
• using NJ for feedings
• On BiPAP at night, cough‐ assist, gurgly voice, diet of Pedisure 41/2 cans over 18 hours
• Comes from several hours away and frequently fails appointments
10/5/2010
Case Study 2
• Physical Exam:
– Thin, alert female, 46pounds and 47 inches tall
– Hypernasal speech
– Audible upper airway secretions, clear lungs with occasional lower airway secretions heard. Upper extremity strength varies 3‐4/5 and has ulnar deviation
10/5/2010
Spinal muscle atrophy patients
• Decreased airway tone
• Decreased ability to compensate for compliance deficient by increasing work of breathing
• Decreased protective refluxes of airways
• Decreased ability to clear lower and upper airway
• Poor gi motility due to due to poor nutrition and decrease stimuli from ambulation
10/5/2010
Case Study 2
• FEES: no movement when patient thought she was swallowing
• Palative care consult, UGI, Pulmonary and GI combined procedure.
• General surgery scheduled G‐tube due to body habitis
10/5/2010
Case Study 3
• Former 26 wk EGA 5 ½ month old male with severe dysphagia, feeding difficulties/aspiration, GERD
• Forceful vomiting developed • VFSS: aspiration with all consistencies, contrast all down posterior tracheal wall
• Multiple ER visits and ultimately admitted when he developed perioral cyanosis
10/5/2010
Case Study 3
10/5/2010
Case Study 3
10/5/2010
Case Study 3
10/5/2010
• ENT and Pulmonary consultations and discussions. Elected combined bronchoscopy to evaluate airway anatomy
• Bronchoscopic Findings: laryngeal cleft (I), EDEMATOUS larynx, subglottic masses, R “pig” bronchous that on close evaluation was a blind pouch divot
• Chest CT Scan for subglottic masses and abnormal carina
• Flexible laryngoscopy showed edema
10/5/2010
GI Consult
• NJ feedings resulted in weight gain
• NPO
• Meds: omeprazole, aldactazide, albuterol and pulmicort
• Examination under anesthesia coordinated with ENT and GI
• EGD and planned Endoscopic Laryngeal Cleft Repair
10/5/2010
EGD
10/5/2010
Case Study 3
10/5/2010
Case Study 3
10/5/2010
Laryngeal Cleft
10/5/2010
Case Study 3
10/5/2010
Case Study 3
10/5/2010
Case Study 3
10/5/2010
Case Study 3
Follow up VFSS: no aspiration • Maladaptive Behavior
• “Feeding Plan”
10/5/2010
Case Study 4
• CC
– 2.7 year old now 3yo born term, had 1 week in special care nursery with 2‐3 days of CPAP (uncertain if nasal or ETT but suspect nasal).
– CHRONIC COUGH. Mom has correlated cough to drinking and a modified barium swallow finds nectar thick to solids fine, thins all aspirated. MRI
normal, pulmonary bronch found no cord closure and pneumonia, UGI and EGD normal, doing speech therapy weekly.
10/5/2010
Case Study 4
• CC‐2
– ENT evaluation: mirror exam larynx found vocal cords closed but was visualized with a “gag”. A question of lateral oral motor strength was raised.
– Plan: Twizzlers laterally….Mom found chewing with not problem.
–FEES then scheduled and findings on film:
10/5/2010
Case Study 4
• CC‐2
– ENT evaluation: mirror exam larynx found vocal cords closed but was visualized with a “gag”. A question of lateral oral motor strength was raised.
– Plan: Twizzlers laterally….Mom found chewing with not problem.
–FEES then scheduled and findings on film:
10/5/2010
10/5/2010
Case Study 4
• CC‐3
– A gelfoam injection was done in the posterior aspect of the L true vocal cord and interarytenoid area.
– Follow up (parent preferred FEES) 3 weeks later…patient had had 2 weeks of thins with no cough, no nebs, CTA and FEES showed:
10/5/2010
10/5/2010
Case Study 5
• CR
– 3 yo premature (24 weeks), IVH, mild CP, s/p TEF repair, dysphagia, reflux, common variable immunodefiency, hearing loss, speech delay, Nissen x
2 and a gtube.
– COUGH SINCE BIRTH. A genetics work up was normal, VFSS showed aspiration of thin liquids and penetration of nectar without aspiration. Her diet included thickened liquids, solids for age and g tube pump feeds 2 x a week.
– Meds: Prevacid, Reglan, Keppra, Flovent, Zyrtec, Singulair, Albuterol
10/5/2010
Tracheo esophageal fistula
• Large airway obstruction due tracheomalacia
• Very poor esophageal motility
• Aspiration damage from the TEF connections especially H‐type
• Associated with laryngeal clefts
10/5/2010
Case Study 5
• CR – 2
ENT evaluation: no distinct physical findings. Discussed with her neurologist who felt not related to head injuries. Discussed with her GI who was concerned may have something to do with her paraesophageal hernia.
10/5/2010
Case Study 5
• CR – 3
– ENT Plan:
• Direct Laryngoscopy and Bronch: no TVC closure seen! SECRETIONS and TEF pocket very close to carina, but not patent.
• FEES: aspiration over interarytenoid area mostly of refluxed liquids.
10/5/2010
10/5/2010
10/5/2010
10/5/2010
10/5/2010
Case Study 5
• CR – 3
– ENT Plan:
• Direct Laryngoscopy and Bronch: no TVC closure seen! SECRETIONS and TEF pocket very close to carina, but not patent.
• FEES: aspiration over interarytenoid area mostly of refluxed liquids.
• Nothing? Boston? Cleft I? Endoscopic repair?
10/5/2010
Case Study 6
• 2month old presents to ER with intermittent respiratory distress
• Pulmonary flexible bronchoscopy found subglottic stenosis, GERD and aspiration
• Treated with three days IV steroids, aggressive GERD therapy then OR for possible dilation or cricoid split • Better so medically managed
10/5/2010
Case Study 6
10/5/2010
Case Study 6
10/5/2010
Case Study 6
• Bronchoscopy at 7 mo found normal trachea and mild laryngomalacia
• Exam at 1 year found no stridor
• Exam at 3years normal • myringotomy tubes only invasive procedure
10/5/2010
GER
• Decreased sensation hypopharynx leads aspiration
• Edema leads to incoordination of laryngeal fn
• Hypersensitivity
• EE
10/5/2010
10/5/2010
10/5/2010
Case Study 7
• 15 month old girl s/p TEF repair as a neonate
• Hospitalized with bronchiolitis and decreased oral intake  feeding refusal
–
–
–
–
Receiving NG feedings but seemed to do worse
Sinus XR: mucosal thickening in both maxillary sinuses Was already being treated with amoxicillin for otitis media
Gastric emptying scan normal
10/5/2010
Diagnosis:
Histological findings in EE
•
•
•
•
•
Basal cell hyperplasia
Vascular papillae elongation
Epithelial eosinophilic infiltration Lamina propria fibrosis
Mucosal Edema (Dahms 2004).
10/5/2010
EGD
10/5/2010
BINGO
10/5/2010
Follow up 4 months later:
10/5/2010
It Takes a Team!
10/5/2010
You are part of the team!
• We have had and continue to welcome Primary Care physician involvement in person or by phone with the team meetings.
• We would welcome constructive comments on ways to help you and your patients in a more effiecent or helpful manner
10/5/2010
Questions and Comments
10/5/2010
References
• Pediatric Aerodigestivev Disorders, Haver, et al, Plural Publishing, San Diego CA, 2009.
• Munyard, P and Bush, A, Arch Dis Child 1996;74:531‐534.
• Pediatric Functinal Endoscopic Evaluation of Swallowing, Miller, et al, Conference, CCMC, 2009.
10/5/2010
References
• “Comparison of Oral Prednisone and Topical Fluticasone in the Treatment of Eosinophilic
Esophagitis: A Randomized Trial in Children”, Schaefer, etal., Clinical Gastroenterlology and Hepatology, 2008; 6:165‐173.