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Pediatric Dysphagia: Complex Case Studies of Patients with Aerodigestive Disorders (2421) Jennifer Perez, M.S., CCC-SLP Ch...
3 SLP’s = 1 Vision <ul><li>All have worked and trained extensively at Children’s Hospital Boston </li></ul><ul><li>Clinica...
Children’s Hospital Boston <ul><li>Acute, urban pediatric hospital </li></ul><ul><li>Associated with Harvard Medical Schoo...
Aero-digestive Disorders <ul><li>Congenital Anomalies of Larynx </li></ul><ul><li>Upper Airway Obstruction  </li></ul><ul>...
Center for  Aero-Digestive Disorders (CADD)
Center for Aero-Digestive Disorders   <ul><li>Multi-disciplinary clinic including pediatric specialists from: </li></ul><u...
Center for Aero-Digestive Disorders <ul><li>Providing comprehensive services to infants and children with complex problems...
Center for Aero-Digestive Disorders <ul><li>Frequently treated medical conditions: </li></ul><ul><ul><li>Asthma </li></ul>...
Center for Aero-Digestive Disorders <ul><li>Logistics—How the CADD works: </li></ul><ul><ul><li>Once per week, patients se...
Importance of the speech-language pathologist  on the Aero-Digestive team <ul><ul><li>Clinical screening for feeding & swa...
Center for Aero-Digestive Disorders <ul><li>Criteria for referral for objective swallowing evaluation: </li></ul><ul><ul><...
Objective Assessment of Swallow Function FEES vs. VFSS <ul><li>FEES  </li></ul><ul><li>Fiberoptic Endoscopic  </li></ul><u...
Management of Dysphagia  <ul><li>Establish safe oral feeding plan : </li></ul><ul><ul><li>? Need for non-oral supplementat...
Congenital Anomalies of the Larynx: Laryngeal Cleft
Laryngeal Cleft <ul><li>Definition: </li></ul><ul><ul><li>Communication between the posterior larynx and esophagus </li></...
Case Study # 1: <ul><li>-5 week old baby boy </li></ul><ul><li>-Clinical symptoms:  </li></ul><ul><li>-coughing while drin...
Case Study # 1 (cont): <ul><li>- VFSS Results : </li></ul><ul><li>-silent aspiration with thin liquids </li></ul><ul><li>-...
Case Study # 1 (cont): <ul><li>-At 11 months of age: </li></ul><ul><li>-Baby assessed for laryngeal cleft (DL/B) </li></ul...
VFSS Clip # 2 Post-Surgery
Case Study # 1 (cont): <ul><li>-Repeat VFSS 12 weeks post surgery  </li></ul><ul><li>-no aspiration with thin liquids </li...
Classification of Laryngeal Clefts <ul><li>According to length: </li></ul><ul><ul><li>Type I:  interarytenoid only </li></...
Types of Laryngeal Clefts <ul><li>Types I and II </li></ul><ul><ul><li>Diagnosis may take months to years </li></ul></ul><...
Classification of Laryngeal clefts Benjamin and Inglis, 1989
<ul><li>Type I </li></ul>Complete cricoid cleft Cleft extending to  thoracic trachea Interarytenoid and  supra glottic cle...
Photos courtesy of Dr. Reza Rahbar, ORL/CHB
Associated Anomalies <ul><li>Aero-Digestive: </li></ul><ul><ul><li>Tracheoesophageal fistulas: 20-37% </li></ul></ul><ul><...
Associated Syndromes <ul><li>Pallister Hall syndrome: </li></ul><ul><ul><li>Hypothalamic hamartoblastoma , Hypopituitarism...
Associated Syndromes <ul><ul><li>At Children’s Hospital Boston, of 81 patients with diagnosis of laryngeal cleft (2003-200...
Incidence of Laryngeal Clefts  (all types) <ul><li>Live birth:  1 in 10,000-1 in 20,000 </li></ul><ul><li>Incidence of lar...
Symptoms of Laryngeal Cleft <ul><li>Cough* </li></ul><ul><li>Wheezing* </li></ul><ul><li>Stridor </li></ul><ul><li>Feeding...
Diagnosis of Laryngeal Cleft <ul><li>High index of suspicion based on: </li></ul><ul><ul><li>Clinical presentation  </li><...
Diagnosis of Laryngeal Cleft <ul><li>Airway endoscopy  (in which laryngeal cleft is suspected)  is needed for definitive d...
Increasing Incidence:  Why? <ul><li>Literature review documents incidence of Type I laryngeal cleft is higher than in the ...
Surgical Repair of Laryngeal Cleft <ul><li>Conventional Treatment: </li></ul><ul><ul><li>Invasive surgery requiring incisi...
Photos courtesy of Dr. Reza Rahbar, Children’s Hospital Boston Endoscopic Repair of Laryngeal Cleft
SLP Perspective: Typical course for patient <ul><li>PCP or other specialist refers for MBS due to chronic pulmonary sympto...
SLP Perspective: Typical course for patient (cont.) <ul><li>6.  CADD team completes direct laryngoscopy/bronchoscopy to co...
Who to consider for laryngeal cleft: <ul><li>Any infant or child with normal development (i.e., no neurogenic, medical and...
Conservative Monitoring  <ul><li>If no surgical intervention, conservative management of dysphagia: </li></ul><ul><ul><li>...
Our experience at CHB <ul><li>2003-2008 </li></ul><ul><ul><li>81 patients referred for evaluation of cough, feeding diffic...
Our experience at CHB <ul><li>- Type I cleft:  100 % reported feeding difficulty with thin liquids </li></ul><ul><li>-Whee...
Case Study # 2: <ul><li>-5 month old baby girl </li></ul><ul><li>-Clinical symptoms:  </li></ul><ul><li>-coughing while dr...
Case Study # 2 (cont): <ul><li>- VFSS Results : </li></ul><ul><li>-silent aspiration with thin and nectar-thick liquids </...
VFSS Clip # 2  Post-op
What the SLP has learned… <ul><li>*Child with aspiration on VFSS who has otherwise normal development, who presents with a...
Fletcher Allen Health Care  <ul><li>Rural, community hospital  </li></ul><ul><li>Level I Trauma Center </li></ul><ul><li>L...
Vermont’s Academic Medical Center <ul><li>In alliance with UVM </li></ul><ul><li>Teaching hospital </li></ul><ul><li>Vermo...
What is Upper Airway Obstruction (UAO)? <ul><li>An obstruction present in the upper airway due to anatomic or physiologic ...
Upper vs. Lower Airway Obstruction <ul><li>Pattern of stridor is key for differential diagnosis of UAO </li></ul><ul><li>I...
Upper Airway Obstruction: Classifications <ul><li>Congenital: </li></ul><ul><li>Choanal atresia </li></ul><ul><li>Cleft li...
Upper Airway Obstruction: Classifications <ul><li>Acute: </li></ul><ul><li>Cases are seen in emergency department </li></u...
Etiologies of UAO <ul><li>Infection/Inflammation </li></ul><ul><li>Accounts for 90% of UAO </li></ul><ul><li>Croup </li></...
Types of UAO <ul><li>Nasopharyngeal: </li></ul><ul><li>Adenoid hypertrophy </li></ul><ul><li>Choanal atresia </li></ul><ul...
Role of Laryngeal-Pharyngeal Reflux and GERD in UAO <ul><li>Erythema </li></ul><ul><li>Post-glottic edema </li></ul><ul><l...
Case Study 1 Pediatric Patient with Congenital UAO: oropharyngeal and laryngeal types
Case 1 (timeline:13 months)  <ul><li>Baby born at 41 6/7 weeks gestational age </li></ul><ul><li>3232 grams </li></ul><ul>...
A baby on ECMO
Case 1 Timeline <ul><li>DOL 0-2 at FAHC </li></ul><ul><li>DOL 2-16 at CHB </li></ul><ul><li>DOL 16 through 10/08/07 at FAH...
Case 1 at FAHC <ul><li>First Feeding Team consult on DOL 19  </li></ul><ul><li>Baby with stridor at rest and feeding </li>...
Case 1 at CHB <ul><li>Baby re-intubated after trial CPAP, but worsening respiratory distress </li></ul><ul><li>Undergoes s...
Case 1 at FAHC <ul><li>Returns to Vermont for trach teaching </li></ul><ul><li>Does baby need G tube? </li></ul><ul><li>Cu...
VFSS results reveal <ul><li>Discoordination of sucking skill </li></ul><ul><li>Trach= “open system” </li></ul><ul><li>Lary...
VFSS: Looking at the whole picture <ul><li>Oral defensiveness </li></ul><ul><li>Poor growth </li></ul><ul><li>Recurrent cl...
Case 1 VFSS 2 Results Reveal <ul><li>5 weeks later </li></ul><ul><li>Trach </li></ul><ul><li>Passy Muir Speaking Valve in ...
Case 1 Re-admitted to FAHC <ul><li>5 weeks later </li></ul><ul><li>Recurrent upper respiratory infections </li></ul><ul><l...
Case 1 Treatment Plan:  “Interim Feeding Plan” <ul><li>When baby is healthy with no URI, emesis, PO feed thin liquids via ...
Case 1: Happy Outcomes <ul><li>Vocal fold mobility returns </li></ul><ul><li>Baby decannulated </li></ul><ul><li>Full PO f...
What we learn  (and questions to ask) <ul><li>Should breastfeeding be allowed if baby with s/s aspiration? </li></ul><ul><...
Case Study 2 Pediatric Patient with Congenital UOA: acute, oropharyngeal type
Case 2 (timeline: 9 months) <ul><li>10 month old admitted to FAHC with respiratory distress and neck edema </li></ul><ul><...
Case 2 at CHB <ul><li>Transferred to CHB for debulking of the mass </li></ul><ul><li>Subsequent pathology reveals a benign...
Case 2  <ul><li>MRI reveals residual tumor in posterior fossa and neck after debulking </li></ul><ul><li>Large enhancing m...
Case 2 VFSS results reveal <ul><li>Silent aspiration with purees and liquids </li></ul><ul><li>Delayed initiation of swall...
Case 2: Treatment plan <ul><li>NPO recommended to PCP </li></ul><ul><li>Baby underwent G tube placement given likely long-...
What we learn  (and questions to ask) <ul><li>Understanding prognosis is essential to make recommendations </li></ul><ul><...
Case Study 3 Pediatric patient with Congenital UAO: laryngeal anomaly type
Case 3 (timeline: 3 months)  <ul><li>Born at 41 weeks gestational age </li></ul><ul><li>4095 grams; LGA </li></ul><ul><li>...
Case 3 <ul><li>Feeding Team re-consulted 3 weeks later after successful extubation </li></ul><ul><li>s/p epiglottoplasty a...
Laryngoscopy results reveal <ul><li>Larynx remarkable for enlarged arytenoid cartilages </li></ul><ul><li>Paradoxical VF m...
VFSS results reveal <ul><li>Day after laryngoscopy </li></ul><ul><li>Aspiration with thin liquids </li></ul><ul><li>No evi...
Case 3: Treatment Plan <ul><li>Recommendations for baby to nipple nectar thick liquids via slow flow nipple </li></ul><ul>...
Case 3: Differential Diagnosis <ul><li>Paradoxical Vocal Fold Movement (PVFM) </li></ul><ul><li>Known an a functional or n...
Case 3: Literature review  <ul><li>Heatley & Swift, 1996 report a case of 4 month old with PVCD related to underlying GERD...
Case 3 <ul><li>PVFM classified as Cortical or Upper Motor Neuron OR Nuclear and Lower Motor Neuron </li></ul><ul><li>In in...
Final Thoughts on what we learn… <ul><li>How do we best manage aspiration in pediatrics? </li></ul><ul><li>Least restricti...
Final Thoughts <ul><li>Upper airway obstruction is an area where the medical SLP needs expertise </li></ul><ul><li>Teaming...
Esophageal Etiologies of Pediatric Dysphagia
South Shore Hospital <ul><li>Regional Medical Center SE MA </li></ul><ul><li>4,800 births/year </li></ul><ul><li>380 inpat...
Anatomy of the Esophagus <ul><li>Refer to the esophagus as the “proximal” and “distal” portions. </li></ul><ul><li>Proxima...
Anatomy & Physiology of the Esophagus <ul><li>After the upper esophageal sphincter relaxes, a reflexive, peristaltic “prim...
Anatomy & Physiology of the Esophagus <ul><li>Stripping wave initiated by the oropharyngeal swallow causing the LES to rel...
Esophageal Dysphagia Occurs When: <ul><li>Amplitude of the stripping wave is too low </li></ul><ul><li>Velocity of the wav...
Etiologies of Esophageal Dysphagia <ul><li>Differential diagnosis from oropharyngeal etiologies </li></ul><ul><li>Co-occur...
Etiologies of Esophageal Dysphagia <ul><li>2 Main Etiologies of Esophageal Dysphagia </li></ul><ul><li>Mechanical Causes <...
Etiologies of Esophageal Dysphagia <ul><li>Mechanical disturbance- usually localize the “hang up” of the food distal to th...
Case Study of a Pediatric Patient with Mechanical Obstruction
VACTERL  or VATER Association <ul><li>Acronym used to describe a series of characteristics which have been found to occur ...
VACTERL Association <ul><li>Babies diagnosed with VACTERL have at least 3 or more of the anomalies. </li></ul><ul><li>Wide...
Esophageal Atresia (EA) & Tracheoesophageal Fistula (TEF) <ul><li>Presence of EA suspected in the post natal period </li><...
EA & TEF <ul><li>Congenital EA represents failure of the esophagus to develop as a continuous passage. </li></ul><ul><li>T...
Case Study <ul><li>4 year 11 month old male with VACTERL Association </li></ul><ul><li>Followed  since infancy through Swa...
Case Study <ul><li>History of aspiration with thin and nectar thick liquids on VFSS first identified at 6 months of age. <...
Case Study <ul><li>Dx with type 1 laryngeal cleft (LC) </li></ul><ul><li>S/P endoscopic repair of LC at 3 years 6 months o...
Case Study <ul><li>6 months s/p LC repair patients VFSS revealed improvement in underlying swallow function </li></ul><ul>...
Case Study <ul><li>Patient returns for repeat VFSS at 4 years 11 months due to complaints “food is getting stuck” </li></u...
Videofluoroscopic Results Reveal: <ul><li>No evidence of aspiration with thin liquids </li></ul><ul><li>No evidence of asp...
Videofluoroscopic Results Reveal: <ul><li>Esophageal phase reveals moderate anastomotic narrowing at the site of the EA re...
Treatment Plan <ul><li>Attending radiologist at VFSS paged the GI physician to discuss dx of a stricture </li></ul><ul><li...
What we learn…….. <ul><li>We must look beyond the pharyngeal swallow </li></ul><ul><li>Most neonates with EA/TEF have some...
What we learn…….. <ul><li>Solid food dysphagia often the first sign the stricture has re-occurred </li></ul><ul><li>As chi...
What we learn…….. <ul><li>VFSS is only one instrumental evaluation that may give rise to esophageal dysphagia </li></ul><u...
Case Study of a Pediatric Patient with Motility Disturbance
Case Study <ul><li>3 month old infant referred for VFSS by pediatric otolaryngologist (ORL) </li></ul><ul><li>Presenting s...
VFSS Results <ul><li>Documentation of silent aspiration with thin, nectar thick and honey thick liquid </li></ul><ul><li>B...
VFSS Results <ul><li>Coordination of suck swallow breathe altered </li></ul><ul><li>Airway protection is compromised </li>...
Treatment Plan <ul><li>Attending radiologist at VFSS paged referring ORL physician </li></ul><ul><li>Patient made NPO by r...
Cricopharyngeal  Dysfunction <ul><li>Cricopharyngeal dysfunction as a cause of oropharyngeal dysphagia in infancy </li></u...
Cricopharyngeal dysfunction <ul><li>Manifested by swallowing difficulties </li></ul><ul><li>Nasopharyngeal regurgitation <...
Case Study Con’t… <ul><li>Patient admitted to the hospital for nasogastric tube feedings </li></ul><ul><li>GI work up reve...
Repeat VFSS  <ul><li>8 weeks s/p dilatation patient returned for repeat VFSS </li></ul><ul><li>Full PO feeding of thin liq...
Treatment Plan <ul><li>Medical team met to discuss the case </li></ul><ul><li>Cleared to continue on thin liquids with pac...
Treatment Options for Cricopharyngeal Achalasia <ul><li>Cricopharyngeal  myotomy </li></ul><ul><ul><li>Surgical division o...
Case study Con’t…. <ul><li>Different treatment modality trialed </li></ul><ul><li>Patient received Botox injection to the ...
Case Study Con’t…. <ul><li>Improvement in underlying swallow function s/p second dilatation </li></ul><ul><li>Aspiration r...
What we learn <ul><li>ASHA Guidelines for Speech Language Pathologists Performing Videofluoroscopic Swallow Studies (2004)...
What we learn <ul><li>Results of the instrumental oropharyngeal swallow evaluation should include an esophageal screening....
Seeking Resources <ul><li>Accessibility to a pediatric medical center that offers multidisciplinary team approach such as ...
Questions?
How to contact us: <ul><li>[email_address] </li></ul><ul><li>[email_address] </li></ul><ul><li>[email_address] </li></ul>
References <ul><li>ASHA 2008 Upper Airway Obstruction References </li></ul><ul><li>Andrews, T. Airway obstruction in crani...
References <ul><li>Arvedson, J. & Lefton-Greif, M. 1998.  Pediatric Videofluoroscopic Swallow Studies:  A Professional Man...
References <ul><li>Walton, J.M.; Tougas, G. Botulinum toxin use in pediatric esophageal achalasia: A case report. Journal ...
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  • Do you gals want this as the introductory slide???? To get things going??
  • Blockage of the upper airway. The following slides are also from Miller &amp; Willging.
  • Koufman, J &amp; Block, C. American Journal of Speech-Language Pathology Vol.17 327-334 November 2008. Inspiratory=BVFP, PVFM, glottic stenosis, Expiratory= “wheezing” asthma, COPD, tumors at or near the thoracic inlet Biphasic=PVFM with asthma, extrinsic or mediastinal compression with from tumor Generally, an inspiratory stridor suggests airway obstruction above the glottis while an expiratory stridor is indicative of obstruction in the lower trachea.
  • SLP and CADD focus is on congenital, although there can be post injury dysphagia from acquired UAO.
  • Acute= 1mm swelling in infant airway can cause big problems due to small diameter of breathing tube PR= PRS is characterized by an unusually small jaw (micrognathia), posterior displacement or retraction of the tongue (glossoptosis), and upper airway obstruction. Incomplete closure of the roof of the mouth ( cleft palate ), is present in the majority of patients, and is commonly U-shaped. TC= a rare genetic disorder characterized by craniofacial deformities . Treacher Collins syndrome is found in 1 in 10,000 births. The typical physical features include downward slanting eyes, a small lower jaw, and malformed or absent ears. Apert=is a congenital disorder characterized by malformations of the skull, face, hands, and feet. It is classified as a branchial arch syndrome and specifically affects the first branchial (or pharyngeal) arch , which is the precursor of the maxilla and mandible . BWS=is an overgrowth disorder present at birth characterized by an increased risk of childhood cancer and certain features. Five common features used to define BWS are: macroglossia (large tongue), macrosomia (birth weight and length &gt;90th percentile), midline abdominal wall defects (omphalocele, umbilical hernia, diastasis recti), ear creases or ear pits , and neonatal hypoglycemia (low blood sugar after birth). [1]
  • Laryngomalacia= terally, &amp;quot;soft larynx &amp;quot;) is a very common condition of infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction.
  • LPR=gastric contents reflux up out of esophagus and into hypopharynx Erythema=inflammation
  • What is PPHN=occurs with a complex interaction of factors results in hypertension (high blood pressure) which forces the blood away from lungs and decreases the supply of O2 to the body. Symptoms are often non-specific. Dx is one a rule out: if baby is still hypoxic despite good mechanical ventilation PPHN must be suspected. Causes of PPHN: severe meconium aspiration, infection, hypothermia, asphyxia. What is Thoracentisis= procedure to remove fluid from the space between the lining of the outside of the lungs (pleura) and the wall of the chest. What isPneuomothorax= air trapped between lung and chest wall What is oscillator= vibratory ventitilation that uses a vibrating diaphragm that alternates between pushing and pulling small volumes of gas within the airway. What is ECMO=Extracorporeal membrane oxygenation=modification of a heart lung bypass machine Needed when less invasive therapy is not effective in maintaining oxygenation, normal acid base status or hemodynamic stability. 2 types: venoarterial (VA) and venovenous (VV) depending on infants needs for heart and lung support vs mainly lung. 5-7 days average length of tx. Hard to determine neurodevelopmental outcome of these babies as they are critically ill at birth.
  • What is stridor=derived from Greek, meaning “creeking” harsh, vibratory sound of variable pitch caused by partial obstruction of the airway, resulting in turbulent airflow Stridor = a sign of upper airway obstruction. In children, laryngomalacia is the most common cause of chronic stridor, while croup is the most common cause of acute stridor. Laryngomalacia= terally, &amp;quot;soft larynx &amp;quot;) is a very common condition of infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction.
  • CPAP= Positive airway pressure ( PAP ) is a method of respiratory ventilation used primarily in the treatment of sleep apnea , for which it was first developed.PAP ventilation is also commonly used for critically ill patients in hospital with respiratory failure , and in newborn infants ( neonates ). In these patients, PAP ventilation can prevent the need for endotracheal intubation , or allow earlier extubation. Laryngomalacia= (literally, &amp;quot;soft larynx &amp;quot;) is a very common condition of infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction Why uncuffed trach in peds?= due to small airway and high risk for scarring. The laryngeal cartilage in infants may yield to pressure and mold more than in older patients. However, in small infants it remains difficult to provide an endotracheal tube small enough to avoid mucosal injury yet large enough to provide adequate ventilatory support and pulmonary toilet. Frequent tube changes result in additional laryngeal trauma. Subglottic stenosis= narrowing of trachea, can be caused by prolonged mechanical ventilation or repeated intubations
  • What is supraglottoplasty= Surgical division or reconstruction of shortened aryepiglottic folds in children with severe stridor or respiratory obstruction due to laryngomalacia. Layngomalacia= common condition of infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction. Tracheostomy PMV= All Passy-Muir Valves (PMVs) have a patented closed position &amp;quot;No Leak&amp;quot; design that restores a closed respiratory system.
  • read, learn and master how to use the phone interpreter
  • chemotherapy
  • neuroectodermal tumor of infancy is a rare neoplasm of neural crest origin that usually occurs in infants under 1 year of age. ハ Most lesions occur within the marrow spaces of the anterior maxilla but some have been outside the jaws, especially in the skull and brain. ハ It appears as a somewhat irregular radiolucency, perhaps multilocular but usually unilocular. The tumor is benign but may grow very rapidly and may invade bone marrow, very rare examples of malignant transformation have been reported. ハ Treatment is surgical removal, somewhat more aggressive than the typical &amp;quot;conservative&amp;quot; removal. ハ One case in 8 recurs with this treatment.
  • What is paradoxical movement in infant? Differential diagnosis made by flexible nasolaryngoscopy with pt awake and crying.
  • Role of GER with this patient Stridor Tracheomalacia Bilateral vocal fold paralysis Bronchoscopy reveals paradoxical movement?
  • Courtesy of Thomas Lahiri, MD Congenital stridor. 2.8 mm flexible bronch thru nares 4/15/08 Often one will see compression of false VF to compensate fro glottal incompetence
  • Laryngeal Dystonia= s a voice disorder characterized by involuntary movements of one or more muscles of the larynx (vocal folds or voice box) during speech. [1] Vocal folds adduct during inhalation and/or exhalation thereby restricting airway opening. Episodic adductor spasms of the VF interfere with normal breathing. Revealing active adductory movement instead of abdcution of the VF during inspiration. Leading to a reduction of the glottic opening. In BVFP VF are found immobile in a paramedian position. PVFM you can see active adductory VF contractions often showing a bulging and medialisation of the anterior 2/3 rds of VF during inspiration.
  • Make more bullets Proximal third of the esophagus made up of striated muscle like the oropharyngeal musculature Distal third of the esophagus (and remainder of the gut) made up of smooth muscle ?Note functional differences of Sriated muscle vs. smooth muscle
  • After the upper esophageal sphincter relaxes and there is pharyngeal clearance of the bolus into the proximal esophagus, a reflexive, peristaltic “primary stripping wave” is initiated. This propels the bolus distally
  • Stripping wave is initiated by the oropharyngeal swallow causing the LES to relax Remains open until the stripping wave has arrived at the LES and the bolus has passed into the stomach Secondary stripping waves originate from within the proximal esophagus (rather than from the oropharyngeal swallow) and propel any residual bolus into the stomach
  • Add globus sensation piece Add mulit phase dysphagia history is often unreliable in the pediatric population
  • Switch format to 2 columns Heading: mechanical Heading: motility
  • Patients usually localize the “hang up” of the food distal to the larynx with a mechanical disturbance Patients with oropharyngeal etiology localize the “hang up” proximal to the larynx On the basis of history and clinical assessment it is often not possible to determine etiology and hence diagnostic testing is indicated If the SLP finds evidence of an esophageal disorder a recommendation is made for appropriate referral to a specialist
  • Imperforate anus is an anus that does not open to the outside of the body C- cardiac anomalies can differ but usually an abnormal hole between parts of the heart ventricula septal defect or atrial septal defect L- limb defects such as absent or displaced thumbs, extra fingers (polydactyly) fused fingers (syndactyly) or a missing bone in the arms or legs
  • No specific genetic or chromosome problem has been identified with VACTERL. VACTERL can be seen with some chromosomal defects such as Trimsomy 18 and is more frequently seen in babies of diabetic mothers. VACERL is most likely caused by mulitple factors.
  • Anastamosis is the surgical connection of 2 tubular structures
  • VFSS one window in time – Anastomosis is the surgical connection of 2 tubular structures
  • Consider describing the dilatation procedure- balloon dilatation to the esphagus Site may stenose again Children with TEF/EA often need multiple dilatations. 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 to “open up” the site of the anastomosis 1 week s/p dilatation patient resumed and tolerated solid foods. Avoid all hard to chew solids/ high risk choking foods. Increased risk of impaction with high choke meats: steak, raw veggies, chicken wings, tough, overcooked meat, nuts. Solid foods that are not pulverized, chewed well also increase risk. Gummy- glutinous foods more likely to stick to the esophageal wall
  • In upright position Add upper GI piece/ complimentary study Next step is upper GI
  • Further GI testing indicated to confirm type of GI /esophageal disorder These tests are ordered and determined by the GI physician Upper GI series includes: barium esophagram: prone position, swallow barium pill
  • Achalasia: Failure to relax, said of muscles, such as sphincters, the normal function of which is persistent contraction with periods of relaxation.
  • 3 month old infant referred for VFSS by pediatric otolaryngologist (ORL) Presenting symptoms coughing and choking during feeding (50-75% of feed/mom) Mother reports “gasping” during bottle feeds PCP placed infant on Zantac with no improvement in symptoms
  • Think about discussing is this a true oropharyngeal dysphagia vs. esophageal dysphagia or co-occurring Aspiration by default Discuss co-occurrence of oropharyngeal dysphagia due to an esophageal etiology which is the failure of the UES to relax
  • Esophageal dysphagia is the primary etiology affecting the oropharyngeal stage Discoordination of SSB
  • Attending radiologist at VFSS paged referring ORL physician Patient made NPO by referring physician Patient admitted to the hospital for further work-up of the etiology of esophageal dysphagia GI service consulted to further assess esophageal dysmotility
  • Cricopharyngeal dysfunction as a cause of oropharyngeal dysphagia in infancy Results from 2 conditions: Failure of relaxation of the upper esophageal sphincter (UES) and/or Cricopharyngeal incoordination, lack of coordination between the contraction of the pharyngeal contraction and the UES relaxation (Mihailocivc, T, &amp; Perisic, V.N., 1992)
  • Pacing method initiated to avoid build up of residue at UES Nectar thick liquids not indicated due to increased build up of the thicker consistency
  • Trade name for Botox: Botulinum toxin (Botox-Allergan) used in pediatric EA esophageal achalasia
  • Describe the complications of botox injection to the UES Can hit the vocal cords
  • Aspiration resolved
  • As stated in the ASHA Instrumental Diagnostic Procedures for Swallowing 1991 and Knowldege and Skilss Needed by SLP’s performing Videofluroscopic Swallowing Studies 2004
  • Transcript of "Pediatric Dysphagia: Complex Case Studies of Patients with ..."

    1. 1. Pediatric Dysphagia: Complex Case Studies of Patients with Aerodigestive Disorders (2421) Jennifer Perez, M.S., CCC-SLP Children’s 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. 2. 3 SLP’s = 1 Vision <ul><li>All have worked and trained extensively at Children’s Hospital Boston </li></ul><ul><li>Clinical specialty in pediatric dysphagia </li></ul><ul><li>High volume of pediatric videofluoroscopy </li></ul><ul><li>Level 3 NICU </li></ul><ul><li>Exposure to wide range of medical/surgical pediatric diagnoses </li></ul><ul><li>Working within the division of ORL </li></ul>
    3. 3. Children’s Hospital Boston <ul><li>Acute, urban pediatric hospital </li></ul><ul><li>Associated with Harvard Medical School </li></ul><ul><li>397 bed comprehensive pediatric healthcare center </li></ul><ul><ul><li>Medical/Surgical ICU </li></ul></ul><ul><ul><li>Cardiac ICU </li></ul></ul><ul><ul><li>Neonatal ICU (Level III) </li></ul></ul><ul><li>204 specialized clinical programs </li></ul>
    4. 4. Aero-digestive Disorders <ul><li>Congenital Anomalies of Larynx </li></ul><ul><li>Upper Airway Obstruction </li></ul><ul><li>Esophageal Obstructive Disorders </li></ul>
    5. 5. Center for Aero-Digestive Disorders (CADD)
    6. 6. Center for Aero-Digestive Disorders <ul><li>Multi-disciplinary clinic including pediatric specialists from: </li></ul><ul><ul><li>Otolaryngology (ORL) </li></ul></ul><ul><ul><li>Gastroenterology </li></ul></ul><ul><ul><li>Pulmonary </li></ul></ul><ul><ul><li>Surgery </li></ul></ul><ul><ul><li>Radiology </li></ul></ul><ul><ul><li>Speech-Language Pathology </li></ul></ul><ul><ul><ul><li>Pediatric Dysphagia specialists </li></ul></ul></ul><ul><ul><ul><li>Voice specialists </li></ul></ul></ul><ul><ul><li>Neurology </li></ul></ul><ul><ul><li>Genetics </li></ul></ul>
    7. 7. Center for Aero-Digestive Disorders <ul><li>Providing comprehensive services to infants and children with complex problems involving the: </li></ul><ul><ul><li>Airway </li></ul></ul><ul><ul><li>Pulmonary tract </li></ul></ul><ul><ul><li>Upper digestive tract </li></ul></ul>
    8. 8. Center for Aero-Digestive Disorders <ul><li>Frequently treated medical conditions: </li></ul><ul><ul><li>Asthma </li></ul></ul><ul><ul><li>Congenital anomalies of the esophagus and trachea </li></ul></ul><ul><ul><li>Croup </li></ul></ul><ul><ul><li>Gastroesophageal reflux </li></ul></ul><ul><ul><li>Iatrogenic or acquired tracheal stenosis </li></ul></ul><ul><ul><li>Laryngeal cleft </li></ul></ul><ul><ul><li>Laryngomalacia </li></ul></ul><ul><ul><li>Lymphatic malformation </li></ul></ul><ul><ul><li>Pneumonia </li></ul></ul><ul><ul><li>Stridor </li></ul></ul><ul><ul><li>Subglottic stenosis </li></ul></ul><ul><ul><li>Tracheoesophageal fistula/esophageal atresia </li></ul></ul>
    9. 9. Center for Aero-Digestive Disorders <ul><li>Logistics—How the CADD works: </li></ul><ul><ul><li>Once per week, patients see ORL, GI, Pulmonary in clinic </li></ul></ul><ul><ul><ul><li>Speech-Pathology available for consultation </li></ul></ul></ul><ul><ul><li>Following clinic, team members meet to discuss and collaborate on findings and determine follow-up plan </li></ul></ul><ul><ul><li>Once per month, CADD conference of various disciplines for review of most complex patients/cases </li></ul></ul>
    10. 10. Importance of the speech-language pathologist on the Aero-Digestive team <ul><ul><li>Clinical screening for feeding & swallowing safety </li></ul></ul><ul><ul><li>Objective assessment of swallow function pre- and/or post-surgical intervention </li></ul></ul><ul><ul><li>Comprehensive feeding evaluation/therapy for sensory-based feeding issues, oral motor delays secondary to complex medical/surgical history </li></ul></ul>
    11. 11. Center for Aero-Digestive Disorders <ul><li>Criteria for referral for objective swallowing evaluation: </li></ul><ul><ul><li>Coughing, choking or sputtering (during or after feeds) </li></ul></ul><ul><ul><li>Wet/junky/congested breath sounds (during or after feeds) </li></ul></ul><ul><ul><li>Chronic wheezing or stridor </li></ul></ul><ul><ul><li>Chronic pneumonia, croup, bronchitis </li></ul></ul><ul><ul><li>Oxygen desaturation/cyanosis (during or after feeds) </li></ul></ul><ul><ul><li>Apnea/bradycardia, etc. with feeds </li></ul></ul><ul><ul><li>Color change with feeds </li></ul></ul><ul><ul><li>Chronic fever of unclear etiology </li></ul></ul>
    12. 12. Objective Assessment of Swallow Function FEES vs. VFSS <ul><li>FEES </li></ul><ul><li>Fiberoptic Endoscopic </li></ul><ul><li>Evaluation of Swallowing </li></ul><ul><li>No radiation exposure </li></ul><ul><li>Invasive-Difficulty maintaining cooperation in children </li></ul><ul><li>12 mo - 4 yrs </li></ul><ul><li>Does not provide information regarding interaction of oral, pharyngeal and esophageal phases of swallowing </li></ul><ul><li>Can assess breastfeeding </li></ul><ul><li>Can view secretions </li></ul><ul><li>Equipment is portable </li></ul><ul><li>Can assess VPI and vocal cord issues </li></ul><ul><li>VFSS </li></ul><ul><li>Videofluoroscopic Swallow Study </li></ul><ul><li>(Modified Barium Swallow Study) </li></ul><ul><li>Exposure to radiation </li></ul><ul><li>Non-invasive </li></ul><ul><li>Dynamic assessment of oral, pharyngeal, and esophageal phases of swallowing </li></ul><ul><li>Cannot assess breastfeeding </li></ul><ul><li>Readily available in most medical centers </li></ul><ul><li>Gold standard assessment of swallowing physiology </li></ul>Adapted from: Arvedson & Lefton-Greif, 1998
    13. 13. Management of Dysphagia <ul><li>Establish safe oral feeding plan : </li></ul><ul><ul><li>? Need for non-oral supplementation </li></ul></ul><ul><ul><li>Diet modification </li></ul></ul><ul><ul><li>Modify delivery method </li></ul></ul><ul><ul><li>Limit volume/length of time </li></ul></ul><ul><ul><li>Determine safest positioning </li></ul></ul><ul><ul><li>Counseling families </li></ul></ul><ul><ul><li>**Collaboration with other medical providers in CADD is essential for decision-making and establishing these dietary changes** </li></ul></ul>
    14. 14. Congenital Anomalies of the Larynx: Laryngeal Cleft
    15. 15. Laryngeal Cleft <ul><li>Definition: </li></ul><ul><ul><li>Communication between the posterior larynx and esophagus </li></ul></ul><ul><ul><li>Failure of the tracheoesophageal septum to develop </li></ul></ul>
    16. 16. Case Study # 1: <ul><li>-5 week old baby boy </li></ul><ul><li>-Clinical symptoms: </li></ul><ul><li>-coughing while drinking thin liquids </li></ul><ul><li>-pneumonia </li></ul><ul><li>-losing weight due to poor feeding </li></ul><ul><li>-Admitted to pulmonary service at CHB </li></ul><ul><li>-NG-tube placed </li></ul><ul><li>-Referred for VFSS </li></ul>
    17. 17. Case Study # 1 (cont): <ul><li>- VFSS Results : </li></ul><ul><li>-silent aspiration with thin liquids </li></ul><ul><li>-consistent deep laryngeal penetration with nectar-thick liquids </li></ul><ul><li>-nasopharyngeal reflux </li></ul><ul><ul><li>- assumed dysphagia due to discoordinated suck-swallow-breathe pattern </li></ul></ul><ul><li>-VFSS repeated no improvement x 2 </li></ul><ul><li>-G-tube placed at 4 months old </li></ul>
    18. 18. Case Study # 1 (cont): <ul><li>-At 11 months of age: </li></ul><ul><li>-Baby assessed for laryngeal cleft (DL/B) </li></ul><ul><li>-found to have Type 1 laryngeal cleft </li></ul><ul><li>-Surgically repaired </li></ul><ul><li>-Repeat VFSS 4 weeks post surgery </li></ul><ul><li>-continued aspiration of thin liquids </li></ul><ul><li>-Repeat VFSS 12 weeks post surgery </li></ul>
    19. 19. VFSS Clip # 2 Post-Surgery
    20. 20. Case Study # 1 (cont): <ul><li>-Repeat VFSS 12 weeks post surgery </li></ul><ul><li>-no aspiration with thin liquids </li></ul><ul><li>-Now: Child 3 years old </li></ul><ul><li>-G-tube removed </li></ul><ul><li>-stable respiratory status </li></ul>
    21. 21. Classification of Laryngeal Clefts <ul><li>According to length: </li></ul><ul><ul><li>Type I: interarytenoid only </li></ul></ul><ul><ul><li>Type II: partial cricoid </li></ul></ul><ul><ul><li>Type III: complete cricoid </li></ul></ul><ul><ul><li>Type IV: extending into thoracic trachea </li></ul></ul>
    22. 22. Types of Laryngeal Clefts <ul><li>Types I and II </li></ul><ul><ul><li>Diagnosis may take months to years </li></ul></ul><ul><li>Types III and IV </li></ul><ul><ul><li>Diagnosed on the first day of life due to severity </li></ul></ul><ul><ul><li>High morbidity associated with Type IV </li></ul></ul>
    23. 23. Classification of Laryngeal clefts Benjamin and Inglis, 1989
    24. 24. <ul><li>Type I </li></ul>Complete cricoid cleft Cleft extending to thoracic trachea Interarytenoid and supra glottic cleft Type I A Type I Type I B Type II Type I Type I C Type III Type II Type II Type III Type III Type III Type IV Classification Petterson Armitage Benjamin Partial cricoid cleft extending below the level of the vocal cords Cleft extending to cervical trachea
    25. 25. Photos courtesy of Dr. Reza Rahbar, ORL/CHB
    26. 26. Associated Anomalies <ul><li>Aero-Digestive: </li></ul><ul><ul><li>Tracheoesophageal fistulas: 20-37% </li></ul></ul><ul><ul><li>Tracheomalacia, laryngomalacia, subglottic stenosis </li></ul></ul><ul><ul><li>Hypoplastic lungs, transposition of the great arteries </li></ul></ul><ul><ul><li>Bifid uvula, cleft lip, cleft palate </li></ul></ul>
    27. 27. Associated Syndromes <ul><li>Pallister Hall syndrome: </li></ul><ul><ul><li>Hypothalamic hamartoblastoma , Hypopituitarism, Polydactyly, Imperforate anus </li></ul></ul><ul><li>Opitz-Frias syndrome: </li></ul><ul><ul><li>Cleft lip palate, Hypertelorism, Airway cleft, Hypospadias </li></ul></ul><ul><li>VACTERL </li></ul><ul><ul><li>Heart and vertebral anomalies , Esophageal atresia , Polydactylie, Absent left radius, Anorectal anomalies </li></ul></ul>
    28. 28. Associated Syndromes <ul><ul><li>At Children’s Hospital Boston, of 81 patients with diagnosis of laryngeal cleft (2003-2008): </li></ul></ul><ul><ul><li>TEF (s/p repair) (N=6) </li></ul></ul><ul><ul><li>Multiple Congenital Anomalies (N=13) </li></ul></ul><ul><ul><ul><li>Trisomy 21 (N=3) </li></ul></ul></ul><ul><ul><ul><li>Fetal Alcohol syndrome (N=1) </li></ul></ul></ul><ul><ul><ul><li>Tetralogy of Fallot (N=2) </li></ul></ul></ul><ul><ul><ul><li>Waardenburg (N=1) </li></ul></ul></ul><ul><ul><ul><li>Scimitar (N=1) </li></ul></ul></ul><ul><ul><ul><li>CHARGE association (N=2) </li></ul></ul></ul><ul><ul><ul><li>Opitz (N=1) </li></ul></ul></ul><ul><ul><ul><li>VACTERL (N=2) </li></ul></ul></ul>
    29. 29. Incidence of Laryngeal Clefts (all types) <ul><li>Live birth: 1 in 10,000-1 in 20,000 </li></ul><ul><li>Incidence of laryngeal cleft increases to 0.6% in patients with co-existing TEF </li></ul><ul><li>Strong association with other anomalies, but also can exist in isolation </li></ul>
    30. 30. Symptoms of Laryngeal Cleft <ul><li>Cough* </li></ul><ul><li>Wheezing* </li></ul><ul><li>Stridor </li></ul><ul><li>Feeding Difficulty </li></ul><ul><li>Aspiration </li></ul><ul><li>Respiratory Distress </li></ul><ul><ul><li>*Most common referral symptoms </li></ul></ul><ul><ul><li>* Symptoms are non-specific </li></ul></ul>
    31. 31. Diagnosis of Laryngeal Cleft <ul><li>High index of suspicion based on: </li></ul><ul><ul><li>Clinical presentation </li></ul></ul><ul><ul><ul><li>Medical history/Systematic chart review </li></ul></ul></ul><ul><ul><li>Interpretation of preoperative studies </li></ul></ul><ul><ul><ul><li>Chest radiograph, VFSS/FEES, Upper GI series </li></ul></ul></ul><ul><ul><li>Thorough endoscopic airway evaluation under general anesthesia </li></ul></ul><ul><ul><ul><li>Direct laryngoscopy, bronchoscopy, esophagoscopy </li></ul></ul></ul><ul><ul><ul><li>Endoscopist MUST perform diligent interarytenoid palpation </li></ul></ul></ul>
    32. 32. Diagnosis of Laryngeal Cleft <ul><li>Airway endoscopy (in which laryngeal cleft is suspected) is needed for definitive diagnosis </li></ul><ul><li>VFSS does not identify laryngeal cleft, but can confirm symptoms of aspiration due to overall neuromuscular discoordination or isolated pharyngeal dysphagia </li></ul>
    33. 33. Increasing Incidence: Why? <ul><li>Literature review documents incidence of Type I laryngeal cleft is higher than in the past </li></ul><ul><li>7.6 % (Chien et al, 2006) </li></ul><ul><li>6.2 % (Parsons et al, 1998) </li></ul><ul><li>7.1 % (Watters & Russell, 2003) </li></ul><ul><li>-Rising incidence may be due to: </li></ul><ul><li>-type of patients referred to aerodigestive clinics </li></ul><ul><li>-higher index of suspicion for presence of laryngeal cleft at time of airway endoscopy (ORL now looking more specifically for laryngeal cleft) </li></ul>
    34. 34. Surgical Repair of Laryngeal Cleft <ul><li>Conventional Treatment: </li></ul><ul><ul><li>Invasive surgery requiring incision in the neck and opening of the larynx </li></ul></ul><ul><li>Endoscopic Repair: </li></ul><ul><ul><li>Laser and suture </li></ul></ul><ul><li>Robotic Repair: </li></ul><ul><ul><li>Work through the oral cavity and in the restricted confines of the airway without impeding breathing </li></ul></ul><ul><ul><li>First performed by Dr. Reza Rahbar at Children’s Hospital Boston </li></ul></ul>
    35. 35. Photos courtesy of Dr. Reza Rahbar, Children’s Hospital Boston Endoscopic Repair of Laryngeal Cleft
    36. 36. SLP Perspective: Typical course for patient <ul><li>PCP or other specialist refers for MBS due to chronic pulmonary symptoms of unclear etiology associated with feeding </li></ul><ul><li>VFSS: Documentation of aspiration with liquids </li></ul><ul><li>SLP modifies diet (i.e,. Nectar-thick liquids) to promote swallowing safety, minimize aspiration </li></ul><ul><ul><li>-(patient may need NG-tube) </li></ul></ul><ul><li>SLP contact PCP to consider referral to CADD </li></ul><ul><li>Patient seen in CADD by ORL, GI, Pulmonary </li></ul>
    37. 37. SLP Perspective: Typical course for patient (cont.) <ul><li>6. CADD team completes direct laryngoscopy/bronchoscopy to confirm diagnosis of laryngeal cleft </li></ul><ul><li>Team decides: </li></ul><ul><ul><li>-conservative management or surgical repair?? </li></ul></ul><ul><li>IF SURGICAL REPAIR: </li></ul><ul><li>8. Maintain diet of thickened liquids for 6-8 weeks after surgery </li></ul><ul><li>Repeat VFSS at 8-12 weeks after surgery, and repeat every 3 months as needed </li></ul><ul><li>If no aspiration, child resumes full oral diet </li></ul>
    38. 38. Who to consider for laryngeal cleft: <ul><li>Any infant or child with normal development (i.e., no neurogenic, medical and genetic etiology) who presents with “isolated swallowing dysfunction” </li></ul><ul><li>Any infant or child with history of Tracheoesophageal fistula (TEF) and aspiration on VFSS </li></ul><ul><li>Any infant or child with a history of chronic aspiration that does not improve over time or with traditional intervention </li></ul>
    39. 39. Conservative Monitoring <ul><li>If no surgical intervention, conservative management of dysphagia: </li></ul><ul><ul><li>Diagnose and treat symptoms of GER </li></ul></ul><ul><ul><li>SLP works with family to modify diet </li></ul></ul><ul><ul><li>Close follow-up with CADD </li></ul></ul>
    40. 40. Our experience at CHB <ul><li>2003-2008 </li></ul><ul><ul><li>81 patients referred for evaluation of cough, feeding difficulty, and aspiration </li></ul></ul><ul><ul><li>74 of 81 patients found to have significant laryngeal cleft (Type I or Type II) </li></ul></ul><ul><ul><li>25 of 74 = conservative monitoring with medical therapy </li></ul></ul><ul><ul><li>49 of 74 = endoscopic CO2 laser repair of Type I and Type II laryngeal cleft </li></ul></ul><ul><ul><ul><li>(age range: 8 mo-15 years) </li></ul></ul></ul>
    41. 41. Our experience at CHB <ul><li>- Type I cleft: 100 % reported feeding difficulty with thin liquids </li></ul><ul><li>-Wheezing and chronic cough most common respiratory symptoms </li></ul><ul><li>-86% patients with aspiration on pre-surgical VFSS </li></ul><ul><li>- 71% patients with Type I =no aspiration on VFSS post-repair </li></ul><ul><li>- 82% patients with Type II =no aspiration on VFSS post repair </li></ul><ul><li>-Overall improvement noted in decreasing cough, normal VFSS, decreasing need for hospitalization </li></ul>
    42. 42. Case Study # 2: <ul><li>-5 month old baby girl </li></ul><ul><li>-Clinical symptoms: </li></ul><ul><li>-coughing while drinking thin liquids </li></ul><ul><li>-wheezing (worsens with feeding) </li></ul><ul><li>-frequent spit ups with feeds </li></ul><ul><li>-Referred for VFSS by PCP </li></ul>
    43. 43. Case Study # 2 (cont): <ul><li>- VFSS Results : </li></ul><ul><li>-silent aspiration with thin and nectar-thick liquids </li></ul><ul><li>-no aspiration with honey-thick liquids </li></ul><ul><li>-no aspiration with purees </li></ul><ul><li>-Discuss results with PCP who refers to CADD </li></ul><ul><li>- Nutrition assessment </li></ul><ul><li>-Airway endoscopy reveals Type 1 laryngeal cleft </li></ul><ul><li>-Patient undergoes surgical repair </li></ul><ul><li>-Repeat VFSS 3 months after laryngeal cleft repair </li></ul>
    44. 44. VFSS Clip # 2 Post-op
    45. 45. What the SLP has learned… <ul><li>*Child with aspiration on VFSS who has otherwise normal development, who presents with any chronic respiratory symptoms is a candidate for CADD work-up </li></ul><ul><li>*Maintain pre-surgical diet at least 6-8 weeks after surgery </li></ul><ul><li>*Do not repeat VFSS too soon after surgery-typically wait 8-12 weeks (minimize radiation exposure) </li></ul><ul><li>*Collaboration with Aero-digestive team is essential! </li></ul>
    46. 46. Fletcher Allen Health Care <ul><li>Rural, community hospital </li></ul><ul><li>Level I Trauma Center </li></ul><ul><li>Level III 20-bed NICU </li></ul><ul><li>10-bed NTU </li></ul><ul><li>30-bed Pediatric Floor </li></ul><ul><li>PICU </li></ul>
    47. 47. Vermont’s Academic Medical Center <ul><li>In alliance with UVM </li></ul><ul><li>Teaching hospital </li></ul><ul><li>Vermont Oncology Center </li></ul><ul><li>Innovative care </li></ul><ul><li>Acute Rehabilitation Center-Fanny Allen </li></ul>
    48. 48. What is Upper Airway Obstruction (UAO)? <ul><li>An obstruction present in the upper airway due to anatomic or physiologic anomalies disrupting respiration and the oral feeding process </li></ul><ul><li>Can be tracheal, laryngeal or pharyngeal in nature </li></ul><ul><li>Miller & Willging, 2007 </li></ul>
    49. 49. Upper vs. Lower Airway Obstruction <ul><li>Pattern of stridor is key for differential diagnosis of UAO </li></ul><ul><li>Inspiratory stridor </li></ul><ul><li>Expiratory stridor </li></ul><ul><li>Biphasic stridor </li></ul>Koufman, J & Block, C. American Journal of Speech-Language Pathology Vol.17 327-334 November 2008.
    50. 50. Upper Airway Obstruction: Classifications <ul><li>Congenital: </li></ul><ul><li>Choanal atresia </li></ul><ul><li>Cleft lip </li></ul><ul><li>Micrognathia </li></ul><ul><li>Laryngomalacia </li></ul><ul><li>Neoplasm </li></ul><ul><li>Laryngeal cleft </li></ul><ul><li>Acquired: </li></ul><ul><li>Croup </li></ul><ul><li>Epiglottitis </li></ul><ul><li>Tracheitis </li></ul><ul><li>Foreign body ingestion </li></ul><ul><li>Inhalation injury </li></ul>The following four slides adapted from Andrews, T. Airway obstruction in craniofacial anomalies: In: Myer, C, Cotton, R, Shott, S. eds. The Pediatric Airway: An Interdisciplinary Approach, Philadelphia, PA: JB Lippencott; 1995:249. Most recently published in Miller & Willging, 2007.
    51. 51. Upper Airway Obstruction: Classifications <ul><li>Acute: </li></ul><ul><li>Cases are seen in emergency department </li></ul><ul><li>Cases of UAO are acquired </li></ul><ul><li>Infection and croup </li></ul><ul><li>Chronic: </li></ul><ul><li>Pierre Robin sequence </li></ul><ul><li>Treacher-Collins syndrome </li></ul><ul><li>Apert syndrome </li></ul><ul><li>Beckwith-Wiedemann syndrome </li></ul>
    52. 52. Etiologies of UAO <ul><li>Infection/Inflammation </li></ul><ul><li>Accounts for 90% of UAO </li></ul><ul><li>Croup </li></ul><ul><li>Epiglottitis </li></ul><ul><li>Tracheitis </li></ul><ul><li>Retropharyngeal abscess </li></ul><ul><li>Peritonsillar abscess </li></ul><ul><li>Accident/Trauma: </li></ul><ul><li>Foreign body ingestion </li></ul><ul><li>Inhalation injury </li></ul><ul><li>Burns </li></ul><ul><li>External trauma to neck </li></ul><ul><li>Post-intubation </li></ul>
    53. 53. Types of UAO <ul><li>Nasopharyngeal: </li></ul><ul><li>Adenoid hypertrophy </li></ul><ul><li>Choanal atresia </li></ul><ul><li>Midface hypoplasia </li></ul><ul><li>Oropharyngeal: </li></ul><ul><li>Macroglossia </li></ul><ul><li>Pharyngeal hypotonia </li></ul><ul><li>Cleft Lip/Palate </li></ul><ul><li>Laryngeal type </li></ul><ul><li>anomalies: </li></ul><ul><li>Laryngeal cleft </li></ul><ul><li>Vocal fold paralysis </li></ul><ul><li>Neoplasm </li></ul><ul><li>Laryngeal Web </li></ul><ul><li>Laryngomalacia </li></ul>
    54. 54. Role of Laryngeal-Pharyngeal Reflux and GERD in UAO <ul><li>Erythema </li></ul><ul><li>Post-glottic edema </li></ul><ul><li>Vocal fold edema </li></ul><ul><li>Decreased sensation </li></ul><ul><li>Do protonics reverse sensory deficits? </li></ul><ul><li>Kirby & Noel, 2007 </li></ul>
    55. 55. Case Study 1 Pediatric Patient with Congenital UAO: oropharyngeal and laryngeal types
    56. 56. Case 1 (timeline:13 months) <ul><li>Baby born at 41 6/7 weeks gestational age </li></ul><ul><li>3232 grams </li></ul><ul><li>Rapid progression of Persistent Pulmonary Hypertension (PPHN) </li></ul><ul><li>Dx Pneumothorax and underwent Thoracentisis </li></ul><ul><li>Intubated and on high frequency oscillator at FAHC </li></ul><ul><li>Transferred to CHB on Day of life (DOL) #2 for ECMO </li></ul>
    57. 57. A baby on ECMO
    58. 58. Case 1 Timeline <ul><li>DOL 0-2 at FAHC </li></ul><ul><li>DOL 2-16 at CHB </li></ul><ul><li>DOL 16 through 10/08/07 at FAHC </li></ul><ul><li>10/08/07-11/20/07 at CHB </li></ul><ul><li>11/20/07 readmitted to FAHC </li></ul>
    59. 59. Case 1 at FAHC <ul><li>First Feeding Team consult on DOL 19 </li></ul><ul><li>Baby with stridor at rest and feeding </li></ul><ul><li>Bronchoscopy revealing significant airway prolapse, laryngomalacia and vocal fold immobility </li></ul><ul><li>Baby on O2 nasal cannula </li></ul><ul><li>Weight loss </li></ul><ul><li>Breastfeeding attempts were made </li></ul>
    60. 60. Case 1 at CHB <ul><li>Baby re-intubated after trial CPAP, but worsening respiratory distress </li></ul><ul><li>Undergoes supraglottoplasty for severe laryngomalacia </li></ul><ul><li>Dx subglottic stenosis </li></ul><ul><li>Fails extubation </li></ul><ul><li>Tracheostomy (Shiley 3.5 uncuffed) </li></ul><ul><li>Ng tube for poor feeding and aspiration </li></ul>
    61. 61. Case 1 at FAHC <ul><li>Returns to Vermont for trach teaching </li></ul><ul><li>Does baby need G tube? </li></ul><ul><li>Cultural differences: Somalian family with 6 kids </li></ul><ul><li>Mother breast fed all children </li></ul><ul><li>Baby now showing signs of oral defensiveness </li></ul>
    62. 62. VFSS results reveal <ul><li>Discoordination of sucking skill </li></ul><ul><li>Trach= “open system” </li></ul><ul><li>Laryngeal penetration with thin and nectar thick liquid-what are the implications? </li></ul><ul><li>Microaspiration </li></ul><ul><li>Silent in nature </li></ul><ul><li>Oral defensiveness </li></ul><ul><li>GERD </li></ul>
    63. 63. VFSS: Looking at the whole picture <ul><li>Oral defensiveness </li></ul><ul><li>Poor growth </li></ul><ul><li>Recurrent clinical signs aspiration with breast milk exiting trach site during nippling </li></ul><ul><li>Baby undergoes G tube placement for supplemental feedings </li></ul>
    64. 64. Case 1 VFSS 2 Results Reveal <ul><li>5 weeks later </li></ul><ul><li>Trach </li></ul><ul><li>Passy Muir Speaking Valve in place </li></ul><ul><li>No documentation of aspiration with thin liquid </li></ul><ul><li>Balance PO (per mouth) and PG (per gavage) feedings </li></ul>
    65. 65. Case 1 Re-admitted to FAHC <ul><li>5 weeks later </li></ul><ul><li>Recurrent upper respiratory infections </li></ul><ul><li>Post-tussive emesis </li></ul><ul><li>Bilateral Infiltrates on CXR </li></ul><ul><li>Cultural differences: everyone eats out of same bowl with hands (and germs) </li></ul>
    66. 66. Case 1 Treatment Plan: “Interim Feeding Plan” <ul><li>When baby is healthy with no URI, emesis, PO feed thin liquids via bottle </li></ul><ul><li>If baby is ill, with URI, emesis, make temporary, alternate modifications to feeding. This could include NPO </li></ul><ul><li>Less conservative, would be nectar thick liquids and/or begin spoon feeding at 6 mo. </li></ul><ul><li>Rely on G tube as primary means of nutrition and hydration </li></ul>
    67. 67. Case 1: Happy Outcomes <ul><li>Vocal fold mobility returns </li></ul><ul><li>Baby decannulated </li></ul><ul><li>Full PO feeder of thin liquids via sippy cup and bottle and soft solids </li></ul><ul><li>Will keep G tube through winter months as back-up for “Interim Feeding Plan” </li></ul><ul><li>Achieving developing milestones in timely age-appropriate manner </li></ul>
    68. 68. What we learn (and questions to ask) <ul><li>Should breastfeeding be allowed if baby with s/s aspiration? </li></ul><ul><li>Is any aspiration allowed? </li></ul><ul><li>The importance of PMV to restore healthy upper airway and “closed system” </li></ul><ul><li>VFSS is only ONE window in time and only a small piece to our overall assessment </li></ul><ul><li>How to embrace and manage cultural differences </li></ul>
    69. 69. Case Study 2 Pediatric Patient with Congenital UOA: acute, oropharyngeal type
    70. 70. Case 2 (timeline: 9 months) <ul><li>10 month old admitted to FAHC with respiratory distress and neck edema </li></ul><ul><li>Tracheostomy </li></ul><ul><li>Biopsied revealed ganglioneuroblstoma </li></ul><ul><li>Baby received cycle of carboplatin and etoposide, which decreased size of tumor temporarily </li></ul><ul><li>Mass increased in size, compromising her airway and necessitating intubation </li></ul>
    71. 71. Case 2 at CHB <ul><li>Transferred to CHB for debulking of the mass </li></ul><ul><li>Subsequent pathology reveals a benign cervical right neck neuroectodermal lesion </li></ul><ul><li>Most notable complication after debulking is poor feeding </li></ul><ul><li>VFSS in Boston reveals silent aspiration of puree and all liquid consistencies </li></ul>
    72. 72. Case 2 <ul><li>MRI reveals residual tumor in posterior fossa and neck after debulking </li></ul><ul><li>Large enhancing mask in the right parapharyngeal space </li></ul><ul><li>Neurosurgery deferred further resection due to low growth potential and stable size on MRI </li></ul><ul><li>Sub total resection: implications for long term and feeding? </li></ul>
    73. 73. Case 2 VFSS results reveal <ul><li>Silent aspiration with purees and liquids </li></ul><ul><li>Delayed initiation of swallow </li></ul><ul><li>Significant pharyngeal weakness and poor pharyngeal constriction </li></ul><ul><li>Post-swallow residue </li></ul>
    74. 74. Case 2: Treatment plan <ul><li>NPO recommended to PCP </li></ul><ul><li>Baby underwent G tube placement given likely long-term nature of impairment </li></ul><ul><li>Parents extremely frustrated by NPO status </li></ul><ul><li>Oral Stimulation program and tactile play with textures in absence of PO intake </li></ul>
    75. 75. What we learn (and questions to ask) <ul><li>Understanding prognosis is essential to make recommendations </li></ul><ul><li>Is any aspiration allowed? </li></ul><ul><li>How do we balance the need for NPO status with developmental feeding needs and family desires to feed the child? </li></ul><ul><li>Is NPO a feasible and reasonable recommendation? </li></ul>
    76. 76. Case Study 3 Pediatric patient with Congenital UAO: laryngeal anomaly type
    77. 77. Case 3 (timeline: 3 months) <ul><li>Born at 41 weeks gestational age </li></ul><ul><li>4095 grams; LGA </li></ul><ul><li>C-section due to fetal distress </li></ul><ul><li>Respiratory distress at birth </li></ul><ul><li>Consulted Feeding Team at DOL 11 (recommended NPO) </li></ul><ul><li>Stridor </li></ul><ul><li>Intubated </li></ul>
    78. 78. Case 3 <ul><li>Feeding Team re-consulted 3 weeks later after successful extubation </li></ul><ul><li>s/p epiglottoplasty and supraglottoplasty for redundant tissue </li></ul><ul><li>Baby with weak cry, aphonic at times </li></ul><ul><li>S/s aspiration during nippling </li></ul><ul><li>Inspiratory stridor continues </li></ul><ul><li>MRI was normal </li></ul>
    79. 79. Laryngoscopy results reveal <ul><li>Larynx remarkable for enlarged arytenoid cartilages </li></ul><ul><li>Paradoxical VF movement with each inspiration </li></ul><ul><li>A-P collapse of trachea was observed dynamically </li></ul><ul><li>No clear pulsatile compression of trachea was seen </li></ul>
    80. 80. VFSS results reveal <ul><li>Day after laryngoscopy </li></ul><ul><li>Aspiration with thin liquids </li></ul><ul><li>No evidence of aspiration with nectar thick liquids </li></ul><ul><li>Ng tube in place </li></ul>
    81. 81. Case 3: Treatment Plan <ul><li>Recommendations for baby to nipple nectar thick liquids via slow flow nipple </li></ul><ul><li>PO ad lib when baby showing interest in nippling </li></ul><ul><li>Gavage feedings continue to supplement until adequate volumes consumed PO </li></ul><ul><li>Repeat VFSS in 6-8 weeks </li></ul>
    82. 82. Case 3: Differential Diagnosis <ul><li>Paradoxical Vocal Fold Movement (PVFM) </li></ul><ul><li>Known an a functional or non-organic disorder </li></ul><ul><li>Etiology suggests psychopathology </li></ul><ul><li>Laryngeal dystonia? </li></ul><ul><li>Neurogenic etiology? </li></ul>
    83. 83. Case 3: Literature review <ul><li>Heatley & Swift, 1996 report a case of 4 month old with PVCD related to underlying GERD </li></ul><ul><li>Omland & Brondbo, 2008 report 4 cases infant of BVCP vs PVCM </li></ul><ul><li>EMG can provide further diagnostic info if the laryngeal muscles have normal electrical activity </li></ul>
    84. 84. Case 3 <ul><li>PVFM classified as Cortical or Upper Motor Neuron OR Nuclear and Lower Motor Neuron </li></ul><ul><li>In infant with no clear organic etiology, can’t be classified under above </li></ul><ul><li>GERD </li></ul><ul><li>Laryngeal Dyskinesia (lack of abductory movement with inhalation) </li></ul>
    85. 85. Final Thoughts on what we learn… <ul><li>How do we best manage aspiration in pediatrics? </li></ul><ul><li>Least restrictive, safest diet </li></ul><ul><li>“ Do no Harm” principle </li></ul><ul><li>Is any aspiration allowed? </li></ul><ul><li>Colin Wallis, MD, Pediatric Pulmonologist, of Great Ormond Street Hospital asks “Is there damage from aspiration?” not is it occurring </li></ul>
    86. 86. Final Thoughts <ul><li>Upper airway obstruction is an area where the medical SLP needs expertise </li></ul><ul><li>Teaming with ENT, Pulmonary, GI, Radiology is essential </li></ul><ul><li>Continuing Education for SLP and always asking questions is essential </li></ul>
    87. 87. Esophageal Etiologies of Pediatric Dysphagia
    88. 88. South Shore Hospital <ul><li>Regional Medical Center SE MA </li></ul><ul><li>4,800 births/year </li></ul><ul><li>380 inpatient beds </li></ul><ul><li>Level 2 Trauma Center </li></ul><ul><li>10 Level 3 NICU </li></ul><ul><li>20 Level 2 NICU </li></ul><ul><li>Pediatric Floor </li></ul><ul><li>Outpatient Pediatric Feeding Clinic </li></ul><ul><li>Clinical Affiliations </li></ul>
    89. 89. Anatomy of the Esophagus <ul><li>Refer to the esophagus as the “proximal” and “distal” portions. </li></ul><ul><li>Proximal third of the esophagus- striated muscle </li></ul><ul><li>Distal third of the esophagus (and remainder of the gut)-smooth muscle </li></ul>
    90. 90. Anatomy & Physiology of the Esophagus <ul><li>After the upper esophageal sphincter relaxes, a reflexive, peristaltic “primary stripping wave” is initiated. </li></ul><ul><li>This propels the bolus distally </li></ul><ul><li>Amplitude of this wave is higher in the distal esophagus </li></ul><ul><li>Traveling at 3-5 cm per second (Rubenstein, J.) </li></ul>
    91. 91. Anatomy & Physiology of the Esophagus <ul><li>Stripping wave initiated by the oropharyngeal swallow causing the LES to relax </li></ul><ul><li>Remains open until the stripping wave has arrived at the LES </li></ul><ul><li>Secondary stripping waves originate from within the proximal esophagus and propel any residual bolus into the stomach </li></ul>
    92. 92. Esophageal Dysphagia Occurs When: <ul><li>Amplitude of the stripping wave is too low </li></ul><ul><li>Velocity of the wave is too slow or too fast </li></ul><ul><li>The wave is so fast the UES and LES contract simultaneously </li></ul><ul><li>The UES or LES fail to relax </li></ul><ul><li>There is mechanical obstruction that cannot be overcome by the stripping wave </li></ul>
    93. 93. Etiologies of Esophageal Dysphagia <ul><li>Differential diagnosis from oropharyngeal etiologies </li></ul><ul><li>Co-occurring oropharyngeal and esophageal etiology </li></ul><ul><li>Careful history is important </li></ul><ul><li>Child should be asked to “point” with one finger to the area they feel there is “hang up” of the bolus </li></ul>
    94. 94. Etiologies of Esophageal Dysphagia <ul><li>2 Main Etiologies of Esophageal Dysphagia </li></ul><ul><li>Mechanical Causes </li></ul><ul><li>Motility Disturbances </li></ul><ul><li>Food impaction is more frequent with mechanical causes </li></ul><ul><li>Patients with mechanical obstruction can typically still swallow liquids </li></ul><ul><li>More pronounced solid food dysphagia </li></ul>
    95. 95. Etiologies of Esophageal Dysphagia <ul><li>Mechanical disturbance- usually localize the “hang up” of the food distal to the larynx </li></ul><ul><li>Oropharyngeal etiology localize the “hang up” proximal to the larynx </li></ul><ul><li>Often not possible to determine etiology and hence diagnostic testing is indicated </li></ul><ul><li>If SLP finds evidence of an esophageal disorder a recommendation is made for appropriate referral to a specialist </li></ul>
    96. 96. Case Study of a Pediatric Patient with Mechanical Obstruction
    97. 97. VACTERL or VATER Association <ul><li>Acronym used to describe a series of characteristics which have been found to occur together </li></ul><ul><li>V =vertebrae, which are abnormal (70% patients) </li></ul><ul><li>A =anal atresia/ imperforate anus (55% patients) </li></ul><ul><li>C = cardiac defects (75% patients) </li></ul><ul><li>T= tracheoesophageal fistula (TEF), an abnormal connection between the trachea and the esophagus </li></ul><ul><li>E= esophageal atresia, (EA) the esophagus does not connect to the stomach </li></ul><ul><li>R= renal defects </li></ul><ul><li>L= limb (arm) defects </li></ul>
    98. 98. VACTERL Association <ul><li>Babies diagnosed with VACTERL have at least 3 or more of the anomalies. </li></ul><ul><li>Wide range of manifestations </li></ul><ul><li>EA with TEF occurs in 70% patients </li></ul><ul><li>EA/TEF can also occur as an isolated defect </li></ul><ul><ul><li>EA/TEF as an isolated condition occurs in 1 in 3,000-5,000 births </li></ul></ul><ul><li>Typically have normal development and intelligence </li></ul>
    99. 99. Esophageal Atresia (EA) & Tracheoesophageal Fistula (TEF) <ul><li>Presence of EA suspected in the post natal period </li></ul><ul><li>Newborn presents with excessive salivation </li></ul><ul><li>Coughing, choking, sneezing. </li></ul><ul><li>Upon oral feeding the infant struggles and may become cyanotic </li></ul><ul><li>Respiratory distress </li></ul>
    100. 100. EA & TEF <ul><li>Congenital EA represents failure of the esophagus to develop as a continuous passage. </li></ul><ul><li>TEF represents an abnormal opening between the trachea and the esophagus </li></ul><ul><li>DX is made, all oral feedings are stopped </li></ul><ul><li>Surgical repair within the first days of life </li></ul>
    101. 101. Case Study <ul><li>4 year 11 month old male with VACTERL Association </li></ul><ul><li>Followed since infancy through Swallowing Disorders Program </li></ul><ul><li>Post natal diagnosis of EA/TEF s/p surgical repair on DOL#3 </li></ul><ul><li>Cardiac diagnosis of Tetrology of Fallot s/p repair (multiple staged repairs) </li></ul><ul><li>G-tube placed in infancy s/p EA/TEF repair </li></ul>
    102. 102. Case Study <ul><li>History of aspiration with thin and nectar thick liquids on VFSS first identified at 6 months of age. </li></ul><ul><li>Cleared to orally feed purees only. </li></ul><ul><li>G-tube to meet hydration, nutritional needs </li></ul><ul><li>No improvement with swallow function over the years </li></ul><ul><li>Referred to CADD clinic at 3 years of age </li></ul>
    103. 103. Case Study <ul><li>Dx with type 1 laryngeal cleft (LC) </li></ul><ul><li>S/P endoscopic repair of LC at 3 years 6 months old </li></ul><ul><li>Pre-surgically patient eating diet of purees, soft dissolvables and solids (hamburger, pasta, grilled chicken, green beans) in limited volume. </li></ul><ul><li>All liquids via g-tube </li></ul>
    104. 104. Case Study <ul><li>6 months s/p LC repair patients VFSS revealed improvement in underlying swallow function </li></ul><ul><li>Patient cleared to take thin and nectar thick liquids </li></ul><ul><li>Patient co-existing sensory based feeding disorder </li></ul><ul><li>Volume of solids limited due to texture sensitivity, hyper-sensitive gag reflex </li></ul><ul><li>Reduced efficiency with chewing </li></ul><ul><li>Receiving motor/sensory based feeding therapy </li></ul>
    105. 105. Case Study <ul><li>Patient returns for repeat VFSS at 4 years 11 months due to complaints “food is getting stuck” </li></ul><ul><li>Patient points to area below the larynx/chest area </li></ul><ul><li>Mother describes patient as “forceful swallow” with facial grimacing, decreased interest in solids, decreased volume of solids </li></ul><ul><li>Asymptomatic with liquids </li></ul>
    106. 106. Videofluoroscopic Results Reveal: <ul><li>No evidence of aspiration with thin liquids </li></ul><ul><li>No evidence of aspiration with solids which pass from the pharynx through the UES </li></ul><ul><li>Normal pharyngeal phase of swallowing </li></ul><ul><li>(confirm this on video clip) </li></ul>
    107. 107. Videofluoroscopic Results Reveal: <ul><li>Esophageal phase reveals moderate anastomotic narrowing at the site of the EA repair </li></ul><ul><li>Piece of chicken impacted at the site of the anastomosis </li></ul><ul><li>Liquid wash downs required to clear the solid </li></ul><ul><li>Continued ingestion of solids can lead to “back up” </li></ul>
    108. 108. Treatment Plan <ul><li>Attending radiologist at VFSS paged the GI physician to discuss dx of a stricture </li></ul><ul><li>Patient placed on diet of liquids/purees only until surgical decision </li></ul><ul><li>Patient underwent dilatation of the esophagus </li></ul><ul><li>1 week s/p dilatation patient resumed and tolerated solid foods. </li></ul><ul><li>Avoid hard to chew solids & high risk choking foods </li></ul><ul><li>Avoid “gummy” foods </li></ul><ul><li>Smaller, more frequent meals </li></ul>
    109. 109. What we learn…….. <ul><li>We must look beyond the pharyngeal swallow </li></ul><ul><li>Most neonates with EA/TEF have some degree of esophageal dysmotility </li></ul><ul><li>Strictures at the site of the anastomosis are common and may require mulitple dilatations </li></ul>
    110. 110. What we learn…….. <ul><li>Solid food dysphagia often the first sign the stricture has re-occurred </li></ul><ul><li>As children mature they are “in tune” to their esophagus and can describe symptoms </li></ul><ul><li>Many older children will self-restrict their diet </li></ul><ul><li>VFSS is an effective procedure for identifying the stricture </li></ul>
    111. 111. What we learn…….. <ul><li>VFSS is only one instrumental evaluation that may give rise to esophageal dysphagia </li></ul><ul><li>Further diagnostic testing may include: </li></ul><ul><ul><li>Upper GI series </li></ul></ul><ul><ul><li>Esophagoduodenscopy (EGD) </li></ul></ul>
    112. 112. Case Study of a Pediatric Patient with Motility Disturbance
    113. 113. Case Study <ul><li>3 month old infant referred for VFSS by pediatric otolaryngologist (ORL) </li></ul><ul><li>Presenting symptoms coughing and choking during feeding </li></ul><ul><li>Mother reports “gasping” during bottle feeds </li></ul><ul><li>PCP placed infant on Zantac with no improvement in symptoms </li></ul>
    114. 114. VFSS Results <ul><li>Documentation of silent aspiration with thin, nectar thick and honey thick liquid </li></ul><ul><li>Build up of residue above the upper esophageal sphincter </li></ul><ul><li>Failure of the UES to relax </li></ul><ul><li>Aspiration results from a build-up/ spill over of liquid due to failure of the UES to relax </li></ul>
    115. 115. VFSS Results <ul><li>Coordination of suck swallow breathe altered </li></ul><ul><li>Airway protection is compromised </li></ul><ul><li>Co-occurrence of oropharyngeal dysphagia along with an esophageal phase dysphagia </li></ul>
    116. 116. Treatment Plan <ul><li>Attending radiologist at VFSS paged referring ORL physician </li></ul><ul><li>Patient made NPO by referring physician </li></ul><ul><li>Patient admitted to the hospital </li></ul><ul><li>GI service consulted </li></ul>
    117. 117. Cricopharyngeal Dysfunction <ul><li>Cricopharyngeal dysfunction as a cause of oropharyngeal dysphagia in infancy </li></ul><ul><li>Multi-phase dysphagia </li></ul><ul><li>Results from 2 conditions: </li></ul><ul><li>Failure of relaxation of the upper esophageal sphincter (UES) and/or </li></ul><ul><li>Cricopharyngeal incoordination </li></ul><ul><li>(Mihailocivc, T, & Perisic, V.N., 1992) </li></ul>
    118. 118. Cricopharyngeal dysfunction <ul><li>Manifested by swallowing difficulties </li></ul><ul><li>Nasopharyngeal regurgitation </li></ul><ul><li>Coughing, choking </li></ul><ul><li>Aspiration pneumonia </li></ul><ul><li>Diagnosis typically made by radiography and motility studies </li></ul><ul><li>Cricopharyngeal dysfunction caused by either primary UES motility disorder or </li></ul><ul><li>May be associated with central nervous system diseases </li></ul>
    119. 119. Case Study Con’t… <ul><li>Patient admitted to the hospital for nasogastric tube feedings </li></ul><ul><li>GI work up revealed cricopharyngeal achalasia </li></ul><ul><li>Patient underwent esophageal dilatation during admission </li></ul><ul><li>D/C home full oral feeder with resolution of symptoms </li></ul><ul><li>f/u with GI physician </li></ul>
    120. 120. Repeat VFSS <ul><li>8 weeks s/p dilatation patient returned for repeat VFSS </li></ul><ul><li>Full PO feeding of thin liquid via bottle in the interim </li></ul><ul><li>Spoon feeding had been initiated </li></ul><ul><li>No intermittent respiratory symptoms </li></ul><ul><li>VFSS reveals qualitative improvement but continued pooling at the UES </li></ul><ul><li>Aspiration with thin liquids documented </li></ul><ul><li>No aspiration with spoon feeding of purees but pooling </li></ul>
    121. 121. Treatment Plan <ul><li>Medical team met to discuss the case </li></ul><ul><li>Cleared to continue on thin liquids with pacing strategy in place </li></ul><ul><li>Alter pacifier for non-nutritive sucking to help clear the residue with subsequent swallows </li></ul><ul><li>Nectar thick liquids not indicated due to increased build up </li></ul><ul><li>Return to GI to discuss need for re-dilatation </li></ul>
    122. 122. Treatment Options for Cricopharyngeal Achalasia <ul><li>Cricopharyngeal myotomy </li></ul><ul><ul><li>Surgical division of the muscle </li></ul></ul><ul><ul><li>Renders the muscle inactive </li></ul></ul><ul><ul><li>Often as “last option” </li></ul></ul><ul><li>Dilatation </li></ul><ul><ul><li>Single balloon catheter dilatation proven to be successful </li></ul></ul><ul><ul><li>Less invasive than myotomy </li></ul></ul><ul><ul><li>Results apparent 24-48 after procedure </li></ul></ul><ul><ul><li>Patient may require repeat dilatations </li></ul></ul><ul><ul><li>Botox injection to the UES </li></ul></ul><ul><ul><li>More commonly used in the adult population </li></ul></ul><ul><ul><li>Efficacy of botox use to LES in pediatrics </li></ul></ul><ul><ul><li>Botox effect may wear off over time </li></ul></ul><ul><ul><li>Need to re-inject </li></ul></ul><ul><ul><li>Risk of injecting the wrong site/muscle </li></ul></ul>
    123. 123. Case study Con’t…. <ul><li>Different treatment modality trialed </li></ul><ul><li>Patient received Botox injection to the UES </li></ul><ul><li>Due to complications from Botox injection patient required hospital admission for NG tube placement </li></ul><ul><li>Decision was made for g-tube placement and repeat dilatation to the UES due to on-going dysphagia and aspiration </li></ul>
    124. 124. Case Study Con’t…. <ul><li>Improvement in underlying swallow function s/p second dilatation </li></ul><ul><li>Aspiration resolved </li></ul><ul><li>Patient progressed to mashed and soft table foods (1 year of age) </li></ul><ul><li>Soft solids pass through site of dilatation without hold up </li></ul><ul><li>Cricopharyngeal muscle still prominent but with improved coordination </li></ul>
    125. 125. What we learn <ul><li>ASHA Guidelines for Speech Language Pathologists Performing Videofluoroscopic Swallow Studies (2004) states </li></ul><ul><li>“ Clinicians should be aware that oropharyngeal swallow function is often altered in patients with esophageal motility disorders and dysphagia.” </li></ul><ul><li>“ SLP’s have knowledge and skills to recognize patient signs and symptoms associated with esophageal dysphagia </li></ul>
    126. 126. What we learn <ul><li>Results of the instrumental oropharyngeal swallow evaluation should include an esophageal screening. </li></ul><ul><li>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) </li></ul><ul><li>“ SLP’s should have sufficient knowledge of esophageal structure and function to make an appropriate referral and plan cooperative management </li></ul>
    127. 127. Seeking Resources <ul><li>Accessibility to a pediatric medical center that offers multidisciplinary team approach such as The Center for Aerodigestive Disorders. </li></ul><ul><li>Underscores the need for graduate training in pediatric dysphagia </li></ul><ul><li>Course work and clinical practicum </li></ul><ul><li>Collaboration among hospital and community based SLP’s for carry over of safe and successful feeding plan in the home setting. </li></ul>
    128. 128. Questions?
    129. 129. How to contact us: <ul><li>[email_address] </li></ul><ul><li>[email_address] </li></ul><ul><li>[email_address] </li></ul>
    130. 130. References <ul><li>ASHA 2008 Upper Airway Obstruction References </li></ul><ul><li>Andrews, T. Airway obstruction in craniofacial anomalies: In: Myer, C, Cotton, R, Shott, S. eds. The Pediatric Airway: An Interdisciplinary Approach, Philadelphia, PA: JB Lippencott; 1995:249. (Most recently published in Miller & Willging, 2007.) </li></ul><ul><li>Koufman, J & Block, C. American Journal of Speech-Language Pathology Vol.17 327-334 November 2008 </li></ul><ul><li>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 265:803-807. </li></ul><ul><li>Mathers-Schmidt, B (2001) Paradoxical Vocal Fold Motion: A Tutorial on a Complex Disorder and the Speech-Language Pathologist’s Role. American Journal of Speech-language Pathology 10: 111-125. </li></ul><ul><li>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. </li></ul><ul><li>Ibrahim, W, Gheriani H, Almohamed, A, Raza, T (2007) Paradoxical vocal cord motion disorder: past, present and future. 83: 164-172 </li></ul><ul><li>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. </li></ul><ul><li>Arvedson, J (2008) Food for Thought on Pediatric Feeding and Swallowing. ASHA Division 13 Perspectives on Swallowing and Swallowing Disorders 17: 110-118. </li></ul><ul><li>Miller, C, Willging, JP (2007) The Implications of Upper-Airway Obstruction on Successful Infant Feeding. Seminars in Speech and Language 28:190-203. </li></ul><ul><li>Zaichkin, J (2002) Newborn Intensive Care: What every parent needs to know. 2nd edition. NICU INK Book Publishers, Santa Rosa, CA. </li></ul><ul><li>Kirby, M, Noel, R. (2007) Nutrition and Gastrointestinal Tract Assessment and Management of Children with Dysphagia. Seminars In Speech and Language 28: 180-189. </li></ul>
    131. 131. References <ul><li>Arvedson, J. & Lefton-Greif, M. 1998. Pediatric Videofluoroscopic Swallow Studies: A Professional Manual with Caregiver Guidelines. San Antonio, TX: Communication Skill Builders </li></ul><ul><li>Rahbar, R. et al. The presentation and management of laryngeal cleft: A 10-year experience. Arch Otolaryngol Head Neck Surg. 2006; 132: 1336-1341. </li></ul><ul><li>Parsons, DS. et al. Type I posterior laryngeal clefts. Laryngoscope. 1998; 108: 403-410. </li></ul><ul><li>Watters K., J. Russel. Diagnosis and management of type I laryngeal cleft. International Journal of Pediatric Otorhinolaryngology. 2003; 67: 591-596. </li></ul><ul><li>Chien, W. et al. “Type I laryngeal cleft: Establishing a functional diagnostic and management algorithm.” International Journal of Pediatric Otorhinolaryngology. 2006, 70: 2073-2079. </li></ul><ul><li>Benjamin B. A Inglis. Minor congenital laryngeal clefts: diagnosis and classification. Ann Otol Rhinol Laryngol 1989; 98: 417-420. </li></ul><ul><li>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. </li></ul>
    132. 132. References <ul><li>Walton, J.M.; Tougas, G. Botulinum toxin use in pediatric esophageal achalasia: A case report. Journal of Pediatric Surgery, 1997, 801-920. </li></ul><ul><li>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. </li></ul><ul><li>Rubenstein, Joel, H. Esophageal Etiologies of Dysphagia: A Guide for SLP’s. Perspctives on Swallowing and Swallowing Disorders (Dysphagia) Volume 16, Number 4, Dec. 2007, 1-6. </li></ul><ul><li>Easterling, Caryn. Food for Thought: What happens in the esophagus, doesn’t stay in the esophagus..So who cares? A Guide for SLP’s. Perspctives on Swallowing and Swallowing Disorders (Dysphagia) Volume 16, Number 4, Dec. 2007, 19-20 </li></ul><ul><li>Mihailovic, T., Perisic, V.N. Balloon dilatation of cricopharyngeal achalasia. Pediatric Radiology (1992), 22; 522-524. </li></ul><ul><li>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. </li></ul><ul><li>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. </li></ul><ul><li>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. </li></ul>
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