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Complex Decision Making in Pediatric Dysphagia


Complex Decision Making in Pediatric Dysphagia Alana Lowry, MS, CCC-SLP Fletcher Allen Health Care Kara Fletcher Larson, MS, CCC-SLP Jennifer Miller, MS, CCC-SLP – PowerPoint PPT presentation

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Title: Complex Decision Making in Pediatric Dysphagia

Complex Decision Making in Pediatric Dysphagia
  • Alana Lowry, MS, CCC-SLP
  • Fletcher Allen Health Care
  • Kara Fletcher Larson, MS, CCC-SLP
  • Jennifer Miller, MS, CCC-SLP
  • Childrens Hospital Boston
  • ASHA November 17, 2006
  • Miami, Florida

Contact Information
  • Kara Fletcher Larson, MS, CCC-SLP
  • Alana Lowry, MS, CCC-SLP
  • Jennifer Miller, MS, CCC-SLP

Incidence of Pediatric Dysphagia
  • 25 in all children
  • 80 in children with developmental disabilities
  • 3-10 of children exhibit severe feeding problems
  • Occur with greater prevalence in children with
    physical disabilities, medical illness and
  • (Manikam Perman 2000)
  • Summarized in Oct. 2006 Brackett, Arvedson
    Manno in SID 13 newsletter

How did we get here?
  • Major pediatric medical center
  • Childrens Hospital Boston, MA
  • 2005 performed 864 pediatric videofluoroscopic
    swallow studies
  • Range in ages from 38 weeks PMA- young adults
    with developmental disabilities (early 20s)

Patient Demographics
  • 50 of our patients fall in the age range of 6
    months- 3 years of age
  • 6 of patients referred from Level 3 NICU
  • 4 of patients referred by partnership with Dana
    Farber Cancer Institute/ Pediatric Oncology
  • 13 of patients referred by the Otolaryngology

Trends in Referral Concerns
  • Given high volume of VFSS performed we began to
    observe trends in subset of patient populations
  • Pediatric Oncology
  • Increased incidence in identification and
    diagnosis of the Type 1 laryngeal cleft

Complex Decision Making
  • Low incidence problems in pediatric dysphagia
  • High stakes for safe and effective management of
    oropharyngeal dysphagia
  • Medical, surgical, ethical and clinical questions
    we face when treating these children
  • Highlight the role of the SLP as the preferred
    provider of dysphagia services.

Pediatric Oncology
  • Patients referred for VFSS with chief complaint
    of coughing and choking with thin liquids
  • All patients referred were undergoing
    chemotherapy consisting of the drug Vincristine
    (enrolled in specific treatment protocol for type
    of cancer)
  • Onset of symptoms occurred 3-14 days during the
    treatment of a 6 week cycle

Chemotherapy AgentVincristine
  • Chemotherapy treats the type of cancer with
    medication that is toxic to tumor cells or kills
    them through interaction with receptors that
    indicate programmed cell death or prevent cell
  • Typically given in cycles
  • Cycle typically lasts 4-6 weeks
  • Period drug administration- resting period

Side Effects of Vincristine Neurotoxicity
  • Involves peripheral, autonomic, and central
  • Primary and dose limiting toxicity of Vincristine
  • Most side effects are dose related and reversible
  • Neurotoxicity can persist for months after
    discontinuation of therapy
  • Rare cases can be permanently disabling

Results of VFSS in Children Receiving Vincristine
  • All patients referred were full oral feeders at
    the time of referral
  • All patients undergoing intravenous
    administration of Vincristine
  • Parents report onset (often sudden) of
    sputtering, coughing and choking mainly with
  • Attending oncologist referred patient for VFSS

Results of VFSS in Children Receiving Vincristine
  • Silent aspiration with thin liquids
  • Silent aspiration with thin and nectar thick
  • Silent aspiration with thin, nectar and honey
    thick liquids
  • No evidence of aspiration with purees or solids

Management of Pharyngeal Dysphagia in Children
with Vincristine Toxicity
  • Results reported back to Oncology Team
  • Based on the extent of aspiration modifications
    to the oral feeding regimen were initiated
  • In cases of aspiration with all liquid
    consistencies discussion regarding non-oral
    supplementation took place with the MD
    Dysphagia Team

Aspiration with Thin Liquid Only
  • Diet of nectar thick liquids
  • Recommend referral to nutrition to ensure
    adequate hydration and child acceptance
  • Report results to Oncology Clinic
  • Medical team to discuss changes to dose/strength
    of Vincristine
  • Develop plan for repeat VFSS once team feels
    neurotoxicity is resolving
  • Parents also report improved clinical status
    which helps guide timeline for reassessment of
    swallow function

Medical Concerns
  • Larger medical concern whether to discontinue
    cycle of Vincristine to avoid further
    exacerbation of the toxicity vs. decreasing the
    dose/strength of the Vincristine.
  • Child may be made NPO with continuation of
    chemotherapy with dose changes.
  • Child put on rest from a swallowing standpoint
    with period of going off the drug
  • Above decision made by attending oncologist with
    input from the Oncology-Dysphagia team

Medical-Ethical Considerations
  • Decision to withhold chemotherapy treatment to
    allow neurotoxicity to improve
  • Parental stressors regarding decision
  • Patients taken off Vincristine for weeks while
    swallow function improves
  • Child continues to orally feed with modifications
    in place

Resolution of Swallow Function
  • Swallow function resolved (returned to
    pre-Vincristine status) in 100 of patients.
  • Range of time it took for swallow function to
    return to normal
  • Normal defined as back to full oral diet of thin
    liquids, purees and solids
  • of VFSS patients underwent until swallow
    function resolved. (at what time intervals).
  • Recurrence once patient resumed Vincristine
  • Yes in some patients
  • Even at reduced strength of drug (50 strength).
  • Oncology team was very conservative with
    re-starting chemotherapy/ altered doses and child
    monitored closely

Case Study Vincristine Toxicity
  • 5/10/04 3 ½ year old girl is diagnosed with
  • acute lymphoblastic leukemia (ALL)
  • Immediately begins chemotherapy (including
  • Throughout 7 months of chemotherapy, pt.
    is seen frequently in clinic for chronic upper
    respiratory tract congestion and persistent

Case Study Vincristine Toxicity
  • 12/27/04 Diagnosed with pneumonia on
    chest x-ray
  • 2/3/05 Pt. referred for initial VFSS by
    oncology team 9 months into chemotherapy
  • VFSS revealed silent aspiration with thin
  • Patient safe to continue to receive
    nectar-thick liquids, purees, and chewable

Case StudyVincristine Toxicity
  • Insert VFSS 1 of silent aspiration with thin
    liquids (2/3/05)

Case StudyVincristine Toxicity
  • 2/4/05 Vincristine component of chemotherapy
    is withheld
  • Pt. remained on nectar-thick liquids, purees,
  • 2/28/05 Repeat VFSS continued to reveal silent
  • with thin liquids
  • Recommendation remain on altered oral diet
  • 4/21/05 Repeat VFSS revealed normal swallow
    function with no documentation of aspiration
    with thin liquids
  • Respiratory status stable

Case StudyVincristine Toxicity
  • Insert VFSS of normal swallow function with no
    aspiration (4/21/05)

Case Study Vincristine Toxicity
  • 4/28/05 Vincristine resumed (50 strength)
  • (Pt. maintained nectar-thick liquid diet)
  • 6/20/05 2 mo. follow-up VFSS revealed silent
    aspiration with thin liquids
  • Recommendation Cont. nectar-thick liquids
  • Pt. continues receiving vincristine
  • Pt. was asymptomatic from respiratory
    standpoint during this time
  • .

Outcome Case StudyVincristine Toxicity
  • 10/1/05 Patient completed course of chemotherapy
  • (No longer receiving vincristine)
  • 11/3/05 Repeat VFSS was normal with no further
    evidence of aspiration with thin liquids
  • Pt. cleared for full oral diet
  • Follow-up Patient tolerated re-introduction of
    thin liquids and maintained stable respiratory

Complex Decision Making in Pediatric Dysphagia
Part 2
  • Type 1 Laryngeal Cleft

What is a Laryngeal Cleft (LC)?
  • Communication between the posterior larynx and
  • Failure of tracheo-esophageal septum to develop

Laryngeal Embryology
  • Trachea and esophagus share common lumen during
  • 35th day of gestation
  • Laryngeal cleft is the failure of the
    interarytenoid tissue or cricoid tissue to fuse
    in the posterior midline

Types of Laryngeal Clefts
  • Four classifications of laryngeal clefts
  • Type 3 and 4 diagnosed on first day of life due
    to severity
  • Type 1 and 2 diagnosis may take months to years.
  • Type 1 is the focus of our talk today.

Classification of Laryngeal Clefts
  • According to length
  • Type 1 interarynenoid only
  • Type 2 partial cricoid
  • Type 3 complete cricoid
  • Type 4 extending into trachea

Classification of Laryngeal Clefts
Benjamin and Inglis, 1989
(No Transcript)
Clinical Signs Symptoms of Type 1 Laryngeal
  • Noisy breathing
  • Inspiratory stridor
  • Coughing choking with feedings
  • Chronic pulmonary infections
  • Aspiration
  • As and Bs with feedings
  • Cyanosis

Differential Diagnosis of Type 1 LC
  • VFSS (MBS)
  • FEES
  • Chest x-ray
  • Referral to pediatric Otolaryngologist and
  • High degree of suspicion of type 1 laryngeal
    cleft (LC)
  • Direct laryngoscopy is needed for definitive
    diagnosis and is the gold standard for diagnosis

Suspicion of Type 1 LC
  • Child presents with normal development with
    exception of isolated swallowing dysfunction
  • No evidence of neurogenic, medical, and genetic
    etiology for swallow dysfunction.

Incidence of Laryngeal Clefts (all types)
  • Rare, less than 0.1
  • Incidence increases to 0.6 in patients with the
    co-existence of TEF and laryngeal cleft
  • Strong association with other anomalies, but in
    our population has often existed in isolation
  • (Cotton Prescott, 1998)

Type 1 LC at Childrens Hospital Boston
  • 30 patients diagnosed with type 1 laryngeal cleft
    from 2000-2005.
  • 21 patients repaired.
  • gt90 patients with improved swallow function
    after repair.

Incidence on the rise
  • Literature review documents incidence of type 1
    laryngeal cleft higher than in the past.
  • 7.6 (Chien et al, 2006)
  • 6.2 (Watters Russell, 2003)
  • 7.1 (Parsons et al, 1998)
  • Are there now more patients with type 1 laryngeal
    cleft or are we getting better at the diagnosis?

Associated Congenital Anomalies with laryngeal
  • Pallister-Hall Syndrome
  • G Syndrome
  • TEF
  • Esophaeal Atresia and Stenosis

Team Approach to Differential Diagnosis
  • SLP (pediatric feeding swallowing specialist)
  • Otolaryngologist (ENT)
  • Pulmonologist
  • Gastroenterologist
  • Radiologist
  • Developmental Pediatrician

Center for Aerodigestive Disorders (CADD)
  • Monthly meeting to review complex cases and
    collaborate on differential diagnosis
  • Multidisciplinary team approach to diagnosis and
    treatment for aerodigestive cases
  • CADD clinic meets 1x per month
  • Patients see GI, ORL, Pulmonary and VFSS on same

Typical course of patient
  • VFSS documentation of aspiration of thin liquids
  • Unable to visualize laryngeal cleft on
  • Patient placed on treatment of thickened liquids
  • PCP referral to Otolaryngologist for further

Alternate treatmentsfor Type 1 LC
  • Identification and management of GERD
  • Thickened liquids
  • NG-tube or G-tube
  • These treatments may be implemented prior to
    surgical repair

Surgical treatment of Type 1 LC
  • Historically, an invasive surgical procedure
  • Endoscopic procedure
  • Robotic Procedure at Childrens Hospital Boston

Laryngeal Cleft Endoscopic repair
Timeline from diagnosis to recovery
  • VFSS
  • ORL consult
  • Direct laryngoscopy
  • Maintenance diet
  • Repair
  • Repeat VFSS 6-8 weeks after repair
  • Full recovery not documented on VFSS until 2-10
    months post surgery

Case Study Laryngeal Cleft
  • 16-month-old boy with normal growth and
  • Admitted to CHB for -respiratory distress
  • -fever of 102
  • -perioral cyanosis
  • -mother reports history of 6 episodes of
    pneumonia in the past 5 months (all LLL)

Case Study Laryngeal Cleft
  • Videofluoroscopic swallow study performed during
  • Revealed
  • silent aspiration with thin liquids
  • silent aspiration with nectar-thick liquids
  • Safe to consume honey-thick liquids, purees and
    chewable solids orally
  • Recommended nutrition consult to assess hydration
    needs on honey-thick liquids

Case StudyLaryngeal Cleft
  • INSERT VFSS HERE of pt. aspirating with thin and
    nectar-thick liquids

Case Study Laryngeal Cleft
  • PCP referral to Otolaryngology (ORL)
  • Direct laryngoscopy and bronchoscopy performed
  • Type I laryngeal cleft diagnosed.
  • 1 month later endoscopic repair of Type I
    laryngeal cleft by ORL
  • Sent home after surgery on honey-thick liquids
    (same pre-operative diet)
  • Repeat VFSS 4 ½ months s/p repair revealed no
    aspiration with thin and nectar-thick liquids
  • Patient cleared for unrestricted oral diet

Summary Vincristine Toxicity in Pediatric
Pharyngeal Dysphagia
  • Low incidence problem but with significant
    consequences for pulmonary health, swallow
    function and treatment decisions.
  • Increased awareness to respiratory symptoms in
    pediatric patients undergoing chemotherapy
  • Decreased referral time.
  • Highlights the importance of the role of the SLP
    on the dysphagia-oncology team.

Complex Decision Making in Pediatric
DysphagiaLowry, Fletcher Larson Miller,
  • Benjamin B, Inglis A. Minor congenital laryngeal
    clefts diagnosis and classification. Ann Otol
    Rhinol Laryngol 198998417-420.
  • Bermudez, M., Fuster, JL, Llinares, E., Galera,
    A, Gonzalez, C. Intraconazole-related increased
    vincristine neurtoxicity case report and review
    of literature, Journal of Pediatric Hematology
    Oncology, 2005, July 27(7) 389-92.
  • Boseley, Mark et al., The utility of fiberoptic
    endoscopic evaluation of swallowing (FEES) in
    diagnosing and treating children with Type 1
    laryngeal clefts. International Journal of
    Pediatric Otorhinolaryngology (2006) 70, 339-343.
  • Chien, Wade et al., Type 1 laryngeal cleft
    Establishing a functional diagnostic and
    management algorithm, International Journal of
    Pediatric Otorhinolaryngology (2006). Article in
  • Cotton, R.T. Prescott, C.A.J. 1998. Congenital
    anomalies of the larynx. In Cotton, R.T. Myer,
    C.M. (eds). Prescribed paediatric otolaryngology
    497-513. Philadelphia Lippincott-Raven.
  • Jeng, MR, Feusner, J. Itraconazole-enhanced
    vincristine neurotoxicity in a child with acute
    lymphoblastic leukemia. Pediatric Hematology
    Oncology. 2001, March 18 (2) 137-42.
  • Langmore, Susan. Evaluation of oropharyngeal
    dysphagia which diagnostic tool is superior,
    Curr. Opin. Otolaryngol. Head Neck Surg. 11
    (2003) 485-489.
  • Parsons, D, Stivers, F, Giovaeto, D, Phillips, S.
    Type1 posterior laryngeal clefts, Laryngoscope
    108, March 1998. 403-410.
  • Schulmeister, Lisa, RN, MN, CS, OCN. Preventing
    Vincristine Sulfate Medication Errors. Oncology
    Nursing Forum, Volume 3, No. 5, E90-E98.
  • Watters, K, Russell, J. Diagnosis and management
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    Otorhinolaryngology. 67, June 2003. 591-596.