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
Children’s Hospital Boston
ASHA November 17, 2006
Miami, Florida
 Contact Information:
 Kara Fletcher Larson, MS, CCC-SLP
 Kara.fletcher@childrens.harvard.edu

 Alana Lowry, MS, CCC-SLP

 Alana.lowry@vtmednet.org

 Jennifer Miller, MS, CCC-SLP

 Jennifer.miller2@childrens.harvard.edu

Lowry, Fletcher, Miller ASHA 2006

 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 prematurity
 (Manikam & Perman 2000)
 Summarized in Oct. 2006 Brackett, Arvedson & Manno in SID #13
Lowry, Fletcher, Miller ASHA 2006
newsletter
 How did we get here?
 Major pediatric medical center
 Children’s Hospital Boston, MA
 2005 performed 864 pediatric
videofluoroscopic swallow studies
 Range in ages from 38 weeks PMA-
young adults with developmental
disabilities (early 20’s)

Lowry, Fletcher, Miller ASHA 2006

 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 Division
 13 % of patients referred by the
Otolaryngology Division

Lowry, Fletcher, Miller ASHA 2006

 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

Lowry, Fletcher, Miller ASHA 2006

 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
Lowry, Fletcher, Miller ASHA 2006
 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 Lowry, Fletcher, Miller ASHA 2006
 Chemotherapy Agent:
Vincristine
 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 division.
 Typically given in cycles
 Cycle typically lasts 4-6 weeks
 Period drug administration- resting
period Lowry, Fletcher, Miller ASHA 2006
 Side Effects of Vincristine:
Neurotoxicity
 Involves peripheral, autonomic, and
central neuropathy
 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
Lowry, Fletcher, Miller ASHA 2006
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 liquids
 Attending oncologist referred patient
for VFSS

Lowry, Fletcher, Miller ASHA 2006

Results of VFSS in Children
Receiving Vincristine

 Silent aspiration with thin liquids

 Silent aspiration with thin and nectar
thick liquids
 Silent aspiration with thin, nectar and
honey thick liquids
 No evidence of aspiration with
purees or solids
Lowry, Fletcher, Miller ASHA 2006
 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
Lowry, Fletcher, Miller ASHA 2006
 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

Lowry, Fletcher, Miller ASHA 2006

 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 decisionLowry,made by attending
Fletcher, Miller ASHA 2006
 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
Lowry, Fletcher, Miller ASHA 2006
 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
treatment
 Yes in some patients
 Even at reduced strength of drug (50% strength).
 Oncology team was very conservative with re-
starting chemotherapy/ altered
Lowry, Fletcher, Miller ASHA 2006 doses and child
 Case Study
Vincristine Toxicity
5/10/04: 3 ½ year old girl is diagnosed
with
acute lymphoblastic leukemia
(ALL)

Immediately begins chemotherapy

(including vincristine)

Throughout 7 months of
chemotherapy, pt. is seen
frequently in clinic for “chronic
upper respiratory tract
congestion and persistent coughing”
Lowry, Fletcher, Miller ASHA 2006
 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
treatments
VFSS revealed silent aspiration with thin
liquids

Patient safe to continue to receive

nectar-thick liquids, purees, and
chewable solids

Lowry, Fletcher, Miller ASHA 2006

 Case Study:
Vincristine Toxicity
 Insert VFSS # 1 of
silent aspiration
with thin liquids
(2/3/05)

Lowry, Fletcher, Miller ASHA 2006

 Case Study
Vincristine Toxicity
2/4/05: Vincristine component of chemotherapy
is withheld

Pt. remained on nectar-thick liquids,

purees, solids

2/28/05: Repeat VFSS continued to reveal silent

aspiration
with thin liquids

Recommendation: remain on altered

oral diet

4/21/05: Repeat VFSS revealed normal swallow

function with noMiller
Lowry, Fletcher, documentation
ASHA 2006 of
 Case Study:
Vincristine Toxicity
 Insert VFSS of
normal swallow
function with no
aspiration
(4/21/05)

Lowry, Fletcher, Miller ASHA 2006

 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

Lowry, Fletcher, Miller ASHA 2006 vincristine
 Outcome: Case Study
Vincristine 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
Lowry, Fletcher, Miller ASHA 2006
Complex Decision Making
in Pediatric Dysphagia
Part 2
Type 1 Laryngeal Cleft
What is a Laryngeal Cleft
(LC)?
 Communication between the
posterior larynx and esophagus
 Failure of tracheo-esophageal
septum to develop

Lowry, Fletcher, Miller ASHA 2006

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

Lowry, Fletcher, Miller ASHA 2006

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.

Lowry, Fletcher, Miller ASHA 2006

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

Lowry, Fletcher, Miller ASHA 2006

Classification of Laryngeal Clefts

Benjamin and Inglis, 1989

Lowry, Fletcher, Miller ASHA 2006
Lowry, Fletcher, Miller ASHA 2006
 Clinical Signs &
Symptoms of Type 1
Laryngeal Cleft
 Noisy breathing
 Inspiratory stridor
 Coughing & choking with feedings
 Chronic pulmonary infections
 Aspiration
 A’s and B’s with feedings
 Cyanosis

Lowry, Fletcher, Miller ASHA 2006

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

Lowry, Fletcher, Miller ASHA 2006

 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.

Lowry, Fletcher, Miller ASHA 2006

 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)

Lowry, Fletcher, Miller ASHA 2006

 Type 1 LC at Children’s
Hospital Boston
 30 patients diagnosed with type 1
laryngeal cleft from 2000-2005.
 21 patients repaired.
 >90% patients with improved
swallow function after repair.

Lowry, Fletcher, Miller ASHA 2006

 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?

Lowry, Fletcher, Miller ASHA 2006

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

Lowry, Fletcher, Miller ASHA 2006

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

Lowry, Fletcher, Miller ASHA 2006

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 day
Lowry, Fletcher, Miller ASHA 2006
Typical course of patient
 VFSS: documentation of aspiration of
thin liquids
 Unable to visualize laryngeal cleft on
fluoroscopy
 Patient placed on treatment of
thickened liquids
 PCP referral to Otolaryngologist for
further assessment

Lowry, Fletcher, Miller ASHA 2006

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

Lowry, Fletcher, Miller ASHA 2006

 Surgical treatment of
Type 1 LC
 Historically, an invasive surgical
procedure
 Endoscopic procedure
 Robotic Procedure at Children’s
Hospital Boston

Lowry, Fletcher, Miller ASHA 2006

 Laryngeal
Cleft
Endoscopic
repair

Lowry, Fletcher, Miller ASHA 2006

 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

Lowry, Fletcher, Miller ASHA 2006

 Case Study
Laryngeal Cleft
16-month-oldboy with normal growth
and development

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 admission:

 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

Lowry, Fletcher, Miller ASHA 2006

 Case Study:
Laryngeal Cleft
 INSERT VFSS HERE
of pt. aspirating
with thin and
nectar-thick liquids

Lowry, Fletcher, Miller ASHA 2006

 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

Lowry, Fletcher, Miller ASHA 2006
 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
treatment.
 Decreased referral time.
 Highlights the importance of the role
of the SLP on the dysphagia-oncology
Lowry, Fletcher, Miller ASHA 2006
 Complex Decision Making in Pediatric Dysphagia
Lowry, Fletcher Larson & Miller, 11-17-06
References
 Benjamin B, Inglis A. Minor congenital laryngeal clefts: diagnosis and classification.
Ann Otol Rhinol Laryngol 1989;98:417-420.
 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 press.
 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 of type 1 laryngeal cleft, Int. J.
Pediatric Otorhinolaryngology. 67, June 2003. 591-596.

Lowry, Fletcher, Miller ASHA 2006