Type 1 Chiari Malformation is a rare congenital defect resulting in the extension of the cerebellar tonsils into the
foramen magnum towards the upper spinal canal1, 2 . This results in pressure on the cerebellum and brainstem, possibly
impacting the flow of the cerebrospinal fluid (CSF). The CSF can accumulate and form a cyst within the spinal cord’s central
canal, causing pressure and damage to the spinal cord (syringomyelia) or brainstem (syringobulbia). Variations in the fluid
pressure on the spinal canal often lead to different symptoms, depending on the sensory and motor cranial and or spinal
nerve pathways affected.
A syrinx extending into the medulla can result in dysphagia, with tongue, palate and pharynx weakness as well as
sensory deficits from trigeminal nerve involvement3, 4, 5 . Symptoms of Chiari Malformation can include but are not limited
to dysarthria, dysphagia, neck pain, headaches, dizziness and balance problems, muscle weakness and altered sensation,
vision changes or hearing loss. Symptoms can manifest during childhood or develop later in life1, 2, 6, 7 .
Case Study
A 19 year old female presented with:
•Increasing headaches, dysphonia, hypernasality, dysphagia.
• Decreased gag reflex.
• MRI results: Type 1 Chiari Malformation, C1 fusion to the skull base, crowding of the foramen magnum, compression
of the medulla and a large cervicothoracic syrinx.
• Paralyzed right vocal fold per ENT assessment.
• Cranial nerves IX and X involvement per Physiatrist examination.
Clinical Swallow Assessment & MBS #1Pre-op (09/2010)
Clinical assessment:
• Patient reports coughing and choking on foods and
liquids, with nasal regurgitation unless she pinched
her nose shut.
• Patient uses a slow deliberate swallowing technique
and an unconscious elimination of more difficult
foods, such as mixed consistencies, to reduce the need
to plug her nose.
MBS results:
•Velopharyngeal Port movement:
• Soft palate observed lying flat against the tongue
during rest without complete closure of the
velopharyngeal port during production of vowels.
•Timing:
• Moderate-severely delayed pharyngeal swallow
demonstrated by thin liquid bolus reaching the
pyriform sinuses before the swallow was triggered.
•Muscle Recruitment:
• During the swallow of thin and nectar liquids a
Passavant's ridge was observed with contraction of the
superior constrictor muscle.
• During the swallow of liquids and solids, recruitment
of the medial constrictor muscle is also visible
intermittently.
• Every swallow required a Passavant's ridge and
effortful posterior tongue base movement and
velopharyngeal port closure remained incomplete.
•UES and LES Function:
• Retro movement or "sticking" of liquid bolus in lower
esophagus was observed during an esophageal
sweep.
•Use of Strategies:
• Slow, smaller boluses appeared to decrease nasal
regurgitation.
• Head turn to the side of vocal fold paralysis (right
side): increased the difficulties, probably due to the
limited cervical movement caused by the fusion of the
C1 vertebra to the skull.
• Head turn to the left: her swallow became more
controlled and she reported more ease in swallowing
liquid.
Treatment
MBS #2: Post-op (11/2010)
Chiari Malformation
Velopharyngeal closure during vowel
production, 09/2010
Surgery:
• Posterior fossa craniotomy, duraplasty, and exploration of the 4th ventricle.
• Relieved pressure on the foramen magnum, brainstem, and cerebellar tonsils.
• Excellent flow of the CSF was noted after exploration of the 4th ventricle.
MBS results:
•
4 days after the decompression surgery.
•
Velopharyngeal Port movement:
•
Slight worsening of the oropharyngeal dysphagia with velopharyngeal insufficiency.
•
Timing:
•
Reduced hyolaryngeal rise and excursion as well as incomplete epiglottic inversion.
•
Silent aspiration with thin liquids and penetration with thin and nectar liquids due to a
moderate-severe delay in the pharyngeal trigger.
•
Muscle Recruitment:
•
No change in the use of the Passavant’s ridge or middle constrictor .
•
Use of strategies:
•
Edema was suspected, resulting in decreased cervical movement, limiting use of
compensatory head turn.
•
Patient’s diet was downgraded to nectar full fluids.
Passavant’s ridge, 11/2010
MBS #3: Pre-treatment (03/2011)
MBS results:
• Velopharyngeal Port movement:
•
Increased elevation of the soft palate during production of the vowels /a/ and /i/.
•
Mildly delayed pharyngeal swallow demonstrated by intermittent entry of thin fluids into
the laryngeal vestibule.
•
Passavant's ridge reduced in size but still required on most swallows along with middle
constrictor recruitment and an effortful posterior tongue base movement.
• Timing:
•
Moderate-severely delayed pharyngeal swallow.
• Muscle Recruitment:
•
Most swallows continued to require the Passavant's ridge, middle constrictor recruitment
and an effortful posterior tongue base movement to sufficiently close the velopharyngeal
port to prevent nasopharyngeal regurgitation.
• Use of Strategies:
• Slow, smaller boluses decreased nasal regurgitation.
• Head turn to the right was ineffective.
• Head turn to the left, mild improvement.
• Chin tuck maneuver and use of straw with thin liquids, no significant improvement.
• General diet with thin liquids recommended.
Passavant’s ridge, 03/2011
MBS # 4: Post-treatment (08/2011)
Nasal regurgitation, 09/2010
Passavant’s ridge, 09/2010
MBS results:
•Velopharyngeal Port movement:
•
Soft palate was observed lying above the tongue in a more elevated position than
previously seen when tongue was at rest.
•
Elevation of the soft palate and closure of the velopharyngeal port was WNL during the
production of the vowels /a/ and /i/.
•
Increase in elevation of the velum during swallowing was observed compared to the
September 2010 and March 2011 studies.
•
Regurgitation occurred in the pharynx, but not in the nasal passage which is a significant
improvement.
•Timing:
•
Slightly improved timing of the pharyngeal trigger and velopharyngeal closure.
•
Moderate-severely delayed pharyngeal swallow demonstrated by thin liquid bolus reaching
the pyriform sinuses before the swallow was triggered.
•
One instance of laryngeal penetration post-swallow of thin liquid.
•Muscle Recruitment:
•
Passavant's ridge continues to be reduced in size from previous studies with less middle
constrictor recruitment.
•UES Function:
•
Improved bolus flow through the upper esophagus due to apparent improvement in UES
pressure from the previous studies.
•Treatment:
•
Expiratory Muscle Strength Training (EMST).
•
Forward Focus (Forward Resonance).
•Methods:
•
EMST- Practiced 7 days a week, 5 times a day with 5 repetitions
with increases in the resistance settings on the EMST 150 device
as the task became easier to maintain a 80% workload.
•
Forward Focus – Daily home practice with 1, 2, 3 syllable words
and short phrases, then question and responses, reading aloud
and later conversation. The resonance was shaped from a
prolonged “mmm” at the beginning of the words or phrases to
natural speech.
•Baseline measurements:
•
EMST- easiest setting on the EMST 150.
•
Forward Focus in 1 syllable words starting with “mmm”.
•Treatment length:
•
EMST began in March 2011 and continued for 22 weeks before
the last MBS.
•
Forward Focus began in May 2011 and continued for 16 weeks
before the last MBS.
•Outcome measures:
•
EMST: Increased setting to most difficult setting on the EMST
150 device.
•
Forward Focus: Patient reports improved voice quality and
loudness in conversation and while reading aloud.
•
Swallowing:
• Improvement noted in velopharyngeal movement during
voicing and swallowing tasks on the MBS.
• Patient reports improved swallowing of thin liquids with no
instances of nasal regurgitation and rare instances of coughing
and food sticking.
Image retrieved from
http://chestjournal.chestpubs.org/content/135/5/1301.full8
References
Velopharyngeal closure during vowel
production, 08/2011
Velopharyngeal closure during
swallowing, 08/2011
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