1. science
2. medicine
3. surgery

REVIEW
URRENT
C
OPINION
Management of unilateral true vocal cord paralysis
in children
Jennifer Setlur and Christopher J. Hartnick
Purpose of review
Historically, information gained from the treatment of unilateral true vocal cord paralysis (UVCP) in adults
was the same used to treat children. Today, there is a growing body of literature aimed specifically at the
treatment of this condition in children. It is an area of growing interest as UVCP can significantly impact a
child’s quality of life.
Recent findings
Children with UVCP may present with stridor, dysphonia, aspiration, feeding difficulties, or a combination
of these symptoms. Diagnosis relies on laryngoscopy, but other adjuncts such as ultrasound and laryngeal
electromyography may also be helpful in making the diagnosis and forming a treatment plan. In many
instances, there is effective compensation by the contralateral vocal fold, making surgical intervention
unnecessary. Children who cannot compensate for a unilateral defect may suffer from significant dysphonia
that can affect their quality of life because their ability to be understood may be diminished. In these
patients, treatment in the form of medialization or reinnervation of the affected recurrent laryngeal nerve
may be warranted.
Summary
UVCP is a well recognized problem in pediatric patients with disordered voice and feeding problems.
Some patients will spontaneously recover their laryngeal function. For those who do not, a variety of
reliable techniques are available for rehabilitative treatment. Improved diagnostics and a growing
understanding of prognosis can help guide therapy decisions along with the goals and desires of the
patient and his or her family.
Keywords
dysphagia, hoarseness, laryngology, pediatric, vocal cord paralysis, voice
INTRODUCTION
The majority of otolaryngologists caring for
children can expect to encounter patients with
unilateral vocal cord paralysis (UVCP). The literature suggests that tertiary care pediatric otolaryngology practices see 4–10 patients a year [1–3].
Whereas bilateral immobility tends to present with
signs and symptoms of airway obstruction, such as
retractions, cyanosis, and even apnea, UVCP is more
frequently associated with noisy breathing and
symptoms associated with an incompetent larynx,
such as difficulty with feeds [1,4]. The focus of this
article is to review the differential diagnosis and
workup of pediatric UVCP, followed by a focus on
diagnostic techniques and the growing variety of
surgical efforts available to treat children.
PRESENTATION
Stridor is present in up to 70% of young children
with UVCP [5]. Additionally, poor feeding, weak cry,
cyanosis, and aspiration are common in neonates
and young children. Hoarseness and poor vocal
projection are more often the chief complaints in
older children and adolescents.
History and physical examination should first
determine the stability of the airway. Once a stable
airway is either assessed or established, the onset of
symptoms in relation to birth, intubation, surgery,
recent illness, or medical therapy can be explored. In
addition to a detailed head and neck examination,
the chest should not be overlooked. History and
Department of Otolaryngology, Massachusetts Eye and Ear Infirmary
and Department of Otology and Laryngology, Harvard Medical School,
Boston, Massachusetts, USA
Correspondence to Christopher J. Hartnick, 243 Charles Street, Boston,
MA 02114, USA. Tel: +1 617 523 7900; e-mail: christopher_hartnick@
meei.harvard.edu
Curr Opin Otolaryngol Head Neck Surg 2012, 20:497–501
DOI:10.1097/MOO.0b013e3283590b56
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Pediatric otolaryngology
physical examination alone is successful in determining cause of unilateral vocal fold paralysis in two
out of three adults with unilateral vocal fold immobility [6].
CAUSES OF UNILATERAL VOCAL CORD
PARALYSIS
The causes leading to UVCP may be congenital or
acquired. The age of the patient along with the
history can help direct the physician to make an
accurate diagnosis. Neonates who suffer cervical
trauma in the setting of a complicated or prolonged
vaginal delivery may present with UVCP [2]. In
other children, central pathologies, such as Arnold
Chiarri malformations, may cause vocal fold immobility in the very young, and it is important to
remember to track the entire course of the recurrent
laryngeal nerve (RLN) from brain and brainstem
down to the chest and back to the neck when
evaluating affected children with no obvious cause.
Congenital left VCP that occurs in association with
thoracic vascular or cardiac anomalies is known as
cardiovocal syndrome [2]. For as many as 30–50% of
patients with UVCP at birth, a cause is never determined [2,7]. When a cause is determined, the most
common of it is typically aberrant thoracic vasculature, but again, in many cases, a cause is not found.
For other children, iatrogenic injury may be the
cause. This can occur in any age group, although it is
seen most often after patent ductus arteriosus (PDA)
ligation [8]. Rates of RLN injury after PDA ligation
between institutions vary widely (8–62%) [9,10].
The rate of UVCP is on the rise due to the increased
number of pediatric cardiothoracic procedures [5].
Extremely low birth weight (<1 kg) children undergoing PDA ligation appear to be at increased risk for
UVCP [10]. UVCP is also associated with repair of
other congenital anomalies, such as tracheoesophageal fistula or esophageal atresia [11 ]. Infection
should not be overlooked as a cause of pediatric
UVCP. Although this is more reported in the adult
literature, children are also at risk. Reports of paralyzed vocal cords as a sequela of Epstein–Barr
virus, polio, and Lyme disease have all been reported
[12–14].
Neoplasms of the central nervous system, neck,
or mediastinum can cause vocal fold paralysis by
invasion. Alternatively, UVCP in the setting of cancer may be related to RLN neuropathy secondary to
vinca alkaloid chemotherapy [15 ]. The majority of
reported cases of VCP after chemotherapy administration have been bilateral, but there have been
some reports of UVCP. It remains unclear exactly
why the process is unilateral in some cases and
bilateral in others. One assumption is that children
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with UVCP secondary to chemotherapy are experiencing a progressive neuropathy that has not yet
advanced, given that neuropathies secondary to this
treatment are sometimes not uniform in onset and
severity. Childhood malignancies, such as lymphoma and sarcoma, are commonly treated with
these types of agents, which should be considered as
the cause of VCP.
INITIAL DIAGNOSIS
When a child is suspected to have UVCP, one should
proceed with a complete evaluation of the airway,
even if the diagnosis is evident. Synchronous airway
lesions including laryngomalacia, tracheomalacia,
subglottic stenosis, intubation granuloma, cricoarytenoid joint fixation, or laryngeal webs can be
present in up to 45% of patients [1]. Patients who
present with a primary aerodigestive complaint
should undergo flexible fiberoptic laryngoscopy in
the office. Scopes as small as 2.9 mm in diameter
allow for improved physical examination of these
patients [16]. Recording these examinations is
particularly helpful, as a real-time view of the larynx
in the infant or small child can be challenging,
and viewing the examination in slow motion is
necessary. In addition, videos can also be archived
for pretreatment and posttreatment comparisons.
There are still rare occasions when a child cannot
be fully evaluated in the office. This is especially true
for the small infant who presents in frank distress
or who has a significant cardiac history. In these
settings, office-based laryngoscopy may be unsafe.
Examination in the operating room with directly
laryngoscopy and bronchoscopy can be done to
assess for other airway lesions. Vocal cord motion
can be assessed via a laryngeal mask airway as the
child is recovering from anesthesia. Even if a good
view of the vocal cords is obtained in this manner,
one should be extremely cautious in proceeding
with treatment planning based on this type of examination. The senior author has had one experience
in which office-based laryngoscopy revealed leftsided UVCP and the patient was brought to the
operating room for a left injection laryngoplasty.
At the time of the procedure, it appeared that the
child suffered from right-sided UVCP. The child was
woken up from anesthesia because of this conflicting examination, and repeat office laryngoscopy
revealed left-sided UVCP. The surgeon felt that perhaps there was an issue of synkinesis not fully
appreciated by either examination (office or operative) alone.
Operative endoscopy to diagnose UVCP is done
at some centers on a more regular basis than at
others. Because this requires general anesthesia,
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Management of unilateral vocal cord paralysis Setlur and Hartnick
the help of well trained pediatric anesthesiologists is
a must in order to develop the correct plane of
anesthesia. This may be met by some resistance from
parents. In patients who cannot tolerate office
laryngoscopy and who cannot undergo operative
examination, some may choose to use ultrasound
to aid in the diagnostic process. A recent study of
13 children with VCP showed that ultrasound can
yield quantitative results that can be used to diagnose immobility [17 ]. Obtaining useful results
from this examination is technician-dependent
and entails a steep learning curve. Nevertheless, it
may be helpful in certain situations.
Assessment of patients with UVCP is not
uniform among providers. The specifics of the
assessment after full evaluation of the airway are
tailored on a case-by-case basis. For instance, an
extensive assessment may not be warranted for
the child who suffers from UVCP after a PDA
ligation. In other instances, in which the cause
may be less clear, examination of the child should
include a thorough physical examination of the
head and neck and magnetic resonance imaging
from the brain to the thoracic inlet to image the
entire course of the RLN. In our practice, we include
Lyme titers as part of this work-up as we live and
work in an endemic area. For the most challenging
cases in which a cause still cannot be found, we
proceed with adjunct diagnostics (see below).
but can take more time than the PVRQoL to
complete.
VOICE-RELATED QUALITY OF LIFE
TREATMENT
For patients who present with a primary voice
complaint, and in general for older children and
adolescents, specific subjective and objective voice
measures should be obtained. A complete evaluation by a voice therapy specialist is important.
The aerodynamic profile allows for subjective
measurement of voice changes following therapy.
A second method of obtaining subjective measurements involves administering quality-of-life surveys. The pediatric voice-related quality of life
questionnaire (PVRQoL) is a validated instrument
that is simple to administer and complete and is
responsive to changes in voice-related quality of life
[18]. It can be used in the general pediatric patient
population and is a 10-question survey that is
scored on a scale of 0–100. Lower scores reflect less
impact on quality of life than higher scores. The
Pediatric Voice Handicap Index (pVHI) is the other
commonly cited vocal quality-of-life instrument
and has been validated in children [19]. Scaled
scores range from 0 to 120 points with higher scores
representing worse perceived quality of life. It is
used to differentiate between the different areas
in a child’s life that a voice disorder can affect,
Much of the decision-making depends not only
upon the age and symptoms of the child but also
upon the skills and experience of the surgeon. The
desires of the parents also play an important role as
do those of the child if he or she is old enough to
participate in the discussion.
The treatment of neonates, or children younger
than school age, should focus on airway protection
and alimentation. Tracheotomy can have a role, but
is usually necessary only if synchronous airway
lesions are present. Speech therapy evaluation and
modified barium swallow can clarify dietary recommendations. Enteral nutrition via gastric or nasogastric tubes may be needed, but simple thickening
of feeds is often all that is necessary.
In many instances (80% of cases of UVCP), there
is effective compensation by the contralateral vocal
fold, making intervention unnecessary. For those
who have a small but persistent glottal aperture
defect (20% of cases), speech therapy can be used
to strengthen compensatory methods of glottic
closure while minimizing risk of hyperfunctional
dysphonia [22]. This can improve feeding in
addition to dysphonia. For children who improve
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ADJUNCT DIAGNOSTICS
Management of UVCP in children can depend on
the underlying cause, as there may be a possibility
that a unilateral deficit may spontaneously recover.
Recovery of function after an 11-year interval has
been previously reported [1]. Adjunct testing may be
helpful in predicting recovery of vocal cord motion.
Intraoperative laryngeal electromyography (LEMG)
has been shown to provide this information and is
safe in children and easy to use. First, it can help
differentiate paralysis from fixation [20]. Second, it
can give general prognostic information as demonstrated by Maturo et al. [21]. In this longitudinal,
prospective study, 25 children with vocal fold
immobility (18 unilateral and seven bilateral)
and a mean age of 21.4 months were followed over
a 1-year period of time. Twelve children had UVCP
after a PDA ligation. Results showed that in these
children, if there are no normal-appearing motor
unit action potentials (MUAPs) by 6 months, then it
is highly unlikely that vocal fold function will
recover. Overall, for children with UVCP irrespective of cause, LEMG accurately predicted the outcome of (return or persistent absence) vocal cord
function in 17 of 18 patients.
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with conservative measures, meaning that the airway is adequate and there are no feeding difficulties,
no further treatment is needed. If there are persistent symptoms despite conservative management,
surgical treatment should be considered. These
patients should be divided into three age groups:
the young infant, the mature adolescent, and the
age in between.
For the very young infant who presents with
aspiration, treatment is focused upon protecting
against aspiration while supporting the airway. In
this group, an injection laryngoplasty may be the
first-line treatment. The availability of a variety
of injectable materials makes this intervention
applicable to a broader group of patients. The
senior author prefers to use Radiesse Voice (Merz
Aesthetics, San Mateo, California, USA) for this procedure. This allows for immediate results, but they
are short-term and the child may need repeated
injections to remain symptom-free. Despite the
need for repeat procedures to maintain effect, it is
a safe procedure in the pediatric population [23 ].
Long-term effects of serial vocal cord injection, such
as scarring, have not been studied. Because injection
laryngoplasty is not a permanent solution, it can be
used in patients who may one day regain vocal cord
function. More invasive and more definitive treatments can then be reserved for patients in whom
function is not expected to return.
For the mature adolescent who is otherwise
healthy and who can tolerate an awake medialization thyroplasty, this may be a viable option. The
patient should be one who can tolerate local anesthesia; this allows the medialization to be fine-tuned
for improved dysphonia, as the patient can phonate
during the procedure while the surgeon manipulates the implant [8,24 ]. The benefit is a
definitive procedure that limits the need for future
intervention. The disadvantage is that a thyroplasty
is static, whereas the size, thickness, and composition of the larynx changes as a person progresses
from childhood to adulthood. Good results
obtained initially may decline as the child ages
and grows. Because this procedure is done to treat
dysphonia, which is not life-threatening, it may be
prudent in some cases to wait for spontaneous
recovery or to abstain from surgical intervention
until a child has gone through puberty in order to
get the best result.
For the child who is in between these ages with a
primary voice disturbance, reinnervation procedure
should be considered [8]. The goal of this procedure
is not to produce a mobile vocal fold, but rather to
restore its tone, thereby medializing it to a position
wherein the contralateral mobile vocal fold can
make adequate contact and allow for appropriate
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glottal closure. The theoretic advantage of this procedure over traditional thyroplasty that makes it
attractive for pediatric application stems from it
being performed while the child is intubated under
general anesthesia without the need for intraoperative patient compliance for vocal tuning; the
results of reinnervation are maintained long-term
[25]. The results of ansa-RLN anastomosis may take
3–6 months to show evidence of effect. The time
delay for reinnervation can be palliated by performing injection laryngoplasty at the same time as the
reinnervation procedure. Reinnervation negates any
chance of spontaneous recovery; therefore, it should
be considered only after an observational period.
LEMG should be performed in the preoperative
assessment period, as any detection of activity
should steer the surgeon away from reinnervation
and toward a more temporary treatment (injection
laryngoplasty). Because the laryngeal skeleton is not
altered, additional phonosurgery is not compromised if needed later in life. Choosing between a
medialization thyroplasty and a reinnervation procedure, as both are long-term solutions, depends
on the individual case and the experience of the
surgeon.
CONCLUSION
Evaluation and treatment of the child with UVCP
requires an understanding of the diverse causes,
aerodigestive sequelae, various diagnostic choices,
and surgical options. As diagnostic techniques continue to improve, physicians and their patients will
have more choices to evaluate the problem without
causing significant morbidity to the patient. Surgical variety is likewise improving, allowing a broader
group of patients to be treated.
Acknowledgements
None.
Conflicts of interest
There are no conflicts of interest.
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READING
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&
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