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Pediatric Rehabilitation, January 2006; 9(1): 6575

Outcome of intensive oral motor and behavioural interventions for


feeding difficulties in three children with Goldenhar Syndrome
E. P. CLAWSON1, K. S. PALINSKI2, & C. A. ELLIOTT1
1

Childrens Hospital, Richmond, VA, USA and 2VCU Medical Center, Richmond, VA, USA

(Received 29 June 2004; accepted 18 February 2005)

Abstract
Objective: The facial anomalies and surgeries associated with Goldenhar Syndrome often lead to feeding problems.
The purpose of this study was to demonstrate the outcome of Goldenhar Syndrome children admitted to the day patient
paediatric feeding programme.
Subjects: Three children with Goldenhar Syndrome and feeding difficulties participated, mean age 3 years. Average length
of stay was 8 weeks. Upon admission all were dependent on tube feedings.
Methods: The children received four therapeutic sessions daily. The sessions included oral motor interventions, behavioural
techniques and a structured meal. Data regarding childrens feeding were collected at admission and discharge.
Results: The patients had 52% acceptance of food/drink at admission and 88.7% at discharge. Expels were 30% at admission
and 14% at discharge. Mouth clean was 9.3% at admission and 97% at discharge. Total inappropriate feeding behaviours
averaged 83% at admission and 27.3% at discharge. Children averaged 3.4 g per meal at admission and 105.4 g at discharge.
Two children were completely weaned from tube feedings by discharge.
Conclusions: Results support the hypothesis that combining oral motor and behavioural interventions offer effective treatment
for children with Goldenhar Syndrome. Many children with Goldenhar Syndrome are reliant on tube feedings and this
regimen allows them to eat orally, thereby improving their nutrition and quality of life.

Keywords: Goldenhar, feeding, oral-motor, behaviour, OAVS, dysphagia

Objetivo: Las anomalas faciales y las cirugas asociadas al Sndrome de Goldenhar generalmente ocasionan problemas de
alimentacion. El proposito de este estudio fue demostrar el resultado en ninos con Sndrome de Goldenhar admitidos al
programa diurno de alimentacion de pacientes pediatricos.
Sujetos: Participaron tres ninos con Sndrome de Goldenhar y dificultad para la alimentacion, con un promedio de edad de
tres anos. El promedio de duracion de la estancia fue de ocho semanas. Antes de la admision todos eran dependientes de
alimentacion por sonda.
Metodos: Los ninos recibieron cuatro sesiones terapeuticas diariamente. Las sesiones incluyeron intervenciones motoras
orales, tecnicas de comportamiento y una comida estructurada. Los datos en relacion a la alimentacion de los ninos fueron
recolectados al ingreso y al egreso.
Resultados: Los pacientes tenan al momento de la admision un 52% de aceptacion de la comida/bebida, y un 88.77% al
egreso. Las expulsiones fueron de 30% a la admision y 14% al egreso. La boca limpia fue de 9.3% a la admision y de 97% al
egreso. El total de los comportamientos de alimentacion inapropiados tuvo un promedio de 83% a la admision y un 27.3%
al egreso. Los ninos tuvieron un promedio de 3.4 gramos por alimento en la admision y de 105.4 gramos al egreso. Dos
ninos fueron separados completamente de las sondas de alimentacion al egreso.
Conclusiones: Los resultados apoyan la hipotesis de que la combinacion de las intervenciones motoras orales y de
comportamiento, ofrecen un tratamiento efectivo para los ninos con Sndrome de Goldenhar. Muchos ninos con Sndrome
de Goldenhar dependen de sondas de alimentacion, y este regimen les permite alimentarse por va oral, y por lo tanto
mejorar su nutricion y su calidad de vida.

Introduction
Goldenhar Syndrome is a constellation of
deformities of the head and neck. The incidence
of this syndrome is 1/30001/5000 live births,

with a male-to-female predominance of 3 : 2 [15].


Facial anomalies associated with Goldenhar
Syndrome include ear abnormalities, periauricular
tags, anotia, mandibular hypoplasia, orbital
dystopia, cervical spine anomalies and ocular

Correspondence: Elizabeth P. Clawson, PhD, LCP, Pediatric Feeding Program, 2924 Brook Road, Childrens Hospital, Richmond, VA 23220, USA.
Tel: (804) 228-5878. Fax: (804) 228-5989. E-mail: bclawson@chva.org
ISSN 13638491 print/ISSN 14645270 online/06/010065-75 2006 Taylor & Francis
DOI: 10.1080/13638490500144809

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E. P. Clawson et al.

dermoids. Cranial nerve palsies as well as multiple


central nervous system anomalies can also be
present. Up to 70% of these anomalies are asymmetric and unilateral. During speech and swallowing, the movement of the affected side of the
oropharynx and larynx is decreased in comparison
to the unaffected side. In children with bilateral
presentations, movement of both sides is decreased
or absent [1]. Children with Goldenhar Syndrome
often have associated cleft lip and palate, which,
along with their small mandibles, often require
surgery [6,7]. There can also be anomalies in the
structure and function of the tongue [4].
The facial anomalies associated with Goldenhar
Syndrome and the surgery these children often go
through lead to multiple feeding problems in children with this disorder. Feeding difficulties related
to facial anomalies can include an under-developed
maxilla or mandile, hemifacial microsomia, high
arched palate, facial palsy, malocclusion, cleft lip
and/or cleft palate, crammed teeth and irregularly
developed pharynx, palate or tongue [8]. These
children often suffer from frequent regurgitation,
swallowing of air and aspiration, which can result in
failure to thrive and pneumonia [7].
Another symptom of Goldenhar Syndrome that
can lead to feeding problems is a decrease in
muscle tone. In a study of patients with Goldenhar
Syndrome, Cohen et al. [9] found 46% of patients
with decreased muscle tone in the oral-facial
musculature. Eighty-two per cent of these patients
had feeding disturbances, swallowing disruption or
failure to thrive. In this study, Cohen et al. suggested
a strong association between oral-facial hypotonia
and feeding difficulties. Yokochi et al. [7] stated that,
although some infants with this syndrome, especially
those having cleft palate, have been empirically
known to have feeding difficulty, this frequency had
not been systematically studied. This study also
concluded that treatment for refusal of oral intake
caused by prolonged tube feeding may also be
necessary in some patients.

Due to the array of facial malformations in


Goldenhar Syndrome, there are various dental and
surgical procedures that restructure the muscles and
bones of the face. These procedures can help
structurally enhance feeding potential. However,
children who undergo multiple craniofacial surgeries
can develop oral motor problems, feeding problems
and oral aversions. Once the physical problems are
resolved and the surgeries are completed, the oral
motor problems are often still present. These feeding
difficulties and learned cycles of oral aversion serve as
barriers to transitioning to oral feedings [6]. By using
a behavioural approach to decrease oral aversion, the
compliance with therapy increases and outcomes are
enhanced [10]. However, there is a lack of research
pertaining to specific feeding interventions for children with Goldenhar Syndrome. More research is
needed to determine the effectiveness of oral and
behaviourally based feeding programmes with medically complex children. The purpose of this study
is to discuss the effectiveness of a comprehensive
behavioural based feeding programme to improve the
feeding abilities of three children with Goldenhar
Syndrome.

Methods
Participants and setting
Three children with Goldenhar Syndrome and
feeding difficulties participated in this study. Names
have been changed to provide patient confidentiality.
Patient characteristics are outlined in Table I.
The mean age of these children was 36 months
old (range 1542 months). The average length of
stay was 37 treatment days (range 2844 days).
Upon admission, all children were dependent on
feeding tubes (two gastrostomy and one jejunostomy) and had no significant oral intake. All children
had participated in ongoing outpatient therapy to
address feeding difficulties. Parents reported no
significant improvements with early intervention
services provided by general paediatric practitioners.

Table I. Characteristics of the three patients with Goldenhar Syndrome included in this study.
Patient characteristic
Age at admission to feeding programme
Gestation at birth
Type of tube feeding
Gastroesophageal reflux
Vision difficulties
Hearing loss
History of cleft lip and palate
Vertebral problems
Tracheostomy
Recurrent otitis media

Susan

Kelly

John

15 months
39 weeks
Gastrostomy
Yes
None
Yes, severe
No
Sacral agenesis
No
Yes

42 months
24 weeks
Gastrostomy
No
Dwayne syndrome
Yes, mild
Yes
Clefting lower thoracic spine
No
Yes

38 months
38 weeks
Jejunostomy
Yes
Blind
Yes, severe
Yes
Scoliosis
Yes
Yes

Behavioural interventions for feeding difficulties


The medical issues of the children were treated as
optimally as possible prior to being admitted to this
programme.

Case 1. Susan was a 15-month-old girl with


gastrostomy tube (G-tube) dependency and whose
Goldenhar Syndrome manifestations include severe
hearing loss requiring bilateral hearing aids, sacral
agenesis, Arnold Chiari malformation, vesicourethral
reflux, laryngomalacia and multiple episodes of otitis
media. She was born near term (39 weeks gestation)
weighing 2.98 kg. Susans surgical history included
the placement of her G-tube and a Nissen fundoplication. Susan primarily used sign language to
communicate. She was allergic to milk products.
Upper endoscopy demonstrated aphthus ulcerations in her oesophagus and colonoscopy done on
the same day revealed lymphonodular hypoplasia,
indicative of milk/protein intolerance. A gastric
emptying scan showed that Susans gastric emptying
was delayed by 120 minutes. Upon arrival to the
feeding programme, Susans medications included
Cefaclor for prophylactic treatment of otitis
media, Omeprazole, for gastroesophageal reflux
and retching, and liquid glycerin enemas daily
for chronic constipation. Susan had mild gross
motor skill delays. At the time of admission,
Susans feeding behaviours included food refusal,
expelling, gagging and retching. She typically pushed
her spoon away, would not open her mouth and
turned her head away when presented with food.
She was receiving 100% of her nutritional needs
via G-tube. A videofluoroscopic swallow study done
prior to admission to the day treatment programme
was within normal limits. Findings were unchanged
during a repeat study done during her admission.

Case 2. Kelly was a 42-month-old girl with G-tube


dependency and whose Goldenhar Syndrome
manifestations included an incomplete cleft lip and
nose, a cleft palate, facial abnormalities including
small mandible, small oral cavity, high palate,
abnormal tongue and periauricular skin tags. She also
had mild hearing loss, clefting of the lower thoracic
spine, Dwaynes Syndrome affecting her eyes, periventricular leukomalacia and lipoma of the brain.
Kelly was 8 months behind developmentally and
suffered from recurrent otitis media. Kelly was a twin
birth at 24 weeks, weighing 530 g. She had retinopathy of prematurity with vision loss and wore
corrective glasses. Kelly also had a small oral cavity,
with an abnormally shaped tongue that deviated to
the right. Kellys past surgical history included laser
surgery for retinopathy of prematurity, gastrostomy
with Nissen fundoplication, left arm z-plasty for

67

amniotic bands, small bowel lysis of adhesions,


costochodral rib graft to her jaw, jaw distraction
surgery, soft palate closure, cleft lip repair, tongue
clipping, tear duct probe/tube surgery, epibulbar
dermoid removal from left eye and periauricular tag
removal. Kellys only medication was Glycopyrrolat,
to decrease oral secretions. At the time of admission,
Kelly was receiving 100% of her caloric needs via Gtube. During the baseline sessions, Kelly was capable
of consuming 2% of her recommended calories by
mouth. She typically refused food and would pack
(holding food for greater than 30 seconds) and expel
when she did accept. She did not actively swallow
any bites at admission. Prior to admission,
a videofluoroscopic swallow study was performed
using a syringe that demonstrated adequate airway
protection; however, there was trace nasal regurgitation, no bolus control, pooling in the anterior floor,
no lip closure and no true swallows were elicited.

Case 3. John was a 38-month-old boy with


tracheostomy tube, G-tube and jejunostomy tube
( J-tube) dependency. Johns Goldenhar Syndrome
characteristics included jaw deformity, unilateral
cleft lip and palate, bilateral microphthalmia with
blindness, unilateral atresia of the right ear and
abnormal cochlea on the left, resulting in profound
deafness, tethered tongue, hemifacial microsomia,
oral pharyngeal inco-ordination with aspiration and
scoliosis. Additional diagnoses included oral aversion, oral dysphagia and gastroesophageal reflux.
John suffered from the Prosanski 3 defect, which
resulted in an unstable airway, for which he received
immediate intubation at birth and a tracheostomy
at 1 week old. John was born via ceasarian section
secondary to pregnancy-induced hypertension at
38 weeks gestation; he weighed 2.52 kg. Johns
past surgical history included unilateral cleft lip
and palate repair, myringotomy tubes, rightsided bone arachnoid hearing aid (BAHA), Nissen
fundoplication
with
gastrostomy,
Rouxen-Y
jejunostomy and rib graft to jaw. He aspirated
thin liquids on his most recent videofluoroscopic
swallow study done prior to admission; however,
he safely swallowed thickened liquids and puree.
John was on day 4 of 10 of Amoxicillin/Clavulanate
Potassium for a left ear infection and Ranitidine
for reflux prophylaxis when he was admitted to
the feeding programme. He had no known drug or
food allergies. Upon admission, John was receiving
100% of his nutrition through his J-tube; his G-tube
was used for venting. He refused foods presented
by mouth and had a history of vomiting when G-tube
feedings had been tried in the past.

68

E. P. Clawson et al.

General procedure including data collection


Admission. Children were referred to the feeding
programme by their general paediatrician secondary
to feeding difficulties. An inter-disciplinary team,
including a paediatric gastroenterologist, a nurse
practitioner, a behavioural psychologist and a nutritionist evaluated the children. The initial evaluation
included a general health assessment, nutritional
evaluation and feeding assessment. These three
children were all placed in the intensive day feeding
programme. The day treatment programme was
a regimen of four meals per day, 5 days per week,
designed for children with moderate-to-severe
feeding problems who were medically stable.
Day programme. The day feeding programme was
carried out by an inter-disciplinary team including a paediatric gastroenterologist, paediatric nurse
practitioner, behavioural psychologist, occupational
therapist, speech-language pathologist, feeding
technicians, registered dieticians, diet technician,
nursing staff, licensed clinical social worker and
case manager. Patients attended the programme
MondayFriday for 6 hours and were provided
four therapeutic meals each day. Each therapeutic
meal included oral motor exercises, followed by oral
feeding. Weekly inter-disciplinary team meetings
were held to review feeding data, calories consumed,
adjustments to tube feedings and the treatment
plan for the coming week.
Baseline sessions. Upon admission to the programme, anthropometric data including height
and weight were collected along with calculation of
average calories consumed and ideal body weight.
Caregivers were instructed to continue the same
schedule of tube feeds. For the first 2 days of the
admission, the parent and the therapist fed the child
without using any therapeutic intervention strategies
to determine the childs skills and amount they
were able to consume during a meal. An oral motor
assessment determined what functional, emerging
and non-productive patterns were present. A reinforcer evaluation was conducted to identify toys
and videos that could be used as motivators during
meals. Positioning was assessed and adjusted to
provide optimal postural stability during meals.
Intervention. The independent variable in this study
was a multi-component package that was made up
of four main components. These included oral
motor exercises, structured meals, behavioural interventions and caregiver training. Oral motor exercises
were performed for 1520 minutes prior to each
oral feeding. These interventions provide assisted

movement to stimulate muscle contraction and


facilitate movement against resistance to build
strength [11]. The goal of these interventions was
to increase functional response to pressure and
movement, specifically increasing the range,
strength, variety and control of movement for the
lips, cheeks, jaw and tongue.
Meals were conducted by the same primary
therapist (speech or occupational therapist) and
feeding technician throughout the admission. All
feeders are trained to greater than 80% reliability
with all data measured prior to independently working with patients. Meal length was determined
according to skill level and endurance. An audible
timer was used to indicate the end of the meal
sessions. Behavioural interventions included differential attention and prolonged presentation of food
to the childs lips until the child opened and accepted
the bite into their mouth. Preferred toys selected
by the individual reinforcement assessments, videos
and verbal praise were used to reward appropriate
feeding behaviours (e.g. accepting food, chewing
and swallowing). Reinforcement was withheld for
negative behaviours such as refusal to take the bite,
expelling food and packing. Toys were returned
and verbal praise given when appropriate behaviour
resumed. Other inappropriate behaviours such as
crying and gagging were treated via extinction and
removal of social attention. This feeding protocol
was carried out as part of each therapeutic meal.

Parent training. All caregivers observed the treatment sessions via video monitor outside the treatment room. Psychologists and social workers
accompanied parents at the viewing stations to
educate as well as address questions and concerns.
Once the child was tolerant of oral motor exercises
with the therapist, parental instruction began.
Parents were instructed using verbal, written and
hands-on demonstration in the implementation
of the prescribed exercises. Prior to initiating
home-based meals, training was also provided in
food preparation and calorie boosting (e.g. puree,
texture grading, food allergies) specific to the childs
needs. At 2 weeks prior to discharge, caregivers
began to be faded into the treatment sessions.
Caregivers received training in handling, facilitation
techniques, use of adaptive feeding utensils, as well
as the three behavioural components of the treatment
protocols: instructions, prompts and consequences
(IPC). They learned and practiced single components of the treatment and did not progress to the
next level of training until they had mastered the skill
at or above 80% accuracy. Finally, the caregiver fed
the child independently while the therapist observed

Behavioural interventions for feeding difficulties


from a video monitor and provided instruction as
needed via a wireless communication system.
Measures
Outcome measures of anthropometric data (weight
and height) were collected at admission and discharge from the programme. Feeding data were also
collected based on percentage of bites accepted,
average length of meal, grams taken per meal,
percentage of bites expelled, total inappropriate
behaviours and IPC at baseline sessions and the
week prior to discharge from the programme.
Child feeding behaviours were recorded for every
feeding session. Positive feeding behaviours were as
follows: (a) an accept was recorded each time the
child opened his/her mouth and allowed the food
or liquid to be placed in the mouth when it was
presented or touched to the lips within 5 seconds of
the presentation; (b) mouth clean was scored by the
absence of food or drink, larger that 1/4 square
inch, in the childs mouth following the acceptance of a bite and before 30 seconds had elapsed.
Negative feeding behaviours were defined as follows:
(a) expels included any food/drink that was in the
mouth and came back out past the border of the lips,
(b) total inappropriate behaviours included the total
number of bites in which negative feeding behaviour
occurred (i.e. refusing to take a bite, expelling,
packing, gagging or emesis).
The nutritional information collected included
calories consumed by mouth per treatment day and
the average grams consumed per meal. Food
was weighed prior to and following each meal;
total grams consumed per meal was the preweight minus the post-weight of all the foods. The
difference scores of all foods were added together,
spilled food and emesis (post-meal bib weight
minus the initial bib weight) were then subtracted
from the total grams to obtain a measure of actual
grams of food consumed. The average calories per
treatment day was a calorie count based on the
gram weight of the foods consumed throughout the
treatment day. Percentage of calories by mouth was
the percentage of their recommended daily intake
that was consumed by mouth.

69

Weight was measured in kilograms using an infant


Health-O-Meter bucket scale. Height was measured
in centimetres. The patients weight and height were
taken at admission and discharge, as well as twice
per week while admitted to the programme.
Specific procedure
The basic behavioural components for each of the
treatment protocols used with the patients are
described below and are outlined in Table II.
Case 1. The focus of Susans therapy was for her
to learn to accept food readily with an open mouth,
increase her skills of lip closure and decrease expels.
Susans treatment began under Protocol 1, which
included continuous tangible reinforcement and
social praise for accepts, prolonged presentations
and extinction for all other inappropriate behaviours.
The therapist had difficulty offering immediate
reinforcement while providing hands on facilitation
for lip closure. As a result, on the 4th day of treatment, Susans reinforcement schedule was switched
to Protocol 2, which included continuous access to
tangible and social reinforcement with cost response
for interrupts. She was fed using a squeeze bottle for
both formula and baby food. The squeeze bottle was
a soft plastic container with a flexible clear plastic
straw that, when pressure was applied, the liquid was
expressed. She began with a 1/8 inch diameter straw.
Significant emphasis was placed on supporting the
chin, inhibiting retraction and facilitating lip closure.
Susans mother was trained in oral motor exercises
and she performed these on Susan prior to each meal
under the supervision of a therapist. During week 3,
skill level had improved to greater than 80% for
accepts and showed and intermittent ability to accept
without expelling. Protocol 3, which added response
cost for expels, was added with re-presentation of the
bite. During week 4, pureed table food presented on
a spoon was begun. Following an increased number
of interrupts, the reinforcement schedule was changed back to continuous reinforcement, while maintaining cost response for expels and re-presentation
(Protocol 4). During week 5, the mother was trained
in the feeding protocol, preparation of the pureed

Table II. Basic behavioural components for each of the treatment protocols used with the patients in this study.
Treatment protocols
Protocol 1
Protocol 2
Protocol 3
Protocol 4

Protocol description
Continuous tangible reinforcement and social praise for accepts, prolonged presentations and extinction
for all other inappropriate behaviours.
Continuous access to tangible and social reinforcement, cost response for interrupts, prolonged presentation
and extinction for all other inappropriate behaviours.
Continuous access to reinforcement with cost response for interrupts and expels. Expels re-presented.
Continuous reinforcement, cost response for expels. Expels re-presented.

70

E. P. Clawson et al.

table food and was transitioned in as the primary


feeder. A large straw squeeze bottle (3/8 inch
diameter) was also introduced during this week.
During week 6, the mother fed all meals under direct
supervision of the therapist. Various cups were tested
with little success and the squeeze bottle was
continued for liquid intake. A larger bowl spoon
was introduced and tolerated. By the end of week 6,
Susan was consuming all of her caloric needs via
pureed table food and formula by mouth. During
week 7, Susans mother fed her for 1 day and then
her father was introduced as the feeder and fed for 3
days. Upon parent request, Susans treatment protocol was changed back to Protocol 3, continuous
access with response cost and re-presentation. The
parents felt this would allow for better generalization
to different settings and increased ease for feeding.
Susans parents were able to follow the protocol with
98% accuracy at the time of discharge.
Case 2. The focus of Kellys treatment was to
increase her acceptance of food by mouth and develop muscle control to form a bolus and co-ordinate
a swallow. During week 1, Kellys treatment began
using Protocol 1 and oral motor exercises. She was
presented with thickened milk in a squeeze bottle
with a 1/8 inch diameter straw. The liquid was
presented centrally with her head slightly extended to
keep the drink from running out of her mouth.
After accepting the drink, Kelly was cued to bring
her chin down in a slightly flexed position. She was
provided with physical and verbal cues to keep her
lips closed and elevate her tongue within her mouth
to initiate a swallow. Her meals lasted 12 minutes at
the start of treatment due to fatigue. During week 2,
Kellys protocol was changed to provide continuous
reinforcement following three facilitated swallows,
because she was too distracted by the television to
be able to work on her swallowing while watching.
During week 3, she was introduced to smooth
solids off a spoon for two out of four meals per day.
Her mother was trained in oral motor exercises and
performed them, under the supervision of a therapist, prior to each meal. During week 4, Kellys
endurance improved and the meal duration
increased to 19 minutes. Her mother was trained in
the feeding protocol and preparation of the pureed
table food. Her mother was then faded in as the
feeder on the last day of week 4 in order to begin
feedings at home over the weekend. During week 5,
Kelly was ill with an ear infection and, as a result,
began having difficulty managing large boluses and
textures. Throughout the week, Kelly demonstrated
increasing levels of packing, particularly with thickened purees. As a result, yogurt was introduced with
success and, by week 7, became part of all meals.

Kellys local speech pathologist attended 1 day of


training during week 7 and was taught the oral motor
exercises and Kellys feeding protocol. Kelly began
producing more mucous this week and at times the
meal needed to be stopped to allow her to expel the
mucous and recover from related gagging. Milk
products were eliminated in an attempt to decrease
mucous production. During week 8, Kellys treatment protocol was changed due to a spike in negative
feeding behaviours as her mother was faded in as the
primary feeder. The change included continuous
reinforcement with the addition of pennies and a
musical timer to target packing and expels. Kellys
father was also faded in as a feeder and was trained
in all oral motor exercises and the feeding protocol
during week 9. Both parents had difficulty manipulating the squeeze bottle to provide adequate
volume; therefore, instruction continued to achieve
the goal of 45 g of liquid per presentation. By
discharge, Kellys parents were able to feed her
independently with 89% IPC accuracy while the
therapist observed from outside the room. During
the last week, Kelly demonstrated significant
decrease in mucous production and improvement
in positive feeding behaviours.
Case 3. Johns goals were to learn to accept food
readily with an open mouth, decrease his gagging
and vomiting and improve his oral motor abilities.
At baseline, John refused all bites of food/drink
and he demonstrated severe defensiveness to the oral
motor exercises. John was started on Protocol 1, with
continuous reinforcement utilized to complete
the oral motor exercises. Yogurt and Pediasure in
large-size straw squeeze bottles were added into
the sessions by the 3rd day of week 1. Placement
was determined to be best on the right side with
lip support. During week 2, Johns mother was
instructed on performing the oral motor exercises
resulting in a dramatic increase in Johns compliance with the exercises. His mother performed
exercises before each meal with therapist supervision.
Johns liquids were thickened to a nectar consistency
to promote improved bolus control. John started
the negative behaviour of turning his head during
week 3. To decrease the refusals, he was given
a verbal prompt to keep his head still, face the feeder
and prolonged presentation was used until he did
not turn away. Following a noticeable increase in
secretions, John began to be regularly suctioned
before and after each meal as well as during the meal
if necessary. An empty spoon was introduced during
the oral motor exercises, but John did not tolerate
it well and demonstrated multiple negative behaviours. During week 4, Johns tolerance of the spoon
increased and yogurt was introduced on the spoon

Behavioural interventions for feeding difficulties


with a change in treatment to re-present expels
(Protocol 4). Johns parents were instructed in the
feeding protocol and began to give the instructions,
prompts and consequences as the therapist fed him.
During week 5, his parents began feeding during the
sessions. He was changed to Protocol 2 for increased
ease of caregivers feeding, which allowed John to
keep the toy and have it removed only when he did
not accept the bite. John had a repeat videofluoroscopic swallow study which indicated no aspiration
on thin liquids, thickened liquids or smooth puree
foods. His swallow was both safe and efficient to
continue increasing his oral intake. Caregivers were
trained in food preparation and calorie boosting.
During week 6, one of Johns primary nurses was
trained in all aspects of the feeding protocol and oral
motor exercises and fed him with direct supervision.
Throughout the week he had been demonstrating
symptoms of a respiratory virus as well as an increase
in negative feeding behaviours during meals.
John was sent home on day 3 of week 6, after being
diagnosed with a severe ear infection. He returned
4 days later, at which time his family came in
for a half day to complete discharge training.
By discharge, Johns parents were able to feed him
independently with 98% IPC accuracy.
Results
All three children were 100% dependent on tube
feedings at admission. During baseline sessions,
they accepted food/drink an average of 52% of the
time. This improved to 88.7% at discharge. Expels
averaged 30% at admission and decreased to 14%
100%

at discharge. Mouth clean (swallowing) improved


10-fold from an average of 9.3% at admission to
97% at discharge. Total inappropriate feeding behaviours averaged 83% at admission and were 27.3% at
discharge. Figures 14 demonstrate each individual
childs improvement from admission to discharge
in accepts (Figure 1), expels (Figure 2), mouth clean
(Figure 3) and total inappropriate behaviour
(Figure 4).
Upon admission, the children were taking an
average of 3.4 g per meal, which increased to
105.4 g per meal at discharge (Figure 5). Similarly,
calories averaged 12.1 kcal per treatment day at
admission and improved to 601.3 kcal per day at
discharge. All children were 100% dependent on
tube feedings for nutrition at admission. This
decreased as eating by mouth improved and upon
discharge tube feeding was only required for 25%
of their nutrition. Two patients, Susan and Kelly,
progressed to eating 100% by mouth and did not
require any nutritional supplementation via gastrostomy tube at discharge.
Susan and Kelly continued to be in contact with
the programme staff following discharge; however,
Johns family did not maintain communication.
Susan and Kelly did not have any hospitalizations
for aspiration or recurrent respiratory infection
following discharge. Susan continues to consume
all of her calories by mouth and her gastrostomy
tube was removed. Kelly underwent additional
craniofacial surgery and consumes the majority of
her calories by mouth; however, requires occasional
gastrostomy supplementation depending on her oral
intake during the day.

98%
94%

90%
80%
74%

Percent Accepts

70%
60%

66%
62%

Susan
50%

John
Kelly

40%
30%

30%

20%
10%
0%
Admission

71

Discharge

Figure 1. Average percent accepts at admission and discharge.

72

E. P. Clawson et al.
100%
90%
80%

Percent Expels

70%
60%
Susan
John

50%
45%

Kelly

40%
32%

30%

21%

20%
13%

10%

15%
6%

0%
Admission

Discharge

Figure 2. Average percent expels at admission and discharge.

100%
95% 96%

100%

Percent Mouth Clean (Swallowing)

90%
80%
70%
60%
Susan
John
Kelly

50%
40%
30%
20%

18%

10%
0%

7%
3%
Admission

Discharge

Figure 3. Average percent mouth clean at admission and discharge.

Case 1
Accepts, expels and mouth clean were the primary
targets for Susans treatment. Susans accepts
(Figure 1) improved to 100% after 1 week of
treatment. Accepts averaged at 98% throughout
her admission, however this dropped slightly to
94% at discharge when her parents were faded in
as the feeders. After accepts had improved, expels
(Figure 2) and mouth clean (Figure 3) were the
next targeted behaviours. With the initiation of

Protocol 3, swallowing increased from 57% to


greater than 95%. At discharge, Susan swallowed
100% of bites within 5 seconds of presentation.
Expels were addressed following the marked
improvement in mouth clean. The change in
reinforcement schedule associated with Protocol 4
brought expels down to 33% by week 4 and
ultimately to 6% upon discharge. Susans total
inappropriate behaviours (Figure 4) remained
greater than 80% until expels and mouth cleans

Behavioural interventions for feeding difficulties

73

100%

Percent Inappropriate Behavior

90%

93%

91%

80%
70%
65%
60%
50%
41%

40%
30%

Susan
John
Kelly

28%

20%
13%

10%
0%
Admission

Discharge

Figure 4. Average percent inappropriate behavior at admission and discharge.

200
180

Total Grams Per Meal

160

154

140
120

114.4

100

Susan
John
Kelly

80
60
47.7
40
20
0

8
0.7 1.5
Admission

Discharge

Figure 5. Average total grams per meal at admission and discharge.

were more directly addressed with Protocol 3 and


4. With the introduction of these two protocols,
inappropriate behaviours decreased to 42%. As skills
continued to be refined, inappropriate behaviours
continued to fade and were noted at only 13% by the
time of discharge. Volume and calories consumed
(Figure 5) increased steadily throughout the course
of treatment. This allowed for less frequent tube
feedings and resulted in Susan taking 100% of her
nutritional needs by mouth at discharge.
Case 2
The use of behavioural techniques to teach oral
motor skill was the primary focus of Kellys
admission. With the initiation of oral motor
exercises, facilitation techniques, verbal cueing and
reinforcing skill development, Kelly demonstrated an

improvement in co-ordination of oral motor musculature. This was shown in a repeat videofluoroscopic
swallow study toward the end of admission, which
demonstrated that Kelly was able to obtain lip
closure, collect the bolus with her tongue and
propel it posteriorly, triggering a normal pharyngeal
swallow in a timely manner. No nasal regurgitation
was noted in this repeat study.
Packing and expels were the primary targeted
behaviours of Kellys treatment. Kellys accepts
(Figure 1) improved immediately from 66% to
99% and stayed high (98% at discharge) with just
the initiation of a reinforcement schedule and the
structured meal. Packing developed once Kelly
began accepting food into her mouth. Initially,
Kelly did not have adequate oral motor skills to
manipulate the bolus and was only able to clean her

74

E. P. Clawson et al.

mouth 3% of the time (Figure 3). Mouth clean


steadily improved as oral motor skills developed. A
final significant improvement was noted during
weeks 89, with a shift in protocol that targeted
swallowing latency and reinforcement for clean
mouth. This resulted in mouth clean at 95% upon
discharge. Expels (Figure 2) dropped from 32%
at baseline to 10% by week 8; however, this rose
to 21% at discharge due to parents learning
how to manipulate the squeeze bottle correctly.
Inappropriate behaviours (Figure 4) were 93% at
admission and steadily declined as skills improved
and were noted at 28% by discharge. Volume and
calories consumed by mouth steadily improved
throughout the course of treatment (Figure 5). At
discharge, Kelly met 100% of her nutritional needs
by mouth.

Case 3
At baseline, John refused all bites of food or drink
and he demonstrated severe defensiveness to the oral
motor exercises. Johns accepts (Figure 1) reached
92% within the first week of admission and remained
high through the course of his treatment until his
discharge. As a result of illness, accepts dropped
from 91% to 74% during his last week of admission.
Mouth clean was addressed after a favourable
response in accepts was noted. By week 2, mouth
clean (Figure 3) improved to 88% and gradually rose
to 96% at discharge. Expels did not require direct
intervention until the spoon was introduced in week
4 and expels rose to 25%. With a change in protocol
and development of skills to eat from a spoon, the
incident of expels decreased and remained at 15%
upon discharge (Figure 2). Johns total inappropriate
(Figure 4) behaviours went down to 25% immediately following the introduction of a structured
reinforcement strategy for accepts. He had a flare
up at week 4 with the introduction of the spoon.
However, with the implementation of Protocol 4,
total inappropriate behaviours went down to 27%.
During the last week of admission, John was ill and
his inappropriate behaviours increased to 41% due to
a rise in refusals and a decrease in accepts. At
baseline, John ate less than 1 g per meal (Figure 5).
By week 2, he was up to 36 g per meal by mouth,
which steadily increased to 67 g by week 5 with the
change in reinforcement schedule. With the onset of
illness, including vomiting, total grams consumed at
discharge decreased to 48 g per meal. Johns tube
feeds were adjusted with the increase in oral feeds
to 25% by mouth at week 5. Johns tube feeds had
to be increased to 99% of his daily intake at discharge
due to his illness.

Discussion
The facial anomalies that most children with
Goldenhar Syndrome demonstrate often interfere
with the ability to consume adequate nutrition
by mouth. Subsequently, many of these children
are reliant upon tube feedings for nutrition. The
results of this study demonstrate that a consistent,
structured model with increased frequency
and intensity of interventions was effective.
Simultaneous use of therapeutic handling/facilitation
techniques, appropriate positioning, consistent application of oral motor exercises and behavioural
interventions to enhance skill development enabled
these children to progress when all other treatment
interventions had failed.
The data for all three children demonstrated
significant improvements in accepting and swallowing food. Improved skill for oral management of the
food/drink bolus led to significant decreases in
expels, packing and total inappropriate behaviours.
Children with this level of medical complexity
require the use of behavioural strategies to overcome
their fears and negative behaviours as well as
reinforce them as they develop appropriate skills.
Therapy that provides repetitive, simultaneous use of
therapeutic tools is essential for measurable progress.
Great success was achieved with increased strength,
range of motion and co-ordination of oral motor
musculature as a result of the consistent oral motor
exercises.
Children with craniofacial anomalies can suffer
from multiple health and psychological issues that
negatively affect their quality of life and that of their
parents and families [12]. Eating with peers is a
social norm. Not being able to eat food by mouth
not only affects nutrition, but also makes these
children different from their peers in yet another
way. These results show that it is possible to help
children with Goldenhar Syndrome eat a significant
part of their daily nutrition by mouth. This ability
to eat with peers is likely to improve their quality
of life and independence in both child-care and
school environments. Parents felt that oral feeding
improved their childs quality of life. They were now
able to participate in the family mealtime as well as
other social settings. While the food preparation and
20 minute structured meals 35 times per day can be
time-consuming for families, parents reported postdischarge that the benefits of watching their child
eat by mouth far outweighed the caregiver burden.
Following discharge, caregivers modified the meal
length and frequency to minimize the impact on the
family schedule. Further research could be done
in this area to assess quality of life in oral feeders
vs tube-fed children in the school environment.

Behavioural interventions for feeding difficulties


There were a few limitations to this study. This
is a case study, which by definition cannot be as
strong as a double-blinded study or a study with
a considerably larger cohort. There were only
three patients in this study and one of the children
became ill prior to concluding the programme.
These children all travelled a long distance to
the institution for treatment and, therefore, received
follow-up care from their local physicians, giving
minimal follow-up data. Further analysis of more
children with Goldenhar Syndrome would indeed
be advantageous. While a double blind study is often
desirable, this is not possible in a situation where oral
motor exercises are extremely important.
In conclusion, many of these children require
tube feedings to achieve their daily nutritional goals.
The three children in this study were all 100%
dependent upon tube feedings for nutrition upon
admission to the day programme. Two of them were
able to consume 100% of their daily nutritional
requirements by mouth by the time they were
discharged from the programme, with no supplementation through their tubes. The ability of this
combined treatment regimen to have such success
in these two children should be a source of great
hope and further research for the oral feeding of
many children with facial abnormalities and tube
dependency.

2.

3.

4.

5.
6.

7.

8.

9.

10.

11.

References
1. DAntonio LL, Rice RD, Fink SC. Evaluation of pharyngeal
and laryngeal structure and function in patients with

12.

75

oculo-auriculo-vertebral spectrum. Cleft Palate-Craniofacial


Journal 1998;35:333341.
FACES: The National Craniofacial Association [Internet].
Chattanooga, TN; 30 April 2004. Goldenhar syndrome.
Available online at: http://www.faces-cranio.org, accessed 2
July 2004.
Hunt JA, Hobar PC. Common craniofacial anomalies:
The facial dysostoses. Plastic and Reconstructive Surgery
2002;110:1711725.
Jones KL. Oculo-auriculo-vertebral spectrum. Smiths recognizable patterns of human malformation. 5th ed. Philadelphia:
W. B. Saunders Company; 1997. pp 640646.
Tinaz M, Oysu C. Goldenhars syndrome. Ear Nose Throat
Journal 2002;81:184.
Stellzig A, Basdra EK, Sontheimer D, Komposch G.
Non-surgical treatment of upper airway obstruction in
oculoauriculovertebral dysplasia: A case report. European
Journal of Orthodontics 1998;20:111114.
Yokochi K, Terasawa S-I, Kono C, Fujishima I. Dysphagia
in children with oculo-auriculo-vertebral spectrum. Dsyphagia
1997;12:222225.
Richard GJ, Hoge DR. Goldenhar syndrome. The source
for syndromes 2. East Moline: LinguiSystems, Inc.; 2000.
pp 5261.
Cohen MS, Samango-Sprouse CA, Stern HJ, Custer DA,
Vaught DR, Saal HM, Tifft CJ, Rosenbaum KN.
Neurodevelopmental profile of infants and toddlers with
oculo-auriculo-vertebral spectrum and the correlation of
prognosis with physical findings. American Journal of
Medical Genetics (Neuropsychiatric Genetics) 1995;
60:535540.
Clawson E, Elliott C, Bishop A. When that mouth just wont
open: Strategies for treating children with severe oral
aversion. Advance for Occupational Therapy Practitioners
2003;19:42.
Beckman D. Oral motor assessment and intervention.
Longwood: Beckman & Associates, Inc, 1995.
Warschausky S, Kay JB, Buchman S, Halberg A, Berger M.
Health-related quality of life in children with craniofacial
anomalies. Plastic and Reconstructive Surgery 2002;110:
409414.

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