Childrens Hospital, Richmond, VA, USA and 2VCU Medical Center, Richmond, VA, USA
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.
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,
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
66
E. P. Clawson et al.
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
67
68
E. P. Clawson et al.
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
69
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.
98%
94%
90%
80%
74%
Percent Accepts
70%
60%
66%
62%
Susan
50%
John
Kelly
40%
30%
30%
20%
10%
0%
Admission
71
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
100%
95% 96%
100%
90%
80%
70%
60%
Susan
John
Kelly
50%
40%
30%
20%
18%
10%
0%
7%
3%
Admission
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
73
100%
90%
93%
91%
80%
70%
65%
60%
50%
41%
40%
30%
Susan
John
Kelly
28%
20%
13%
10%
0%
Admission
Discharge
200
180
160
154
140
120
114.4
100
Susan
John
Kelly
80
60
47.7
40
20
0
8
0.7 1.5
Admission
Discharge
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.
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.
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