Infernational Journal of Pediatric Otorhinolaryngology, 24 (1992) 63-71 G 1992 Elsevier Science Publishers B.V. All rights reserved 0165876/92/$05.00
PEDOT
63
00794
Swallowing disorders in a population of children with cerebral palsy * Eric T. Waterman
‘, Peter J. Koltai ‘, Jane Capria Downey ’ and Anthony T. Cacace ’
’ Dkision of Otolatyngology, ’ Section of Pediatric Otolaryngoiogy, Albany Medical College, Alban) NY 12208 (U.S.A.) and -’ Center for the Disabled, Albuny NY 12208 (U.S.A.) (Received (Accepted
Key words: Dysphagia;
Cerebral
1 November 29 November
1991)
1991)
palsy
Abstract
One of the disabilities in patients with cerebral palsy (CP) is dysphagia. To establish the prevalence of dysphagia in a population of children with CP, and to determine if any factors are related to dysphagia, we studied 56 CP patients, 5-21 years, enrolled in a primary school for the disabled. Fifteen patients (27%) had either radiographic or clinical evidence of dysphagia. These 15 patients were compared to the remaining 41 patients without dysphagia. Using data obtained from chart review and interviews with speech pathologists, several factors that contributed to dysphagia were found. These included: bite reflexes, slowness of oral intake, poor trunk control, inability to feed independently, anticonvulsant medication, coughing with meals, choking, and pneumonia. We also noted trends in the following factors: presence of tongue thrusting, presence of drooling, severity of CP, poor head control, severity of mental retardation, seizures, and speech disorders. Factors not related to the presence of dysphagia include: subject age, cause of CP, and type of CP. Early, aggressive work-up and identification in CP patients with the risk factors outlined above can reduce the associated pulmonary complications.
* Presented at the Annual Meeting of the American Hawaii. Correspondence to: P.J. Koltai, Section of Pediatric NY 12208, U.S.A.
Society of Pediatric Otolaryngology,
Otolaryngology,
Albany
Medical
May 10. 1991, College,
Albany.
64
Introduction
Cerebral palsy (CP) is defined as ‘a disorder of movement and posture due to a defect or lesion of the immature brain’ [l]. The lesion, which interferes with central nervous system maturation, is non-progressive. The incidence of cerebral palsy is estimated at 7 in 1000 live births [lo]. The severity varies greatly among subjects. In the mildest case, there are minimal motor weakness and normal cognitive abilities. In these instances, subjects function normally in society without obvious handicaps. In contrast, there is a large population of patients that have central motor handicaps accompanied by speech, hearing, and vision deficits, with or without mental retardation and seizure disorders. One additional disability that has been reported is difficulty feeding. Just as the CP patient may have abnormal posturing of the, head and trunk, and motor disturbances of the face, lips, and tongue, so too there can be less apparent abnormal pharyngeal and laryngeal mobility which can greatly impair the patient’s ability to eat. Although there is an abundance of literature dealing with the management of feeding problems in disabled children [2,4,5,7], there is no documentation in the literature of the frequency of swallowing disorders among children with CP. The goals of this study are to establish the prevalence of dysphagia in a population of children with cerebral palsy, as well as to compare these patients with CP patients with normal swallowing to determine which factors relate to the presence of dysphagia.
Materials
and methods
The subjects in this study comprised 101 schoolchildren in the Kevin G. Langan School at the Cerebral Palsy Center for the Disabled (Albany, NY). Data was obtained by reviewing medical records. The study was approved by the Human Research Review Committee of the CP Center. Patients with genetic, muscle, degenerative, or neuromuscular disorders, spinal diseases/ anomalies, postnatal trauma, or any central nervous system insult occurring after the first year of life, and any patient in whom a diagnosis of cerebral palsy was unclear, were excluded from the study. A staff neurologist at the Center for the Disabled was consulted when there was doubt regarding the neurologic diagnosis. Of the 101 records reviewed, 56 patients, 5-21 years, were identified as having cerebral palsy and form the sample for this study. Medical charts were reviewed for demographic, neurologic, surgical, and feeding data. This was obtained from the initial matriculation assessment, yearly medical and neurologic evaluations, as well as from screening exams performed by physical, occupational, and/or speech therapists. Following the chart review, a computerized database was instituted with available software (Enable/OA, The Software Group, Ballston Lake, NY). The subjects were divided into two groups based on the presence or absence of swallowing disorders. The x2 test was used to compare differences in various parameters between groups. A significance level of P < 0.05was selected a priori.
65
Results Fifteen subjects (27%) had swallowing disorders. Eight of these had abnormalities documented on barium swallow radiographs or videofluoroscopic studies. Of these eight., one had findings limited to the oral stage of swallowing, whereas the remainder had abnormalities of the pharyngeal stage. Five of these subjects aspirated. Seven subjects had clinical evidence of dysphagia without radiographic documentation: Subject 1 had a severe seizure disorder, at times having almost constant seizure activity. During these periods he experienced dysphagia. Subject 2 had recurrent aspiration pneumonia and was noted to have difficulty in swallowing and congestion during meals. Subject 3 appeared to have delayed swallowing and had undergone Nissen fundoplication for severe gastroesophageal (GE) reflux. Subject 4 had severe neck spasms which interfered with swallowing. During meals. he would often hold his breath, cough, and become plethoric. Subject 5 had a history of severe GE reflux for which she had a Nissen fundoplication and gastrostomy. She receives nutrition via gastrostomy as well as orally, but suffers from recurrent pneumonia. Subject 6 had a history of aspiration pneumonia, including one life-threatening episode requiring prolonged hospitalization. Subject 7 had severe, chronic drooling that interferes with swallowing. This group of I5 patients was compared to the remaining 41 patients with normal swallowing. The age of the patients ranged from 5 to 21 years, with a median age of 14 years. The children with dysphagia were fairly equally distributed over all ages (Fig. 1). Table I summarizes the parameters studied. Although the percentage of dysphagic patients with tongue thrusting and bitt reflexes during meals was higher than in the normal patients, only the difference in bite reflex was statistically significant. The increase in severity of drooling in dysphagic patients approached statistical significance. The rate of oral intake was significantly lower in the dysphagic group.
m
Normal
ezzd Oysphagia
10
I5
Age Fig. 1. Age distribution
of subjects. No significant
(years)
difference
was noted.
between
the normal
and dysphagic group
66
TABLE I Parameters studied. P-ualue indicates difference between normal and dysphagia group Number of patients Normal
Dysphagia
Severity of speech disorder Mild Moderate-severe Non-verbal
26
1 14
Tongue thrust No Yes
31 10
7 5
Bite reflex No Yes
36 5
5 7
Severity of drooling None Mild Moderate Severe
10 18 7 1
Rate of oral intake Normal Moderately slow Very slow
18 15 1
2 1 8
History of seizures No Yes
15 26
1 14
P < 0.10
Taking anti-convulsants No Yes
26 14
3 12
P < 0.01
Ability to feed self Independent Assistance Dependent
20 3 17
0
P < 0.005
2 12
Head control Good Fair Poor
18 10 13
2 4 9
Trunk control Good Fair Poor
13 13 15
0
Coughing with meals Never Rarely Occasionally Frequently
30 1 7 1
7 8
0
P < 0.10
P < 0.005
P < 0.10
P < 0.005
P < 0.05
5 10 0
2 6 7
P < 0.005
TABLE
I (continued)
Number
ofpatients
Normal
Dysphugirr
Choking with meals (2 + episodes in 3 months) No
40
3
Ye\
0
12
Episodes of pneumonia None
34
7
I-2
4
4
3 or more
3
4
P < 0.05
Diet Regular
or chopped
Modified
39
2
2
12
Several neurologic factors related to dysphagia. There was a statistically significant difference between groups in trunk control, ability to feed independently, and whether or not the subject received anticonvulsant medications. Other consistent but non-significant trends included more severe CP, poorer head control, lower cognitive function, more frequent seizures, and poorer speech proficiency in the dysphagia group. Factors predisposing to cerebral palsy were not associated with the presence or absence of dysphagia, nor was the type of cerebral palsy. The primary neurologic factors are summarized in Figs. 2-5. A potential significant problem is that of aspiration. There was a significant association between the presence of dysphagia and frequency of coughing with meals as well as a history of two or more choking episodes in 3 months. Overall, a large percentage (27%) of the children studied had a history of at least one episode of pneumonia. Dysphagic patients had significantly more frequent episodes.
30 v)
;
25
.-a, :
20
+0
15
k
10
f 2
m E?J
Normal
Dyaphagia
5 0 Mild
Moderate
Severity Fig. ‘7. Severity
of CP, defined
as mild for ambulatory
Severe
of CP subjects, moderate
with assistance, and severe for non-ambulatory
for subjects who ambulate
subjects.
68 10 v)
9
2 W c
a
I? Lc 0 L W 2 2
m Normal ezzd Dysphagia
7 6 5 4 : 1 0 Mild
None
Degree
of
Fig. 3.
Moderate
Mental
Cognitive
Severe
Profound
Retardation
function.
m m
Sp Puad
Sp Di
Type
Mixed
Normal Oytphogia
Other
of CP
Fig. 4. Cause of CP. A, unknown; B, perinatal asphyxia; C, prematurity; D. perinatal infection; E, postnatal infection; F, stroke/hemorrhage; G, other.
Normal ezzd Dyaphogio
25 r
m
A
B
C Cause
D
E
F
G
of CP
Fig. 5. Type of CP. Sp Quad, spastic quadraparesis; Sp Di, spastic diplegia.
TABLE
II
Relwant surgical history Procedure
Number of putients
Gastrostomy T&A Tracheotomy Salivary dive] sion Nissen fundoplication Uvulectomy Tonsillectomy
4 2 2 2 2
(7%:) (4%‘) (4%) (4%) (4%;) I (2%) 1 (2%)
Forty-two of the 56 patients (75%) in the study ate a regular or chopped diet; 39 of 41 patients (95%) in the normal group, and only 3 of 15 patients (20%) in the dysphagia group. Relevant surgical histories of the 56 patients studied are shown in Table II. The number of patients undergoing any single procedure was too small to allow statistical analysis. Only a few patients in the study had weight above the 5th percentile on standard age vs weight growth charts. This data did not appear to be related to the presence of dysphagia.
Discussion
Dysphagia may be defined as difficulty with any of the four .phases of swallowing, i.e. oral preparation, oral stage, pharyngeal stage, or esophageal stage. There is ample literature describing abnormal oromotor findings in patients with CP (e.g. abnormal tongue thrust, increased or decreased gag reflexes, oral hypersensitivity, bite reflexes, and lingual and labial dysfunction) [2,6,8]. Most of the children we studied have CP with some degree of orofacial involvement and thus have abnormalities in the oral preparatory or oral stages of swallowing. Although difficulty with these stages, particularly when there is loss of tongue control, may result in aspiration, most of these disruptions result only in the inability to eat a regular diet or loss of the pleasure of eating for the patient and feeder. Based on standard growth charts, the low percentile weights of nearly all patients in the study appear to be related more to the degree of neurologic involvement and consequent muscle-wasting than to true malnutrition. Biochemical measurements such as serum albumin, total lymphocyte count, transferrin, and nitrogen balance, as well as anthropometric measurements such as triceps skin-fold thickness or bone age, would be more accurate in assessing the nutritional state of a patient with CP, While an increase in tongue thrusting, bite reflex, and slowness of oral intake (all of which indicate oromotor involvement) were apparent, it is the difficulty with pharyngeal swallowing that is more dangerous to the patient since it may result in aspiration.
The possible areas of neurologic damage resulting in abnormal pharyngeal swallowing in cerebral palsy patients are numerous. Although in most cases the cause of cerebral palsy is unknown, many of these children have ischemic brain damage secondary to ischemia at birth. The site of damage depends on the maturity of the brain at the time of birth. Periventricular white matter infarction, including involvement of the corticofugal pyramidal tracts and oral-facial fibers involved in sucking and mastication, is characteristically seen in the pre-term brain [3]. Children suffering birth asphyxia at full term more commonly have cerebral edema, cortical necrosis, cortical-white matter ‘boundary zone’ infarction, and necrosis of thalamic and brainstem nuclei [9]. The association between severity of mental retardation and dysphagia seen in this study is consistent with the fact that much of eating is learned behavior. In addition, the automaticity of collecting and propelling the bolus into the pharynx to initiate normal reflex swallowing exists due to familiarity. Children with severe cognitive disabilities may interpret the presentation of food as an unpleasant stimulus causing choking and thus be unable to make an effective cortical decision to properly propel the bolus along the dorsum of the tongue. Normal speech requires both adequate cognitive function and intact oromotor skills. The association we found between dysphagia and poor speech production skills suggests a relationship between these variables. A large proportion of the children in this study had some degree of drooling (78%). For most of these children, drooling represents a social or hygiene problem for the patient or caregiver. It also is a visible consequence of the loss of oromotor control. Two patients in this series had surgical treatment for their drooling. In this study, 8 of 15 dysphagic children had at least one episode of pneumonia. While we cannot directly correlate episodic aspiration and pneumonia, the frequency of coughing, choking and pneumonia indicates that dysphagic children with CP suffer from a high incidence of respiratory complications. Our results clearly demonstrate that children with CP who have dysphagia can be divided into two groups. The first group comprises those children with less severe involvement who have only oromotor dysphagia. These children present significant feeding problems, primarily due to slow eating which can be extremely labor-intensive, thus present a great financial responsibility. However, these children generally do not have a health risk associated with eating; as such they have a social and therapeutic, rather than a medical, disability. The second group includes those children who have pharyngeal dysphagia demonstrable on pharyngoesophagram. This is manifest clinically as frequent coughing, episodic aspiration, and occasional pneumonia. These children are at medical risk with routine feeding even when done by capable therapists despite the fact that most have been in intensive swallow therapy programs. It is these patients who pose the greatest problems in management and raise important issues about the liability faced by therapists doing the feeding, the desirability to intervene surgically with gastrostomy, the compound financial, burden of expensive feeding programs, and the medical consequences of chronic aspiration It is our goal with studies such as this one, as well as ongoing investigations with this population, to
71
better define the criteria between dysphagia.
the medical and surgical therapy
of severe
Acknowledgement
We would like to thank the staff of the Center assistance in this study.
for the Disabled for their
References I Bax. M.C.O.. Terminology and classification of cerebral palsy. Developmental Medicine and Child Neurology. 1064; 6: 295-297. 2 Helfrich-Miller, K.R., et al., Dysphagia: its treatment in the profoundly retarded patient with cerebral palsy. Arch. Phys. Med. Rehabil., lY86; 67: 520-525. 3 Iriki A., et al.. Feeding behavior in mammals: corticobulbar projection is reorganized during conversion from sucking to chewing. Dev. Brain Res., 1988; 44: 189-196. 4 Jones. P.M., Feeding disorders in children with multiple handicaps. Dev. Med. and Child Neuroogy. 1989: 31: 404-406. 5 Logemann. J.A.. Evaluation and Treatment of’ Swallowing Disorders. San Diego, CA: f‘ollegc-Iii11 Press: 1983. 6 Love, R.J.. Speech performance, dysphagia and oral reflexes in cerebral palsy. J. Speech Hear Dis., 1980; 45: 59-7s. 7 Morris. SE., Program Guidelines for Children with Feeding Problems. Edison. NJ: Childcraft Education Corporation; 1977. 8 Mueller, fl., Feeding. In: Groher, M.E., ed. Handling the Young Cerebral Palsied Child at Home. New York. NY: Dutton; 197.5: 113-132. Y Pape, K.E.. Wigglesworth, J.S.. Haemorrhage, Ischaemia and the Perinatal Brain. Philadelphia, PA: JB Lippincott Co; 1979: 196. IO Thompson. G.H., Rubin, I.L., Bilenker. Comprehensive Management of Cerebral Palsy. Grune & Stratton: 1983: 19.
PEDOT
63
00794
Swallowing disorders in a population of children with cerebral palsy * Eric T. Waterman
‘, Peter J. Koltai ‘, Jane Capria Downey ’ and Anthony T. Cacace ’
’ Dkision of Otolatyngology, ’ Section of Pediatric Otolaryngoiogy, Albany Medical College, Alban) NY 12208 (U.S.A.) and -’ Center for the Disabled, Albuny NY 12208 (U.S.A.) (Received (Accepted
Key words: Dysphagia;
Cerebral
1 November 29 November
1991)
1991)
palsy
Abstract
One of the disabilities in patients with cerebral palsy (CP) is dysphagia. To establish the prevalence of dysphagia in a population of children with CP, and to determine if any factors are related to dysphagia, we studied 56 CP patients, 5-21 years, enrolled in a primary school for the disabled. Fifteen patients (27%) had either radiographic or clinical evidence of dysphagia. These 15 patients were compared to the remaining 41 patients without dysphagia. Using data obtained from chart review and interviews with speech pathologists, several factors that contributed to dysphagia were found. These included: bite reflexes, slowness of oral intake, poor trunk control, inability to feed independently, anticonvulsant medication, coughing with meals, choking, and pneumonia. We also noted trends in the following factors: presence of tongue thrusting, presence of drooling, severity of CP, poor head control, severity of mental retardation, seizures, and speech disorders. Factors not related to the presence of dysphagia include: subject age, cause of CP, and type of CP. Early, aggressive work-up and identification in CP patients with the risk factors outlined above can reduce the associated pulmonary complications.
* Presented at the Annual Meeting of the American Hawaii. Correspondence to: P.J. Koltai, Section of Pediatric NY 12208, U.S.A.
Society of Pediatric Otolaryngology,
Otolaryngology,
Albany
Medical
May 10. 1991, College,
Albany.
64
Introduction
Cerebral palsy (CP) is defined as ‘a disorder of movement and posture due to a defect or lesion of the immature brain’ [l]. The lesion, which interferes with central nervous system maturation, is non-progressive. The incidence of cerebral palsy is estimated at 7 in 1000 live births [lo]. The severity varies greatly among subjects. In the mildest case, there are minimal motor weakness and normal cognitive abilities. In these instances, subjects function normally in society without obvious handicaps. In contrast, there is a large population of patients that have central motor handicaps accompanied by speech, hearing, and vision deficits, with or without mental retardation and seizure disorders. One additional disability that has been reported is difficulty feeding. Just as the CP patient may have abnormal posturing of the, head and trunk, and motor disturbances of the face, lips, and tongue, so too there can be less apparent abnormal pharyngeal and laryngeal mobility which can greatly impair the patient’s ability to eat. Although there is an abundance of literature dealing with the management of feeding problems in disabled children [2,4,5,7], there is no documentation in the literature of the frequency of swallowing disorders among children with CP. The goals of this study are to establish the prevalence of dysphagia in a population of children with cerebral palsy, as well as to compare these patients with CP patients with normal swallowing to determine which factors relate to the presence of dysphagia.
Materials
and methods
The subjects in this study comprised 101 schoolchildren in the Kevin G. Langan School at the Cerebral Palsy Center for the Disabled (Albany, NY). Data was obtained by reviewing medical records. The study was approved by the Human Research Review Committee of the CP Center. Patients with genetic, muscle, degenerative, or neuromuscular disorders, spinal diseases/ anomalies, postnatal trauma, or any central nervous system insult occurring after the first year of life, and any patient in whom a diagnosis of cerebral palsy was unclear, were excluded from the study. A staff neurologist at the Center for the Disabled was consulted when there was doubt regarding the neurologic diagnosis. Of the 101 records reviewed, 56 patients, 5-21 years, were identified as having cerebral palsy and form the sample for this study. Medical charts were reviewed for demographic, neurologic, surgical, and feeding data. This was obtained from the initial matriculation assessment, yearly medical and neurologic evaluations, as well as from screening exams performed by physical, occupational, and/or speech therapists. Following the chart review, a computerized database was instituted with available software (Enable/OA, The Software Group, Ballston Lake, NY). The subjects were divided into two groups based on the presence or absence of swallowing disorders. The x2 test was used to compare differences in various parameters between groups. A significance level of P < 0.05was selected a priori.
65
Results Fifteen subjects (27%) had swallowing disorders. Eight of these had abnormalities documented on barium swallow radiographs or videofluoroscopic studies. Of these eight., one had findings limited to the oral stage of swallowing, whereas the remainder had abnormalities of the pharyngeal stage. Five of these subjects aspirated. Seven subjects had clinical evidence of dysphagia without radiographic documentation: Subject 1 had a severe seizure disorder, at times having almost constant seizure activity. During these periods he experienced dysphagia. Subject 2 had recurrent aspiration pneumonia and was noted to have difficulty in swallowing and congestion during meals. Subject 3 appeared to have delayed swallowing and had undergone Nissen fundoplication for severe gastroesophageal (GE) reflux. Subject 4 had severe neck spasms which interfered with swallowing. During meals. he would often hold his breath, cough, and become plethoric. Subject 5 had a history of severe GE reflux for which she had a Nissen fundoplication and gastrostomy. She receives nutrition via gastrostomy as well as orally, but suffers from recurrent pneumonia. Subject 6 had a history of aspiration pneumonia, including one life-threatening episode requiring prolonged hospitalization. Subject 7 had severe, chronic drooling that interferes with swallowing. This group of I5 patients was compared to the remaining 41 patients with normal swallowing. The age of the patients ranged from 5 to 21 years, with a median age of 14 years. The children with dysphagia were fairly equally distributed over all ages (Fig. 1). Table I summarizes the parameters studied. Although the percentage of dysphagic patients with tongue thrusting and bitt reflexes during meals was higher than in the normal patients, only the difference in bite reflex was statistically significant. The increase in severity of drooling in dysphagic patients approached statistical significance. The rate of oral intake was significantly lower in the dysphagic group.
m
Normal
ezzd Oysphagia
10
I5
Age Fig. 1. Age distribution
of subjects. No significant
(years)
difference
was noted.
between
the normal
and dysphagic group
66
TABLE I Parameters studied. P-ualue indicates difference between normal and dysphagia group Number of patients Normal
Dysphagia
Severity of speech disorder Mild Moderate-severe Non-verbal
26
1 14
Tongue thrust No Yes
31 10
7 5
Bite reflex No Yes
36 5
5 7
Severity of drooling None Mild Moderate Severe
10 18 7 1
Rate of oral intake Normal Moderately slow Very slow
18 15 1
2 1 8
History of seizures No Yes
15 26
1 14
P < 0.10
Taking anti-convulsants No Yes
26 14
3 12
P < 0.01
Ability to feed self Independent Assistance Dependent
20 3 17
0
P < 0.005
2 12
Head control Good Fair Poor
18 10 13
2 4 9
Trunk control Good Fair Poor
13 13 15
0
Coughing with meals Never Rarely Occasionally Frequently
30 1 7 1
7 8
0
P < 0.10
P < 0.005
P < 0.10
P < 0.005
P < 0.05
5 10 0
2 6 7
P < 0.005
TABLE
I (continued)
Number
ofpatients
Normal
Dysphugirr
Choking with meals (2 + episodes in 3 months) No
40
3
Ye\
0
12
Episodes of pneumonia None
34
7
I-2
4
4
3 or more
3
4
P < 0.05
Diet Regular
or chopped
Modified
39
2
2
12
Several neurologic factors related to dysphagia. There was a statistically significant difference between groups in trunk control, ability to feed independently, and whether or not the subject received anticonvulsant medications. Other consistent but non-significant trends included more severe CP, poorer head control, lower cognitive function, more frequent seizures, and poorer speech proficiency in the dysphagia group. Factors predisposing to cerebral palsy were not associated with the presence or absence of dysphagia, nor was the type of cerebral palsy. The primary neurologic factors are summarized in Figs. 2-5. A potential significant problem is that of aspiration. There was a significant association between the presence of dysphagia and frequency of coughing with meals as well as a history of two or more choking episodes in 3 months. Overall, a large percentage (27%) of the children studied had a history of at least one episode of pneumonia. Dysphagic patients had significantly more frequent episodes.
30 v)
;
25
.-a, :
20
+0
15
k
10
f 2
m E?J
Normal
Dyaphagia
5 0 Mild
Moderate
Severity Fig. ‘7. Severity
of CP, defined
as mild for ambulatory
Severe
of CP subjects, moderate
with assistance, and severe for non-ambulatory
for subjects who ambulate
subjects.
68 10 v)
9
2 W c
a
I? Lc 0 L W 2 2
m Normal ezzd Dysphagia
7 6 5 4 : 1 0 Mild
None
Degree
of
Fig. 3.
Moderate
Mental
Cognitive
Severe
Profound
Retardation
function.
m m
Sp Puad
Sp Di
Type
Mixed
Normal Oytphogia
Other
of CP
Fig. 4. Cause of CP. A, unknown; B, perinatal asphyxia; C, prematurity; D. perinatal infection; E, postnatal infection; F, stroke/hemorrhage; G, other.
Normal ezzd Dyaphogio
25 r
m
A
B
C Cause
D
E
F
G
of CP
Fig. 5. Type of CP. Sp Quad, spastic quadraparesis; Sp Di, spastic diplegia.
TABLE
II
Relwant surgical history Procedure
Number of putients
Gastrostomy T&A Tracheotomy Salivary dive] sion Nissen fundoplication Uvulectomy Tonsillectomy
4 2 2 2 2
(7%:) (4%‘) (4%) (4%) (4%;) I (2%) 1 (2%)
Forty-two of the 56 patients (75%) in the study ate a regular or chopped diet; 39 of 41 patients (95%) in the normal group, and only 3 of 15 patients (20%) in the dysphagia group. Relevant surgical histories of the 56 patients studied are shown in Table II. The number of patients undergoing any single procedure was too small to allow statistical analysis. Only a few patients in the study had weight above the 5th percentile on standard age vs weight growth charts. This data did not appear to be related to the presence of dysphagia.
Discussion
Dysphagia may be defined as difficulty with any of the four .phases of swallowing, i.e. oral preparation, oral stage, pharyngeal stage, or esophageal stage. There is ample literature describing abnormal oromotor findings in patients with CP (e.g. abnormal tongue thrust, increased or decreased gag reflexes, oral hypersensitivity, bite reflexes, and lingual and labial dysfunction) [2,6,8]. Most of the children we studied have CP with some degree of orofacial involvement and thus have abnormalities in the oral preparatory or oral stages of swallowing. Although difficulty with these stages, particularly when there is loss of tongue control, may result in aspiration, most of these disruptions result only in the inability to eat a regular diet or loss of the pleasure of eating for the patient and feeder. Based on standard growth charts, the low percentile weights of nearly all patients in the study appear to be related more to the degree of neurologic involvement and consequent muscle-wasting than to true malnutrition. Biochemical measurements such as serum albumin, total lymphocyte count, transferrin, and nitrogen balance, as well as anthropometric measurements such as triceps skin-fold thickness or bone age, would be more accurate in assessing the nutritional state of a patient with CP, While an increase in tongue thrusting, bite reflex, and slowness of oral intake (all of which indicate oromotor involvement) were apparent, it is the difficulty with pharyngeal swallowing that is more dangerous to the patient since it may result in aspiration.
The possible areas of neurologic damage resulting in abnormal pharyngeal swallowing in cerebral palsy patients are numerous. Although in most cases the cause of cerebral palsy is unknown, many of these children have ischemic brain damage secondary to ischemia at birth. The site of damage depends on the maturity of the brain at the time of birth. Periventricular white matter infarction, including involvement of the corticofugal pyramidal tracts and oral-facial fibers involved in sucking and mastication, is characteristically seen in the pre-term brain [3]. Children suffering birth asphyxia at full term more commonly have cerebral edema, cortical necrosis, cortical-white matter ‘boundary zone’ infarction, and necrosis of thalamic and brainstem nuclei [9]. The association between severity of mental retardation and dysphagia seen in this study is consistent with the fact that much of eating is learned behavior. In addition, the automaticity of collecting and propelling the bolus into the pharynx to initiate normal reflex swallowing exists due to familiarity. Children with severe cognitive disabilities may interpret the presentation of food as an unpleasant stimulus causing choking and thus be unable to make an effective cortical decision to properly propel the bolus along the dorsum of the tongue. Normal speech requires both adequate cognitive function and intact oromotor skills. The association we found between dysphagia and poor speech production skills suggests a relationship between these variables. A large proportion of the children in this study had some degree of drooling (78%). For most of these children, drooling represents a social or hygiene problem for the patient or caregiver. It also is a visible consequence of the loss of oromotor control. Two patients in this series had surgical treatment for their drooling. In this study, 8 of 15 dysphagic children had at least one episode of pneumonia. While we cannot directly correlate episodic aspiration and pneumonia, the frequency of coughing, choking and pneumonia indicates that dysphagic children with CP suffer from a high incidence of respiratory complications. Our results clearly demonstrate that children with CP who have dysphagia can be divided into two groups. The first group comprises those children with less severe involvement who have only oromotor dysphagia. These children present significant feeding problems, primarily due to slow eating which can be extremely labor-intensive, thus present a great financial responsibility. However, these children generally do not have a health risk associated with eating; as such they have a social and therapeutic, rather than a medical, disability. The second group includes those children who have pharyngeal dysphagia demonstrable on pharyngoesophagram. This is manifest clinically as frequent coughing, episodic aspiration, and occasional pneumonia. These children are at medical risk with routine feeding even when done by capable therapists despite the fact that most have been in intensive swallow therapy programs. It is these patients who pose the greatest problems in management and raise important issues about the liability faced by therapists doing the feeding, the desirability to intervene surgically with gastrostomy, the compound financial, burden of expensive feeding programs, and the medical consequences of chronic aspiration It is our goal with studies such as this one, as well as ongoing investigations with this population, to
71
better define the criteria between dysphagia.
the medical and surgical therapy
of severe
Acknowledgement
We would like to thank the staff of the Center assistance in this study.
for the Disabled for their
References I Bax. M.C.O.. Terminology and classification of cerebral palsy. Developmental Medicine and Child Neurology. 1064; 6: 295-297. 2 Helfrich-Miller, K.R., et al., Dysphagia: its treatment in the profoundly retarded patient with cerebral palsy. Arch. Phys. Med. Rehabil., lY86; 67: 520-525. 3 Iriki A., et al.. Feeding behavior in mammals: corticobulbar projection is reorganized during conversion from sucking to chewing. Dev. Brain Res., 1988; 44: 189-196. 4 Jones. P.M., Feeding disorders in children with multiple handicaps. Dev. Med. and Child Neuroogy. 1989: 31: 404-406. 5 Logemann. J.A.. Evaluation and Treatment of’ Swallowing Disorders. San Diego, CA: f‘ollegc-Iii11 Press: 1983. 6 Love, R.J.. Speech performance, dysphagia and oral reflexes in cerebral palsy. J. Speech Hear Dis., 1980; 45: 59-7s. 7 Morris. SE., Program Guidelines for Children with Feeding Problems. Edison. NJ: Childcraft Education Corporation; 1977. 8 Mueller, fl., Feeding. In: Groher, M.E., ed. Handling the Young Cerebral Palsied Child at Home. New York. NY: Dutton; 197.5: 113-132. Y Pape, K.E.. Wigglesworth, J.S.. Haemorrhage, Ischaemia and the Perinatal Brain. Philadelphia, PA: JB Lippincott Co; 1979: 196. IO Thompson. G.H., Rubin, I.L., Bilenker. Comprehensive Management of Cerebral Palsy. Grune & Stratton: 1983: 19.
