ISSUES I N CLINICAL NEUROSCIENCE
The Persistent Vegetative State in Chddren: . Report of The Chdd Neurology bociety Mucs Commmee n
Stephen Ashwal, MD, James F. Bale, Jr, MD, David L. Coulter, MD, Robert Eiben, MD, Bhuwan P. Garg, MD, Alan Hill, MD, Edwin C. Myer, MD, &chard E. Nordgren, MD, D. Alan Shewmon, MD, Theodore R. Sunder, MD, Russell W. Walker, MD (The CNS Ethics Committee)
Increasing concern about children in a persistent vegetative state (PVS) prompted a survey of members of the Child Neurology Society regarding aspects of the diagnosis and management of this disorder. Major findings of those responding to this survey (26% response rate) were as follows: (1) 93% believed that a diagnosis of PVS can be made in children, but only 16% believed that this applied to infants younger than 2 months and 70% in the 2-month to 2-year group; (2) a period of 3 to 6 months was believed to be the minimum observation period required before a diagnosis of PVS could be made; ( 3 )86% believed that the age of the patient would affect the duration of time needed to make the diagnosis of PVS; (4)78% thought a diagnosis of PVS could be made in children with severe congenital brain malformations; ( 5 ) 75% believed that neurodiagnostic studies would be of value and supportive of the clinical diagnosis of PVS; (6) members’ opinions as to the average life expectancy (in years) for the following age groups after the patients were considered vegetative were: newborn to 2 months, 4.1; 2 months to 2 years, 5.5; 2 to 7 years, 7.3; and more than 7 years, 7.4; ( 7 ) 20% believed that infants and children in a PVS experience pain and suffering; and (8) 75% “never” withhold fluid and nutrition from infants and children in a PVS and 28% “always” give medication for pain and suffering. The data suggest that child neurologists believe PVS can be diagnosed in children after 2 years of age, but that in younger children a more cautious approach is indicated. The findings suggest reasonable agreement concerning many of these issues, and it may be possible at this time to develop a position statement on the medical aspects of PVS in children. Ashwal S, Bale JF Jr, Coulter DL, Eiben R, Garg BP, Hill A, Myer EC, Nordgren RE, Shewmon DA, Sunder TR, Walker RW (the CNS Ethics Committee). The persistent vegetative state in children: report of The Child Neurology Society Ethics Committee. Ann Neurol 1992;32:570-576
Since the original description of the persistent vegetative state (PVS) by Jennett and Plum [l] in 1972, increasing attention has been focused on this condition in adults but only more recently in children [2-10). The PVS has been defined as a state of “wakefulness without awareness” o r “permanent unconsciousness.” Criteria necessary for the definition of this condition in adults have, in general, been well accepted by clinically determining the absence of neocortical function El 1). However, it is evident that these criteria cannot be applied directly to infants and children, especially during the newborn period. In addition, the prognosis and management of these children has not previously been examined. The objective of this study was to obtain the opinions of child neurologists concerning the persistent vegetative state in infants and children by means of an extensive questionnaire mailed to the members of the Child Neurology Society (CNS).
Methods In March 1991, 960 questionnaires were mailed to 775 active, 155 junior, and 30 emeritus and affiliate members of the Child Neurology Society with a second mailing in June. The questionnaire contained 32 items that surveyed opinions from the membership of the CNS regarding the clinical features, neurodiagnosticevaluation, and management of infants and children with PVS. In addition, basic data regarding the experience and practice characteristics(e.g., academic versus private practice) of the respondents were obtained. Completed questionnaires were collated by members of the Ethics Committee, entered into a personal computer, and descriptive statistics were geneiated. Data are expressed as ranges or means ? 1 SD.
Received Dec 24,1991, and in revised form Mar 10,1992. Accepted for publication Mar 10, 1992.
Address correspondence to Dr Ashwal, Department of Pediatrics, Loma k n d a University School of Medicine, Lorna Lnda, CA 92350.
570
Results Of the 960 questionnaires mailed, 250 were completed and returned by 227 Active and 23 Junior members by September, 1991. All 250 returned surveys were
Copyright (9 1992 by the American Neurological Association
used in the calculation of the results that have been tabulated. Sixty-three percent of active members had primary academic appointments and 35% were engaged in private practice. Junior members were either in training or had recently completed a fellowship. For the active members, the number of years after training approximated less than 5 years (19%), 5 to 10 years (28%), 10 to 20 years (35%), and more than 20 years (19%). The percentage of time that members devoted to the clinical care of children with neurological disorders was as follows: 0 to 25% (5%), 25 to 50% (16%), 50 to 75% (39%), and 75 to 100% (40%). Diagnosis of PVS in Infants and Children Most respondents (93%) agreed with the following statement: PVS in infants and children can be diagnosed based on the appropriate clinical neurological examination for age, knowledge of the insult causing brain injury, and an appropriate period of observation depending on the age of the patient and nature of the insult.
There was a clear-cut difference for different age groups. Only 16% of members believed the diagnosis of PVS could be made in newborns up to 2 months of age and 70% agreed that diagnosis was feasible in infants 2 months to 2 years of age. In children older than 2 years, the overwhelming majority of child neurologists (92-94%) believed that a diagnosis of PVS could be reliably made. Definition of PVS In adults with PVS, 10 clinical characteristics have been commonly used and generally accepted as constituting an operational definition of PVS 1111. Child neurologists’ opinions as to which of these criteria would “apply,” be “supportive,” or be “necessary,” are presented in Table 1. Seven of these criteria were considered to apply to infants and children by more than
80% of members. Six of these seven criteria were deemed necessary by a significant majority of members ( 6 7 4 4 % ) . These criteria primarily reflected assessment of cortical functions associated with wakefulness, cognition, language, and behavior. The seventh criteria (“spontaneous eye movements but no sustained tracking”) was deemed necessary by only 53% of members. The remaining three criteria (nos. 8-10), which reflect brainstem, autonomic, or vegetative functions, were considered applicable (53-75%) or necessary (35-5396) to a lesser degree. The data suggest that child neurologists defined PVS in infants and children in terms of the persistent loss of higher cortical functions rather than the persistence of vegetative functions.
Neurodiagnostic Testing Few respondents considered making the diagnosis of PVS based only on clinical criteria in “all” (5%) or a “majority” (20%) of patients. Thus, of the 75% who recommended neurodiagnostic testing, 1596 recommended testing in “some,” 31% in a majority, and 29% in all children. Whether such testing was considered “useful” or “mandatory” depended on the type of study as outlined in Table 2. Interestingly, as many as 10% of respondents felt that demonstration of electrocerebral silence by electroencephalography was mandatory before making a diagnosis of PVS. Electroencephaiographic (EEG), computed tomographic (CT)/ magnetic resonance imaging (MRI), and cerebral blood flow (CBF) studies were thought to be more useful than evoked-response data. Duration of Observation: Dependence on Nature of Insult and Age of Patient According to most respondents (84%), the minimum observation period required to make a diagnosis of PVS would vary, depending on the nature of the insult.
Table I. D&nition of PVS in Infants and Childven
1. Wakefulness without awareness
2. 3. 4. 5. 6. 7. 8. 9.
Eyes-open unconsciousness No “voluntary” action or behavior No “cognitive” response No “voluntary” language Inability to follow commands Spontaneous eye movements but no sustained tracking Intact brainstem reflexes and sleep-wake cycles Spontaneous breathing, but chewing and swallowing impaired 10. Bowel and bladder incontinence
Apply (%)
Supportive (5%)
Necessary
95 94 91 90 84 83 83 75 75 53
16 33 23 22 29 22 47 53 65 53
84 67 77 78 71 78 53 47 35 47
(96)
Data given as percentage of respondents (n = 250). PVS = persistent vegetative state.
Issues in Clinical Neuroscience: Ashwal et al: Persistent Vegetative State
571
Table 2. Value of Neurodiagnostic Testing for PVS Diagnosis in Infants and Children ~~
~
~~
~~~
~
~~~
Test
Useful (%)
Mandatory (%)
Isoelectric EEG in the presence of brainstem function CTJMRI scan documenting extensive or progressive atrophy of the cerebral cortex Absent CBF in the presence of brainstem function SSEP showing no response BAER showing no response VER showing no response PET scan showing cortical metabolic activity lower than 40 to 50%
67 64
10 17
64 53 46 43 42
Data presented as percentage of respondents (n == 250) who believed that a specific test was useful or mandatory. PVS = persistent vegetative stare; EEG = electroencephalogram; CT = computed tomography; MRI = magnetic resonance imaging; CBF = cerebral blood flow; SSEP = somatosensory-evoked response; BAER = brainstem auditory-evoked response; VER = visual-evoked response; PET = positron emission tomography.
Table 3. Minimum Observation Periodjor Diagnosis of PVS in Infants and Children Insult Head trauma Bacterial meningitis Encephalitis Cardiorespiratory arrest Birth asphyxia
SIDS Near drowning Strangulation Carbon monoxide Hemorrhagic shock encephalopathy syndrome Age of child Newborn to 2 months 2 months to 2 years 2 to 7 years >7 years
Observation Period (mo; mean k 1 SD)
Range (mo)
(0.9) (0.6) (1.4) (1.1) (0.8) (1.0) (1.2) (1.2)
0.5-24 0.5-24 0.5-24 0.5-24 0.25-48 0.5-48 0.5-24 0.25-24 0.25-36 0.5-24
5.7 (1.6)
0.25-24
4.6 (1.4) 3.8 (1.5) 3.1 (0.5)
0.25-24 0.25-24 0.25-24
5.9 4.1 4.1 3.3 4.4 3.5 3.4 3.2 3.7 3.4
(0.9) (1.2)
PVS = persistent vegetative stare; SIDS = sudden infant death syndrome.
As outlined in Table 3, this ranged between 3 and 6 months for most types of injuries. In addition, 86% believed that a longer observation period was indicated for younger patients before the vegetative state could be considered irreversible. Many (78%) believed that PVS could be diagnosed in infants and children with congenital brain malformations. Those who disagreed only considered the diagnosis of PVS after acquired brain insults. However, congenital malformations appear to be an uncommon cause of PVS compared with acquired nervous system insults (Table 4). Among the malformations that members stated were associated with a diagnosis of PVS were hydranencephaly, anen572 Annals of Neurology
Vol 32 No 4
October 1992
cephaly, holoprosencephaly, lissencephaly, migrational disorders, schizencephaly, severe hydrocephalus, encephaloceles, and atelencephaly. Fifty-nine percent of members would not make a diagnosis of PVS in infants or children who have an absent cerebral cortex, determined by MRI or CT scan, if these infants show additional behavioral responses that are inconsistent with being vegetative. Preualence and Life Expectancy Table 5 outlines average estimates as to the number of infants and children seen or diagnosed per year with PVS by child neurologists and their opinions, based on their experience, of the average life expectancy in the four different age groups. Although most believed survival would fall between 4 and 7 years, several respondents (less than 10%)indicated that they had occasionally observed much longer life expectancies, in the range of 5 to 20 years. American Academy of Nezrrology Position Paper on PVS In 1989, the American Academy of Neurology (AAN) published a position paper and commentary on certain aspects of the care and management of the PVS patient C3, 41. This position statement defined the PVS, expressed the opinion that artificial nutrition and hydration should be considered forms of medical treatment, clarifed the ethical positions that patients or their surrogates should make personal decisions concerning treatment, and stated that there are no medicallethical distinctions between withholding and withdrawing treatment. There was no discussion in this position statement or the commentary that referred specifically to PVS in children. In reference to this AAN position paper, 92% of CNS members agreed with its content as it related to adults, but only 72% believed this statement was applicable to infants and children.
Table 4. Causes of PVS in Infants and Children Insult
Newborn to 2 Months
2 Months to 2 Years
2 to 7 Years
>7 Years
Asphyxia Trauma Infection Near drowning Malformations
116 22
86 111 117
73 145 90
0 48 18 26
69
96
0 14 39
0 0 47
62 124 68 29 0 0 25
337 402 34 1 194 48 32 137
296
436
45 1
308
1,491
SIDS Other
Total
66
Total
Data indicate the number of patients seen in each category per year, based on 250 respondentsto questionnaire.The category “Other” includes metabolic and degenerative disorders, tumors, etc. PVS = persistent vegetative state; SIDS = sudden infant death syndrome.
Table 5 . Prevalence and L$e Expectancy of Infants and Children with PVS Predicted Life Expectancy (Yr)
* 1 SD
Ranse
Age
Prevalencea
Mean
Newborn to 2 months 2 months to 2 years 2 to 7 years >7 years
263 460 475 465 1,663
4.1 (0.7) 5.5 (0.9) 7.3 (1.7) 7.4 (1.8)
0.5-20 0.5-20 0.5-20 0.5-20
...
...
Total
‘Total reported for that age group per year by all 250 questionnaire respondents. PVS = persistent vegetative state.
Treatment Practices Child neurologists stated that of the infants and children they had seen in the past 2 years, their role was that of consultant (68%) or primary care provider (32%). They believed they were the “primary decision maker” in regard to issues of withholding or withdrawing treatment 44% of the time. Many provided acute (55%) and long-term (37%) counseling to families. Based on their experience, 80% of child neurologists did not (but 20% did) believe that infants and children with PVS experience pain and suffering. Their opinions as to recommendations for treatment are outlined in Table 6. Almost all child neurologists (99%)recommended supportive care, such as fluids, nutrition, and “routine” use of antibiotics, and the implementation of a “do not resuscitate” order in the hospital. Most (755%) stated they “never” recommend the withholding of fluids and nutrition. Also, 75% indicated that they recommend the use of medications to alleviate pain and suffering as follows: “always” (28%), “frequently” (14%), and “sometimes” (33%). Limitations of Study The Ethics Committee had to assess whether the data were a reasonable reflection of the opinions of the
membership of the CNS. The return rate (26%) was considered acceptable and similar to previous questionnaires mailed to the membership and in line with other mailings, such as balloting for officers of the Society. The data were also presented to the membership at the annual Society meeting for open discussion. It is the Committee’s impression that these results do reflect the member’s experience and opinions as to their practices or beliefs concerning the issue of PVS in infants and children. In addition, the manuscript reporting the data of this study was reviewed and approved by the Executive Committee of the CNS. A second concern related to the range of responses for certain questions such as time estimates of life expectancy or observation periods required for diagnosis of PVS. From Tables 3 and 5, it appears that the variances of the data (i.e., standard deviations) are relatively “tight” in both groups, reflecting a reasonable consensus opinion. However, approximately 5 to 10% of members did express much longer estimates of survival, such as 10 to 20 years or even longer (see Table 5). We believe this is an accurate indication of “real life” situations in which some patients, especially with modern technology and care, have the potential for prolonged survival.
Issues in Clinical Neuroscience: Ashwal et al: Persistent Vegetative State
573
Table 6. Treatment Practices in Infants and Children with PVS ~~~
~~
Supportive care (i.e., fluids, nutrition, routine antibiotics, excluding gastrostomy) supportive care including gastrostomy Supportive care, gastrostomy, tracheostomy DNR order in hospital DNR idout home and hospital Withholding of fluids Withholding of fluid and nutrition Medications to alleviate pain and suffering
AlwaysiFrequently (%)
Sometimes (%)
75
24
61 32 76 77 6 3 42
35 54 23 18 20 22 32
Never (%) 1
4 14
1 5 74 75
26
Data given as percentage of respondents (n = 250). PVS = persistent vegetative state; DNR = do not resuscitate.
Discussion It is clear that the majority of child neurologists who responded to this questionnaire believe the diagnosis of PVS can be made in children after 2 years of age, but most are reluctant to consider this diagnosis in infants and children less than 2 months of age. Immaturity of the nervous system (44%), uncertainty of the and to a lesser extent, uncertainty examination (40
The Persistent Vegetative State in Chddren: . Report of The Chdd Neurology bociety Mucs Commmee n
Stephen Ashwal, MD, James F. Bale, Jr, MD, David L. Coulter, MD, Robert Eiben, MD, Bhuwan P. Garg, MD, Alan Hill, MD, Edwin C. Myer, MD, &chard E. Nordgren, MD, D. Alan Shewmon, MD, Theodore R. Sunder, MD, Russell W. Walker, MD (The CNS Ethics Committee)
Increasing concern about children in a persistent vegetative state (PVS) prompted a survey of members of the Child Neurology Society regarding aspects of the diagnosis and management of this disorder. Major findings of those responding to this survey (26% response rate) were as follows: (1) 93% believed that a diagnosis of PVS can be made in children, but only 16% believed that this applied to infants younger than 2 months and 70% in the 2-month to 2-year group; (2) a period of 3 to 6 months was believed to be the minimum observation period required before a diagnosis of PVS could be made; ( 3 )86% believed that the age of the patient would affect the duration of time needed to make the diagnosis of PVS; (4)78% thought a diagnosis of PVS could be made in children with severe congenital brain malformations; ( 5 ) 75% believed that neurodiagnostic studies would be of value and supportive of the clinical diagnosis of PVS; (6) members’ opinions as to the average life expectancy (in years) for the following age groups after the patients were considered vegetative were: newborn to 2 months, 4.1; 2 months to 2 years, 5.5; 2 to 7 years, 7.3; and more than 7 years, 7.4; ( 7 ) 20% believed that infants and children in a PVS experience pain and suffering; and (8) 75% “never” withhold fluid and nutrition from infants and children in a PVS and 28% “always” give medication for pain and suffering. The data suggest that child neurologists believe PVS can be diagnosed in children after 2 years of age, but that in younger children a more cautious approach is indicated. The findings suggest reasonable agreement concerning many of these issues, and it may be possible at this time to develop a position statement on the medical aspects of PVS in children. Ashwal S, Bale JF Jr, Coulter DL, Eiben R, Garg BP, Hill A, Myer EC, Nordgren RE, Shewmon DA, Sunder TR, Walker RW (the CNS Ethics Committee). The persistent vegetative state in children: report of The Child Neurology Society Ethics Committee. Ann Neurol 1992;32:570-576
Since the original description of the persistent vegetative state (PVS) by Jennett and Plum [l] in 1972, increasing attention has been focused on this condition in adults but only more recently in children [2-10). The PVS has been defined as a state of “wakefulness without awareness” o r “permanent unconsciousness.” Criteria necessary for the definition of this condition in adults have, in general, been well accepted by clinically determining the absence of neocortical function El 1). However, it is evident that these criteria cannot be applied directly to infants and children, especially during the newborn period. In addition, the prognosis and management of these children has not previously been examined. The objective of this study was to obtain the opinions of child neurologists concerning the persistent vegetative state in infants and children by means of an extensive questionnaire mailed to the members of the Child Neurology Society (CNS).
Methods In March 1991, 960 questionnaires were mailed to 775 active, 155 junior, and 30 emeritus and affiliate members of the Child Neurology Society with a second mailing in June. The questionnaire contained 32 items that surveyed opinions from the membership of the CNS regarding the clinical features, neurodiagnosticevaluation, and management of infants and children with PVS. In addition, basic data regarding the experience and practice characteristics(e.g., academic versus private practice) of the respondents were obtained. Completed questionnaires were collated by members of the Ethics Committee, entered into a personal computer, and descriptive statistics were geneiated. Data are expressed as ranges or means ? 1 SD.
Received Dec 24,1991, and in revised form Mar 10,1992. Accepted for publication Mar 10, 1992.
Address correspondence to Dr Ashwal, Department of Pediatrics, Loma k n d a University School of Medicine, Lorna Lnda, CA 92350.
570
Results Of the 960 questionnaires mailed, 250 were completed and returned by 227 Active and 23 Junior members by September, 1991. All 250 returned surveys were
Copyright (9 1992 by the American Neurological Association
used in the calculation of the results that have been tabulated. Sixty-three percent of active members had primary academic appointments and 35% were engaged in private practice. Junior members were either in training or had recently completed a fellowship. For the active members, the number of years after training approximated less than 5 years (19%), 5 to 10 years (28%), 10 to 20 years (35%), and more than 20 years (19%). The percentage of time that members devoted to the clinical care of children with neurological disorders was as follows: 0 to 25% (5%), 25 to 50% (16%), 50 to 75% (39%), and 75 to 100% (40%). Diagnosis of PVS in Infants and Children Most respondents (93%) agreed with the following statement: PVS in infants and children can be diagnosed based on the appropriate clinical neurological examination for age, knowledge of the insult causing brain injury, and an appropriate period of observation depending on the age of the patient and nature of the insult.
There was a clear-cut difference for different age groups. Only 16% of members believed the diagnosis of PVS could be made in newborns up to 2 months of age and 70% agreed that diagnosis was feasible in infants 2 months to 2 years of age. In children older than 2 years, the overwhelming majority of child neurologists (92-94%) believed that a diagnosis of PVS could be reliably made. Definition of PVS In adults with PVS, 10 clinical characteristics have been commonly used and generally accepted as constituting an operational definition of PVS 1111. Child neurologists’ opinions as to which of these criteria would “apply,” be “supportive,” or be “necessary,” are presented in Table 1. Seven of these criteria were considered to apply to infants and children by more than
80% of members. Six of these seven criteria were deemed necessary by a significant majority of members ( 6 7 4 4 % ) . These criteria primarily reflected assessment of cortical functions associated with wakefulness, cognition, language, and behavior. The seventh criteria (“spontaneous eye movements but no sustained tracking”) was deemed necessary by only 53% of members. The remaining three criteria (nos. 8-10), which reflect brainstem, autonomic, or vegetative functions, were considered applicable (53-75%) or necessary (35-5396) to a lesser degree. The data suggest that child neurologists defined PVS in infants and children in terms of the persistent loss of higher cortical functions rather than the persistence of vegetative functions.
Neurodiagnostic Testing Few respondents considered making the diagnosis of PVS based only on clinical criteria in “all” (5%) or a “majority” (20%) of patients. Thus, of the 75% who recommended neurodiagnostic testing, 1596 recommended testing in “some,” 31% in a majority, and 29% in all children. Whether such testing was considered “useful” or “mandatory” depended on the type of study as outlined in Table 2. Interestingly, as many as 10% of respondents felt that demonstration of electrocerebral silence by electroencephalography was mandatory before making a diagnosis of PVS. Electroencephaiographic (EEG), computed tomographic (CT)/ magnetic resonance imaging (MRI), and cerebral blood flow (CBF) studies were thought to be more useful than evoked-response data. Duration of Observation: Dependence on Nature of Insult and Age of Patient According to most respondents (84%), the minimum observation period required to make a diagnosis of PVS would vary, depending on the nature of the insult.
Table I. D&nition of PVS in Infants and Childven
1. Wakefulness without awareness
2. 3. 4. 5. 6. 7. 8. 9.
Eyes-open unconsciousness No “voluntary” action or behavior No “cognitive” response No “voluntary” language Inability to follow commands Spontaneous eye movements but no sustained tracking Intact brainstem reflexes and sleep-wake cycles Spontaneous breathing, but chewing and swallowing impaired 10. Bowel and bladder incontinence
Apply (%)
Supportive (5%)
Necessary
95 94 91 90 84 83 83 75 75 53
16 33 23 22 29 22 47 53 65 53
84 67 77 78 71 78 53 47 35 47
(96)
Data given as percentage of respondents (n = 250). PVS = persistent vegetative state.
Issues in Clinical Neuroscience: Ashwal et al: Persistent Vegetative State
571
Table 2. Value of Neurodiagnostic Testing for PVS Diagnosis in Infants and Children ~~
~
~~
~~~
~
~~~
Test
Useful (%)
Mandatory (%)
Isoelectric EEG in the presence of brainstem function CTJMRI scan documenting extensive or progressive atrophy of the cerebral cortex Absent CBF in the presence of brainstem function SSEP showing no response BAER showing no response VER showing no response PET scan showing cortical metabolic activity lower than 40 to 50%
67 64
10 17
64 53 46 43 42
Data presented as percentage of respondents (n == 250) who believed that a specific test was useful or mandatory. PVS = persistent vegetative stare; EEG = electroencephalogram; CT = computed tomography; MRI = magnetic resonance imaging; CBF = cerebral blood flow; SSEP = somatosensory-evoked response; BAER = brainstem auditory-evoked response; VER = visual-evoked response; PET = positron emission tomography.
Table 3. Minimum Observation Periodjor Diagnosis of PVS in Infants and Children Insult Head trauma Bacterial meningitis Encephalitis Cardiorespiratory arrest Birth asphyxia
SIDS Near drowning Strangulation Carbon monoxide Hemorrhagic shock encephalopathy syndrome Age of child Newborn to 2 months 2 months to 2 years 2 to 7 years >7 years
Observation Period (mo; mean k 1 SD)
Range (mo)
(0.9) (0.6) (1.4) (1.1) (0.8) (1.0) (1.2) (1.2)
0.5-24 0.5-24 0.5-24 0.5-24 0.25-48 0.5-48 0.5-24 0.25-24 0.25-36 0.5-24
5.7 (1.6)
0.25-24
4.6 (1.4) 3.8 (1.5) 3.1 (0.5)
0.25-24 0.25-24 0.25-24
5.9 4.1 4.1 3.3 4.4 3.5 3.4 3.2 3.7 3.4
(0.9) (1.2)
PVS = persistent vegetative stare; SIDS = sudden infant death syndrome.
As outlined in Table 3, this ranged between 3 and 6 months for most types of injuries. In addition, 86% believed that a longer observation period was indicated for younger patients before the vegetative state could be considered irreversible. Many (78%) believed that PVS could be diagnosed in infants and children with congenital brain malformations. Those who disagreed only considered the diagnosis of PVS after acquired brain insults. However, congenital malformations appear to be an uncommon cause of PVS compared with acquired nervous system insults (Table 4). Among the malformations that members stated were associated with a diagnosis of PVS were hydranencephaly, anen572 Annals of Neurology
Vol 32 No 4
October 1992
cephaly, holoprosencephaly, lissencephaly, migrational disorders, schizencephaly, severe hydrocephalus, encephaloceles, and atelencephaly. Fifty-nine percent of members would not make a diagnosis of PVS in infants or children who have an absent cerebral cortex, determined by MRI or CT scan, if these infants show additional behavioral responses that are inconsistent with being vegetative. Preualence and Life Expectancy Table 5 outlines average estimates as to the number of infants and children seen or diagnosed per year with PVS by child neurologists and their opinions, based on their experience, of the average life expectancy in the four different age groups. Although most believed survival would fall between 4 and 7 years, several respondents (less than 10%)indicated that they had occasionally observed much longer life expectancies, in the range of 5 to 20 years. American Academy of Nezrrology Position Paper on PVS In 1989, the American Academy of Neurology (AAN) published a position paper and commentary on certain aspects of the care and management of the PVS patient C3, 41. This position statement defined the PVS, expressed the opinion that artificial nutrition and hydration should be considered forms of medical treatment, clarifed the ethical positions that patients or their surrogates should make personal decisions concerning treatment, and stated that there are no medicallethical distinctions between withholding and withdrawing treatment. There was no discussion in this position statement or the commentary that referred specifically to PVS in children. In reference to this AAN position paper, 92% of CNS members agreed with its content as it related to adults, but only 72% believed this statement was applicable to infants and children.
Table 4. Causes of PVS in Infants and Children Insult
Newborn to 2 Months
2 Months to 2 Years
2 to 7 Years
>7 Years
Asphyxia Trauma Infection Near drowning Malformations
116 22
86 111 117
73 145 90
0 48 18 26
69
96
0 14 39
0 0 47
62 124 68 29 0 0 25
337 402 34 1 194 48 32 137
296
436
45 1
308
1,491
SIDS Other
Total
66
Total
Data indicate the number of patients seen in each category per year, based on 250 respondentsto questionnaire.The category “Other” includes metabolic and degenerative disorders, tumors, etc. PVS = persistent vegetative state; SIDS = sudden infant death syndrome.
Table 5 . Prevalence and L$e Expectancy of Infants and Children with PVS Predicted Life Expectancy (Yr)
* 1 SD
Ranse
Age
Prevalencea
Mean
Newborn to 2 months 2 months to 2 years 2 to 7 years >7 years
263 460 475 465 1,663
4.1 (0.7) 5.5 (0.9) 7.3 (1.7) 7.4 (1.8)
0.5-20 0.5-20 0.5-20 0.5-20
...
...
Total
‘Total reported for that age group per year by all 250 questionnaire respondents. PVS = persistent vegetative state.
Treatment Practices Child neurologists stated that of the infants and children they had seen in the past 2 years, their role was that of consultant (68%) or primary care provider (32%). They believed they were the “primary decision maker” in regard to issues of withholding or withdrawing treatment 44% of the time. Many provided acute (55%) and long-term (37%) counseling to families. Based on their experience, 80% of child neurologists did not (but 20% did) believe that infants and children with PVS experience pain and suffering. Their opinions as to recommendations for treatment are outlined in Table 6. Almost all child neurologists (99%)recommended supportive care, such as fluids, nutrition, and “routine” use of antibiotics, and the implementation of a “do not resuscitate” order in the hospital. Most (755%) stated they “never” recommend the withholding of fluids and nutrition. Also, 75% indicated that they recommend the use of medications to alleviate pain and suffering as follows: “always” (28%), “frequently” (14%), and “sometimes” (33%). Limitations of Study The Ethics Committee had to assess whether the data were a reasonable reflection of the opinions of the
membership of the CNS. The return rate (26%) was considered acceptable and similar to previous questionnaires mailed to the membership and in line with other mailings, such as balloting for officers of the Society. The data were also presented to the membership at the annual Society meeting for open discussion. It is the Committee’s impression that these results do reflect the member’s experience and opinions as to their practices or beliefs concerning the issue of PVS in infants and children. In addition, the manuscript reporting the data of this study was reviewed and approved by the Executive Committee of the CNS. A second concern related to the range of responses for certain questions such as time estimates of life expectancy or observation periods required for diagnosis of PVS. From Tables 3 and 5, it appears that the variances of the data (i.e., standard deviations) are relatively “tight” in both groups, reflecting a reasonable consensus opinion. However, approximately 5 to 10% of members did express much longer estimates of survival, such as 10 to 20 years or even longer (see Table 5). We believe this is an accurate indication of “real life” situations in which some patients, especially with modern technology and care, have the potential for prolonged survival.
Issues in Clinical Neuroscience: Ashwal et al: Persistent Vegetative State
573
Table 6. Treatment Practices in Infants and Children with PVS ~~~
~~
Supportive care (i.e., fluids, nutrition, routine antibiotics, excluding gastrostomy) supportive care including gastrostomy Supportive care, gastrostomy, tracheostomy DNR order in hospital DNR idout home and hospital Withholding of fluids Withholding of fluid and nutrition Medications to alleviate pain and suffering
AlwaysiFrequently (%)
Sometimes (%)
75
24
61 32 76 77 6 3 42
35 54 23 18 20 22 32
Never (%) 1
4 14
1 5 74 75
26
Data given as percentage of respondents (n = 250). PVS = persistent vegetative state; DNR = do not resuscitate.
Discussion It is clear that the majority of child neurologists who responded to this questionnaire believe the diagnosis of PVS can be made in children after 2 years of age, but most are reluctant to consider this diagnosis in infants and children less than 2 months of age. Immaturity of the nervous system (44%), uncertainty of the and to a lesser extent, uncertainty examination (40
