Evaluation of Mortality Nonpreventable, By Frans W.J. Hazebroek,
in Surgical Neonates Over a lo-Year Period: Permissible, and Preventable Death
Dick Tibboel,
Karin Leendertse-Verloop,
Albert
P. Bos, Gerard C. Madern,
and Jan C. Molenaar Rotterdam,
The Netherlands
l The mortality pattern and causes of death in surgical neonates were evaluated over the period of 1980 through 1990 in order to assess whether any deaths might have been prevented. In the study period 1,010 neonates were admitted, 104 of whom died (10%). These 104 neonates formed our study population. The Clinical Classification System (CCS) and the Therapeutic Intervention Scoring System (TISS) were used to assess the severity of illness. All 104 nonsurvivors were seriously ill (CCS 3 and 4; TISS > 20). The mortality pattern was classified in three groups. Group A (nonpreventable death) included 58 deaths, 48 of which were due to the underlying disease. The other 8 patients died of a nonpreventable complication of treatment. Eleven of the patients in group B (permissible death) had (multiple) congenital anomalies associated with chromosomal anomalies. Three of them were not treated and in the other 8 treatment was initially started but later withdrawn. In most of the other 14 patients further treatment was withdrawn because of serious postoperative complications or inoperable cardiac anomalies. Group C (preventable death) consisted of 23 patients. Six of 10 had irreversible brain damage due to prolonged hypoxemia. In the other 13 patients of group C, death was due to sepsis acquired in the postoperative period and treated inadequately. Staphylococcus epidermidis was the most frequent isolated pathogen (7/ 13). Copyright o 1991 by W.B. Saunders Company INDEX WORDS: Mortality, genital anomalies.
surgical neonates; mortality,
con-
T
HE PRESENT-DAY quality of perioperative care has a positive effect on morbidity and mortality in neonates with congenital anomalies. Therefore, we take pride in talking and writing about our good treatment results. Nevertheless, a number of these children still die, despite trained intensive care unit (ICU) physicians and nurses and wellequipped ICUs. Analysis of mortality provides an opportunity for developing preventive strategies to improve survival. Would a study of the pattern of mortality in neonates with congenital anomalies show
From the Department of Pediatric Surgery, Medical School of Erasmus University and Sophia Children’s Hospital, Rotterdam, The Netherlands. Presented at the 42nd Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Boston, Massachusetts, October 6- 7, 1990. Address reprint requests to F. W.J. Hazebroek, MD, Department of Pediatric Swgery, Sophia Children’s Hospital, PO Box 70029, 3000 LL Rotterdam, The Netherlands. Copyright o 1991 by WB. Saunders Company 0022-3468/91/2609-0010$03.00l0
1058
that any deaths might have been prevented? In order to evaluate this premise, we retrospectively evaluated the mortality pattern and causes of death in our pediatric surgical ICU (PSICU). MATERIALS
AND METHODS
From January 1980 to January 1990 all neonates who were admitted within 1 month after birth to the PSICLJ of the Sophia Children’s Hospital, Rotterdam, The Netherlands, and who died during hospitalization, were considered for our retrospective evaluation. The different parameters for these patients were assessed, such as duration of gestation, birth weight, sex, primary diagnosis on admission, treatment, and infant’s age at the time of death. All neonates were examined by a consultant in genetics. During a pilot analysis we decided to classify mortality pattern in three groups described as follows: group A: nonpreventable death-patients with serious life-threatening disorders who died despite maximal care; group B: permissible death-patients with (mostly congenital) disorders in whom death is the result of withholding or withdrawal of life-supporting surgical and/or medical intervention: and group C: preventable death-patients who died due to errors in management in which death possibly could have been avoided. Our patients were also evaluated in terms of severity of illness and quantity of care using the Clinical Classification System (CCS) and the Therapeutic Intervention Scoring System (TISS). CCS was assessed according to the definition of Cullen et al’: class I patients were not admitted to the ICU; class II patients were physiologically stable and were admitted to the ICU only for monitoring or observation; class III patients were physiologically stable requiring intensive nursing and monitoring; and class IV patients were unstable needing frequent assessment and interventions by ICU physicians and nurses. The TISS assesses the severity of the patient’s illness by quantitating the therapeutic intervention required for care. Each intervention is graded from 1 to 4 points depending on the time and effort required and the complexity of the procedure.’ CCS score and the maximal numbers of TISS points were scored during the first 24-hour period after admission and during the 24-hour period before death. The data were collected from each patient’s daily ICU flowsheet and medical file. Collection of data and classifying mortality pattern and causes of death were done by a senior registrar (K.L.-V.) not directly involved in the treatment of the patients. RESULTS
In the study period a total of 3,243 children of all ages were admitted to our PSICU, 152 of whom died (4.7%). This group of patients included 1,010 children admitted within 1 month after birth, 104 of whom died (10%). These 104 neonates formed our study population. Sixty-five of the 104 neonates (62.5%) were born prematurely (gestational age < 37 weeks). JournalofPediatric Surgery, Vol26. No 9 (September), 1991: pp 1058-1063
MORTALITY IN SURGICAL NEONATES
1059
Eighty-eight of the 104 patients had single or multiple congenital anomalies. Multiple congenital anomaiies were defined as two or more life-threatening congenital disorders. In 67 of these patients (76%) multiple congenital anomalies, congenital diaphragmatic hernia, and digestive tract anomalies were the primary diagnoses on admission. In the other 16 neonates, necrotizing enterocolitis was the most frequent primary diagnosis. A complete list of the primary diagnoses on admission is given in Table 1. No patients were CCS class I or II. On admission 78 patients were class IV and 26 were class III. On the day of death, all but 17 were class IV. In groups A and C TISS scores were almost identical. The TISS score for group B was markedly lower during the 24-hour period before death (Table 2). In group A (nonpreventable death), we found that 48 of the 56 deaths were due to the underlying disease. The other 8 patients died because of a nonpreventable complication of treatment (Table 3). Group B (permissible death) consisted of 25 patients. Eleven of the 25 patients had (multiple) congenital anomalies associated with chromosomal anomalies. In 8 of these 11 patients treatment was initially started but was later withdrawn (Table 4). In most of the other 14 patients, often with multiple congenital anomalies, further treatment was withdrawn because of the complexity of different congenital anomalies or serious complications in the postoperative period such as intracranial hemorrhage (Table 5). The deaths of 23 patients were preventable (group C) because of errors in management. In 10 of these 23, death was the result of complications of treatment
Table 2. Mortality Groups in Relation to TISS No.
TISS Scores Study
Group
I points)
Nonpreventable death (n = 56) group A
o-9
of Patients
Day of Day of Admission Death
-
-
10-19
6
1
20-39
48
49
2
6
>40 Permissible death (n = 25) group B
o-9
1
4
10-19
7
12
20-39
16
8
1
1
240 Preventable death (n = 23) group C
o-9
-
-
10-19
5
2
20-39
17
18
1
3
>40
not related to infections in the postoperative period. Six of these 10 had irreversible brain damage due to prolonged hypoxemia. The different errors in these 10 patients are listed in Table 6. In the other 13 patients of group C, death was due to sepsis acquired in the postoperative period (Table 7). Staphylococcus epidemidis appeared to be the most frequently isolated pathogen (7/13), especially in the first years of this survey, and was treated inadequately. Of the gram-negative bacteria, Enterococcus, Klebsiella, and Pseudomonas species were most frequently found. In 10 of these 13 patients antibiotic therapy appeared not to have been in accordance with the microbiology culture and in 4 of them the central venous catheter had not been removed. Two patients never received antibiotics despite clinical and laboratory indicators of sepsis. In the remaining patient, sepsis had been diagnosed but treatment was started too late. DISCUSSION
Table 1. Primary Diagnosis on Admission No.
of Patients
Total
Premature
Multiple congenital anomalies
26
18
Congenital diaphragmatic hernia
21
8
Digestive tract
20
17
Congenital anomalies
Cardiac anomaly
8
3
Abdominal wall defect
7
4
Congenital adenomatoid malformation of lung
2
1
Teratoma
3
2
Liver hemangioma Total Other diseases Necrotizing enterocolitis
1 88
13
Sepsis
1
Hyaline membrane disease
1
Subdural hematoma
1
Total
16
53
11 1 12
Despite the importance of pediatric intensive care, little effort has been made to evaluate associated mortality. In 1976, Hecker and Warkotsch reported the results of a study3 with postoperative mortality percentages varying from 42% in neonates to 7% in older children. Mortality rates have been reported recently in only a few other studies, and range from 8% to 22%,4-7depending on composition of the study group (medical and/or surgical patients; whether or not included prematures). Our mortality rates, 4.7% for all admitted patients and 10% for our study group of neonates only, including premature babies, lie below the average mortality percentages. Comparison with other centers is difficult, because most data in the literature refer to combined medical and surgical pediatric ICU patients. Various scoring systems for ICU patients have been developed in recent years.x,9 These scoring
HAZEBROEK ET AL
1060
Table 3. Nonpreventable
Death (Group A): Primary Diagnosis in Relation to Cause of Death Cause No. of
Priman/Diagnosis
Patients
of Death
Underlying Disease
Complication of Treatment 2
MCA
11
9
CDH
17
17
SpecialRemarks Postoperative sepsis
Esophageal atresia
1
1
Postoperative sepsis
Pylorus atresia
1
1
Epidermolysis with sepsis
Meconium ileus
1
1
Postoperative sepsis
Meconium peritonitis
1
1
Postoperative sepsis
Cardiac anomaly
7
7
Omphalocele
2
1
HMD
1
Teratoma in cerebra
1
Sepsis
1
Sinus cavernosus bleeding
Sacrococcygeal teratoma
1
1
Congenital adenomatoid malformation of lung
2
2
Lung hypoplasia
NEC*
9
9
Sepsis/MOF
HMD
1
1
Not confirmed NEC
Subdural hematoma
1
1
Intrauterine trauma, car accident mother
56
48
Total Abbreviations:
MCA, multiple congenital anomalies;
CDH, congenital
Bleeding in tumor
8 diaphragmatic
hernia; NEC, necrotizing enterocolitis;
HMD, hyaline
membrane disease; MOF, multiple organ failure. *One patient with trisomy 21.
systems are useful for assessing severity of illness, therapeutic interventions, and cost-effectiveness calculations, for comparing patient populations, and also for predicting mortality.” We used the CCS and TISS scoring systems, because both are (also in retrospect) applicable to pediatric patients. Most of our patients were classified in CCS class IV on admission as well as before death, requiring frequent assessment and therapy by skilled nursing staff and physicians. Because it is assumed that severity of illness is indirectly related to quantity of care needed, TISS is an indirect assessment of severity of illness. In groups A and C were found most identical TISS scores, which indicate that both groups received an equal quantity of treatment. The TISS scores for group B were, in the 24-hour period before death, markedly lower than for groups A and C because
treatment was withdrawn, permitting these patients to die. In group A, 84% of deaths were due to the underlying disease. Despite recent developments in pediatric surgery,” patients die notwithstanding our advanced surgical, medical, and technical interferences. In 3 of the 11 patients from group B with chromosomal anomalies, immediately after admission, lifethreatening congenital anomalies were diagnosed combined with trisomy 13 or 18. Considering this as a condition incompatible with life, we withheld therapy to these newborns. In three other patients of group B (without chromosomal anomalies), we withheld therapy because during laparotomy the remaining small bowel length was incompatible with life. Alternative treatment such as small bowel transplantation was
Table 4. Permissible Death (Group 6): Primary Diagnosis in Relation to Cause of Death in 11 Patients With Chromosomal Anomalies Treatment
Chromosomal Primary Diagnosis
Anomaly
Withheld
Withdrawn
Most Serious Anomaly
and/or
Special Remarks
+
Omphalocele
Trisomy 13
MCA
Trisomy 18
+
Tetralogy of Fallot
MCA
Trisomy 13
+
Tetralogy of Fallot
Hirschsprung’s disease
Trisomy 21
+
Necrotizing enterocolitis with wound dehiscention
Omphalocele
Trisomy 13
+ +
Esophageal atresia
Trisomy 18
MCA
Tetrasomy 22
+
Complex cardiac anomaly
CDH
Trisomy 18
+
Ventilation started awaiting chromosomal investigation
MCA
Trisomy 21
+
AVSD, duodenal atresia, dysplastic kidneys with renal failure
Esophageal atresia
Trisomy 18
+
Gastrostomy, awaiting chromosomal investigation
MCA
Trisomy 21
+
Prune-belly syndrome, dysplasic kidneys with renal failure
Total
11
3
8
Abbreviations: MCA. multiple congenital anomalies: CDH, congenital diaphragmatic hernia; AVSD, atrioventricular septal defect.
MORTALITY IN SURGICAL NEONATES
1061
Table 5. Permissible Death (Group B): Primary Diagnosis in Relation to Cause of Death in 14 Patients Without Chromosomal Anomalies
PrimaryDiagnosis Multiple congenital anomalies
No. of Patients Withheld
Withdrawn
Special Remarks
7
Postoperative intracranial hemorrhage (4); peritonitis with bowel and wound de-
7
hiscention (1); inoperable cardiac anomaly (2) Malrotation
1
Small bowel atresia
2
Volvulus with small bowel necrosis Short bowel incompatible with life
Meconium peritonitis
1
Cystic fibrosis with sepsis
Gastroschisis
1
Premature, postoperative intracranial hemorrhage
Omphalocele
1
Postoperative intracranial hemorrhage
Sepsis
1
Suspected necrotizing enterocolitis, unrelenting hypoxemia
Total
3
11
considered as experimental treatment, which was not applied. The clinical picture in 5 patients pointed to a chromosomal anomaly, and we started full treatment of the children in expectation of the chromosomal investigation. After the suspected chromosomal anomaly was confirmed, treatment was withdrawn, permitting the child to die. In a number of children (listed in Tables 4 and 5), postoperative complications or unsalvageable cardiac or renal anomalies leading to renal insufficiency were considered to be so serious that it was decided to discontinue treatment. Such a decision is taken after thorough consultation with attending physicians, nursing staff, and parents.” A reasonably informed decision cannot always be made in a few hours, but may require accumulation of data over several days. Deciding whether a severely ill or handicapped newborn should be sustained on lifesupport systems or therapies, or should be permitted to die, is a difficult issue facing modern society.‘3.14 In our age of technical progress the pediatric surgeon and pediatric intensivist should be aware, more than ever before, of the problem of whether all that is technically feasible should always be carried out. We should take care not to take the position that everything that is possible on medical-technical grounds be done at all times. A permanent multidisci-
plinary medical-ethical infant care review committee was set up in the Sophia Children’s Hospital years ago. As recommended by the American Academy of Pediatrics, our review committee has a consultative role rather than a therapeutic decision-making role.15 Thus, it only functions as a sounding-board for contemplating the ethical implications of our daily work and provides well-considered advice. We had to classify 23 deaths as preventable, and these deaths are particularly concerning. In 10 patients, death was not related to infection during the postoperative period but to errors in surgical or medical treatment. By understanding the causes of these errors, it becomes possible to prevent the different sources of iatrogenic disease.16 Total parenteral nutrition using central venous catheters is widely used in the management of surgical neonates. For over 10 years, we have been using the Broviac silastic catheter, which is always placed by a cutdown in the operating room. A well-known serious complication is catheter sepsis, with an incidence in neonates ranging from 9% to 26%.” Twelve patients from group C died because of inadequate treatment of gram-positive or gram-negative sepsis. Eight of them had a Broviac catheter: in four cases it was not removed while the infection failed to respond clinically. In these four patients, catheter-related
Table 6. Preventable Death (Group C): Primary Diagnosis in Relation to Complications of Treatment Postoperative Primary Diagnosis Teratoma
Not Related to Infections During the
Period Error in Management
Cause of Death Hypoxemia
Surgery delayed, intubation injury
Hirschsprung’s disease
Sepsis + MOF
Colostoma on aganglionic bowel, enterocolitis with bowel perforation
Liver hemangioma
Sepsis + hypoxemia
Ligation a. hepatica delayed, inadequate medical treatment
Esophageal atresia
Cerebral bleeding, hypoxemia
Inadequate medical treatment after operative correction
Cardiac anomaly
Hemorrhagic shock
Retroperitoneal bleeding, delayed surgical management
Esophageal atresia
Hypoxemia
Bronchopulmonary
Omphalocele
Sepsis + MOF
Error in surgical technique, stomach perforation + peritonitis
Meconium ileus
Sepsis + MOF
Inadequate medical treatment of shock
CDH
Hypoxemia
Unrecognized pneumothorax during surgical procedure
CDH
Hypoxemia
Endotracheal tube located in esophagus
neck
Abbreviations: CDH, congenital diaphragmatic hernia; MOF, multiple organ failure.
dysplasia, epilepsy not recognized
HAZEBROEK ET AL
1062
Table 7. Preventable Deaths (Group C): Primary Diagnosis in Relation to Complications of Treatment Primary Diagnosis
Infections
cvc
+ _
Sepsis + MOF, Proteus
Inadequate sepsis treatment
Sepsis + MOF, Pseudomonas
NEC, inadequately treated with antibiotics
+
Sepsis + MOF, S epidermidis + Enterococcus
Inadequate antibiotics, CVC not removed
Sepsis + hypoxemia, Klebsieita
Sepsis not treated Inadequate antibiotics
MCA
_
Duodenal atresia*
+
NEC
+ _
CDH
Related to Postoperative Error in Management
Cause of Death
Stoma
_
Sepsis, Pseudomonas
Meconium peritonitis
+
+
Sepsis, S epidermidis
Inadequate antibiotics, CVC not removed
Small bowel atresia
+
Sepsis + MOF, Klebsiella
Inadequate antibiotics, CVC not removed
Duodenal atresia’
MCA
+
+ _
Sepsis + MOF, S epidermidis
Inadequate antibiotics
MCA
+
+
Sepsis, S epidermidis
Only gram-negative bacteria treated
+ Enterococcus
NEC
+
NEC
+
+ _
Sepsis, S epidermidis
+ Enterococcus
Inadequate antibiotics
NEC
+
+
Sepsis, S epidermidis
+ S aureus
Inadequate antibiotics
Meconium ileus
+
+
Sepsis, S epidermidis
Sepsis + MOF, Enterobacter
+ Klebsiella
Inadequate antibiotics, CVC not removed
No antibiotics treatment
Abbreviations: MCA, multiple congenital anomalies; NEC, necrotizing enterocolitis: CDH, congenital diaphragmatic hernia; MOF, multiple
organ
failure; CVC, central venous catheter.
sepsis may have been a preventable factor. Clinical management of children with a central venous catheter remains controversial. If sepsis is diagnosed, removal of the catheter is not always possible (for example, in newborns with enterostomies and a high stoma production) for whom the catheter is a “life line”). Systemic antibiotics without removal of the catheter has be successful in infants with catheterrelated sepsis.‘7-2o In a number of our patients the antibiotic management has not been adequate. Apart from a number of gram-negative species (Enterococcus, Klebsiella, and Pseudomonas), S epidermidis appeared to be the most isolated pathogen. The latter has long been considered as a skin contaminant in blood cultures, as in a number of our patients, especially in the first years of this study. This organism was recognized 5 years ago as an important clinical pathogen in the malnour-
ished, immunosuppressed, or otherwise seriously ill neonate.‘9’21Subsequently, after 1986 we did not have any preventable deaths related to S epidemtidis infection. The most important lessons we have learned from this retrospective survey is that the overall mortality rate is not very high in comparison to other centers, although we realize that exact comparison is impossible because data from comparable studies ar.e lacking. Second, evaluation of withholding or withdrawal of life-sustaining care is difficult yet important and helps us to formulate humane standards of intensive care. Third, some errors cannot really be prevented, but recognition of errors and retrospective discussion of what went wrong will undoubtedly have some preventive value. Last but not least, a more adequate sepsis therapy might have further reduced overall mortality.
REFERENCES 1. Cullen DJ,
JM, Briggo BA, et al: Therapeutic comparison
tion systems: patient 2. Keene AR, Cullen DJ: Therapeutic Update 1983. Crit Care Med ll:l-3,1983 A: survey of the causes of death in patients in pediatric Prog Surg 13:1.5-21,1979 P, P: Pediatric care: that influence Crit Care Med 10:34-37,1982 Pollack UE, et al: Evaluation intensive 1984 6. MM, Ruttiman prediction outcome intensive 1987 7. Beaufils of pediatric care in Intens Care Med 13:65-70,1987 Matthew of intensive scoring Intens Care Med 15:79-83,1989
Pollack
in intensive
10. MM, Ruttemann risk of mortality (PRISM) 1988 11. Altman RP: What’s new in pediatric rics 85:1121-1124,199O Smedira and withdrawel support ill. N Engl J Med 322:309-315,199O surgery. Theoret 1987 14. J: Approaches ethical making in the neonatal care unit. Am J Dis Child 142:825-830,1988 J, Grodin MA, et al: Live-saving therapy A questionaire Pediatrics 16. Vincer MJ, Murray JM, Yuill A, et al: Drug errors and incidents intensive
MORTALITY
IN SURGICAL NEONATES
17. Roberts JP, Gollow IJ: Central venous catheters in surgical neonates. J Pediatr Surg 25:632-634,199O 18. King DR, Komer M, Hoffman J, et al: Broviac catheter sepsis: The natural history of an iatrogenic infection. J Pediatr Surg 20:728-733,1985 19. Scherer LR, West KW, Weber TR, et al: Staphylococcus epidemidis sepsis in pediatric patients: Clinical and therapeutic considerations. J Pediatr Surg 19:358-361.1984
1063
20. Grisoni ER, Mehita SK, Connors AF: Thrombosis and infection complicating central venous catheterization in neonates. J Pediatr Surg 21:772-776, 1986 21. Freeman J, Epstein MF, Smith NE, et al: Extra hospital stay and antibiotic usage with nosocomial coagulase-negative staphylococcal bacteremia in two neonatal intensive care unit populations. Am J Dis Child 144:324-329, 1990
in Surgical Neonates Over a lo-Year Period: Permissible, and Preventable Death
Dick Tibboel,
Karin Leendertse-Verloop,
Albert
P. Bos, Gerard C. Madern,
and Jan C. Molenaar Rotterdam,
The Netherlands
l The mortality pattern and causes of death in surgical neonates were evaluated over the period of 1980 through 1990 in order to assess whether any deaths might have been prevented. In the study period 1,010 neonates were admitted, 104 of whom died (10%). These 104 neonates formed our study population. The Clinical Classification System (CCS) and the Therapeutic Intervention Scoring System (TISS) were used to assess the severity of illness. All 104 nonsurvivors were seriously ill (CCS 3 and 4; TISS > 20). The mortality pattern was classified in three groups. Group A (nonpreventable death) included 58 deaths, 48 of which were due to the underlying disease. The other 8 patients died of a nonpreventable complication of treatment. Eleven of the patients in group B (permissible death) had (multiple) congenital anomalies associated with chromosomal anomalies. Three of them were not treated and in the other 8 treatment was initially started but later withdrawn. In most of the other 14 patients further treatment was withdrawn because of serious postoperative complications or inoperable cardiac anomalies. Group C (preventable death) consisted of 23 patients. Six of 10 had irreversible brain damage due to prolonged hypoxemia. In the other 13 patients of group C, death was due to sepsis acquired in the postoperative period and treated inadequately. Staphylococcus epidermidis was the most frequent isolated pathogen (7/ 13). Copyright o 1991 by W.B. Saunders Company INDEX WORDS: Mortality, genital anomalies.
surgical neonates; mortality,
con-
T
HE PRESENT-DAY quality of perioperative care has a positive effect on morbidity and mortality in neonates with congenital anomalies. Therefore, we take pride in talking and writing about our good treatment results. Nevertheless, a number of these children still die, despite trained intensive care unit (ICU) physicians and nurses and wellequipped ICUs. Analysis of mortality provides an opportunity for developing preventive strategies to improve survival. Would a study of the pattern of mortality in neonates with congenital anomalies show
From the Department of Pediatric Surgery, Medical School of Erasmus University and Sophia Children’s Hospital, Rotterdam, The Netherlands. Presented at the 42nd Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Boston, Massachusetts, October 6- 7, 1990. Address reprint requests to F. W.J. Hazebroek, MD, Department of Pediatric Swgery, Sophia Children’s Hospital, PO Box 70029, 3000 LL Rotterdam, The Netherlands. Copyright o 1991 by WB. Saunders Company 0022-3468/91/2609-0010$03.00l0
1058
that any deaths might have been prevented? In order to evaluate this premise, we retrospectively evaluated the mortality pattern and causes of death in our pediatric surgical ICU (PSICU). MATERIALS
AND METHODS
From January 1980 to January 1990 all neonates who were admitted within 1 month after birth to the PSICLJ of the Sophia Children’s Hospital, Rotterdam, The Netherlands, and who died during hospitalization, were considered for our retrospective evaluation. The different parameters for these patients were assessed, such as duration of gestation, birth weight, sex, primary diagnosis on admission, treatment, and infant’s age at the time of death. All neonates were examined by a consultant in genetics. During a pilot analysis we decided to classify mortality pattern in three groups described as follows: group A: nonpreventable death-patients with serious life-threatening disorders who died despite maximal care; group B: permissible death-patients with (mostly congenital) disorders in whom death is the result of withholding or withdrawal of life-supporting surgical and/or medical intervention: and group C: preventable death-patients who died due to errors in management in which death possibly could have been avoided. Our patients were also evaluated in terms of severity of illness and quantity of care using the Clinical Classification System (CCS) and the Therapeutic Intervention Scoring System (TISS). CCS was assessed according to the definition of Cullen et al’: class I patients were not admitted to the ICU; class II patients were physiologically stable and were admitted to the ICU only for monitoring or observation; class III patients were physiologically stable requiring intensive nursing and monitoring; and class IV patients were unstable needing frequent assessment and interventions by ICU physicians and nurses. The TISS assesses the severity of the patient’s illness by quantitating the therapeutic intervention required for care. Each intervention is graded from 1 to 4 points depending on the time and effort required and the complexity of the procedure.’ CCS score and the maximal numbers of TISS points were scored during the first 24-hour period after admission and during the 24-hour period before death. The data were collected from each patient’s daily ICU flowsheet and medical file. Collection of data and classifying mortality pattern and causes of death were done by a senior registrar (K.L.-V.) not directly involved in the treatment of the patients. RESULTS
In the study period a total of 3,243 children of all ages were admitted to our PSICU, 152 of whom died (4.7%). This group of patients included 1,010 children admitted within 1 month after birth, 104 of whom died (10%). These 104 neonates formed our study population. Sixty-five of the 104 neonates (62.5%) were born prematurely (gestational age < 37 weeks). JournalofPediatric Surgery, Vol26. No 9 (September), 1991: pp 1058-1063
MORTALITY IN SURGICAL NEONATES
1059
Eighty-eight of the 104 patients had single or multiple congenital anomalies. Multiple congenital anomaiies were defined as two or more life-threatening congenital disorders. In 67 of these patients (76%) multiple congenital anomalies, congenital diaphragmatic hernia, and digestive tract anomalies were the primary diagnoses on admission. In the other 16 neonates, necrotizing enterocolitis was the most frequent primary diagnosis. A complete list of the primary diagnoses on admission is given in Table 1. No patients were CCS class I or II. On admission 78 patients were class IV and 26 were class III. On the day of death, all but 17 were class IV. In groups A and C TISS scores were almost identical. The TISS score for group B was markedly lower during the 24-hour period before death (Table 2). In group A (nonpreventable death), we found that 48 of the 56 deaths were due to the underlying disease. The other 8 patients died because of a nonpreventable complication of treatment (Table 3). Group B (permissible death) consisted of 25 patients. Eleven of the 25 patients had (multiple) congenital anomalies associated with chromosomal anomalies. In 8 of these 11 patients treatment was initially started but was later withdrawn (Table 4). In most of the other 14 patients, often with multiple congenital anomalies, further treatment was withdrawn because of the complexity of different congenital anomalies or serious complications in the postoperative period such as intracranial hemorrhage (Table 5). The deaths of 23 patients were preventable (group C) because of errors in management. In 10 of these 23, death was the result of complications of treatment
Table 2. Mortality Groups in Relation to TISS No.
TISS Scores Study
Group
I points)
Nonpreventable death (n = 56) group A
o-9
of Patients
Day of Day of Admission Death
-
-
10-19
6
1
20-39
48
49
2
6
>40 Permissible death (n = 25) group B
o-9
1
4
10-19
7
12
20-39
16
8
1
1
240 Preventable death (n = 23) group C
o-9
-
-
10-19
5
2
20-39
17
18
1
3
>40
not related to infections in the postoperative period. Six of these 10 had irreversible brain damage due to prolonged hypoxemia. The different errors in these 10 patients are listed in Table 6. In the other 13 patients of group C, death was due to sepsis acquired in the postoperative period (Table 7). Staphylococcus epidemidis appeared to be the most frequently isolated pathogen (7/13), especially in the first years of this survey, and was treated inadequately. Of the gram-negative bacteria, Enterococcus, Klebsiella, and Pseudomonas species were most frequently found. In 10 of these 13 patients antibiotic therapy appeared not to have been in accordance with the microbiology culture and in 4 of them the central venous catheter had not been removed. Two patients never received antibiotics despite clinical and laboratory indicators of sepsis. In the remaining patient, sepsis had been diagnosed but treatment was started too late. DISCUSSION
Table 1. Primary Diagnosis on Admission No.
of Patients
Total
Premature
Multiple congenital anomalies
26
18
Congenital diaphragmatic hernia
21
8
Digestive tract
20
17
Congenital anomalies
Cardiac anomaly
8
3
Abdominal wall defect
7
4
Congenital adenomatoid malformation of lung
2
1
Teratoma
3
2
Liver hemangioma Total Other diseases Necrotizing enterocolitis
1 88
13
Sepsis
1
Hyaline membrane disease
1
Subdural hematoma
1
Total
16
53
11 1 12
Despite the importance of pediatric intensive care, little effort has been made to evaluate associated mortality. In 1976, Hecker and Warkotsch reported the results of a study3 with postoperative mortality percentages varying from 42% in neonates to 7% in older children. Mortality rates have been reported recently in only a few other studies, and range from 8% to 22%,4-7depending on composition of the study group (medical and/or surgical patients; whether or not included prematures). Our mortality rates, 4.7% for all admitted patients and 10% for our study group of neonates only, including premature babies, lie below the average mortality percentages. Comparison with other centers is difficult, because most data in the literature refer to combined medical and surgical pediatric ICU patients. Various scoring systems for ICU patients have been developed in recent years.x,9 These scoring
HAZEBROEK ET AL
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Table 3. Nonpreventable
Death (Group A): Primary Diagnosis in Relation to Cause of Death Cause No. of
Priman/Diagnosis
Patients
of Death
Underlying Disease
Complication of Treatment 2
MCA
11
9
CDH
17
17
SpecialRemarks Postoperative sepsis
Esophageal atresia
1
1
Postoperative sepsis
Pylorus atresia
1
1
Epidermolysis with sepsis
Meconium ileus
1
1
Postoperative sepsis
Meconium peritonitis
1
1
Postoperative sepsis
Cardiac anomaly
7
7
Omphalocele
2
1
HMD
1
Teratoma in cerebra
1
Sepsis
1
Sinus cavernosus bleeding
Sacrococcygeal teratoma
1
1
Congenital adenomatoid malformation of lung
2
2
Lung hypoplasia
NEC*
9
9
Sepsis/MOF
HMD
1
1
Not confirmed NEC
Subdural hematoma
1
1
Intrauterine trauma, car accident mother
56
48
Total Abbreviations:
MCA, multiple congenital anomalies;
CDH, congenital
Bleeding in tumor
8 diaphragmatic
hernia; NEC, necrotizing enterocolitis;
HMD, hyaline
membrane disease; MOF, multiple organ failure. *One patient with trisomy 21.
systems are useful for assessing severity of illness, therapeutic interventions, and cost-effectiveness calculations, for comparing patient populations, and also for predicting mortality.” We used the CCS and TISS scoring systems, because both are (also in retrospect) applicable to pediatric patients. Most of our patients were classified in CCS class IV on admission as well as before death, requiring frequent assessment and therapy by skilled nursing staff and physicians. Because it is assumed that severity of illness is indirectly related to quantity of care needed, TISS is an indirect assessment of severity of illness. In groups A and C were found most identical TISS scores, which indicate that both groups received an equal quantity of treatment. The TISS scores for group B were, in the 24-hour period before death, markedly lower than for groups A and C because
treatment was withdrawn, permitting these patients to die. In group A, 84% of deaths were due to the underlying disease. Despite recent developments in pediatric surgery,” patients die notwithstanding our advanced surgical, medical, and technical interferences. In 3 of the 11 patients from group B with chromosomal anomalies, immediately after admission, lifethreatening congenital anomalies were diagnosed combined with trisomy 13 or 18. Considering this as a condition incompatible with life, we withheld therapy to these newborns. In three other patients of group B (without chromosomal anomalies), we withheld therapy because during laparotomy the remaining small bowel length was incompatible with life. Alternative treatment such as small bowel transplantation was
Table 4. Permissible Death (Group 6): Primary Diagnosis in Relation to Cause of Death in 11 Patients With Chromosomal Anomalies Treatment
Chromosomal Primary Diagnosis
Anomaly
Withheld
Withdrawn
Most Serious Anomaly
and/or
Special Remarks
+
Omphalocele
Trisomy 13
MCA
Trisomy 18
+
Tetralogy of Fallot
MCA
Trisomy 13
+
Tetralogy of Fallot
Hirschsprung’s disease
Trisomy 21
+
Necrotizing enterocolitis with wound dehiscention
Omphalocele
Trisomy 13
+ +
Esophageal atresia
Trisomy 18
MCA
Tetrasomy 22
+
Complex cardiac anomaly
CDH
Trisomy 18
+
Ventilation started awaiting chromosomal investigation
MCA
Trisomy 21
+
AVSD, duodenal atresia, dysplastic kidneys with renal failure
Esophageal atresia
Trisomy 18
+
Gastrostomy, awaiting chromosomal investigation
MCA
Trisomy 21
+
Prune-belly syndrome, dysplasic kidneys with renal failure
Total
11
3
8
Abbreviations: MCA. multiple congenital anomalies: CDH, congenital diaphragmatic hernia; AVSD, atrioventricular septal defect.
MORTALITY IN SURGICAL NEONATES
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Table 5. Permissible Death (Group B): Primary Diagnosis in Relation to Cause of Death in 14 Patients Without Chromosomal Anomalies
PrimaryDiagnosis Multiple congenital anomalies
No. of Patients Withheld
Withdrawn
Special Remarks
7
Postoperative intracranial hemorrhage (4); peritonitis with bowel and wound de-
7
hiscention (1); inoperable cardiac anomaly (2) Malrotation
1
Small bowel atresia
2
Volvulus with small bowel necrosis Short bowel incompatible with life
Meconium peritonitis
1
Cystic fibrosis with sepsis
Gastroschisis
1
Premature, postoperative intracranial hemorrhage
Omphalocele
1
Postoperative intracranial hemorrhage
Sepsis
1
Suspected necrotizing enterocolitis, unrelenting hypoxemia
Total
3
11
considered as experimental treatment, which was not applied. The clinical picture in 5 patients pointed to a chromosomal anomaly, and we started full treatment of the children in expectation of the chromosomal investigation. After the suspected chromosomal anomaly was confirmed, treatment was withdrawn, permitting the child to die. In a number of children (listed in Tables 4 and 5), postoperative complications or unsalvageable cardiac or renal anomalies leading to renal insufficiency were considered to be so serious that it was decided to discontinue treatment. Such a decision is taken after thorough consultation with attending physicians, nursing staff, and parents.” A reasonably informed decision cannot always be made in a few hours, but may require accumulation of data over several days. Deciding whether a severely ill or handicapped newborn should be sustained on lifesupport systems or therapies, or should be permitted to die, is a difficult issue facing modern society.‘3.14 In our age of technical progress the pediatric surgeon and pediatric intensivist should be aware, more than ever before, of the problem of whether all that is technically feasible should always be carried out. We should take care not to take the position that everything that is possible on medical-technical grounds be done at all times. A permanent multidisci-
plinary medical-ethical infant care review committee was set up in the Sophia Children’s Hospital years ago. As recommended by the American Academy of Pediatrics, our review committee has a consultative role rather than a therapeutic decision-making role.15 Thus, it only functions as a sounding-board for contemplating the ethical implications of our daily work and provides well-considered advice. We had to classify 23 deaths as preventable, and these deaths are particularly concerning. In 10 patients, death was not related to infection during the postoperative period but to errors in surgical or medical treatment. By understanding the causes of these errors, it becomes possible to prevent the different sources of iatrogenic disease.16 Total parenteral nutrition using central venous catheters is widely used in the management of surgical neonates. For over 10 years, we have been using the Broviac silastic catheter, which is always placed by a cutdown in the operating room. A well-known serious complication is catheter sepsis, with an incidence in neonates ranging from 9% to 26%.” Twelve patients from group C died because of inadequate treatment of gram-positive or gram-negative sepsis. Eight of them had a Broviac catheter: in four cases it was not removed while the infection failed to respond clinically. In these four patients, catheter-related
Table 6. Preventable Death (Group C): Primary Diagnosis in Relation to Complications of Treatment Postoperative Primary Diagnosis Teratoma
Not Related to Infections During the
Period Error in Management
Cause of Death Hypoxemia
Surgery delayed, intubation injury
Hirschsprung’s disease
Sepsis + MOF
Colostoma on aganglionic bowel, enterocolitis with bowel perforation
Liver hemangioma
Sepsis + hypoxemia
Ligation a. hepatica delayed, inadequate medical treatment
Esophageal atresia
Cerebral bleeding, hypoxemia
Inadequate medical treatment after operative correction
Cardiac anomaly
Hemorrhagic shock
Retroperitoneal bleeding, delayed surgical management
Esophageal atresia
Hypoxemia
Bronchopulmonary
Omphalocele
Sepsis + MOF
Error in surgical technique, stomach perforation + peritonitis
Meconium ileus
Sepsis + MOF
Inadequate medical treatment of shock
CDH
Hypoxemia
Unrecognized pneumothorax during surgical procedure
CDH
Hypoxemia
Endotracheal tube located in esophagus
neck
Abbreviations: CDH, congenital diaphragmatic hernia; MOF, multiple organ failure.
dysplasia, epilepsy not recognized
HAZEBROEK ET AL
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Table 7. Preventable Deaths (Group C): Primary Diagnosis in Relation to Complications of Treatment Primary Diagnosis
Infections
cvc
+ _
Sepsis + MOF, Proteus
Inadequate sepsis treatment
Sepsis + MOF, Pseudomonas
NEC, inadequately treated with antibiotics
+
Sepsis + MOF, S epidermidis + Enterococcus
Inadequate antibiotics, CVC not removed
Sepsis + hypoxemia, Klebsieita
Sepsis not treated Inadequate antibiotics
MCA
_
Duodenal atresia*
+
NEC
+ _
CDH
Related to Postoperative Error in Management
Cause of Death
Stoma
_
Sepsis, Pseudomonas
Meconium peritonitis
+
+
Sepsis, S epidermidis
Inadequate antibiotics, CVC not removed
Small bowel atresia
+
Sepsis + MOF, Klebsiella
Inadequate antibiotics, CVC not removed
Duodenal atresia’
MCA
+
+ _
Sepsis + MOF, S epidermidis
Inadequate antibiotics
MCA
+
+
Sepsis, S epidermidis
Only gram-negative bacteria treated
+ Enterococcus
NEC
+
NEC
+
+ _
Sepsis, S epidermidis
+ Enterococcus
Inadequate antibiotics
NEC
+
+
Sepsis, S epidermidis
+ S aureus
Inadequate antibiotics
Meconium ileus
+
+
Sepsis, S epidermidis
Sepsis + MOF, Enterobacter
+ Klebsiella
Inadequate antibiotics, CVC not removed
No antibiotics treatment
Abbreviations: MCA, multiple congenital anomalies; NEC, necrotizing enterocolitis: CDH, congenital diaphragmatic hernia; MOF, multiple
organ
failure; CVC, central venous catheter.
sepsis may have been a preventable factor. Clinical management of children with a central venous catheter remains controversial. If sepsis is diagnosed, removal of the catheter is not always possible (for example, in newborns with enterostomies and a high stoma production) for whom the catheter is a “life line”). Systemic antibiotics without removal of the catheter has be successful in infants with catheterrelated sepsis.‘7-2o In a number of our patients the antibiotic management has not been adequate. Apart from a number of gram-negative species (Enterococcus, Klebsiella, and Pseudomonas), S epidermidis appeared to be the most isolated pathogen. The latter has long been considered as a skin contaminant in blood cultures, as in a number of our patients, especially in the first years of this study. This organism was recognized 5 years ago as an important clinical pathogen in the malnour-
ished, immunosuppressed, or otherwise seriously ill neonate.‘9’21Subsequently, after 1986 we did not have any preventable deaths related to S epidemtidis infection. The most important lessons we have learned from this retrospective survey is that the overall mortality rate is not very high in comparison to other centers, although we realize that exact comparison is impossible because data from comparable studies ar.e lacking. Second, evaluation of withholding or withdrawal of life-sustaining care is difficult yet important and helps us to formulate humane standards of intensive care. Third, some errors cannot really be prevented, but recognition of errors and retrospective discussion of what went wrong will undoubtedly have some preventive value. Last but not least, a more adequate sepsis therapy might have further reduced overall mortality.
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IN SURGICAL NEONATES
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