Académique Documents
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of Mortality Factors
By Hans Skari, Kristin Bjornland, Guttorm Haugen, Thore Egeland, and Ragnhild Emblem
Oslo, Norway
Conclusions: Prenatal diagnosis of CDH, presence of associated major malformations, and the study population have a
major influence on mortality rate. The very high mortality
rate in studies of fetuses with a prenatal diagnosis of CDH
should be taken into account in prenatal counselling.
J Pediatr Surg 35:1187-1197. Copyright r 2000 by W.B.
Saunders Company.
INDEX WORDS: Congenital diaphragmatic hernia, metaanalysis, selection bias, systematic review, associated anomalies, associated major malformations, prenatal diagnosis,
side of hernia, timing of surgery, prenatal ultrasound, termination of pregnancy, intrauterine fetal demise, hidden mortality, neonatal mortality, perinatal outcome.
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SKARI ET AL
RESULTS
1189
Table 1. Frequency of Associated Major Malformations and Mortality Rates in Fetuses With Prenatally Diagnosed CDH
Proportion
IUFD
(%)
True
Postnatal
Mortality
Rate
(%)
Total
Mortality
Rate Excl.
TOP
(%)
Total
Mortality
Rate
(%)
Study
No.
Associated
Major
Malformations
(%)
al9
Adzick et
Adzick et al10
Bollmann et al16
Bronshtein et al75
Calisti et al76
Crawford et al77
Dommergues et al78
Fox et al79
Geary et al80
Germain et al81
Guibaud et al82
Howe et al109
Manni et al83
Meizner and Levy84
Sharland et al85
Teixeira et al86
Thorpe-Beeston et al87
94
38
33
15
6
19
135
24
34
32
40
48
28
11
28
36
36
16.0
28.9
72.7
33.3
16.7
15.8
32.6
16.7
47.1
43.8
35.0
43.8
53.6
NA
23.6
25.0
47.2
1.1
10.5
21.2
46.7
0.0
5.3
28.2
NA
44.1
NA
30.0
20.8
7.1
45.5
20.0
50.0
41.7
3.2
20.6
26.9
0.0
0.0
0.0
4.1
8.3
5.3
NA
3.6
10.5
11.5
0.0
6.8
0.0
4.8
78.9
66.7
73.7
25.0
83.0
50.0
71.0
31.8
66.7
81.3
55.5
61.7
82.6
100.0
56.1
64.3
50.0
79.6
73.5
80.8
25.0
83.3
50.0
72.2
37.5
68.4
81.3
57.1
65.8
84.6
100.0
59.1
64.3
52.4
79.8
76.3
84.5
60.0
83.3
52.6
80.0
37.5
82.4
81.3
70.0
72.9
85.7
100.0
67.3
82.1
72.2
Pooled
676
33.7
22.9
7.2
66.9
69.1
75.6
Proportion
TOP
(%)
NOTE. The summary calculations only include the studies providing data on the respective variables.
Abbreviation: NA, not available.
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SKARI ET AL
Study
al88
Adolph et
Al-Hathal et al89
Azarow et al7
Benjamin et al90
Butt et al91
Chan et al5
Cuniff et al92
de la Hunt et al23
Finer et al93
Frenckner et al1
Hansen et al94
Haugen et al95
Lessin et al96
Macken and Thompson19
Miguet et al110
Nakayama et al8
Pfleghaar et al97
Reyes et al3
Ruff et al98
Skari et al99
Steimle et al100
Thibeualt and
Haney101
West et al102
Wilson et al6
Pooled
Prenatal
Diagnosis
(%)
Associated
Major
Malformations
(%)
Visible
Postnatal
Mortality
Rate
(%)
18
21
223
108
90
19
92
54
65
43
75
10
123
NA
52.3
15.2
NA
NA
78.9
NA
59.3
63.1
23.3
NA
50.0
NA
11.1
9.5
15.7
39.8
12.2
52.6
39.1
1.9
NA
2.3
25.3
10.0
0.8
16.7
19.1
45.3
61.1
43.3
79.0
47.8
48.2
21.5
9.3
46.7
20.0
59.4
11
18
20
55
22
26
8
111
63.6
61.1
45.0
42.0
40.9
NA
25.0
34.2
9.1
22.2
40.0
9.1
18.2
61.5
12.5
25.2
36.4
27.8
75.0
50.9
18.2
61.5
37.5
36.0
90
111
196
NA
NA
44.9
8.9
33.3
48.0
36.7
46.9
46.9
1,609
38.2
23.3
44.6
No.
1191
Table 3. Population-Based Studies on CDH: Frequency of Prenatal Diagnosis, Frequency of Associated Major Malformations
and Mortality Rates
Associated
Major
Malformations
(%)
Proportion
TOP
(%)
Proportion
IUFD
(%)
Hidden
Postnatal
Mortality
Rate
(%)
Visible
Postnatal
Mortality
Rate
(%)
True
Postnatal
Mortality
Rate
(%)
Total
Mortality
Rate
(%)
Study
No.
Prenatal
Diagnosis
(%)
Cannon et al12
Forrester and Merz104
Harrison et al15
Jaffray and Makinlay11
Moore et al105
Puri and Gorman103
Scott and Renwick106
Steinhorn et al13
Torfs et al107
Wenstrom et al108
96
51
70
44
39
36
9
48
237
65
53.1
23.5
NA
23.5
87.2
NA
0.0
31.3
NA
18.5
46.9
41.2
NA
NA
25.6
58.3
NA
25.0
41.7
27.7
5.2
9.8
NA
NA
20.5
NA
NA
NA
0.8
NA
2.2
2.2
NA
NA
9.7
30.6
11.1
NA
4.3
21.5
NA
NA
52.9
36.4
10.7
60.0
NA
8.3
NA
NA
NA
NA
30.3
46.4
32.0
40.0
NA
34.1
NA
NA
40.4
51.1
67.1
65.9
39.3
76.0
50.0
39.6
58.2
47.1
44.8
56.9
NA
NA
56.4
83.3
55.6
NA
60.3
58.5
Pooled
695
37.2
39.3
4.7
8.2
34.9
35.7
54.2
58.2
NOTE. The summary calculations only include the studies providing data on the respective variables.
Abbreviation: NA, not available.
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SKARI ET AL
Study
Category
Proportion
TOP (%)
Proportion
IUFD (%)
Hidden
Postnatal
Mortality
Rate (%)
22.9
NA
4.7
7.2
NA
8.2
12.9
NA
34.9
Visible
Postnatal
Mortality
Rate (%)
True
Postnatal
Mortality
Rate (%)
Total
Mortality
Rate Excl.
TOP (%)
Total
Mortality
Rate (%)
61.3
44.6
35.7
66.9
NA
54.2
69.1
NA
56.5
75.6
NA
58.2
Place and
Period (Births)
Langham et al26
No.
Birth
Prevalence
166
0.17
70
0.18
21
0.19
310
0.20
0.22
498
0.23
51
0.25
65
0.27
45
0.27
136
0.31
116
0.31
237
0.33
91
0.35
48
0.36
20
0.41
77
0.57
1,950
0.25
1193
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