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Mode of delivery and neonatal bleeds in hemophilia
hemophilia 0.7% (1 of 140) (P=0.009) where only one major bleed in the neonatal period was reported.
The analysis of major bleeds in severe hemophilia only showed the same significant results regarding vaginal instrumental delivery as a risk factor compared to vaginal delivery without instruments (P<0.0001; RR 7.28) and CS prior to labor (P=0.0004; RR 6.05) (Table 3); therefore, these groups were kept together in the further analysis.
Family history of hemophilia
In 466 neonates there was a known family history of hemophilia. In 445 cases, no family history of hemophilia was known; these patients represent sporadic cases where no influence on mode of delivery could be made. The rate of CS differed significantly between the groups (P=0.009): 35.8% (167 of 466) in the group with known family histo- ry were born by CS and 27.6% (123 of 445) of the spo- radic cases (Table 5). The reason for planned CS in the group of known family history was hemophilia carrier sta- tus and/or known hemophilia status of the child in 45.2% (38 of 84 cases), in 14.3% (12 of 84 cases) due to a combi- nation of hemophilia status and maternal/fetal issues, and in 29.8% not related to hemophilia (n=16 cases, maternal; n=9 cases, fetal). The reasons recorded for a planned CS in the group with no known family history of hemophilia (n=49) were maternal reasons in 65.3% (n=32), fetal rea- sons in 24.5% (n=12), combined reasons in 4.1% (n=2), and Other in 6.1% (n=3). Vaginal instrumental deliveries occurred less often when a family history was known (18 of 466) than in the group with no known family history (48 of 445) (P=0.00038).
However, there was no significant difference in the fre- quency of major bleeds and ICH between the group with known family history (KFH) and the group with no known family history (NFH) (P=0.87 and P=0.37, respec- tively). In the KFH group, we found an overall frequency of 4.7% (22 of 466) for major bleeds and 1.7% (8 of 466) for ICH. In comparison, in NFH the frequency was of 4.5% (20 of 445) for major bleeds and 2.7% (12 of 445) for ICH. For more detailed information see Table 5.
Prenatal diagnosis
Prenatal diagnosis was performed in 13.7% (62 of 466) of the children with a known family history of hemophil-
Table 4. Pairwise comparisons between groups on mode of delivery.
ia. In this group, the rate of CS was significantly higher (32 of 62; 51.6% vs. 135 of 404, 33.4%) than in the group with known family history and no PND (P=0.0068).
Mortality
One child with no known family history died after CS in labor due to ICH in the neonatal period. The patient was diagnosed with ICH at six days of age and the diag- nosis of hemophilia A was made on the same day; this child was included in the analysis.
No other deaths related to major bleeds or ICH were reported.
Counseling of a pregnant carrier of hemophilia
In counseling a pregnant carrier, the decision to be taken is between a planned CS (in most cases prior to labor) compared to planned vaginal delivery which can result in vaginal delivery with or without instruments or CS during labor. Patients with planned vaginal delivery (n=703) had a non-instrumental vaginal delivery in 77% (541 of 703), an instrumental delivery in 9.7% (68 of 703), and a CS dur- ing labor in 9.9% (70 of 703); in 24 patients it was unknown if the vaginal delivery was with or without instruments (3.4%). Patients with planned CS (n=134) had a CS prior to labor in 93.2% (125 of 134) and in 9 patients the CS was performed in labor (6.8%) (Table 6). We com- pared planned CS (n=134) to planned vaginal delivery (n=703) for the whole cohort, and no significant difference could be seen for both ICH (P=0.75) and major bleeds (P=0.82). The frequencies for ICH were 1.5% (2 of 134) for planned CS and 2.4% (17 of 703) for planned vaginal delivery. Frequencies for major bleeds were 3.7% (5 of 134) for planned CS and 4.8% (34 of 703) for planned vagi- nal delivery. (See Table 6 for an overview.) We also com- pared the subgroup of patients with known family history of hemophilia and compared the number of major bleeds and ICH in planned CS (n=84) and planned vaginal deliv- ery (n=327); even here, there was no significant difference (P=0.777 for major bleeds, P=1 for ICH).
Discussion
In this multicenter study, no statistical difference was
Reference group
Vaginal delivery without instruments
Vaginal delivery without instruments
Vaginal delivery without instruments
Cesarian prior to labor
Cesarian during labor
Cesarian prior to labor
Comparison group
Vaginal instrumental
Cesarian prior to labor
Cesarian during labor
Vaginal
instrumental
Vaginal instrumental
Cesarian during labor
ICH P-value; RR (95%CI)
§*0.0005; 6.96 (2.61,18.60)
1.0000; 1.08
(0.23,5.03)
0.3709; 1.71 (0.37,7.92)
*0.0100; 6.43
(1.37,30.12)
0.0814; 4.07 (0.87,18.92)
0.6417; 1.58
(0.23,11.01)
Major bleeding P-value; RR (95%CI)/severe hemophilia only
§*<0.0001; 7.39 (3.63,15.05)/ §*<0.0001; 7.28 (3.60,14.72)
0.3758; 1.55 (0.57,4.21)/
0.3408; 1.20 (0.4.;3.58)
*0.0110; 3.42 (1.43,8.22)/ *0.0209; 3.20 (1.34; 7.66)
§*0.0012; 4.78(1.78,12.84)/
§*0.0004; 6.05 (2.07, 17.72)
0.0918; 2.16 (0.91,5.10)/ 0.1263; 2.27 (0.97, 5.32)
0.2207; 2.22 (0.73,6.74)/
0.09396; 2.66 (0.81, 8.76)
ICH: intracranial hemorrhage; RR. relative risk; CI: Confidence Interval. *Significant at significance level P<0.05; §significant after Bonferroni correction P<0.0083.
haematologica | 2019; 104(10)
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