Background

Benckiser's hemorrhage was first described in 1831 by R. Benckiser. It is a pure fetal haemorrhage resulting from the tearing of one or more umbilical vessels occurring during the rupture of the membranes. This follows a velmentous insertion of the cord. It is a rare obstetric accident, its frequency is estimated at 1/4000 deliveries (Nohuz et al., 2015). Its prognosis is formidable. Indeed, despite the progress of neonatal resuscitation, the mortality of this syndrome remains more than 50%. This pejorative prognosis can be explained in part by a delay or a lack of knowledge of the diagnosis.

The aim of our work is to specify the means and the possibilities of an early prenatal diagnosis that can improve the fetal prognosis.

1 Observation

Ms Z.O., Aged 30, second gesture, prim parous blood group. O positive, with no particular antecedents noteworthy, pregnant at 39 weeks’ gestation, followed 4 times by a gynecologist for placenta anterior low inserted type I of the Bessis classification. She consulted us for the start of work. The examination at admission had found an arterial tension at 12/8, an uterine height at 31 cm, uterine contractions present and regular at the rate of 3 contr./10mn. At the vaginal touch the cervix was open at 1 cm, the presentation was cephalic and the pouch of the water was intact. Ultrasound monitoring showed a eutrophic fetus in cephalic presentation and a still low placenta inserted. Fetal heart rhythm recording demonstrated a reactive tracing with a base frequency of 130 bps / min.

An amniotomy was performed at 4 cm following the occurrence of genital haemorrhage of medium abundance made of bright red blood with the appearance at the fetal heart rhythm recording of repetitive and deep decelerations (Figure 1). Benckiser's hemorrhage diagnosis was evoked and an emergency caesarean section was performed, allowing the extraction of a boy of 3400 g of apgar 2/4/4 with intense mucosal skin pallor.

Neonatal resuscitation was started in the delivery room and continued in the neonatal department where a biological check-up was performed and showed an anemia at 9.1 g / ml and an arterial PH at 7.18. A vascular filling with serum albumin at a rate of 1 g / kg and a blood transfusion with two pockets of globular O-negative pellets were performed. The development was favorable and the newborn had left the service on the 4^th day of life.

Macroscopic examination of the placenta showed a velamentous cord insertion with rupture of a main vascular bronchus (Figure 2).

2 Discussion

Benckiser's hemorrhage is a serious obstetric event involving fetal life expectancy through exsanguination. Fortunately rare, its frequency varies from 1/2000 to 1/5000 deliveries (Heckel et al., 1993; Nohuz et al., 2015). This frequency is higher in multiple pregnancies.

Many risk factors have been reported; it is essential to know them in order to make a prenatal oriented screening of this fearful affection. These risk factors are represented by: the placenta inserted low, bipartita and multilobulate, aberrant cotyledon and the velamentous cord insertion (Carbonnel et al., 2007; Chmait et al., 2010; Gagnon et al., 2010). Indeed, in the presence of a velamentous y insertion of the cord associated with a placenta praevia, the incidence of vasa praevia is estimated at 1/50 (Hasegawa et al., 2011; Nishtar and Wood, 2012). Moreover, in many authors, in vitro fertilization (IVF) would be a risk factor, since it multiplies the risk by ten (Baulies et al., 2007; Chmait et al., 2010; Cipriano et al., 2010; Gagnon et al., 2010; Hasegawa et al., 2011; Nishtar and Wood, 2012). Of these, it would be desirable to carry out systematic screening in patients with risk factors.

This vicious cord implantation, which instead of being inserted into the placenta, is inserted on the membranes and its vascular ramifications, not protected by Wharton's jelly, bind the placenta "bare" thereby in addition to the risks potential asphyxia by compression or fetal hemorrhage that may occur during pregnancy (Kazadi, 1991). The severity of this insertion resides in the occurrence of fetal haemorrhage by vascular shredding during the spontaneous or artificial rupture of the membranes (Kazadi, 1991; Heckel et al., 1993; Régis et al., 2006). This hemorrhagic accident complicates one case for 50 velamentous insertions.

It is also reported, but in an exceptional way, that this haemorrhage may be secondary to preavia pathways of the chorionic vessels outside any velamentous insertion. This aberrant path is most often linked to an aberrant cotyledon or to a very eccentric insertion of the cord. In our observation, haemorrhage was secondary to a velamentous insertion of the cord on a low inserted placenta type I of Bessis.

The clinical picture includes a typical symptomatic triad (Kazadi, 1991; Heckel et al., 1993; Régis et al., 2006; Aissi et al., 2013):

1- Painless genital hemorrhage, often important, concomitant with rupture of the membranes and which persists after the amniotomy.

2- Acute and immediate fetal suffering, rapidly unfavorable evolution towards fetal death by exsanguination in utero.

3- A constant maternal general condition.

Such symptomatology should prompt Benckiser's hemorrhage diagnosis and indicate immediate fetal extraction (Chmait et al., 2010). However, this diagnosis may be more delicate because of the clinical polymorphism of this disease: fetal haemorrhage may be delayed compared to rupture of membranes and occurs only late in labor when the vascular wound occurs during enlargement of the membrane orifice by the fetal presentation (Aissi et al., 2013), it can be broken during iatrogenic maneuvers such as the setting of scalp electrode. This delay between the rupture of the membranes and the occurrence of hemorrhage can be extended from a few minutes to several hours.

Fetal hemorrhage may also be absent or stopped by compression of the vascular wound by fetal presentation or thrombosis, under these conditions fetal distress may be absent and bleeding is labeled as isolated but severity Of this form is the risk of recurrence of the haemorrhage with a very reserved fetal prognosis.

The rupture of these preavia vessels can occur with intact membranes, and cause an in utero or externalized haemorrhage, so the diagnosis is extremely difficult. Its etiopathogenesis is imprecise (histological lesion of endarteritis, rupture of an umbilical vein of a very short cord).

Finally, there are completely asymptomatic forms when the rupture spares the vascular ramifications and the presentation progresses without incident while repressing these vascular branches. In these cases, the diagnosis is retrospectively examined for delivery (Aissi et al., 2013). In our observation the clinical picture was complete of which made the diagnosis easy to take care in time.

The differential diagnosis is that of any hemorrhage in the third trimester. It arises with:

1- The placenta preavia: The association of these two pathologies is frequent. The fundamental element of the diagnosis is that the haemorrhage of the placenta preavia is anterior to the rupture of the membranes and can cease shortly afterwards, whereas the hemorrhage of Benckiser succeeds it.

2- The retro placental hematoma: The haemorrhage is made of minimal black blood, anterior to the rupture of the membranes with alteration of the maternal general state; often in the context of pre-eclampsia.

3- Uterine rupture: Fetal suffering and haemorrhage are common for both diseases, but the context (scar or multiparity uterus) associated with an alteration of the patient's general condition makes it possible to correct the diagnosis.

4- Low genital haemorrhages: They do not affect the fetus. Their diagnosis is based on simple speculum examination.

The severity of the condition justifies the need for early diagnosis to improve this extremely unfavorable fetal prognosis. This diagnosis should ideally be made before the rupture of the membranes. It can be done: to the vaginal touch which recognizes exceptionally the preavia vessels by palpating fibrous, hard, pulsatile cords circulating in the membranes.

Amnioscopy, which is used less and less, makes it possible to make the diagnosis of certainty of the condition and thus prevents cases of exsanguination by indicating an extraction with intact membranes. Many cases diagnosed by amnioscopy and favorable development have been reported.

Giannopoulos (Giannopoulos and Carver, 1987) reported for the first time the ultrasound diagnosis of a preavia vessel and since this examination was proposed as a diagnostic element. Ultrasound allows the detection of vessels passing between the presentation and the internal orifice of the neck (Baulies et al., 2007; Carbonnel et al., 2007; Chmait et al., 2010; Cipriano et al., 2010; Hasegawa et al., 2011). Later it was shown that this method is not reproducible because of the difficulties of visualizing small diameter vessels buried in the bottom of the pelvis. This examination should therefore be coupled to the vaginal color doppler to improve the visualization of vascular flow (Baulies et al., 2007; Cipriano et al., 2010; Hasegawa et al., 2011; Nishtar and Wood, 2012; Ruiter et al., 2015).

In case of haemorrhage, the diagnosis is based on the detection of fetal hemoglobin. This search must not delay the extraction decision. Numerous tests have been proposed; In particular that of Kleihauer. Hemoglobin electrophoresis was also used. The major disadvantage of these two tests is the length of time required for making a sufficiently long 30 minutes to 1 hour. Some authors (Kazadi, 1991) recommend the APT test or alkaline denaturation resistance test, it is faster realization, its principle is based on a colorimetric reaction observable with the naked eye. This test requires a large blood volume (2 to 3 ml) and only in the laboratory, which limits its use.

Currently, other colorimetric tests are reported, all short-circuit centrifugation and workable in a few minutes (Heckel et al., 1993). The Jones test appears to be the most relevant because of sensitivity and specificity of 100% (Jones et al., 1987). This test consists of mixing 9 volumes of blood taken at 1% sodium hydroxide in a test tube or on filter paper, fetal hemoglobin resists alkaline denaturation and the sample retains the red color at reverse maternal hemoglobin does not resist is the mixture takes in two minutes a brown coloration.

In spite of the progress of neonatal resuscitation, Benckiser's haemorrhage is one of the rare conditions for which the fetal prognosis remains appalling with a perinatal mortality rate which varies from 75 to 100% of the cases for the singletons (Heckel et al., 1993; Régis et al., 2006; Aissi et al., 2013) this prognosis would be better for multiple pregnancies but it remains severe (Heckel et al., 1993).

The therapeutic management of this obstetric emergency must therefore not suffer any delay:

With intact membranes, caesarean section should be performed without delay (Heckel et al., 1993). Attempts vaginal delivery while keeping the membranes intact until complete dilatation are prohibited, there is always a risk of sudden decompensation.

With broken membrane,if the fetus is alive, the caesarean section must be performed urgently. If the fetus is deceased, the vaginal delivery is the rule in the absence of obstetrical contraindication. In our observation, the rapidity of cesarean delivery coupled with intensive neonatal resuscitation was at the origin of a favorable outcome of the fetus.

3 Conclusions

Benckiser Haemorrhage is a rare condition. Its clinical symptomatology is nonspecific and its diagnosis is often made only when examining the delivery. Despite the progress of neonatal resuscitation, the fetal prognosis remains formidable.

Authors’ contributions

R.H: Editing and supervision, read and approved the final manuscript; D.A: participated in the drafting of the observation, read and approved the final manuscript; B.N: participated in the drafting of the discussion, read and approved the final manuscript; A.A: checking references.

Acknowledgements

We thank the pediatric department of Ibn El Jazzar Hospital, Kairouan.

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