Spontaneous hemoperitoneum in pregnancy due to rupture of uterine vessels in woman with endometriosis: a case report

The current study represents a clear case of spontaneous hemoperitoneum in pregnancy (SHiP) as direct consequence of spontaneous rupture of uterine vessels due to endometriotic lesions in an otherwise uncomplicated pregnancy. Endometriosis has increased in recent decades and is frequently associated with infertility, pelvic pain, and dysmenorrhea. Endometriosis is a very complex condition that could impact sexuality, quality of life and psychology of affected woman. Although these aspects could not be correlated to the severity of disease, they have an important role on psychological wellbeing and interpersonal relationships [6] [7]. Endometriosis can be correlated with pregnancy complications, such as severe preeclampsia, placental abruption, placental abnormalities, premature rupture of membranes, preterm birth, and retained placenta [8] [9]. SHiP is a rare and potentially life-threatening condition that occurs in pregnant women out-of-labor in 61% of cases, of which 39% happened between 33-37 weeks of gestation [10] [11]. According to the International Network of Obstetric Survey Systems (INOSS), SHiP is defined as a non- traumatic intraperitoneal hemorrhage during pregnancy up to 42 days postpartum, excluding ectopic pregnancy, uterine rupture and cesarean section-associated bleeding [12]. In literature, trends regarding parity, age, and length of gestation in patients with SHiP have not been documented [1]. In a review of 25 cases of SHiP, endometriosis has been recognized as the major risk factor and the spontaneous rupture of uterine vessels or direct bleeding of endometriotic lesions were the most common findings . Moreover, in a recent systematic review by Lier et al., the authors reported that the SHiP was associated with rupturing utero-ovarian vessels in 57% of cases, endometriotic implants in 23% of cases, hemorrhagic nodules in 2% of cases, and a combination of these events in 20% of cases [13]. Furthermore, in almost half of the patients reported in the literature, the diagnosis of endometriosis was misunderstood until the laparotomic visualization of endometriotic lesions and the histological confirmation [10]. Conversely, in our case, the diagnosis of endometriosis was already known at the time of clinical presentation, and the histopathologic examination of the samples confirmed the presence of decidualized ectopic endometrial tissue. It is well known that the phenomenon of decidualization during the first trimester of pregnancy consists of the loss of pigmentation and fibrosis of endometriotic implants [10]. Recently, it has been supposed that the SHiP is linked to an involution of the decidualization process due to the decrease of progesterone levels and a supposed progesterone resistance. This mechanism causes the production of chemokine, proinflammatory cytokine, metalloproteinases, apoptotic factors, cell death, and bleeding [10]. The incidence of SHiP may be influenced by the use of assisted reproductive techniques (ART), as women with endometriosis could overcome subfertility/infertility problems [14]. The use of ART is linked to a high dosage of progesterone, which can facilitate the process of decidualization. In a recent review of 362 pregnancies reported from 2010 to 2018, Benaglia et al. documented that the Manuscript accepted for publication 4 frequency of SHiP in women with endometriosis submitted In vitro fertilization is 0.3% [15]. However, in our case, the patient had a spontaneous pregnancy. Besides endometriosis and ART as risk factors, a recent prospective population-based study reported some additional factors associated with SHiP, such as multiple pregnancies, ≥35 years of age in mothers, and previous abdominal surgery [11]. Although the recent evidence, the etiopathogenesis of this condition remains unclear. Increased venous pressure in utero-ovarian circulation due to pregnancy status or muscular activity such as defecation and coughing could be possibly implicated in the pathophysiology of SHiP [16]. In the literature, three factors have been described as explanations for spontaneous rupture of uterine vessels: vessels leakage caused by endometriosis-linked chronic inflammation; adhesions between vessels with relative tensions; decidualization of endometrial foci [5] [17] [18]. Our patient had either a diagnosis of deep infiltrating endometriosis or pelvic adhesions. Indeed, during the surgery, adhesiolysis was performed. Moreover, in a few cases, the origin of the bleeding remains unknown, even during laparotomy. During the surgery of our patient, arteries and superficial veins of the posterior surface of the uterus and right parametria have been involved in the bleeding. The surgical visualization of the endometriotic implants’ bleeding and the medical history of our patient suggest that the phenomenon of decidualization of endometriotic foci lead to massive and sudden hemoperitoneum in our patient. In all cases of spontaneous hemoperitoneum in pregnancy, the onset symptoms were acute or subacute abdominal pain, free abdominal fluid, hypovolemic shock, and decreasing values of hemoglobin [10]. A prompt differential diagnosis is a crucial requirement. Placental abruption, uterine rupture, placenta percreta, appendix, hepatic, and splenic ruptures are the most common preoperative misdiagnosis. Vascular sources of hemoperitoneum in pregnancy should be considered as a result of the rupture of a visceral abdominal artery aneurysm such as splenic artery [1]. Our patient’s symptoms were similar to clinical presentation described in the literature. Lier et al. reported the most common signs of presentation of SHiP: subacute abdominal pain (94.9%), a decreased level of hemoglobin (62.7%), imaging showing free peritoneal fluid (62.7%) [13]. The sensibility of contrast enhanced computed tomography in the identification of bleeding is documented, but maternal and fetal potential risks from ionizing radiation have to be considered. Ultrasonography could be helpful for the detection and monitoring of abdominal free fluid, but the real diagnosis is often obtained only by laparotomic exploration. In a preterm pregnancy, the decision making should be balanced between risks related to prematurity, delayed diagnosis, and maternal complications. In our case, the worsening symptoms and the hemoglobin drop level guided our decision on laparotomy. Several questions remain unknown about the management of endometriosis in pregnancy: whether any medical or surgical treatment of endometriosis in the preconception period would add any benefit and prevent pregnancy complications such as SHiP; whether adopting any particular management in pregnancies with a previous diagnosis of endometriosis (in terms of follow-up and mode of delivery); whether to choose any specific flowchart in case of a pregnant woman with acute abdomen and free blood abdominal liquid. Yet, endometriotic lesions should be considered a possible cause of hemoperitoneum during the third trimester of pregnancy [5]. Rapid diagnosis and prompt intervention are essential to correctly manage such complicated cases.

In conclusion, our case represents a rare case of SHiP related to endometriosis confirmed by histologic examination. Moreover, a prompt diagnosis of SHiP was crucial in managing this unique Manuscript accepted for publication 5 clinical scenario without either maternal or fetal complications. Exploring the association between the diffusion of endometriosis and the severity of SHiP could be a new challenge. In consideration of the risk of spontaneous rupture of uterine vessels and SHiP, physicians should be aware that prompt diagnosis and interventions are crucial to minimize maternal and fetal/neonatal morbidity and mortality. More attention to SHiP, with a particular focus on endometriosis as a cause, would help prevent maternal and fetal adverse events. Abbreviations SHiP: Spontaneous Hemoperitoneum in Pregnancy; ART: assisted reproductive techniques. COMPLIANCE WITH ETHICAL STANDARDS Authors contribution A.M., F.F.: Conceptualization; G.Z: Writing – original draft; A.M, F.F.: Writing – review & editing. A.M., G.Z., G.P ., A.M. F.F.: Data curation, Validation. Funding None. Study registration Not applicable. Disclosure of Interests The authors declare that they have no conflict of interests. Ethical Approval Not applicable. Informed consent Written informed consent was obtained from the patient for publication of this case report and accompanying images. Data sharing The data that support the findings of this study are available from the corresponding author upon reasonable request.

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