Stephanie H. Guseh1 , Daniela A. Carusi1 , Andrea Tinelli2, 3, 4, 5 and Antonio R. Gargiulo6
Department of Obstetrics and Gynecology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
Department of Obstetrics and Gynecology, Vito Fazzi Hospital, Lecce, Italy
Laboratory of Human Physiology, The International Translational Medicine and Biomodelling Research Group, Department of Informatics and Applied Mathematics, Moscow Institute of Physics and Technology (State University), Dolgoprudny, Moscow Region, Russia
Institute of Physics and Technology (State University), Moscow, Russia
Division of Experimental Endoscopic Surgery, Imaging, Technology and Minimally Invasive Therapy, Department of Obstetrics & Gynecology, Vito Fazzi Hospital, Lecce, Italy
Center for Infertility and Reproductive Surgery and Center for Robotic Surgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
Stephanie H. Guseh
Daniela A. Carusi
Antonio R. Gargiulo (Corresponding author)
Rupture of the gravid uterus is a rare obstetrical emergency that is immediately and simultaneously life threatening to both mother and fetus. An infrequent occurrence during labor and at the end of a pregnancy, this complication is even more infrequent during the first 20 weeks of gestation (Fig. 11.1). Although the overall rate of uterine rupture has increased significantly over the last few decades – likely paralleling the rising rate of cesarean deliveries , uterine rupture early in gestation is still extremely uncommon (Fig. 11.2). Here, we review in detail the risk factors for early uterine rupture in addition to the common interventions for this rare catastrophic event.
A spontaneous posterior uterine rupture, detected by transvaginal ultrasonography in a patient at 11 weeks of pregnancy (a); the fetus inside the gestational sac, expelled with placenta in abdominal cavity (b)
An urgent laparotomy for anterior uterine rupture at 14 weeks of pregnancy; uterine rupture is highlighted under the index finger of the surgeon, in the black ring
Uterine rupture refers to complete separation of the uterine wall in the absence of direct penetration (Fig. 11.3) and is described almost exclusively in pregnancy. The gravid uterus is highly vascular, leading to significant hemorrhage when arterial branches and vascular sinuses become exposed. Rupture may occur in three contexts: labored, in which one area of the uterus separates under the contractile myometrial forces; spontaneous, referring to a tear in the uterus in the absence of regular contractions and traumatic, in which blunt force pressure disrupts the myometrium. In contrast, uterine perforation refers to an iatrogenic tear in the myometrium, caused by surgical instruments or trauma from a penetrating object (Fig. 11.4). Unlike later pregnancy, during which labor accounts for the majority of uterine ruptures, rupture in the first half of pregnancy is generally spontaneous.
A spontaneous uterine rupture, detected during a laparotomy in a patient at 19 weeks; uterine rupture refers to complete separation of the uterine wall in the absence of direct penetration
The figure shows a uterine perforation by hysterometer, in a uterus with anterior multiple fibroids; this condition can facilitate a subsequent uterine perforation (by creating an iatrogenic tear in the myometrium)
11.3 Risk Factors
Certain risk factors place women at risk of suffering an early rupture, most notably uterine anomalies (Fig. 11.5), abnormal placentation, prior uterine surgery (Fig. 11.6), ectopic pregnancies (Fig. 11.7), and uterine tissue abnormalities – such as those following radiation exposure. Particularly when combined, the above features in a patient’s history should alert the clinician to the possibility of an early, spontaneous rupture.
The ultrasonographic transvaginal image shows a bicornuate uterus at 7 weeks of pregnancy, a risk factor for uterine rupture, especially in case of previous uterine surgery
A surgically scared uterus removed laparotomically during second trimester of pregnancy for a spontaneous rupture and massive hemoperitoneum; the photo shows the placental fragments attached to uterine walls
An ectopic pregnancy leading to a successive uterine rupture
11.4 Uterine Anomalies
Uterine rupture during a woman’s first pregnancy is an extremely rare occurrence. A rupture in the unscarred uterus, during either the first or early second trimesters, is nearly always the result of a congenital uterine anomaly . Uterine anomalies occur in up to 9.8 % of the general population, many of which are undiagnosed prior to a woman’s first attempt at conception . Such anomalies include arcuate, septate, bicornuate, didelphys, and unicornuate uteri. There have been no studies defining the impact of uterine anomalies on uterine rupture exclusively prior to 20 weeks of gestation. Even studies investigating pregnancies later in gestation have shown discrepant findings. One group found an 8 % rate of uterine rupture during labor in women with uterine anomalies, which was statistically different than the control group . A more recent cohort, however, found no difference and recorded no incidences of rupture in their study population .
There is likely a greater rate of early uterine rupture among anomalies that confine a growing pregnancy to a restricted space or have poorly developed musculature, such as bicornuate, didelphys, or unicornuate uteri . Rupture typically occurs in the late first or early second trimester in pregnancies within a communicating rudimentary horn, as the malformed organ fails to adequately expand . The incidence of such pregnancies has been reported to be approximately as common as 1/40,000 to 1/150,000 pregnancies [42, 58]. Although such pregnancies are prone to rupture and life-threatening hemorrhage, early identification and intervention has led to a marked reduction in mortality – from 23 % around 1900 to less than 0.5 % in the year 2000 .
Case reports of spontaneous uterine rupture secondary to implantation in a bicornuate or unicornuate uterus have ranged in timing from 9 to 20 weeks. All of these ruptures were treated with emergent laparotomy secondary to acute abdominal pain and evidence of active hemorrhage [10, 23, 28]. Of note, one additional report of rupture of a pregnancy within a noncommunicating uterine horn was described in the setting of an 18-week induction-termination with misoprostol .
11.5 Abnormal Placentation
In addition to uterine anomalies that restrict a growing pregnancy, the presence of abnormal placentation in normal and abnormal uteri represents another large group of women who are at risk of early uterine rupture [3, 15, 30, 38, 47, 50, 62].
Abnormally adherent and invasive placentas are believed to result from a defect in the uterine decidua basalis and can only formally be diagnosed on pathologic review .
Morbidly adherent placentation may be classified as one of three subtypes: placenta accreta, in which chorionic villi attach directly to the myometrium; placenta increta, in which there is partial invasion of the myometrium; or placenta percreta, in which there is complete penetration of chorionic villi through the uterine wall .
Although major risk factors for placenta accreta include the presence of a placenta previa or history of prior uterine surgery (including manual extractions of the placenta), accretas have been described without these risk factors. The incidence of placenta accreta is approximately 1 in 500, and this number has been steadily rising, likely secondary to the increasing rate of cesarean deliveries [40, 65].
Although increasing efforts are being directed at early recognition of placenta accreta through advances in sonography, several reports in the literature describe the event of uterine rupture as the index sign of abnormal placentation as early as 14 weeks .
Ruptures have been most often described in the setting of placenta percreta, with complete placental invasion through the uterine wall or a prior scar. Because progressive placental invasion into and through the myometrium is a painless process, patients may not present until the time of a uterine rupture, when intraperitoneal bleeding results in abdominal pain or signs of shock [3, 47]. Such early uterine ruptures, especially in patients without known risk factors for placenta accreta, can result in sudden morbidity and mortality .
11.6 Uterine Anomalies and Abnormal Placentation
Although there are selected cases of uterine rupture in the setting of a uterine anomaly and an otherwise normally developing pregnancy, there are many more reported instances of uterine rupture secondary to abnormal placentation within a malformed uterus [4, 44, 58]. These cases of rupture similarly presented between 14 and 16 weeks of gestational age with severe abdominal pain and evolving shock and were found to have a ruptured rudimentary horn at the time of laparotomy [4, 44, 58].
The first case of placenta accreta described in a rudimentary horn pregnancy was in 1983 . Since that time, other case series have been published highlighting the increased incidence of placenta accreta within rudimentary horns and their ensuing tendency to cause preterm, often previable, deliveries. One review identified 8 cases of placenta accreta among a total of 97 rudimentary horn pregnancies, all of which were delivered preterm and 7 of which suffered spontaneous rupture . One proposed theory behind the higher incidence of abnormally adherent placentation among pregnancies within uterine anomalies is the scant decidualization within underdeveloped horns .
11.7 Prior Uterine Surgery
Perhaps the most widely known risk factor for uterine rupture is a history of prior uterine surgeries, including cesarean section, myomectomy, cornual resection, and metroplasty [21, 37]. However, these are most often reported as labored ruptures in the second half of pregnancy.
Spontaneous rupture of a prior uterine scar has also been reported, particularly when a transmural incision was used outside of the lower uterine segment [19, 46]. Again, these more often occur in the second half of pregnancy, when the uterus is more distended. While labor technically does not occur until the second half of pregnancy, early uterine ruptures have been described during medical terminations of pregnancy (Fig. 11.8), during which contractions are induced with uterotonic agents, such as oxytocin or prostaglandins [5, 7].
The image shows an early uterine ruptures during medical terminations of pregnancy by oxytocin stimulation: surgeons performed an urgent laparotomy for complete placental-fetal expulsion in abdomen for uterine rupture at 18 weeks
Prior uterine surgery appears to be a significant risk factor for uterine rupture in this clinical situation, though is it not considered an absolute contraindication [5, 20].
11.8 Prior Uterine Surgery and Abnormal Placentation
One additional group of patients at risk of early uterine rupture includes women with a combination of prior uterine surgery and abnormal placentation. This cohort of women comprises a majority of case reports that exist in the literature regarding rupture during the first and early second trimesters. Case reports of such events include ruptures ranging from 9 to 20 weeks of gestation, most of which present with acute abdominal pain and progressive signs of hypovolemic shock [9, 16, 22, 25, 49, 61].
Early uterine ruptures have been described both in women with abnormal placentation at the site of a prior low transverse hysterotomy, prior classical incision, or prior hysterotomy for a myomectomy (Fig. 11.9) [13, 56]. Such cases include cesarean scar implantations, in which embryos implant directly into the uterine wall in a small dehiscence at the site of a previous cesarean delivery . Speculation remains as to whether some cases of spontaneous rupture in early pregnancy at the site of a prior hysterotomy might, in fact, represent undiagnosed cesarean scar implantations . Although most case reports describe placenta accreta at the site of prior cesarean sections (Fig. 11.10), there are also case reports of rupture in the setting of abnormal placentation following uterine curettage alone .
A case of spontaneous rupture in early pregnancy at the site of a prior hysterotomy for myomectomy
A placenta accreta at the site of prior cesarean sections could be a possible risk factor for uterine rupture
Unfortunately, given the small number of patients with uterine rupture at early gestational ages, no quantitative studies exist to define the absolute risk of early uterine rupture when accreta is observed within a uterine scar. Similarly, the relative risks of early rupture based on hysterotomy location and early accreta diagnosis have not been determined. The concern for rupture is highest when the pregnancy is located eccentrically in the uterine scar as opposed to low, but centrally located in the uterine cavity. The close proximity to the uterine serosa raises the potential for extension outside of the uterus and rupture of the scar.
11.9 Ectopic Pregnancies
Similar to the pathology behind pregnancies implanted in uterine anomalies or those with abnormally adherent placentas, some ectopic pregnancies are also at risk of early uterine rupture given their atypical implantation. Ectopic pregnancies overall are increasing recently due to the rising rates of pelvic inflammatory disease, cesarean sections, and assisted reproductive technologies [32, 67].
Locations of ectopic pregnancies that might lead to a uterine rupture prior to 20 weeks include implantations within the cervix (Fig. 11.11), the cornua (Fig. 11.12), the interstitial portion of the fallopian tube (Fig. 11.13), and within a defective scar from a previous cesarean section (11.14, 11.15, and 11.16) [35, 55, 63, 67].
A cervical pregnancy with the uterus removed by hysterectomy (b), with the ruptured cervix in the white ring
The transvaginal ultrasonographic image of a cornual pregnancy
Laparoscopic image of ectopic pregnancy located in the interstitial portion of the fallopian tube, with initial hemoperitoneum
A cesarean implantation growing into the uterine cavity, in a first trimester gestation within a cesarean scar
A pregnancy growth into the cervix, in a first trimester gestation within a cesarean scar
A dehiscence encountered at the time of hysterectomy, in a first trimester gestation within a cesarean scar
Diagnosis of these pregnancies often requires a high index of suspicion in addition to expert imaging, ideally including transvaginal sonography (Fig. 11.17). It is also important to remember the possibility of heterotopic pregnancies, in which one pregnancy is implanted within the uterine cavity and another is implanted ectopically .
A transvaginal ultrasonographic scan showing a cervical pregnancy at 7 weeks
Early uterine ruptures have been described both in known and in previously undiagnosed ectopic pregnancies [55, 63]. Known ectopic pregnancies are occasionally managed conservatively, either because they are thought to be resolving or because the diagnosis is yet uncertain and there is hope for an ongoing, viable pregnancy [2, 57].
Even with close monitoring, these pregnancies are at significant risk of unexpected rupture . Cesarean scar implantations have been managed expectantly with the hope that the pregnancy will grow within the uterine cavity; however, ruptures have been reported also in this scenario .
11.10 Tissue Abnormalities
There are a few case reports detailing spontaneous rupture of abnormal myometrium prior to 20 weeks. For example, a patient with Turner syndrome is reported to have experienced a spontaneous rupture at 14 weeks in the setting of an IVF pregnancy, with a history notable only for a hysteroscopic polyp resection . Interestingly, patients with Turner syndrome are known to be at risk of having hypoplastic uteri, and thus, it has been suggested that they be treated with higher doses of estrogen for endometrial preparation during IVF cycles . Even still, these pregnancies should be followed very closely and with a high index of suspicion for uterine pathology and ensuing sequelae.
There is also one case report of a posterior-fundal rupture secondary to placenta percreta in a patient with a remote history of childhood chronic myeloid leukemia, who had received whole body irradiation as part of her cancer treatment . The etiology of this rupture was thought to be caused by classic radiation-induced injury including delayed scarring and fibrosis as well as endometrial atrophy of the uterus and its blood supply .
Given that early spontaneous uterine rupture prior to 20 weeks is an acute process, most patients described in the literature present in a dramatic fashion – with severe abdominal pain, intra-abdominal bleeding, and often impending signs of hypovolemic shock. In these settings, the most common management strategy is an urgent laparotomy and often hysterectomy (Fig. 11.18). As techniques in minimally invasive surgery become more advanced, however, an increasing number of case reports describe the use of laparoscopy (Fig. 11.19) for diagnosis and treatment at the time of an early uterine rupture [26, 36, 45, 59].
An hysterectomy for a uterine rupture at 15 weeks with patient hypovolemic shocked for massive hemorrhage
Laparoscopic treatment of an ectopic pregnancy into the left interstitial portion of the fallopian tube
Depending on the findings at the time of exploratory surgery, a decision can be made regarding definitive surgery with hysterectomy versus an attempt at conservative management, even in laparoscopy. Hemodynamic stability and, if known, patients’ desire for future fertility might influence the decision at the time of surgery. Intraoperative strategies range from oversewing the uterine defect [1, 8], to removing the pregnancy alone [60, 68], to resecting a noncommunicating uterine horn [10, 59], to subtotal or total hysterectomy (Fig. 11.20) . Interestingly, there have been reports of heterotopic pregnancies in which an ectopic pregnancy leading to uterine rupture is successfully removed followed by successful conservation of a viable, intrauterine pregnancy . Guidelines and outcome statistics exist for operative management of late uterine rupture and in cases of abnormal placentation [17, 61], however, early uterine rupture remains too rare for an expert opinion, and its management relies on the clinical judgment of individual care providers. It is not known whether a patient who experiences early uterine rupture can safely carry a future pregnancy – a factor that should be taken into account when considering conservative management.
Hysterectomy in 45-year-old women with interstitial pregnancy and fibrotic uterus
Given the acuity and morbidity of a uterine rupture, any opportunity to intervene prior to the rupture should be considered and discussed with the patient. Unfortunately, intervention usually requires pregnancy termination at these early gestational ages, which may be a difficult decision for patients and their families.
In general, women diagnosed with a cesarean scar or rudimentary horn implantation in the first trimester are offered termination of pregnancy. This avoids the risks of extrauterine pregnancy extension and uterine rupture and may allow for successful medical management or treatment with minimally invasive surgery [12, 31, 48]. Expectant management of cesarean scar implantations has been linked to both uterine rupture and live births, albeit with a high risk of hysterectomy for placenta accreta (Rotas 2006; ). Criteria have yet to be established that will distinguish which of these patients may carry their pregnancies to viability and which are high risk for early rupture. Patients opting for expectant management should be monitored with ultrasound, and termination should be reconsidered if the pregnancy begins to extrude outside of the normal uterine cavity (Fig. 11.21).
The image shows the pregnancy extruding outside of the normal uterine cavity
Because implantations within rudimentary uterine horns increase the risk of rupture, some advocate for routine. Removal of such pregnancies has recently been reported using a minimally-invasive approach . However, it is not clear that any other management of uterine anomalies, such as metroplasty for a septate uterus, will improve patient outcomes.
Uterine rupture during the first half of pregnancy is one of the most morbid complications that the gravid woman may face. Although a number of individual reports have been published in the literature, there remains a paucity of data and experience from which to define and quantify risks or predict outcomes. The sudden morbidity and mortality associated with early uterine ruptures demands that this diagnosis remain high in the differential when a woman presents with acute onset abdominal pain, a positive pregnancy test, and signs or symptoms of intra-abdominal bleeding, as prompt recognition and surgical treatment of early uterine ruptures is critical (Fig. 11.22). Early pregnancy termination should be considered when an early pregnancy is identified within a rudimentary uterine horn or is eccentrically located within a prior cesarean section scar or uterine cornua.
A urgent laparotomy for second trimester uterine rupture and hemoperitoneum: the rupture is on the uterine anterior body
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