Abstract
A 39-year-old G5P3A1 woman at 30 weeks of gestation with a twin pregnancy presented to the hospital with abdominal and epigastric pain. Her pain was initially attributed to uterine contractions. Following the diagnosis of intrauterine fetal death (IUFD), she was admitted to the labor and delivery unit for induction, and the twins were delivered with Apgar scores of 0. The patient's hemoglobin level dropped to 7 g/dL, prompting the administration of packed red blood cell (pRBC) transfusions, which appropriately improved her hemoglobin. She was discharged five days later with stable vital signs.
However, due to a rapid deterioration in her clinical condition on the day of discharge, she presented to another referral hospital. Based on severe hypotension, a hemoglobin level of 7.2 g/dL, and an ultrasound reporting abundant free intra-abdominal fluid, the patient was immediately transferred to the operating room.
Approximately 4.5 liters of blood and clots were evacuated from the abdominal cavity, and a splenectomy was performed following the diagnosis of a ruptured splenic artery aneurysm (SAA). Due to her deteriorating general condition and recurrent bleeding from the surgical site, she was taken back to the operating room the following day, where an additional 2 liters of blood were evacuated. Ultimately, despite aggressive resuscitation efforts, the patient developed bradycardia, became unresponsive, and expired in the intensive care unit (ICU) secondary to recurrent hemorrhage from the surgical site.
Research Insight
· Ruptured splenic artery aneurysm (SAA) must be considered a primary differential diagnosis in pregnant patients presenting with acute abdominal pain, given its life-threatening nature.
· Prompt diagnosis and immediate therapeutic intervention are pivotal in mitigating the high risk of maternal and fetal mortality associated with this condition.
· Successful outcomes necessitate a highly coordinated, multidisciplinary approach involving obstetricians, general and vascular surgeons, anesthesiologists, and intensive care specialists.
· Because the clinical manifestations of SAA rupture are often nonspecific and can mimic more common causes of acute abdomen in pregnancy, clinicians must maintain a high index of clinical suspicion to avoid catastrophic diagnostic delays.
Extended Abstract
Background
Splenic Artery Aneurysm (SAA) is defined as a pathological dilation of the splenic artery exceeding 1 cm in diameter. Although rare, it is frequently discovered incidentally on ultrasound or during an autopsy. This condition is more prevalent among women, particularly multiparous women. The prevalence of SAA in women of reproductive age is reported to be less than 0.1%; however, because 95% of cases are asymptomatic, its true prevalence remains unknown. The most dangerous complication of SAA is rupture, which can lead to maternal (75%) and fetal mortality (95%). Two-thirds of these ruptures occur in pregnant women during the third trimester. The diverse anatomical and physiological changes that occur during pregnancy can complicate the diagnosis, evaluation, and management of these patients.
This article reports a rare case of ruptured SAA in a pregnant woman in her third trimester, which resulted in the death of the mother and her twin fetuses.
Case Report
A 39-year-old woman (G5P3A1) at 30 weeks of gestation with a twin pregnancy presented to the hospital in February 2022 complaining of severe abdominal pain, nausea, lethargy, and diaphoresis. Upon initial examination, her blood pressure was 64/42 mmHg, heart rate was 42 beats per minute, respiratory rate was 14 breaths per minute, and oxygen saturation was 98%. Her abdomen was slightly distended, and no fetal heart tones were detected. No other abnormalities, abnormal discharge, or cervical dilation were observed on examination.
The patient reported a history of palpitations beginning in the fifth month of her current pregnancy, alongside a history of mitral and tricuspid regurgitation. Initial laboratory results were as follows: Creatinine: 1.33 mg/dL, Hematocrit: 24.9%, Hemoglobin: 10.1 g/dL, and HbA1c: 1.6%. No abnormalities were noted on her electrocardiogram (ECG). The abdominal pain was initially attributed to uterine contractions. Due to the confirmation of IUFD, the patient was admitted to the labor and delivery ward for induction of labor with oxytocin. The twins (one male, one female) were delivered with Apgar scores of 0.
Given the COVID-19 pandemic and the patient's suspicious symptoms, an infectious disease consultation was requested as per the specialist's recommendations, the patient was isolated, and a PCR test was performed, which returned positive. A chest computed tomography (CT) scan revealed atelectasis in the posterior lung base, with no evidence of COVID-19-related pulmonary involvement. Limited abdominal CT slices revealed hyperdense free abdominal fluid suggestive of hemoperitoneum, as well as a hematoma measuring 88 × 180 mm, located anterior to the stomach.
The following day, due to a drop in hemoglobin to 6.5 g/dL, a surgical consultation was obtained, and two units of pRBCs were transfused. Abdominal fluid was evaluated and noted to be clear. During her hospitalization, tachycardia with heart rates up to 120 bpm was recorded. Given her history of palpitations, she was prescribed propranolol; the consulting cardiologist suggested the tachycardia could also be secondary to the COVID-19 infection. An abdominal ultrasound demonstrated a normal liver span with a maximum diameter of 158 mm, and a moderate amount of free fluid was visualized in the peritoneal cavity.
On the sixth day of hospitalization, noting the stability of her vital signs, a hemoglobin level of 11 g/dL, and an overall improved general condition, the attending physician discharged the patient. She was prescribed enoxaparin and thoroughly counseled regarding warning signs.
Upon returning home, the patient’s clinical condition rapidly deteriorated. On the same day, she was transferred to a referral hospital exhibiting signs of severe psychomotor agitation and a precipitous drop in hemoglobin. Her initial hemoglobin at the second center was 7.8 g/dL. Based on her severe agitation and clinical presentation, she was referred to the tertiary hospital in Tabriz with a working diagnosis of a possible pulmonary embolism.
Emergency investigations revealed bloody intra-abdominal fluid and a further drop in hemoglobin to 7.2 g/dL, prompting immediate transfer to the operating room. During the laparotomy, 4 liters of blood and 600 mL of clots were evacuated from the peritoneal cavity. Active bleeding was identified in the left upper quadrant (LUQ), inferior to the spleen. The primary source of hemorrhage was manually compressed. Following consultation with the senior general surgery resident, who then scrubbed into the procedure, a ruptured splenic artery aneurysm was diagnosed, and a splenectomy was performed. The splenic artery and vein were ligated, and hemostasis at the splenectomy bed was meticulously verified. Once hemostasis was assured, the abdominal wall was closed. Intraoperatively, the patient received 4 units of pRBCs, 4 units of fresh frozen plasma (FFP), and 3 units of platelets.
Postoperatively, the patient was transferred to the intensive care unit (ICU). While in the ICU, bleeding was noted from the corrugated drain site. The patient subsequently developed bradycardia, prompting the initiation of cardiopulmonary resuscitation (CPR) and chest compressions. To identify the source of the hemorrhage, she was transported back to the operating room while receiving respiratory support. Upon reopening the abdomen, 2 liters of fresh blood were suctioned from the peritoneal cavity. The abdominal cavity was explored, bleeding vessels were ligated, and the hemorrhagic bed was packed and compressed. The following day, the patient experienced recurrent hemorrhage from the surgical site and again developed bradycardia. Unfortunately, she did not respond to resuscitative measures and passed away.
Discussion
This report emphasizes that the timely diagnosis of a splenic artery aneurysm, a rare complication (0.23%), can be life-saving. The diverse clinical manifestations of an SAA make early diagnosis challenging, particularly during pregnancy. The symptoms of SAA are broad and varied, including diffuse abdominal pain (especially in the left upper quadrant), epigastric pain radiating to the left shoulder (Kehr’s sign), syncope, dyspnea, nausea and vomiting. These symptoms mimic other obstetric complications, such as preterm labor, placental abruption and preeclampsia. Fetal distress and mortality in these cases are typically secondary to maternal hypovolemia, shock and their downstream sequelae.
A high index of clinical suspicion for a ruptured SAA can be life-saving, particularly in pregnant women presenting with abdominal pain and severe hypotension. In a similar case report of a patient who presented to Bentol-Hoda Hospital in Bojnourd with sudden and severe abdominal pain at 20 weeks of gestation, the mother's life was saved via emergency surgery, although the fetus was found to be without a heartbeat 24 hours postoperatively. Despite successful maternal survival in many instances, the maternal mortality rate associated with ruptured splenic aneurysm is reported to be 21.9%, with a neonatal mortality rate of 15.6%. In another documented case, maternal death occurred at 6 weeks of gestation following a SAA rupture.
The risk of aneurysm rupturing does not typically correlate with its size. Approximately 62% of ruptures occur spontaneously. Due to the exceptionally high risk of rupture and associated mortality during pregnancy, elective surgery is recommended during pregnancy and even for asymptomatic women of reproductive age. Ninety-five percent of patients are asymptomatic. A ruptured splenic aneurysm is a devastating complication that occurs in 20-50% of cases during pregnancy. The onset is abrupt; however, in 20-25% of cases, rupture occurs in two stages. This "double-rupture" phenomenon temporarily increases the chance of survival by providing a vital window for definitive diagnosis and management. Nevertheless, if left untreated, this latent period is invariably followed by catastrophic cardiovascular collapse and intra-abdominal hemorrhage within 48 hours.
Based on her clinical course, it appears the patient in this report had a two-stage rupture. A delay in diagnosis secondary to a lack of clinical suspicion, an inadequate definitive surgical evaluation during the first admission, and the premature discharge of the patient from the first medical center can be considered the primary underlying factors contributing to this fatal outcome. When surgery for SAA is performed in the setting of hypotension, bleeding may appear to have ceased; however, hemorrhage rapidly resumes once the blood pressure is corrected and normalizes. In the presented case, due to the delayed diagnosis, the emergent nature of the surgery following the rupture, and the patient's poor hemodynamic state, this physiological phenomenon was likely underappreciated intraoperatively.
A strong degree of clinical suspicion is essential for diagnosing a ruptured splenic aneurysm. In the setting of cardiovascular collapse, aggressive volume resuscitation combined with immediate surgical intervention is imperative and improves both maternal and fetal survival rates. When presented with the clinical signs of SAA, physicians frequently prioritize more common differential diagnoses, such as uterine rupture, placental abruption, amniotic fluid embolism, or other surgical emergencies like a perforated peptic ulcer. Angiography is the gold standard for diagnosis, while imaging modalities such as CT scans and abdominal X-rays can assist in clinical decision-making. However, in pregnant women, Doppler ultrasound is considered the preferred initial imaging modality.
Conclusion
A ruptured SAA during pregnancy is a rare but acutely life-threatening complication, consistently associated with high rates of maternal and fetal morbidity and mortality. A strong clinical suspicion facilitating the early diagnosis of an SAA rupture increases the probability of survival for both the mother and the fetus. Recognizing the critical severity of this complication, if an SAA is suspected, swift action and the coordinated efforts of a multidisciplinary medical team are paramount for achieving a successful outcome.
Practical Implications of Research
A high index of clinical suspicion for the early detection of a ruptured splenic artery aneurysm significantly increases the chances of maternal and fetal survival. Given the critical and life-threatening nature of this complication, timely intervention and the coordinated efforts of a multidisciplinary team are essential for improving outcomes.