HELLP Syndrome with Hepatic and Thoracic Complications: Insights From a Case of Spontaneous Liver Rupture

Introduction

HELLP syndrome is a pregnancy-associated clinical syndrome primarily characterized by hemolysis, elevated liver enzymes, and thrombocytopenia [1]. Reports indicate that among women with HELLP syndrome, 39% develop spontaneous hepatic subcapsular hematomas, with 0.5–2% progressing to hepatic rupture, which is associated with maternal and fetal mortality rates of 17% and 38%, respectively [2]. The etiology and pathogenesis of hepatic subcapsular hematoma remain incompletely understood. Previous studies suggest that vascular endothelial injury serves as the primary pathophysiological mechanism, leading to vasospasm, platelet activation, and intravascular fibrin deposition. Hepatic vasospasm and fibrin deposition within the sinusoids result in hepatocellular damage, thereby elevating liver enzyme levels. Additionally, impaired sinusoidal blood flow can cause hepatic congestion and enlargement, potentially progressing to subcapsular or intrahepatic hemorrhage and subsequent hematoma formation. While hematomas can occur in either the left or right hepatic lobe, the right lobe is more frequently affected [3]. Factors such as vomiting, labor, or time delay can increase intra-abdominal pressure, potentially triggering rupture of the subcapsular hematoma into the peritoneal cavity and leading to massive hemorrhage. Due to the nonspecific clinical presentation, diagnosis is often challenging, resulting in delayed intervention. To date, no published literature has documented cases of hepatic rupture complicated by thoracic hemorrhage in pregnant women. In this report, we present a case of a 37-week pregnant woman who initially presented with epigastric discomfort, likely due to undiagnosed HELLP syndrome. Upon subsequent evaluation following symptom exacerbation, she was found to have a ruptured subcapsular hematoma in the left hepatic lobe, resulting in intraperitoneal hemorrhage and intrauterine fetal death. During treatment, thoracic hemorrhage developed; however, through multidisciplinary collaboration, aggressive resuscitation, and stepwise interventions, a favorable maternal outcome was achieved.

Case Report

This study was approved by the Ethics Committee of Bozhou Hospital of Anhui Medical University (Approval No. bzyy20250232), and the patient signed an informed consent form. The patient was a 29-year-old pregnant woman at 37 weeks of gestation (gravida 3, para 2) with a body mass index (BMI) of 29.6 kg/m2. Three days prior to admission, her blood pressure was measured at 150/90 mmHg at an outside hospital, but no further attention was given, and no repeat measurements were taken. Four hours before admission, she developed diffuse abdominal pain. She denied any history of trauma, predominantly in the right upper quadrant, prompting her to seek medical attention at a local hospital. Due to her history of cholelithiasis for over 3 years, symptomatic treatment was administered. Three hours before admission, she noticed the absence of fetal movement with no improvement in abdominal pain, leading to her transfer to our hospital. She reported no symptoms of preeclampsia, such as headache, visual disturbances, dyspnea, or edema. Emergency ultrasound at our hospital revealed fatty liver, postprandial gallbladder, gallstones, and peritoneal effusion. During the examination, fetal heart sounds were absent, and peritoneal fluid was noted, although the exact cause remained unclear, with no evidence of uterine rupture. Initial laboratory results showed hemoglobin (HGB) at 119 g/L (normal 110–150) and platelets (PLT) at 100×109/L (normal 100–300), with an admission blood pressure of 110/65 mmHg. At the time of admission, obtaining urine samples promptly for analysis was not feasible. Liver function test results were pending. The patient’s condition was complex, and a multidisciplinary consultation was immediately initiated to discuss subsequent treatment. Due to persistent right upper-quadrant pain, triple-rule-out computed tomography angiography (CTA) was performed to exclude acute coronary syndrome, pulmonary embolism, and aortic dissection. The CTA revealed no significant abnormalities in the coronary arteries, aorta, or pulmonary arteries but confirmed substantial peritoneal effusion and increased density at the edge of the left lobe of the liver (Figure 1). Repeat laboratory tests showed an HGB drop to 81 g/L, PLT at 86×109/L, normal creatinine levels, white cell count 13.16×109/L (normal 3.5–9.5), serum ammonia 38 μmol/L (normal 18–72), blood glucose 10.32 mmol/L (normal 4.10–5.90), alanine aminotransferase at 625 U/L (normal <35), lactate dehydrogenase (LDH) at 2352 U/L (normal 120–246), total bilirubin at 64.7 μmol/L (normal 3.4–17.1), fibrinogen (FIB)1.37g/L (normal 2.0–4.0), prothrombin time (PT) 17.80 s (normal 11.8–14.8 s), D-dimer 17.12 μg/ml (normal <0.5), and normal serum amylase. Given the patient’s progressive hypotension, hemorrhagic shock, and deteriorating consciousness status, coupled with a closed cervical os and rapid clinical deterioration precluding imminent vaginal delivery, the decision was made to expand the indications for cesarean delivery. Under endotracheal general anesthesia, the patient underwent an emergency exploratory laparotomy and fetal extraction through a standard midline vertical abdominal incision combined with a low transverse uterine incision. Upon entering the abdominal cavity, a large amount of dark red blood and clots (approximately 1600 mL) was observed. Blood transfusion was initiated immediately, and a stillborn fetus (2520 g) was delivered via rapid cesarean section, with complete placental expulsion and no signs of placental abruption or indentations.

After uterine suturing, the uterus and bilateral adnexa appeared intact. However, active intra-abdominal bleeding persisted, accompanied by a further blood pressure drop (60/35 mmHg). Suspecting hemorrhagic shock secondary to hepatic rupture due to HELLP syndrome, vasopressors and continued blood transfusion were administered. The surgical incision was extended cephalad around the umbilicus to the subxiphoid region after the discovery of coagulopathic bleeding originating from the upper abdominal cavity. Surgical exploration confirmed a ruptured left hepatic lobe with a 6-cm laceration, subcapsular hematoma, and active bleeding, along with patchy changes and scattered subcapsular hematomas (unruptured) in the right lobe. Given the difficulty in achieving hemostasis, the left hepatic laceration was sutured, and compression hemostasis was applied (Figure 2). Following hemostatic suturing, 3 non-suction silicone closed drains were strategically positioned in the post-hepatic space (Morison’s pouch), splenic fossa, and pelvic cavity (Douglas pouch) to establish passive drainage. The total intraoperative blood loss was approximately 3000 mL, with 50 mL of dark brown urine output via catheterization. During the surgery, the patient was transfused with 1800 mL of fresh frozen plasma (FFP), 40 units of cryoprecipitate, and 14 units of leukocyte-reduced packed red blood cells (LR-PRBCs). However, no platelet transfusions were administered due to time constraints in platelet procurement. Postoperatively, the patient was transferred to the ICU for vital signs monitoring and symptomatic treatment.

Ten hours after surgery, the abdominal drainage volume continued to increase, with 600 mL of dark bloody fluid collected over 4 hours. Repeat hemoglobin levels dropped significantly to 47 g/L. Bedside ultrasound revealed an irregular heterogeneous echo (97×50 mm) in the right hepatic lobe and fluid accumulation in the abdominopelvic cavity, suggesting persistent active bleeding. Given the patient’s critical condition and inability to tolerate repeat laparotomy, our hospital immediately initiated a multidisciplinary consultation. Emergency TAE was performed, successfully controlling the hemorrhage (Figure 3). The patient was transferred back to the ICU for continued management, including antibiotic therapy, coagulation correction, and organ support.

On postoperative day 3, the patient’s oxygen saturation remained around 91%. Bedside ultrasound revealed a massive left pleural effusion. Ultrasound-guided thoracentesis yielded non-coagulable bright red blood with the following laboratory parameters: HGB 66g/L, PLT 55×109/L, pleural fluid hematocrit 0.154 L/L versus peripheral blood hematocrit 0.206 L/L (normal 0.35–0.45), PT 20.20 s, and FIB 1.94g/L. These findings were indicative of active left thoracic hemorrhage. The exact source of bleeding remained unclear. Chest CT confirmed a massive left pleural effusion (Figure 4A), prompting placement of a closed thoracic drainage system, which drained copious amounts of dark red bloody fluid. Supportive treatment was administered accordingly.

During ICU supportive care, the patient developed postoperative high fever (body temperature 39°C) and elevated infection markers: C-reactive protein 126.50 mg/L (normal <10), procalcitonin 0.05 ng/mL (normal <0.05), and lactic acid 5.3 mmol/L (normal 0.10–2.70). Considering the history of open abdominal trauma and thoracic hemorrhage, there was a risk of multidrug-resistant bacterial infection (such as extended-spectrum beta-lactamase-producing Enterobacteriaceae, methicillin-resistant Staphylococcus aureus) or mixed infection. An empirical broad-spectrum antibiotic regimen was selected: meropenem (covering gram-negative bacteria, including Pseudomonas aeruginosa) combined with vancomycin. After 48 hours of treatment, the patient’s body temperature and inflammatory markers significantly decreased, indirectly validating the effectiveness of the regimen. Blood culture results showed no bacterial growth. Drainage fluid cultures were obtained for further evaluation. Subsequent observation showed gradual reduction in drainage volume, which was attributed to residual intra-abdominal hematoma rather than active bleeding. Throughout treatment, the patient received a total of 40 units of LR-PRBCs and 7800 ml of FFP. On postoperative day 15, with significant reduction in ventilator support parameters and stable hemodynamic monitoring meeting weaning criteria, the patient underwent multidisciplinary team evaluation. A sequential spontaneous breathing trial (SBT) was successfully completed, followed by a protocolized weaning and extubation procedure. After 21 days of postoperative care with stabilized condition, the patient was transferred to the Obstetrics Department for continued management.

During the 35th postoperative day, while under transitional care in the Obstetrics Department, the patient continued to experience intermittent fever, likely due to absorption fever. Close monitoring was maintained with serial infection marker evaluations. We implemented enhanced nutritional support and encouraged appropriate ambulation to prevent thrombosis. Serial bedside thoracic ultrasound and CT surveillance revealed significant right pleural effusion (Figure 4B) measuring approximately 80 mm in depth, necessitating right-sided closed thoracic drainage. The drained fluid appeared dark yellow in color. Antibiotic therapy was continued throughout this period, with pleural fluid cultures obtained, all returning negative results. The patient achieved progressive clinical recovery and indicators of laboratory assays, culminating in discharge on postoperative day 48 after full symptom resolution and hemodynamic stabilization. One month after discharge, she returned for a follow-up examination. All laboratory parameters had normalized, and thoracic-abdominal ultrasound and CT imaging revealed postoperative changes in the liver along with a small amount of bilateral pleural effusion. She currently reports no significant discomfort symptoms.

Discussion

HELLP syndrome complicated by hepatic subcapsular hematoma is a rare yet critical obstetric emergency. When the hematoma enlarges beyond a critical threshold, spontaneous rupture can occur, leading to intraperitoneal hemorrhage and potentially hemorrhagic shock, posing life-threatening risks to both mother and fetus [4]. This condition typically manifests during the third trimester or within several days postpartum, with a predilection for the right hepatic lobe. Clinical outcomes are closely related to both the hematoma location and timeliness of intervention [5]. The clinical presentation of hepatic subcapsular hematoma and subsequent rupture is often nonspecific, potentially including right upper-quadrant pain, nausea, vomiting, hemoperitoneum, shock, or fetal distress. Definitive diagnosis primarily relies on intraoperative findings complemented by imaging modalities such as CT and ultrasound [6,7]. In this case, the patient presented to the Emergency Department with epigastric and hypogastric pain accompanied by cessation of fetal movement. Her medical history included cholelithiasis, and elevated blood pressure (150/90 mmHg) had been documented at an outside hospital prior to admission, though not adequately addressed. Ultrasound findings revealed fatty liver, ascites, and abnormal laboratory test indicators, leading to a high suspicion of acute fatty liver of pregnancy (AFLP). In the absence of other pregnancy-related liver dysfunction disorders, the case also met 6 criteria of the Swansea diagnostic standards [8]. However, her laboratory tests indicated elevated blood glucose and significant liver dysfunction, which are key clinical features distinguishing between AFLP and HELLP syndrome. The initial normal blood pressure readings at presentation likely reflected masking of hypertensive manifestations by subsequent hemorrhage. The definitive intraoperative diagnosis confirmed HELLP syndrome complicated by hepatic rupture and hemorrhagic shock. The differential diagnosis of HELLP syndrome is complex and requires differentiation from acute abdominal conditions such as placental abruption, uterine rupture, acute cholecystitis, and acute pancreatitis. This study shows that when a pregnant woman experiences sudden upper-abdominal pain accompanied by hypertension or thrombocytopenia, immediate evaluation of liver function, LDH, coagulation profile, and abdominal ultrasound to screen for perihepatic hematoma should be performed.

Regarding treatment strategies, conservative treatment should be used in cases where the mother and fetus are in a stable state and there is no immediate indication for termination of the pregnancy, as spontaneous absorption typically occurs within several months [9]. However, close monitoring of maternal symptoms, physical signs, and hematoma size is essential, along with avoidance of factors that can cause sudden increases in intra-abdominal pressure. For cases of ruptured hepatic subcapsular hematoma, early aggressive fluid resuscitation to prevent shock must be accompanied by effective surgical hemostasis. Treatment options depend on the extent of rupture and can include compression hemostasis, suturing, or partial hepatectomy. TAE is suitable for cases with limited bleeding surfaces or when surgical hemostasis is difficult to achieve. In refractory hepatic hemorrhage or rapidly progressing acute liver failure, liver transplantation should be considered [10,11]. In this case, the patient presented with intraperitoneal hemorrhage exceeding 1500 mL, and intraoperative exploration revealed a ruptured left hepatic lobe with active bleeding. Although surgical suturing and compression hemostasis were initially performed, increased bloody drainage postoperatively indicated persistent active bleeding. While surgical hemostasis remains the preferred approach, our patient’s hemodynamic instability and poor general condition made repeat laparotomy extremely high risk. Ultimately, TAE was successfully employed to control the active hepatic hemorrhage while minimizing surgical trauma. This case shows that for refractory hemorrhage, aggressive intervention is necessary but requires multidisciplinary collaboration and precise execution to optimize outcomes.

There have been no reported cases of spontaneous liver rupture complicated by thoracic hemorrhage due to HELLP syndrome. García et al suggested that patients with HELLP syndrome may experience spontaneous rupture of small vessels in the diaphragm or thoracic cavity due to coagulation dysfunction and vascular endothelial injury [12]. This type of bleeding is often occult in the early stages and may not be easily detected. However, as the bleeding volume increases, it gradually manifests as pleural effusion and hemorrhagic shock, similar to the postoperative thoracic hemorrhage observed in our case. During surgery, hepatic rupture with hemorrhage was identified, resulting in an intraperitoneal blood loss exceeding 4000 ml. Blood can enter the thoracic cavity through diaphragmatic lymphatic vessels or via direct penetration of the diaphragm, leading to hemothorax. Additionally, elevated intra-abdominal pressure, along with liver suturing for hemostasis and interventional hemostatic procedures, can contribute to thoracic bleeding. Therefore, it is hypothesized that the postoperative thoracic hemorrhage in this case may have resulted from a combination of multiple factors. The patient was treated promptly with thoracic closed drainage and blood transfusion, leading to gradual improvement of the thoracic hemorrhage.

This patient presented with postoperative recurrent fever and elevated infection indicators, indicating potential abdominal or thoracic infections. Postoperative pleural effusion and intra-abdominal blood accumulation may have been infection sources, necessitating close monitoring of infection markers and prompt adjustment of antibiotic therapy. While maternal recovery ultimately progressed favorably, the fetal outcome proved fatal despite maternal stabilization. This tragic fetal death may be attributed to HELLP syndrome-induced pathophysiological cascades: maternal systemic vasospasm and microthrombi formation severely compromised placental blood supply, culminating in irreversible hypoxic-ischemic injury. For such high-risk pregnancies, current guidelines recommend initiating low-dose aspirin prophylaxis at 12 weeks’ gestation, continuing through 36 weeks, or delivery after individualized bleeding risk assessment [13]. Additionally, enhanced obstetric surveillance through increased frequency of prenatal evaluations is strongly advised to optimize maternal–fetal outcomes.

Conclusions

This case demonstrates the severity and complexity of HELLP syndrome complicated by liver rupture and thoracic hemorrhage. Early recognition, timely intervention, and multidisciplinary collaboration are crucial for successful treatment. For patients with hypertensive disorders during pregnancy, enhanced prenatal monitoring and management are essential to prevent disease progression. Further research is needed to explore the pathogenesis and preventive strategies of HELLP syndrome to improve maternal and fetal outcomes.