Spontaneous rupture of broad ligament vein in twin pregnancy: a case report and literature review

Abstract

Spontaneous uterine vein rupture in pregnancy is rare. This current case report presents a case of broad ligament vein rupture in spontaneous twin pregnancy that is even rarer. A female in her early 30s at the third trimester of a twin pregnancy presented with headache and left upper abdominal pain for 2 days. After admission, her blood pressure was 158/112 mmHg accompanied with increased blood lipids and proteinuria. Ultrasonography did not show any abnormalities. The main diagnoses included twin pregnancy with abdominal pain of unknown aetiology, preeclampsia and hyperlipidaemia. Her blood pressure and symptoms did not improve after administration of medications. An emergency caesarean section and laparotomy were then performed. Intraoperatively, bright red non-coagulated blood was observed within the pelvis and removed. After two healthy female babies were delivered, pelvic exploration revealed haematomas in the broad ligaments bilaterally and haemostatic sutures and clips were applied to successfully treat the ruptured vein. In cases of unexplained abdominal pain accompanied with fetal distress and haemodynamic instability, especially in twin pregnancy that can cause uterus over-expansion and broad ligament over-stretch, the possibility of spontaneous uterine vein rupture should be considered. Rapid diagnosis, immediate fluid replacement and prompt surgical intervention are essential for the safety of the mother and child.

Introduction

Spontaneous rupture of the uterine vein in pregnancy is rare, with an incidence of approximately 0.5 in 10 000 births. ¹ However, it is a serious condition with a prenatal mortality rate of 26.9%. ² Although the exact aetiology of spontaneous uterine vein rupture in pregnancy remains unknown, endometriosis and assisted reproductive technologies might represent risk factors in its occurrence and severity.^3,4 Spontaneous rupture of the uterine vein can occur at any time during pregnancy but it is more common in late pregnancy.^5,6 The most common site of rupture is the broad ligament. ⁶ The absence of a clear trigger and typical signs and symptoms can lead to misdiagnosis or delayed diagnosis, which may endanger both the mother and the baby's safety. This current report describes a case of broad ligament vein spontaneous rupture in twin pregnancy, which is even rarer.

Case report

In August 2023, a female patient in her early 30s at the third trimester of a spontaneous twin pregnancy (G3P0) complained of headache and left upper abdominal pain for 2 days and was admitted to the Department of Obstetrics and Gynaecology, China-Japan Friendship Hospital, Beijing, China. Her previous prenatal checkups were uneventful. Gestational diabetes mellitus was diagnosed in mid-pregnancy and her blood glucose was under control with diet and exercise. Before admission her blood pressure was 160/100 mmHg. The patient was evaluated for preeclampsia with urinary protein testing and the result showed urinary proteins 2+. Physical examination showed good fetal movements, no tightness in the chest and abdomen, no vaginal bleeding and no abnormal vaginal discharge. The patient had a history of miscarriage. After a recent miscarriage that happened 3 years ago, she was diagnosed with atypical antiphospholipid syndrome combined with hypothyroidism. Diagnosis of atypical antiphospholipid syndrome was based on previous pregnancy losses and antiphospholipid antibodies being tested positive. She was initially administered 100 mg aspirin oral once a day that tapered to 75 mg oral once a day, 3500 IU low molecular heparin subcutaneous injection once a day and 75 µg levothyroxine sodium oral once a day from the 8th week of the current gestation.

After admission, her physical examination showed the following: body temperature, 36.6 °C; pulse rate, 86 beats/min; respiration rate, 20 breaths/min; blood pressure, 158/112 mmHg; and she had oedema of both lower limbs, ++. Light pressure pain was felt in the left upper abdomen. The patient’s pregnancy-related characteristics were as follows: uterine height, 38 cm; abdominal circumference, 102 cm; fetal heart rate, 140 beats/min for one fetus and 156 beats/min for the other; contractions, irregular; fetal position, cephalic/buttock position; estimated fetal weights, one with 2600 g and the other 2800 g. An ultrasound examination did not show any significant abnormalities. The non-stress test was reactive. The patient was given 10 mg dexamethasone intramuscular injection once to promote fetal lung maturation, a loading dose of 5 g magnesium sulphate intravenous infusion followed by a continuous intravenous infusion of 1 g magnesium sulfate every hour to relieve spasms, and 100 mg labetalol oral once and 20 mg nifedipine oral once to reduce blood pressure. An emergency laboratory test showed the following: total white blood cell count, 9.7 × 10⁹/l; neutrophil percentage, 75.8%; platelet count, 203 × 10⁹/l; haemoglobin, 125 g/l; total cholesterol, 20.55 mmol/l (normal range, <5.20 mmol/l); triglycerides, 36.06 mmol/l (normal range, <1.70 mmol/l); high-density lipoprotein cholesterol 2.82 mmol/l (normal range, 1.00–2.20 mmol/l); blood amylase 111 IU/l (normal range, 35–135 IU/l); sodium, 130.8 mmol/l (normal range, 135–145 mmol/l); uric acid, 494 μmol/l (normal range, 150–420 µmol/l); alkaline phosphatase, 167 IU/l (normal range, 35–100 IU/l). All coagulation parameters were normal. Abnormalities were not found in echocardiography and ophthalmology consultation.

The initial diagnoses were as follows: (i) severe preeclampsia; (ii) abdominal pain with unknown aetiology (iii) hyperlipidaemia; (iv) acute pancreatitis (v) twin pregnancy, 35 weeks + 1 day of gestation, G3P0; (vi) gestational diabetes mellitus; (vii) hypothyroidism in pregnancy; and (viii) atypical antiphospholipid syndrome.

The medications that had been administered showed little effect with blood pressure being maintained at around 160/105 mmHg and there was no relief of the headache and left upper abdominal pain. In view of these observations, an emergency caesarean section and laparotomy were performed. Approximately 300 ml of bright red non-coagulated blood was removed from the pelvis before two live female babies were delivered, both with a postnatal Apgar score of 10. The umbilical artery blood pH was 7.25. Intraoperative exploration revealed haematomas in the broad ligaments of the uterus bilaterally, with smooth surfaces, no rupture and no obvious active bleeding. The haematoma of the anterior leaf of the right broad ligament was approximately 2 cm × 2 cm; the haematoma of the posterior leaf of the right broad ligament was approximately 2 cm × 5 cm; the haematoma of the anterior leaf of the left broad ligament was approximately 2 cm × 3 cm; and the haematoma of the posterior leaf of the left broad ligament was approximately 10 × 10 cm (Figure 1). Haemostatic sutures or clips were applied to treat the ruptured vein successfully. Total intraoperative bleeding was approximately 1500 ml and 4 units of red blood cells and 800 ml plasma were transfused.

Figure 1.

Intraoperative image of a patient in her early 30s at the third trimester of a spontaneous twin pregnancy (G3P0) who presented with a headache and left upper abdominal pain lasting for 2 days showing a haematoma of the posterior leaf of the left broad ligament. The black arrow points to the ruptured posterior leaf of the left broad ligament. The colour version of this figure is available at: http://imr.sagepub.com.

The intravenous infusion of 1 g magnesium sulfate every hour was continued and 1.5 g cefoperazone sodium/sulbactam sodium intravenous infusion twice a day combined with 0.5 g metronidazole intravenous infusion twice a day for 3 days were administered for post-procedure prophylaxis. Postoperative recovery was uneventful and the initial diagnosis of acute pancreatitis was ruled out because of mainly the disappearance of abdominal pain after operation. The patient was discharged 5 days after the surgical procedures. Both neonates developed jaundice within 24 h after birth and received phototherapy for 1 week. They both recovered and were discharged thereafter. The reporting of this study conforms to CARE guidelines. ⁷ All patient details are de-identified and patient consent to treatment was obtained.

Discussion

A search of the PubMed® database was conducted using the terms ‘spontaneous haemoperitoneum’ and ‘twin pregnancy’ without any timeline restrictions. Relevant references in the identified studies were also thoroughly reviewed. The result of this literature search demonstrated that spontaneous rupture of uterine veins is rare.^2,8–17 Uterine vein rupture can occur in the following locations: the surface of uterine fibroids, the broad ligament of the uterus, foci of endometriosis and the site of placenta accrete. Risk factors for the spontaneous rupture of uterine vessels include endometriosis, twin pregnancies, connective tissue disease and artificially assisted reproduction pregnancies.^2,8,9 A previous review summarized 25 cases of uterine vessel rupture from 20 case reports and found that 52% of patients had endometriosis and 90% of vascular ruptures occurred in the posterior wall of the uterus and parametrium. ¹⁰ The pathogenesis of uterine vein ruptures may be related to the following factors: (i) thin walls of pelvic veins with poor elasticity and absence of venous valves during pregnancy; (ii) compression of the inferior vena cava by the enlarged pregnant uterus, impeding venous reflux; (iii) slow venous blood flow accompanied with a hypercoagulable state; (iv) varicose veins; and (v) uterine vascular malformation.

A prospective population-based cohort study of nine Italian regions covering 75% of the national births found 29 cases of spontaneous haemoperitoneum in 741 516 women between November 2017 and March 2020, giving an incidence rate of 0.04 per 1000 births. ⁸ Maternal age  ≥35 years, multiple pregnancies and assisted reproductive technology were risk factors for the condition. ⁸ Of the 29 cases, 26 (89.7%) had abdominal pain and 27 (93.1%) underwent caesarean section. The median blood loss was 1900 ml (range, 400–4000 ml). ⁸ The current patient had a twin pregnancy and twin fetuses can cause excessively high tension of the uterine, increasing the risk of venous rupture. Uterus venous rupture in twin pregnancy is extremely rare with <10 cases being reported thus far in the literature.^11–17

The current patient had severe preeclampsia, poor blood pressure control and hyperlipidaemia. These factors may contribute to vein rupture by damaging the endothelium and reducing venous blood flow. Moreover, she was pregnant with twin fetuses requiring more uterine blood flow, which can be associated with broad ligament veins rupture. Twin fetuses cause over-expansion of the uterus and over-stretching of the broad ligaments, which may lead to vein rupture. The patient in the present case report had high blood pressure and proteinuria on examination, which could easily make the clinician focus on these seemingly more serious clinical manifestations and ignore the minor abdominal and lower back pain. Fortunately, this patient underwent emergency surgery and spontaneous rupture of the broad ligamentous veins was detected intraoperatively, leading to timely management and safety of the mother and her two babies.

Antiphospholipid syndrome, a rare disease characterized by the occurrence of arterial or venous thrombotic events, is associated with an increased risk of preeclampsia. ¹⁸ However, the link between atypical antiphospholipid syndrome and preeclampsia in the current patient remains undetermined. Anticoagulation and antiplatelet therapies are recommended for the management of antiphospholipid syndrome. ¹⁹ The current patient received aspirin and low molecular heparin, which together increases bleeding risk and might may have contributed to the haemorrhage observed in the patient.

Spontaneous rupture of the uterine vein during pregnancy should be differentiated from other acute abdominal conditions, such as placental abruption, uterine rupture, placenta accrete and rupture of abdominal organs. When a patient presents with unexplained abdominal or lower back pain accompanied by haemodynamic abnormalities such as decreased blood pressure and increased heart rate, an urgent blood test, ultrasound exam, pelvic computed tomography scan or pelvic magnetic resonance imaging should be performed to assist the differential diagnosis. A previous review of 25 cases of spontaneous peritoneal haemorrhage demonstrated that bleeding was not diagnosed on abdominal ultrasonography in all cases. ¹⁰ This may be related to the low amount of bleeding over a short period of time. Once an abdominopelvic effusion is identified, rapid rehydration along with laparotomy may be considered. If non-clotting blood is detected, a prompt caesarean section should be performed and the site of bleeding be identified and sutured. While most cases of spontaneous uterine vein rupture in the third trimester have been treated with surgical intervention, ⁸ management of uterine vein rupture in early or mid-pregnancy is poorly documented. Regardless of the gestation time when uterine vein rupture occurs, in our opinion the decision to treat the patient conservatively or surgically should depend on the patient's haemodynamic stability.

In conclusion, in cases of unexplained abdominal pain accompanied with fetal distress and hemodynamic instability especially in twin pregnancy that can cause uterus over-expansion and broad ligament over-stretch, the possibility of spontaneous uterine vein rupture should be considered. Rapid diagnosis, immediate fluid replacement and prompt surgical intervention are essential for the safety of the mother and child.