Clinical presentation and maternal-fetal outcomes of mirror syndrome: A case series of 10 affected pregnancies

Background Mirror Syndrome, also known as Ballantyne syndrome, is a rare condition with fewer than 120 cases described in the literature. A simultaneous edematous state of the mother, fetus and placenta is pathognomonic, with the maternal condition frequently presenting with signs and symptoms similar to that of preeclampsia. Objective Our aim was to add to the international body of literature through identification of all cases of Mirror Syndrome at two Canadian tertiary obstetric centres and characterize the maternal presentation, laboratory findings, and perinatal outcomes. Methodology We performed a retrospective chart review of all cases of fetal hydrops from two tertiary centres in Winnipeg (Manitoba, Canada) between 2000 and 2019. There were 276 cases of fetal hydrops during this period, of which 10 cases satisfied the diagnostic criteria for Mirror Syndrome where maternal and perinatal outcomes were analysed. Results The median gestational age at diagnosis with Mirror Syndrome was 23weeks and 3 days of gestation and at birth was 25 weeks and 0 days of gestation. The majority of women were multiparous (80%) and had elevated maternal body mass index (median 33 kg/m2). The most common maternal clinical findings included weight gain (100%) and hypertension (90%). The most common laboratory findings included low hematocrit (100%), hypoalbuminemia (80%), anemia (70%) and hyperuricemia (70%). Structural anomalies were observed in 50% of cases, over half of the fetuses were stillborn (66.7%) and one quarter of pregnancies resulted in neonatal deaths (25%). The median time until maternal improvement of Mirror Syndrome was 2 days postpartum. Conclusion Mirror Syndrome affected 3.6% of all cases of fetal hydrops in our cohort, and showed associations with multiparity, elevated BMI, hemodilution, hypoalbuminemia, anemia and hyperuricemia. Delivery is frequently required for fetal and/or maternal indications and symptoms usually improved rapidly after delivery.

A very preterm infant born to mother of mirror syndrome secondary to fetomaternal hemorrhage: a case report

Background Mirror syndrome (MS) is defined as maternal edema with fetal hydrops and placental edema with different etiologies, such as rhesus isoimmunization and twin-twin transfusion syndrome. Herein, we showcased a unique MS case secondary to fetomaternal hemorrhage (FMH). Case presentation A 32-year-old gravida 2 para 0 woman diagnosed with fetal hydrops was admitted to our hospital. Maternal laboratory tests revealed anemia, slightly increased creatinine and uric acid levels, hypoproteinemia, and significantly increased alpha-fetoprotein and hemoglobin-F levels. Therefore, FMH was diagnosed initially. Two days after admission, the woman had unexpectedly progressive anasarca and started to feel chest distress, palpitations, lethargy, and oliguria, and MS was suspected. An emergency cesarean section was performed to terminate the pregnancy. The maternal clinical symptoms and laboratory tests rapidly improved after delivery. A very preterm infant with a 2080-g birthweight at 31 weeks gestation survived after emergency cesarean section, active resuscitation, emergency blood transfusion, abdominocentesis, and advanced life support. Conclusions FMH could develop into MS, providing new insights into the etiology of MS. Once MS is diagnosed, emergency cesarean section might be an alternative treatment. The very preterm infant survived with a favorable long-term outcome, and a well-trained perinatal work team is needed for such cases.

Fetal hydropsia: challenges in etiologies

Introduction: Fetal hydrops is defined as the presence of abnormal fluid collections in two or more extravascular fetal compartments and body cavities. There are about 150 different underlying causes known today potentially leading to this fetal alteration. Objective: To analyze the etiologies involved in the occurrence of cases of fetal hydrops. Methods: A systematic literature review was carried out using the MedLine, Pubmed and Scielo databases, from 2015 to 2021, using the expressions: "fetal, hydrop, etiologies." Discussion: Fetal hydrops is divided into immune and non-immune. Immune results from anemia secondary to erythroblastosis by alloimmunization, so when there is maternal exposure to fetal antigens, it generates an immune response that results in the production of antibodies. History of blood transfusions, previous births, trauma and a history of alloimmunization are characterized as risk factors. Thus, immunoprophylaxis with anti-D immunoglobulin is indicated for all RhD negative pregnant women, with RhD positive male partner, with abundant fetal maternal hemorrhage during childbirth or events with potential sensitizer in the prenatal period. Conclusion: For an effective treatment, it is essential to identify the type of fetal hydrops in the patient and then the etiology of the disease, which is quite variable in Non-Immune Fetal Hydrops.

Fatal Association of Mirror and Eisenmenger Syndrome during the COVID-19 Pandemic

Mirror syndrome (MS) or Ballantyne’s syndrome is a rare maternal condition that can be life-threatening for both mother and fetus. The condition is characterized by maternal signs and symptoms similar to those seen in preeclampsia in the setting of fetal hydrops. Despite recent advances in the field of maternal-fetal medicine, the etiopathogenesis of MS remains elusive. For patients and doctors, the COVID-19 pandemic has become an extra hurdle to overcome. The following case illustrates how patients’ non-compliance associated with mirror syndrome and SARS-CoV-2 infection led to the tragic end of a 19-year-old patient. Therefore, knowledge of the signs and symptoms of mirror syndrome should always be part of the armamentarium of every obstetrician.

Clinical characteristics and risk factors of mirror syndrome: a retrospective case-control study

Background Mirror syndrome (MS) is a rare obstetric disorder complicated with high maternal morbidity and fetal mortality. MS is often misdiagnosed or underdiagnosed due to the low incidence and lack of awareness of its diverse features. This study aimed to summarise the etiology, clinical characteristics, and risk factors of MS among mothers with fetal hydrops. Methods This retrospective case-control study included 37 pregnant women with fetal hydrops in the second and third trimesters from 58,428 deliveries performed at the Third Affiliated Hospital of Sun Yat-Sen University between January 2012 and December 2020. Cases were categorized as MS and non-MS according to the presence or absence of maternal mirroring symptoms. Binary logistic regression was performed for analysis. Results Fourteen women developed MS with an overall incidence of 0.024% (14/58,428) and 37.8% (14/37) in the fetal hydrops cases. Among the 11 MS cases with known associated etiologies, seven had alpha thalassemia major. Onset of fetal hydrops was later (27.8 vs. 23.0 weeks) and the rate of placental thickening was higher (85.7% vs. 34.8%) in the MS group than in the non-MS group (P < 0.05). Regarding maternal characteristics, the MS group had higher maternal morbidity (85.7% vs. 8.7%), more weight gain (9.0 vs. 5.5 kg), higher rates of hypertension (35.7 vs. 0%) and proteinuria (64.3% vs. 4.3%), and lower levels of hemoglobin (88 vs. 105 g/L) and serum albumin (25.8 vs. 35.0 g/L) than the non-MS group (P < 0.05). Logistic regression analysis showed that onset of fetal hydrops at ≥24 weeks and placental thickening were associated with the risk of MS among fetal hydrops cases (OR 15.83, 95% CI 1.56–160.10 and OR 8.63, 95% CI 1.29–57.72, respectively). Conclusions MS is relatively common among fetal hydrops cases in the late second and third trimesters, and alpha thalassemia major is the main etiology for fetal hydrops and also MS in this population. Complicated with high maternal morbidity, the key maternal features of MS include more weight gain, hemodilution, and hypertension. Among those with fetal hydrops, the onset time of ≥24 weeks and placental thickening are risk factors for MS.

Non-immune fetal hydrops in late pregnancу: antenatal ultrasound monitoring and pregnancy outcomes in a series of 14 cases

Background. Fetal hydrops is the accumulation of extracellular fluid in two or more fetal cavities, often in combination with subcutaneous edema. An isolated accumulation of fluid only in the abdominal, pleural, or pericardial cavities is described as ascites, pleural effusion (hydrothorax), pericardial effusion (hydropericardium). Features of the pathogenesis of non-immune hydrops fetalis (NIHF) are the follow: high hydrophilicity of fetal tissues, obstruction of the lymphatic vessels, impaired lymph return, congestive heart failure, obstruction of venous return, changes in fetal venous pressure. All these factors lead to the release of fluid from cells and tissues into the «third» spaces – the abdominal, thoracic, pericardial cavities, as well as the subcutaneous space. The oncotic pressure of fetal plasma proteins is not of great importance in the formation of the fetal circulating blood volume. Currently, all the links in the pathogenesis of hydrops fetalis syndrome with various etiological factors are not fully known. The prevalence of NIHF is unknown because it is difficult to collect relevant data; many cases of the disease are not diagnosed until intrauterine fetal death or may spontaneously resolve during the prenatal period. Currently, up to 90% of all cases of fetal dropsy are attributed to NIHF. Purpose – acquaintance of the medical community with the diagnosis and treatment of non-immune hydrops as well as the analysis of clinical features, ultrasound monitoring and perinatal outcomes of 14 cases of non-immune fetal hydrops onset in a second half of pregnancy. Materials and methods. The material for the study were publications and results of clinical trials found in the databases Scopus, Web of Science Core Collection and PubMed for the period 2009–2020 and the analytic report of the own series of 14 cases of non-immune hydrops fetalis (NIHF) of various origins. During the period of 2005–2020 under the supervision in the Ultrasound Department of Kharkiv regional hospital with regional perinatal center there were 14 pregnant women with NIHF diagnosed in a second half of pregnancy. Clinical features are described, ultrasound images and Doppler monitoring are given, perinatal / postnatal results are studied. Results and discussion. The analysis of clinical features, ultrasound monitoring and pregnancy outcomes of 14 cases of non-immune fetal hydrops developed in the second half of pregnancy in presented series was carried out. 6/14 fetuses had structural anatomical defects (lung sequestration, СDH, myasthenia gravis, megacystis and hydronephrosis, epidermolysis bullosa, meconium peritonitis, intestinal atresia). Mortality rate (including perinatal and infant losses) was as high as 9/14 cases (64.2%): 3 of antenatal, 4 of neonatal, 2 of infant death). Surgical treatment was performed on 2 newborns. 5 newborns had apparently a favorable clinical postnatal outcome. In 2 cases, spontaneous resolution with complete regression of hydrops was observed (parvovirus-B19 and idiopathic NIHF). Complete recovery of fetus (spontaneous regression of hydrops without any deterioration and pathological consequences) was observed in 1 case. Conclusions. Antenatal ultrasound monitoring of fetus with NIHF is based on the assessment of PSV CMA, ductus venous, umbilical vein, atrioventricular flow. According to the results of the study, it was revealed that the cardiovascular profile of the fetus with NIHF is disturbed earlier, and the placental profile and arterial Doppler-later. Normal umbilical artery Doppler do not exclude the possibility of an adverse outcome, including intrauterine fetal demise. Extended Doppler monitoring is essential at NIHF. All neonates with NIHF in an antenatal anamnesis require postnatal follow-up.