Ballantyne syndrome (BS) also called mirror syndrome is defined by the presence of a clinical triad that includes fetal hydrops and placental and maternal edema. Here we present a clinical case of a 34-year-old woman with a 29 weeks’ pregnancy, who developed BS and fetal loss probably due to failure in prompt recognition of a rapidly growing sacrococcygeal teratoma (SCT). Due to similarities in clinical presentation with preeclampsia and the importance in early identification of the source for BS, we underwent a literature review in order to identify significant signs and symptoms, as well as sonographic changes, in order to help clinicians to make this prompt recognition, identification of the cause, and early management of BS, which will have an important impact in maternal and fetal survival.
Ballantyne syndrome (BS) also called mirror syndrome or triple edema is defined by the presence of a clinical triad that includes fetal hydrops and placental and maternal edema [
It is extremely important for clinicians to identify similarities and differences between BS and preeclampsia, since diagnosis and management of BS might be possible when the source is readily identified [
A 34-year-old woman gravida 5, para 3, abortion 1 presented to the ER of our hospital, at 29 weeks’ gestation, due to uterine contractions that increased in frequency and intensity in the last 5 hours, with no other symptomatologies added (Figure
Timeline of patient’s clinical evolution with diagnostic tests and treatments. SCT: sacrococcygeal teratoma.
Single female sex without vitality spontaneously delivered, with hydrops and macerated skin, weight: 1730 g, with a grayish and hemorrhagic sacral mass, later described as a Type I sacrococcygeal teratoma (SCT).
In the present case, the diagnosis of BS was established after the findings of fetal hydrops, placental edema, and mild maternal edema. Presumably in this case, BS developed secondary to SCT. The SCT was not evident in the first ultrasound at 24 weeks’ gestation, neither were fetal hydrops; however polyhydramnios was present at that time. SCT is a rare congenital disease of the newborn that happens in 1 out of 20,000-40,000 pregnancies, predominantly in female newborns with a ratio of 3:1 [
The etiopathogenic mechanisms of BS remain partially understood; it has been suggested that the placenta is the source of the problem, since resolution of the pregnancy with the removal of the placenta resolves the mirroring edema [
In the fetus, the development of hydrops secondary to SCT can be explained by a high output failure caused by either anemia due to tumor hemorrhage and/or an arteriovenous shunt in a low resistance, rapidly growing tumor [
Furthermore, rapid growth of SCT (>150 cm3 per week) and hypervascularity are associated with increased risk in perinatal mortality [
In order to distinguish BS from preeclampsia, one important variable is time for presentation since BS can present earlier, with the first becoming evident as soon as 16 weeks’ gestation and the latter presenting after 20 weeks’ gestation [
Clinical considerations in establishing a differential diagnosis for BS.
Variable | PE | BS | Case Report |
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Maternal | |||
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Gestational age | < 20 | 16-34 | 24-29 |
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Edema | ++ | ++ | + |
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Hypertension | +++ | + | - |
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Proteinuria | +++ | + | + |
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Hyperreflexia (OTR) | + | - | - |
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Liver function test abnormalities | +++ | + | + |
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Creatinine | ++ | + | + |
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Urea | ++ | + | + |
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Creatinine:urea ratio | ++ | + | + |
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Thrombocytopenia | +++ | + | - |
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Uterine bleeding | ++ | + | - |
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Renal failure | ++ | +++ | - |
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Pulmonary edema | + | +++ | - |
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Anemia | Hemoconcentration | Hemodilution | Hemodilution |
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Placental edema | - | +++ | +++ |
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Fetal | |||
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Hydrops | + | +++ | +++ |
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Polyhydramnios | + | +++ | +++ |
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Cardiac affection | + | ++ | Unknown |
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Intrauterine growth restriction | + | - | - |
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Associated tumor | - | + | + |
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Fetal death | + | ++ | ++ |
Maternal rapid weight gain, marked skin edema, and uterine distention can also be distinguishable features in BS, whereas elevated blood pressure and proteinuria can be present in both [
Renal failure can appear in both; however, it has been described in a higher frequency for BS; postpartum hemorrhage and pulmonary edema have also been described in BS [
First-trimester uterine artery Doppler ultrasound examination, placental growth factor, and pregnancy-associated plasma protein-A help to predict pregnancy complications associated with uteroplacental insufficiency such as preeclampsia before the onset of clinical features [
Development of BS can increase the risk of preeclampsia and vice versa; we believe that there is a common ground for the development of both pathologies, a ground that favors an angiogenic-antiangiogenic imbalance that might contribute to an even worse prognosis when both entities coexist; therefore it is mandatory to alert physicians about this possibility in order to remain vigilant and prescribe early treatment when indicated.
Maternal complications of BS, such as hypertension and pulmonary edema, should be immediately treated if there is a decision to wait with continuous observation and the administration of diuretics, calcium channel blockers, and beta-blockers [
We presented a case report with BS probably following to the development of SCT after 24 weeks’ gestation, with polyhydramnios, hydrops fetalis, placental edema, and mild maternal edema. Since the most common cause for the development of hydrops fetalis is preeclampsia, we thought it could be important to identify clinical differences that could help in a prompt diagnosis since management of both entities is different, especially in BS where the source for endothelial dysfunction can be recognized prenatally. Preeclampsia and BS share some similarities in clinical presentation; even more, they can occur simultaneously, increasing the risk of maternal and fetal death. Some clinical considerations that point towards BS in the mother are time of onset (<20 weeks), edema, renal affection, and hemodilution; in the fetus ultrasound examination: polyhydramnios, cardiac affection, and fetal anomalies, including tumors. Finally, we would like to emphasize that in our case the initial manifestation was polyhydramnios; therefore, it is mandatory that patients with this abnormality are adequately observed during the following weeks’ gestation with a Doppler ultrasound or fetal MRI for the evaluation of hydrops fetalis, and, in the case of a tumor, growth rate and vascularity. Then, each case should be evaluated for either early termination of pregnancy or fetal surgical intervention, in order to decrease the risk of fetal mortality, as well as maternal morbidity and mortality associated to BS.
Informed consent was obtained from the patient; her personal data was protected always, during the elaboration and publication of the manuscript.
This article does not contain studies with human participants or animal subjects of experimentation.
The authors declare that they have no conflicts of interest.
Silvia F. Navarro-Perez, Karen Corona-Fernandez, and María G. Zavala-Cerna were responsible for gathering information; Silvia F. Navarro-Perez, Karen Corona-Fernandez, María G. Zavala-Cerna, José L. Rodriguez-Chavez, and A. Bañuelos-Franco carried out the analysis of the information and medical literature; Silvia F. Navarro-Perez, Karen Corona-Fernandez, José L. Rodriguez-Chavez, A. Bañuelos-Franco, and María G. Zavala-Cerna were responsible for draft writing and approval of the final version; María G. Zavala-Cerna was responsible for draft submission.