Ballantyne Syndrome in Rhesus Isoimmunised Pregnancy

Introduction

Ballantyne syndrome or Mirror syndrome or triple edema syndrome is a rare and dangerous disorder affecting pregnant women. It describes unusual association of fetal and placental hydrops with maternal edema. It was first described in 1882 by John William Ballantyne. Awareness of the syndrome is important due to associated fetal and maternal risks [1]. We present a case of Ballantyne syndrome in Rhesus isoimmunised pregnancy with severe fetal hydrops.

Case Report

A 28 year old woman, Gravida 4, Para 3 Living 2 was referred from a peripheral hospital at 35 weeks of gestation with diagnosis of Rhesus (D) isoimmunised pregnancy. She had three full term vaginal deliveries with delivery of hydropic fetus in her last delivery two years back. Her blood group was AB negative. There was no history of taking anti D immunoglobulin in any of the pregnancies. Ultrasound examination at the time of admission revealed marked fetal hydrops with ascites (Fig. 1) and pleural and pericardial effusion with cardiomegaly (Fig. 2). Indirect Coombs’ test titer was 1:256. Cordocentesis and intra uterine transfusion (IUT) was performed on the day of admission. Cordocentesis revealed haemoglobin of 2.9 g/dl and hematocrit of 7.8%. Post transfusion haemoglobin and hematocrit were 8.1 g/dl and 24.6% respectively. Second IUT was carried out on day four of admission with post transfusion haemoglobin of 11.2 g/dl. On day two of admission she developed marked edema over face, lower limbs and anterior abdominal wall together with slight rise in blood pressure (140/90 mm Hg). All her biochemical parameters were normal. About 12 hours after second IUT she went into spontaneous labour. Emergency caesarean section was performed for associated ascites, pleural and pericardial effusion. Maternal ascites was noted during caesarean section. Hydropic male fetus weighing 3010 gm was delivered (Fig. 3). Placenta weighed 2915 g and was pale and edematous with fluid oozing from it. New born was put on ventilator support, but couldn't maintain oxygen saturation and died on day two of life.

Fig. 1.

Fetal hygiene with ascites.

Fig. 2.

Pleural and pericardial effusions with cardiomegaly.

Fig. 3.

Hydrophic fetus.

Discussion

Ballantyne syndrome or mirror syndrome or triple edema syndrome is a rare complication seen in pregnancy. It was first described in association with severe foeto-placental hydrops caused by rhesus immunization. The name ‘Mirror syndrome’ refers to similarity between maternal edema and foeto-placental hydrops. Ballantyne syndrome has several characteristics; edema is always a key feature, albuminuria usually mild and preeclampsia unusual. The exact pathophysiology of this rare disorder remains unclear. This syndrome may also be associated with a variety of obstetrical problems that may range from fetal hydrops caused by fetal congenital malformations [2, 3, 4], fetal infection e.g. by maternal-fetal parvovirus infection and metabolic disorders. Some authors have suggested placental hypoxia as the main causative factor for this syndrome [5].

This serious complication occurs only if the placenta is grossly edematous as was seen in our case, while it is rare if the fluid accumulation is limited to the fetal body. It may be difficult to distinguish between Ballantyne syndrome and preeclampsia. Platelet count, aspartate transaminase, alanine transaminase and haptoglobin are usually not affected in this syndrome and this may be used to distinguish it from the hemolytic anemia, elevated liver enzyme and low platelet count (HELLP) syndrome. When the specific cause of fetal hydrops can be identified and corrected by in utero treatment, reversion of Ballantyne syndrome has been reported [6]. Total or partial reduction of the placental edema may be responsible for this reversion. When specific cause can not be identified or corrected, immediate delivery is necessary in order to avoid fetal death and maternal complications.

Conflicts of Interest

None identified