An unusual form of mirror syndrome: a case report

The Journal of Maternal-Fetal and Neonatal Medicine, 2012; Early Online: 1–3 © 2012 Informa UK, Ltd. ISSN 1476-7058 print/ISSN 1476-4954 online DOI: 10.3109/14767058.2012.722734

SHORT REPORT

An unusual form of mirror syndrome: a case report Annamaria Giacobbe, Roberta Grasso, Maria Lieta Interdonato, Antonio Simone Laganà, Giacobbe Valentina, Onofrio Triolo & Alfredo Mancuso

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Department of Gynecological, Obstetrical Sciences and Reproductive Medicine, University Hospital, Messina, Italy We describe a case report of massive vulvar edema in an 18-year-old primigravida at 38 gestational weeks associated with placentomegaly and hydramnios.

Aim: Mirror syndrome is a triad consisting of fetal hydrops, maternal edema and placentomegaly. Its pathogenesis is unclear and it is frequently mistaken for preeclampsia, even though distinguishing features can be identified. It is associated with an increase in fetal mortality and maternal morbility. Methods: We report an uncommon case of mirror syndrome, which appeared late in pregnancy (38 weeks) in a young nulliparous and characterized by sudden and massive vulvar edema, with placentomegaly and hydramnios but without fetal hydrops. Results: Our report is an interesting example of an unusual form of Mirror syndrome for several reasons. First of all, the gestational age in which the disorder appeared differs remarkably from the data of literature; in our case, clinical signs and symptoms appeared only at 37 weeks. Another difference consists in the lack of hypertension that represents the second most common symptom associated and explains the difficulty to differentiate this syndrome from preeclampsia. Conclusions: Although mirror syndrome is associated with an increase in perinatal mortality, in the case we reported the late onset of the disorder associated with the medical treatment and the timely decision to perform a caesarean section allowed the birth of a healthy baby.

Case report An 18-year-old woman, gravida 1, para 0, group B rhesus-positive, was referred at 37 weeks of gestation to our Department because of a massive vulvar edema (Figure 1). On admission, physical examination revealed a fairly good general state, normal blood pressure (120/80 mmHg), weight of 54 Kg, height of 156 cm and body temperature of 36.4°C. Abdominal examination evidenced a gravid uterine fundus equivalent to gestational age, a fetus in cephalic presentation and a normal fetal heart rate. On physical examination, the pregnant woman had moderate edema of the ankles and legs while external genitalia were swollen without vaginal discharge and digital vaginal examination was difficult due to tenderness. A Foley catheter was inserted to relieve urinary retention. Maternal laboratory tests revealed mild anemia (hemoglobin 11.6 g %, hematocrit 35%), hypoalbuminaemia (2.76 g/dL) and mild proteinuria (1+ on urinalysis 350 mg/24 h). Renal (creatinine 0.7 mg/dL, uric acid 5.3 mg/dL) and hepatic function (alanine transaminase 15 U/L, aspartate transaminase 8 U/L, γ-glutamyl transpeptidase 5 U/L) were normal. Other maternal signs and symptoms such as oliguria, headache, visual disturbances and low platelets were absent. The woman and her husband were not consanguineous and were apparently healthy and there was no family history of congenital malformations. The conception of the pregnancy was spontaneous and its course was regular with a weight gain comprised in the normal range (12 kg). The pregnant woman was on no medication and she gave no history of trauma or contact exposure. There was no history of drug or alcohol use and no recent infections. TORCH serology was negative and the patient had not undergone first-trimester maternal serum screening for Down syndrome. Obstetric ultrasound showed a male fetus with biometry equivalent to the gestational age with regular development of all the examined organs. No signs of hydrops or localized edema were evidenced in fetal district while an increased amniotic fluid (AFI 27) and placentomegaly were evidenced Figure 2. In consideration of vulvar massive edema, proteinuria and placentomegaly at US examination, the diagnosis of mirror syndrome was made, albumin was administered and the use of

Keywords: Ballantyne’s syndrome, hydrops, mirror syndrome, placentomegaly, vulvar edema

Introduction John W. Ballantyne in 1892 first described a syndrome characterized by the combination of maternal edema, fetal hydrops and placentomegaly due to rhesus alloimmunization [1]. This rare disease is also known as “Mirror Syndrome” owing to the presence of maternal edema that “mirrors” the fetal and placental conditions. Mirror syndrome can be associated with both immunological and nonimmunological causes of fetal hydrops such as twin–twin transfusion syndrome, structural fetal malformations, viral infections, fetal arrythmias and placental or fetal tumors. The pathogenesis is poorly understood and this syndrome is often related to preeclampsia because of clinical signs common to both disorders. Like preeclampsia, it is characterized by adverse perinatal outcome with poor fetal prognosis and increased maternal morbidity [2]. As it is uncommon and frequently underdiagnosed, its incidence is not clear and few cases have been reported in literature [3].

Correspondence: Alfredo Mancuso, Department of Gynecological, Obstetrical Sciences and Reproductive Medicine, University Hospital, Policlinico “G. Martino,” Dip. Scienze Ginecologiche, Via Consolare Valeria 1, 98125 Messina, Italy. Tel: 00390902212965. Fax: 00390902212201. E-mail: [email protected]; [email protected]

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Figure 3. Ano-rectal edema.

Figure 1. Massive vulvar edema.

A caesarean section was performed and a healthy newborn of 2670 g was delivered with Apgar score of 7 and 9 at the first and the fifth minute respectively. The placenta was noted to be enlarged and grossly edematous, weighing 1050 g without signs of chorioamnionitis. Postoperative course was characterized by moderate hypertension (150/100 mm Hg) that required antihypertensive treatment with methyldopa. Therapy with albumin was continued, small doses of furosemide were administered to reduce the edema and low molecular weight heparin was considered since one of the reasons might have been venous thrombosis. The maternal edema progressively decreased over seven postpartum days and the patient and her baby were discharged. The histological evaluation of section stained with haematoxylin and eosin evidenced the placental anomalies with large, edematous, pale villi surrounded by an increased amount of fibrin deposition.

Discussion

Figure 2. Placentomegaly.

analgesic in small doses was considered in order to mitigate the discomfort of the patient but no significant improvement was recorded. While in the ward, blood pressure values and diuresis remained regular, but the clinical condition of the pregnant woman worsened with a widening of genital edema to the anorectal region and the onset of edema involving, above all, face and lower limbs Figure 3. Two days after admission, because of the progressive increase of proteinuria (900 mg/24 h) and the onset of labour pains, the decision for delivery was taken but the vaginal route was excluded owing to the local conditions.

The presence of a triad of maternal edema, fetal hydrops and placentomegaly has been referred to in several ways, such as pseudotoxemia, maternal hydrops syndrome, early onset preeclampsia, triple edema or, more frequently, as Ballantyne’s syndrome or Mirror syndrome [4,5]. We report an interesting example of an unusual form of Mirror syndrome for several reasons. First of all, the gestational age in which the disorder appeared differs remarkably from the data of literature in which it ranges from 22 to 28 weeks of gestation; in our case, clinical signs and symptoms appeared only at 37 weeks. Another difference consists in the lack of hypertension that represents the second most common symptom associated (58%) and explains the difficulty to differentiate this syndrome from preeclampsia. Even in our case, according to the proteinuria, preeclampsia was considered in the differential diagnosis but the absence of hypertension and low hematocrit allowed us to rule it out. Even

The Journal of Maternal-Fetal and Neonatal Medicine


Mirror syndrome 3 if its pathogenesis is not clear, it has been suggested that the hemodilution might be an important criterium for better characterization of this syndrome as opposed to preeclampsia marked by hemoconcentration [6]. In our case, a moderate increase in blood pressure was recorded only in puerperium and it emphasized the importance of carefully monitoring these patients also after delivery. In the second place, the characteristic triad is not totally represented: the fetus was not compromised although placentomegaly and hydramnios were described. In our opinion, the late onset of the syndrome could explain the partial involvement of placental district because it did not have time to determine fetal repercussions, represented by hydrops. Although mirror syndrome is associated with an increase in perinatal mortality, in the case we reported the late onset of the disorder associated with the medical treatment and the timely decision to perform a caesarean section allowed the birth of a healthy baby.

© 2012 Informa UK, Ltd.

Declaration of Interest: The authors report no conflicts of interest.

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