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An unusual form of mirror syndrome: A case report

Article  in  The journal of maternal-fetal & neonatal medicine: the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the
International Society of Perinatal Obstetricians · September 2012
DOI: 10.3109/14767058.2012.722734 · Source: PubMed

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Annamaria Giacobbe Maria Lieta Interdonato

Università degli Studi di Messina Azienda Ospedaliera Niguarda Ca' Granda
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Antonio Simone Laganà Onofrio Triolo

Università degli Studi dell'Insubria Università degli Studi di Messina
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 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
Department of Gynecological, Obstetrical Sciences and Reproductive Medicine, University Hospital, Messina, Italy
Aim: Mirror syndrome is a triad consisting of fetal hydrops,
maternal edema and placentomegaly. Its pathogenesis is
J Matern Fetal Neonatal Med Downloaded from informahealthcare.com by Miss Lindsay Duncan on 09/25/12
unclear and it is frequently mistaken for preeclampsia, even
though distinguishing features can be identified. It is associ-
ated 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
For personal use only.
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.
Keywords:  Ballantyne’s syndrome, hydrops, mirror syndrome,
placentomegaly, vulvar edema
Introduction
John W. Ballantyne in 1892 first described a syndrome character-
ized 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 pres-
ence of maternal edema that “mirrors” the fetal and placental
conditions. Mirror syndrome can be associated with both immu-
nological 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 peri-
natal outcome with poor fetal prognosis and increased maternal
morbidity [2].
As it is uncommon and frequently underdiagnosed, its inci-
dence 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: amancuso@unime.it; annamaria_8119@yahoo.it
1
We describe a case report of massive vulvar edema in an
18-year-old primigravida at 38 gestational weeks associated with
placentomegaly and hydramnios.
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
 2   A. Giacobbe et al.
J Matern Fetal Neonatal Med Downloaded from informahealthcare.com by Miss Lindsay Duncan on 09/25/12
For personal use only.
Figure 1.  Massive vulvar edema.
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 ano-
rectal 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 Journal of Maternal-Fetal and Neonatal Medicine
Figure 3.  Ano-rectal 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 hyperten-
sion (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 post-
partum days and the patient and her baby were discharged.
The histological evaluation of section stained with haema-
toxylin and eosin evidenced the placental anomalies with large,
edematous, pale villi surrounded by an increased amount of fibrin
deposition.
Discussion
The presence of a triad of maternal edema, fetal hydrops and plac-
entomegaly has been referred to in several ways, such as pseudo-
toxemia, 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 gesta-
tion; 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

if its pathogenesis is not clear, it has been suggested that the
hemodilution might be an important criterium for better char-
acterization 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 empha-
sized the importance of carefully monitoring these patients also
after delivery.
In the second place, the characteristic triad is not totally repre-
sented: 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
J Matern Fetal Neonatal Med Downloaded from informahealthcare.com by Miss Lindsay Duncan on 09/25/12
decision to perform a caesarean section allowed the birth of a
healthy baby.
For personal use only.
© 2012 Informa UK, Ltd.
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Mirror syndrome  3
Declaration of Interest: The authors report no conflicts of interest.
References
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Sarioglu N. Mirror syndrome: a systematic review of fetal associated
conditions, maternal presentation and perinatal outcome. Fetal Diagn
Ther 2010;27:191–203.
2. Midgley DJ, Harding K. The mirror syndrome. Eur J Obstet Gynecol
Reprod Biol 2000;88:201–202.
3. Vidaeff AC, Pschirrer ER, Mastrobatista JM, Gilstrap LC III, Ramin SM.
Mirror syndrome: a case report. J Reprod Med. 2002 Sep;47(9):770–774.
4. van Selm M, Kanhai HH, Gravenhorst JB. Maternal hydrops syndrome:
a review. Obstet Gynecol Surv 1991;46:785–788.
5. Carbillon L, Oury JF, Guerin JM, Azancot A, Blot P. Clinical biological
features of Ballantyne syndrome and the role of placental hydrops.
Obstet Gynecol Surv 1997;52:310–314.
6. Prefumo F, Pagani G, Fratelli N, Benigni A, Frusca T. Increased
concentrations of antiangiogenic factors in mirror syndrome
complicating twin-to-twin transfusion syndrome. Prenat Diagn
2010;30:378–379.