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Mirror Syndrome After Fetoscopic Laser Treatment - a Case Report Síndrome Do Espelho Após Tratamento Laser Por Fetoscopia-Casoclínico

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Mirror Syndrome After Fetoscopic Laser Treatment - a Case Report Síndrome Do Espelho Após Tratamento Laser Por Fetoscopia-Casoclínico THIEME 576 Case Report Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report Síndrome do espelho após tratamento laser por fetoscopia-casoclínico Ana Maria Simões Brandão1 Ana Patrícia Rodrigues Domingues1 Etelvina Morais Ferreira Fonseca1 Teresa Maria Antunes Miranda1 José Paulo Achando Silva Moura1 1 Obstetrics Unit A, Maternidade Dr. Daniel de Matos, Centro Address for correspondence Ana Brandão, Medical Resident, Rua Hospitalar e Universitário de Coimbra (CHUC), Faculdade de Miguel Torga, 3030-165 Coimbra, Portugal Medicina, Universidade de Coimbra, Portugal (e-mail: [email protected]; [email protected]). Rev Bras Ginecol Obstet 2016;38:576–579. Abstract Mirror syndrome is a rare disease with unknown pathophysiology that can be present in different diseases that can cause fetal hydrops. The prognosis is usually bad with a high Keywords perinatal mortality. We report an unusual form of mirror syndrome that manifested ► mirror syndrome itself only after a successful treatment for fetal hydrops (caused by twin-twin ► fetoscopic laser transfusion syndrome, in Quinteros stage IV) was performed. This syndrome was treatment controlled by medical treatment, and despite the usually bad prognosis seen in these ► twin-twin transfusion cases, we could extend the pregnancy from the 23rd to the 34th week of gestation, syndrome resulting in the birth of 2 live infants. Resumo Asíndromedoespelhoéumadoençarara,defisiopatologia desconhecida, que se manifesta em situações obstétricas responsáveis pela presença de hidrópsia fetal. Habitualmente o prognóstico é reservado, uma vez que se associa a elevadas taxas de Palavras-chave mortalidade perinatal. O presente caso clínico trata de uma situação de síndrome do ► síndrome do espelho espelho que se manifestou, atipicamente, após o tratamento eficazparaahidrópsia ► tratamento a laser fetal associada à síndrome de transfusão feto-fetal. fetoscópico Apesar do mau prognóstico associado a estas situações, conseguiu-se controlar a ► síndrome de situação apenas com tratamento médico e, desta forma, prolongar a gravidez durante transfusão feto-fetal 12 semanas. Introduction maternal edema virtually mirrors the edema of the fetus. The incidence is not clear since it is a rare disease, often – Mirror syndrome, also called Ballantyne’s or triple edema underdiagnosed.1 3 syndrome, was described in 1982 by John Ballantyne as a Fetal hydrops of any etiology, immune and nonimmune combination of maternal edema, fetal and placental hydrops. (►Table 1), can be associated with mirror syndrome, which This syndrome is a complication of fetal hydrops in which the appears when fetal symptoms become severe. Nevertheless, received DOI http://dx.doi.org/ Copyright © 2016 by Thieme-Revinter May 19, 2016 10.1055/s-0036-1593895. Publicações Ltda, Rio de Janeiro, Brazil accepted ISSN 0100-7203. September 27, 2016 Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report Brandão et al. 577 Table 1 Different causes associated with mirror syndrome Immune Rh iso immunization Nonimmune Viral infections Parvovirus B19 Human parvovirus Coxsackie virus Structural fetal or placental malformations Sacrococcygeal teratoma Aneurysm of Galen’svein Ebstein’sanomaly Placental chorioangioma Multiple pregnancy Twin-twin transfusion syndrome Fetal arrhythmia the syndrome’s pathogenesis and pathophysiology are still essentially hemodynamic, with implications for the cardio- unknown, and it is associated with a high perinatal vascular systems of both twins, presenting itself more se- – mortality.1 5 verely in the recipient twin, who usually has a progressive According to a few cases described in the literature, the cardiomyopathy as an adaptive fetal response to hemody- gestational age of appearance of this syndrome ranges from namic, hormonal and biochemical stressors.12,13 22 to 29 weeks.2,4 However, other reports suggest a sporadic Fetoscopic laser ablation of the placental anastomoses is earlier or later gestational age of manifestation.2,6 the primary treatment of TTTS, having a deep effect on fetal Symptoms of maternal edema include weight gain; ele- hemodynamics and on the reversal of cardiac dysfunction vated blood pressure with or without proteinuria; oliguria; and valvulopathy, even in severe cases.5,9,13 When mirror tachycardia and tachypnea; acute respiratory failure due to syndrome complicates TTTS, the laser treatment also has the pulmonary edema; and laboratorial signs of hemodilution potential to reverse this complication.8 So far, there have with mild anemia and elevated liver enzymes, thus resem- been very few cases reported in the literature in which bling other complications such as preeclampsia or HELLP mirror syndrome occurred after laser therapy for syndrome.1,3,7 It has been suggested that the presence of TTTS.5,11,14 We report one such unusual case in which mirror hemodilution might be an important criterion to better syndrome presented itself only after fetoscopic laser therapy differentiate mirror syndrome from preeclampsia, which is was done for fetal hydrops in a twin gestation. associated with hemoconcentration.3,8 The similarity between these diseases has led some Case Report authors to designate mirror syndrome as a preeclampsia- like syndrome.7 Several studies and case reports have A 38 year-old woman with a spontaneous monochorionic, emerged in which authors have focused on a possible pla- diamniotic twin pregnancy was referred to our institution at cental dysfunction, where upregulation of antiangiogenic 9 weeks for a multiple pregnancy follow-up. Her personal factors seems to be responsible for the preeclampsia-like medical history included ankylosing spondylitis and hyper- – clinical pictures seen in mirror syndrome.3,4,7 9 somnia, a B Rh-positive blood type, and a previous forceps Mirror syndrome can be reversible when the underlying and breech delivery (gesta 3 para 2). An amniocentesis was factors are identified and corrected.3 Improvement in the performed for advanced maternal age, and it revealed a maternal condition after treating the cause of fetal hydrops normal karyotype in both fetuses. The first ultrasounds has been reported.10 When it is not possible to reverse the were normal, although on the 16th week, a minimal differ- main factor, the prognosis of the syndrome is usually poor, ence in amniotic fluid was found between both fetuses. This with high perinatal mortality, and prompt termination of difference was maintained on subsequent weeks (maximum pregnancy is often recommended.11 Delivery, of course, vertical pocket of 2.4 versus 7.4 cm), but without the criteria should be in accordance with gestational age and maternal of a TTTS. stability.3 Support therapy (diuretics and antihypertensive At 23 weeks of gestation, an ultrasound showed acute fetal therapy) for maternal stability is crucial in preventing the deterioration with oligohydramnios versus polyhydramnios, deterioration of the mother’s condition. discordant bladders, and the recipient twin presented severe Mirror syndrome is also a rare complication of twin-twin hydrops and cardiomegaly (►Fig. 1). The diagnosis of TTTS in transfusion syndrome (TTTS), a severe complication associ- Quintero stage IV was made. Umbilical artery Doppler flow ated with monochorionic twin pregnancies due to the un- indices were abnormal (altered pulsatility index) in the balanced flow of blood from one twin (donor) to its co-twin recipient twin, and his echocardiogram showed a cardiome- (recipient) through arteriovenous anastomoses deep within galy with reduced cardiac ejection fraction and a narrow the shared placental lobules.5,8 It is a progressive disease, aorta. Rev Bras Ginecol Obstet Vol. 38 No. 11/2016 578 Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report Brandão et al. Fig. 1 Ultrasound findings of the fetus with hydrops (A and B), cardiomegaly (C) and polydramnios (D). Since our institution does not have experience with laser The postnatal echocardiogram of the ex-recipient twin treatment for TTTS, we contacted and sent the gravid to the showed biventricular hypertrophy and distal dilatation of Clínic Barcelona Hospital Universitari, where a successful the left ventricle with contraction anomaly and hypertrabe- fetoscopic laser therapy was performed. The mother and the culation. Twenty months later, this twin presents no cardiac fetuses responded well on the first hours after this proce- symptoms and has a normal cardiac auscultation, with an dure. However, on the day after, the mother presented an echocardiogram revealing lateral and apical trabeculation acute shortness of breath in a context of intense generalized but a normal ejection fraction. The ex-donor twin has no edema and oliguria, without signs of hypertension or pro- cardiac problem. teinuria. Analytically, she presented a progressive dilutional Despite the physical development being good in both anemia (hemoglobin [Hgb] ¼ 8.3 g/dL; hematocrit [Htc] twins, they are manifesting some delayed motor develop- ¼ 25.7%), normal renal function (Naþ ¼ 134; creatinine ment: sitting without support only at 13 months and walking ¼ 0.47), normal hepatic function, and negative protein to at 17 months. creatinine ratio in urine (0.3). Diuretics (furosemide three ’ times a day ) were introduced into the mother stherapy. Discussion Four days afterward, she was discharged and arrived clinically stable at our obstetrics unit, where diuretics treat- Mirror syndrome is a rare condition, where maternal edema ment, clinical and analytical controls were maintained
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