Síndrome de Ballantyne: segundo episodio en una embarazada. Reporte de caso y revisión de la bibliografía / Ballantyne syndrome: Second episode in a pregnant woman. Case report and literature review

Resumen ANTECEDENTES: El síndrome de Ballantyne es un cuadro poco frecuente asociado con hidrops fetal, en el que la madre refleja los síntomas fetales. Es decisivo diferenciarlo de la preeclampsia porque comparten signos de hipertensión y proteinuria. Su etiopatogenia se desconoce pero se han propuesto teorías asociadas con el desequilibrio entre factores angiogénicos y antiangiogénicos. CASO CLÍNICO: Paciente de 29 años, controlada en la consulta de Medicina Materno-Fetal debido al antecedente de síndrome de Ballantyne en el embarazo previo. En la ecografía de control a las 26 semanas se detectaron placentomegalia, ascitis fetal e incremento del líquido amniótico. Las ecografías posteriores demostraron polihidramnios e hidrops a las 28 semanas. Enseguida de la aparición del edema se estableció el diagnóstico de síndrome del espejo recidivante e hidrops no inmunitario. Se hospitalizó para drenaje del líquido. La amniorrexis se produjo a las 29 + 6 semanas. Una semana después se inició la dinámica uterina y el embarazo finalizó a las 31 semanas, después de la ruptura prematura de membranas. El neonato fue un varón de 3200 g, Apgar 2-6-8 al minuto, 5 y 10 minutos, respectivamente. Después del estudio postnatal se estableció el diagnóstico de perforación ileal múltiple. El recién nacido requirió 5 intervenciones quirúrgicas, con posoperatorio tórpido y se dio de alta a los 3 meses de vida. CONCLUSIONES: El síndrome del espejo es infradiagnosticado, a pesar de su potencial para complicar gravemente el embarazo asociado con hidrops. La recuperación de la madre suele ser favorable a los pocos días de posparto aunque la morbilidad y la mortalidad fetal son elevadas.

Abstract BACKGROUND: Ballantyne syndrome is characterized by the triad: fetal, placental and maternal edema. It is an uncommon condition associated with fetal hydrops, in which mother reflects fetal symptoms. It is essential to differentiate from preeclampsia, since there are common signs such as hypertension and proteinuria. Etiopathogenesis is unknown, although theories associated with an imbalance between angiogenic and antiangiogenic factors have been postulated. Treatment consists of ending the pregnancy or improving the fetal situation. CLINICAL CASE: We present the case of a 29-years pregnant woman controlled in the Maternal-Fetal Medicine Unit due to the history of Ballantyne Syndrome in the previous gestation. In the follow-up ultrasound performed at 26-weeks, placentomegaly, fetal ascites and increased amniotic fluid were detected. Subsequent ultrasounds showed polyhydramnios and fetal hydrops at 28-weeks. After maternal edema began, she was diagnosed with recurrent Mirror Syndrome and non-immune hydrops. Admission was indicated and amniodrainage was performed due to symptomatic polyhydramnios. Finally, premature rupture of membranes occurred at 29+6-weeks. She started uterine dynamic after one week, ending in a preterm delivery at 31-weeks after premature rupture of membranes. A 3200gr male was born with Apgar Scores 2-6-8 at 1, 5 and 10min respectively and, after postnatal study, he was diagnosed with multiple ileal perforation. Five surgical interventions were necessary, with a complicated postoperative period and could be discharged at 3 months of age. CONCLUSIONS: Mirror syndrome is an underdiagnosed pathology of unknown incidence that can seriously complicate gestation associated with fetal hydrops. Maternal recovery is favorable few days after delivery, but it leads to high fetal morbi-mortality.

Síndrome en espejo como manifestación del desequilibrio angiogénico: Apropósito de un caso / The mirror syndrome as a manifestation of angiogenic imbalance: a propos of a case

El síndrome en espejo es una entidad obstétrica poco frecuente, cuya triada clásica es hidropesía fetal, edema materno generalizado y placentomegalia. Presentamos el caso de una gestante de 25 semanas, con diagnóstico de hidropesía fetal por anemia severa y criterios de preeclampsia severa con óbito fetal, en el contexto del síndrome en espejo. Revisamos la literatura con énfasis en la anti-angiogénesis como origen fisiopatológico de la enfermedad.

The mirror syndrome is a rare obstetric condition whose classical triad is fetal hydrops, generalized maternal edema and placentomegaly. We present the case of a 25 weeks pregnant woman with diagnosis of fetal hydrops due to severe anemia presenting criteria of severe preeclampsia with fetal death, in the context of mirror syndrome. We review the literature with emphasis on anti-angiogenesis as the pathophysiological origin of the disease.

Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report / Síndrome do espelho após tratamento laser por fetoscopia - caso clínico

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 perinatal mortality. We report an unusual form of mirror syndrome that manifested itself only after a successful treatment for fetal hydrops (caused by twin-twin transfusion syndrome, in Quinteros stage IV) was performed. This syndrome was controlled by medical treatment, and despite the usually bad prognosis seen in these cases, we could extend the pregnancy from the 23rd to the 34th week of gestation, resulting in the birth of 2 live infants.

Resumo A síndrome do espelho é uma doença rara, de fisiopatologia 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 mortalidade perinatal. O presente caso clínico trata de uma situação de síndrome do espelho que se manifestou, atipicamente, após o tratamento eficaz para a hidrópsia fetal associada à síndrome de transfusão feto-fetal. Apesar do mau prognóstico associado a estas situações, conseguiu-se controlar a situação apenas com tratamento médico e, desta forma, prolongar a gravidez durante 12 semanas.

Síndrome de transfusión intergemelar estadio III, manejado con fotocoagulaición láser guiada por fetoscopia, complicado con Síndrome de Ballantyne y RCIU selectivo del feto donante: reporte de caso y revisión de la literatura / A case report and literature review in twin-twin transfusion syndrome stage III, fetoscopy -guided laser therapy, complicated with ballantyne syndrome and selective IUGR of fetus donor

Objetivo: se presenta el caso de una paciente primigestante con embarazo monocorial biamniótico y síndrome de transfusión intergemelar estadio III, diagnosticado en la semana 23 de gestación. El manejo se llevó a cabo con foto-coagulación láser selectiva y secuencial de vasos comunicantes. Se presentó complicación con síndrome de Ballantyne y RCIU selectivo del feto donante. Desembarazada en la semana 35 con buen resultado perinatal. El objetivo es realizar una revisión actualizada de la literatura del síndrome de Ballantyne. Materiales y métodos: se revisaron las bases de datos PubMed, Cochrane, MEDLINE y libros de la especialidad, relativos al término “síndrome de Ballantyne”, sin restricción en la fecha de publicación. Conclusión: la fotocoagulación láser selectiva y secuencial de vasos comunicantes es el manejo de elección para el síndrome de transfusión fetofetal. La restricción de crecimiento selectivo del feto donante en estadio III se puede presentar en el 40 a 70% de los casos. El síndrome de Ballantyne puede ser secundario a edema placentario sin hidrops fetal; su sospecha puede ayudar a identificar cuáles pacientes se podrían beneficiar de un manejo activo de esta patología.

Objective: a 21 years old primigravid patient with biamniotic monochorial twin pregnancy and stage III twin-to -twin transfusion syndrome, diagnosed during week 23 of gestation is presented. She was managed with selective, sequential laser photocoagulation of the communicating blood vessels which was complicated by Ballantyne’s syndrome (mirror syndrome) and selective intrauterine growth restriction (IUGR) of the donor fetus. She delivered during week 35 with good perinatal result. The object was to make an updated review of the literature about Ballantyne’s syndrome. Materials and methods: the PubMed/ MEDLINE, Cochrane databases were searched as well as books on the specialization, related to the term “Ballantyne’s syndrome”, with no restriction regarding publication date. Conclusion: selective, sequential laser photocoagulation of the communicating blood vessels is the management of choice for twintwin fetus transfusion syndrome. Selective growth restriction of the donor fetus in stage III could be presented in 40% to 70% of cases. Ballantyne’s syndrome could be secondary to placental edema without fetal hydrops; suspecting it, helps to identify which patients could benefit from active management of this pathology.