Displasia mesenquimal placentaria asociada con restricción del crecimiento intrauterino de inicio temprano: reporte de un caso / Placental mesenchymal dysplasia associated with early onset intrauterine growth restriction: a case report

Resumen ANTECEDENTES: La displasia mesenquimal placentaria es una anomalía de la vasculatura y del tejido conectivo placentario que altera la estructura y la función de las vellosidades, se asocia con un desenlace perinatal adverso. CASO CLÍNICO: Paciente de 21 años, primigesta, con 15.2 semanas de embarazo establecidas por fetometría, con placenta corporal anterior, de morfología anormal, con múltiples imágenes hipoecogénicas que semejan racimos de uvas, sin flujo vascular en el Doppler color. La valoración ecográfica de las 33 semanas reportó fetometría para 27.2 semanas, anhidramnios y placentomagelia, con alteración en los parámetros de la flujometría Doppler. En la prueba sin estrés se diagnosticó restricción del crecimiento intrauterino estadio IV. La imagen del feto fue heterogénea en el flanco derecho de 4.7 x 4.1 cm. El embarazo finalizó por cesárea con una recién nacida con Apgar 8-9, de 930 gramos, placenta agrandada con vellosidades hidrópicas. El estudio histopatológico reportó: displasia mesenquimal placentaria. La resección del tumor abdominal fue electiva, por vía laparoscópica, al cuarto mes de vida, con diagnóstico histopatológico de hamartoma mesenquimal hepático. CONCLUSIÓN: La displasia mesenquimal placentaria es una anomalía placentaria benigna, relacionada con restricción del crecimiento intrauterino de inicio temprano que puede evolucionar, incluso, a la muerte del feto por hipoxia crónica debido a las anomalías en la vasculatura microscópica que afectan el desarrollo de las vellosidades terminales e interfiere en el intercambio de gases, nutrientes y electrolitos en el espacio intervelloso.

Abstract BACKGROUND: Placental mesenchymal dysplasia is an anomaly of the vasculature and placental connective tissue which alters the structure and function of the villi, being associated with an adverse perinatal outcome. CLINICAL CASE: Patient 21 years old, primigestation, with 15.2 weeks of pregnancy established by fetometry, with anterior body placenta, abnormal morphology, with multiple hypoechogenic images resembling clusters of grapes, without vascular flow in color Doppler. Ultrasound evaluation at 33 weeks, reporting fetometry for 27.2 weeks, anhydramnios and placentomagelia, with alteration in Doppler parameters, as well as in a non-stress test diagnosing fetal growth restriction stage IV. Fetus with heterogeneous image on the right flank of 4.7 x 4.1 cm. Abdominal pregnancy interruption was decided, obtaining a live female newborn, Apgar 8/9, weight 930 grams, enlarged placenta with hydropic villi. The histopathology study reports placental mesenchymal dysplasia. Abdominal tumor resection was performed electively by laparoscopy at 4 months of age with a histopathological diagnosis of hepatic mesenchymal hamartoma. CONCLUSION: Placental mesenchymal dysplasia is a benign placental anomaly, related to early-onset intrauterine growth restriction that can even progress to fetal death by chronic hypoxia due to abnormalities in the microscopic vasculature that affect the development of the terminal villi and interfere with the exchange of gases, nutrients and electrolytes in the intervillous space.

Clinicopathological analysis of 5 cases of placental mesenchymal dysplasia / 临床与实验病理学杂志

Purpose To explore the clinicopathological features, diagnosis, differential diagnosis and prognosis of placental mesenchymal dysplasia. Method The clinicopathological data of 5 cases with placental mesenchymal dysplasia were retrospectively analysed and related literatures were also re-viewed. Results All of 5 patients were consciously fetal movement disappeared or found abnormal ultrasound results at routine examination of the pregnancy. The placentas were enlarged, partly with oedematous "grape-like" cysts. On histologic exami-nation, enlarged villi with varying degrees of edema contained abnormal thick walled fetal blood vessels. The chorionic vessels were expanded and congested, and some chorionic villi showed mesenchymal cell hyperplasia. In immunohistochemical staining, p57 was positive, and Ki-67 showed low expression. There was no the trophoblastic proliferation. It's mainly differential diagnosis was hydatidiform mole.2 cases were accompanied with stillbirth. Conclusion The diagnosis of placental mesenchymal dysplasia can be confirmed by pathology examination. When a cystic placenta is detected by ultrasound examination, placental mesenchymal dysplasia should be considered in the differential diagnosis.

Disomía uniparental-duplicación paterna de la región del cromosoma 11p15.5 como causa de displasia mesenquimatosa placentaria / Placental mesenchymal dysplasia due to uniparental disomy/paternal duplication of 11p15.5

Resumen ANTECEDENTES La displasia mesenquimatosa de la placenta es una enfermedad rara que en la ecografía se observa con vesículas en forma de racimo de uvas. Puede provocar complicaciones en el embarazo, como: restricción del crecimiento intrauterino, muerte intrauterina y parto pretérmino. CASO CLÍNICO Paciente de 31 años con displasia mesenquimatosa placentaria diagnosticada en la ecografía del primer trimestre de embarazo. Se apreció una zona con formaciones econegativas de 14 x 20 mm, con escasa captación del doppler color y que terminó de confirmarse en el segundo trimestre, con una biopsia corial. El embarazo trascurrió con normalidad hasta la semana 33, que fue cuando se detectó la restricción del crecimiento intrauterino. Debido a la alta incidencia de complicaciones obstétricas derivadas de éste se decidió la inducción del parto en la semana 37 de la gestación. CONCLUSIONES El diagnóstico de displasia mesenquimatosa placentaria requiere seguimiento estrecho del embarazo e inducir su finalización entre las semanas 37-38 para aminorar las complicaciones perinatales.

Abstract BACKGROUND Placental mesenchymal dysplasia is a rare disease of the placenta which presents with vesicles in the form of a cluster of grapes on ultrasound. It can cause pregnancy complications such as: intrauterine growth restriction, intrauterine death, and preterm birth CLINICAL CASE A 31-year-old patient with placental mesenchymal dysplacia diagnosed on the ultrasound of the first trimester of pregnancy, in which an area with 14 x 20 mm econegative formations was observed with little uptake of the color Doppler and which was confirmed in the second trimester by corial biopsy of that area. The pregnancy was normal until week 33, after which an intrauterine growth restriction was detected. Due to the high incidence of obstetric complications of this entity, an induction of labor was decided at week 37 of gestation. CONCLUSIONS Whenever this type of pathology is diagnosed, it is recommended a close follow-up of pregnancy and an induction of labor around 37-38 weeks of gestation, due to the perinatal complications presents.

Displasia mesenquimal de placenta: revisión sistemática / Placental mesenchymal dysplasia: systematic review

Resumen OBJETIVOS: Describir las características clínicas y las repercusiones perinatales de la displasia mesenquimal de placenta. MÉTODO: Revisión sistemática de la bibliografía disponible en las bases PubMed, EMBASE y Scopus en donde se buscaron los términos "displasia mesenquimal de placenta" y "pseudomola parcial". Criterios de inclusión: artículos de casos o series que contuvieran los apartados de diagnóstico histológico confirmado y reporte de al menos 75% de los datos clínicos establecidos. Como ejemplo se comunica un caso clínico de los autores. RESULTADOS: Se encontraron 202 artículos, casi todos de casos clínicos o series de casos. Se descartaron los duplicados y los que no cumplieron los criterios de inclusión; finalmente la serie quedó formada por 88 publicaciones con 104 casos clínicos. CONCLUSIONES: La displasia mesenquimal de placenta es una anomalía poco conocida, infradiagnosticada y poco publicada. En la ecografía simula una mola parcial, casi siempre con un cariotipo fetal diploide y altas concentraciones de alfafetoproteína. Es frecuente su asociación con prematurez, rotura prematura de membranas, retraso del crecimiento intrauterino, malformaciones fetales, síndrome de Beckwith-Wiedemann y muerte perinatal.

Abstract OBJECTIVES: To describe the clinical characteristics and perinatal outcomes of placental mesenchymal dysplasia. MATERIALS AND METHODS: Systematic review of the medical literature under the terms "placental mesenchymal dysplasia", "partial pseudomole". Inclusion criteria for the review were: confirmed histological diagnosis and presence of at least 75% of established clinical data. The systematic review was performed by searching for cases or series published in PubMed, EMBASE, Scopus databases. We present 1 clinical case of our institution. RESULTS: A total of 202 articles were found, most of them corresponding to clinical cases or case series. Duplicates were discarded and those that did not meet inclusion criteria were excluded. Finally, the series consisted of 88 publications with 104 clinical cases. CONCLUSIONS: Placental mesenchymal dysplasia is a poorly understood, underdiagnosed, and poorly published clinical entity. Placental mesenchymal dysplasia echocardiography simulates a partial spring, but usually presents a diploid fetal karyotype and elevated levels of alpha-fetoprotein. It is frequently associated with prematurity, premature rupture of membranes, intrauterine growth retardation, fetal malformations, Beckwith-Wiedemann syndrome and perinatal death.

Mesenchymal dysplasia of placenta.

A rare case of placental mesenchymal dysplasia (PMD) in a 26-year-old patient is reported. Ultrasound scan at 17 weeks of gestation showed placenta with multiple cystic spaces and a normal appearing fetus. Following delivery of a term live baby, histological examination of the placenta was suggestive of PMD. The early recognition of this rare condition by characteristic ultrasonographic fi ndings is herein emphasized and hence that PMD is distinguished from molar pregnancy.

Displasia mesenquimal placentaria: caso clínico / Placental mesenchymal dysplasia: a case report

La displasia mesenquimal placentaria es una entidad poco conocida, de etiología incierta y subdiagnosticada. Frecuentemente, es confundida con enfermedad trofoblástica gestacional debido a que se presenta con hallazgos ultrasonográficos caracterizados por una placenta engrosada, con quistes e imágenes hipoecogénicas y niveles de gonadotrofina coriónica humana normales o levemente aumentados. El feto es frecuentemente viable y puede manifestar retraso del crecimiento intrauterino, prematurez o asociarse al síndrome de Beckwith-Wiedemann. Se presenta el caso de una mujer joven con un parto pretérmino con placentomegalia, sospecha de mola hidatidiforme parcial y un recién nacido pequeño para la edad gestacional.

The placental mesenchymal dysplasia is a not well known entity, with an uncertain etiology and under diagnosed. It is frequently confused with gestational trophoblastic disease because of its ultrasonographic features of a thick placenta, cysts and hypoechogenic images, with normal or slightly increased levels of human chorionic gonadotrophic hormone. The fetus is often viable and can manifest intrauterine growth restriction, prematurity or be associated with Beckwith-Wiedemann syndrome. We present a case report of a young woman with a preterm delivery, placentomegaly, suspicious of a partial hydatidiform mole and a low growth newborn.

Placental mesenchymal dysplasia: A report of two cases with review of literature.

Placental mesenchymal dysplasia (PMD) is a recently recognized, rare placental vascular anomaly characterized by placentomegaly and grape-like vesicles mimicking partial molar pregnancy. It is associated with significant fetal morbidity and mortality. We describe the histologic features of PMD in two different cases with different disease outcomes, one in a preterm intrauterine death (IUD) and another in a live birth. Placental examination in both the cases revealed large placenta with multiple vesicles and mesenchymal dysplasia.

Placental Mesenchymal Dysplasia Associated with a Fetal Unilateral Multicystic Dysplastic Kidney: A Case Report / 대한주산의학회잡지

Placental mesenchymal dysplasia (PMD) is a rare placental disorder that may coexist with a normal fetus but is frequently associated with fetal growth restriction, fetal demise, prematurity, and Beckwith-Wiedemann syndrome, as well as with various fetal anatomical and vascular anomalies. In this case, ultrasonography performed at 14 weeks of gestation revealed multiple, variable sized cysts in the placenta along with a morphologically normal fetus. Serial sonographic examinations found that the fetus became small for gestational age and had a dysplastic right kidney. After delivery at 33 weeks of gestation, histopathologic examination of the placenta showed enlarged, hydropic stem villi with cavity formation and absence of trophoblastic proliferation and pseudoinclusions, all of which were consistent with PMD. Neonatal abdominal ultrasonography showed multiple cysts in the right kidney consistent with a multicystic dysplastic condition. This appears to be the first case of PMD associated with fetal multicystic dysplastic kidney.

Displasia mesenquimatosa de la placenta: caso clínico / Placental mesenchymal dysplasia: case report

Placental mesenchymal dysplasia (PMD) is a rare placental anomaly characterized by placentomegaly and grapelike vesicles resembling a molar placenta by ultrasound. A clinical case is presented and we will discuss the differential diagnosis, possible associations and perinatal management.

La displasia mesenquimatosa de la placenta es una anormalidad rara de la placenta caracterizada por una placenta grande con dilataciones quísticas similares a una mola parcial a la ultrasonografía. Se presenta un caso clínico y se discutirá el diagnóstico diferencial, posible asociaciones y manejo perinatal.