Placental Mesenchymal Dysplasia
Placental Mesenchymal Dysplasia
Placental mesenchymal dysplasia (PMD) is a rare condition of placentomegaly and abnormal chorionic villi often clinically mistakenly as partial hydatidiform mole. However, it is clinicopathologically distinct with high incidence of intrauterine growth restriction (IUGR) and fetal death. This study presents 11 new PMD cases and provides a meta-analysis of the associated IUGR and fetal death rates. The cases were identified between 1971 and 2005, mostly from consultation files. To our knowledge, 71 PMD cases have previously been reported; 15 of these were associated with Beckwith-Wiedemann syndrome (BWS). With the addition of our new results, among all cases without BWS, 50% had IUGR and 43% had intrauterine fetal demise (IUFD) or neonatal death. Females represented 82% of cases. Thus, PMD is associated with high IUGR and IUFD/neonatal death rates and disproportionally affects females. The pathogenesis is yet unknown. The current understanding and hypotheses involving PMD are discussed.
First described in 1991 by Moscoso and colleagues, placental mesenchymal dysplasia (PMD) is a rare lesion. Sonographically, PMD shows findings similar to those of partial hydatidiform mole, such as enlarged placentas with multicystic, anechoic regions (giving a moth-eaten appearance), and widely distributed, large, edematous villi as seen under gross examination. PMD has distinct clinicopathologic features. Unlike molar pregnancies, characterized by absent or malformed fetuses, PMD usually features a normal fetus and the pregnancy often extends into the third trimester. About 20% of the fetuses with PMD also have Beckwith-Wiedemann syndrome (BWS), a condition characterized by macrosomia, visceromegaly, hemihyperplasia (hemihypertrophy), macroglossia, omphalocele, and adrenal cytomegaly that is recognizable prenatally or in infancy. It is unknown whether cases with BWS are distinct from cases without BWS; however, their placental findings are morphologically similar.
In previous case reports and small series, intrauterine growth restriction (IUGR) and intrauterine fetal demise (IUFD) often were noted. The purposes of this article are to describe 11 new cases and to review the literature with attention to the rates of associated IUGR and IUFD in PMD.
Abstract and Introduction
Abstract
Placental mesenchymal dysplasia (PMD) is a rare condition of placentomegaly and abnormal chorionic villi often clinically mistakenly as partial hydatidiform mole. However, it is clinicopathologically distinct with high incidence of intrauterine growth restriction (IUGR) and fetal death. This study presents 11 new PMD cases and provides a meta-analysis of the associated IUGR and fetal death rates. The cases were identified between 1971 and 2005, mostly from consultation files. To our knowledge, 71 PMD cases have previously been reported; 15 of these were associated with Beckwith-Wiedemann syndrome (BWS). With the addition of our new results, among all cases without BWS, 50% had IUGR and 43% had intrauterine fetal demise (IUFD) or neonatal death. Females represented 82% of cases. Thus, PMD is associated with high IUGR and IUFD/neonatal death rates and disproportionally affects females. The pathogenesis is yet unknown. The current understanding and hypotheses involving PMD are discussed.
Introduction
First described in 1991 by Moscoso and colleagues, placental mesenchymal dysplasia (PMD) is a rare lesion. Sonographically, PMD shows findings similar to those of partial hydatidiform mole, such as enlarged placentas with multicystic, anechoic regions (giving a moth-eaten appearance), and widely distributed, large, edematous villi as seen under gross examination. PMD has distinct clinicopathologic features. Unlike molar pregnancies, characterized by absent or malformed fetuses, PMD usually features a normal fetus and the pregnancy often extends into the third trimester. About 20% of the fetuses with PMD also have Beckwith-Wiedemann syndrome (BWS), a condition characterized by macrosomia, visceromegaly, hemihyperplasia (hemihypertrophy), macroglossia, omphalocele, and adrenal cytomegaly that is recognizable prenatally or in infancy. It is unknown whether cases with BWS are distinct from cases without BWS; however, their placental findings are morphologically similar.
In previous case reports and small series, intrauterine growth restriction (IUGR) and intrauterine fetal demise (IUFD) often were noted. The purposes of this article are to describe 11 new cases and to review the literature with attention to the rates of associated IUGR and IUFD in PMD.
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