Lay Summary: The Effects of Pseudoxanthoma Elasticum (PXE) on the Placenta and Pregnancy

October 12, 2011
By Lisa Ratanaprasatporn, Intern, PXE International

Early literature on pregnancy in women with pseudoxanthoma elasticum (PXE) contained reports of severe complications, such as gastrointestinal hemorrhage, congestive heart failure, and cardiac arrhythmia. This led healthcare providers to advise women with PXE against becoming pregnant. However, more recent studies do not support that recommendation and to date, there is no strong evidence linking PXE to complications during pregnancy for the mother or the fetus. (See PXE and Pregnancy)

In an epidemiological survey conducted in 2000 by PXE International, many women with pseudoxanthoma elasticum reported placental calcifications during pregnancy. This information led to a cooperative study of the placenta in PXE. Dr. Lionel Bercovitch and Sharon Terry collected placentae from donors, and a team led by Dr. Ivonne Pasquali-Ronchetti in Italy analyzed and compared them to control placentae. In 2001, the results were published by Gheduzzi et al in a paper titled “The placenta in pseudoxanthoma elasticum: clinical, structural and immunochemical study” in the journal Placenta.

In the study, the features of placentae at term from 14 control and 15 PXE-affected women were compared in order to better understand if and how abnormal mineral accumulation in pseudoxanthoma elasticum might affect placental function. There were no marked differences between placentae of PXE patients and controls, with regard to weight, size, infarct (damage due to obstruction of local blood supply), blood clots, and inflammation. However, cell death and mineralization appeared statistically more pronounced in PXE. The placentae in patients with PXE had significantly increased mineral deposition, especially on the maternal side of the placenta. Thus, mineralization of the placenta is more pronounced in women with PXE than women without PXE. However, the calcification does not appear to significantly affect placental function or fetal well being. Women with pseudoxanthoma elasticum seldom experience problems during pregnancy. In all cases, pregnancy, fetus growth, and delivery were normal.

The uncomplicated pregnancy and normal delivery of a child by women with PXE was illustrated in a 2008 paper titled “Placental Calcification in Pseudoxanthoma Elasticum” published in the Annals Academy of Medicine by Wei Chan Tan and Charles H. Rodeck. In this study, Tan and Rodeck reported a case of a 37-year-old pregnant white woman with PXE. She was diagnosed with PXE at the age of 26 with the characteristic eye and skin lesions of PXE. Her prenatal imaging showed an extensively calcified placenta known to be associated with PXE. Despite the calcification of the placenta, her pregnancy was normal. The woman delivered a healthy baby boy at the 38th week by Caesarean section. The child had normal weight and normal growth. The boy did not show any characteristic skin lesions at birth and remained well at 6 months of age. In addition, the mother was well throughout the pregnancy. She did not experience aggravation of skin lesions, hypertension, or gastrointestinal bleeding. She did not have any drastic changes in abdominal skin quality or excessive stretch marks.

In conclusion, most women with pseudoxanthoma elasticum show no serious complications during pregnancy. Some pregnancies are associated with worsening of skin manifestations, but more recent reports showed no complications in the mother or the baby during the pregnancy or the first few months following the birth. Therefore, there is no basis for advising women with PXE to avoid becoming pregnant based on recent reports and findings.
 

References

Gheduzzi D, Taparelli F, Quaglino D Jr, Di Rico C, Bercovitch L, Terry S, Singer DB, Pasquali-Ronchetti I. The placenta in pseudoxanthoma elasticum: clinical, structural and immunochemical study. Placenta. 2001 Jul;22(6):580-90.

Tan WC, Rodeck CH. Placental calcification in pseudoxanthoma elasticum. Ann Acad Med Singapore. 2008 Jul;37(7):598-600.