-by Leanne Dahlgren, MD

My colleagues and I are very interested in helping women who have PXE obtain the best care possible during pregnancy. We invite women who have PXE and have experienced pregnancy to read the abstract provided below and to tap into the website address provided at the end. Please complete the questionnaire in the knowledge that this is a protected website and that your responses will be confidential. We hope to obtain enough information about pregnancy among women with PXE to advise physicians through medical literature about what to look for and how to protect such women. Thank you for participating in this study.

Background: Pseudoxanthoma elasticum (PXE) is a rare hereditary disorder characterized by systemic degeneration of elastic tissue. It affects the skin, eye, cardiovascular system and gastrointestinal system. Most women have normal pregnancies, but gastrointestinal bleeding, ruptured aneurysm, and hypertension have been reported. Damage to the pelvic floor is theorized.

Case: We report a case of a 27 year-old primigravida with PXE. Pregnancy was uncomplicated. A term primary elective caesarean delivery was performed to protect the pelvic floor and reduce the chance of retinal hemorrhage. As well, an apronectomy was performed to improve cosmetic results of abdominal skin healing.

Conclusion: Primary elective caesarean delivery should be considered. Further studies are warranted to establish the effects of pregnancy and delivery on the pelvic floor in women with PXE.

We would like to determine if women with PXE are at higher risk of different complications of skin and tissue healing as a result of pregnancy. You will be helping women with PXE everywhere if you agree to fill out our short survey (5 minutes). Your responses remain anonymous. We hope to learn more about the effects of pregnancy on PXE and determine the optimal management of pregnancy in women with PXE. Thank you for your time.

Please click on the below website address OR copy or type this address into the address bar on your internet home page. It will link you to the web based survey.

http://www.surveymonkey.com/s.aspx?sm=bAJHnSe6GsndGtr_2f3gTmjg_3d_3d