Background: Pseudoxanthoma Elasticum PXE is a rare inherited connective tissue disease characterized by degenerative changes due to calcification and fragmentation of elastic fibers on the skin, eyes and cardiovascular system.
There are very few reports of Labour Analgesia (LA) in pregnant women with PXE. We found only 2 cases in the literature.
The aim of our study is to describe the analgesic approach and discuss the main anaesthetic and surgical particularities of these parturients.
Case Report: A 37 year-old pregnant woman, ASA II, With PXE, dyslipidemia, obesity. She had a history of infertility with medically assisted reproduction, which resulted in 2 pregnancies followed by abortion with organ dysfunction and need for ICU care. The present pregnancy was naturally conceived and was held without complications besides an elective cervical cerclage at 17w.
The examination showed yellow spots on folds and fibrotic streaks in the retina, all of these PXE features, and cardiomegaly on the chest x-ray.
Based on the literature and in the face of her condition, we proposed Regional Analgesia RA: epidural and a normal delivery that the patient accepted.
Labour progressed uneventful and delivery time was reduced through the use of forceps. During placental removal the umbilical cord ruptured, which led us to the OR for the internal manual removal of the remaining placenta under close monitoring providing a safer procedure. The procedure was performed under an epidural bolus and sedation with good control of vital signs.
In the postpartum period, there were no obstetric or anesthetic complications.
Learning points: Close monitoring and control of heart rate and blood pressure is critical, due to premature development of atherosclerosis and risk of myocardial infarction.
RA allows for better haemodynamic stability during childbirth. There is less chance of bleeding from injured arteries and reduced cardiac work.
There may be bleeding from the retina secondary to the Valsalva maneuver during the second stage of delivery. Instrumentation is recommended to shorten its duration.
Beware that necrotic changes and placental mineralization are more common in these pregnant women, which can present as a complication during labour.
This case of success using RA for labour in patients with PXE may be helpful aiding in the most appropriate analgesic choice in these cases, taking into account the few cases described and anaesthetic implications still poorly known.

Pseudoxanthoma Elasticum in pregnancy - anesthetic and surgical considerations