Background and Aims Pseudoxanthoma elasticum (PXE) is a rare heritable disorder of connective tissue calcification that mainly affects skin, eyes and cardiovascular system. There are several disease features that may influence the anaesthetic management such as the development of arrhythmias, premature ischaemic heart disease, difficult airway management, haemorrhagic complications including the theoretical risk of epidural hematoma. Since there’s only a few cases described in the literature, we aim to report a case of neuro-axial labour analgesia in a parturient with PXE.

Methods This case reports a 39-year-old nulliparous woman with PXE diagnosed at the age of 29 with the typical skin and ophthalmological features. Cardiac evaluation ruled out any abnormalities. She had no previous history of anaesthetic procedures and she was diagnosed with gestational diabetes controlled with diet. At 38-week gestation the labour was induced due to premature rupture of membranes and an epidural catheter was inserted at L3-L4 for labour analgesia under ASA standard monitoring. After an initial bolus of 0.2% ropivacaine together with sufentanil the analgesia was maintained with 0.2% ropivacaine boluses hourly.

Results During labour she remained hemodynamically stable with a good analgesic control. Four hours later a female newborn was born via eutocic uneventful delivery. Neurologic evaluation was performed in the postpartum period excluding any complications from epidural catheter placement.

Conclusions A good analgesic control is essential for preventing hemodynamic changes that might be harmful for these patients overcoming the risk of epidural hematoma. The rare cases of this disease make it essential to report the anaesthetic management especially during pregnancy.