Spinal anesthesia for scheduled cesarean delivery in a patient with phacomatosis pigmentovascularis type V.

Abstract

Background:

Phacomatosis pigmentovascularis (PPV) is a rare and usually sporadic congenital disorder marked by the coexistence of vascular malformations and pigmentary mosaicism. Although many patients have predominantly cutaneous disease, extracutaneous involvement (neurologic, ophthalmologic, musculoskeletal, and vascular) has been reported. In pregnancy physiological changes and peripartum hypercoagulability may exacerbate vascular complications. In these cases safety of neuraxial anesthesia depends on careful assessment for neuraxial or intracranial vascular malformations and appropriate anticoagulation management.

Case report:

A 30-year-old primigravida at 39 weeks’ gestation with PPV type V (phacomatosis cesiomarmorata; cutis marmorata telangiectatica congenita with dermal melanocytosis) was scheduled for elective cesarean delivery. Comorbidities included bilateral ocular melanosis with raised intraocular pressure and a prior right lower-limb deep venous thrombosis 4 years earlier. Pregnancy was otherwise uncomplicated apart from diet-controlled gestational diabetes. She received enoxaparin 40 mg twice daily during pregnancy, brinzolamide eye drops and cetirizine. Enoxaparin was withheld for 24 hours preoperatively. Examination revealed characteristic pigmentary lesions; cardiopulmonary and neurologic findings were normal. Hematologic and coagulation tests and echocardiography were unremarkable. Electrocardiography showed a short PR interval. Following multidisciplinary review, spinal anesthesia was performed at L4–L5 with hyperbaric bupivacaine 10 mg, fentanyl 20 μg, and morphine 100 μg. Cesarean delivery and recovery were uneventful with stable hemodynamics and effective postoperative analgesia.

Conclusion:

Cesarean delivery under spinal anesthesia can be safely accomplished in pregnant women with PPV when individualized multidisciplinary evaluation excludes clinically significant systemic involvement. Neuraxial risk is minimized through strict anticoagulation timing and vigilant perioperative monitoring.