Anesthesia for cesarean in patient with lymphangioleiomyomatosis

Case report

Abstract

Introduction: Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology, classically described in reproductive age women and, occasionally, reported in postmenopausal. Gestation in these patients is high risk, since the physiological changes of gestation add to those of LAM, worsening maternal cardiorespiratory status. Case Report: A 29-year-old pregnant woman with LAM and collapsing segmental and focal glomerulosclerosis (ESRF) diagnosed two years before had an indication of interruption of pregnancy during the 35th gestational
week, due to worsening renal function. Spirometer examination revealed significant restrictive disorder. She was hospitalized during the 34th week of gestation and underwent steroid therapy for fetal lung maturation, when worsening of renal function and cesarean section were indicated. Combined regional anesthesia was chosen. Comments: LAM has a prevalence of 1 to 2.6 / 1,000,000 women. It is associated with Tuberous Sclerosis or idiopathic one.. Combined regional anesthesia was adopted because of the difficulty in managing the airway. Hormonal factors appear to play a role in the initiation and progression of LAM.