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Perioperative management of a parturient with severe preeclampsia, lymphangioleiomyomatosis, and respiratory failure on veno-venous extracorporeal membrane oxygenation (ECMO) undergoing cesarean section.
Abstract Number: F-48
Abstract Type: Case Report/Case Series
Extracorporeal membrane oxygenation (ECMO) provides a bridge for oxygenation and ventilation for patients with cardiopulmonary failure. ECMO has been used in pregnancy to manage acute respiratory distress syndrome (ARDS) secondary to H1N1 influenza infection, with one case describing successful cesarean section (CS) performed while on ECMO (1,2). We describe the first case of a parturient with hypoxemic respiratory failure secondary to H1N1 influenza and lymphangioleiomyomatosis (LAM), requiring ECMO support, who underwent urgent CS for preeclampsia and HELLP syndrome. A healthy 37 year old G2P0010 parturient at 21 weeks gestation was admitted to an outside hospital for H1N1 pneumonia following 5 days of cough and fever. She developed severe ARDS, was intubated, and then transferred to our facility for further management. Chest CT demonstrated diffuse cystic changes consistent with lymphangioleiomyomatosis. Veno-venous ECMO was initiated on day 16 for refractory hypoxemia secondary to diffuse alveolar hemorrhage, but anticoagulation was withheld to prevent further bleeding. On hospital day 21, at 24 weeks 3 days gestation, the patient developed severe preeclampsia and HELLP syndrome and was taken to the operating room for urgent CS. Perioperative anesthetic management focused on mitigating the potential risks of disseminated intravascular coagulation (DIC), severe hemorrhage from surgical bleeding and uterine atony, and cardiovascular collapse from ECMO circuit thrombosis. A balanced anesthetic with isoflurane, fentanyl, and dexmedetomidine minimized the risk of iatrogenic uterine atony. Vascular cystic lesions consistent with LAM were noted within the myometrium during hysterotomy, and ongoing bleeding was noted from the hysterotomy site after repair. A full complement of blood products and uterotonics were administered for estimated loss of 25% of total blood volume; DIC did not develop. Adequate ECMO flow rates and oxygenation were maintained throughout the case. She remained intubated postoperatively and was successfully weaned from ECMO 3 days later. LAM is a rare estrogen-responsive systemic disease found primarily in women of childbearing age, characterized by abnormal smooth muscle proliferation and cystic destruction of the lungs, uterus, and other organs (3). Hormonal and immunosuppressive therapy may slow disease progression, but severe cases may require lung transplantation. The juxtaposition of hypoxemic respiratory failure with risk of massive hemorrhage and thrombosis encountered in this challenging case is of particular interest to the obstetric anesthesiologist.
1. Brown CM: Severe influenza A virus (H1N1) infection in pregnancy. Obstetr Gynecol 2010, 115:412-414.
2. Panarello et al. Cesarean section during ECMO support. Minerva Anestesiol 2006, 77(6): 654-657.
3. Gargari S, et al. Pregnancy complicated by lymphangioleiomyomatosis. Arch Iranian Med 2009; 12(2):182–185.