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Cesarean delivery in a patient with tuberous sclerosis and lymphangioleiomyomatosis (LAM)
Abstract Number: F-51
Abstract Type: Case Report/Case Series
Our patient is a 31 year old gravida 2, para 1 female who presented for preoperative evaluation prior to her scheduled cesarean delivery. The patient is 173cm and 72 kg. Her medical history is significant for tuberous sclerosis complicated by cortical and ocular tubers however she denies any focal neurological deficits. Her past surgical history includes Cesarean delivery (2013) and right pneumonectomy (2010) for recurrent pneumothoraces. Her baseline pulmonary function tests (PFTs) demonstrated a mild obstructive and severely restrictive ventilatory defect. Additionally, she has lymphangioleiomyomatosis (LAM) syndrome that is complicated by renal/hepatic angiomyolipomas. Renal and hepatic function tests are normal. She also notes sacroilitis complicated by low back pain. The patient’s transthoracic echocardiogram showed normal cardiac function and anatomy. Third trimester fetal echocardiogram revealed a mild-to-moderate LVOT obstruction. Her exercise tolerance was not limited on the day of initial consult; she is employed as an engineer which requires moderate physical activity and denies dyspnea despite her respiratory pathology. Her medications include albuterol, which she uses infrequently, and prenatal vitamins. She has a Mallampati class one airway with full range of neck motion, a thyromental distance of 7 cm, and good mouth opening. Examination of her spine showed no obvious deformity. Her delivery plan is for repeat Cesarean delivery in order to limit maternal morbidity associated with LAM syndrome. Her anesthetic plan is for spinal anesthesia with bupivacaine, fentanyl, and morphine. Pregnant patients with tuberous sclerosis are at increased risk for adverse maternal and fetal outcomes. The pregnancy may be complicated by preterm labor, preeclampsia, and fetal demise (1). Women with LAM syndrome are often advised to avoid pregnancy due to higher rates of pneumothorax, premature birth, and miscarriage (2). Patients with a reduced pulmonary reserve after pneumonectomy may have worsening of respiratory symptoms during pregnancy secondary to the increased oxygen consumption and minute ventilation and decreased functional residual capacity (FRC). Spinal anesthesia was safely employed in this patient for a repeat urgent Cesarean delivery and was without complication.
1. "Maternal and Fetal Tuberous Sclerosis Complicating Pregnancy: A Case Report and Overview of the Literature" Jeremy A King, MD and David M Stamilio, MD, MSCE. American Journal of Perinatology (2005) 22, 103-108.
2. "Pregnancy experiences among women with lymphangioleiomyomatosis" Marsha M Cohen, Anette M Freyer, Simon R Johnson. Respiratory Medicine (2009) 103, 766-772.