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Anesthetic Management of an Obstetric Patient with Lymphangioleiomyomatosis
Abstract Number: SUN-25
Abstract Type: Case Report/Case Series
We present a case report of a 33 year old G1P0 at 37 weeks who arrived for induction of labor due to chronic placental abruption. Her past medical history includes lymphangioleiomyomatosis (LAM), bilateral renal angiomyolipomas (AML), hypertension, type II diabetes, asthma, transverse myelitis at 13 years old (residual right foot drop), and morbid obesity (BMI 46). Five years prior, rupture of an AML required blood transfusion and embolization; however, the diagnosis of LAM was not confirmed until she presented with hemoptysis in the 1st trimester of this pregnancy. Imaging confirmed multiple thin-walled cystic lesions throughout the lungs. During the 2nd trimester, she presented for another embolization of her renal AMLs due to concern for rupture of a rapidly enlarging AML (largest was 18 cm). Of note, she had a possible cortical tuber on a MRI Head scan, although genetic testing for tuberous sclerosis was non-diagnostic. After a multidisciplinary meeting, the plan was to have packed red blood cells typed and crossed, standard monitoring, additional peripheral IV access, and a labor epidural for pain control and hemodynamic stability. She was subsequently diagnosed with severe preeclampsia during her induction and was started on IV magnesium therapy. The remainder of her labor progressed uneventfully. She delivered vaginally, a healthy baby boy without any further interventions and was discharged on postpartum day 2.
Lymphangioleiomyomatosis (LAM) is a rare disorder resulting from abnormal smooth muscle proliferation in the lung, kidney, and axial lymphatics. Cystic destruction of the lung with progressive pulmonary dysfunction and the presence of abdominal tumors (eg. angiomyolipomas, lymphangioleiomyomas) characterize the disease. LAM typically occurs in premenopausal women, suggesting some involvement of hormones in disease pathogenesis. Thus, pregnancy may be advised against due to the possibility of disease exacerbation. Additionally, about 30% of patients diagnosed with tuberous sclerosis, a genetic disorder that results in benign tumor growths, have LAM. Patients are at increased risk for pneumothoraces, lymphatic obstruction, hemorrhage from blood vessels due to increased blood volume in pregnancy, and neurologic complications if the central nervous system is involved. This case report highlights the importance of prior imaging and a multidisciplinary approach to management of a patient with a complicated and rare disease.
References: 1. Moss, J. Lymphangioleiomyomatosis. 2015 Jul 27, 2015; Available from: http://emedicine.medscape.com/article/299545-overview.