#6931 TWO CASES OF PREGNANCY IN PATIENTS WITH TUBEROUS SCLEROSIS COMPLEX

Abstract

Abstract Background and Aims Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder that is characterized by formation of benign tumors of the brain, kidney, eyes, heart, lung, liver and skin. The disease is frequently associated with adverse pregnancy outcomes. Our aim is to present two cases of Tuberous sclerosis complex in pregnancy which might be illustrative in the management of these patients. Method The first patient was a 32-year-old woman with a history of renal angiomyolipoma (AML) and pulmonic lymphangiomyomathosis (LAM) due to Tuberous sclerosis complex diagnosed at a young age. In the 35th gestational week she had an emergency Cesarean section due to acute retroperitoneal bleeding from angiomyolipoma of the right kidney and gave birth to a preterm male infant (birth mass 2077 g, Apgar 4/6). The patient did not take any medications during the course of pregnancy. Since 2015 she is being treated with everolimus (5 mg/day) due to complications for pulmonic lymphangiomyomathosis, subependymomas and epilepsy and AMLs. After eight years of treatment renal AMLs and LAM and subependymomas show no dynamic in size or number. The second patient was a 29-year-old nulliparous woman who has presented with seizures at the age of 2 and was diagnosed with TSC. At 37th gestational week of a twin pregnancy she gave birth to one male neonate (birth mass 2660 g, Apgar 8/9), while the other fetus died in utero. During the course of the pregnancy she was treated with phenobarbital, folic acid and iron supplementation. Her epilepsy is well-controlled and AMLs show no dynamic in size or number. Results: Conclusion We have showed two cases with relatively good outcomes which have long-term stable disease. Female patients with TSC have increased risk of hemorrhagic complications of AMLs during pregnancy and it can be complicated by intrauterine growth restriction. A multidisciplinary approach is needed in the planning and monitoring pregnancies in these patients to achieve optimal outcomes.