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Neuraxial Anesthesia for Assisted Vaginal Delivery in Parturient with Autosomal Dominant Tuberous Sclerosis Complex
Abstract Number: RF3BC-417
Abstract Type: Case Report Case Series
Tuberous sclerosis complex (TSC) is an autosomal dominant multiorgan disease, resulting from mutations in TSC1 or TSC2 genes, that cause benign hamartomas. CNS lesions cause epilepsy and learning disabilities. Large, vascular renal angiomyolipomas (AMLs) are second leading cause of premature death. Ventricular rhabdomyomas are highly specific and often seen in prenatal ECHO of an affected fetus. Other affected organs include lungs (Lymphangioleiomyomatosis/LAM, cystic parenchymal disease), lymphatic system and retina. (1) (2)
Literature on pregnancy outcomes in TSC patients is limited but increased risk of preeclapmsia, preterm labor, fetal growth restriction, oligo/poly-hydramnios, and fetal demise has been observed (3) (4). Other possible complications include rupture of large renal AMLs during labor or worsening of underlying pulmonary disease or seizure disorder (5) (6).
We present a case of 34 year old G2P1 with TSC complicated by CKDIII secondary to large bilateral renal AMLs with near complete replacement by fatty tissue, seizures (on Levetiracetam), and pulmonary LAM. MRI brain showed multiple hamartomas but no mass effect, hydrocephalus or hemorrhage. Maternal TTE was normal but fetal ECHO at 30w4d revealed multiple rhabdomyomas (largest 2.6 cm) with moderate tricuspid regurgitation, in addition to CNS and renal lesions. From multidisciplinary meeting, IOL was recommended with assisted second stage to avoid maternal Valsalva due to concern for spontaneous rupture of renal AMLs. We decided to avoid GETA and PPV in setting of known cystic parenchymal disease. MRI lumbar spine was obtained to rule out spinal AVMs which showed multiple sclerotic spinal lesions. Neuroradiology was consulted and recommended L paramedian approach at L4-5 for neuraxial anesthesia. Actual induction was at 36w5d, earlier than originally planned, due to concern for atypical preeclampsia with transaminitis and rising Creatinine. IR and Cell Saver were consulted for possible emergent embolization. Inpatient Nephrology followed patient closely. CSE was performed on hospital day 3. Nitrous oxide was avoided for reasons discussed above. Patient progressed to complete on day 4, followed by uncomplicated FAVD (EBL 100 cc) and Apgar scores of 8 and 8.
Overall, a thorough evaluation of risks associated with labor and anesthesia in this patient with extensive multi-organ disease was performed. A safe anesthetic plan with multidisciplinary involvement was developed that resulted in successful vaginal delivery.
1. Leung AK et al., 2007, Journal of Pediatric Health care, pp. 108-14
2. Northup H et al., 2013, Pediatric Neurology, pp. 243-254
3. Terry AR et al., 2015, American Journal of obstetrics and gynecology, pp. 108-109
4. King, JA et al., 2005, American Journal of Perinatology, pp. 103-8.
5. Khaddoura K et al. 2019, Respiratory Medicine Case Reports, pp. 63-67
6. Iruloh C et al., 2013, Journal of Obstetrics and Gynecology, pp. 542-546