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Case report: Anesthetic management of a pregnant patient with tuberous sclerosis.
Abstract Number: F-49
Abstract Type: Case Report/Case Series
21 year old gravida 1 para 0 female at 36 weeks gestation, with past medical history of sickle cell trait and tuberous sclerosis with associated epilepsy, was admitted to the antepartum ward with intrauterine growth restriction. Despite antiepileptic medication, the patient continued to have weekly seizures. The patient remained in the antepartum unit for 2 weeks until she underwent a planned trial of labor with oxytocin, which occurred at 38 weeks and 1 day.
The patient received an epidural for pain control during her trial of labor. Due to failure to progress, the decision was made to proceed with a primary cesarean delivery. Unfortunately, the epidural was not working appropriately and the patient refused replacement of the epidural. So the anesthesia team elected to proceed with general anesthesia and endotracheal intubation. No imaging was performed during her admission and prior to interventions performed by the anesthesia team. The intraoperative course was complicated by right uterine artery extension and uterine atony, with an estimated blood loss of 1500mL. The patient received 15-methylprostaglandin F2 alpha and methylergonovine, which resolved the atony. For the duration of the procedure, the patient was tachycardic, but otherwise hemodynamically stable and able to maintain normal oxygen saturations. The patient was extubated in the operating room without difficulty and her post-operative evaluation revealed no apparent complications. The patient’s postoperative course was unremarkable.
Tuberous sclerosis is an autosomal dominant genetic disorder that is characterized by epilepsy, cognitive deficits and/or learning disabilities, skin lesions, and benign tumors in multiple sites. The most common renal lesions are angiomyolipomas, which can lead to renal hemorrhage and kidney disease. Some patients may develop pulmonary lesions known as lymphangioleiomyomatosis, which can lead to pulmonary hemorrhage and pneumothorax intraoperatively. Similarly, hamartomas, tumors, and other lesions may develop in the central nervous system and are also at risk for bleeding (1,2). Few case reports exist regarding the anesthetic management of a pregnant patient with tuberous sclerosis. This case report highlights the potential complications that can occur during anesthetic management of these patients and the role of multidisciplinary management that would have allowed for appropriate imaging prior to induction of labor.
1. Causse-Mariscal A, Palot M, Visseaux H, Daigremont-Botmans C, Malinovsky JM. Labor analgesia and cesarean section in women affected by tuberous sclerosis: report of two cases. Int J Obstet Anesth. 2007 Jul;16(3):277-80. Epub 2007 Mar 6.
2. Petrikovsky BM, Vintzileos AM, Cassidy SB, Egan JF. Tuberous sclerosis in pregnancy. Am J Perinatol. 1990 Apr;7(2):133-5. Review.