Matthew J Rabito1, Alan David Kaye2. 1. Department of Anesthesiology, Louisiana State University Health Sciences Center, New Orleans, LA. 2. Department of Anesthesiology, Louisiana State University Health Sciences Center, New Orleans, LA ; Department of Pharmacology, Louisiana State University Health Sciences Center, New Orleans, LA.
Abstract
BACKGROUND: Tuberous sclerosis complex (TSC), also known as Bourneville disease, is an inherited, progressive neurocutaneous disorder characterized by the potential for hamartoma formation throughout the body. TSC is an autosomal dominant genetic disorder, but more than two-thirds of cases are sporadic. METHODS: Clinical manifestations and treatment options are discussed. Both surgical and anesthetic perioperative considerations are described in this review. RESULTS: Routine monitoring is appropriate for minor surgical procedures for patients with TSC who have mild disease manifestations. More extensive monitoring is indicated for major procedures that have the potential for significant blood loss and for patients with more severe pathology. Postoperatively, TSC patients should be admitted for monitoring and treatment after more extensive procedures or if significant organ dysfunction occurs. Postoperative complications, which may be related to either the surgery or the TSC pathology itself, may have origins in many different organs and may include seizures, severe hypertension, and bradyarrhythmias. CONCLUSION: TSC is a rare disease with a highly variable clinical presentation and provides a multitude of challenges for the patient, the family, and the healthcare team.
BACKGROUND:Tuberous sclerosis complex (TSC), also known as Bourneville disease, is an inherited, progressive neurocutaneous disorder characterized by the potential for hamartoma formation throughout the body. TSC is an autosomal dominant genetic disorder, but more than two-thirds of cases are sporadic. METHODS: Clinical manifestations and treatment options are discussed. Both surgical and anesthetic perioperative considerations are described in this review. RESULTS: Routine monitoring is appropriate for minor surgical procedures for patients with TSC who have mild disease manifestations. More extensive monitoring is indicated for major procedures that have the potential for significant blood loss and for patients with more severe pathology. Postoperatively, TSC patients should be admitted for monitoring and treatment after more extensive procedures or if significant organ dysfunction occurs. Postoperative complications, which may be related to either the surgery or the TSC pathology itself, may have origins in many different organs and may include seizures, severe hypertension, and bradyarrhythmias. CONCLUSION: TSC is a rare disease with a highly variable clinical presentation and provides a multitude of challenges for the patient, the family, and the healthcare team.