Anesthesia concerns for children with tuberous sclerosis.

Abstract

Tuberous sclerosis (TS) is a relatively rare, autosomal dominant syndrome that displays high genetic penetrance in affected families. It is identified by a classic triad of symptoms including epilepsy, skin lesions, and mental retardation. Tuberous sclerosis causes hamartomas in multiple organ systems, including the brain, skin, heart, kidneys, lungs, and liver. Awareness of the signs and symptoms and the organs involved is critical to provide safe and effective anesthesia care. We describe a 10-year-old girl with TS scheduled to receive a general anesthetic for laser treatment of facial angiofibromas. The patient had several coexisting maladies from TS, including hypertension, autism, seizure disorder, cardiac rhabdomyomas, developmental delay, and bilateral polycystic renal disease. The laser procedure was performed, and there were no surgical or anesthetic complications. However, the potential for complications due to TS remained high throughout the provision of anesthesia care. Increased knowledge of TS and diligence in anesthesia practice can greatly reduce these risks.

• Authors: Steve Septer, Edward S Thompson, Ann Willemsen-Dunlap
• Journal: AANA journal (AANA J) Vol. 74 Issue 3 Pg. 219-25 (Jun 2006) ISSN: 0094-6354 [Print] United States
• PMID: 16786916 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Anesthesia, General (adverse effects, methods, nursing)
• Angiofibroma (etiology, surgery)
• Child
• Developmental Disabilities (etiology)
• Echocardiography
• Facial Neoplasms (etiology, surgery)
• Female
• Genes, Dominant (genetics)
• Heart Neoplasms (diagnostic imaging, etiology)
• Humans
• Hypertension (etiology)
• Incidence
• Laser Therapy
• Nursing Assessment
• Patient Care Planning
• Penetrance
• Perioperative Care (methods, nursing)
• Polycystic Kidney Diseases (etiology)
• Rare Diseases
• Rhabdomyoma (diagnostic imaging, etiology)
• Seizures (etiology)
• Tuberous Sclerosis (diagnosis, epidemiology, genetics, surgery)