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Open Emergency Repair of a Thoracoabdominal Aortic Aneurysm on a 12-Year-Old Boy With Tuberous Sclerosis

Julian M Corso-Ramirez1, Mariana Molina López1, Paula Camila Flórez2

  • 1Vascular and Endovascular Surgery Research Group, Fundación CardioInfantil- La Cardio, Bogotá D.C, Colombia.

Vascular and Endovascular Surgery
|June 15, 2023
PubMed

Insights

Tuberous sclerosis complex (TSC) in a pediatric patient led to a large thoracoabdominal aortic aneurysm. Surgical repair was successful, and the patient was diagnosed with de novo TSC.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Pediatric Neurology

Background:

  • Tuberous sclerosis complex (TSC) is a genetic disorder causing tumors and affecting multiple organs.
  • Vascular anomalies are common in pediatric TSC patients.
  • Aortic aneurysms have been associated with TSC.

Observation:

  • A 12-year-old boy presented with a large Crawford type IV thoracoabdominal aortic aneurysm (97 × 70 mm).
  • The patient had no prior diagnosis of TSC.
  • The aneurysm was a de novo manifestation.

Findings:

  • Open surgical repair of the aortic aneurysm was performed using a multibranched Dacron graft.
  • The patient was diagnosed with Tuberous Sclerosis Complex (TSC) following clinical and imaging evaluation.
  • The surgical repair was successful with an uneventful recovery.

Implications:

  • This case highlights the potential for severe vascular complications, like aortic aneurysms, in pediatric TSC.
  • Early diagnosis and management of TSC are crucial for preventing or treating associated vascular issues.
  • Surgical intervention can be effective for managing large aortic aneurysms in young patients with TSC.

Keywords:
Tuberous sclerosiscase reportpediatric aneurysmsthoracoabdominal aortic aneurysm

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