Interesting Cases CRT 2013 Nishant Gupta MD PDCC

Interesting Cases CRT 2013 Nishant Gupta MD PDCC, Rachel F. Oser MD UAB at Birmingham, Alabama

Clinical Scenario • • 5 -yr-old boy H/o Tuberous sclerosis Low IQ, Cutaneous features of TS Incidentally detected bilateral renal Angiomyolipoma (AML) • Left sided- 5. 3 cm X 4. 4 cm X 3. 2 cm (Long. X AP X Lat. ) • Right sided- 1. 0 cm X 0. 9 cm X 0. 4 cm (Long. X AP X Lat. )

Imaging Left Renal AML Coronal Axial

What to Do Next? • Nothing • Medical management • Surgery • Local percutaneous treatment • Endovascular Management- TOC

Pre-Procedure Details • BMP • Renal function tests • CBC • Coagulation profile • • PT PTT INR Platelet count

Cincinnati Protocol Reduction of Post-Angiomyolipoma-Embolization-Syndrome (PES) Pre-embolization For reduction of Post. Angiomyolipomaembolization syndrome • Prophylactic first-generation cephalosporin • Intravenous dose of 250 mg/m 2 of methylprednisolone (maximum dose, 260 mg) Post-embolization • 4 doses of antibiotics, with subsequent oral dosing • POD-1: 2 mg/kg of prednisone in 3 divided doses per day for 2 days, with a maximum dose of 60 mg/d • Every 2 days, the dose was tapered to finish approximately 14 days after embolization • For the first day after embolization, patients also were administered acetaminophen every 6 hours orally if needed Bissler et al. Am J of Kid Dis. Vol. 30, 2002; pg 966 -71

Procedural Details • • General Anesthesia Femoral Route, Seldinger’s Technique Dilute contrast media with saline (50: 50 dilution) Aortogram Selective renal angiograms Micro catheter (2. 4 Fr) and Glidewire (0. 018“) Embolization Particles (Embozene. TM) • 250 -350 microns

Procedural Details

- Post embolization

Follow-up • Pain • VAS • Fever During and after 2 week course of steroids • > 100. 5 F • Serum creatinine levels • Before • 1 month after embolization • Follow-up imaging (CT/MR) after 3 months

Follow-up

Discussion • Tuberous Sclerosis Complex (TSC)- Autosomal Dominant inherited disease • 75% patients with TSC develop renal AML • Distort and damage renal parenchyma • Can lead to hemorrhage (microaneurysms & macroaneurysms as well as dysplastic arteries) • Renal failure • Long term complication • Leading cause of death in adults Ou YC et al. Chung Hua I Hsueh Tsa Chih (Taipei) 48: 217 -223, 1991 Hartmut PH et al. Semin Pediat r Neurol 5: 269 -275, 1999 Clarke A et al. Nephrol Dial Transplant 14: 988 -991, 1999

Renal Angiomyolipoma • Indication of embolization • Prophylactic embolization of AML with size >4 cm • Acute hemorrhage even with smaller AML • PES • Symptoms similar to PES of any other organ • Frequently seen (60 -70% cases) • Significant reduction using Cincinnati Protocol • Increased Patient tolerance • More acceptance • Reproducible results

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