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Abstract: TH-PO492

Renal Autotransplant in a Pediatric Patient with Neurofibromatosis Type 1 (NF1) and Renal Artery Stenosis (RAS)

Session Information

  • Pediatric Nephrology - I
    November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Beebe, Morgan Elizabeth, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Wala, Samantha Jane, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Kallash, Mahmoud, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Nathan, Jaimie D., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Rasmussen, Sara Kirsten, Nationwide Children's Hospital, Columbus, Ohio, United States
Introduction

Patients with NF1 are at increased risk for developing HTN, with RAS being the most common etiology. When medication fails, surgery may be indicated. We present a case of a 6-year-old boy with NF1 and RAS whose HTN was refractory to medical therapy and balloon dilation, but successfully treated with renal autotransplant.

Case Description

A 6-year-old boy with NF1 presented to our nephrology clinic with HTN. While his laboratory findings were unrevealing, computed tomography angiography (CTA) of the renal vessels revealed high-grade stenosis of the proximal right renal artery. He subsequently underwent arterial dilation and balloon angioplasty performed with IR. Following this, he presented in hypertensive urgency on dual-medical therapy. Repeat CTA redemonstrated severe right-sided stenosis; IR deemed the patient inappropriate for re-intervention. With 45% function of the right kidney on MAG-3 scan, our surgical colleagues offered renal autotransplant for consideration, which the family accepted. Six months postoperatively, his antihypertensive burden decreased from four-drug therapy to amlodipine monotherapy, with home blood pressures averaging 100/60 mmHg.

Discussion

Patients with NF1 are at increased risk of developing HTN. In those with RAS, a stepwise treatment approach from least-to-most invasive is preferred. If multidrug therapy and endovascular interventions fail, nephrectomy is then considered. Because stenosis may recur in the remaining kidney, renal autotransplant can be a preferred intervention for the treatment of refractory HTN with RAS in patients with preserved renal function. While more technically challenging in pediatric patients, this intervention confers the benefit of retaining the native kidney.

Graft in situ