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Diagnosis and Treatment of Middle Aortic Syndrome in a Pediatric Patient with Previous Renal Transplant
Jared T Gowen, Lauren Story-Hefta, Mohammed Moursi
University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, AR

INTRODUCTION: Middle aortic syndrome (MAS) is a developmental abdominal aortic coarctation resulting in a segmental narrowing that can affect the renal and visceral arteries. We present a 14-year-old female with past medical history significant for a Nissen fundoplication as an infant, as well as bilateral renal dysplasia resulting in end stage renal disease who underwent a deceased donor kidney transplant in 2014. In 2023 the patient was undergoing routine duplex ultrasound for graft evaluation and it was noted that her aorta appeared diminutive. A CTA was obtained showing significant celiac artery stenosis due to median arcuate ligament compression and narrowing of the abdominal aorta distal to the SMA that extended just proximal to the aortic bifurcation where her renal allograft was implanted. She had hypertension on losartan but normal creatinine and renal function. Her femoral pulses were diminished and she reported symptoms consistent with life-limiting claudication.
METHODS: On 6/8/2023, the patient underwent an open repair procedure which included a supra-celiac to distal aortic bypass, as well as a median arcuate ligament release. A subcostal incision was used and the viscera were rotated to the right. An occlusive supraceliac clamp was placed and a bypass was performed from the supraceliac aorta to the infrarenal aorta just above the renal transplant using 12 mm ringed PTFE via a left retrorenal tunnel. The proximal anastomosis was performed in an end-to-side fashion and the distal anastomosis was performed in an end-to-end fashion using running prolene suture. Clamp time was 16 minutes in total, and during this time the transplanted kidney was placed on ice for renal protection. Post-operatively, distal pulses were palpable and urine output was adequate. Her postoperative course was relatively benign aside from a left pleural effusion requiring a pigtail catheter for drainage, and an AKI with a peak creatinine of 1.1.
RESULTS: At the 1-year postoperative appointment, she was seen in clinic with no complications. Her blood pressure medication had been cut in half. Her creatinine normalized. The patient additionally reported vastly improved lower extremity exertional symptoms.
CONCLUSIONS: This is a unique presentation of MAS. She presented with an incidental finding on routine imaging, however after further investigation it was found that she was symptomatic regarding her hypertension and claudication with impeded blood flow to the transplanted kidney and her bilateral lower extremities. Her life-limiting claudication, along with the need to preserve adequate flow to her renal allograft were both indications to proceed with operative repair. She is now a 10th grade girl who is able to run and jump just like her peers.


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