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      Renal artery aneurysm induced by neurofibromatosis type 1: A case report and review of the endovascular interventions for this rare vasculopathy

      research-article
      , MD, FRCSC, FACS, RVPI a , * ,
      Medicine
      Lippincott Williams & Wilkins
      endovascular interventions, neurofibromatosis type 1, renovascular hypertension, renal artery aneurysm

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          Rationale:

          The autosomal dominant condition known as neurofibromatosis type 1 (NF-1) is characterized by café au lait macules and neurofibromatosis. Aneurysms in renal arteries are rare. Renal artery aneurysm (RAA) can be successfully treated with endovascular procedures; however, successful cases in NF-1 adults have not been reported.

          Patient concerns:

          Here, we report the case of a 30-year-old female suffering from NF-1. The patient presented to the emergency department with complaints of chronic, poorly controlled hypertension. A left RAA was found om the computed tomography angiography (CTA).

          Diagnoses:

          A left renal artery aneurysm was diagnosed using CTA during workup for secondary hypertension.

          Interventions:

          Selective angiographym of the left renal artery confirmed a fusiform aneurysm of the distal renal artery. A self-expandable covered stent was placed, and a completion angiogram demonstrated good aneurysm sealing and contrast flow to the left kidney.

          Outcomes:

          The patient’s blood pressure improved after the procedure. Her medications were lowered to almost half of their baseline doses, and hydralazine was discontinued. On the follow-up visit after 4 months, the patient reported his home-measured systolic blood pressure to be less than 120 mm Hg. A repeated CTA of the abdomen showed post-left RAA repair with a covered stent and interval improvement of the left kidney.

          Lessons:

          RAA caused by NF-1 are manageable and feasible with endovascular intervention.

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          Most cited references23

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          Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference.

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            Is Open Access

            Revised diagnostic criteria for neurofibromatosis type 1 and Legius syndrome: an international consensus recommendation

            Purpose By incorporating major developments in genetics, ophthalmology, dermatology, and neuroimaging, to revise the diagnostic criteria for neurofibromatosis type 1 (NF1) and to establish diagnostic criteria for Legius syndrome (LGSS). Methods We used a multistep process, beginning with a Delphi method involving global experts and subsequently involving non-NF experts, patients, and foundations/patient advocacy groups. Results We reached consensus on the minimal clinical and genetic criteria for diagnosing and differentiating NF1 and LGSS, which have phenotypic overlap in young patients with pigmentary findings. Criteria for the mosaic forms of these conditions are also recommended. Conclusion The revised criteria for NF1 incorporate new clinical features and genetic testing, whereas the criteria for LGSS were created to differentiate the two conditions. It is likely that continued refinement of these new criteria will be necessary as investigators (1) study the diagnostic properties of the revised criteria, (2) reconsider criteria not included in this process, and (3) identify new clinical and other features of these conditions. For this reason, we propose an initiative to update periodically the diagnostic criteria for NF1 and LGSS.
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              The Society for Vascular Surgery clinical practice guidelines on the management of visceral aneurysms

              These Society for Vascular Surgery Clinical Practice Guidelines describe the care of patients with aneurysms of the visceral arteries. They include evidence-based size thresholds for repair of aneurysms of the renal arteries, splenic artery, celiac artery, and hepatic artery, among others. Specific open surgical and endovascular repair strategies are also discussed. They also describe specific circumstances in which aneurysms may be repaired at smaller sizes than these size thresholds, including in women of childbearing age and false aneurysms. These Guidelines offer important recommendations for the care of patients with aneurysms of the visceral arteries and long-awaited guidance for clinicians who treat these patients.
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                Author and article information

                Journal
                Medicine (Baltimore)
                MD
                Medicine
                Lippincott Williams & Wilkins (Hagerstown, MD )
                0025-7974
                1536-5964
                07 July 2023
                07 July 2023
                : 102
                : 27
                : e34216
                Affiliations
                [a ] Division of Vascular Surgery, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
                Author notes
                * Correspondence: Sultan AlSheikh, Division of Vascular Surgery, Department of Surgery, College of Medicine, King Saud University, 11322 Riyadh, Saudi Arabia (e-mail: sualsheikh@ 123456ksu.edu.sa ).
                Author information
                https://orcid.org/0000-0003-4528-1662
                Article
                00044
                10.1097/MD.0000000000034216
                10328702
                37417633
                252d46e6-8a50-4054-88ad-5c2566a548bf
                Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 20 April 2023
                : 15 June 2023
                Categories
                7100
                Research Article
                Clinical Case Report
                Custom metadata
                TRUE

                endovascular interventions,neurofibromatosis type 1,renovascular hypertension,renal artery aneurysm

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