PMID- 37915684
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20231103
IS  - 2049-0801 (Print)
IS  - 2049-0801 (Electronic)
IS  - 2049-0801 (Linking)
VI  - 85
IP  - 11
DP  - 2023 Nov
TI  - Rare giant renal artery aneurysm in neurofibromatosis type 1 patient: a case 
      report.
PG  - 5748-5751
LID - 10.1097/MS9.0000000000001329 [doi]
AB  - INTRODUCTION AND IMPORTANCE: Neurofibromatosis type 1 (NF1) is a genetic disorder 
      characterised by multiple neurofibromas, cafe-au-lait spots, and iris hamartomas. 
      The variety of vasculopathies that can occur in NF1 make it difficult for 
      clinicians to accurately follow-up patients. Most cases of vasculopathies are 
      stenotic, and, in few cases, aneurysms may form. CASE PRESENTATION: A 35-year-old 
      male presented with extreme left flank pain for the past 2 days. His physical 
      examination revealed whole-body several cafe-au-lait skin macules, a subcutaneous 
      lesion, and a palpable abdominal mass in the left flank. His laboratory workup 
      was within normal ranges. A multi-slice computed tomography and computed 
      tomography angiogram with contrast outlined a giant left renal artery aneurysm 
      (RAA). A kidney salvage surgery was planned. However, due to ectatic dilatation 
      and large extension of the aneurysm, the affected renal artery branches and renal 
      vein were found unfit for auto-transplantation during the surgical procedure and 
      a total nephrectomy was necessary. Symptoms improved significantly 
      postoperatively and no complications developed. CLINICAL DISCUSSION: RAA is an 
      uncommon finding in NF1 patients. Diagnosis is often dependent on computed 
      tomography angiogram. Management techniques are conservative, endovascular, or 
      surgical. In few surgical cases, a total nephrectomy may be necessary if 
      auto-transplantation is not feasible. CONCLUSION: Despite its rarity, the 
      diagnosis of RAA should be considered in patients with NF1 presenting with flank 
      pain. Moreover, early screening for renal vasculopathies can evade critical 
      surgical outcomes including a total nephrectomy. Hence, the authors recommend a 
      total vascular workup for these patients, consisting of doppler ultrasound and, 
      if necessary, a multi-slice computed tomography with contrast.
CI  - Copyright (c) 2023 The Author(s). Published by Wolters Kluwer Health, Inc.
FAU - Jawad, Ali
AU  - Jawad A
AD  - Faculty of Medicine, Damascus University.
FAU - Hannouneh, Zein Alabdin
AU  - Hannouneh ZA
AD  - Faculty of Medicine, Al Andalus University for Medical Sciences, Tartus, Syrian 
      Arab Republic.
FAU - Soqia, Jameel
AU  - Soqia J
AD  - Faculty of Medicine, Damascus University.
FAU - Al Nahhas, Zaher
AU  - Al Nahhas Z
AD  - Department of Radiology, Damascus Hospital, Damascus.
FAU - Ahmed, Adnan
AU  - Ahmed A
AD  - Department of Urology, Faculty of Medicine.
FAU - Nahas, Mohamad Ali
AU  - Nahas MA
AD  - Head of Vascular and Endovascular Surgery Division, Al-Assad University Hospital, 
      Damascus University.
LA  - eng
PT  - Journal Article
DEP - 20230915
PL  - England
TA  - Ann Med Surg (Lond)
JT  - Annals of medicine and surgery (2012)
JID - 101616869
PMC - PMC10617891
OTO - NOTNLM
OT  - case report
OT  - nephrectomy
OT  - neurofibromatosis type 1
OT  - renal artery aneurysm
OT  - vasculopathy
COIS- The authors declare no conflict of interest.
EDAT- 2023/11/02 06:43
MHDA- 2023/11/02 06:44
PMCR- 2023/09/15
CRDT- 2023/11/02 04:04
PHST- 2023/08/26 00:00 [received]
PHST- 2023/09/10 00:00 [accepted]
PHST- 2023/11/02 06:44 [medline]
PHST- 2023/11/02 06:43 [pubmed]
PHST- 2023/11/02 04:04 [entrez]
PHST- 2023/09/15 00:00 [pmc-release]
AID - AMSU-D-23-01856 [pii]
AID - 10.1097/MS9.0000000000001329 [doi]
PST - epublish
SO  - Ann Med Surg (Lond). 2023 Sep 15;85(11):5748-5751. doi: 
      10.1097/MS9.0000000000001329. eCollection 2023 Nov.