BACKGROUND: Fibromuscular dysplasia (FMD) and neurofibromatosis type 1 (NF1) are the most common causes of pediatric renal artery stenosis (RAS) in western countries, and characterization of their angiographic features could aid in an accurate diagnosis and in treatment. OBJECTIVE: This study characterizes renal angiographic findings in pediatric fibromuscular dysplasia (FMD) and neurofibromatosis type 1(NF1). MATERIALS AND METHODS: We reviewed 68 angiograms performed over 11 years on 43 children with renovascular hypertension (20 male, 23 females; ages 1 month to -19 years; median/average 9.8 years). Ten patients were diagnosed with NF1, and 33 had presumed FMD. The frequency, extent and distribution of lesions were determined and analyzed. RESULTS: Stenosis was found in 91% of patients (n=39/43), with 86% showing stenosis of 1st or 2nd order arteries, and 12% distal to 2nd order. Stenoses in multiple 1st/2nd order arteries were found in 32% of patients, and 36/43 patients had 1-2 stenoses. The most common lesion was a ≤ 5 mm stenosis in a 1st/2nd order artery, in 42%. Mean percentage of stenosis in a 1st/2nd order vessel was 62%; ≥ 70% stenosis was found in 53%, and ≥ 90% stenosis in 29%. Bilateral disease was noted in 30% of patients. Intraparenchymal disease, distal to 2nd order branches, was seen in 30%. Aneurysms were seen in 28%, beading in 19% and collaterals in 51% (associated with ≥ 70% stenosis). Mid-aortic narrowing was seen in 16%, more often in patients with NF1. CONCLUSION: We provide a descriptive characterization of renal angiographic findings in pediatric FMD and NF1.
BACKGROUND:Fibromuscular dysplasia (FMD) and neurofibromatosis type 1 (NF1) are the most common causes of pediatric renal artery stenosis (RAS) in western countries, and characterization of their angiographic features could aid in an accurate diagnosis and in treatment. OBJECTIVE: This study characterizes renal angiographic findings in pediatric fibromuscular dysplasia (FMD) and neurofibromatosis type 1(NF1). MATERIALS AND METHODS: We reviewed 68 angiograms performed over 11 years on 43 children with renovascular hypertension (20 male, 23 females; ages 1 month to -19 years; median/average 9.8 years). Ten patients were diagnosed with NF1, and 33 had presumed FMD. The frequency, extent and distribution of lesions were determined and analyzed. RESULTS:Stenosis was found in 91% of patients (n=39/43), with 86% showing stenosis of 1st or 2nd order arteries, and 12% distal to 2nd order. Stenoses in multiple 1st/2nd order arteries were found in 32% of patients, and 36/43 patients had 1-2 stenoses. The most common lesion was a ≤ 5 mm stenosis in a 1st/2nd order artery, in 42%. Mean percentage of stenosis in a 1st/2nd order vessel was 62%; ≥ 70% stenosis was found in 53%, and ≥ 90% stenosis in 29%. Bilateral disease was noted in 30% of patients. Intraparenchymal disease, distal to 2nd order branches, was seen in 30%. Aneurysms were seen in 28%, beading in 19% and collaterals in 51% (associated with ≥ 70% stenosis). Mid-aortic narrowing was seen in 16%, more often in patients with NF1. CONCLUSION: We provide a descriptive characterization of renal angiographic findings in pediatric FMD and NF1.
Authors: Richard B Towbin; Daniel J Pelchovitz; Anne Marie Cahill; Kevin M Baskin; Kevin E C Meyers; Bernard S Kaplan; Clare A McClaren; Derek J Roebuck Journal: J Vasc Interv Radiol Date: 2007-05 Impact factor: 3.464
Authors: T A Sos; T G Pickering; K Sniderman; S Saddekni; D B Case; M F Silane; E D Vaughan; J H Laragh Journal: N Engl J Med Date: 1983-08-04 Impact factor: 91.245
Authors: Rebecca Green; Xiaokui Gu; Eva Kline-Rogers; James Froehlich; Pamela Mace; Bruce Gray; Barry Katzen; Jeffrey Olin; Heather L Gornik; Ann Marie Cahill; Kevin E Meyers Journal: Pediatr Nephrol Date: 2015-11-02 Impact factor: 3.714
Authors: Patricia K Castelli; Jonathan R Dillman; David B Kershaw; Shokoufeh Khalatbari; James C Stanley; Ethan A Smith Journal: Pediatr Radiol Date: 2013-09-15