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Renal nutcracker syndrome

MedGen UID:
465238
•Concept ID:
C3178770
•
Disease or Syndrome
Synonyms: Nutcracker Syndrome, Renal; Nutcracker Syndromes, Renal; Renal Nutcracker Syndrome; Renal Nutcracker Syndromes; Renal Vein Entrapment Syndrome
SNOMED CT: Left renal vein entrapment syndrome (717267005); Renal nutcracker syndrome (717267005); Left renal vein (LRV) entrapment syndrome (717267005); NCS - nutcracker syndrome (717267005); Mesoaortic compression of left renal vein (717267005)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0019105
Orphanet: ORPHA71273

Definition

Compression of the left renal vein between the superior mesenteric artery and the abdominal aorta causing an increase in the pressure gradient between the left renal vein and the inferior vena cava. The thin septae between the veins and the collecting system in the renal fornices rupture, with resultant left sided haematuria. Most symptomatic cases present in the third or fourth decade of life, with women being more commonly affected than men. Three types of renal nutcracker syndrome have been defined: anterior nutcracker syndrome, posterior nutcracker syndrome and combined nutcracker syndrome. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRenal nutcracker syndrome
Follow this link to review classifications for Renal nutcracker syndrome in Orphanet.

Professional guidelines

PubMed

The Journey and Barriers to Treatment of Patients With Renal Nutcracker Syndrome.
Charondo LDB, Hamouche F, Stoller M
Urology 2022 Nov;169:250-255. Epub 2022 Aug 18 doi: 10.1016/j.urology.2022.08.013. PMID: 35987378
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
Diagnosis and treatment of the pelvic congestion syndrome.
O'Brien MT, Gillespie DL
J Vasc Surg Venous Lymphat Disord 2015 Jan;3(1):96-106. Epub 2014 Jun 25 doi: 10.1016/j.jvsv.2014.05.007. PMID: 26993690

Recent clinical studies

Etiology

Venous Compressive Disorders.
Young VA, Obi C, Oladini LK, Josephs SC, Hofmann LV
Tech Vasc Interv Radiol 2024 Jun;27(2):100964. Epub 2024 Jun 14 doi: 10.1016/j.tvir.2024.100964. PMID: 39168547
Treatment of Nutcracker Syndrome with Left Renal Vein Transposition and Endovascular Stenting.
Sarikaya S, Altas O, Ozgur MM, Hancer H, Yilmaz F, Karagoz A, Ozer T, Aksut M, Ozen Y, Kirali K
Ann Vasc Surg 2024 May;102:110-120. Epub 2024 Jan 29 doi: 10.1016/j.avsg.2023.11.036. PMID: 38296038
Nutcracker Syndrome: Challenges in Diagnosis and Surgical Treatment.
Dieleman F, Hamming JF, Erben Y, van der Vorst JR
Ann Vasc Surg 2023 Aug;94:178-185. Epub 2023 Apr 5 doi: 10.1016/j.avsg.2023.03.030. PMID: 37023926
The Symptoms-Varices-Pathophysiology classification of pelvic venous disorders: A report of the American Vein & Lymphatic Society International Working Group on Pelvic Venous Disorders.
Meissner MH, Khilnani NM, Labropoulos N, Gasparis AP, Gibson K, Greiner M, Learman LA, Atashroo D, Lurie F, Passman MA, Basile A, Lazarshvilli Z, Lohr J, Kim MD, Nicolini PH, Pabon-Ramos WM, Rosenblatt M
J Vasc Surg Venous Lymphat Disord 2021 May;9(3):568-584. Epub 2021 Jan 30 doi: 10.1016/j.jvsv.2020.12.084. PMID: 33529720
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209

Diagnosis

Nutcracker Syndrome: Challenges in Diagnosis and Surgical Treatment.
Dieleman F, Hamming JF, Erben Y, van der Vorst JR
Ann Vasc Surg 2023 Aug;94:178-185. Epub 2023 Apr 5 doi: 10.1016/j.avsg.2023.03.030. PMID: 37023926
Nutcracker syndrome.
Franco-Mesa C, Gloviczki P, Erben Y
J Cardiovasc Surg (Torino) 2021 Oct;62(5):467-471. Epub 2021 Jun 9 doi: 10.23736/S0021-9509.21.11923-8. PMID: 34105927
The Symptoms-Varices-Pathophysiology classification of pelvic venous disorders: A report of the American Vein & Lymphatic Society International Working Group on Pelvic Venous Disorders.
Meissner MH, Khilnani NM, Labropoulos N, Gasparis AP, Gibson K, Greiner M, Learman LA, Atashroo D, Lurie F, Passman MA, Basile A, Lazarshvilli Z, Lohr J, Kim MD, Nicolini PH, Pabon-Ramos WM, Rosenblatt M
J Vasc Surg Venous Lymphat Disord 2021 May;9(3):568-584. Epub 2021 Jan 30 doi: 10.1016/j.jvsv.2020.12.084. PMID: 33529720
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
Venous Compression Syndromes.
White JM, Comerota AJ
Vasc Endovascular Surg 2017 Apr;51(3):155-168. doi: 10.1177/1538574417697208. PMID: 28330436

Therapy

Venous Compressive Disorders.
Young VA, Obi C, Oladini LK, Josephs SC, Hofmann LV
Tech Vasc Interv Radiol 2024 Jun;27(2):100964. Epub 2024 Jun 14 doi: 10.1016/j.tvir.2024.100964. PMID: 39168547
Nutcracker Syndrome: How to Diagnose It and When/How Should It Be Treated in the Pelvic Venous Disease Population.
Chait J, Sen I, Kalra M
Tech Vasc Interv Radiol 2021 Mar;24(1):100734. Epub 2021 Apr 16 doi: 10.1016/j.tvir.2021.100734. PMID: 34147193
Nutcracker syndrome.
Franco-Mesa C, Gloviczki P, Erben Y
J Cardiovasc Surg (Torino) 2021 Oct;62(5):467-471. Epub 2021 Jun 9 doi: 10.23736/S0021-9509.21.11923-8. PMID: 34105927
A systematic review on management of nutcracker syndrome.
Velasquez CA, Saeyeldin A, Zafar MA, Brownstein AJ, Erben Y
J Vasc Surg Venous Lymphat Disord 2018 Mar;6(2):271-278. Epub 2017 Dec 29 doi: 10.1016/j.jvsv.2017.11.005. PMID: 29292117
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209

Prognosis

Venous Compressive Disorders.
Young VA, Obi C, Oladini LK, Josephs SC, Hofmann LV
Tech Vasc Interv Radiol 2024 Jun;27(2):100964. Epub 2024 Jun 14 doi: 10.1016/j.tvir.2024.100964. PMID: 39168547
Nutcracker syndrome.
Franco-Mesa C, Gloviczki P, Erben Y
J Cardiovasc Surg (Torino) 2021 Oct;62(5):467-471. Epub 2021 Jun 9 doi: 10.23736/S0021-9509.21.11923-8. PMID: 34105927
The Symptoms-Varices-Pathophysiology classification of pelvic venous disorders: A report of the American Vein & Lymphatic Society International Working Group on Pelvic Venous Disorders.
Meissner MH, Khilnani NM, Labropoulos N, Gasparis AP, Gibson K, Greiner M, Learman LA, Atashroo D, Lurie F, Passman MA, Basile A, Lazarshvilli Z, Lohr J, Kim MD, Nicolini PH, Pabon-Ramos WM, Rosenblatt M
J Vasc Surg Venous Lymphat Disord 2021 May;9(3):568-584. Epub 2021 Jan 30 doi: 10.1016/j.jvsv.2020.12.084. PMID: 33529720
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
The nutcracker syndrome.
Venkatachalam S, Bumpus K, Kapadia SR, Gray B, Lyden S, Shishehbor MH
Ann Vasc Surg 2011 Nov;25(8):1154-64. Epub 2011 Mar 25 doi: 10.1016/j.avsg.2011.01.002. PMID: 21439772

Clinical prediction guides

Venous Compressive Disorders.
Young VA, Obi C, Oladini LK, Josephs SC, Hofmann LV
Tech Vasc Interv Radiol 2024 Jun;27(2):100964. Epub 2024 Jun 14 doi: 10.1016/j.tvir.2024.100964. PMID: 39168547
A systematic review on nutcracker syndrome and proposed diagnostic algorithm.
Nastasi DR, Fraser AR, Williams AB, Bhamidi V
J Vasc Surg Venous Lymphat Disord 2022 Nov;10(6):1410-1416. Epub 2022 Aug 23 doi: 10.1016/j.jvsv.2022.08.003. PMID: 36007798
The Symptoms-Varices-Pathophysiology classification of pelvic venous disorders: A report of the American Vein & Lymphatic Society International Working Group on Pelvic Venous Disorders.
Meissner MH, Khilnani NM, Labropoulos N, Gasparis AP, Gibson K, Greiner M, Learman LA, Atashroo D, Lurie F, Passman MA, Basile A, Lazarshvilli Z, Lohr J, Kim MD, Nicolini PH, Pabon-Ramos WM, Rosenblatt M
J Vasc Surg Venous Lymphat Disord 2021 May;9(3):568-584. Epub 2021 Jan 30 doi: 10.1016/j.jvsv.2020.12.084. PMID: 33529720
Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines.
Ananthan K, Onida S, Davies AH
Eur J Vasc Endovasc Surg 2017 Jun;53(6):886-894. Epub 2017 Mar 27 doi: 10.1016/j.ejvs.2017.02.015. PMID: 28356209
The nutcracker syndrome.
Venkatachalam S, Bumpus K, Kapadia SR, Gray B, Lyden S, Shishehbor MH
Ann Vasc Surg 2011 Nov;25(8):1154-64. Epub 2011 Mar 25 doi: 10.1016/j.avsg.2011.01.002. PMID: 21439772

Recent systematic reviews

Systematic review of endovascular versus laparoscopic extravascular stenting for treatment of nutcracker syndrome.
Fuentes-Perez A, Bush RL, Kalra M, Shortell C, Gloviczki P, Brigham TJ, Li Y, Erben Y
J Vasc Surg Venous Lymphat Disord 2023 Mar;11(2):433-441. Epub 2022 Oct 29 doi: 10.1016/j.jvsv.2022.10.001. PMID: 36404475
A systematic review on nutcracker syndrome and proposed diagnostic algorithm.
Nastasi DR, Fraser AR, Williams AB, Bhamidi V
J Vasc Surg Venous Lymphat Disord 2022 Nov;10(6):1410-1416. Epub 2022 Aug 23 doi: 10.1016/j.jvsv.2022.08.003. PMID: 36007798
Nutcracker syndrome in children: Appearance, diagnostics, and treatment - A systematic review.
Meyer J, Rother U, Stehr M, Meyer A
J Pediatr Surg 2022 Nov;57(11):716-722. Epub 2022 Jan 1 doi: 10.1016/j.jpedsurg.2021.12.019. PMID: 35065803
A systematic review on management of nutcracker syndrome.
Velasquez CA, Saeyeldin A, Zafar MA, Brownstein AJ, Erben Y
J Vasc Surg Venous Lymphat Disord 2018 Mar;6(2):271-278. Epub 2017 Dec 29 doi: 10.1016/j.jvsv.2017.11.005. PMID: 29292117
Posterior nutcracker syndrome - a systematic review.
Park JH, Lee GH, Lee SM, Eisenhut M, Kronbichler A, Lee KH, Shin JI
Vasa 2018 Jan;47(1):23-29. Epub 2017 Nov 22 doi: 10.1024/0301-1526/a000670. PMID: 29165061

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