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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
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
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
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
Surgical and endovascular interventions for nutcracker syndrome.
Avgerinos ED, McEnaney R, Chaer RA
Semin Vasc Surg 2013 Dec;26(4):170-7. Epub 2014 Jun 25 doi: 10.1053/j.semvascsurg.2014.06.014. PMID: 25220323

Diagnosis

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
Doppler US and CT Diagnosis of Nutcracker Syndrome.
Kim SH
Korean J Radiol 2019 Dec;20(12):1627-1637. doi: 10.3348/kjr.2019.0084. PMID: 31854150Free PMC Article
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
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

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.
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
Localized solitary left renal vein thrombus complicating nutcracker syndrome: a case report and review of the literature.
Nakashima T, Sahashi Y, Kanamori H, Ohno Y, Okura H
CEN Case Rep 2020 Aug;9(3):252-256. Epub 2020 Apr 3 doi: 10.1007/s13730-020-00467-9. PMID: 32246272Free PMC Article
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

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
The nutcracker syndrome. Morphology and clinical aspects of the important vascular variations: a systematic study of 112 cases.
Orczyk K, Łabetowicz P, Lodziński S, Stefańczyk L, Topol M, Polguj M
Int Angiol 2016 Feb;35(1):71-7. Epub 2015 May 14 PMID: 25972135

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