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Disease Profile
Renal nutcracker syndrome
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Unknown
Age of onset
Adult
ICD-10
-
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Nutcracker syndrome; Left renal vein entrapment syndrome; RNS
Categories
Digestive Diseases; Kidney and Urinary Diseases
Summary
Renal nutcracker
Symptoms
The most common signs and symptoms may include:[1][2][4]
- Blood in the urine (hematuria) which can occasionally cause
anemia requiring blood transfusions. - Abdominal or flank pain that may radiate to the thigh and buttock. Pain may be worsened by sitting, standing, walking, or riding in a vehicle that shakes.
- Varicocele in men almost always occurring on the left side.
- Chronic fatigue syndrome and fatigue symptoms.
- Pelvic congestion syndrome, causing symptoms such as chronic
pelvic pain, pain during intercourse, painful or difficult urination, painful menstrual cramps during periods, and polycystic ovaries. - Orthostatic proteinuria.
- Orthostatic intolerance (feeling light-headed or having palpitations when standing upright).
Cause
- Anterior NCS, which is the most common form, occurs when the normally-placed left renal vein (the vein that carries blood purified by the left kidney) is compressed between two arteries (the abdominal aorta and the superior mesenteric artery).
- Posterior NCS generally occurs when the left renal vein is compressed between an artery (usually the abdominal aorta) and the bones of the spine.
Some cases of mild NCS in children may be due to changes in body proportions associated with growth. The reason NCS occurs or causes symptoms in adults is less clear.[1][2] The following may increase the risk of developing NCS as a result of compression of the left renal vein:[1][4][2]
- Pancreatic tumors
- Swelling of the para-aortic
lymph nodes (a group of lymph nodes in front of the base of the spine) - Retroperitoneal
tumors - Abdominal aortic aneurysm
- Left renal vein
duplication , and an abnormally placed right renal artery - Nephroptosis of the left kidney when the kidney drops down into an abnormal position in the pelvis while a person goes from sitting to standing
Lordosis - Low body mass index (BMI)
Diagnosis
Treatment
Surgery may be considered in the following instances:[4]
- Gross hematuria (when urine is visibly discolored by blood)
- Severe symptoms (flank or abdominal pain,
anemia , autonomic dysfunction, impairment of renal function) - If conservative management is not effective after a specific period of time, depending on the patient's age (generally after 24 months in patients under 18, and after 6 months in adults)
The surgical technique depends upon each person's anatomy and how likely it is to relieve symptoms.[3] The use of a combination of surgical techniques is increasingly being recognized as a means to alleviate symptoms of NCS.[4]
Related diseases
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
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Compression of LRV leading to loin pain and hematuria can be seen in pancreatic neoplasms, paraortic lymphoadenopathy, retroperitoneal masses, overarching testicular artery, lordosis, reduced retroperitoneal and mesenteric fat or too much fibrolymphatic tissue between SMA and AA.
Visit the Orphanet disease page for more information.
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Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Social Networking Websites
- Visit the following Facebook groups related to Renal nutcracker syndrome:
Renal Nutcracker Syndrome Support Group
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
In-Depth Information
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Renal nutcracker syndrome. Click on the link to view a sample search on this topic.
References
- Gulleroglu K, Gulleroglu B & Baskin E. Nutcracker syndrome. World J Nephrol. November 2014; 3(4):277-281. https://www.researchgate.net/publication/267872522_Nutcracker_syndrome.
- Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc. June 2010; 85(6):552-559. https://www.mayoclinicproceedings.org/article/S0025-6196(11)60346-7/abstract.
- Said SM, Gloviczki P, Kalra M, Oderich GS, Duncan AA, D Fleming M, Bower TC. Renal nutcracker syndrome: surgical options. Semin Vasc Surg. March, 2013; 26(1):35-42. https://www.ncbi.nlm.nih.gov/pubmed/23932560.
- Ananthan K, Onida S, Davies AH. Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines. Eur J Vasc Endovasc Surg. Jun 2017; 53(6):886-894. https://www.ncbi.nlm.nih.gov/pubmed/28356209.
- Policha A, Lamparello P, Sadek M, Berland T, Maldonado T. Endovascular Treatment of Nutcracker Syndrome. Ann Vasc Surg. October, 2016; 36:https://www.ncbi.nlm.nih.gov/pubmed/27321979.
- Atkinson TH, Love M, Messer J. Gross hematuria caused by nutcracker syndrome and segmental pseudoaneursym. Can J Urol. April, 2015; 22(2):7745-7747. https://europepmc.org/abstract/med/25891340.
- Del Canto Peruyera P, Vaquero Lorenzo F, Vallina-Victorero Vazquez MJ, Alvarez Salgado A, Vicente Santiago M, Botas Velasco M, Alvarez Fernandez LJ. Recurrent hematuria caused by nutcracker syndrome. Ann Vasc Surg. May, 2014; 28(4):1036.e15-9. https://www.ncbi.nlm.nih.gov/pubmed/24321267.
- Chen YM, Wang IK, Ng KK, Huang CC. Nutcracker syndrome: an overlooked cause of hematuria. Chang Gung Med J. October, 2002; 25(10):700-705. https://www.ncbi.nlm.nih.gov/pubmed/12518783.
- Hartung O, Grisoli D, Boufi M, Marani I, Hakam Z, Barthelemy P, Alimi YS. Endovascular stenting in the treatment of pelvic vein congestion caused by nutcracker syndrome: lessons learned from the first five cases. J Vasc Surg. August, 2005; 42(2):275-280. https://www.ncbi.nlm.nih.gov/pubmed/16102626.
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