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Disease Profile

Renal nutcracker syndrome

Prevalence
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

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ICD-10

-

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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Other names (AKA)

Nutcracker syndrome; Left renal vein entrapment syndrome; RNS

Categories

Digestive Diseases; Kidney and Urinary Diseases

Summary

Renal nutcracker syndrome (NCS) is a condition that occurs when the left renal vein (the vein that carries blood purified by the left kidney) becomes compressed. Signs and symptoms can vary from person to person. Some people may not have symptoms, while others develop severe and persistent symptoms. Symptoms may include blood in the urine (hematuria), orthostatic proteinuria, flank pain and/or abdominal pain. Some cases of mild NCS in children may be due to changes in body proportions associated with growth. It is less clear why NCS occurs or causes symptoms in adults. Treatment ranges from surveillance (in less severe cases) to various types of surgery. The surgical technique depends upon each person's anatomy and how likely it is to relieve symptoms.[1][2][3]

Symptoms

The symptoms and severity of renal nutcracker syndrome can vary from person to person. Some people may not have any symptoms (especially children), while others have severe and persistent symptoms. Symptoms are often worsened by physical activity.[1][2]

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

NCS is generally divided into 2 types: anterior and posterior.[1][4]

  1. 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).
  2. 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

A diagnosis of NCS may first be suspected based on the characteristic signs and symptoms. Other conditions that cause similar symptoms are often first ruled out. The diagnosis is ultimately confirmed by results of various imaging studies, including:[1][4]

Treatment

The best treatment for each person with renal nutcracker syndrome (NCS) is a controversial topic.[4] In general, for people with mild hematuria or with mild and tolerable symptoms, conservative management is recommended.[4] This generally consists of regular surveillance and observation. This is because many patients will have complete resolution of symptoms without any significant intervention.[1][2] Medical therapy may be recommended to improve orthostatic proteinuria and/or renal perfusion.[4]

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]

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

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

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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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