Nutcracker phenomenon

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Nutcracker phenomenon, also known as left renal vein entrapment, refers to compression of the left renal vein, most commonly between the aorta and the superior mesenteric artery, with impaired blood outflow often accompanied by distention of the distal portion of the vein.[1] Normally, the left renal vein brings blood out of the left kidney and into the inferior vena cava, the body’s largest vein. Compression of the left renal vein can cause blood to flow backward into other nearby veins.

The term nutcracker syndrome most often refers to the classic symptoms that can arise from the nutcracker phenomenon, e.g. hematuria, abdominal pain (classically left flank or pelvic pain) and orthostatic proteinuria. [2][3][4] Some patients with nutcracker phenomenon don't have all these classic symptoms though (hematuria for example is obligatory in nutcracker syndrome[3]), but still suffers from other symptoms arising from this vein compression disorder.[3][5] Hence, both the terms nutcracker syndrome and nutcracker phenomenon can be used to describe symptomatic left renal vein entrapment. There's a wide spectrum of clinical presentations and diagnostic criteria are not well defined, which frequently results in delayed or incorrect diagnosis.[1]

Nutcracker phenomenon has been linked to CFS and dysautonomia in several medical journal articles, both in pediatric and adult patients. Theories regarding the various ways in which nutcracker phenomenon might affect autonomic function include:

  1. severe congestion in the kidney may cause the expansion of the renal venous bed, which would affect the renin-angiotensin system.[6]
  2. severe congestion in the adrenal medulla, which is innervated by sympathetic nerves, may disturb a complex set of central neural connections controlling the sympathoadrenal system. [6]
  3. overproduction or night retention of catecholamines.[6]

Nutcracker phenomenon's connection to ME/CFS & dysautonomia[edit | edit source]

Paper # 1[edit | edit source]

Hammami et al. published a case-study titled "A Tough Nut to Crack: Chronic Fatigue Syndrome and Abdominal Pain Attributed to Nutcracker Syndrome". The patient, a 24-year-old male had a seven-year history of chronic fatigue syndrome, chronic pelvic pain syndrome, and vague abdominal pain. He also suffered from orthostatic hypotension, and he showed a possible tachycardic response during a tilt table test. He also reported symptoms of “slowed thinking” and an inability to exercise without feeling lightheaded. He underwent surgery to insert a left renal vein stent. His symptoms resolved soon after the intervention. [7]

Paper # 2[edit | edit source]

This paper was titled "Does severe nutcracker phenomenon cause pediatric chronic fatigue?". According to the abstract, the authors (Takahashi et al.) had 9 pediatric CFS patients at their clinic who were intermittently or persistently absent from school. The patients had been suspected to be burdened with psychosomatic disorders, having orthostatic hypotension, postural tachycardia, or other autonomic dysfunction symptoms. When they investigated the cause of moderate orthostatic proteinuria in some of the patients, they found by chance that they suffered from severe nutcracker phenomenon. Further investigation revealed that nutcracker phenomenon was present in all 9 children complaining of chronic fatigue, even those who did not have orthostatic proteinuria. They conclude in their abstract (NC=nutcracker phenomenon): "Their symptoms filled the criteria of chronic fatigue syndrome or idiopathic chronic fatigue (CFS/CF). An association between severe NC and autonomic dysfunction symptoms in children with CFS/CF has been presented."[8]

The full-text is hard to find, but some other more details can be found in another paper[9] referring to this paper and on Dysautonomia Information Network's website[6]. The patients are reported to have suffered from a broad range of symptoms including; chronic fatigue, headache, lightheadedness, dizziness, abdominal pain, unrefreshing sleep, muscle pain, joint pain, sore throat, low-grade fever, afebrile chills in hot summer and depression.

The authors of this study point out that the classic symptom of nutcracker phenomnen is renal bleeding (presenting as micro or macro-hematuria). In their experience, non-CFS patients with nutcracker phenomenon typically had hematuria, but the patients with CFS-associated nutcracker phenimenon had no renal bleeding. Some of these patients did report fibromyalgia type pain. Some patients had proteinuria, others had no urinary abnormalities. The authors of this study had some theories regarding the various ways in which nutcracker phenomenon might affect autonomic function: First, severe congestion in the kidney may cause the expansion of the renal venous bed, which would affect the renin-angiotensin system. Secondly, severe congestion in the adrenal medulla, which is innervated by sympathetic nerves, may disturb a complex set of central neural connections controlling the sympathoadrenal system. On the other hand, overproduction or night retention of catecholamines might be responsible for the various symptoms of pediatric chronic fatigue syndrome.

Paper # 3[edit | edit source]

Another paper from Takahashi et al. was titled "An effective "transluminal balloon angioplasty" therapy for pediatric chronic fatigue syndrome with nutcracker phenomenon"[10]. Unfortunately, the abstract and full-text are not easily found, but another paper[9] describes the findings in this paper.

It's about a 13 year old girl who suffered from orthostatic hypotension, tachycardia and chronic fatigue syndrome. The patient was also diagnosed with nutcracker phenomenon, and was successfully treated with transluminal balloon angioplasty of the compressed left renal vein between the aorta and superior mesenteric artery. Both her dysautonomia and CFS improved after the intervention.

Paper # 4[edit | edit source]

A third paper from Takahashi et al. was titled "An ultrasonographic classification for diverse clinical symptoms of pediatric nutcracker phenomenon". They tested 93 pediatric patients for nutcracker phenomenon (56 with idiopathic renal bleeding, 14 with massive orthostatic proteinuria and 23 with severe orthostatic intolerance). Left renal vein occlusion (nutcracker phenomenon) was observed in 70% of the patients with severe orthostatic intolerance, and in contrast in 18% and 14% for idiopathic renal bleeding and massive orthostatic proteinuria, respectively. [11]

Paper # 5[edit | edit source]

Takemura et al. published a paper describing four adolescents diagnosed with nutcracker syndrome. Three of these patients had previously been diagnosed with orthostatic disturbance and suffered from various symptoms including fainting, tachycardia, headache and abdominal pain.[12]

Paper # 6

A case-report from Daily et al. titled "Nutcracker syndrome: symptoms of syncope and hypotension improved following endovascular stenting" describes a 19-year-old woman diagnosed with nutcracker syndrome. She suffered from syncope, sometimes multiple episodes in one day. Her other symptoms included unilateral hematuria, nausea, lower abdominal pain and weight loss. After she was treated with stenting of her left renal vein, her symptoms improved drastically and she had no episodes of syncope and her blood pressure normalized.[13]

Paper # 7

In a paper titled "Newly-identified symptoms of left renal vein entrapment syndrome mimicking orthostatic disturbance"[5], Koshimichi et al. describes the symptoms of 53 pediatric patients diagnosed with left renal vein entrapment syndrom (another term for nutcracker phenomenon). 22 of these 53 patients (42%) suffered from orthostatic disturbance. 15 of these 22 patients suffered from general malaise and fatigue, palpitation or shortness of breath in 14, severe abdominal pain in 10, increased pulse (21 beats per minute or more) on standing in 2 patients.

There was an absence of micro/macro-hematuria 11 of these 22 patients. 13 of 22 patients had no proteinuria. 4 of 22 patients had neither micro/macro-hematuria or proteinuria.

Treatment with midodrine significantly decreased orthostasis scores. The most severe patients (6 of 22) had either low urinary cortisol or plasma cortisol, persistent in some and intermittent in some. These patients improved after being given a low oral dose of fludrocortisone acetate to maintain sufficient blood cortisol.

Epidemiology[edit | edit source]

Nutcracker syndrome/phenomenon is often described as "rare" in the medical literature, even though there's indications that it's very likely a severely under-diagnosed condition.[1][3][14][15] For example, left renal vein entrapment was observed in 10.9% of patients undergoing abdominal contrast-enhanced MDCT scans according to one study.[16]

Varicocele is a know complication of nutcracker phenomenon. About 15 % of all men globally are affected by varicocele.[17] According to two separate studies, 30%[18] to 100%[19] of varicocele patients have nutcracker phenomenon. If one extrapolates these numbers, 4,5-15% of all men suffer from nutcracker phenomenon. A minuscule percentage of these patients are ever diagnosed with nutcracker phenomenon.

The reason why nutcracker phenomenon is severely under-diagnosed might be because of several factors:

  1. wide spectrum of symptoms[1][3][5][7][8][10][11][12], many of which are not well known in the medical community to be linked to nutcracker phenomenon.
  2. not all patients experience classic nutcracker syndrome symptoms like hematuria, abdominal pain, proteinuria[5][10][8]
  3. diagnostic criteria are not well defined[1]
  4. entrapment of the left renal vein is not easily detectable using conventional means[20][1]. Standard CT is for example insufficient to diagnose nutcracker phenomenon.[1]

Diagnosis[edit | edit source]

This quote is from Dysautonomia Information Network's website[6], where Dr. Takahashi (the author of some of the studies above linking nutcracker phenomenon to CFS & orthostatic intolerance) explains the methods used to diagnose nutcracker phenomenon:

"The methods used to diagnose nutcracker phenomenon include Doppler US, MRI and three-dimensional helical computed tomography. Dr. Takahashi (personal communication, September 8, 2002) explains the procedures for testing as follows: Conventional ultrasound requires patients to be examined for left renal vein obstruction in 4 positions: supine, semisitting, upright and prone. Nonvisualization of the left renal vein lumen or absence of the left renal vein wall between the aorta and superior mesenteric artery is regarded as signifying left renal vein obstruction. Doppler color flow imaging can be used to locate a blue-colored blood stream flowing to the dorsal direction. This is a collateral vein flowing from the left renal vein into the paravertebral vein. With MRI, oblique coronal images along the left renal vein, and also axial images, are recommended to visualize the collateral veins around the left renal vein."

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Kurklinsky, Andrew K.; Rooke, Thom W. (2010-6). "Nutcracker Phenomenon and Nutcracker Syndrome". Mayo Clinic Proceedings. 85 (6): 552–559. doi:10.4065/mcp.2009.0586. ISSN 0025-6196. PMC 2878259. PMID 20511485. Check date values in: |date= (help)
  2. ↑ "Nutcracker syndrome". Wikipedia. April 13, 2020.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 Scholbach, Thomas (2007). "From the nutcracker-phenomenon of the left renal vein to the midline congestion syndrome as a cause of migraine, headache, back and abdominal pain and functional disorders of pelvic organs". Medical Hypotheses. 68 (6): 1318–1327. doi:10.1016/j.mehy.2006.10.040. ISSN 0306-9877. PMID 17161550.
  4. ↑ Kurklinsky, Andrew K.; Rooke, Thom W. (2010-6). "Nutcracker Phenomenon and Nutcracker Syndrome". Mayo Clinic Proceedings. 85 (6): 552–559. doi:10.4065/mcp.2009.0586. ISSN 0025-6196. PMC 2878259. PMID 20511485. Check date values in: |date= (help)
  5. ↑ 5.0 5.1 5.2 5.3 Koshimichi, Machiko; Sugimoto, Keisuke; Yanagida, Hidehiko; Fujita, Shinsuke; Miyazawa, Tomoki; Sakata, Naoki; Okada, Mitsuru; Takemura, Tsukasa (2012-05). "Newly-identified symptoms of left renal vein entrapment syndrome mimicking orthostatic disturbance". World journal of pediatrics: WJP. 8 (2): 116–122. doi:10.1007/s12519-012-0349-1. ISSN 1867-0687. PMID 22573421. Check date values in: |date= (help)
  6. ↑ 6.0 6.1 6.2 6.3 6.4 "What Causes POTS?". Dysautonomia Information Network (DINET). Retrieved May 1, 2020.
  7. ↑ 7.0 7.1 "A Tough Nut to Crack: Chronic Fatigue Syndrome and Abdominal Pain Attributed to Nutcracker Syndrome". www.oatext.com. Retrieved April 30, 2020.
  8. ↑ 8.0 8.1 8.2 Takahashi, Y.; Ohta, S.; Sano, A.; Kuroda, Y.; Kaji, Y.; Matsuki, M.; Matsuo, M. (2000-03). "Does severe nutcracker phenomenon cause pediatric chronic fatigue?". Clinical Nephrology. 53 (3): 174–181. ISSN 0301-0430. PMID 10749295. Check date values in: |date= (help)
  9. ↑ 9.0 9.1 Shin, Jae Il; Lee, Jae Seung (2006-05). "Comment on Gastrointestinal Symptoms Associated with Orthostatic Intolerance". Journal of Pediatric Gastroenterology and Nutrition. 42 (5): 588. doi:10.1097/01.mpg.0000215310.71040.c0. ISSN 0277-2116. Check date values in: |date= (help)
  10. ↑ 10.0 10.1 10.2 Takahashi, Y.; Sano, A.; Matsuo, M. (2000-01). "An effective "transluminal balloon angioplasty" therapy for pediatric chronic fatigue syndrome with nutcracker phenomenon". Clinical Nephrology. 53 (1): 77–78. ISSN 0301-0430. PMID 10661488. Check date values in: |date= (help)
  11. ↑ 11.0 11.1 Takahashi, Y.; Sano, A.; Matsuo, M. (2005-07). "An ultrasonographic classification for diverse clinical symptoms of pediatric nutcracker phenomenon". Clinical Nephrology. 64 (1): 47–54. doi:10.5414/cnp64047. ISSN 0301-0430. PMID 16047645. Check date values in: |date= (help)
  12. ↑ 12.0 12.1 Takemura, T.; Iwasa, H.; Yamamoto, S.; Hino, S.; Fukushima, K.; Isokawa, S.; Okada, M.; Yoshioka, K. (2000-09). "Clinical and radiological features in four adolescents with nutcracker syndrome". Pediatric Nephrology (Berlin, Germany). 14 (10–11): 1002–1005. doi:10.1007/s004670050062. ISSN 0931-041X. PMID 10975316. Check date values in: |date= (help)
  13. ↑ Daily, Ryan; Matteo, Jerry; Loper, Todd; Northup, Martin (2012-12). "Nutcracker syndrome: symptoms of syncope and hypotension improved following endovascular stenting". Vascular. 20 (6): 337–341. doi:10.1258/vasc.2011.cr0320. ISSN 1708-5381. PMID 22734085. Check date values in: |date= (help)
  14. ↑ "Renal nutcracker syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved May 1, 2020.
  15. ↑ Poyraz, Ahmet K; Firdolas, Fatih; Onur, Mehmet R; Kocakoc, Ercan (2013-03). "Evaluation of left renal vein entrapment using multidetector computed tomography". Acta Radiologica. 54 (2): 144–148. doi:10.1258/ar.2012.120355. ISSN 0284-1851. Check date values in: |date= (help)
  16. ↑ Poyraz, Ahmet K; Firdolas, Fatih; Onur, Mehmet R; Kocakoc, Ercan (2013-03). "Evaluation of left renal vein entrapment using multidetector computed tomography". Acta Radiologica. 54 (2): 144–148. doi:10.1258/ar.2012.120355. ISSN 0284-1851. Check date values in: |date= (help)
  17. ↑ "Varicocele". Wikipedia. April 16, 2020.
  18. ↑ Mohamadi, Afshin; Ghasemi-Rad, Mohammad; Mladkova, Nikol; Masudi, Sima (2010). "Varicocele and Nutcracker Syndrome". Journal of Ultrasound in Medicine. 29 (8): 1153–1160. doi:10.7863/jum.2010.29.8.1153. ISSN 1550-9613.
  19. ↑ Unlu, Murat; Orguc, Sebnem; Serter, Selim; Pekindil, Gokhan; Pabuscu, Yuksel (2007). "Anatomic and hemodynamic evaluation of renal venous flow in varicocele formation using color Doppler sonography with emphasis on renal vein entrapment syndrome". Scandinavian Journal of Urology and Nephrology. 41 (1): 42–46. doi:10.1080/00365590600796659. ISSN 0036-5599. PMID 17366101.
  20. ↑ Matsukura, Hiro; Arai, Miwako; Miyawaki, Toshio (2005-02). "Nutcracker phenomenon in two siblings of a Japanese family". Pediatric Nephrology (Berlin, Germany). 20 (2): 237–238. doi:10.1007/s00467-004-1682-y. ISSN 0931-041X. PMID 15599773. Check date values in: |date= (help)

renal involving, related to or in the area of the kidneys

dysautonomia disorders of the autonomic nervous system that cause disturbances in all or some autonomic functions, may cause problems regulating autonomic functions, including heart rate, blood pressure, body temperature, and digestion. Can cause symptoms including lightheadedness, fainting, unstable blood pressure, and orthostatic intolerance.

somatic symptom disorder A psychiatric term to describe an alleged condition whereby a person's thoughts somehow cause physical symptoms. The actual existence of such a condition is highly controversial, due to a lack of scientific evidence. It is related to other psychiatric terms, such as "psychosomatic", "neurasthenia", and "hysteria". Older terms include "somatization", "somatoform disorder", and "conversion disorder". Such terms refer to a scientifically-unsupported theory that claims that a wide range of physical symptoms can be created by the human mind, a theory which has been criticized as "mind over matter" parapsychology, a pseudoscience.

tachycardia An unusually rapid heart beat. Can be caused by exercise or illness. A symptom of postural orthostatic tachycardia syndrome (POTS). (Learn more: www.heart.org)

chronic fatigue (CF) - Persistent and abnormal fatigue is a symptom, not an illness. It may be caused by depression, multiple sclerosis, fibromyalgia, chronic fatigue syndrome or many other illnesses. The term "chronic fatigue" should never be confused with the disease chronic fatigue syndrome.

dysautonomia disorders of the autonomic nervous system that cause disturbances in all or some autonomic functions, may cause problems regulating autonomic functions, including heart rate, blood pressure, body temperature, and digestion. Can cause symptoms including lightheadedness, fainting, unstable blood pressure, and orthostatic intolerance.

lightheadedness the condition of being dizzy or on the verge of fainting

supine lying on the back or with the face upward

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