Abstract
The incidence of NCP and NCS is unknown and there have been no accurate figures on the prevalence of these conditions. Generally, NCS has been thought rare but probably overlooked and underreported. In the United States, NCS is registered as a rare disease in the GARD (genetic and rare disease) information center of the National Institute of Health and the Orphanet site, a portal for rare disease and orphan drugs. In the website of the GARD information center, it is mentioned that the exact prevalence of renal nutcracker syndrome is unknown partly because there have been no standard diagnostic criteria, and partly because symptoms can vary among people with the condition.
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Further Reading
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https://2020.ksum.or.kr/file/KSUM_2020_Program_Abstract_Book.pdf, page 228
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https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=71273
https://rarediseases.info.nih.gov/diseases/11971/renal-nutcracker-syndrome
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3.1.1 1. NCS Registered at NIH GARD and Orphanet Sites
Screenshot image of the website of the GARD (Genetic and Rare Diseases) Information Center of the NIH where NCS is registered as a rare disease. It is mentioned that the exact prevalence of renal nutcracker syndrome is unknown partly because there have been no standard diagnostic criteria, and partly because symptoms can vary (or be absent) among people with the condition. It is also mentioned that while it has been described as “rare” in the medical literature, some have suggested its incidence is likely underestimated. (https://rarediseases.info.nih.gov/diseases/11971/renal-nutcracker-syndrome)
Screenshot image of the website of the Orphanet where NCS is registered as a rare disease. (https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=71273)
3.1.2 2. Prevalence of NCP and NCS in K-Radiology Clinic (Presented in 2020 KSUM Annual Congress)
Prevalence of NCP and NCS presented in 2020 annual congress of the Korean Society of Ultrasound in Medicine. (https://2020.ksum.or.kr/file/KSUM_2020_Program_Abstract_Book.pdf, page 228). (A) Among the 1223 patients examined with Doppler US, approximately 30% (372 patients, 30.4%) were found to have NCP and approximately 50% (184 patients, 49.5%) of those 372 cases with NCP may be diagnosed as NCS. (B) The prevalence of NCP and NCS was higher in women than in men (34% vs 26% in NCP and 55% vs 42% in NCS prevalence among NCP cases)
A 28-year-old woman with a history of proteinuria for 20 years. Because there were no causes for the proteinuria other than NCP, this patient was diagnosed as NCS. (A) Greyscale US image shows narrowing of the am-LRV (arrow). (B) CDUS image of the LRV shows bright-colored blood flow (arrowheads) from the point where it is compressed between the AA (a) and SMA (s). (C) SDUS at the am-LRV shows a PFV of 147 cm/s. (D) A diagram showing where (arrow) this patient belongs to
A 60-year-old woman with intermittent left flank pain. Because there were no causes for the left flank pain other than NCP, this patient was diagnosed as NCS. (A) Greyscale US image shows dilated h-LRV (h) and narrowing of the am-LRV (arrow). (B) CDUS image of the LRV shows bright-colored blood flow (arrowheads) from the point where it is compressed between the AA (a) and SMA (s). h: h-LRV. (C) SDUS at the am-LRV shows a PFV of 220 cm/s. (D) A diagram showing where (arrow) this patient belongs to
A 56-year-old woman with microscopic hematuria and proteinuria. Because there were no causes for microscopic hematuria and proteinuria other than NCP, this patient was diagnosed as NCS. (A) Greyscale US image shows the LRV compressed posteriorly by the RRA (open arrow). Note SMA (s) does not compress the LRV. (B) CDUS image of the LRV shows bright-colored blood flow (arrowheads) from the point where it is compressed by the RRA (open arrow). (C) SDUS at the am-LRV shows a PFV of 118 cm/s. (D) A diagram showing where (arrow) this patient belongs to
A 49-year-old woman with NCP. This patient did not have any signs or symptoms related to NCP. (A) Greyscale US image shows the LRV stretched (arrow) over the AA (a). (B) CDUS image of the LRV shows bright-colored blood flow (arrowhead) from the point where it is stretched over the AA (a). (C) SDUS at the am-LRV with the patient keeping quiet breathing shows a PFV between 100 and 200 cm/s. A measured a PFV was 178 cm/s. (D) A diagram showing where (arrow) this patient belongs to
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KIM, S.H. (2022). Prevalence of NCP and NCS. In: Radiology Illustrated: Nutcracker Phenomenon and Nutcracker Syndrome. Radiology Illustrated. Springer, Singapore. https://doi.org/10.1007/978-981-16-6218-8_3
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DOI: https://doi.org/10.1007/978-981-16-6218-8_3
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