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Branagan WT, Reynolds N, Pshak T, Nydam T. Psychosocial Trends in Autotransplant Patients with Nutcracker Syndrome: A Single Center's Experience. Ann Vasc Surg 2025; 119:11-13. [PMID: 40320208 DOI: 10.1016/j.avsg.2025.04.123] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2025] [Revised: 04/09/2025] [Accepted: 04/10/2025] [Indexed: 05/24/2025]
Affiliation(s)
- William T Branagan
- Department of Psychiatry, University of Colorado School of Medicine, Aurora, CO; Colorado Center for Transplantation Care, Research and Education (CCTCARE), Aurora, CO.
| | - Nicole Reynolds
- Department of Psychiatry, University of Colorado School of Medicine, Aurora, CO; Colorado Center for Transplantation Care, Research and Education (CCTCARE), Aurora, CO
| | - Thomas Pshak
- Colorado Center for Transplantation Care, Research and Education (CCTCARE), Aurora, CO; Department of Surgery, University of Colorado School of Medicine, Aurora, CO
| | - Trevor Nydam
- Colorado Center for Transplantation Care, Research and Education (CCTCARE), Aurora, CO; Department of Surgery, University of Colorado School of Medicine, Aurora, CO
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Athish KK, Kumar NP, Nayak-Rao S. Varying clinical presentations of nutcracker syndrome: a case report. J Med Case Rep 2025; 19:150. [PMID: 40170126 PMCID: PMC11963672 DOI: 10.1186/s13256-025-05156-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2024] [Accepted: 02/14/2025] [Indexed: 04/03/2025] Open
Abstract
INTRODUCTION The term nutcracker syndrome was initially established in 1972 to refer to the compressive effects on the left renal vein due to a narrow angle between the abdominal aorta and the superior mesenteric artery. Although the precise prevalence of nutcracker syndrome remains unknown, an incidence of up to 4% has been described in patients presenting with hematuria. The peak age of incidence is between 10 and 30 years, and it is equally prevalent among both genders, though earlier studies showed a predominantly female preponderance. About 70-80% of patients present initially with hematuria, though left flank pain and proteinuria may also be presenting symptoms. A high index of suspicion and appropriate imaging often helps in aiding diagnosis. CASE PRESENTATION In this case report, we present here two South Asian young females aged 23 and 30 years old. They presented with gross painless hematuria of a duration of 2 years (Case 1) and nonspecific symptoms of abdominal pain (Case 2), and they were diagnosed with nutcracker syndrome on investigation. Both patients were diagnosed by computed tomography angiography with defined aortomesenteric angles diagnostic of nutcracker syndrome. Patient 1 underwent saphenous venous bypass grafting and repositioning of left renal vein with symptomatic improvement, while patient 2 was managed conservatively and continues to be on close follow-up. In addition, we present a brief review of this syndrome so that better insight is obtained regarding diagnosis and management. CONCLUSION The diagnosis of nutcracker syndrome needs to be considered in patients who present with unexplained hematuria or proteinuria. Diagnosis by appropriate imaging studies is necessary, and treatment is dictated by the severity of symptoms. Surgical therapy remains the front-line treatment; however, endovascular techniques are becoming favored owing to advancements in stent technology.
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Affiliation(s)
- K K Athish
- Department of Nephrology, Sri Devaraj Urs Academy of Higher Education and Research, Tamaka, Kolar, 563103, Karnataka, India
| | - N Prasanna Kumar
- Department of Nephrology, Sri Devaraj Urs Academy of Higher Education and Research, Tamaka, Kolar, 563103, Karnataka, India
| | - Shobhana Nayak-Rao
- Department of Nephrology, Sri Devaraj Urs Academy of Higher Education and Research, Tamaka, Kolar, 563103, Karnataka, India.
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Wu W, Aisha A, Chang F, Jianghui Z, Shuai T, Zheng L, Chen F. Establishment and analysis of an animal model for nutcracker syndrome. Int Urol Nephrol 2025; 57:419-426. [PMID: 39422826 DOI: 10.1007/s11255-024-04245-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2024] [Accepted: 10/13/2024] [Indexed: 10/19/2024]
Abstract
BACKGROUND This study investigated the effects of renal vein congestion of varying durations on kidney damage by establishing an animal model of Nutcracker Syndrome (NCS). In addition, we assessed whether renal damage improved after relieving the congestion. METHOD We established a rat model of NCS and relieved renal congestion through secondary surgery, with renal vein reperfusion confirmed by animal ultrasound. Histopathological changes in congested kidneys were evaluated at 3, 6, and 9 days using HE and Masson staining, and the expression of renal injury factors was determined using q-PCR. RESULTS Renal tissue pathology changes were evident with prolonged obstruction time. Compared to the sham-operated group, no apparent fibrosis was observed in the kidneys at 3 days post-congestion, whereas fibrosis was most severe at 9 days post-congestion, with no significant improvement observed even after blood flow restoration. Six days post-congestion relief, some alleviation of renal fibrosis occurred. q-PCR results indicated a significant reduction in the expression of fibrosis markers after 3 or 6 days of renal vein ligation release. Following blood flow restoration on the 9th day post-congestion, no significant changes in fibrosis markers were noted. CONCLUSION Prolonged renal vein congestion in a rat model of NCS resulted in severe kidney damage and significant fibrosis after 9 days. While relieving congestion within 6 days led to some improvement, no reduction in fibrosis occurred after 9 days, underscoring the importance of congestion duration in renal pathology.
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Affiliation(s)
- Wensong Wu
- Department of Urology, The Third Central Clinical College of Tianjin Medical University, Tianjin, 300170, China
| | - Awuti Aisha
- Department of Urology, The Third Central Clinical College of Tianjin Medical University, Tianjin, 300170, China
| | - Fan Chang
- Department of Urology, The Third Central Hospital of Tianjin, 83 Jintang Road, Tianjin, 300170, China
- Department of Urology, Nankai University Affinity the Third Central Hospital, Tianjin, 300170, China
| | - Zhang Jianghui
- Department of Urology, The Third Central Hospital of Tianjin, 83 Jintang Road, Tianjin, 300170, China
- Department of Urology, Nankai University Affinity the Third Central Hospital, Tianjin, 300170, China
| | - Tang Shuai
- Department of Urology, The Third Central Hospital of Tianjin, 83 Jintang Road, Tianjin, 300170, China
- Department of Urology, Nankai University Affinity the Third Central Hospital, Tianjin, 300170, China
| | - Lv Zheng
- Department of Urology, The Third Central Hospital of Tianjin, 83 Jintang Road, Tianjin, 300170, China
- Department of Urology, Nankai University Affinity the Third Central Hospital, Tianjin, 300170, China
| | - Fangmin Chen
- Department of Urology, The Third Central Hospital of Tianjin, 83 Jintang Road, Tianjin, 300170, China.
- Department of Urology, Nankai University Affinity the Third Central Hospital, Tianjin, 300170, China.
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Sarikaya S, Hancer H, Karasu H, Kırali K. [MSB-31] Treatment of Nutcracker Syndrome: Outcomes with Left Renal Vein Transposition and Stenting. TURK GOGUS KALP DAMAR CERRAHISI DERGISI 2024; 32:054-55. [PMID: 40322112 PMCID: PMC12045219 DOI: 10.5606/tgkdc.dergisi.2024.msb-31] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/14/2024] [Accepted: 09/29/2024] [Indexed: 05/08/2025]
Abstract
Objective This study aimed to share our experiences and outcomes with left renal vein (LRV) transposition and endovascular stenting in treating Nutcracker syndrome. Methods Data of 20 female Nutcracker syndrome patients (mean age: 24 years) who underwent LRV transposition (n=15) or stenting (n=5) between July 2019 and June 2024 were retrospectively reviewed. Primary endpoints were morbidity and mortality. Secondary endpoints included late complications, patency, freedom from reintervention, and resolution of symptoms. Results There were no major complications or mortality after either procedure. The most common signs and symptoms associated with LRV entrapment were left flank pain (100%, n=20), proteinuria (88%, n=15), and hematuria (47%, n=9). After both procedures, classical symptoms resolved in 89.5% (n=17) of patients for left flank pain, 57.8% (n=11) for proteinuria, and 82.3% (n=15) for hematuria. Four patients required reintervention [three after LRV transposition (two for occlusion and one for stenosis) and one after stenting (occlusion)]. The one-year primary patency rate was 87%, and the primary assisted patency rate was 100%. One-year primary patency rates were 91% for the transposition group and 75% for the stent group. The one-year freedom from reintervention was 83%. Conclusion Both procedures can be used as primary treatments and have their advantages. This study shows that both methods are safe and effective.
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Affiliation(s)
- Sabit Sarikaya
- Koşuyolu Heart Training and Research Hospital, İstanbul, Türkiye
| | - Hakan Hancer
- Koşuyolu Heart Training and Research Hospital, İstanbul, Türkiye
| | - Hasan Karasu
- Koşuyolu Heart Training and Research Hospital, İstanbul, Türkiye
| | - Kaan Kırali
- Koşuyolu Heart Training and Research Hospital, İstanbul, Türkiye
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Maharaj D, Mohammed SR, Caesar K, Dindyal S. Nutcracker syndrome: a case-based review. Ann R Coll Surg Engl 2024; 106:396-400. [PMID: 38038139 PMCID: PMC11060856 DOI: 10.1308/rcsann.2023.0090] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/23/2023] [Indexed: 12/02/2023] Open
Abstract
The nutcracker phenomenon, also known as left renal vein entrapment, occurs when there is extrinsic compression of the left renal vein, most often between the abdominal aorta and the superior mesenteric artery. Nutcracker syndrome refers to the constellation of clinical symptoms that may arise from the nutcracker phenomenon, typically inclusive of haematuria, flank/pelvic pain, orthostatic proteinuria and (in male patients) varicocele. We provide a short review of the nutcracker syndrome including various diagnostic and therapeutic modalities. We utilise our own experience with a patient as a case study and highlight the modern management option of endovascular stenting.
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Affiliation(s)
- D Maharaj
- St Clair Medical Centre, Trinidad and Tobago
| | - SR Mohammed
- University of the West Indies, Trinidad and Tobago
| | - K Caesar
- St Clair Medical Centre, Trinidad and Tobago
| | - S Dindyal
- Mid and South Essex NHS Foundation Trust, UK
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Sarikaya S, Altas O, Ozgur MM, Hancer H, Yilmaz F, Karagoz A, Ozer T, Aksut M, Ozen Y, Kirali K. Treatment of Nutcracker Syndrome with Left Renal Vein Transposition and Endovascular Stenting. Ann Vasc Surg 2024; 102:110-120. [PMID: 38296038 DOI: 10.1016/j.avsg.2023.11.036] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2023] [Revised: 10/30/2023] [Accepted: 11/13/2023] [Indexed: 02/26/2024]
Abstract
BACKGROUND Nutcracker syndrome is a rare condition that occurs as a result of the entrapment of the left renal vein (LRV) between the aorta and the superior mesenteric artery. It is typically associated with symptoms such as left flank pain, hematuria, proteinuria, and pelvic congestion. The current treatment approach may be conservative in the presence of tolerable symptoms, and surgical or hybrid and stenting procedures in the order of priority in the presence of intolerable symptoms. The aim of this study is to review our experiences to evaluate the results of both methods in this series in which we have a greater tendency toward surgery instead of stenting. METHODS The clinical data of consecutive patients with nutcracker syndrome who underwent LRV transposition and LRV stenting between July 2019 and October 2023 were retrospectively reviewed. The patients were divided into 2 groups based on the methods of treatment: surgical and stenting. For procedure selection, LRV transposition was primarily recommended, with stenting offered to those who declined. Primary end points were morbidity and mortality. Secondary end points included late complications, patency, freedom from reintervention, and resolution of symptoms. Standard basic statistics and survival analysis methods were employed. RESULTS Nineteen patients with nutcracker syndrome (female: 100%) were treated with LRV stentings (n = 5) and LRV transposition (n = 14). The mean age was 24 (20-27, interquartile range [IQR]) years. The mean follow-up was 23 (9-32, IQR) months. There were no major complications and mortality after both procedures. The most frequent sign and symptom associated with LRV entrapment were left flank pain 100% (n = 19), proteinuria 88% (n = 15), and hematuria 47% (n = 9). The mean peak velocity ratio on Doppler ultrasound was 6.13 (6-6.44, IQR). Aortomesenteric angle, beak angle (beak sign), and mean diameter ratio on computed tomography were 26° (22.6-28.5, IQR), 25° (23.9-28, IQR), and 5.3 (5-6, IQR), respectively. Venous pressure measurements were only used to confirm the diagnosis in 5 patients in the stenting group. The measured renocaval gradient was 4 (3.9-4.4, IQR) mm Hg. After both procedures, the classical symptoms, including left flank pain, proteinuria, and hematuria, resolved in 89.5% (n = 17), 57.8% (n = 11), and 82.3% (n = 15) of the cases, respectively. A total of 4 patients required reintervention, 3 patients after LRV transposition (occlusion, n = 2; stenosis, n = 1), and 1 patient after stenting (occlusion, n = 1). The 1-year and 3-year primary patency for the 19 patients was 87% and 80%, respectively. Three-year primary-assisted patency was 100%. Similarly, the 1-year and 3-year freedom from reintervention rate was 83% and 72%, respectively. Additionally, the 1-year and 3-year primary patency for the surgical group was 91% and 81%, respectively, and the 1-year and 3-year primary patency for the stenting group was 75%. CONCLUSIONS Nutcracker syndrome should be kept in mind in cases where flank pain and hematuria cannot be associated with kidney diseases. Radiographic evidence must be accompanied by serious symptoms to initiate the treatment of nutcracker syndrome with LRV transposition and endovascular stenting procedures. Both procedures, along with their respective advantages and disadvantages, can be preferred as primary treatments for nutcracker syndrome. Our study demonstrates that both procedures can be safely and effectively performed, yielding good outcomes.
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Affiliation(s)
- Sabit Sarikaya
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey.
| | - Ozge Altas
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Mustafa Mert Ozgur
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Hakan Hancer
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Fatih Yilmaz
- Department of Cardiology, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Ali Karagoz
- Department of Cardiology, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Tanıl Ozer
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Mehmet Aksut
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Yucel Ozen
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
| | - Kaan Kirali
- Department of Cardiovascular Surgery, Kosuyolu Heart Training and Research Hospital, Istanbul, Turkey
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Gavrilov SG, Karalkin AV, Mishakina NY, Grishenkova AS. Effect of left renal vein compression stenosis on functional status of left kidney in patients with pelvic venous disorders. J Vasc Surg Venous Lymphat Disord 2024; 12:101668. [PMID: 37625506 PMCID: PMC11523412 DOI: 10.1016/j.jvsv.2023.08.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/24/2023] [Revised: 07/24/2023] [Accepted: 08/15/2023] [Indexed: 08/27/2023]
Abstract
OBJECTIVE We evaluated the effect of left renal vein (LRV) compression stenosis on the functional state of the left kidney in patients with pelvic venous disorders (PeVDs). METHODS We examined 162 female patients with PeVD and diagnosed LVR compression stenosis using duplex ultrasound (DUS) in 40. Patients with clinical manifestations of PeVD (n = 26) had symptoms and signs of pelvic venous congestion but without pain in the left flank of the abdomen, typical for nutcracker syndrome (NS). The remaining patients were asymptomatic (n = 14). The parameters measured with DUS included the angle of the superior mesenteric artery (SMA) with the aorta, the LRV diameter (Dhilum/Dstenosis) ratio, and LRV velocity (Vstenosis/Vhilum) ratio. All the patients underwent laboratory testing (complete blood count, urinalysis, and biochemical blood testing) and dynamic renal scintigraphy to assess the secretory and evacuation functions of the renal tubular system. RESULTS The laboratory tests revealed no abnormalities, including no hematuria or proteinuria, in either group. The Dhilum/Dstenosis and Vstenosis/Vhilum ratios varied from 2.8 to 5.2 and from 2.9 to 8.3, respectively, and did not differ between the symptomatic and asymptomatic patients. All 40 patients with LRV compression stenosis were diagnosed with left gonadal vein reflux with a mean duration of 4.7 ± 0.6 seconds and 2.2 ± 0.6 seconds in the symptomatic and asymptomatic patients, respectively (P = .005). Eight patients had signs of NS on DUS, including five in the symptomatic group (SMA angle, 34.8° ± 2.7°; Dhilum/Dstenosis ratio, 5.2 ± 0.2; and Vstenosis/Vhilum ratio, 5.7 ± 0.4) and three in the asymptomatic group (SMA angle, 35° ± 2.8°; Dhilum/Dstenosis ratio, 5; and Vstenosis/Vhilum ratio, 5 ± 0.5). The groups did not differ significantly in the DUS parameters. Scintigraphy did not reveal any cases of secretory or evacuation dysfunction of the left kidney, including in the patients with DUS signs of NS. The maximum uptake time, elimination half-life, and effective renal plasma flow were within the normal ranges. CONCLUSIONS LRV compression stenosis without hematuria has no significant effects on the functional state of the left kidney, irrespective of the disease severity. In patients with PeVDs, dynamic renal scintigraphy provides an objective assessment of left kidney function.
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Affiliation(s)
- Sergey G Gavrilov
- Savelyev University Surgical Clinic, Pirogov Russian National Research Medical University, Moscow, Russia.
| | - Anatoly V Karalkin
- Savelyev University Surgical Clinic, Pirogov Russian National Research Medical University, Moscow, Russia
| | - Nadezhda Yu Mishakina
- Savelyev University Surgical Clinic, Pirogov Russian National Research Medical University, Moscow, Russia
| | - Anastasiya S Grishenkova
- Savelyev University Surgical Clinic, Pirogov Russian National Research Medical University, Moscow, Russia
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Muacevic A, Adler JR, Almansour AA, Alghamdi A, Alsubhi MA. Nutcracker Phenomenon: A Rare Incidental Finding. Cureus 2022; 14:e32822. [PMID: 36570111 PMCID: PMC9774047 DOI: 10.7759/cureus.32822] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/22/2022] [Indexed: 12/24/2022] Open
Abstract
The nutcracker phenomenon, or left renal vein (LRV) entrapment syndrome, occurs when there is compression of the LRV, mostly between the abdominal aorta and the superior mesenteric artery. Patients with nutcracker syndrome (NCS) may present with various symptoms, with the most common being hematuria, left flank pain, varicocele in males, proteinuria, and anemia. Our 22-year-old male patient presented with abdominal pain without hematuria. Insidiously, we made the diagnosis of NCS with this unusual presentation. Some studies have proposed a relationship between rapid weight loss in a short period of time and the appearance of NCS. We recommend that healthcare providers suspect NCS in patients who present with abrupt severe abdominal discomfort, particularly in situations that coincide with rapid weight loss for an unknown reason.
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Pan Y, Wang S, Liu L, Liu X. The SLC6A19 gene mutation in a young man with hyperglycinuria and nephrolithiasis: a case report and literature review. BMC Urol 2022; 22:190. [PMID: 36434624 PMCID: PMC9700881 DOI: 10.1186/s12894-022-01147-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2022] [Accepted: 11/08/2022] [Indexed: 11/27/2022] Open
Abstract
BACKGROUND Hyperglycinuria is a rare disorder, with few reported cases, caused by either a defect in glycine metabolism or a disturbance in renal glycine reabsorption. Genetic findings of hyperglycinuria are rare and have not previously been reported in Chinese young men. CASE PRESENTATION A 24-year-old man presented with a compliant of bilateral lumbago for 1 month. Abdominal computed tomography revealed bilateral kidney stones and right upper ureteral dilatation. The 24-h urine analysis showed high urine oxalate levels of 63 mg/day. Analysis of amino acids in urine revealed that his urinary glycine levels were abnormally high (2.38 µmol/mg creatinine). Whole-exome sequencing detected the SLC6A19 variant c.1278 C > T p. (Cys426). Flexible ureteroscopy with holmium laser lithotripsy was conducted twice to remove his bilateral nephrolithiasis. Postoperative stone biochemical composition analysis revealed that the stones were composed of approximately 70% calcium oxalate monohydrate and 30% calcium oxalate dihydrate. The patient was subsequently diagnosed with hyperglycinuria. Three months after the stone surgery, ultrasonography revealed one nodule under the right thyroid lobe during a health checkup. His serum parathyroid hormone (PTH) levels increased to 392.3 pg/mL. Resection of the right parathyroid nodule was performed, and the histopathological examination confirmed right parathyroid adenoma. During the 2-year follow-up period, nephrolithiasis did not relapse, and serum PTH, calcium, and phosphorus levels were normal. CONCLUSION The SLC6A19 gene may have been significant in the development of hyperglycinuria in a Chinese young man. Further evaluation for the possibility of a glycine excretion disorder could be considered when encountering nephrolithiasis.
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Affiliation(s)
- Yang Pan
- grid.412645.00000 0004 1757 9434Department of Urology, Tianjin Medical University General Hospital, No. 154 Road Anshan, Heping District, 30052 Tianjin, China
| | - Shangren Wang
- grid.412645.00000 0004 1757 9434Department of Urology, Tianjin Medical University General Hospital, No. 154 Road Anshan, Heping District, 30052 Tianjin, China
| | - Li Liu
- grid.412645.00000 0004 1757 9434Department of Urology, Tianjin Medical University General Hospital, No. 154 Road Anshan, Heping District, 30052 Tianjin, China
| | - Xiaoqiang Liu
- grid.412645.00000 0004 1757 9434Department of Urology, Tianjin Medical University General Hospital, No. 154 Road Anshan, Heping District, 30052 Tianjin, China
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Meyer J, Rother U, Stehr M, Meyer A. Nutcracker syndrome in children: Appearance, diagnostics, and treatment - A systematic review. J Pediatr Surg 2022; 57:716-722. [PMID: 35065803 DOI: 10.1016/j.jpedsurg.2021.12.019] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2021] [Revised: 12/13/2021] [Accepted: 12/14/2021] [Indexed: 10/31/2022]
Abstract
BACKGROUND The nutcracker syndrome (NCS) is defined by compression of the left renal vein (LVR) and may present with a wide variety of symptoms. Due to its rarity in pediatric patients, incidence, diagnostics and performed therapy regimen are widely undefined. To this date, there are only case reports and small collectives of pediatric patients described but comprehensive research is lacking. METHODS A systematic literature research on pediatric NCS was carried out on Medline and Scopus databases according to PRISMA principles using predefined search terms and inclusion criteria. The PROSPERO registered review (CRD42021237415) identified patients' characteristics regarding age, sex, clinical symptoms, applied diagnostic methods and treatment options. RESULTS In total 47 articles were included. Overall, 423 children (218 boys and 205 girls) with diagnosed NCS were included in the analysis. Mean age was 12.0 (boys 12.9, girls 12.0) years. Hematuria was most common presentation (55.5%), followed by proteinuria (49.9%). Classical flank pain was only detected in 19.1% of patients. Sonographic evaluation was the most commonly used diagnostic tool (99%). Invasive diagnostic studies were performed in 97 children. 86.8% patients were treated conservatively and 94.9% showed complete resolution (42.8%) or at least improvement (52.2%) of symptoms. Type of operative treatment comprised of open surgery with transposition of LRV, endovascular stenting and laparoscopy. CONCLUSIONS Overall, data quality regarding NCS in children is poor. However, conservative approach in pediatric patients is recommended and should be regarded first treatment option. Diagnostic and treatment should follow a defined algorithm when NCS is suspected. Sufficient observation and follow-up must be assured in all patients to get significant results in this heterogenous syndrome. LEVEL OF EVIDENCE V-IV.
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Affiliation(s)
- Johannes Meyer
- Department for Pediatric Surgery and Pediatric Urology, Cnopfsche Children´s Hospital, St.-Johannis Mühlgasse 19, Nürnberg 90419, Germany.
| | - Ulrich Rother
- Department of Vascular Surgery, University Hospital Erlangen, Krankenhausstraße 12, Erlangen 91054, Germany
| | - Maximilian Stehr
- Department for Pediatric Surgery and Pediatric Urology, Cnopfsche Children´s Hospital, St.-Johannis Mühlgasse 19, Nürnberg 90419, Germany
| | - Alexander Meyer
- Department of Vascular Surgery, University Hospital Erlangen, Krankenhausstraße 12, Erlangen 91054, Germany
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Jiang Y, Gan Z, Wang Q, Chen Y, Jiang Y. Bibliometric and visual analysis of research on nutcracker syndrome from 1974 to 2021: A systematic review. Medicine (Baltimore) 2022; 101:e29939. [PMID: 35945728 PMCID: PMC9351850 DOI: 10.1097/md.0000000000029939] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/05/2023] Open
Abstract
BACKGROUND At present, researchers have obtained fruitful results in the study of nutcracker syndrome (NCS), but there is still a lack of systematic research on the overall status of this disease. This article aims to describe the past and current status of research into NCS, and predict future research trends and popular research topics. METHODS Using bibliometric and visualization methods, 552 articles related to NCS collected from the Scopus database from 1974 to 2021 were analyzed from multiple perspectives. RESULTS Overall, the amount of literature related to NCS is on the rise every year, and the number of citations is the turning point in 2006. The United States has the largest number of publications and has the most extensive cooperation with other countries. The main contents of the co-authored study focused on the symptoms, surgical procedures, and concomitant diseases of NCS. Keywords such as peak velocity, ultrasonography, orthostatic proteinuria, etc appeared earlier, whereas diagnosis, chronic pelvic pain, endovascular stents, etc appeared later. CONCLUSIONS The literature utilization rate of NCS is relatively insufficient. The pathogenesis and pathological mechanisms need to be further studied, and the diagnostic criteria and surgical methods will continue to be favored by clinicians.
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Affiliation(s)
- Yuchang Jiang
- School of Basic Medicine, Chengdu University of Traditional Chinese Medicine, Chengdu, China
| | - Zaili Gan
- Institute of Chinese Medicine Literature, Nanjing University of Chinese Medicine, Nanjing, China
| | - Qinsheng Wang
- Traditional Chinese Medicine Hospital of Jiangbei District, Chongqing, China
| | - Yang Chen
- College of acupuncture and massage, Chengdu University of traditional Chinese Medicine, Chengdu, China
| | - Yong Jiang
- School of Basic Medicine, Chengdu University of Traditional Chinese Medicine, Chengdu, China
- *Correspondence: Yong Jiang, School of Basic Medicine, Chengdu University of Traditional Chinese Medicine, No. 37 Shierqiao Road, Jinniu District, Chengdu 610072, China (e-mail: )
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Franco-Mesa C, Gloviczki P, Erben Y. Nutcracker Syndrome. THE JOURNAL OF CARDIOVASCULAR SURGERY 2021; 62:467-471. [PMID: 34105927 DOI: 10.23736/s0021-9509.21.11923-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
We provide a concise review of Nutcracker Syndrome with its history, incidence, clinical presentation and currently available diagnostic criteria and treatment options. This should help any clinician identify and better serve patients with rare venous disorders such as this. Although the literature is scarce, clinicians should be keen to diagnose and treat patients with this potentially debilitating syndrome.
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Affiliation(s)
- Camila Franco-Mesa
- Division of Vascular and Endovascular Surgery, Mayo Clinic, Jacksonville, FL, USA
| | - Peter Gloviczki
- Division of Vascular and Endovascular Surgery, Mayo Clinic, Rochester, MN, USA
| | - Young Erben
- Division of Vascular and Endovascular Surgery, Mayo Clinic, Jacksonville, FL, USA -
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An unusual case of nutcracker phenomenon treated by percutaneous stenting. JOURNAL DE MÉDECINE VASCULAIRE 2021; 46:152-154. [PMID: 33990291 DOI: 10.1016/j.jdmv.2021.03.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/10/2020] [Accepted: 02/22/2021] [Indexed: 11/24/2022]
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14
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Karami M, Kouhi H, Sadatmadani SF, Sadeghi B, Rostamiyan N, Hashemzadeh M. Splenic vein enlargement, a rare cause of nutcracker syndrome. Clin Case Rep 2021; 9:e03833. [PMID: 34084474 PMCID: PMC8142411 DOI: 10.1002/ccr3.3833] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2020] [Revised: 11/01/2020] [Accepted: 11/13/2020] [Indexed: 11/13/2022] Open
Abstract
Abnormal enlargement of the splenic vein is one of the etiologies of nutcracker syndrome that should be considered when examining the causes of this syndrome. Because knowing rare etiologies can help correct diagnose and reduce mortality.
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Affiliation(s)
- Mehdi Karami
- Department of RadiologySchool of Medicine, Isfahan University of Medical SciencesIsfahanIran
| | - Hossein Kouhi
- Department of RadiologySchool of Medicine, Isfahan University of Medical SciencesIsfahanIran
| | | | - Bahar Sadeghi
- Department of RadiologySchool of Medicine, Isfahan University of Medical SciencesIsfahanIran
| | - Narges Rostamiyan
- Department of RadiologySchool of Medicine, Isfahan University of Medical SciencesIsfahanIran
| | - Mozhdeh Hashemzadeh
- Department of Medical Library and Information ScienceSchool of Management and Medical Information Sciences, Clinical Informationist Research Group, Isfahan University of Medical SciencesIsfahanIran
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15
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Falsetti L, Martino GP, Mattioli M, Menetti S, Serra C. Chronic abdominal pain and haematuria in internal medicine: do not forget about the Nutcracker syndrome. Intern Emerg Med 2021; 16:443-445. [PMID: 32951161 DOI: 10.1007/s11739-020-02426-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/07/2020] [Accepted: 06/27/2020] [Indexed: 10/23/2022]
Affiliation(s)
- Lorenzo Falsetti
- Internal and Sub-Intensive Medicine Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti"di Ancona, Via Conca 10, Ancona, Italy.
| | | | - Massimo Mattioli
- UOC Emergency Department, Azienda Ospedaliera "Ospedali Riuniti Marche Nord", Pesaro, Italy
| | - Saverio Menetti
- Internal Medicine, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy
| | - Carla Serra
- Department of Organ Failure and Transplantation, Ultrasound Unit, S.Orsola-Malpighi Hospital, Bologna, Italy
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16
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Granata A, Distefano G, Sturiale A, Figuera M, Foti PV, Palmucci S, Basile A. From Nutcracker Phenomenon to Nutcracker Syndrome: A Pictorial Review. Diagnostics (Basel) 2021; 11:101. [PMID: 33440614 PMCID: PMC7826835 DOI: 10.3390/diagnostics11010101] [Citation(s) in RCA: 29] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2020] [Revised: 01/02/2021] [Accepted: 01/06/2021] [Indexed: 02/07/2023] Open
Abstract
Left renal vein (LRV) entrapment, also known as nutcracker phenomenon if it is asymptomatic, is characterized by abnormality of outflow from the LRV into the inferior vena cava (IVC) due to extrinsic LRV compression, often accompanied by demonstrable lateral (hilar) dilatation and medial (mesoaortic) stenosis. Nutcracker syndrome, on the other hand, includes a well-defined set of symptoms, and the severity of these clinical manifestations is related to the severity of anatomic and hemodynamic findings. With the aim of providing practical guidance for nephrologists and radiologists, we performed a review of the literature through the PubMed database, and we commented on the definition, the main clinical features, and imaging pattern of this syndrome; we also researched the main therapeutic approaches validated in the literature. Finally, from the electronic database of our institute, we have selected some characteristic cases and we have commented on the imaging pattern of this disease.
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Affiliation(s)
- Antonio Granata
- Nephrology and Dialysis Unit, “Cannizzaro” Hospital, 95026 Catania, Italy; (A.G.); (A.S.)
| | - Giulio Distefano
- Radiology Unit I, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital “Policlinico—San Marco”, University of Catania, 95123 Catania, Italy; (P.V.F.); (S.P.); (A.B.)
| | - Alessio Sturiale
- Nephrology and Dialysis Unit, “Cannizzaro” Hospital, 95026 Catania, Italy; (A.G.); (A.S.)
| | - Michele Figuera
- Radiology Unit II, University Hospital “Policlinico—San Marco”, 95123 Catania, Italy;
| | - Pietro Valerio Foti
- Radiology Unit I, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital “Policlinico—San Marco”, University of Catania, 95123 Catania, Italy; (P.V.F.); (S.P.); (A.B.)
| | - Stefano Palmucci
- Radiology Unit I, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital “Policlinico—San Marco”, University of Catania, 95123 Catania, Italy; (P.V.F.); (S.P.); (A.B.)
| | - Antonio Basile
- Radiology Unit I, Department of Medical Surgical Sciences and Advanced Technologies “GF Ingrassia”, University Hospital “Policlinico—San Marco”, University of Catania, 95123 Catania, Italy; (P.V.F.); (S.P.); (A.B.)
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17
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Byun E, Kim H, Park S, Han Y, Kwon TW, Cho YP. Body Mass Index of Patients with Nutcracker Syndrome. Phlebology 2020. [DOI: 10.37923/phle.2020.18.3.51] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- Eunae Byun
- Division of Vascular Surgery, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
| | - Hyangkyoung Kim
- Division of Vascular Surgery, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
| | - Seunghee Park
- Division of Vascular Surgery, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
| | - Youngjin Han
- Division of Vascular Surgery, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
| | - Tae-Won Kwon
- Division of Vascular Surgery, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
| | - Yong-Pil Cho
- Division of Vascular Surgery, Department of Surgery, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea
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18
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Ribeiro FS, Puech-Leão P, Zerati AE, Nahas WC, David-Neto E, De Luccia N. Prevalence of left renal vein compression (nutcracker phenomenon) signs on computed tomography angiography of healthy individuals. J Vasc Surg Venous Lymphat Disord 2020; 8:1058-1065. [DOI: 10.1016/j.jvsv.2020.04.005] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2020] [Accepted: 04/08/2020] [Indexed: 01/18/2023]
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Yadav A, Maley W, Singh P. An Unusual Case of Proteinuria in a Kidney Donor. Kidney Int Rep 2020; 5:1360-1362. [PMID: 32775841 PMCID: PMC7403544 DOI: 10.1016/j.ekir.2020.05.012] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2020] [Revised: 04/28/2020] [Accepted: 05/04/2020] [Indexed: 11/29/2022] Open
Affiliation(s)
- Anju Yadav
- Division of Nephrology, Department of Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA
| | - Warren Maley
- Division of Transplantation, Department of Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania, USA
| | - Pooja Singh
- Division of Nephrology, Department of Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA
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20
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Microsurgical gonadal-inferior epigastric vein anastomosis to treat the nutcracker phenomenon with left gonadal vein varices with reflux. Int Urol Nephrol 2020; 52:1629-1635. [PMID: 32338319 DOI: 10.1007/s11255-020-02478-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2020] [Accepted: 04/16/2020] [Indexed: 10/24/2022]
Abstract
OBJECTIVE To evaluate the effectiveness and safety of microsurgical gonadal-inferior epigastric vein anastomosis for the treatment of the nutcracker phenomenon (NCP) associated with left gonadal vein varices with reflux. METHODS Thirty-five patients with NCP associated with left gonadal vein varices with reflux diagnosed in our hospital from June 2016 to June 2018 were included. All patients underwent a shunt operation consisting of microsurgical gonadal-inferior epigastric vein anastomosis, and the patients were followed up for 1 year. RESULTS All patients were successfully operated on, with an average operation time of 96.5 ± 12.3 min. After a 1-year follow-up, the symptom of gross hematuria disappeared in 3 patients (including 1 woman). For the other 32 patients, the sperm concentration (27.43 ± 8.68 × 106/ml) and motility (33.06 ± 4.27%) postoperatively were significantly higher than that preoperatively (16.21 ± 6.43 × 106/ml and 23.48 ± 4.43%, respectively) (P < 0.05); among these patients, 2 had natural pregnancies with their spouses. The peak velocity (PV) at the aortomesenteric portion of the left renal vein (LRV) and the PV ratio between the aortomesenteric and hilar portion of the LRV significantly decreased after surgery (117.9 ± 30.4 cm/s vs 76.6 ± 18.5 cm/s; 7.3 ± 0.7 vs 4.1 ± 0.4). Two patients had complications of mild hydroceles requiring no intervention, and no major complications were observed during and after surgery. CONCLUSION Our results suggest that the microsurgical gonadal-inferior epigastric vein anastomosis is both effective and safe to treat patients with gonadal varicose veins caused by the nutcracker phenomenon.
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Nakashima T, Sahashi Y, Kanamori H, Ohno Y, Okura H. Localized solitary left renal vein thrombus complicating nutcracker syndrome: a case report and review of the literature. CEN Case Rep 2020; 9:252-256. [PMID: 32246272 DOI: 10.1007/s13730-020-00467-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2020] [Accepted: 03/13/2020] [Indexed: 11/28/2022] Open
Abstract
Nutcracker syndrome (NCS) refers to compression of the left renal vein (LRV) between the abdominal aorta and the superior mesenteric artery (SMA). The clinical presentation of NCS includes hematuria, abdominal and left flank pain, gonadal varices, and varicocele formation. Theoretically, thrombosis can occur in the LRV in patients with NCS. However, an isolated solitary left renal vein thrombus (LRVT) complicating NCS is rare. In addition, the clinical features of an LRVT complicating NCS remain unclear. We describe a 43-year-old woman presenting with an asymptomatic LRVT complicating NCS. She was referred to our hospital for investigation of dysfunctional uterine bleeding, and detailed examination revealed endometrial cancer. Computed tomography angiography (CTA) and Doppler ultrasonography revealed compression of the LRV between the aorta and the SMA, as well as an LRVT. CTA performed 4 months after the administration of an anticoagulant showed complete disappearance of the LRVT. We have also included a review of published reports describing LRVT complicating NCS and discussed the clinical features of such a presentation.
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Affiliation(s)
- Takashi Nakashima
- Department of Cardiology, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu, 501-1194, Japan.
| | - Yuki Sahashi
- Department of Cardiology, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Hiromitsu Kanamori
- Department of Cardiology, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Yasushi Ohno
- Department of Respirology, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Hiroyuki Okura
- Department of Cardiology, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu, 501-1194, Japan
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Eighteen years of experience with pediatric nutcracker syndrome: the importance of the conservative approach. J Pediatr Urol 2020; 16:218.e1-218.e6. [PMID: 32044256 DOI: 10.1016/j.jpurol.2019.12.003] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2018] [Accepted: 12/02/2019] [Indexed: 12/29/2022]
Abstract
INTRODUCTION Nutcracker syndrome (NS) defines an entity generated by the compression of the left renal vein resulting in venous hypertension, which transmitted in a retrograde direction may cause hematuria, proteinuria, and varicocele. The literature concerning exclusively pediatric patients is very rare. OBJECTIVE To report the authors' experience with pediatric NS in the last 18 years. STUDY DESIGN This is a retrospective review of the patients followed up in the authors' center with diagnosis of NS based on clinical and imaging tests (ultrasound, computed tomography/magnetic resonance imaging, and phlebography). The primary outcome was the success of the conservative approach in the study patients. RESULTS AND DISCUSSION A total of 21 patients were diagnosed with NS and followed up for a mean period of 52.3 months (37.1-67.5). The mean age at diagnosis was 11.7 years (9.9-13.4). The most frequent symptom of presentation was hematuria in 16 patients (76.2%), being macroscopic in 75% patients and related to physical exercise in 42.9% patients. Other symptoms were left varicocele in 7 patients (33%) and proteinuria in 6 patients (28.6%). Mild to moderate cases received conservative treatment (change of physical activity, postural hygiene), which achieved resolution of symptoms in 16 patients (76.2%). Five cases (23.8%) finally needed a more aggressive approach. Two (9.5%) of them required endovascular procedures (intravascular stent in the renal vein in 1 case and embolization of the spermatic vein in 1 case); in one (4.8%) of the patients, transposition of the left renal vein and kidney autotransplantation were performed, and 2 (9.5%) of the patients with mild cases required surgical correction of the varicocele. CONCLUSIONS Hematuria, usually macroscopic and related to physical exercise, is the most frequent symptom in pediatric patients with NS. The authors advocate studying the aortomesenteric junction by abdominal ultrasound in patients with varicocele or with intermittent macroscopic hematuria. Diagnosis is based on non-invasive tests; phlebography should be reserved for severe cases that require an interventionist attitude. A long period of conservative treatment is the first approach for pediatric patients with NS.
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Neppala P, Chau HS, Sood D, Berumen J, Mekeel KL. Renal Autotransplantation for Nutcracker Kidney after Prior Right Nephrectomy. Am Surg 2020. [DOI: 10.1177/000313482008600217] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Affiliation(s)
- Pushpa Neppala
- University of California San Diego School of Medicine La Jolla, California
| | - Harrison S. Chau
- Department of Surgery University of California, San Diego La Jolla, California
| | - Divya Sood
- Department of Surgery University of California, San Diego La Jolla, California
| | - Jennifer Berumen
- Division of Transplantation and Hepatobiliary Surgery Department of Surgery University of California, San Diego La Jolla, California
| | - Kristin L. Mekeel
- Division of Transplantation and Hepatobiliary Surgery Department of Surgery University of California, San Diego La Jolla, California
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Nishio Y, Kawano Y, Hara S. Nutcracker syndrome complicated with intestinal malrotation. BMJ Case Rep 2019; 12:12/10/e231230. [PMID: 31604719 DOI: 10.1136/bcr-2019-231230] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2023] Open
Abstract
Nutcracker syndrome (NCS) is a pathological condition in which the left renal vein (LRV) is compressed between the superior mesenteric artery (SMA) and aorta. NCS can predispose patients to the onset of chronic kidney disease because of persistent increase in LRV pressure. Although NCS in children is often idiopathic, it can also be caused by underlying pathologies such as retroperitoneal tumours. To the best of our knowledge, there have been no reports regarding paediatric cases of NCS complicated with intestinal malrotation. Here, we report the case of a 12-year-old girl with intestinal malrotation complicated with NCS whose haematuria resolved after surgical intervention for intestinal malrotation. The present case findings indicate that intestinal malrotation with concomitant weight loss is a potential underlying aetiology in NCS. Thus, when NCS is especially diagnosed with gastrointestinal symptoms, intestinal malrotation should be considered as an underlying aetiology.
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Affiliation(s)
- Yosuke Nishio
- Pediatrics, TOYOTA Memorial Hospital, Toyota, Aichi, Japan
| | | | - Shinya Hara
- Pediatrics, TOYOTA Memorial Hospital, Toyota, Aichi, Japan
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Wang H, Guo YT, Jiao Y, He DL, Wu B, Yuan LJ, Li YY, Yang Y, Cao TS, Zhang B. A minimally invasive alternative for the treatment of nutcracker syndrome using individualized three-dimensional printed extravascular titanium stents. Chin Med J (Engl) 2019; 132:1454-1460. [PMID: 31205104 PMCID: PMC6629333 DOI: 10.1097/cm9.0000000000000255] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/25/2018] [Indexed: 01/17/2023] Open
Abstract
BACKGROUND This study was designed to evaluate the clinical and radiographic outcomes of patients with nutcracker syndrome (NCS) who were treated with three-dimensional printing (3DP) extravascular titanium stents (EVTSs). The 3DP EVTS was expected to release the hypertension of the left renal vein (LRV) produced by its compression between the superior mesenteric artery (SMA) and the aorta without causing any complications. METHOD The pre-operative kidney model of each patient was printed out to enable surgical planning. After that, the EVTS was designed based on the LRV's primitive physiologic structure using computer-aided design software, and each stent was printed out with a precision setting of 20 μm. Seventeen patients who had been suffering from NCS underwent laparoscopic 3DP EVTS placement. The surgical procedure was designed for the placement of EVTS, taking great care in positioning and fixing the stent. Surgical data, which included patient demographic characteristics as well as pre- and post-operative test results, were collected and analyzed. RESULTS The mean duration of surgery was 75 ± 9 min, and the mean blood loss was 20 ± 5 mL. Computed tomography examinations revealed that the pre- and post-operative angle between the SMA and the aorta ranged from 18.7° ± 4.3° to 48.0° ± 8.8° (P < 0.05); in patients with left varicocele, the mean diameter of the left spermatic vein ranged from 3.7 ± 0.5 to 1.3 ± 0.2 mm (P < 0.05). Moreover, Doppler ultrasound examinations showed that the peak velocity of blood flow at the hilar area ranged from 12.4 ± 3.3 to 18.5 ± 3.4 cm/s (P < 0.05). No side effects were observed in the 24 to 42 months following surgery. CONCLUSION The findings after 2 years of follow-up suggest that the 3DP EVTS is a safe and effective minimally invasive alternative for the treatment of NCS.
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Affiliation(s)
- He Wang
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Yi-Tong Guo
- Department of Ultrasound Diagnosis, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
- 3D Printing Research Center of The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Yong Jiao
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Da-Li He
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Bin Wu
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Li-Jun Yuan
- Department of Ultrasound Diagnosis, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
- 3D Printing Research Center of The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Yan-Yan Li
- Department of Radiology, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Yong Yang
- Department of Ultrasound Diagnosis, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Tie-Sheng Cao
- Department of Ultrasound Diagnosis, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
- 3D Printing Research Center of The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
| | - Bo Zhang
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
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Sugaya K, Nishijima S, Kadekawa K, Noguchi K, Ashitomi K, Ueda T, Yamamoto H. Pelvic venous congestion induces lower urinary tract dysfunction in rats. Biomed Res 2018; 39:269-277. [PMID: 30531156 DOI: 10.2220/biomedres.39.269] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
Pelvic venous congestion (PC) is thought to be related to several diseases of the lower urinary tract (LUT). We examined the characteristics of the LUT in rats with PC. To create PC, female rats were anesthetized with isoflurane, and the bilateral common iliac veins and bilateral uterine veins were ligated. At 1-8 weeks after either ligation or sham surgery, we performed cystometry with or without administration of carbazochrome sodium sulfonate hydrate or propiverine hydrochloride, histologic examination of the bladder, blood flow imaging, assessment of locomotor activity, measurement of urinary 8-hydroxydeoxyguanosine (8-OHdG) and nitric oxide metabolites (NOx), and the Evans blue dye extravasation test. PC elevated frequency of urination after 2-6 weeks, and caused a decrease of spontaneous locomotor activity. In addition, there was a decrease of bladder blood flow, an increase of bladder vascular permeability, an increase of urinary 8-OHdG, a decrease of urinary NOx, and mild inflammatory changes of the bladder. In rats with PC, frequency of urination was normalized by administration of propiverine or carbazochrome. Rats with PC may be used as a model of PC associated with high frequency of urination, and this model may be useful when developing treatment for LUT symptoms associated with PC.
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Affiliation(s)
| | | | | | | | | | - Tomoyuki Ueda
- The Institute for Animal Experiments, Faculty of Medicine, University of the Ryukyus
| | - Hideyuki Yamamoto
- Department of Biochemistry, Graduate School of Medicine, University of the Ryukyus
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Shi Y, Yang H, Feng Z, Chen F, Zhang H, Wu Z. Evaluation of posterior nutcracker phenomenon using multisection spiral CT. Clin Radiol 2018; 73:1060.e9-1060.e16. [PMID: 30224187 DOI: 10.1016/j.crad.2018.07.110] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2018] [Accepted: 07/30/2018] [Indexed: 10/28/2022]
Abstract
AIM To prospectively evaluate the epidemiological, anatomical classification, clinical importance, and radiological features of posterior nutcracker phenomenon (PNP) in patients examined with multisection spiral computed tomography (MSCT). MATERIAL AND METHODS Patients who underwent abdominal enhanced MSCT from January 2012 to July 2016 were evaluated retrospectively. Combined with the blood, normal biochemistry, and urine tests, patients were assessed retrospectively for renal vein diameter measurements, the compression ratio of blood vessels, gender differences, anatomical classification, percentage of proteinuria and haematuria, and clinical symptoms. RESULTS The study included 6,225 consecutive patients, PNP was observed in 2.06% involving the CLRV in 1.22% and retro-aortic left renal vein in 0.84%. The proportion of posterior nutcracker syndrome (PNS) was lower in PNP (17.2%). There was no difference by sex and age in the prevalence of PNP (p>0.05). In patients with urological symptoms, the number of type II abnormalities was more frequent than type Ia and type Ib abnormalities. Meanwhile, the number of type II abnormalities was frequent than the other two types. There was a significant difference between PNS and asymptomatic PNP (aPNP) in all measured values (p<0.001), the ratio was >4, especially in type II abnormalities (ratio >5.0). CONCLUSIONS PNP is a type of left renal vein entrapment. It is exceptionally rare and might be underdiagnosed. MSCT and measured values can be useful in diagnosis and recognition.
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Affiliation(s)
- Y Shi
- Department of Radiology, The First Affiliated Hospital of Medical School of Zhejiang University, Hangzhou, 310003, PR China
| | - H Yang
- Department of Radiology, The First Affiliated Hospital of Medical School of Zhejiang University, Hangzhou, 310003, PR China
| | - Z Feng
- Department of Radiology, The First Affiliated Hospital of Medical School of Zhejiang University, Hangzhou, 310003, PR China
| | - F Chen
- Department of Radiology, The First Affiliated Hospital of Medical School of Zhejiang University, Hangzhou, 310003, PR China
| | - H Zhang
- Department of Vascular Surgery, The First Affiliated Hospital of Medical School of Zhejiang University, Hangzhou, 310003, PR China
| | - Z Wu
- Department of Vascular Surgery, The First Affiliated Hospital of Medical School of Zhejiang University, Hangzhou, 310003, PR China.
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28
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de Macedo GL, Dos Santos MA, Sarris AB, Gomes RZ. Diagnosis and treatment of the Nutcracker syndrome: a review of the last 10 years. J Vasc Bras 2018; 17:220-228. [PMID: 30643508 PMCID: PMC6326141 DOI: 10.1590/1677-5449.012417] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
The nutcracker syndrome is characterized by a group of clinical manifestations caused by compression of the Left Renal Vein. The main symptoms are: macro and micro hematuria, proteinuria, and flank pain. Diagnosis is usually made after excluding other causes, because there are no clinical criteria for diagnosis. Confirmation is by Doppler ultrasonography or computed tomography. Treatment can vary, depending on patient characteristics and the severity of the symptoms, while conservative treatment, open surgery, and endovascular surgery may be employed. Currently, open surgery is still the first-line treatment, but some less invasive approaches are gaining acceptance.
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Affiliation(s)
| | | | - Andrey Biff Sarris
- Universidade Estadual de Ponta Grossa - UEPG, Departamento de Medicina Ponta Grossa, PR, Brasil
| | - Ricardo Zanetti Gomes
- Universidade Estadual de Ponta Grossa - UEPG, Departamento de Medicina Ponta Grossa, PR, Brasil
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Nalcacioglu H, Ceyhan Bilgici M, Tekcan D, Genc G, Bostanci Y, Yakupoglu YK, Sarikaya S, Ozkaya O. Nutcracker Syndrome in Children: Role of Doppler Ultrasonographic Indices in Detecting the Pattern of Symptoms. J Clin Med 2018; 7:jcm7080214. [PMID: 30104539 PMCID: PMC6111325 DOI: 10.3390/jcm7080214] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2018] [Revised: 07/29/2018] [Accepted: 08/08/2018] [Indexed: 12/20/2022] Open
Abstract
The purpose of this study was to evaluate the clinical characteristics of 44 pediatric patients who were diagnosed as having nutcracker syndrome (NCS). We also investigated the left renal vein Doppler ultrasonography (DUS) results, to determine whether or not there was an association between clinical symptoms and DUS findings among these patients. The clinical data from 44 pediatric patients who were diagnosed as having NCS from January 2008 to December 2015 were retrospectively reviewed. We grouped the patients according to the presenting symptoms as symptomatic (loin pain; macroscopic hematuria or both) and non-symptomatic (microscopic hematuria and proteinuria were detected incidentally) and evaluated the left renal vein DUS indices in these two groups separately. Asymptomatic NCS was found in 27 (61.4%) patients; 21 (47.7%) of whom were admitted for the evaluation of proteinuria. The most frequent presenting symptoms were left flank pain (20.5%) and macroscopic hematuria (13.6%); and 2 (4.5%) patients presented with a combination of left flank pain and macroscopic hematuria. The mean ratio of the diameter of the hilar portion of the left renal vein (LRV) to that of the aortomesenteric portion was 4.36 ± 1.55. The mean ratio of the peak velocity (PV) between the two sites of the LRV was 7.32 ± 2.68 (3.1–15.6). The differences in the ratio of the diameters were statistically significant between the two groups and significantly higher in children with asymptomatic NCS (p = 0.025). The PV ratios of the LRV (p = 0.035) were significantly higher in asymptomatic children with NCS than in the symptomatic group. Our study identifies that increased compression ratio of the LRV entrapment is most observed in orthostatic proteinuria and microscopic hematuria.
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Affiliation(s)
- Hulya Nalcacioglu
- Pediatric Nephrology Department, Ondokuz Mayis University Faculty of Medicine, 55220 Samsun, Turkey.
| | - Meltem Ceyhan Bilgici
- Pediatric Radiology Department, Ondokuz Mayis University Faculty of Medicine, 55220 Samsun, Turkey.
| | - Demet Tekcan
- Pediatric Nephrology Department, Ondokuz Mayis University Faculty of Medicine, 55220 Samsun, Turkey.
| | - Gurkan Genc
- Pediatric Nephrology Department, Ondokuz Mayis University Faculty of Medicine, 55220 Samsun, Turkey.
| | - Yakup Bostanci
- Urology Department, Ondokuz Mayis University Faculty of Medicine, 55220 Samsun, Turkey.
| | | | - Saban Sarikaya
- Pediatric Urology Department, Ondokuz Mayis University Faculty of Medicine, 55220 Samsun, Turkey.
| | - Ozan Ozkaya
- Pediatric Nephrology Department, Istinye University Faculty of Medicine, 34010 Istanbul, Turkey.
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Hipertensión arterial secundaria como manifestación clínica del síndrome de nutcracker. ANGIOLOGIA 2018. [DOI: 10.1016/j.angio.2018.04.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
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Hangge PT, Gupta N, Khurana A, Quencer KB, Albadawi H, Alzubaidi SJ, Knuttinen MG, Naidu SG, Oklu R. Degree of Left Renal Vein Compression Predicts Nutcracker Syndrome. J Clin Med 2018; 7:jcm7050107. [PMID: 29738433 PMCID: PMC5977146 DOI: 10.3390/jcm7050107] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2018] [Revised: 05/02/2018] [Accepted: 05/03/2018] [Indexed: 11/16/2022] Open
Abstract
Nutcracker syndrome (NS) refers to symptomatic compression of the left renal vein (LRV) between the abdominal aorta and superior mesenteric artery with potential symptoms including hematuria, proteinuria, left flank pain, and renal venous hypertension. No consensus diagnostic criteria exist to guide endovascular treatment. We aimed to evaluate the specificity of LRV compression to NS symptoms through a retrospective study including 33 NS and 103 control patients. The size of the patent lumen at point of compression and normal portions of the LRV were measured for all patients. Multiple logistic regression analyses (MLR) assessing impact of compression, body mass index (BMI), age, and gender on the likelihood of each symptom with NS were obtained. NS patients presented most commonly with abdominal pain (72.7%), followed by hematuria (57.6%), proteinuria (39.4%), and left flank pain (30.3%). These symptoms were more commonly seen than in the control group at 10.6, 11.7, 6.8, and 1.9%, respectively. The degree of LRV compression for NS was 74.5% and 25.2% for controls (p < 0.0001). Higher compression led to more hematuria (p < 0.0013), abdominal pain (p < 0.006), and more proteinuria (p < 0.002). Furthermore, the average BMI of NS patients was 21.4 and 27.2 for controls (p < 0.001) and a low BMI led to more abdominal pain (p < 0.005). These results demonstrate a strong correlation between the degree of LRV compression on imaging in diagnosing NS.
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Affiliation(s)
- Patrick T Hangge
- Department of General Surgery, Mayo Clinic, Phoenix, AZ 85054, USA.
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
| | - Nikhil Gupta
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
- Tufts University School of Medicine, Boston, MA 02111, USA.
| | - Aditya Khurana
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
- Mayo Clinic School of Medicine, Mayo Clinic, Scottsdale, AZ 85259, USA.
| | - Keith B Quencer
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
- Division of Interventional Radiology, Department of Radiology, University of Utah, Salt Lake City, UT 84112, USA.
| | - Hassan Albadawi
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
| | - Sadeer J Alzubaidi
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
| | - M-Grace Knuttinen
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
| | - Sailendra G Naidu
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
| | - Rahmi Oklu
- Division of Vascular and Interventional Radiology, Minimally Invasive Therapeutics Laboratory, Mayo Clinic, Phoenix, AZ 85054, USA.
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What Each Clinical Anatomist Has to Know about Left Renal Vein Entrapment Syndrome (Nutcracker Syndrome): A Review of the Most Important Findings. BIOMED RESEARCH INTERNATIONAL 2017; 2017:1746570. [PMID: 29376066 PMCID: PMC5742442 DOI: 10.1155/2017/1746570] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 04/14/2017] [Accepted: 10/12/2017] [Indexed: 12/21/2022]
Abstract
Nutcracker syndrome (NCS) is the most common term for compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. The development of NCS is associated with the formation of the left renal vein (LRV) from the aortic collar during the sixth to eighth week of gestation and abnormal angulation of the superior mesenteric artery from the aorta. Collateralization of venous circulation is the most significant effect of NCS. It includes mainly the left gonadal vein and the communicating lumbar vein. Undiagnosed NCS may affect retroperitoneal surgery and other radiological and vascular procedures. The clinical symptoms of NCS may generally be described as renal presentation when symptoms like haematuria, left flank pain, and proteinuria occur, but urologic presentation is also possible. Radiological methods of confirming NCS include Doppler ultrasonography as a primary test, retrograde venography, which can measure the renocaval pressure gradient, computed tomography angiography, which is faster and less traumatic, intravascular ultrasound, and magnetic resonance angiography. Treatment can be conservative or surgical, depending on the severity of symptoms and degree of LRV occlusion. Nutcracker syndrome is worth considering especially in differential diagnosis of haematuria of unknown origin.
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Siddiqui WJ, Bakar A, Aslam M, Arif H, Bianco BA, Trebelev AE, Kelepouris E, Aggarwal S. Left Renal Vein Compression Syndrome: Cracking the Nut of Clinical Dilemmas - Three Cases and Review of Literature. AMERICAN JOURNAL OF CASE REPORTS 2017; 18:754-759. [PMID: 28680033 PMCID: PMC5511006 DOI: 10.12659/ajcr.905324] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Case series Patient: Female, 36 • Female, 41 • Female, 36 Final Diagnosis: Left renal vein compression syndrome Symptoms: Hematuria Medication: — Clinical Procedure: Percutaneous intervention • nephrectomy Specialty: Nephrology
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Affiliation(s)
- Waqas Javed Siddiqui
- Department of Medicine, Division of Nephrology and Hypertension, Drexel University College of Medicine, Philadelphia, PA, USA.,Hahnemann University Hospital, Philadelphia, PA, USA
| | - Abu Bakar
- Department of Medicine, Dow University of Health Sciences, Karachi, Pakistan
| | - Muhammad Aslam
- Department of Medicine, Dow University of Health Sciences, Karachi, Pakistan
| | - Hasan Arif
- Department of Medicine, Division of Nephrology and Hypertension, Drexel University College of Medicine, Philadelphia, PA, USA.,Hahnemann University Hospital, Philadelphia, PA, USA
| | - Brian A Bianco
- Hahnemann University Hospital, Philadelphia, PA, USA.,Department of Interventional Radiology, Drexel University College of Medicine, Philadelphia, PA, USA
| | - Alexander E Trebelev
- Hahnemann University Hospital, Philadelphia, PA, USA.,Department of Interventional Radiology, Drexel University College of Medicine, Philadelphia, PA, USA
| | - Ellie Kelepouris
- Department of Medicine, Division of Nephrology and Hypertension, Drexel University College of Medicine, Philadelphia, PA, USA.,Hahnemann University Hospital, Philadelphia, PA, USA
| | - Sandeep Aggarwal
- Department of Medicine, Division of Nephrology and Hypertension, Drexel University College of Medicine, Philadelphia, PA, USA.,Hahnemann University Hospital, Philadelphia, PA, USA
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Ananthan K, Onida S, Davies A. Nutcracker Syndrome: An Update on Current Diagnostic Criteria and Management Guidelines. Eur J Vasc Endovasc Surg 2017; 53:886-894. [DOI: 10.1016/j.ejvs.2017.02.015] [Citation(s) in RCA: 141] [Impact Index Per Article: 17.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2016] [Accepted: 02/13/2017] [Indexed: 02/06/2023]
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Zucker EJ, Ganguli S, Ghoshhajra BB, Gupta R, Prabhakar AM. Imaging of venous compression syndromes. Cardiovasc Diagn Ther 2016; 6:519-532. [PMID: 28123973 DOI: 10.21037/cdt.2016.11.19] [Citation(s) in RCA: 71] [Impact Index Per Article: 7.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/20/2023]
Abstract
Venous compression syndromes are a unique group of disorders characterized by anatomical extrinsic venous compression, typically in young and otherwise healthy individuals. While uncommon, they may cause serious complications including pain, swelling, deep venous thrombosis (DVT), pulmonary embolism, and post-thrombotic syndrome. The major disease entities are May-Thurner syndrome (MTS), variant iliac vein compression syndrome (IVCS), venous thoracic outlet syndrome (VTOS)/Paget-Schroetter syndrome, nutcracker syndrome (NCS), and popliteal venous compression (PVC). In this article, we review the key clinical features, multimodality imaging findings, and treatment options of these disorders. Emphasis is placed on the growing role of noninvasive imaging options such as magnetic resonance venography (MRV) in facilitating early and accurate diagnosis and tailored intervention.
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Affiliation(s)
- Evan J Zucker
- Division of Cardiovascular Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA;; Department of Radiology, Stanford University School of Medicine, Stanford, CA 94305, USA
| | - Suvranu Ganguli
- Division of Interventional Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
| | - Brian B Ghoshhajra
- Division of Cardiovascular Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
| | - Rajiv Gupta
- Division of Cardiovascular Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA;; Division of Neuroradiology, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
| | - Anand M Prabhakar
- Division of Cardiovascular Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
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Patil AT, Sanjay K, Govindraj M. Nutcracker syndrome in children presenting with recurrent gross hematuria. APOLLO MEDICINE 2016. [DOI: 10.1016/j.apme.2015.02.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
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Di Carlo M, Gaudiano C, Busato F, Pucci S, Schiavina R, Vagnoni V, Golfieri R. Anterior Nutcracker Syndrome with Left Gonadal Vein Varicosities on Multiphasic Computed Tomography: An Unexpected Cause of Pyeloureteral Junction Obstruction. Urol Int 2016; 97:482-484. [DOI: 10.1159/000381270] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2014] [Accepted: 02/24/2015] [Indexed: 11/19/2022]
Abstract
The anterior nutcracker syndrome is defined by the compression of the left renal vein between the aorta and superior mesenteric artery, usually related to the occurrence of hematuria. We report the case of an uncommon complication of the nutcracker syndrome. A 75-year-old woman was referred to our institution for left flank pain without hematuria. Multiphasic computer tomography urography showed a condition of left renal vein entrapment between the aorta and superior mesenteric artery with the development of left gonadal vein varicosities at the level of the renal hilum; a pyeloureteral junction compression with dilation of the pyelocalyceal system coexisted. To our knowledge, this is the first report of the association between nutcracker syndrome and pyeloureteral junction obstruction.
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An Unusual Course of Segmental Renal Artery Displays a Rare Case of Hilar Nutcracker Phenomenon. Case Rep Med 2015; 2015:249015. [PMID: 26448765 PMCID: PMC4584064 DOI: 10.1155/2015/249015] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2015] [Revised: 09/01/2015] [Accepted: 09/01/2015] [Indexed: 01/08/2023] Open
Abstract
Nutcracker phenomenon or renal vein entrapment is classically seen as a compression of renal vein in between abdominal aorta and superior mesenteric artery with patients being asymptomatic or clinically manifested in the form of nutcracker syndrome as proteinuria, hematuria, flank pain, pelvic congestion in women, and varicocele in men. In this report, we are presenting a case of rare variant of nutcracker phenomenon along with brief review of anatomy, pathophysiology, public health, and clinical significance of nutcracker syndrome. On a routine dissection of an adult male cadaver, we noticed an unusual arrangement of the structures at the hilum of the left kidney showing entrapment of renal vein between left anterior inferior and posterior segmental renal arteries. The variation in the course of left anterior inferior segmental renal artery leads to compression of left renal vein at renal hilum. Therefore, we have named this rare abnormal anatomical entity as hilar nutcracker phenomenon. The structures in the right renal hilum are normal. The objective of this paper is to report an unusual but important variant of nutcracker phenomenon and also give collective knowledge of such anatomical variations in renal vasculature that will help in diagnosing and treating such rare renal disorder.
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40
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Gaudiano C, Pucci S, Busato F, Di Carlo M, Schiavina R, Vagnoni V, Pultrone CV, Corcioni B, Golfieri R. Three Unusual Cases of Nutcracker Syndrome Caused by Increased Blood Flow within the Left Renal Vein. Urol Int 2015; 96:484-7. [PMID: 26343488 DOI: 10.1159/000437000] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2015] [Accepted: 06/17/2015] [Indexed: 11/19/2022]
Abstract
Nutcracker syndrome (NCS) refers to the compression of the left renal vein (LRV) between the aorta and the superior mesenteric artery. It can cause both microscopic and gross haematuria, with or without flank pain. The diagnosis is often delayed in a majority of symptomatic patients. On the other hand, the use of CT in routine abdominal explorations has increased the detection of the compression of the LRV in healthy and asymptomatic patients, but its diagnostic value remains uncertain. In this paper, we report 3 cases of the NCS associated with an increased blood flow in the LRV, due to different conditions, which we believe could produce the appearance of clinical symptoms.
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Affiliation(s)
- Caterina Gaudiano
- Department of Radiology, University of Bologna, Sant'Orsola-Malpighi Hospital, Bologna, Italy
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Gulleroglu K, Gulleroglu B, Baskin E. Nutcracker syndrome. World J Nephrol 2014; 3:277-281. [PMID: 25374822 PMCID: PMC4220361 DOI: 10.5527/wjn.v3.i4.277] [Citation(s) in RCA: 85] [Impact Index Per Article: 7.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/06/2014] [Revised: 07/16/2014] [Accepted: 09/17/2014] [Indexed: 02/06/2023] Open
Abstract
The nutcracker phenomenon [left renal vein (LRV) entrapment syndrome] refers to compression of the LRV most commonly between abdominal aorta and superior mesenteric artery. Term of nutcracker syndrome (NCS) is used for patients with clinical symptoms associated with nutcracker anatomy. LRV entrapment divided into 2 types: anterior and posterior. Posterior and right-sided NCSs are rare conditions. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. Symptoms include hematuria, orthostatic proteinuria, flank pain, abdominal pain, varicocele, dyspareunia, dysmenorrhea, fatigue and orthostatic intolerance. Existence of the clinical features constitutes a basis for the diagnosis. Several imaging methods such as Doppler ultrasonography, computed tomography angiography, magnetic resonance angiography and retrograde venography are used to diagnose NCS. The management of NCS depends upon the clinical presentation and the severity of the LRV hypertension. The treatment options are ranged from surveillance to nephrectomy. Treatment decision should be based on the severity of symptoms and their expected reversibility with regard to patient's age and the stage of the syndrome.
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Muddana N, Pai R, Nahum E, Sureshkumar KK. Quiz page october 2014: unexplained hematuria in a thin, middle-aged man. Am J Kidney Dis 2014; 64:A21-A24. [PMID: 25257332 DOI: 10.1053/j.ajkd.2014.04.035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2014] [Accepted: 04/07/2014] [Indexed: 11/11/2022]
Affiliation(s)
- Neeharika Muddana
- Division of Nephrology and Hypertension, Department of Medicine, Allegheny General Hospital, Pittsburgh, PA
| | - Rima Pai
- Division of Nephrology and Hypertension, Department of Medicine, Allegheny General Hospital, Pittsburgh, PA
| | - Elmer Nahum
- Department of Radiology, Allegheny General Hospital, Pittsburgh, PA
| | - Kalathil K Sureshkumar
- Division of Nephrology and Hypertension, Department of Medicine, Allegheny General Hospital, Pittsburgh, PA.
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Velásquez-Jones L, Medeiros M, Patiño-Ortega M, Guerrero-Kanan R, Valadez-Reyes MT, Valverde-Rosas S, Del Moral-Espinosa I, Franco-Alvarez I, Romo-Vázquez JC. [Nutcracker syndrome: Cause of non-glomerular hematuria and massive proteinuria]. BOLETIN MEDICO DEL HOSPITAL INFANTIL DE MEXICO 2014; 71:298-302. [PMID: 29421619 DOI: 10.1016/j.bmhimx.2014.10.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2014] [Accepted: 10/02/2014] [Indexed: 10/23/2022] Open
Abstract
BACKGROUND Nutcracker syndrome caused by compression of the left renal vein between the aorta and superior mesenteric artery is a non-glomerular cause of left renal bleeding and left varicocele. It has also been recognized to be an important cause of orthostatic proteinuria. CASE REPORT A 17-year-old male was evaluated due to recurrent macroscopic hematuria. Physical examination showed left varicocele. Body mass index 16.3 kg/m2. Urinalysis demonstrated hematuria and massive proteinuria. Renal biopsy showed mild mesangial glomerular proliferation. Cystoscopy showed hematuria originating from the left ureter. Doppler ultrasonography and contrast-enhanced computed angiotomography revealed a peak velocity of the left renal vein of 20cm/s, ratio of peak velocity of aortomesenteric and hilar portions of left renal vein of 7.7 and enlargement of the left renal vein in the hilar portion. With a diagnosis of nutcracker syndrome, the patient received conservative treatment. During follow-up, progressive remission of the recurrent episodes of hematuria and proteinuria was observed. The patient had no clinical symptoms or abnormal urinalysis. At 13 months of follow-up the body mass index was 19 kg/m2. CONCLUSIONS This case shows the relationship between the increase in body mass index and remission of nutcracker syndrome, manifested as left varicocele, hematuria and massive proteinuria. All symptoms disappeared with the increase of body mass index, probably due to increase in retroperitoneal fat with improvement of the aortomesenteric angle of the left renal vein.
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Affiliation(s)
- Luis Velásquez-Jones
- Departamento de Nefrología Dr. Gustavo Gordillo Paniagua, Hospital Infantil de México Federico Gómez, México D.F., México.
| | - Mara Medeiros
- Laboratorio de Investigación en Nefrología y Metabolismo Mineral Óseo, Hospital Infantil de México Federico Gómez, México D.F., México
| | - Mauricio Patiño-Ortega
- Departamento de Nefrología Dr. Gustavo Gordillo Paniagua, Hospital Infantil de México Federico Gómez, México D.F., México
| | - Ricardo Guerrero-Kanan
- Departamento de Nefrología Dr. Gustavo Gordillo Paniagua, Hospital Infantil de México Federico Gómez, México D.F., México
| | | | - Saúl Valverde-Rosas
- Departamento de Nefrología Dr. Gustavo Gordillo Paniagua, Hospital Infantil de México Federico Gómez, México D.F., México
| | - Irma Del Moral-Espinosa
- Departamento de Nefrología Dr. Gustavo Gordillo Paniagua, Hospital Infantil de México Federico Gómez, México D.F., México
| | - Isidro Franco-Alvarez
- Departamento de Nefrología Dr. Gustavo Gordillo Paniagua, Hospital Infantil de México Federico Gómez, México D.F., México
| | - José Carlos Romo-Vázquez
- Departamento de Nefrología Dr. Gustavo Gordillo Paniagua, Hospital Infantil de México Federico Gómez, México D.F., México
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Quevedo HC, Arain SA, Abi Rafeh N. Systematic review of endovascular therapy for nutcracker syndrome and case presentation. CARDIOVASCULAR REVASCULARIZATION MEDICINE 2014; 15:305-7. [DOI: 10.1016/j.carrev.2014.04.008] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2014] [Revised: 04/11/2014] [Accepted: 04/14/2014] [Indexed: 01/29/2023]
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Avgerinos ED, McEnaney R, Chaer RA. Surgical and endovascular interventions for nutcracker syndrome. Semin Vasc Surg 2014; 26:170-7. [PMID: 25220323 DOI: 10.1053/j.semvascsurg.2014.06.014] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
Nutcracker syndrome is a rare condition of left renal vein entrapment manifesting with hematuria, flank pain, and, occasionally, pelvic congestion in females or varicocele in males. Diagnosis requires a high index of suspicion upon careful history delineation. The gold standard for definite confirmation remains venography with renocaval pressure gradient. Treatment is mainly guided by the severity of symptoms. For the majority of centers, it appears that surgery remains the first-line therapy, however, endovascular alternatives are rapidly evolving into the field with favorable outcomes. This article reviews current concepts on nutcracker syndrome with particular focus on contemporary surgical and endovascular techniques and their outcomes.
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Affiliation(s)
- Efthymios D Avgerinos
- Division of Vascular Surgery, University of Pittsburgh, School of Medicine, 200 Lothrop Street, Suite A1011, Pittsburgh, PA 15213
| | - Ryan McEnaney
- Division of Vascular Surgery, University of Pittsburgh, School of Medicine, 200 Lothrop Street, Suite A1011, Pittsburgh, PA 15213
| | - Rabih A Chaer
- Division of Vascular Surgery, University of Pittsburgh, School of Medicine, 200 Lothrop Street, Suite A1011, Pittsburgh, PA 15213.
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Abstract
Patients with pelvic congestion syndrome present with otherwise unexplained chronic pelvic pain that has been present for greater than 6 months, and anatomic findings that include pelvic venous insufficiency and pelvic varicosities. It remains an underdiagnosed explanation for pelvic pain in young, premenopausal, usually multiparous females. Symptoms include noncyclical, positional lower back, pelvic and upper thigh pain, dyspareunia, and prolonged postcoital discomfort. Symptoms worsen throughout the day and are exacerbated by activity or prolonged standing. Examination may reveal ovarian tenderness and unusual varicosities-vulvoperineal, posterior thigh, and gluteal. Diagnosis is suspected by clinical history and imaging that demonstrates pelvic varicosities. Venography is usually necessary to confirm ovarian vein reflux, although transvaginal ultrasound may be useful in documenting this finding. Endovascular therapy has been validated by several large patient series with long-term follow-up using standardized pain assessment surveys. Embolization has been shown to be significantly more effective than surgical therapy in improving symptoms in patients who fail hormonal therapy. Although there has been variation in approaches between investigators, the goal is elimination of ovarian vein reflux with or without direct sclerosis of enlarged pelvic varicosities. Symptom reduction is seen in 70 to 90% of the treated females despite technical variation.
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Affiliation(s)
- Janette D Durham
- Department of Radiology, University of Colorado Denver, Aurora, Colorado
| | - Lindsay Machan
- Department of Radiology, University of British Columbia Hospital, Vancouver, BC, Canada
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Gracia-Calvo LA, Durán ME, Martín-Cuervo M, Vieítez V, Jiménez J, Ezquerra LJ. Persistent Hematuria as a Result of Chronic Renal Hypertension Secondary to Nephritis in a Stallion. J Equine Vet Sci 2014. [DOI: 10.1016/j.jevs.2013.12.014] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Jin M, Hu P, Ding R, Chen P, Qiu Q, Wu J, Liu S, Xie Y, Chen X. Effectiveness of supine/standing urinalysis for differential diagnosis of left renal vein entrapment syndrome combined with or without glomerulopathy. Nephrology (Carlton) 2014; 19:332-8. [PMID: 24629105 DOI: 10.1111/nep.12227] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/07/2014] [Indexed: 01/29/2023]
Abstract
AIM To assess the effectiveness of supine/standing urinalysis for differential diagnosis of left renal vein entrapment syndrome (LRVES) combined with or without glomerulopathy. METHODS The enrolled patients with abnormal urinalysis and LRVES demonstrated by Doppler sonography were guided to perform a supine/standing urinalysis. RESULTS Fifty-two patients were enrolled. Most of them were adolescents (aged 14-29 years, 73.1%) and with low body mass index (BMI, mean BMI, 19.8 ± 2.4 kg/m(2)). Seventeen cases (32.7%) manifested orthostatic urine abnormalities (OUA, proteinuria and/or haematuria show negative in supine while positive after 15 min standing), two patients who had undergone renal biopsies both showed no evidence of kidney lesions, another two patients were changed from abnormal to normal urinalysis after weight gain. The remaining 35 cases (67.3%) manifested non-orthostatic urine abnormalities (NOUA, proteinuria and/or haematuria show positive both in supine and standing), 15 patients had undergone renal biopsies and showed different degrees of glomerulopathy. After prednisone/immunosuppression therapy, four patients with glomerulonephritis were changed from the NOUA to the OUA classification. Statistics analyses showed that serum total protein and albumin levels were significantly lower (P = 0.028, 0.007, respectively) and urinary protein was significantly higher (P = 0.007) in the NOUA group than in the OUA group. CONCLUSION After the indication of LRVES by ultrasound, patients with OUA likely have only LRVES, while patients with NOUA likely also have glomerulopathy. Supine/standing urinalysis combined with Doppler sonography can be helpful for differential diagnosis of LRVES combined with or without glomerulopathy.
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Affiliation(s)
- Meiling Jin
- State Key Laboratory of Kidney Disease, Institute of Nephrology, Chinese PLA General Hospital, Beijing, China; Medical College, Nankai University, Tianjin, China
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Park SJ, Kim SM, Won JH, Lim HS. A Case of Secondary Hypertension Associated with the Nutcracker Phenomenon. Korean Circ J 2014; 44:434-6. [PMID: 25469147 PMCID: PMC4248617 DOI: 10.4070/kcj.2014.44.6.434] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/17/2013] [Revised: 01/27/2014] [Accepted: 04/29/2014] [Indexed: 11/11/2022] Open
Abstract
A 25-year-old Korean woman was referred for uncontrolled hypertension. Laboratory examination revealed increased plasma renin activity and microscopic hematuria. Computed tomography demonstrated compression of the left renal vein (LRV) between the aorta and superior mesenteric artery; however, both renal arteries were intact and there was no adrenal mass. Renal vein catheterization showed external compression with a pressure gradient of up to 8 mm Hg between the LRV and the inferior vena cava. Plasma renin activity in the LRV was almost five times higher than that in the right renal vein. In this patient, renin-dependent hypertension was caused by renal congestion due to LRV obstruction.
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Affiliation(s)
- Se-Jun Park
- Department of Cardiology, Ajou University School of Medicine, Suwon, Korea
| | - Sun-Mi Kim
- Department of Cardiology, Ajou University School of Medicine, Suwon, Korea
| | - Je-Hwan Won
- Department of Radiology, Ajou University School of Medicine, Suwon, Korea
| | - Hong-Seok Lim
- Department of Cardiology, Ajou University School of Medicine, Suwon, Korea
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