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Li C, Wang YW, Sheng H, Jin D, Shu ZX, Li M, Li SG. Case Report: Nutcracker Syndrome Triggered by Rapid Weight Loss in a Patient With Systemic Lupus Erythematosus. Int J Rheum Dis 2025; 28:e70190. [PMID: 40130708 DOI: 10.1111/1756-185x.70190] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2024] [Revised: 02/23/2025] [Accepted: 03/17/2025] [Indexed: 03/26/2025]
Abstract
Nutcracker syndrome (NCS) is a rare vascular disorder characterized by compression of the left renal vein, typically causing hematuria, proteinuria, and flank pain. Although NCS is often linked with weight loss and anatomical variations, no previous reports have connected it to systemic lupus erythematosus (SLE). We describe a 44-year-old male with SLE who developed NCS after rapid weight loss, presenting with abdominal pain, hematuria, and proteinuria. Imaging confirmed left renal vein compression between the aorta and the superior mesenteric artery. This finding suggests that significant weight reduction in SLE may trigger NCS by altering retroperitoneal fat and vascular structures. A literature review reveals a consistent association between NCS and marked weight loss, as well as possible coexistence with other vascular compression syndromes, such as superior mesenteric artery syndrome. Clinicians should consider NCS in SLE patients with sudden weight changes to ensure timely diagnosis and prevent complications.
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Affiliation(s)
- Chen Li
- Department of Dermatology, Tianjin Institute of Integrative Dermatology, Tianjin Academy of Traditional Chinese Medicine Affiliated Hospital, Tianjin, China
- Department of Rheumatology, Fangshan Hospital, Beijing University of Chinese Medicine, Beijing, China
| | - Yu-Wei Wang
- Department of Cardiology, Yidu Central Hospital of Weifang, Weifang, China
| | - Han Sheng
- School of Clinical Medicine, Beijing University of Chinese Medicine, Beijing, China
| | - Di Jin
- Department of Rheumatology, Weifang People's Hospital, Weifang, China
| | - Zi-Xuan Shu
- School of Traditional Chinese Medicine, Beijing University of Chinese Medicine, Beijing, China
| | - Ming Li
- Department of Rheumatology, Weifang People's Hospital, Weifang, China
| | - Sheng-Guang Li
- Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China
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2
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Figueroa AV, Cifuentes S, Ulloa JH, Raffetto J. Iliac venous stenting as adjunct in the management of symptomatic orthostatic hypotension in iliac vein compression. J Vasc Surg Cases Innov Tech 2024; 10:101483. [PMID: 40027266 PMCID: PMC11868757 DOI: 10.1016/j.jvscit.2024.101483] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2023] [Accepted: 02/29/2024] [Indexed: 03/05/2025] Open
Abstract
Orthostatic hypotension (OH) is a common cardiovascular disorder with high prevalence in the elderly. Concomitant venous return impairment may worsen the autonomic response and accentuate the symptoms. We detailed a patient with severe OH, prominent varicosities, and hemosiderin deposition in lower limbs. After excluding autonomic and neurological etiology, a computed tomography venography revealed significant common iliac vein compression. The patient was deemed to benefit from venous stenting. At a 2-year follow-up, the patient reported symptom improvement with adequate stent patency. Venous stenting may aid in the treatment of patients with severe OH symptoms without clear etiology and impairment of venous return.
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Affiliation(s)
- Andres V. Figueroa
- Division of Vascular and Endovascular Surgery, Fundacion Santa Fe de Bogota, University Hospital, Bogota, Colombia
| | - Sebastian Cifuentes
- Division of Vascular and Endovascular Surgery, Fundacion Santa Fe de Bogota, University Hospital, Bogota, Colombia
| | - Jorge H. Ulloa
- Division of Vascular and Endovascular Surgery, Fundacion Santa Fe de Bogota, University Hospital, Bogota, Colombia
- Universidad de Los Andes Medical School, Bogota, Colombia
| | - Joseph Raffetto
- Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA
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Dewald CLA, Becker LS, Meyer BC. Interventional Therapy of Pelvic Venous Disorders (PeVD). ROFO-FORTSCHR RONTG 2024; 196:921-927. [PMID: 38373714 DOI: 10.1055/a-2229-4100] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/21/2024]
Abstract
BACKGROUND Pelvic venous disorders (PeVD) are an underdiagnosed cause of chronic pelvic pain in women. They are caused by venous insufficiency of the pelvic or ovarian veins, leading to the development of mainly periuterine and periovarian varices. It is a progressive disease if left untreated and can cause swelling, dyspareunia, dysmenorrhea, and other symptoms, some non-specific, that affect the patient's quality of life. Interventional therapies are a central component of the treatment of PeVD, with a variety of techniques available for both diagnosis and treatment. METHOD This review provides an overview of the pathophysiologic background, diagnosis, and, most importantly, interventional treatment options for PeVD. RESULTS There is a lack of standardized nomenclature and internationally accepted diagnostic criteria for PeVD as well as randomized controlled trials demonstrating clinical success. However, in clinical trials, endovascular therapy for PeVD has been shown to be safe and effective. This review presents the various interventional techniques for the treatment of PeVD, including embolization, stenting, and sclerotherapy. CONCLUSION The importance of PeVD is receiving growing recognition. Recent advances, such as the development of the Symptoms-Varices-Pathophysiology (SVP) classification, provide an impetus to standardize nomenclature and are the first step toward systematizing disease management. Interventional therapies provide a safe and tailored minimally invasive treatment option for patients with PeVD. KEY POINTS · Pelvic venous disorders are an underdiagnosed condition with frequently delayed diagnosis and debilitating symptoms.. · Until now, the PeVD nomenclature has been imprecise, with terms like May-Thurner syndrome/Nutcracker syndrome.. · Interventional approaches are effective and play a central role in PeVD treatment.. CITATION FORMAT · Dewald CL, Becker LS, Meyer BC. Interventional Therapy of Pelvic Venous Disorders (PeVD). Fortschr Röntgenstr 2024; 196: 921 - 927.
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Affiliation(s)
| | - Lena Sophie Becker
- Diagnostic and Interventional Radiology, Hannover Medical School, Hannover, Germany
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Wang J, Li H, Huang X, Hu H, Lian B, Zhang D, Wu J, Cao L. Adult vasovagal syncope with abdominal pain diagnosed by head-up tilt combined with transcranial doppler: a preliminary study. BMC Neurol 2024; 24:118. [PMID: 38600450 PMCID: PMC11005138 DOI: 10.1186/s12883-024-03623-1] [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: 07/17/2023] [Accepted: 04/04/2024] [Indexed: 04/12/2024] Open
Abstract
BACKGROUND Syncope is a common condition that increases the risk of injury and reduces the quality of life. Abdominal pain as a precursor to vasovagal syncope (VVS) in adults is rarely reported and is often misdiagnosed.. METHODS We present three adult patients with VVS and presyncopal abdominal pain diagnosed by synchronous multimodal detection (transcranial Doppler [TCD] with head-up tilt [HUT]) and discuss the relevant literature. RESULTS Case 1: A 52-year-old man presented with recurrent decreased consciousness preceded by six months of abdominal pain. Physical examinations were unremarkable. Dynamic electrocardiography, echocardiography, head and neck computed tomography angiography, magnetic resonance imaging (MRI), and video electroencephalogram showed no abnormalities. Case 2: A 57-year-old woman presented with recurrent syncope for 30 + years, accompanied by abdominal pain. Physical examination, electroencephalography, and MRI showed no abnormalities. Echocardiography showed large right-to-left shunts. Case 3: A 30-year-old woman presented with recurrent syncope for 10 + years, with abdominal pain as a precursor. Physical examination, laboratory analysis, head computed tomography, electrocardiography, and echocardiography showed no abnormalities. Syncope secondary to abdominal pain was reproduced during HUT. Further, HUT revealed vasovagal syncope, and synchronous TCD showed decreased cerebral blood flow; the final diagnosis was VVS in all cases. CONCLUSIONS Abdominal pain may be a precursor of VVS in adults, and our findings enrich the clinical phenotypic spectrum of VVS. Prompt recognition of syncopal precursors is important to prevent incidents and assist in treatment decision-making. Abdominal pain in VVS may be a sign of sympathetic overdrive. Synchronous multimodal detection can help in diagnosing VVS and understanding hemodynamic mechanisms.
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Affiliation(s)
- Jingyi Wang
- Faculty of Chinese Medicine, Macau University of Science and Technology, Macau, China
- Department of Neurology, Zhuhai Hospital of Integrated Traditional Chinese and Western Medicine, Zhuhai, China
| | - Hua Li
- Department of Neurology, Zhuhai Hospital of Integrated Traditional Chinese and Western Medicine, Zhuhai, China
- Affiliated Hospital of the Faculty of Chinese Medicine, Macao University of Science and Technology, Macau, China
| | - Xuming Huang
- Department of Gastroenterology, Shenzhen baoan Shiyan People's Hospital, Shenzhen, China
| | - Huoyou Hu
- Department of Neurology, The First Affiliated Hospital of Shenzhen University, Shenzhen, China
| | - Baorong Lian
- Shantou University Medical College, Shantou University, Shantou, China
| | - Daxue Zhang
- Clinical Medical College of Shenzhen Second People's Hospital, Anhui Medical University, Hefei, China
| | - Jiarui Wu
- The First School of Clinical Medicine, Guangdong Medical University, Zhanjiang, China
| | - Liming Cao
- Department of Neurology, The First Affiliated Hospital of Shenzhen University, Shenzhen, China.
- Clinical Medical College of Shenzhen Second People's Hospital, Guangxi University of Chinese Medicine, Nanning, China.
- Hunan Provincial Key Laboratory of the Research and Development of Novel Pharmaceutical Preparations, Changsha Medical University, Changsha, China.
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Donnelly L, Turner B, Davies AH. Atypical case of coexistent vascular compression syndromes: median arcuate ligament syndrome and nutcracker syndrome. BMJ Case Rep 2023; 16:e257754. [PMID: 37989330 PMCID: PMC10668132 DOI: 10.1136/bcr-2023-257754] [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] [Indexed: 11/23/2023] Open
Abstract
Vascular compression syndromes, including median arcuate ligament syndrome (MALS) and nutcracker syndrome (NCS), are poorly understood and frequently delayed diagnoses. This case describes a young adult female presenting with chronic vomiting, abdominal pain and weight loss, with dependence on nasogastric feeding. Subsequent to her gastrointestinal symptoms, she developed haematuria and orthostatic intolerance. Investigations confirmed NCS and possible MALS, with superadded gastroparesis and bradygastria. Under the joint care of general and vascular surgeons, she underwent a gastrojejunostomy and panelled renocaval bypass which led to partial resolution of her symptoms. It is hypothesised that gastroparesis may have been caused by MALS via a neurogenic mechanism, or coexistent compression of the duodenum by the superior mesenteric artery. This case highlights the difficulty in diagnosis of vascular compression syndromes, the overlap between the conditions and the potential for multiple coexistent conditions which complicate diagnosis and lead to increased lead-time and morbidity for patients.
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Affiliation(s)
- Liam Donnelly
- London North West University Healthcare NHS Trust, London, UK
| | | | - Alun H Davies
- Academic Section of Vascular Surgery, Imperial College London, London, UK
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Manzano JP, de Pinho JHS, de Oliveira Azambuja T, Ambrogini C, Bruna PC. Robot-assisted extravascular stent for nutcracker syndrome: A case report. Urol Case Rep 2022; 46:102311. [PMID: 36606097 PMCID: PMC9807993 DOI: 10.1016/j.eucr.2022.102311] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2022] [Revised: 12/20/2022] [Accepted: 12/24/2022] [Indexed: 12/26/2022] Open
Abstract
Nutcracker syndrome (NCS) is a rare condition caused by compression of the left renal vein (LRV) between the aorta and superior mesenteric artery. Surgical treatment is reserved for patients with severe symptoms and failure of conservative treatment. A 31-year-old woman diagnosed in adolescence with NCS has had recurrent pain since age 15. For 30 days, she has had severe left flank pain and microhematuria. The patient underwent extravascular stent placement around the LRV with the Da Vinci Xi. In 30 months of follow-up, the patient has no pain and the LRV remained without compressions or thrombosis.
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Affiliation(s)
- João Pádua Manzano
- Federal University of São Paulo, São Paulo, Brazil,Brazilian Institute of Robotic Surgery, Moriah Hospital, São Paulo, Brazil
| | | | | | - Claudio Ambrogini
- Brazilian Institute of Robotic Surgery, Moriah Hospital, São Paulo, Brazil
| | - Paulo Collet Bruna
- Brazilian Institute of Robotic Surgery, Moriah Hospital, São Paulo, Brazil
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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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Yoshino K, Tomari K, Kato A, Yagi T. Nutcracker Syndrome Discovered after Syncope with Abdominal Pain as a Prodromal Symptom. Indian J Pediatr 2022; 89:518. [PMID: 35143038 DOI: 10.1007/s12098-021-04061-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2021] [Accepted: 11/18/2021] [Indexed: 11/25/2022]
Affiliation(s)
- Keisuke Yoshino
- Department of General Pediatrics, Okinawa Prefectural Nanbu Medical Center and Children's Medical Center, Arakawa, Okinawa, Japan
| | - Kouki Tomari
- Department of General Pediatrics, Okinawa Prefectural Nanbu Medical Center and Children's Medical Center, Arakawa, Okinawa, Japan. .,, 118-1 Arakawa, Haebaru-cho, Okinawa, 901-1193, Japan.
| | - Akio Kato
- Department of Pediatric Cardiology, Okinawa Prefectural Nanbu Medical Center and Children's Medical Center, Arakawa, Okinawa, Japan
| | - Takeshi Yagi
- Department of Pediatric Hematology and Oncology, Okinawa Prefectural Nanbu Medical Center and Children's Medical Center, Arakawa, Okinawa, Japan
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He D, Liang J, Wang H, Jiao Y, Wu B, Cui D, Cao T, Li Y, Wang J, Zhang B. 3D-Printed PEEK Extravascular Stent in the Treatment of Nutcracker Syndrome: Imaging Evaluation and Short-Term Clinical Outcome. Front Bioeng Biotechnol 2020; 8:732. [PMID: 32719785 PMCID: PMC7347745 DOI: 10.3389/fbioe.2020.00732] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2020] [Accepted: 06/10/2020] [Indexed: 11/13/2022] Open
Abstract
Minimally invasive options are safe and reliable alternatives for the treatment of nutcracker syndrome (NCS). After continued efforts, our team successfully devised a new and effective therapeutic method: 3D-printed extravascular stenting of the left renal vein. From December 2017 to May 2019, 28 patients (25 men and 3 women) from different parts of China between 18 and 37 years old (mean, 23.6 years) diagnosed with NCS were admitted for laparoscopic 3D-printed extravascular stenting treatment. The post-operative follow-up duration was 6-24 months (median, 16.3 months). Technical success of the operation was achieved in all patients. After treatment, the NCS symptoms all patients resolved or improved during the follow-up period, without relapse. Most symptoms, including macro-/microhematuria, proteinuria, and flank/abdominal pain, tended to resolve within 3-6 months after the surgery; other symptoms, such as left-sided varicocele, also showed varying degrees of improvement at different times post-operatively. Perioperative complications were noted in two patients, including transient and mild lymphatic leakage, without any adverse effects. All extravascular stents were visualized on computed tomography and Doppler ultrasound scans, and no migration or any side effects occurred during the entire follow-up period. Compared to endovascular stenting or polytetrafluoroethylene artificial vessel procedures, 3D-printed polyetheretherketone extravascular stenting has more advantages in terms of stent design and rigidity and approach rationality while successfully preventing stent migration and thrombosis. Therefore, this method may serve as an accurate and effective treatment for NCS patients.
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Affiliation(s)
- Dali He
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Jiahe Liang
- Department of Ultrasound Diagnostic, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Hengen Wang
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Yong Jiao
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Bin Wu
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Dong Cui
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Tiesheng Cao
- Department of Ultrasound Diagnostic, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Yanyan Li
- Department of Radiology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
| | - Jing Wang
- School of Chemical Engineering and Technology, Xi'an Jiaotong University, Xi'an, China
| | - Bo Zhang
- Department of Urology, Tangdu Hospital, The Fourth Military Medical University, Xi'an, China
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Banon S, Skaribas I. Serial ganglion impar blocks in a patient with nutcracker syndrome refractory to left renal vein transposition: a case report. J Med Case Rep 2020; 14:102. [PMID: 32620128 PMCID: PMC7333409 DOI: 10.1186/s13256-020-02398-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2019] [Accepted: 05/08/2020] [Indexed: 12/17/2022] Open
Abstract
Background Nutcracker syndrome is a rare disorder caused by compression of the left renal vein, usually between the aorta and the superior mesenteric artery. It typically presents with left-sided abdominal pain and hematuria. Left renal vein transposition is the most commonly employed surgical technique to alleviate the compression. Case presentation A 22-year-old Caucasian man with a known diagnosis of nutcracker syndrome had undergone left renal vein transposition 1 year before presentation without any subsequent pain relief. In addition, his surgery was complicated by massive blood loss and a 1-week-long stay in an intensive care unit (ICU); as such, he was not amenable to further surgical intervention or stenting to treat his underlying pathology. His symptoms included constant sharp left-sided flank, perineal, and testicular pain. A series of ganglion impar blocks were performed every 3–4 months over the course of 5 years with substantial pain relief achieved. Conclusions Our case report highlights a treatment option that has not yet been described for patients with pain secondary to nutcracker syndrome refractory to surgical intervention.
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Affiliation(s)
- Shawn Banon
- Baylor College of Medicine, Houston, TX, USA.
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11
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Bin Dahman HA, Aljabry AO. A case report of a young girl with recurrent hematuria: a missed diagnosis - renal nutcracker syndrome. BMC Nephrol 2019; 20:349. [PMID: 31488074 PMCID: PMC6727525 DOI: 10.1186/s12882-019-1508-6] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2019] [Accepted: 07/31/2019] [Indexed: 01/19/2023] Open
Abstract
BACKGROUND Nutcracker syndrome is an easily missed cause of hematuria in children. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Intermittent hematuria and orthostatic proteinuria with or without abdominal or flank pain are the common clinical manifestations. Presence of variable non-specific symptoms and non-significant physical findings results in a delayed diagnosis. CASE PRESENTATION We present a ten -year -old girl with four episodes of painless gross hematuria and recurrent microscopic hematuria since the age of two years. Doppler ultrasound showed left renal vein compression while 3 D computerized tomography angiography confirmed the diagnosis of an anterior nutcracker. The patient was conservatively treated with nutritional support (pediasure complete formula and high calorie food), iron supplements and followed up, monitored for anemia, hypertension and renal insufficiency. CONCLUSION Nutcracker syndrome is a rare cause of recurrent gross hematuria in children. A high index of suspicion and proper imaging is needed to reach a proper diagnosis and avoid the psychological and financial stress on the family.
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Affiliation(s)
- Haifa Ali Bin Dahman
- Pediatric Department, Hadhramout University College of Medicine, Mukalla, Hadhramout governorate, Yemen.
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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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Park JH, Lee GH, Lee SM, Eisenhut M, Kronbichler A, Lee KH, Shin JI. Posterior nutcracker syndrome - a systematic review. VASA 2017; 47:23-29. [PMID: 29165061 DOI: 10.1024/0301-1526/a000670] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Posterior nutcracker syndrome (PNCS) is the entrapment of the left renal vein between the aorta and the vertebral column. Although uncommon, it is still an important diagnosis due to the high morbidity associated with the risk of secondary anaemia from haematuria, from long-term left renal vein hypertension, vascular thrombosis, and even blood clots in the urinary system. A literature search of PubMed and EMBASE databases was performed and 27 publications containing 27 cases were included for the final analysis. The following frequency of clinical signs and symptoms was noted: twenty-five patients had haematuria, 13 patients had flank pain, and two had hypertension. Overall, male-female distribution was balanced and there were more adult than paediatric (age < 18 years) patients. All symptoms of patients with conservative treatment were either well-controlled or under spontaneous resolution. Conservative management instead of surgical treatment should be preferred in most cases. Taken together, despite the low incidence of PNCS, its recognition and management are highly important. This systematic study explores the evidence base for conservative and medical options.
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Affiliation(s)
- Jae Hyon Park
- 1 Yonsei University College of Medicine, Severance Hospital, Seoul, Republic of Korea.,a These authors contributed equally to this paper
| | - Gi Hoon Lee
- 2 Yonsei University Wonju College of Medicine, Wonju, Republic of Korea.,a These authors contributed equally to this paper
| | - Seul Mi Lee
- 3 Department of Pediatrics, Yonsei University College of Medicine, Seoul, Republic of Korea.,4 Division of Pediatric Nephrology, Severance Children's Hospital, Seoul, Republic of Korea
| | - Michael Eisenhut
- 5 Luton & Dunstable University Hospital NHS Foundation Trust, Lewsey Road, Luton, United Kingdom
| | - Andreas Kronbichler
- 6 Department of Internal Medicine IV, Medical University Innsbruck, Innsbruck, Austria
| | - Keum Hwa Lee
- 3 Department of Pediatrics, Yonsei University College of Medicine, Seoul, Republic of Korea.,4 Division of Pediatric Nephrology, Severance Children's Hospital, Seoul, Republic of Korea
| | - Jae Il Shin
- 3 Department of Pediatrics, Yonsei University College of Medicine, Seoul, Republic of Korea.,4 Division of Pediatric Nephrology, Severance Children's Hospital, Seoul, Republic of Korea.,7 Institute of Kidney Disease Research, Yonsei University College of Medicine, Seoul, Republic of Korea
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Nutcracker syndrome: A rare cause of left flank pain that can also manifest as unexplained pelvic pain. Joint Bone Spine 2016; 84:557-562. [PMID: 27932281 DOI: 10.1016/j.jbspin.2016.10.006] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/21/2016] [Indexed: 02/07/2023]
Abstract
Nutcracker syndrome (NCS) is symptomatic unilateral renal venous hypertension due to compression of the left renal vein between the superior mesenteric artery and aorta (anterior NCS) or between the aorta and spine (posterior NCS). The left ovarian or spermatic vein empties into the left renal vein and is an additional site of venostasis in about half the cases of NCS. The presenting symptom of NCS in about half the cases is atypical left flank pain suggesting a disorder of the lower ribs or thoracolumbar spinal junction, particularly as the pain worsens with standing and increased lumbar lordosis. NCS may be suggested by any combination of the following manifestations: hematuria, which is often only microscopic; orthostatic proteinuria; varicocele and infertility; dyspareunia and other gynecological symptoms; varicose veins in the pelvis, buttocks, or upper thighs; orthostatic hypotension and fatigue; and abdominal pain. Narrowing of the left renal vein on imaging studies is required but far from sufficient to establish the diagnosis. Several converging clinical findings and a marked pressure gradient between the left renal vein and inferior vena cava must be present also. Urological procedures and vascular surgery are being superseded by endovascular stenting with or without simultaneous treatment of the acquired gonadal vein insufficiency by embolization.
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Barman N, Palese M. Robotic-Assisted Laparoscopic Donor Nephrectomy of Patient With Nutcracker Phenomenon. EXP CLIN TRANSPLANT 2016; 16:212-215. [PMID: 27210521 DOI: 10.6002/ect.2015.0335] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
We report the case of a 30-year-old male patient undergoing a robotic-assisted laparoscopic left donor nephrectomy, where compression of the left renal vein between the superior mesenteric artery and aorta was noted on magnetic resonance angiography before the operation. The patient was diagnosed with nutcracker phenomenon and was noted to be asymptomatic at that time. This is the first reported case to date of a patient with nutcracker phenomenon who underwent a robotic-assisted laparoscopic donor nephrectomy. This article also reviews the current literature on nutcracker phenomenon and nutcracker syndrome.
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Affiliation(s)
- Naman Barman
- From the Icahn School of Medicine at Mount Sinai, NY, USA
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Micro- and macroscopic hematuria caused by renal vein entrapment: systematic review of the literature. Pediatr Nephrol 2016; 31:175-84. [PMID: 25627663 DOI: 10.1007/s00467-015-3045-2] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2014] [Revised: 01/05/2015] [Accepted: 01/05/2015] [Indexed: 01/17/2023]
Abstract
BACKGROUND Hematuria secondary to renal vein entrapment is mentioned only passing in textbooks and reviews. METHODS We performed a search of the National Library of Medicine database for peer-reviewed publications using the terms "renal vein" or "nutcracker" and "hematuria". RESULTS We identified 187 published reports/studies that covered 736 patients, of whom 288 had microscopic hematuria and 448 had macroscopic hematuria. The patient cohort comprised 159 patients aged ≤17 years. Abdominal pain was absent in approximately 65% of all patients, and a clinically relevant left-sided varicocele was observed in 29% of the male patients. A normal pre-aortic left renal vein and an anomalous anatomy were noted in 680 and 56 patients, respectively. The body mass index (BMI) was lower in patients with renal vein entrapment than in the controls, with a regression of hematuria correlating with an increase in BMI. A surgical procedure was attempted in 34% of the patients, of which the most common were endovascular stenting and transposition of the renal vein distally into the vena cava. CONCLUSIONS In cases of unexplained hematuria with or without abdominal pain, clinicians should consider the diagnosis of renal vein congestion, especially in males with varicocele. Ultrasonic Doppler flow scanning is the recommended initial diagnostic modality in these patients. Expectation management is advised in the great majority of cases.
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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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Yih NDC, Chyen LH, Cunli Y, Jaywantraj PS, Isip ABC, Anil SA. Renosplenic shunting in the nutcracker phenomenon: a discussion and paradigm shift in options? A novel approach to treating nutcracker syndrome. Int J Angiol 2014; 23:71-6. [PMID: 24627622 DOI: 10.1055/s-0033-1348883] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
The nutcracker syndrome is a rare clinical manifestation of symptoms caused by the compression of the left renal vein by an overriding superior mesenteric artery, an anatomical variant otherwise known as the nutcracker phenomenon. Usually present in women and children, when symptomatic, it commonly presents with hematuria, proteinuria, and chronic pelvic pain. Effective modalities of treatment apart from conservative management, include both invasive surgical procedures such as renal vein transposition and autotransplantation of the kidney and more popular recently, the less invasive endovascular stenting. Both options, however, are not without complications, such as, retroperitoneal hematomas or stent migration, thrombosis and restenosis. We now present a case of spontaneous renosplenic shunting in a 68-year-old lady of Chinese descent with the nutcracker syndrome-the first of such cases to be ever reported in a patient with no preexisting predilection for chronic liver disease and portosystemic shunting. Despite having significant pelvic venous congestion as evident on computed tomography scans, she remained asymptomatic. This may present a novel paradigm shift for the treatment of the nutcracker syndrome -surgical creation of a renosplenic bypass instead of current modalities, an alternative solution which can be performed laparoscopically and is without problems related to stent use. The creation of laparoscopic splenorenal bypass has been reported once thus far in Cleveland Ohio by Chung and Gill with good symptomatic improvement but no further studies since to validate its long-term effectiveness.
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Affiliation(s)
- Ng Deborah Chieh Yih
- Department of General Medicine, Tan Tock Seng Hospital, Singapore ; Department of General Surgery, Tan Tock Seng Hospital, Singapore
| | - Lee Hwee Chyen
- Department of General Medicine, Tan Tock Seng Hospital, Singapore
| | - Yang Cunli
- Department of Diagnostic Radiology, Tan Tock Seng Hospital, Singapore
| | - Punamiya Sundeep Jaywantraj
- Department of Diagnostic Radiology, Tan Tock Seng Hospital, Singapore ; Subspecialty of Interventional Radiology, Tan Tock Seng Hospital, Singapore
| | | | - Sule Ashish Anil
- Department of General Medicine, Tan Tock Seng Hospital, Singapore ; Subspecialty of Vascular Medicine, Tan Tock Seng Hospital, Singapore
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He Y, Wu Z, Chen S, Tian L, Li D, Li M, Jin W, Zhang H. Nutcracker syndrome--how well do we know it? Urology 2013; 83:12-7. [PMID: 24139744 DOI: 10.1016/j.urology.2013.08.033] [Citation(s) in RCA: 83] [Impact Index Per Article: 6.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2013] [Revised: 08/12/2013] [Accepted: 08/17/2013] [Indexed: 02/07/2023]
Abstract
Nutcracker syndrome (NCS), which is caused by compression of the left renal vein between the abdominal aorta and the superior mesenteric artery, leads to a series of clinical symptoms including hematuria, proteinuria, flank pain, and varicocele. The diagnosis of NCS is difficult due to variations in normal anatomy. Treatment, which ranges from observation to nephrectomy, remains controversial. We conducted a review based on the related literature and our experience with hundreds of cases. We summarize the characteristics of NCS, the different measurements used in diagnosis, and the current treatment options. We present our diagnostic criteria and recommend endovascular stenting as the primary option for NCS.
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Affiliation(s)
- Yangyan He
- Department of Vascular Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China
| | - Ziheng Wu
- Department of Vascular Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China
| | - Shanwen Chen
- Department of Urology, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China
| | - Lu Tian
- Department of Vascular Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China
| | - Donglin Li
- Department of Vascular Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China
| | - Ming Li
- Department of Vascular Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China
| | - Wei Jin
- Department of Vascular Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China
| | - Hongkun Zhang
- Department of Vascular Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, People's Republic of China.
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