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Nutcracker syndrome
Radiography xxx (2014) 1e2
Contents lists available at ScienceDirect
Radiography journal homepage: www.elsevier.com/locate/radi
Case report
Nutcracker syndrome Ingrid Jolley* Sandringham Hospital, Alfred Health, 193 Bluff Road, Sandringham, Victoria 3191, Australia
a r t i c l e i n f o
a b s t r a c t
Article history: Received 8 February 2014 Accepted 19 February 2014 Available online xxx
Purpose: The purpose of this case study is to highlight the symptoms of the Nutcracker Syndrome (NCS), the methods of clinical investigations and the importance of differential diagnosis. Introduction: The NCS refers to left renal vein entrapment caused by abnormal branching patterns of the superior mesenteric artery from the aorta.1,2 Clinical case presentation: A 27 years old female presented to the emergency department with complaints of abdominal discomfort, bloating, loose bowel motions and irregular micro-haematuria. The radiologist’s report indicated the findings from computed tomography examination to be consistent with anterior NCS. Discussion: In most of the NCS cases the clinical symptoms are non-specific.3 The syndrome is caused by a vascular disorder, but its clinical manifestation can relate to a wide range of abdominal, urological, endovascular or gynaecological pathologies.4 Conclusion: Nutcracker Syndrome is a relatively rare disease and underdiagnosed may lead to left renal vein thrombosis. Ó 2014 Published by Elsevier Ltd on behalf of The College of Radiographers.
Keywords: Left renal vein Nutcracker syndrome Diagnosis
Introduction The Nutcracker Syndrome, also known as the Left Renal Vein (LRV) Entrapment Syndrome, was initially described by anatomist Grant in 1937.1 The first clinical study on NCS was published by ElSadr et al. in 1950.1 Left renal vein entrapment is caused by an abnormal branching pattern of Superior Mesenteric Artery (SMA) with an acute, (less than 90 ), aortic-mesenteric angle.2 With this branching configuration the aorta and SMA resemble the jaws of a nutcracker and the LRV is considered “a nut” between them.2 This condition has also been termed as the anterior Nutcracker, as the posterior Nutcracker occurs when the LRV is compressed in a decreased space between the aorta and the vertebra.2 The “posterior” entrapment of the LRV is considered to be a variation of NCS.2 The case description A 27-year old female presented to the emergency department with complaints of abdominal discomfort, bloating, loose bowel motions and irregular micro-haematuria. The possibility of pregnancy was excluded. Clinical examinations involved abdominalpelvic computed tomography (CT) with an application of intra
* Tel.: þ61 (0) 402076258. E-mail address: [email protected].
venous contrast media and biochemical blood and urine tests. The results from the patient’s biochemical blood and urine tests were within the normal range. The CT scan demonstrated compression of the left renal vein between the aorta and SMA. According to the Radiologist’s report, the findings from CT examination were consistent with NCS (Figs. 1 and 2). No other medical imaging methods or biochemical tests were applied to this particular case. The patient was observed within the emergency department for 4 h before being discharged and referred to general practitioner for further observation and management.
The discussion In most of the NCS cases the clinical symptoms are non-specific.3 They could manifest as abdominal pain or discomfort, pelvic congestion syndrome, dyspareunia, orthostatic pain, post-coital pain and varices in pelvic, vulvar, gluteal and thigh regions.3 Although the syndrome is caused by a vascular disorder, its clinical manifestation can relate to a wide range of abdominal, urological, endovascular or gynaecological pathologies, therefore; the differential diagnosis is important.4 The main clinical symptoms of Nutcracker Syndrome involve left flank and abdominal pain that can be accompanied by microscopic or macroscopic haematuria.3 The development of haematuria is usually correlated to the
http://dx.doi.org/10.1016/j.radi.2014.02.006 1078-8174/Ó 2014 Published by Elsevier Ltd on behalf of The College of Radiographers.
Please cite this article in press as: Jolley I, Nutcracker syndrome, Radiography (2014), http://dx.doi.org/10.1016/j.radi.2014.02.006
2
I. Jolley / Radiography xxx (2014) 1e2
associated with compromised communication system between dilated venous sinuses and adjacent renal calices.3 Under-diagnosed and untreated NCS may lead to a development of left renal vein thrombosis due to blood congestion within compressed portion of the vessel.3,5 The medical imaging modalities that are applied for diagnosis of NCS include CT, magnetic resonance imaging (MRI), digital subtraction angiography (DSA) and Doppler sonography.6,7 The CT and MRI tests can demonstrate the precise LRV compression point and these modalities are usually applied for initial investigations.6 The retrograde left renal vein DSA is considered the reference standard in the diagnosis of NSC, as this method of examination allows the visualization of the LRV compression, the evaluation of venous reflux and the detection of collaterals.6 The Doppler sonography can provide the measurements of venous blood flow velocity with the respect to compression point and may demonstrate 69%e90% sensitivity and 89%e100% specificity in diagnosis of NCS.1,7 The limitations for this case study are associated with unavailability of DSA or sonographic investigations and inaccessibility of patient’s follow-up tests. Conclusion Figure 1. Sagittal CT image of NCS showing compressed left renal vein (LRV). Ao e aorta, SMA e superior mesenteric artery.
Nutcracker Syndrome develops when LRV is compressed between the aorta and SMA. The clinical symptoms associated with this condition are non-specific in most of the cases, therefore; the clinical investigations should employ medical imaging for differential diagnosis. Un-detected and untreated NCS may lead to the development of LRV thrombosis. Conflict of interest None declared. Acknowledgements The author thanks Helen Kavnoudis, Research Manager, Department of Radiology, Alfred Health and Ngon Tran, Senior Supervisor, Department of Radiology, Alfred Health for technical support with this manuscript. References
Figure 2. Axial CT image of NCS showing compressed left renal vein (LRV). Ao e aorta, SMA e superior mesenteric artery.
processes where the compression of LRV increases the flow gradient between the LRV and inferior vena cava (IVC) up to 14 mmHg.3,4 The elevated flow gradient may lead to rupture of thin-walled septa between the smaller veins and the collecting system in renal fornix.3 Another cause for heamaturia may be
1. Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc 2010;6:552e9. 2. Cuellar i Calabria H, Quiroga Gomez S, Sebastia Cerqueda C, Boye de la Presa R, Miranda A, Alvarez-Castells A. Nutcracker or left renal vein compression phenomenon: multidetector computed tomography findings and clinical significance. Eur Radiol 2005;15:1745e51. 3. Ahmed K, Sampath R, Khan MS. Current trends in the diagnosis and management of renal nutcracker syndrome: a review. Eur J Vasc Endovasc Surg 2006;31: 410e6. 4. Scultetus AH, Villavicencio JL, Gillespie DL. The nutcracker syndrome: its role in the pelvic venous disorders. J Vasc Surg 2001;5:812e9. 5. Mallat F, Hmida W, Jaidane M, Mama N, Mozbah F. Nutcracker syndrome complicated by left renal vein thrombosis. Case Rep Urol 2013;2013:168057 [Epub 2013 Nov 24] doi: 10.1155/2013/168057. 6. Eliahou R, Sosna J, Bloom AI. Between a rock and a hard place: clinical and imaging features of vascular compression syndromes. RadioGraphics 2012;32: E33e49. 7. Takebayashi S, Ueki T, Ikeda N, Fujikawa A. Diagnosis of the nutcracker syndrome with color Doppler sonography: correlation with flow patterns on retrograde left renal venography. AJR 1999;172:39e43.
Please cite this article in press as: Jolley I, Nutcracker syndrome, Radiography (2014), http://dx.doi.org/10.1016/j.radi.2014.02.006
Contents lists available at ScienceDirect
Radiography journal homepage: www.elsevier.com/locate/radi
Case report
Nutcracker syndrome Ingrid Jolley* Sandringham Hospital, Alfred Health, 193 Bluff Road, Sandringham, Victoria 3191, Australia
a r t i c l e i n f o
a b s t r a c t
Article history: Received 8 February 2014 Accepted 19 February 2014 Available online xxx
Purpose: The purpose of this case study is to highlight the symptoms of the Nutcracker Syndrome (NCS), the methods of clinical investigations and the importance of differential diagnosis. Introduction: The NCS refers to left renal vein entrapment caused by abnormal branching patterns of the superior mesenteric artery from the aorta.1,2 Clinical case presentation: A 27 years old female presented to the emergency department with complaints of abdominal discomfort, bloating, loose bowel motions and irregular micro-haematuria. The radiologist’s report indicated the findings from computed tomography examination to be consistent with anterior NCS. Discussion: In most of the NCS cases the clinical symptoms are non-specific.3 The syndrome is caused by a vascular disorder, but its clinical manifestation can relate to a wide range of abdominal, urological, endovascular or gynaecological pathologies.4 Conclusion: Nutcracker Syndrome is a relatively rare disease and underdiagnosed may lead to left renal vein thrombosis. Ó 2014 Published by Elsevier Ltd on behalf of The College of Radiographers.
Keywords: Left renal vein Nutcracker syndrome Diagnosis
Introduction The Nutcracker Syndrome, also known as the Left Renal Vein (LRV) Entrapment Syndrome, was initially described by anatomist Grant in 1937.1 The first clinical study on NCS was published by ElSadr et al. in 1950.1 Left renal vein entrapment is caused by an abnormal branching pattern of Superior Mesenteric Artery (SMA) with an acute, (less than 90 ), aortic-mesenteric angle.2 With this branching configuration the aorta and SMA resemble the jaws of a nutcracker and the LRV is considered “a nut” between them.2 This condition has also been termed as the anterior Nutcracker, as the posterior Nutcracker occurs when the LRV is compressed in a decreased space between the aorta and the vertebra.2 The “posterior” entrapment of the LRV is considered to be a variation of NCS.2 The case description A 27-year old female presented to the emergency department with complaints of abdominal discomfort, bloating, loose bowel motions and irregular micro-haematuria. The possibility of pregnancy was excluded. Clinical examinations involved abdominalpelvic computed tomography (CT) with an application of intra
* Tel.: þ61 (0) 402076258. E-mail address: [email protected].
venous contrast media and biochemical blood and urine tests. The results from the patient’s biochemical blood and urine tests were within the normal range. The CT scan demonstrated compression of the left renal vein between the aorta and SMA. According to the Radiologist’s report, the findings from CT examination were consistent with NCS (Figs. 1 and 2). No other medical imaging methods or biochemical tests were applied to this particular case. The patient was observed within the emergency department for 4 h before being discharged and referred to general practitioner for further observation and management.
The discussion In most of the NCS cases the clinical symptoms are non-specific.3 They could manifest as abdominal pain or discomfort, pelvic congestion syndrome, dyspareunia, orthostatic pain, post-coital pain and varices in pelvic, vulvar, gluteal and thigh regions.3 Although the syndrome is caused by a vascular disorder, its clinical manifestation can relate to a wide range of abdominal, urological, endovascular or gynaecological pathologies, therefore; the differential diagnosis is important.4 The main clinical symptoms of Nutcracker Syndrome involve left flank and abdominal pain that can be accompanied by microscopic or macroscopic haematuria.3 The development of haematuria is usually correlated to the
http://dx.doi.org/10.1016/j.radi.2014.02.006 1078-8174/Ó 2014 Published by Elsevier Ltd on behalf of The College of Radiographers.
Please cite this article in press as: Jolley I, Nutcracker syndrome, Radiography (2014), http://dx.doi.org/10.1016/j.radi.2014.02.006
2
I. Jolley / Radiography xxx (2014) 1e2
associated with compromised communication system between dilated venous sinuses and adjacent renal calices.3 Under-diagnosed and untreated NCS may lead to a development of left renal vein thrombosis due to blood congestion within compressed portion of the vessel.3,5 The medical imaging modalities that are applied for diagnosis of NCS include CT, magnetic resonance imaging (MRI), digital subtraction angiography (DSA) and Doppler sonography.6,7 The CT and MRI tests can demonstrate the precise LRV compression point and these modalities are usually applied for initial investigations.6 The retrograde left renal vein DSA is considered the reference standard in the diagnosis of NSC, as this method of examination allows the visualization of the LRV compression, the evaluation of venous reflux and the detection of collaterals.6 The Doppler sonography can provide the measurements of venous blood flow velocity with the respect to compression point and may demonstrate 69%e90% sensitivity and 89%e100% specificity in diagnosis of NCS.1,7 The limitations for this case study are associated with unavailability of DSA or sonographic investigations and inaccessibility of patient’s follow-up tests. Conclusion Figure 1. Sagittal CT image of NCS showing compressed left renal vein (LRV). Ao e aorta, SMA e superior mesenteric artery.
Nutcracker Syndrome develops when LRV is compressed between the aorta and SMA. The clinical symptoms associated with this condition are non-specific in most of the cases, therefore; the clinical investigations should employ medical imaging for differential diagnosis. Un-detected and untreated NCS may lead to the development of LRV thrombosis. Conflict of interest None declared. Acknowledgements The author thanks Helen Kavnoudis, Research Manager, Department of Radiology, Alfred Health and Ngon Tran, Senior Supervisor, Department of Radiology, Alfred Health for technical support with this manuscript. References
Figure 2. Axial CT image of NCS showing compressed left renal vein (LRV). Ao e aorta, SMA e superior mesenteric artery.
processes where the compression of LRV increases the flow gradient between the LRV and inferior vena cava (IVC) up to 14 mmHg.3,4 The elevated flow gradient may lead to rupture of thin-walled septa between the smaller veins and the collecting system in renal fornix.3 Another cause for heamaturia may be
1. Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc 2010;6:552e9. 2. Cuellar i Calabria H, Quiroga Gomez S, Sebastia Cerqueda C, Boye de la Presa R, Miranda A, Alvarez-Castells A. Nutcracker or left renal vein compression phenomenon: multidetector computed tomography findings and clinical significance. Eur Radiol 2005;15:1745e51. 3. Ahmed K, Sampath R, Khan MS. Current trends in the diagnosis and management of renal nutcracker syndrome: a review. Eur J Vasc Endovasc Surg 2006;31: 410e6. 4. Scultetus AH, Villavicencio JL, Gillespie DL. The nutcracker syndrome: its role in the pelvic venous disorders. J Vasc Surg 2001;5:812e9. 5. Mallat F, Hmida W, Jaidane M, Mama N, Mozbah F. Nutcracker syndrome complicated by left renal vein thrombosis. Case Rep Urol 2013;2013:168057 [Epub 2013 Nov 24] doi: 10.1155/2013/168057. 6. Eliahou R, Sosna J, Bloom AI. Between a rock and a hard place: clinical and imaging features of vascular compression syndromes. RadioGraphics 2012;32: E33e49. 7. Takebayashi S, Ueki T, Ikeda N, Fujikawa A. Diagnosis of the nutcracker syndrome with color Doppler sonography: correlation with flow patterns on retrograde left renal venography. AJR 1999;172:39e43.
Please cite this article in press as: Jolley I, Nutcracker syndrome, Radiography (2014), http://dx.doi.org/10.1016/j.radi.2014.02.006