Nutcracker syndrome is a vascular compression disorder and refers to the compression of the left renal vein between the superior mesenteric artery (SMA) and aorta. This can lead to renal venous hypertension, resulting in rupture of thin-walled veins into the collecting system with resultant haematuria.
Nutcracker syndrome should not be confused with superior mesenteric artery syndrome (Wilkie syndrome) also a superior mesenteric artery compression disorder, where the SMA compresses the third part of the duodenum (the two conditions however may be associated).
There is a slightly greater female predilection.
The most common clinical manifestation of the nutcracker syndrome is intermittent macroscopic haematuria 3. Repeated episodes of gross haematuria and left flank pain should be present. Haematuria should be from the left ureteric orifice only 4. In the absence of clinical features, renal vein narrowing is usually developmental and not causing any pressure gradient, and thus should not be labelled as nutcracker syndrome.
- may occur simultaneously with SMA syndrome
- an association with a thin or asthenic body habitus has long been noted.
Radiographic features are similar on ultrasound, Doppler ultrasound, CT, MRI, and conventional angiography:
- reduced aortic-SMA angle (the normal angle between aorta and SMA is approximately 45° (38-65°)
- left renal vein stenosis
- collateral pathways: main collateral pathway is the left gonadal vein which will display early enhancement during portal venous phase
- pressure gradient >3 mm Hg on renal venography
- compression ratio (CR) given by the equation CR=P-C/C i.e. diameter of pre-compressed vein (P) minus diameter of compressed vein (C) divided by the diameter of the compressed vein (C). A compression ratio above 2.25 is highly sensitive and specific for Nutcracker syndrome 10.
Persistent haematuria can precipitate renal vein thrombosis 4.
Treatment and prognosis
Treatment should be started strictly when it is causing symptoms (haematuria and left flank pain). Surgical treatment modalities have their inherent complications and should be contemplated only when strongly indicated. A few of the reported surgical choices are:
- left renal vein transposition 5
- superior mesenteric artery transposition
- endovascular stent-graft placement 6
History and etymology
The first clinical report of this syndrome was made by El-Sadr and Mina in 1950 while the term "nutcracker syndrome" is thought to have been first used by de Schepper in 1972 7.
- 1. Little AF, Lavoipierre AM. Unusual clinical manifestations of the Nutcracker Syndrome. (2002) Australasian radiology. 46 (2): 197-200. Pubmed
- 2. Kavukcu S, Kasap B, Göktay Y, Seçil M. Doppler sonographic indices in diagnosing the nutcracker phenomenon in a hematuric adolescent. (2004) Journal of clinical ultrasound : JCU. 32 (1): 37-41. doi:10.1002/jcu.10222 - Pubmed
- 3. 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. (1999) AJR. American journal of roentgenology. 172 (1): 39-43. doi:10.2214/ajr.172.1.9888735 - Pubmed
- 4. Muraoka N, Sakai T, Kimura H, Uematsu H, Tanase K, Yokoyama O, Itoh H. Rare causes of hematuria associated with various vascular diseases involving the upper urinary tract. Radiographics : a review publication of the Radiological Society of North America, Inc. 28 (3): 855-67. doi:10.1148/rg.283075106 - Pubmed
- 5. Bo Almgren, Magne Juhl. Superior mesenteric artery syndrome complicating treatment with balanced traction: A case report. (2009) Acta Orthopaedica Scandinavica. doi:10.3109/17453677708985106
- 5. Hohenfellner M, D'Elia G, Hampel C, Dahms S, Thüroff JW. Transposition of the left renal vein for treatment of the nutcracker phenomenon: long-term follow-up. (2002) Urology. 59 (3): 354-7. Pubmed
- 6. Chen W, Chu J, Yang JY, Li HP, Zhuang WQ, Huang YH, Guo WB. Endovascular stent placement for the treatment of nutcracker phenomenon in three pediatric patients. (2005) Journal of vascular and interventional radiology : JVIR. 16 (11): 1529-33. doi:10.1097/01.RVI.0000178259.87608.EC - Pubmed
- 7. Lamba R, Tanner DT, Sekhon S, McGahan JP, Corwin MT, Lall CG. Multidetector CT of vascular compression syndromes in the abdomen and pelvis. Radiographics : a review publication of the Radiological Society of North America, Inc. 34 (1): 93-115. doi:10.1148/rg.341125010 - Pubmed
- 8. Fong JK, Poh AC, Tan AG, Taneja R. Imaging findings and clinical features of abdominal vascular compression syndromes. (2014) AJR. American journal of roentgenology. 203 (1): 29-36. doi:10.2214/AJR.13.11598 - Pubmed
- 9. Kurklinsky AK, Rooke TW. Nutcracker phenomenon and nutcracker syndrome. (2010) Mayo Clinic proceedings. 85 (6): 552-9. doi:10.4065/mcp.2009.0586 - Pubmed
- 10. Hangge, Patrick, Gupta, Nikhil, Khurana, Aditya, Quencer, Keith, Albadawi, Hassan, Alzubaidi, Sadeer, Knuttinen, M-Grace, Naidu, Sailendra, Oklu, Rahmi. Degree of Left Renal Vein Compression Predicts Nutcracker Syndrome. (2018) Journal of Clinical Medicine. 7 (5): 107. doi:10.3390/jcm7050107