Nutcracker Syndrome

Navya

Pharm.D Intern, Kauvery Hospital, Alwarpet, Chennai, Tamil Nadu

Introduction

Nutcracker syndrome (NCS) is defined by the compression of the left renal vein (LRV) as it travels between the abdominal aorta and the superior mesenteric artery (SMA). This compression blocks the vein and ability to drain blood from the left kidney, leading to increased venous pressure in the left renal vein. The rise in pressure encourages the development of alternative vein pathways and causes backward flow into the pelvic and lumbar veins. Chronic pain is a typical symptom of this condition [1]. Nutcracker syndrome, also known as left renal vein entrapment syndrome, was first described anatomically by Grant in 1937. The condition gets its name from the anatomical arrangement in which the left renal vein is squeezed between the aorta and the superior mesenteric artery, resembling a nut caught in the jaws of a nutcracker. This arrangement causes narrowing in the aorta mesenteric part of the LRV and may lead to dilation of its lower part [2]. Early reports considered NCS a rare condition. However, more patients are now being diagnosed with NCS, especially those who had earlier been treated for kidney inflammation or kidney disease, presenting with symptoms like blood in the urine or protein in the urine. The exact frequency of NCS is not yet known. NCS can occur at any age, from childhood to old age, though it is more common in young and middle-aged adults. During puberty, the rapid growth in height and development of the vertebrae reduce the angle between the superior mesenteric artery and the aorta. The condition may be more common in females and women, though at least one study found no difference between genders. A low body mass index has been linked to a higher risk of NCS (3). Nutcracker syndrome occurs when the left renal vein is compressed as it passes between the abdominal aorta and the superior mesenteric artery. This compression causes a variety of symptoms, including blood in the urine, protein in the urine, flank pain, and varicocele [3]. The symptoms are thought to result from a significant increase in pressure within the renal vein, which is then transferred to the veins that drain the kidney. This high pressure is believed to damage the thin walls that separate the veins from the collecting system in the kidney area, leading to blood in the urine or protein in the urine [4]. This study included 23 patients diagnosed with NCS or NCP. The medical records of patients referred to our institution between 1999 and 2012, who had one or more of the following symptoms: abdominal pain, flank pain, blood in the urine (either visible or microscopic), or protein in the urine, and were later diagnosed with NCS or NCP, were reviewed in retrospect [5]. Computed tomography (CT), magnetic resonance imaging (MRI), and Doppler ultrasound (DUS) are all suitable imaging methods for evaluating patients suspected of having NCS. However, a definitive diagnosis usually requires an invasive procedure, such as catheter venography combined with pressure measurements [6]. Patients with NCS under 18 years of age are generally treated without surgery, as the anatomical changes that happen during growth and puberty may change the angle between the superior mesenteric artery and the aorta, thus reducing the compression on the left renal vein [7]. Transposition of the left renal vein is a safe and effective treatment option for NCS in suitable paediatric patients [8]. Different surgical methods have been used to relieve the pressure on the veins, the most common being moving the left renal vein to a more distant part of the inferior vena cava. There is limited long-term data on the results of surgical treatments. Recently, endovascular stenting of the left renal vein has been introduced as an alternative treatment method [9].

Case Presentation

A 9years old male patient with the above-mentioned complaints and clinical findings. After all pre-operative investigation, pre aesthetic evaluation and informed consent from parents he was taken up on Left Renal vein decompression on 01/09/2026. Post operatively, he was treated with intravenous analgesic and other supportive medication. He remained clinically and haemodynamically stable during the hospital stay and tolerated oral feeds very well and hence discharged with following advice.

Diagnosis: Nutcracker syndrome with Hematuria.

Background

Nutcracker syndrome is a rare syndrome which is characterized by the compression of the left renal vein (LRV) between the aorta and superior mesenteric artery which lead to chronic pain. Hematuria and proteinuria are the common symptoms of NCS.

Presentation

The patient presented with hematuria on strenuous activities for 3years, last episode during last week, Epistaxis once in 15days for 4years which increases during last episode and left flank pain in everyday evaluated extensively and diagnosed as Nutcracker syndrome with hematuria.

Treatment

The patient underwent a left renal decompression, a surgical procedure which relieves compression of the left renal vein as it passess between the aorta and the superior mesenteric artery.

Procedure

Patient in supine position, general anesthesia was given via IV access left hand. Cefuroxime and Paracetamol was given intravenously. Midline laparotomy made from xiphisternum to umbilicus. Small bowel mobilised to the right and cephalad and packed and protected. Transeverse colon and mesocolon lifted upwards and posterior peritoneum opened over the aortic region. Left renal vein was found to be distended proximal to the compression site between the SMA and aorta. Lumbar vein draining into the LRV were doubly ligated and divided. After complete mobilisation of the left renal vein ostium, no stenosis or fibrous narrowing was noted. Hence decision was made to perform LRV-IVC bypass. In the right high, a longitudinal medial incision was made and the great saphenous vein was harvested. As a single segment of GSV with adequate calibre was not available, the required conduit was constructed by spilcing two GSV segments longitudinally for the required length. The GSV was reversed and valves were removed after opening. After adequate heparinisation, partial side clamping of the LRV was done. The GSV panel graft was anastomosed to the LRV using 7-0 prolene by end-to-end technique. IVC side clamping was then done below the LRV insertion. The graft was anastomosed to the IVC using 7-0 prolene by end-to-end technique. Before de clamping, de airing/flushing was done. Clamps were released and the LRV and graft were found to be filling normally. No bleeding was noted from either anastomotic side. Total intraoperative heparin is 3000 units.

Outcome

The surgery was successfully done. Pereitoneum closed over the IVS< aorta, LRV and graft bypass. Bowel positioned intra-peritoneally in normal position. No intraperitoneal bleeding. Bowel normal. Laparotomy wound closed using 1-0 PDS for linea alba, 3-0 vicryl for subcutaneous layer and staples for skin. Leg wound closed using 3-0 vicryl and3-0 monocryl. Sterile dressing applied.

Follow-up

The patient will need to be closely monitor the urine output, vital signs and kidney function test.

Discussion

This case report describes a 9years old male patient with nutcracker syndrome with hematuria who underwent a Left renal vein decompression to manage his condition. Nutcracker syndrome is a which is also known as left vein entrapment syndrome, in which compression of the left renal vein (LRV) as it travels between the abdominal aorta and the superior mesenteric artery (SMA). This compression blocks the vein and ability to drain blood from the left kidney, leading to increased venous pressure in the left renal vein [1]. The patient with nutcracker syndrome undergoes symptoms like blood in the urine, protein in the urine, flank pain, and varicocele due to the compression [3]. Left renal vein decompression is done under general anesthesia and successfully in releving the pressure on the left renal vein which is squeezed by surrounding blood vessels, which reduce the flank pain and other symptoms. Oral intake and urine output are normal. The patient recovered from the hospital after 5days of admission.

The case report highlights the importance and early diagnosis and treatment of

the nutcracker syndrome, as it needs conservative management, as this condition is associated with hematuria [2]. Renal vein transportation is reporting a good result and relatively less morbidity [10]. Although this procedure is associated with potential complication, with maximum recovery. His condition need regular follow up and kidney function monitoring, as it causes renal ischemia, anastomoses of the renal artery and ureter [11].

The etiology of NCS is being unknown and remain unclear. This condition occurs especially in children in the time of adolescence period, as it need a conservative management, endovascular intervention and regular follow-up. Surgical treatment will improve the quality of the life. Though without treatment it can leads to left renal vein thrombosis and resulting kidney damage. In such case like NCS associated with hematuria as it leads to anemia requiring blood transfusion and other complication [2]. This case emphasizes the importance of early diagnosis and management of NCS, and the benefits of renal vein decompression in treating NCS associated with hematuria. Further research is needed to evaluate the long term follow-up data regarding renal function, hematuria, proteinuria, growth and recurrence after treatment in pediatric NCS.

Conclusion

This is the case report highlights the eventual management of Nutcracker syndrome in a 9years old male with hematuria. NCS is defined by the compression of the left renal vein as it travels between the abdominal aorta and the superior mesenteric artery. [1] NCS is an unknown condition which affect the children of adolescence, of all gender. It indicates the symptoms like flank pain which is caused due to the compression of the left renal vein, hematuria, proteinuria and varicocele. Renal vein decompression is a procedure had done, which relieves the pressure of the left renal vein which is squeezed by the blood vessels. After the procedure, the patient’s renal function need to be monitor and also oral input and output. Conservative management should be required. If this condition left untreated it can leads to renal failure. In general, renal vein decompression can significantly improve the quality of the patient with NCS.

Funding: No funding source

Conflict of interest: None declared

Ethical approval: Not required

Reference

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