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Nutcracker syndrome or renal vein entrapment syndrome, occurs when the arteries near the kidney compress the left renal vein.
It gets its name because this compression is like a nutcracker crushing a nut.
There are 2 types:
- Anterior nutcracker syndrome, is the most common and occurs when the normally-placed left renal vein is compressed between the abdominal aorta and the superior mesenteric artery.
- Posterior nut cracker syndrome occurs when the left renal vein is compressed between an artery (usually the abdominal aorta) and the bones of the spine.
It is postulated that the compression of the left renal vein increases blood flow in other nearby veins and their engorgement causes the symptoms such as flank pain and blood in urine.
Nutcracker syndrome risk factors and prognosis
Nutcracker syndrome is a rare disorder that can affect adults or children, though the prevalence and specific causes are unknown.
It’s more common in women in their 30s and 40s who are tall and slim. Symptoms may appear after rapid weight loss.
Nutcracker syndrome is not hereditary, though it may be triggered by multiparity or pregnancy.
The prognosis for renal nutcracker syndrome is unclear.
In some cases, more commonly in children, it can resolve spontaneously.
However, without treatment, it is suggested it may predispose a person to left renal vein thrombosis and kidney damage. In cases presenting with hematuria, it can lead to anemia
Nutcracker syndrome symptoms
Nutcracker syndrome symptoms are many and very variable in severity. They can be asymptomatic especially in children. Symptoms are often worsened by physical activity.
The most common symptoms of nutcracker syndrome include:
- Flank pain (abdominal pain).
- Blood in urine (hematuria).
- Venous pelvic congestion symptoms in women, (pain and heaviness in the pelvis or genital area, pain during sexual intercourse, Severe menstrual cramping, Gluteal and vulvar varicose veins ).
- Men may develop varicoceles.
Other symptoms of nutcracker phenomenon can include:
- Leg Varicose veins.
- Painful urination.
- Low energy
- orthostatic intolerance (light-headedness or palpitations when standing upright).
Nutcracker Syndrome Investigations
Initialy a duplex ultrasound scan is undertaken, a CT venogram is then considered
Nutcracker Syndrome Treatment
The best treatment for renal nutcracker syndrome is controversial.
Nutcracker syndrome treatments can vary based on age, symptoms, and their severity.
In mild symptoms no treatment is needed.
Invasive treatment can involve endovenous stenting or surgery.
In this CT venogram the left renal vein is enlarged by compression between the aorta and the superiormesenteric artery.
Antiphospholipid syndrome (APS)
What is APS
APS is an autoimmune disorder which increases the risk of having a blood clot in the vein causing a deep vein thrombosis (DVT) or in the artery resulting in a stroke or heart attack. There is an increased risk of miscarriages.
Who gets APS
APS affects all ages, including children and babies, but is usually diagnosed between 20 and 50 years old. It affects women four times as often as men.
Although there is a genetic predisposition to APS it is likely that environmental factors precipitate the syndrome. It is not clear what these are. They may include viral infections (such as cytomegalovirus (CMV) or parovirus B19), bacterial infections (such as e.coli and food poisoning) and certain medications (such ass the oral contraceptive pill and anti-epileptic drugs) .
- Symptoms and complications may not be present and some with APS are undiagnosed.
- A consequence of developing blood clots in the blood vessels depends on where they occur:
- In veins can result in a DVT, PE and superficial thrombophlebitis
- In arteries can result in high blood pressure, stroke and heart attacks
- In small vessels skin vessels can result in Livedo reticularis The skin is blotchy red or blue in appearance. Some people also develop ulcers (sores) and nodules (bumps). These symptoms are worse in cold weather
- There is an increased risk of miscarriages and pre-eclampsia
- People with APS may also experience more vague symptoms , which can be significant. These include
- balance and mobility problems
- vision problems, such as double vision
- speech and memory problems
- a tingling sensation or pins and needlesin arms and legs
- headachesor migraines
APS is diagnosed on the combination of blood results and clinical features.
There needs to be a positive test for one of the autoantibodies on 2 or more occasions at least 12 weeks apart.
The autoantibodies tested for are
- lupus anticoagulant (e.g., dilute Russell viper venom test or DRVVT and hexagonal phase phospholipid neutralization test) Need to be present for a positive test
- anticardiolipin antibodies: Needs to be present at medium or high levels for a positive test
- anti-beta2-glycoprotein I antibodies. Needs to be present at a high level (>99th percentile)
A diagnosis of APS can only be made after 2 abnormal blood test results, with at least a 12-week gap between them. This is because harmless antiphospholipid antibodies can sometimes develop in the body for short periods of time. Usually, this is the result of an infection or a side effect of medication, such as antibiotics.
A diagnosis of APS in those with raised autoantibodies can usually be confirmed if there has been both
- 1 or more confirmed blood clots
- Complications of pregnancy
- 1 or more unexplained late miscarriages at or after week 10 of your pregnancy
- 1 or more premature births at or before week 34 of your pregnancy
- 3 or more unexplained early miscarriages before week 10 of your pregnancy
Although there’s no cure for APS, the risk of developing blood clots can be greatly reduced if it’s correctly diagnosed.
Most people with APS need to take anticoagulant or antiplatelet medication daily for life.
Low dose Aspirin is recommended if blood tests show abnormal antiphospholipid antibodies, without a history of blood clots. Clopidogrel is used as an alternative. .
Warfarin is recommended if blood tests show abnormal antiphospholipid antibodies, with a history of blood clots. As yet the DOACs are considered to be an inferior alternative.
When pregnant the Warfarin or aspirin is changed to heparin injections. This results in an 80% chance of a successful pregnancy
If blood clots develop despite warfarin treatment or the symptoms suddenly become severe, heparin injections may be needed.
Take all steps to reduce the risk of developing blood clots. These include not smoking, eating a healthy, balanced diet, regular exercise, maintaining a healthy weight
Complications: Note on Catastrophic antiphospholipid syndrome (CAPS)
CAPS is a serious complication of APS.
- It occurs in < 1% of people with APS.
- Blood clots suddenly form throughout the body, resulting in multiple organ failure.
- The cause is not clear, but 20% follow an infection, trauma or surgery.
- The symptoms usually develop suddenly and rapidly get worse.
- Treatment is on the intensive care unit to support the failing body and provide high-dose anticoagulants to stop the blood clots extending
- With the best available treatment, 50% of people die.