Renal nutcracker syndrome

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ORPHA:71273Q27.8
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1Active trials2Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Renal nutcracker syndrome, also called left renal vein entrapment syndrome or nutcracker phenomenon, is a condition where the left kidney's main vein (the left renal vein) gets squeezed or compressed as it passes between two large blood vessels in the abdomen — the aorta and the superior mesenteric artery. Think of it like a nutcracker squeezing a nut: the vein gets pinched, which causes blood to back up and pressure to build inside the kidney's blood vessels. This increased pressure can cause blood to leak into the urine, pain in the left side or lower back, and other symptoms. The condition affects people of all ages but is most often diagnosed in children, teenagers, and young adults. Symptoms can range from mild — such as finding blood in the urine during a routine test — to more severe, including significant pain, anemia from blood loss, and in some cases pelvic pain or swelling of veins in the pelvis. Treatment depends on how severe the symptoms are. Many younger patients, especially children, are managed with watchful waiting because the condition sometimes improves on its own as the body grows. For those with more serious symptoms, options include minimally invasive procedures to place a small stent inside the vein, or surgery to reposition the vein. The outlook for most people is good, especially with appropriate care.

Also known as:

Key symptoms:

Blood in the urine (urine may look pink, red, or cola-colored)Left-sided flank or lower back painPelvic pain or discomfort, especially in womenVaricocele (swollen veins in the scrotum) in malesFatigue or tiredness due to anemia from ongoing blood lossProtein in the urine detected on lab testsAbdominal pain, often on the left sidePainful or heavy periods in womenFeeling of fullness or pressure in the abdomenDizziness or lightheadedness, especially when standing

Clinical phenotype terms (25)— hover any for plain English
Renal artery stenosisHP:0001920Varicose veinsHP:0002619Low back painHP:0003419Dilatation of mesenteric arteryHP:0011934VaricoceleHP:0012871Flank painHP:0030157Pelvic painHP:0034267Vulval varicose veinHP:0100677Orthostatic hypotensionHP:0001278
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

7 events
Dec 2025A Long-term Study of the Safety and Effectiveness of RAP-219 in Adults With Focal Onset Seizures

Rapport Therapeutics Inc. — PHASE2

TrialRECRUITING
Oct 2025BHV-7000 Responsive Neurostimulation System (RNS) Study

Yale University — PHASE1

TrialACTIVE NOT RECRUITING
Sep 2024Lumbar Vein Embolization for Chronic Headaches With Nutcracker Physiology

Mayo Clinic — NA

TrialRECRUITING
Aug 2022Network Effects of Therapeutic Deep Brain Stimulation

The University of Texas Health Science Center, Houston — NA

TrialRECRUITING
Aug 2022RNS System NAUTILUS Study

NeuroPace — PHASE3

TrialACTIVE NOT RECRUITING
Apr 2021Clinical and Electrographic Changes in Responsive Neurostimulation System (RNS) Patients with Acupuncture Treatment

The University of Texas Health Science Center, Houston — NA

TrialRECRUITING
Jun 2015RNS® System Epilepsy Post-Approval Study

NeuroPace

TrialACTIVE NOT RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Renal nutcracker syndrome.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
N/A1 trial
Lumbar Vein Embolization for Chronic Headaches With Nutcracker Physiology
N/A
Actively Recruiting
PI: Zlatko Devcic, MD (Mayo Clinic) · Sites: Jacksonville, Florida · Age: 1899 yrs

Specialists

2 foundView all specialists →
MM
Martha Morrell, MD
STANFORD, CA
Specialist
PI on 3 active trials
ZM
Zlatko Devcic, MD
JACKSONVILLE, FL
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Renal nutcracker syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Renal nutcracker syndrome

Disease timeline:

New recruiting trial: Network Effects of Therapeutic Deep Brain Stimulation

A new clinical trial is recruiting patients for Renal nutcracker syndrome

New recruiting trial: A Long-term Study of the Safety and Effectiveness of RAP-219 in Adults With Focal Onset Seizures

A new clinical trial is recruiting patients for Renal nutcracker syndrome

New recruiting trial: Clinical and Electrographic Changes in Responsive Neurostimulation System (RNS) Patients with Acupuncture Treatment

A new clinical trial is recruiting patients for Renal nutcracker syndrome

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Is my case mild enough to be monitored without treatment, or do I need an intervention now?,What are the risks and benefits of stenting versus surgery for my specific situation?,How often do I need follow-up tests, and what should I watch for at home?,Could my symptoms — such as pelvic pain or heavy periods — be related to this condition?,Is there a risk of long-term kidney damage if I choose watchful waiting?,What signs or symptoms should prompt me to seek emergency care?,Are there any activity restrictions I should follow while being monitored?

Common questions about Renal nutcracker syndrome

What is Renal nutcracker syndrome?

Renal nutcracker syndrome, also called left renal vein entrapment syndrome or nutcracker phenomenon, is a condition where the left kidney's main vein (the left renal vein) gets squeezed or compressed as it passes between two large blood vessels in the abdomen — the aorta and the superior mesenteric artery. Think of it like a nutcracker squeezing a nut: the vein gets pinched, which causes blood to back up and pressure to build inside the kidney's blood vessels. This increased pressure can cause blood to leak into the urine, pain in the left side or lower back, and other symptoms. The condition

How is Renal nutcracker syndrome inherited?

Renal nutcracker syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Are there clinical trials for Renal nutcracker syndrome?

Yes — 1 recruiting clinical trial is currently listed for Renal nutcracker syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Renal nutcracker syndrome?

2 specialists and care centers treating Renal nutcracker syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.