When Kidneys Move: Nephroptosis in hEDS

For people living with hypermobile Ehlers-Danlos syndrome (hEDS), unexplained pain and strange, positional symptoms are often dismissed or misattributed. One lesser known but potentially relevant condition in this community is nephroptosis, sometimes called floating kidney. While considered rare in the general population, nephroptosis may be underrecognized in people with connective tissue disorders, including hEDS.

What Is Nephroptosis?

Nephroptosis is a condition in which one or both kidneys drop downward when a person moves from lying down to standing. Normally, kidneys are held in place by connective tissue, fat, and supporting structures. In nephroptosis, this support is insufficient, allowing the kidney to move excessively—sometimes several centimeters.

This abnormal movement can cause stretching or kinking of blood vessels, ureters, or surrounding nerves, leading to pain and other symptoms that often change with posture.

Why Does Nephroptosis Matter in hEDS?

hEDS is characterized by connective tissue laxity, which affects joints, skin, blood vessels, and internal organs. Ligaments and fascial supports throughout the body may be more elastic or fragile than normal.

In theory—and increasingly in patient experience—this laxity may also affect the renal fascia and supporting tissues, making people with hEDS more vulnerable to kidney mobility. Despite this, nephroptosis is rarely discussed in hEDS care guidelines, and many clinicians are unfamiliar with its relevance to connective tissue disorders.

Signs and Symptoms of Nephroptosis

Symptoms can vary widely and may come and go depending on body position. Commonly reported symptoms include:

Pain-Related Symptoms

• Flank pain (one or both sides)

• Lower back pain

• Abdominal or pelvic pain

• Pain that worsens when standing or walking and improves when lying down

• Pain after prolonged upright posture

  
  

Urinary and Systemic Symptoms

• Nausea or vomiting

• Urinary frequency or urgency

• Hematuria (blood in urine)

• Recurrent urinary tract infections

• Sensation of internal “pulling” or pressure

Overlap With hEDS Symptoms

Many nephroptosis symptoms overlap with common hEDS complaints, such as:

• Chronic pain

• Dysautonomia-like symptoms

• Gastrointestinal discomfort

• Fatigue

This overlap can make nephroptosis easy to miss or dismiss, especially when imaging is done only while lying flat.

How Common Is Nephroptosis in hEDS?

There is no reliable prevalence data specifically for nephroptosis in hEDS. In the general population, nephroptosis is considered rare, but historically it was likely underdiagnosed due to limitations in imaging.

Among people with hEDS and related connective tissue disorders, nephroptosis may be:

• More common than recognized

• Underdiagnosed due to lack of upright imaging

• Misattributed to musculoskeletal or gastrointestinal causes

  Most evidence currently comes from case reports, small studies, and patient communities, not large-scale epidemiological research. This represents a significant gap in medical knowledge.

  
  

  Several factors contribute to underdiagnosis:

  • Standard CT scans and ultrasounds are usually performed lying down

  • Symptoms may be labeled as “functional” or psychosomatic

  • Lack of awareness among clinicians

  • Overlap with more familiar hEDS complications

    
    

  In some cases, the kidney may appear completely normal on supine imaging, even when symptoms are severe.

  How Is Nephroptosis Diagnosed? Diagnosis often requires positional imaging, such as:

  
  

  • Upright or standing ultrasound

  • IVP (intravenous pyelogram) performed both supine and upright

  • Dynamic imaging comparing kidney position in different postures

  Clinical history—especially postural pain relief when lying down—is also an important clue.

  
  

  Treatment Options

  Depend on symptom severity

  
  

  Conservative Management:

  • Abdominal or lumbar support garments

  • Activity modification

  • Pain management strategies

  • Monitoring kidney function

    
    

    In severe, refractory cases, nephropexy (surgical fixation of the kidney) may be considered. Outcomes can vary, especially in connective tissue disorders, and surgery requires careful risk-benefit discussion.

    
    

    For people with hEDS, unexplained pain is too often normalized or minimized. Recognizing nephroptosis as a possible contributor to positional pain empowers patients to advocate for appropriate evaluation and helps clinicians think beyond just joints and muscles when patients have hEDS.

    

    Unsplash Photo