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Implications of Erdheim-Chester disease and percutaneous renal access

Lael Stieglitz1, Vernon M Pais

  • 1Department of Surgery, Section of Urology, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire, USA.

The Canadian Journal of Urology
|December 16, 2020
PubMed

Insights

Erdheim-Chester disease (ECD) can cause retroperitoneal fibrosis, complicating kidney stone removal. Understanding these fibrotic changes is crucial for successful surgical planning and patient outcomes in urology.

Area of Science:

  • Urology
  • Histiocytosis
  • Retroperitoneal Fibrosis

Background:

  • Erdheim-Chester disease (ECD) is a rare systemic histiocytosis.
  • Urologic manifestations are common in ECD patients.
  • Retroperitoneal fibrosis is a significant ECD manifestation, often encasing the kidneys.

Observation:

  • A case involving a patient with a large kidney stone burden requiring percutaneous nephrolithotomy is presented.
  • The patient also had Erdheim-Chester disease with associated retroperitoneal fibrotic changes.

Findings:

  • Retroperitoneal fibrosis in ECD can present challenges during urologic procedures.
  • The dense inflammatory rind associated with ECD can complicate surgical access and manipulation.

Implications:

  • Awareness of ECD-related retroperitoneal fibrosis is vital for urologists.
  • Perioperative counseling and surgical planning must account for these fibrotic changes.
  • Informed planning can improve the likelihood of successful surgical outcomes for patients with ECD and kidney stones.

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