Coding Rules Current as at 18-Oct-2016 08: 31

Published 15 June 2015,
for implementation 01 July 2015.

Ref No: Q2837 | Published On: 15-Jun-2015 | Status: Current

SUBJECT: Retroperitoneal fibrosis without ureteric obstruction.

Q:

How do you code retroperitoneal fibrosis without ureteric obstruction?

A:

Retroperitoneal fibrosis (RPF) (Ormond’s disease) is a rare condition that results in inflammation and fibrosis within the retroperitoneal space. The inflammation and fibrosis usually begins around the distal aorta and spreads toward the inferior vena cava, kidneys and surrounding structures, resulting in compression.

Signs and symptoms of RPF are dependent upon the specific body structure that is being compressed by the fibrotic mass. When RPF surrounds and compresses the ureters it causes obstruction and may lead to hydronephrosis and consequently kidney failure if left untreated. Compression of the inferior vena cava results in deep venous thrombosis and lower extremity oedema. Impaired blood flow in the intestines may lead to necrosis.

Approximately 70% of the cases of RPF are idiopathic. Other causes include drug-induced, malignancies, complication of external beam radiotherapy, certain infections (tuberculosis, histoplasmosis) or complication of previous abdominal surgery or trauma.

The indexing and classification of idiopathic retroperitoneal fibrosis (Ormond’s disease) in ICD-10-AM is consistent with ICD-10 and other ICD-10 modifications (ICD-10-CM, ICD-10-CA), which defaults to N13.5 Kinking and stricture of ureter without hydronephrosis via the index pathways: