Definition
Proliferation of aberrant fibroinflammatory tissue surrounds the infrarenal portion of the abdominal aorta, IVC and the iliac vessels.
Frequently entrap and obstruct the ureters and eventually leads to renal failure.
Types and causes
Idiopathic ( Ormand disease) – 70 % of RPF, Males > Females, 40 – 60 years of age.
Secondary RPF –
Radiation
Medication ( Hydralazine, methyldopa, ergotamines)
Inflammation ( Pancreatitis, pyelonephritis)
Malignancy ( Desmoplastic reaction, lymphoma)
Asbestos.
Epidimeology
Males > Females, 40 – 60 years.
Males > Females, 40 – 60 years.
Pathology
Macroscopic
Hard grey white plaque encasing retroperitoneal structure.
Macroscopic
Hard grey white plaque encasing retroperitoneal structure.
Microscopic –
Active stage – Immature fibrotic process, capillary proliferation, inflammatory cells, tissue edema.
Late stage – Reduced celluluar activity, collagen hyalinosis,
IHC – IgG positive.
Active stage – Immature fibrotic process, capillary proliferation, inflammatory cells, tissue edema.
Late stage – Reduced celluluar activity, collagen hyalinosis,
IHC – IgG positive.
Clinical features
Malaise, anorexia, weight loss, low grade fever, ureteral entrapment – Obstructive uropathy, renal vessel involvement – Renovascular hypertension, Lymphatic compression – Lower limb edema
Gonadal vessel compression – Varicocele, hydrocele.
Lab findings
Increased ESR, CRP, ANA, RA factor and renal parameters.
Increased ESR, CRP, ANA, RA factor and renal parameters.
Imaging findings
1. Ultrasound –
Hypoechoic, well defined irregularly contoured retroperitoneal mass anterior to lower lumbar spine
Hypoechoic, well defined irregularly contoured retroperitoneal mass anterior to lower lumbar spine
2.IVU and retrograde pyelography –
Medial deviation of middle third of ureters.
Tapering of lumen of bilateral middle ureter.
Proximal resultant hydroureteronephrosis, delayed excretion of contrast in obstructive uropathy.
Medial deviation of middle third of ureters.
Tapering of lumen of bilateral middle ureter.
Proximal resultant hydroureteronephrosis, delayed excretion of contrast in obstructive uropathy.
3.MDCT
Mostly idiopathic and benign RPF.
Soft tissue density in the periaortic region extending from level of renal arteries to iliac vessels,
Encasement of the ureters and inferior vena cava.
Enhancement in active stage, no enhancement in delayed stage.
Mostly idiopathic and benign RPF.
Soft tissue density in the periaortic region extending from level of renal arteries to iliac vessels,
Encasement of the ureters and inferior vena cava.
Enhancement in active stage, no enhancement in delayed stage.
(importance of CT – 1)To diagnose the cause of retroperitoneal fibrosis 2) Follow up ).
MRI
( Importance – 1) High contrast resolution 2) Assessment of urinary tract without contrast)
Early stage – High T2W signal with marked contrast enhancement.
Late stage – Low T2W signal with absent or low enhancement.
( Importance – 1) High contrast resolution 2) Assessment of urinary tract without contrast)
Early stage – High T2W signal with marked contrast enhancement.
Late stage – Low T2W signal with absent or low enhancement.
PET
18F- FDG PET shows increased uptake in the lesion in active disease and absent uptake in inactive disease.
18F- FDG PET shows increased uptake in the lesion in active disease and absent uptake in inactive disease.
Differentials
Erdheim chester disease.
Extramedullary hematopoesis.
Retroperitoneal lymphoma
Erdheim chester disease.
Extramedullary hematopoesis.
Retroperitoneal lymphoma
Author - Dr sayana