Retroperitoneal fibrosis is a proliferative process of inflammation and fibrosis occurring in elderly men that may encase affected structures, with the ureters frequently exhibiting medial deviation on intravenous pyelography. Rpf may be primary idiopathic most common or secondary e. Pathology outlines idiopathic retroperitoneal fibrosis of colon. This abnormal tissue growth often spreads to affect the tubes that carry urine from the kidney to the bladder ureters. Retroperitoneal fibrosis and the spectrum of chronic. Early on, the individual may experience dull pain in the abdomen that progresses, swelling of one. Retroperitoneal fibrosis on intravenous pyelogram radiology case. Retroperitoneal fibrosis can occur at any age but appears most frequently between the ages of 40 and. It may present with lower back pain, kidney failure, hypertension, deep vein thrombosis, and other obstructive. Retroperitoneal fibrosis rpf is characterized by the presence of an aberrant fibroinflammatory retroperitoneal tissue that usually develops around the infrarenal portion of the abdominal aorta and the iliac arteries.
Sign up for an individual subscription to the oxford textbook of nephrology. It can block the tubes that carry urine from the kidney to the bladder. Colon tumor idiopathic retroperitoneal fibrosis of colon. Jun 23, 2014 retroperitoneal fibrosis is thought to be an autoimmune response to an insoluble lipid that has leaked through a thinned arterial wall from atheromatous plaques. The mass can affect the flow of urine to the kidneys and may also affect other abdominal organs.
Our family of members encourages all patients, caregivers, family, friends, and interested medical professionals to join. Retroperitoneal fibrosis rpf is the abnormal growth of white and woody tissue on and around the structures of the abdomen that are outside the peritoneum. Retroperitoneal fibrosis is a rare inflammatory disorder causing increased fibrotic deposition in the retroperitoneum, often leading to ureteral obstruction. Retroperitoneal fibrosis rpf is an uncommon disease characterized by a fibrous reaction that takes place in the periaortic retroperitoneum and often entraps the ureters causing obstructive uropathy. Retroperitoneal fibrosis or ormonds disease is a disease featuring the proliferation of fibrous tissue in the retroperitoneum, the compartment of the body containing the kidneys, aorta, renal tract, and various other structures. Causes retroperitoneal fibrosis occurs when extra fibrous tissue forms in. Retroperitoneal fibrosis commonly happens within the fibrous tissue. It is an uncommon fibrotic reaction in the retroperitoneum. Retroperitoneal fibrosis is a rare disorder that blocks the tubes ureters that carry urine from the kidneys to the bladder. Patient is a 40yearold african american male with no significant past medical history who presented with a fourmonth history of low back.
Retroperitoneal fibrosis radiology linkedin slideshare. Combined prednisone and mycophenolate mofetil appears to be an effective therapeutic option for patients with retroperitoneal fibrosis. Retroperitoneal fibrosis is a rare condition that occurs when the ureters tubes that carry urine from the kidneys to the bladder become blocked due to a mass located behind the stomach and intestines. This patient, known to have retroperitoneal fibrosis, has medial deviation of the ureters on ivp. Retroperitoneal fibrosis penn state hershey medical center. Retroperitoneal fibrosis rpf is a rare disease where inflammatory scar tissue in the back of the abdominal cavity wraps itself around blood vessels, the ureter and nerves. Though a standard procedure for treating this disorder has not been determined, medication and surgery are two viable options. Fibromatosis, mesenteric, retroperitoneal and pelvic retroperitoneal fibrosis. Retroperitoneal fibrosis genetics home reference nih.
Sep 01, 2017 retroperitoneal fibrosis rpf is a condition that occurs when extensive fibrosis develops in the retroperitoneum, usually centred over the anterior aspect of the fourth and fifth lumbar vertebrae. Retroperitoneal fibrosis rpf is a rare condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space. Retroperitoneal fibrosis rpf comprises a spectrum of rare diseases hallmarked by the presence of an aberrant fibroinflammatory tissue that usually develops around the infrarenal portion of the abdominal aorta and the iliac arteries and frequently entraps neighbouring structures such as the ureters and the inferior vena cava. This process may spread contiguously and involve adjacent. Retroperitoneal fibrosis is a rare inflammatory disorder in which abnormal formation of fiberlike tissue fibrosis occurs behind the membrane that lines the cavity of the abdomen peritoneum. We report a case of a 40yearold male with an incidental finding of retroperitoneal fibrosis. Retroperitoneal fibrosis knowledge for medical students.
Retroperitoneal fibrosis causes, treatment, diagnosis. The retroperitoneal space retroperitoneum is the anatomical space sometimes a potential space in the abdominal cavity behind retro the peritoneum. The description of two cases by ormond in 1948 established retroperitoneal fibrosis as a clinical entity. Management of idiopathic retroperitoneal fibrosis from the. Retroperitoneal fibrosis radiology reference article.
Retroperitoneal fibrosis definition of retroperitoneal. Retroperitoneal fibrosis also called as ormonds disease named after john kelso ormond who rediscovered the condition in 1948. Retroperitoneal fibrosis on intravenous pyelogram radiology. This condition develops if tissue overgrowth blocks the ureters, the tubes that transport urine from the kidneys to the bladder. Since 7yeasrs my parents and inlaws are going to dr. Retroperitoneal fibrosis multimedia encyclopedia health. Oct 08, 2019 retroperitoneal fibrosis rpf is characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centered over the anterior surface of the fourth and fifth lumbar vertebrae and resulting in entrapment and obstruction of retroperitoneal structures, notably the ureters. Retroperitoneal fibrosis knowledge for medical students and. In 70% of patients with retroperitoneal fibrosis, the etiology of ormonds disease is unclear.
Retroperitoneal fibrosis is a rare cause of acute renal failure arf with only a handful of cases reported in literature. Structures that are not suspended by mesentery in the abdominal cavity and that lie between the parietal. Retroperitoneal fibrosis rpf is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. Retroperitoneal trauma the space in the abdominal cavity behind the membrane that makes the lining of the cavity.
Causes etiology of retroperitoneal fibrosis unknown etiology. Mediastinal widening may result from a softtissue mass associated with mediastinal fibrosis. Liang cheng, in urologic surgical pathology fourth edition, 2020. It may extend from the renal pedicle to below the pelvic brim. Retroperitoneal fibrosis rpf is a rare condition of variable etiology characterized by the presence of an aberrant fibroinflammatory tissue in the periaortic retroperitoneum that frequently entraps neighboring structures such as the ureters, causing obstructive uropathy and renal failure. It has no specific delineating anatomical structures. This can cause a range of problems including back pain, kidney blockage or renal failure. Introduction retroperitoneal fibrosis rpf is a condition characterized by inflammation and fibrosis surrounding the infrarenal aorta and other retroperitoneal structures 1. Anatomically, inflammation and fibrosis start surrounding the large artery, called the aorta, that. Retroperitoneal fibrosis is a disorder in which inflammation and extensive scar tissue fibrosis occur in the back of the abdominal cavity, behind retro the membrane that surrounds the organs of the digestive system the peritoneum. Retroperitoneal fibrosis rpf is a rare disease, hallmarked by inflammation.
The retroperitoneal mass extends in craniocaudal direction from the renal hilum to the sacral promontory. Retroperitoneal fibrosis radiology reference article radiopaedia. Retroperitoneal fibrosis rpf is a rare but multifaceted disease which. Retroperitoneal fibrosis occurs when extra fibrous tissue forms in the area behind the stomach and intestines. An awardwinning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, gi, cardiac and musculoskeletal diseases. Retroperitoneal fibrosis an overview sciencedirect topics. Rpf is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic. Retroperitoneal fibrosis rpf is a condition characterized by inflammation and fibrosis surrounding the infrarenal aorta and other retroperitoneal structures. Cosgrove, in clinical ultrasound third edition, 2011. Organs are retroperitoneal if they have peritoneum on their anterior side only. Retroperitoneal fibrosis is a rare collagen vascular disorder of unclear cause. Uncommon causes of secondary retroperitoneal fibrosis include trauma and major abdominal surgery 4. Symptoms of such are deep vein thrombosis, renal failure, hypertension and the most common is the lower back pain. Pain, often accompanied by significant weight loss, constipation, and constitutional symptoms, is a common presenting symptom.
Seventeen patients with idiopathic retroperitoneal fibrosis and ureteral entrapment followed in our unit for at least 1 year were selected for this study. One assumed mechanism of disease is an autoimmune process with periaortitis as the initial pathophysiological. Historically, treatment has focused on relieving the patients ureteral obstruction with double j stents or percutaneous nephrostomy tubes followed by more definitive resolution. Tamoxifen monotherapy in the treatment of retroperitoneal fibrosis. Retroperitoneal fibrosis is a severe disease that affects the ureters, causing renal insufficiency in threequarters of patients. The optimal treatment is far from being established. Longterm outcome of idiopathic retroperitoneal fibrosis.
Idiopathic and secondary forms of retroperitoneal fibrosis. Retroperitoneal fibrosis world support group offers both a warm and comforting welcome and an unrivaled learning experience to build selfawareness of this rare condition. Retroperitoneal fibrosis rf is a clinical entity characterized by the progressive proliferation of connective tissue that rarely forms a mass. Fibrous tissue covers the retroperitoneal structures such as the aorta, vena cava, ureters and psoas muscle. Retroperitoneal fibrosis rpf, is a condition that has previously been described as chronic periaortitis. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Retroperitoneal fibrosis rpf is an uncommon disease that is characterized by. Historically, treatment has focused on relieving the patients ureteral obstruction with double j stents or percutaneous nephrostomy tubes followed by more definitive resolution by surgical ureterolysis. The obstruction hampers the ureters ability to transport urine from the kidneys to the bladder. Retroperitoneal fibrosis is a rare condition caused by overgrowth of tissue behind the stomach and intestines.
Retroperitoneal fibrosis is a proliferation of fibrous tissue generally confined to the central and paravertebral regions of the retroperitoneum in the perirenal space between the renal hila and the dome of the bladder. Retroperitoneal fibrosis is an uncommon fibrotic reaction that is thought to have been first described by the french urologist albarran in 1905 as ureteral obstruction secondary to fibrotic changes in the retroperitoneal space. Retroperitoneal fibrosis rpf is a rare chronic wooden fibroinflammatory process surrounding the infrarenal part of abdominal aorta and iliac vessels with frequent encasement of ureters hence the arising obstructive uronephropathy and, in addition, the inferior vena cava 1, 2. Retroperitoneal fibrosis treatment, retroperitoneal. Retroperitoneal fibrosis is a rare condition that occurs when the tubes that carry urine from the kidneys to the bladder become blocked due to a mass. Retroperitoneal fibrosis idiopathic retroperitoneal fibrosis. Retroperitoneal fibrosis genetic and rare diseases. Retroperitoneal fibrosis most often manifests as a paraspinal, welldemarcated but irregular retroperitoneal mass that is isodense to surrounding muscle figs. The fibrotic tissue typically surrounds the infrarenal aorta, inferior vena cava and iliac vessels. The tissue forms a mass or masses that can block the tubes that carry urine from the kidney to the bladder. Retroperitoneal fibrosis is a slowly progressive disorder in which the tubes.
Retroperitoneal fibrosis is associated with cancer in approximately eight percent of cases, but up to 70 percent of cases are idiopathic and have no known. In up to 15% of patients, rf is associated with other fibrotic processes like riedels thyroiditis, fibrous pseudotumour of the orbit, sclerosing cholangitis and mediastinal fibrosis 22, 23. The disease is part of a spectrum of entities that have a common pathogenic process consisting of an inflammatory response to. Patient is a 40yearold african american male with no significant past medical history who presented with a. Retroperitoneal fibrosis is a rare condition in which the ureters tubes that carry urine from the kidneys to the bladder are obstructed by a mass located behind the stomach and intestines. Introduction retroperitoneal fibrosis rpf encompasses a range of diseases characterized by proliferation of aberrant fibroinflammatory tissue, which usually surrounds the infrarenal portion of the abdominal aorta, inferior vena cava, and iliac vessels.
Retroperitoneal and pelvic fibromatosis stanford university. Retroperitoneal fibrosis is a rare autoimmune related disorder that blocks the tubes ureters that carry urine from the kidneys to the bladder. Retroperitoneal fibrosis causes, treatment, diagnosis, symptoms. The disorder may cause pain in the abdomen that worsens with time, pain or swelling of the. Pdf retroperitoneal fibrosis in young female researchgate.
It is an uncommon fibrotic reaction in the retroperitoneum that typically presents with ureteric obstruction. It occurs when excess fibrous tissue develops in the space behind your stomach and intestine called the. Jun 20, 2016 retroperitoneal fibrosis is a slowly progressive disorder in which the tubes that carry urine from the kidneys to the bladder ureters and other abdominal organs or vessels become blocked by a fibrous mass and inflammation in the back of the abdomen. Ormonds disease or secondary retroperitoneal fibrosis. Retroperitoneal fibrosis is a rare condition thats also known as ormonds disease. The disease process typically begins with clinical symptoms of flank pain, and unexplained weight loss. Causes retroperitoneal fibrosis occurs when extra fibrous tissue forms in the area behind the stomach and intestines. Retroperitoneal fibrosis rpf is a rare chronic wooden fibroinflammatory process surrounding the infrarenal part of abdominal aorta and iliac vessels with frequent encasement of ureters hence. Idiopathic retroperitoneal fibrosis irf is a rare disease characterized by a fibrotic reaction that affects retroperitoneal organs, especially the urinary tract. Pathology outlines idiopathic retroperitoneal fibrosis.
Both benign and malignant associations have been described, rendering differentiation of these entities of paramount importance because sinister pathology alters the diagnosis. Retroperitoneal fibrosis is a slowly progressive disorder in which the tubes that carry urine from the kidneys to the bladder ureters and other abdominal organs or vessels become blocked by a fibrous mass and inflammation in the back of the abdomen. The symptoms and signs associated with retroperitoneal fibrosis are nonspecific. Retroperitoneal fibrosis rpf, ormonds disease is a rare disease of unknown etiology, characterized by inflammation and fibrosis of the retroperitoneum resulting in compression and encasement of the ureter, andor the retroperitoneal blood vessels. The disorder may cause pain in the abdomen that worsens with time, pain or swelling of the legs, decreased urine output, and swelling of the scrotum in men. Intravenous urography was once the modality of choice for the. Initial fibrosis tends to begin near the aorta and the iliac arteries, extending through the retroperitoneum to involve the ureters. Retroperitoneal fibrosis symptoms, diagnosis, treatments. Retroperitoneal fibrosis world support nord national. Retroperitoneal fibrosis rpf is a rare condition of variable etiology characterized by the presence of an aberrant fibroinflammatory tissue in the periaortic retroperitoneum that frequently entraps neighboring structures such as.
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