Presentation
Children may present with hip or gluteal pain. [patient.info]
Cytomorphology of Erdheim-Chester disease presenting as a retroperitoneal soft tissue lesion. [cytojournal.com]
His comorbid history included ischemic heart disease, with a myocardial infarction 11 years before this presentation. [acgcasereports.gi.org]
Severe abdominal pain may also present due to hemorrhage brought about by the death of intestinal tissues. Men may experience testicular swelling while women may present with endometriosis secondary to anatomic distortion. [allhealthsite.com]
Diagnosis RPF is a diagnosis of exclusion, depending on the presenting complaint and examination to which investigations are conducted. The non-specific nature often results in late diagnosis when disease is advanced. [fastbleep.com]
Gastrointestinal
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Abdominal Pain
A patient has been referred for abdominal sonography with abdominal pain and increased serum amylase. You detect a fluid pocket in the left pararenal space. [quizlet.com]
The patient may also experience nausea, vomiting, confusion and reduced consciousness in the presence of kidney failure Inflammation of veins and deep vein thrombosis Severe abdominal pain with bleeding Other symptoms like fever, weight loss, malaise, [medindia.net]
Later symptoms include: decreased urine output, no urine output (anuria), nausea, vomiting, changes in thinking caused by kidney failure and build-up of toxic chemicals in the blood, and severe abdominal pain with hemorrhaging (due to death of intestinal [aarda.org]
Symptoms are malaise, fever, and abdominal pain. Diagnosis is by CT. Treatment is with drainage, either surgical or percutaneous. Antibiotics are ancillary. [merckmanuals.com]
pain with hemorrhaging (due to death of intestinal tissue) Abdominal CT scan is the best way to find a retroperitoneal mass. [medlineplus.gov]
Workup
See Workup for more detail. [emedicine.medscape.com]
X-Ray
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X-Ray Abnormal
Abscesses near the diaphragm may result in chest x-ray abnormalities such as ipsilateral pleural effusion, elevated or immobile hemidiaphragm, lower lobe infiltrates, and atelectasis. CBC and blood cultures should be done. [merckmanuals.com]
Treatment
Improvement of both diseases after treatment with steroids and immunosuppresive agents. Nephrol Dial Transplant. 1999;14:1303-05. Grotz W, et al. Treatment of retroperitoneal fibrosis by mycophenolate mofetil and corticosteroids. [rarediseases.org]
Historically, treatment has focused on relieving ureteral obstruction, using stents followed by open or laparoscopic ureterolysis to relocate the ureter. [broadcastmed.com]
Treatment for retroperitoneal fibrosis The treatment used for RPF depends on the underlying cause, the severity of the condition and the degree of damage caused by any complications. [betterhealth.vic.gov.au]
Treatment Options For Retroperitoneal Fibrosis Treatment of retroperitoneal fibrosis will depend whether the cause is known or whether it is idiopathic. The modality of treatment is medication and surgery. [tandurust.com]
Biopsy should also be done if the location of fibrosis is atypical or if there is an inadequate response to initial treatment. [5] Treatment [ edit ] In the absence of severe urinary tract obstruction (which generally requires surgery with omental wrapping [en.wikipedia.org]
Prognosis
Prognosis Overall the prognosis is positive in non-malignant RPF, however it varies depending on the location of the obstruction, the degree of renal insufficiency, and the establishment of appropriate management to prevent renal failure. [fastbleep.com]
[…] has a poor prognosis. [patient.info]
Also, those with non-syndromic (sporadic) tumors have a comparatively better prognosis than individuals with tumors that are seen in association with a genetic disorder (syndromic) In most cases, the prognosis of small-sized solitary tumors is excellent [dovemed.com]
Prognosis The prognosis for retroperitoneal fibrosis would depend on the extent of the fibrosis as well as the invasion and damage to other surrounding organs especially to the kidneys. [allhealthsite.com]
What is the Prognosis of a Patient suffering from Retroperitoneal Fibrosis? Prognosis or outcome of retroperitoneal fibrosis depends on the extent of kidney damage. [medindia.net]
Etiology
Primary treatment is directed at assumed autoimmune and inflammatory etiologies of RPF, initially with a prolonged course of corticosteroids. [bradyurology.blogspot.com]
Displacement of the aorta and IVC anteriorly away from the vertebral bodies is suggestive of malignant etiology. Invasion and disruption of bone and/or soft tissue structures suggests an aggressive process - infection or malignancy. [radiopaedia.org]
Most frequent isolates are Intra-Abdominal Abscesses Location Etiology Organisms Intraperitoneal Subphrenic Right or left lower quadrant Interloop Paracolic Pelvic Postoperative; perforation of hollow viscus, appendicitis, diverticulitis, or tumor; Crohn [merckmanuals.com]
Although etiology is unknown, immunodeficiency, aberrant autoimmunity and chronic inflammation may contribute to CD pathophysiology. [academic.oup.com]
Epidemiology
Epidemiology Retroperitoneal fibrosis is an uncommon disease that affects 1 person per 200,000-500,000 population per year, depending on the specific location. [news-medical.net]
Epidemiology Retroperitoneal fibrosis is rare disease. Idiopathic retroperitoneal fibrosis accounts for over 70% of cases. [patient.info]
Epidemiology and Etiology In large, cross-sectional studies RPF is a rare disease, presenting in only 0.1 to 1 per 100,000 to 200,000 people.[ 1, 2 ] It is more common in men than women (ratio 2-3:1) and typically presents later in life - in the 6 th [bradyurology.blogspot.com]
Because of the rarity of this disease, epidemiological data like prevalence or incidence are not available. The average age of patients with unicentric Castleman’s disease is around 30 to 40 years old. [jmedicalcasereports.biomedcentral.com]
References 1. de Pontual L, Ovetchkine P, Rodriguez D, Grant A, Puel A, et al. (2008) Rhinoscleroma: a French national retrospective study of epidemiological and clinical features. Clin Infect Dis 47: 1396–1402. View Article Google Scholar 2. [journals.plos.org]
Pathophysiology
Although etiology is unknown, immunodeficiency, aberrant autoimmunity and chronic inflammation may contribute to CD pathophysiology. [academic.oup.com]
Therefore, it remains to be solved whether, as seen in our patient, minimal change nephrotic syndrome is pathophysiologically related with or a casual finding of IRF. [kjim.org]
View Article PubMed Google Scholar Casper C: The aetiology and management of Castleman disease at 50 years: translating pathophysiology to patient care. Br J Haematol. 2005, 129: 3-17. 10.1111/j.1365-2141.2004.05311.x. [jmedicalcasereports.biomedcentral.com]
Pathophysiology Anatomy of the retroperitoneum The retroperitoneal space is bordered anteriorly by the posterior parietal peritoneum, posteriorly by the transversalis fascia, and superiorly by the diaphragm. [emedicine.medscape.com]
[…] unknown about the behavior of the IgG4 molecule in vivo, the pathways through which this immunoglobulin participates in disease, and whether or not the role of IgG4 is primary or secondary. 20, 21, 22 In time, discoveries pertaining to the etiology and pathophysiology [nature.com]
Prevention
Corticosteroids are also used supportively to prevent nausea. Also preventing nausea as one of the symptoms of retroperitoneal fibrosis. Tamoxifen Tamoxifen is a drug given to block the effects of the hormone estrogen. [ehealthwall.com]
Since the majority of cases cannot be linked to any specific cause, prevention may not be possible. [healthline.com]
Other complications like chronic kidney failure and chronic obstructive uropathy may be prevented if retroperitoneal fibrosis is diagnosed and treated early. References: [allhealthsite.com]
Surgery Surgery is used in retroperitoneal fibrosis to separate the ureters from the obstructing mass and cover them with fatty tissue from the abdomen called omentum to prevent further obstruction. [medindia.net]
Surgical ureterolysis can obviously relieve the obstruction of ureter, and can prevent the further deterioration of renal function, but it cannot prevent the progression of the primary disease [ 17 ]. [omicsonline.org]