Fournier gangrene is a rare form of infection involving the perineal and genital area. It is more commonly encountered in men, and people affected by immunosuppressive conditions (diabetes mellitus, human immunodeficiency virus (HIV) infection, etc). A sudden onset of fever with severe pain and swelling of the scrotal or perineal area, with the skin exhibiting purulent discharge and redness. In the absence of an early diagnosis, Fournier gangrene can be fatal. A comprehensive clinical assessment followed by appropriate laboratory and imaging studies are crucial steps of the workup.
Presentation
The first signs of Fournier gangrene (also known as cutaneous necrotizing fasciitis) stem from the invasion of bacterial microorganisms from other sources into the scrotal or perineal areas [1] [2] [3]. The lower gastrointestinal system (the colon and the rectum), the urogenital system, and the skin are listed as the most important sources of bacterial spread, with perianal abscesses, appendicitis, infection of the Bartholin's gland, coital injury, and episiotomy-induced wound infections being the main portals of entry [2] [3]. The most common pathogens are Escherichia coli, Klebsiella pneumoniae, Staphylococcus aureus, and Bacteroides fragilis; many studies, however, highlight the polymicrobial nature of Fournier gangrene [1] [4]. The clinical presentation is distinguished by an abrupt onset of fever (often > 38°C) accompanied by severe pain in the scrotal, perineal, or perianal region [1] [2] [3] [5]. Marked localized swelling is observed in most cases and frequently produces a purulent discharge with a foul odor [1] [2] [3] [5]. Crepitus is also observed in the majority of patients [1] [2] [3]. Fournier gangrene poses a significant risk to the individual's life due to the rapid spread of infection into distant tissues, leading to sepsis and systemic signs of tachycardia and multiorgan failure [1] [2] [3]. Mortality rates have been established to be as high as 20-30% [6], meaning that an early diagnosis is of essential importance.
Entire Body System
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Swelling
The swelling of subcutaneous plane involved the flank regions, mainly on the left side. The investigations were performed (full blood count, blood urea, electrolytes, coagulation profile). [ncbi.nlm.nih.gov]
Marked localized swelling is observed in most cases and frequently produces a purulent discharge with a foul odor. Crepitus is also observed in the majority of patients. [symptoma.com]
A 46-year-old man with uncontrolled type 2 diabetes and alcoholic liver disease presented to the emergency department with painful swelling in the scrotum and perianal region. [nejm.org]
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Lymphedema
Oanna Meyer Ganz, Raphaël Gumener, Pascal Gervaz, Julien Schwartz and Brigitte Pittet-Cuénod, Management of unusual genital lymphedema complication after Fournier’s gangrene: a case report, BMC Surgery, 12, 1, (2012). [doi.org]
Gastrointestinal
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Rectal Mass
Following acute management a thorough examination including digital rectal examination was performed which detected an extensive rectal mass with extension of necrotic points to the anal area. [casesjournal.biomedcentral.com]
Cardiovascular
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Cardiomegaly
In addition there was morbid obesity (body mass index 46.9), cirrhosis of the liver, and marked focal coronary artery atherosclerosis with significant cardiomegaly. [ncbi.nlm.nih.gov]
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Chest Pain
The patient deteriorated clinically 8-11 days postoperatively with delirium, chest pain and severe hypertension followed by hypotension and thrombocytopaenia. Abdominal CT scan revealed bilateral adrenal haemorrhage. [ncbi.nlm.nih.gov]
Musculoskeletal
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Low Back Pain
CASE: A 30-year old primigravid woman in the third trimester of pregnancy presented with a history of low back pain. Subsequently, a diagnosis of perianal sepsis and associated Fournier gangrene was established. [ncbi.nlm.nih.gov]
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Back Pain
CASE: A 30-year old primigravid woman in the third trimester of pregnancy presented with a history of low back pain. Subsequently, a diagnosis of perianal sepsis and associated Fournier gangrene was established. [ncbi.nlm.nih.gov]
Skin
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Erythema
Physical examination revealed edema (91.4%), erythema (88.6%) and perineal skin necrosis (60%). All the patients underwent surgical debridement of necrotic tissue. In 54.3% reoperations were necessary for new surgical debridements. [ncbi.nlm.nih.gov]
Examination of the patient's perineum was consistent with Fournier's gangrene and included significant erythema, edema, and calor of the penis and scrotum with a large malodorous eschar. [hindawi.com]
Fournier's gangrene: A horrendous infection of the genitalia that causes severe pain in the genital area (in the penis and scrotum or perineum) and progresses from erythema (redness) to necrosis (death) of tissue. Gangrene can occur within hours. [medicinenet.com]
[…] promote anaerobes and cause rapid dissemination Obliterative endarteritis plays a key role in pathogenesis Diagrams / tables Images hosted on other servers: Fournier gangrene severity index Clinical features Patients present with genital induration, pain, erythema [pathologyoutlines.com]
Workup
The diagnosis of Fournier gangrene rests on a thorough patient history and a detailed physical examination [2] [3]. During the interview, the physician should obtain information regarding the onset of symptoms and their progression, whereas assessment of risk factors is crucial for determining the risk of complications. Diabetes mellitus, HIV infection, and alcohol abuse are some of the principal comorbidities that significantly elevate the risk for the development of Fournier gangrene, meaning that their presence should be taken into consideration [1]. Furthermore, recent instrumentation (such as catheterization, urogenital or colonic biopsy, gynecological procedures, genital piercing, trauma, recent delivery or septic abortion) have also been implicated in the pathogenesis [1] [2] [3]. A full examination of the genital and perineal areas can reveal the typical features of an advanced infection and is perhaps the crucial component of the workup. Although Fournier gangrene is primarily a clinical diagnosis, various laboratory and imaging studies may be employed. A complete blood count (showing anemia, thrombocytopenia, and leukocytosis), glucose abnormalities (both hyperglycemia and hypoglycemia can be detected), impaired kidney function parameters (creatinine and urea), as well as hypoalbuminemia are nonspecific findings, whereas arterial blood gases (ABGs), a full coagulation panel, serum electrolytes (sodium and potassium) and samples for microbiological investigations (mainly cultures) should be obtained as well [1] [3] [5]. Radiography, ultrasonography, and computed tomography (CT) might be useful for evaluating the abdomen and the perineal area [1] [2] [3] [5].
Serum
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Decreased Platelet Count
Increased age, body mass index, and white blood cell count, and decreased platelet count were all associated with an increased risk of 30-day mortality. Copyright 2015 Elsevier Inc. All rights reserved. [ncbi.nlm.nih.gov]
Microbiology
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Blood Culture Positive
At autopsy, the most striking finding was deep necrotic ulceration of the scrotum with exposure of underlying deep muscles and testicles, with blood cultures positive for Escherichia coli. [ncbi.nlm.nih.gov]
Treatment
Treatment was significantly longer in patients with a comorbidity, such as diabetes or liver cirrhosis due to alcohol abuse. CONCLUSION: Early diagnosis as well as intensive and aggressive treatment are key to successful treatment of FG. [ncbi.nlm.nih.gov]
Prognosis
The aim of this study was to analyze the prognosis and treatment effectiveness of this fulminant infectious disease. Forty-one patients were admitted to our hospital with the diagnosis of Fournier gangrene between January 1998 and December 2006. [ncbi.nlm.nih.gov]
It is considered a urological emergency with a poor prognosis due to its high mortality rate (ranging 15-50% 3 ). [radiopaedia.org]
Etiology
Although there were no etiological factors in 25 patients (71%), various etiological factors were found in 10 patients (29%). Multiple debridements were performed in the majority of the cases. The overall mortality rate was 40%. [ncbi.nlm.nih.gov]
Epidemiology
Because to our knowledge there are no population based data, we used a national database to investigate the epidemiology of Fournier's gangrene. [dx.doi.org]
The aim of this study was to analyze the epidemiological and clinical characteristics of Fournier gangrene along with the variables that influence disease course and mortality in patients treated in our department. [ncbi.nlm.nih.gov]
Pathophysiology
An overview is given for each, including causes, pathophysiology, diagnosis, treatment, and new developments. The focus for priapism is on diagnosis and distinguishing high-flow from low-flow forms, as the latter requires emergent treatment. [ncbi.nlm.nih.gov]
Abstract Despite advances in the evaluation, treatment, and pathophysiological understanding of necrotizing soft-tissue infections, Fournier's gangrene remains a life-threatening urological emergency. [nature.com]
Pathophysiology Localized infection adjacent to a portal of entry is the inciting event in the development of Fournier gangrene. [emedicine.medscape.com]
Prevention
Prevention and early diagnosis are essential. Prompt aggressive intervention is warranted to maximize outcomes. [ncbi.nlm.nih.gov]
Can You Prevent It? There are some steps you can take to lower your chances of getting Fournier’s gangrene: If you have diabetes, check your genitals and surrounding areas for wounds or signs of infection, as well as for swelling or drainage. [webmd.com]
Women who have had a pus-producing bacterial infection (abscess) in the vaginal area, a surgical incision in the vagina and perineum to prevent tearing of skin during delivery of a child (episiotomy), an abortion resulting in fever and an infection of [healthcommunities.com]
References
- Thwaini A, Khan A, Malik A, et al. Fournier’s gangrene and its emergency management. Postgrad Med J. 2006;82(970):516-519.
- Chennamsetty A, Khourdaji I, Burks F, Killinger KA. Contemporary diagnosis and management of Fournier’s gangrene. Ther Adv Urol. 2015;7(4):203-215.
- Mallikarjuna MN, Vijayakumar A, Patil VS, Shivswamy BS. Fournier’s Gangrene: Current Practices. ISRN Surg. 2012;2012:942437.
- Eke N. Fournier's gangrene: a review of 1726 cases. Br J Surg. 2000;87(6):718-728.
- Chen SY, Fu JP, Wang CH, Lee TP, Chen SG. Fournier gangrene: a review of 41 patients and strategies for reconstruction. Ann Plast Surg. 2010;64(6):765-769.
- Singh A, Ahmed K, Aydin A, Khan MS, Dasgupta P. Fournier's gangrene. A clinical review. Arch Ital Urol Androl. 2016;88(3):157-164.