You are in: eMedicine Specialties > Urology > Infections and Related Inflammatory Conditions Fournier GangreneArticle Last Updated: Jan 29, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Thomas Santora, MD, Associate Professor, Director of Regional Resource Trauma Center, Department of Surgery, Temple University Medical Center Thomas Santora is a member of the following medical societies: American Association for the Surgery of Trauma, American College of Surgeons, American Trauma Society, Association for Academic Surgery, and Society for Surgery of the Alimentary Tract Coauthor(s): Daniel B Rukstalis, MD, Chief, Associate Professor, Department of Surgery, Division of Urology, Medical College of Pennsylvania-Hahnemann University Editors: Alex Jacocks, MD, Program Director, Professor, Department of Surgery, University of Oklahoma School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; David L Morris, MD, PhD, Professor, Department of Surgery, St George Hospital, University of New South Wales, Australia; J Stuart Wolf, Jr, MD, FACS, David A Bloom Professor of Urology, Director, Division of Minimally Invasive Urology, Department of Urology, University of Michigan Medical Center; Stephen W Leslie, MD, FACS, Founder and Medical Director of the Lorain Kidney Stone Research Center, Clinical Assistant Professor, Department of Urology, Medical College of Ohio Author and Editor Disclosure Synonyms and related keywords: Fournier gangrene, Fournier's gangrene, idiopathic gangrene of the penis and scrotum, spontaneous fulminant gangrene of the scrotum, necrotizing fasciitis of the scrotum, necrotizing fasciitis of the male genitalia, infectious gangrene of the scrotum and penis, scrotal gangrene, synergistic gangrene of the male genitalia, gangrenous erysipelas of the scrotum, streptococcal gangrene of the scrotum, necrotizing fasciitis INTRODUCTIONSection 2 of 10
  Fournier gangrene is a necrotizing infection that involves the soft tissues of the male genitalia. In modern-day vernacular, Fournier gangrene is a specific form of necrotizing fasciitis (see Image 2), a general term introduced in 1951 by Wilson to describe infection of soft tissue that involves the deep and superficial fascia, regardless of location. Originally, the term Fournier gangrene was used to describe idiopathic gangrene of the genitalia; however, it has also has been used to describe most soft tissue necrotizing infections of the perineum, independent of cause. Modern-day use of the term Fournier gangrene should be restricted to describe infections that primarily involve the genitalia. The indiscriminate use of this eponym makes comparing the results of clinical series or defining a reliable occurrence rate difficult. History of the ProcedureIn 1764, Baurienne originally described an idiopathic, rapidly progressive soft tissue necrotizing gangrene of the male genitalia. However, Jean-Alfred Fournier, a Parisian venereologist who practiced his trade from 1860-1902, is more commonly associated with this disease, which bears his name. A transcript for one of Fournier's clinical lectures in 1883 presents a case of perineal gangrene in an otherwise healthy young man. This paper, written initially in French and translated recently by Alexander Corman, provides historical insight into the practice of medicine at the time. In his presentation, Fournier reviews the systemic and local factors that predispose to this fulminate process. Although Fournier did not emphasize the role of diabetes in this paper, even then diabetes was known as the leading predisposing systemic factor. Local factors related to trauma of the genitalia accounted for the vast majority of the cases of genital gangrene. Fournier describes in anecdotes some of the misconceptions of the times that created this condition, including the practice of nighttime ligation of the prepuce to control enuresis or an attempted birth control technique practiced by an adulterating man to avoid impregnating his married lover. Since Fournier's description, subsequent knowledge has shown that it has an identifiable cause and that it frequently manifests in a more indolent fashion. Trauma to the genitalia continues to be a frequently recognized vector for the introduction of bacteria that initiate the infectious process. FrequencyIn a review of the topic in 1992, Paty and coworkers calculated that approximately 500 cases have been reported in the literature since Fournier's 1883 report, yielding a prevalence of 1 case in 7500 persons. Using MEDLINE and its abstracted journals, other researchers have reported approximately 600 cases of Fournier gangrene in the world literature since 1996. The frequency of the disease has not likely changed appreciably; rather, the apparent increase in the number of cases in the literature most likely results from increased reporting. Fortunately, Fournier gangrene is an uncommon, but not rare, disease. No seasonal variation occurs, and the disease is not indigenous to any region of the world, although the largest clinical series originate from the African continent. EtiologyAlthough originally described as idiopathic gangrene of the genitalia, Fournier gangrene has an identifiable cause in approximately 95% of cases. The necrotizing process commonly originates from an infection in the anorectum, the urogenital tract, or the skin of the genitalia.
Comorbid diseases that compromise the immune system have been implicated as necessary predisposing factors for the development of Fournier gangrene. The following are common predisposing comorbidities:
PathophysiologyThe following are pathognomonic findings upon pathologic evaluation of the involved tissue:
Infection represents an imbalance between (1) host immunity, which is frequently compromised by one or more of the above comorbid systemic processes, and (2) the virulence of the causative microorganisms. The etiologic factors allow the portal for entry of the microorganism into the perineum, the compromised immunity provides a favorable environment to initiate the infection, and the virulence of the microorganism promotes the rapid spread of the disease. Microorganism virulence (see Image 1) results from the production of toxins or enzymes that create an environment conducive to rapid microbial multiplication. In a 1924 series of Chinese men with necrotizing infections, Meleney reported that streptococcal species were the predominant organisms recovered from cultures. Meleney attributed the necrotizing infection to this sole organism; however, subsequent clinical series have emphasized the multiorganism nature of most cases of necrotizing infection, including Fournier gangrene. Presently, recovering only streptococcal species is unusual; rather, streptococcal organisms are cultured along with as many as 5 other organisms. The following are common causative microorganisms:
Most authorities believe the polymicrobial nature of this disease is necessary to create the synergy of enzyme production that promotes rapid multiplication and spread of the infection. For example, one microorganism might produce the enzymes necessary to cause coagulation of the nutrient vessels. Thrombosis of these nutrient vessels reduces local blood supply; thus, tissue oxygen tension falls. The resultant tissue hypoxia allows growth of facultative anaerobes and microaerophilic organisms. These latter microorganisms, in turn, may produce enzymes (eg, lecithinase, collagenase), which lead to digestion of fascial barriers, thus fueling the rapid extension of the infection. The fascial necrosis and digestion are hallmarks of this disease process; this is important to appreciate because it provides the surgeon with a clinical marker of the extent of tissue involvement. Specifically, if the fascial plane can be separated easily from the surrounding tissue by blunt dissection, it is quite likely to be involved with the ischemic-infectious process; therefore, any such dissected tissue should be excised. Far-advanced or fulminate disease can spread from the fascial envelopment of the genitalia throughout the perineum, along the torso, and, occasionally, into the thighs. ClinicalThe hallmark of Fournier gangrene is intense pain and tenderness in the genitalia. The clinical course usually progresses through the following phases:
The systemic effects of this process vary from local tenderness with no toxicity to florid septic shock. In general, the greater the degree of necrosis, the more profound the systemic effects. The typical patient is an elderly male in his sixth or seventh decade of life with comorbid diseases; females are not immune to this disease but are affected much less frequently. The following are pitfalls in the clinical detection of Fournier gangrene (see Image 3):
RELEVANT ANATOMYSection 3 of 10
The complex anatomy (see Image 5) of the male external genitalia influences the initiation and progression of Fournier gangrene. This infectious process involves the superficial and deep fascial planes of the genitalia. As the microorganisms responsible for this infection multiply, infection spreads along the anatomical fascial planes, often sparing the deep muscular structures and, to variable degrees, the overlying skin. This phenomenon has implications for both initial debridement and subsequent reconstruction. Therefore, a working knowledge of the anatomy of the male lower urinary tract and external genitalia is critical for the clinician treating a man with Fournier gangrene. Skin and superficial fascia Because Fournier gangrene is predominately an infectious process of the superficial and deep fascial planes, understanding the anatomic relationship of the skin and subcutaneous structures of the perineum and abdominal wall is important. The skin cephalad to the inguinal ligament is backed by Camper fascia, which is a layer of fat-containing tissue of varying thickness and the superficial vessels to the skin that run through it. Scarpa fascia forms another distinct layer deep to Camper fascia. In the perineum, Scarpa fascia blends into Colles fascia (also known as the superficial perineal fascia), while it is continuous with Dartos fascia of the penis and scrotum. Several important anatomic relationships should be considered. A potential space between the Scarpa fascia and the deep fascia of the anterior wall (external abdominal oblique) allows for the extension of a perineal infection onto the anterior abdominal wall. Superiorly, Scarpa and Camper fascia coalesce and attach to the clavicles, ultimately limiting the cephalad extension of an infection that may have originated in the perineum. Colles fascia is attached to the pubic arch and the base of the perineal membrane, and it is continuous with the superficial Dartos fascia of the scrotal wall. The perineal membrane is also known as the inferior fascia of the urogenital diaphragm and, together with Colles fascia, defines the superficial perineal space. This space contains the membranous urethra, bulbar urethra, and bulbourethral glands. In addition, this space is adjacent to the anterior anal wall and ischiorectal fossae. Infectious disease of the male urethra, bulbourethral glands, perineal structures, or rectum can drain into the superficial perineal space and can extend into the scrotum or onto the anterior abdominal wall up to the level of the clavicles. Vascular supply to the skin of the lower abdomen and genitalia Branches from the inferior epigastric and deep circumflex iliac arteries supply the lower aspect of the anterior abdominal wall. Branches of the external and internal pudendal arteries supply the scrotal wall. With the exception of the internal pudendal artery, each of these vessels travels within Camper fascia and, therefore, can become thrombosed in the progression of Fournier gangrene. If thrombosed, the viability of the skin of the anterior scrotum and perineum is jeopardized. Often, the posterior aspect of the scrotal wall supplied by the internal pudendal artery remains viable and can be used in the reconstruction following resolution of the infection. Penis and scrotum The contents of the scrotum, namely the testicles, epididymides, and cord structures, are invested by several fascial layers distinct from the Dartos fascia of the scrotal wall. Again, several important anatomic relationships should be considered. The most superficial layer of the testis and cord is the external spermatic fascia, which is continuous with the external aponeurosis of the superficial inguinal ring (external abdominal oblique). The next deeper layer is the internal spermatic fascia, which is continuous with the transversalis fascia. A deep fascia termed Buck fascia covers the erectile bodies of the penis, the corpora cavernosa, and the anterior urethra. Buck fascia fuses to the dense tunica albuginea of the corpora cavernosa, deep in the pelvis. The fascial layers described in this section do not become involved with an infection of the superficial perineal space and can limit the depth of tissue destruction in a necrotizing infection of the genitalia. The corpora cavernosa, urethra, testes, and cord structures are usually spared in Fournier gangrene, while the superficial and deep fascia and the skin are destroyed. WORKUPSection 4 of 10
Lab Studies
Imaging Studies
Histologic FindingsThe pathognomonic findings upon pathologic evaluation of the involved tissue include (1) necrosis of the superficial and deep fascial planes, (2) fibrinoid coagulation of the nutrient arterioles, (3) polymorphonuclear cell infiltration, and (4) microorganisms identified within the involved tissues. The characteristic finding that most commonly indicates Fournier disease is fibrinoid thrombosis of the nutrient vessels that supply the superficial and deep fascia. A frequent occurrence is widespread necrosis of the fascia with acute inflammatory cell infiltration, necrotic debris, and frequent demonstration of causative microorganisms within the tissues. This extensive inflammatory process is frequently present deep to intact skin, which is often minimally involved with the inflammatory process until late in the disease. TREATMENTSection 5 of 10
Medical therapyTreatment of Fournier gangrene involves several modalities, including restoration of normal organ perfusion. In patients who present with systemic toxicity manifesting as hypoperfusion or organ failure, aggressive resuscitation to return normal organ perfusion and function must take precedence over diagnostic maneuvers, especially if these diagnostic studies could compromise the resuscitative interventions. Treatment also involves the institution of broad-spectrum antibiotic therapy. The antibiotic spectrum should cover staphylococci, streptococci, the Enterobacteriaceae family of organisms, and anaerobes. A reasonable empiric regimen might consist of ciprofloxacin and clindamycin. Clindamycin is particularly useful in the treatment of necrotizing soft tissue infections due to its gram-positive and anaerobic spectrum of activity. Clindamycin has been shown in animal models of streptococcal infection to have superior response rates compared with penicillin or erythromycin, even if treatment was delayed (Stevens, 1998). If initial tissue stains (ie, KOH stain) show fungi, add an empiric antifungal agent such as amphotericin B or caspofungin. In cases associated with sepsis syndrome, therapy with intravenous immunoglobulin (IVIG), which is thought to neutralize superantigens such as the streptotoxins (A, B) believed to mitigate the exaggerated cytokine response, has been shown to be a good adjuvant to appropriate antibiotic coverage and complete surgical debridement (Cawley, 1999). Hyperbaric oxygen, if available, has shown some promising results. One needs to balance this therapy with the stability of the patient, and surgical therapy must not be delayed. Surgical therapy
COMPLICATIONSSection 6 of 10
The main complication associated with Fournier disease is unresolved sepsis, often caused by one of the following:
OUTCOME AND PROGNOSISSection 7 of 10
To date, all studies are clinical series reviewed retrospectively. Therefore, drawing reliable prognostication information from these studies is scientifically unsound. Given that proviso, survival may be inversely proportional to the age of the patient, the extent of systemic toxicity upon admission, and, possibly, the extent of the local tissue involvement. In some studies, Fournier disease that originates from diseases of the anorectum produces poorer outcomes compared with other causes. In the 600 cases of Fournier gangrene discovered during a MEDLINE search dating back to 1996, 100 deaths occurred (16.5%). In the series that included more than 20 patients, the mortality rate ranged from 4-54%, with most studies reporting mortality rates of 20-30%. The prognosis following reconstruction is usually good. Approximately 50% of men with penile involvement have pain with arousal. This pain is often related to limited mobility of the genitalia because of scarring. Consultation with a psychiatrist may be beneficial for some patients in order to deal with the emotional stress of a different body image. If extensive soft tissue is lost, lymphatic drainage may be impaired; thus, edema and cellulitis may result. Use of external support ware may be beneficial to minimize this postoperative problem. FUTURE AND CONTROVERSIESSection 8 of 10
The role of hyperbaric oxygen therapy needs to be clarified with a prospective controlled trial. The role of topical agents in wound care also requires further investigation. Although reports from Africa extol the beneficial chemical effects of unprocessed honey, the salutatory effect of honey likely is related to its physical property of hyperosmolarity. Therefore, honey holds little advantage over other hygroscopic agents. The application of growth hormones and other trophic agents holds the potential to promote more rapid wound healing. The use of vacuum dressing technologies to hasten the wound closure has only recently been used to treat these wounds. MULTIMEDIASection 9 of 10
REFERENCESSection 10 of 10
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