Fournier Gangrene

Disease Overview

Fournier gangrene is an acute necrotic infection of the scrotum; penis; or perineum. It is characterized by scrotum pain and redness with rapid progression to gangrene and sloughing of tissue. Fournier gangrene is usually secondary to perirectal or periurethral infections associated with local trauma, operative procedures, or urinary tract disease.

Since 1950, more than 1,800 cases for study have been reported in English language medical literature. This disease occurs worldwide and, although it is recognized more frequently among male adults, has been identified also among women and children. Treatment usually consists of the surgical removal (debridement) of extensive areas of dead tissue (necrosis, necrotic) and the administration of broad-spectrum intravenous antibiotics. Surgical reconstruction may follow where necessary.

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Synonyms

• Fournier disease
• Fournier's disease
• Fourniers disease
• Fournier's gangrene
• Fourniers gangrene
• gangrene, Fournier
• gangrene, Fournier's
• necrotizing fasciitis of the perineum and genitalia
• synergistic necrotizing fasciitis of the perineum and genitalia

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Signs & Symptoms

Symptoms include fever, general discomfort (malaise), moderate to severe pain and swelling in the genital and anal areas (perineal) followed by rankness and smell of the affected tissues (fetid suppuration) leading to full blown (fulminating) gangrene. Rubbing the affected area yields the distinct sounds (crepitus) of gas in the wound and of tissues moving against one another (palpable crepitus). In severe cases, the death of tissue can extend to parts of the thighs, through the abdominal wall and up to the chest wall.

This disease is commonly found in conjunction with other disorders (comorbidity), especially those that weaken the immune system. Some disorders that increase the predisposition to Fournier gangrene are diabetes mellitus, profound obesity, cirrhosis, interference with the blood supply to the pelvis, and various malignancies.

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Causes

Portals of entry for the bacteria, fungi, and/or viruses responsible for a particular case of Fournier gangrene are generally colorectal, urogenital or cutaneous in origin. Anorectal abscesses, urinary tract infections, surgical instrumentation and other contributing factors have all been implicated. Some cases continue to be of unknown cause (idiopathic). Why this process occasionally develops in individuals with common ailments is still not understood.

There are many ways for the virulent microorganism to gain access to the host, where the compromised immunological system is unable to prevent the infection from taking hold. The virulence of the resulting disorder is thought to be enhanced by the toxins and enzymes produced by the combination of microorganisms (synergy).

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Affected populations

The mean age of presentation is about 50 years, but the range of patient ages in reported cases is from eight days to 90 years. Fournier gangrene is diagnosed more frequently among males. It may be that the high male to female ratio in the diagnosis is the result of the lack of recognition of this entity among women by physicians. It is believed that the male to female proportion may be anywhere from 5:1 to 10:1.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Fournier gangrene. Comparisons may be useful for a differential diagnosis.

Epididymitis is inflammation of the long, tightly coiled tube behind each testicle (epididymis) that carries sperm from the testicle to the spermatic duct. Affected individuals usually have painful swelling of the one epididymitis and the associated testicle. In some cases, the second testicle may also be tender. In addition, affected individuals have fever, painful swelling and redness (erythema) of the scrotum, and/or inflammation of the tube from which urine is carried from the bladder (urethritis). The two main forms of epididymitis are the sexually-transmitted form and the nonspecific bacterial form. (For more information on this disorder, choose “Epididymitis” as your search term in the Rare Disease Database.)

Gas gangrene is a severe form of tissue death usually caused by bacteria that do not need oxygen (anaerobes) to survive, such as Clostridium perfringens. It can also result from infections caused by Group A Streptococcus bacteria and Staphlococcus aureus and Vibrio vulnificus. Clostridium bacteria in an environment of low oxygen concentration produce toxins that cause tissue death and associated symptoms. Gas gangrene is rare, with only 1,000 to 3,000 cases occurring in the United States annually.

Hydrocele is a fluid-filled sack along the spermatic cord within the scrotum. Hydroceles are common in the newborn infant. They may be unilateral or bilateral and result when there is failure of the tract through which the testis descends from the abdomen into the scrotum to close. Peritoneal fluid drains through the open tract from the abdomen into the scrotum where it becomes trapped causing enlargement of the scrotum. Hydroceles may also be caused by inflammation or trauma of the testicle or epididymis or by fluid or blood obstruction within the spermatic cord. The incidence of this type of hydrocele is higher in older men.

Orchitis is an inflammation of one or both of the testicles, often caused by infection. Orchitis may be caused by numerous bacterial and viral organisms. It is usually a consequence of epididymitis (see above). The most common viral cause of orchitis is mumps. Approximately 30% of patients who have mumps will develop orchitis during the course of the illness. It is most common in post-pubertal boys (rare before 10 years of age). It usually manifests 4 to 6 days after the onset of mumps. In one-third of boys who get orchitis caused by mumps, testicular atrophy (shrinking of the testicles) will result.

Debilitating illnesses, such as diabetes, alcoholism, HIV infection, and malnutrition seem to increase the susceptibility of a person to Fournier gangrene but are not related disorders.

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Diagnosis

The diagnosis is basically made on clinical findings. Ultrasound evaluation may achieve early differentiation between Fournier gangrene and an acute inflammatory process, such as epididymitis or orchitis. Computed tomography may help to determine the portal of entry and extension of the process, but is not indispensable and should not delay surgical treatment.

X-ray studies are useful to confirm the location and extent of gas distribution in the wounds. Ultrasonography is useful to detect gases and/or fluids, but patients with severe pain may not be able to tolerate the pressures on the skin required to obtain an acceptable image. Computerized tomographic (CT) images are preferred because they resolve smaller amounts of soft tissue gases and fluids.

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Standard Therapies

Treatment

It is critical to recognize the disorder and to initiate aggressive resuscitation and administration of broad-spectrum intravenous antibiotics as quickly as possible. Such antibiotics must be followed by urgent surgical debridement of all affected dead (necrotic) skin and subcutaneous tissue involved, with repeated removal of wound margins as necessary. If colorectal or urogenital origin is established, source control is imperative, in accordance with each case. Patients with severe blood infection (sepsis) are at increased risk for developing blood clots (thrombembolic phenomena) and may require medication to reduce the risk for thrombosis Reconstructive surgery is undertaken, once infection is under control.

Colostomy remains controversial as a means of decreasing fecal contamination. Foley catheters generally get rid of urine adequately.

When available, a burn center may be a good location for the treatment of patients with necrotizing soft-tissue surgical infections, including Fournier gangrene.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

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References

TEXTBOOKS
Corman ML [ed.]. Corman’s colon and rectal surgery. 6th ed. Philadelphia : Wolters Kluwer Health/Lippincott Williams & Wilkins, 2013.

REVIEW ARTICLES
Shyam DC(1), Rapsang AG. Fournier’s gangrene. Surgeon. 2013 Aug;11(4):222-32.

Park H(1), Copeland C, Henry S, Barbul A. Complex wounds and their management. Surg Clin North Am. 2010 Dec;90(6):1181-94.

Mopurgo E, Galandiuk S. Fournier’s gangrene. Surg Clin North Am. 2002;1213-24.

Korhonen K. Hyperbaric oxygen therapy in acute necrotizing infections with a special reference to the effects on tissue gas tensions. Ann Chir Gynaecol Suppl. 2000;214:7-36.

Yagham RJ, Al-Jaberi TM, Bani-Hani I. Fournier’s gangrene: changing face of the disease. Dis Colon Rectum. 2000;43:1300-08.

Eke N. Fournier’s gangrene: a review of 1726 cases. Br J Surg. 2000;87:718-28.

JOURNAL ARTICLES
Korkut M, Icoz G, Dayangac M, et al. Outcome analysis in patients with Fournier’s gangrene: report of 45 cases. Dis Colon Rectum. 2003;46:649-52.

Norton KS, Johnson LW, Perry T, et al. Management of Fournier’s gangrene: an eleven year retrospective analysis of early recognition, diagnosis, and treatment. Am Surg. 2002;68:709-13.

Xeropotamos NS, Nousias VE, Kappas AM. Fournier’s gangrene: diagnostic approach and therapeutic challenge. Eur J Surg. 2002;168:91-95.

Faucher LD, Morris SE, Edelman LS, Phil M, Saffle JR. Burn center management of necrotizing soft-tissue surgical infections in unburned patients. Am J Surg. 2001; 182:563-569.

Fillo J, Cervenakov I, Labas P, et al. Fournier’s gangrene: can aggressive treatment save life. Int Urol Nephrol. 2001;33:533-36.

Nisbet AA, Thompson IM. Impact of diabetes mellitus on the presentation and outcomes of Fournier’s gangrene. Urology. 2000; 60(5):775-779

Corman JM, Moody JA, Aronson WJ. Fournier’s gangrene in a modern surgical setting: improved survival with aggressive management. BJU Int. 1999;84:85-88.

Burciaga-Alvarado A, Bracho-Riquelme RL, Betancourt-Valdivia JC. La Gangrena de Fournier: Serie de casos del Hospital General “C” de Durango, SSA. Cirugía (Durango, Mexico). 1995;4(1):11-17.

INTERNET
Pais VM, Santora T, Rukstalis DB. Fournier Grangrene. Medscape. Last Update May 07, 2019 . Available at https://emedicine.medscape.com/article/2028899-overview Accessed April 23, 2020.

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