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Fournier's syndrome: Synergistic gangrene of the scrotum
Fournier’s Syndrome: Synergistic Gangrene of the Scrotum Ross Rudolph, MD,* Cleveland, Ohio Mark Soloway, MD, Cleveland, Ohio Ralph G. DePalma, MD, Cleveland, Ohio Lester Persky, MD, Cleveland, Ohio
Fournier’s gangrene [I] is a rare malignant surgical infection of the scrotum. Because its clinical presentation is protean, the syndrome is frequently misdiagnosed and inadequately treated. Although local manifestations eventually draw clinical attention to the proper diagnosis, presentation as systemic illness or an acute abdomen may be misleading. If not recognized and treated early in its course, this infection may be fatal. When the process is viewed as a specific type of synergistic gangrene, management and therapy can be planned more logically. We report three successfully treated cases of Fournier’s syndrome, illustrating various presentations, problems in management, and specific approaches to therapy. Case Reports Case I. The patient, a forty-seven year old chronic alcoholic male had a one week history of increasing scrotal pain and swelling accompanied by fever and chills. There had been no urethral discharge or instrumentation. On physical examination, the temperature was 103.8OF, pulse rate 110 per minute, and blood pressure 100/60 mm Hg. Examination revealed an acutely ill man with scleral icterus and hepatomegaly. The penis and scrotum were markedly edematous with bilateral inguinal tenderness and adenopathy. The hematocrit was 40 per cent, and white blood cell count 6,700/mm3. Total bilirubin was 4.8 mg, serum gluFrom the Department of Surgery, Case Western Reselw, University Medicine, University Hospitals of Cleveland. and Cleveland Veterans Administratfon Hospital. C@elsnd, Ohio. Present address and address for reprintrequests: Departmentof Plasticand Reconstructhre Surgery,MedicalColIeQs of Wisconsin, 8700 WestWisconsin Avenue, Mlhvaukee. Wisconsin 53??26.
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Volume 129, May 1975
tamic oxalacetic acid 125 units, and serum protein 2.3 mg/lOO cc. Results of urinalysis were normal. The patient was treated with ampicillin, 1 gm every six hours. However, scrotal swelling and tenderness persisted and progressed. The hematocrit fell to 28 per cent. On the seventh hospital day foul-smelling yellowbrown purulent material drained from the scrotal area, and gas was palpated in subcutaneous tissues of the scrotum and perineum. Distention of the large and small bowel progressed to marked paralytic ileus requiring intestinal suction. (Figure 1.) Sigmoidoscopy and barium enema did not reveal communication of the scrotal process with the colon. Immediate Gram’s stain study of the scrotal exudate revealed a mixed infection with both gram-positive cocci and gram-negative bacilli. Cultures later grew Klebsiella, Bacteroides, and Escherichia coli. At operation the necrotic scrotal tissue was widely debrided; sinus tracts near the urethra extending into the abdominal wall under Scarpa’s fascia were opened widely. (Figure 2.) The wounds were treated with fine mesh gauze moistened with Dakin’s solution. Systemic antibiotic therapy with lincomycin and chloramphenicol, 1 gm every six hours, was begun. Serial debridement was performed every two days for eight days. As necrotic scrotal tissue cleared, the paralytic ileus and signs of systemic illness resolved. The abnormal liver function test results returned to normal values by the twenty-first day. The wounds began to granulate, and after two more weeks all exposed areas had a healthy appearance. (Figure 3.) The remaining
scrotal skin was mobilized and sutured with chromic catgut.
(Figure 4.) Penrose drains were placed and re-
moved one week later. The wounds healed comoletelv: * “I no subsequent noted.
genitourinary
abnormalities
have been
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The patient was returned to the operating room twice in the ensuing four days for debridement and dressing changes. His temperature returned to normal in the next two days, and signs of acute systemic illness resolved. Three weeks later the wounds were clean and granulating. The scrotal remnants were mobilized and sutured together to cover the exposed left testicle and cord.
Figure 1. Case 1. Abdominal roentgenogram marked dilatation of large and small bowel.
showing
Case II. The patient, a thirty-two year old man, was admitted with fever, chills, and scrotal swelling. One month prior to admission he had undergone urethral dilatation for stricture. On physical examination the temperature was 102’F, pulse rate 120 per minute, and blood pressure 104/80 mm Hg. The patient was acutely ill with marked swelling of the left scrotal area. The hematocrit was 26 per cent, and white blood cell count was 13,000/mm3 (87 per cent polymorphonuclear cells). An initial diagnosis of scrotal abscess was made; incision and drainage of the scrotum revealed foul-smelling purulent material. Immediate Gram’s stain study showed gram-positive cocci and gram-negative bacilli. Cultures later grew Klebsiella and Bacteroides. Urine culture was noncontributory. After dilatation of a stricture in the proximal urethra, a urethral catheter was inserted. Despite high doses of ampicillin, the patient’s condition did not improve and the temperature rose to 105°F. Within forty-eight hours after initial incision and drainage, an area of ascending cellulitis extended from the left hemiscrotum to the left inguinal and suprapubic areas. Extensive drainage was carried out through an incision from the base of the left scrotum to the left anterior superior iliac spine. The appearance of the scrotum was similar to that in the first case. Much of the necrotic left side of the scrotum was resected, but the testicle and spermatic cord were preserved. Dakin’s solution was used locally; lincomycin and chloramphenicol, 1 gm of each every six hours, were administered systemically.
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Case III. The patient, a sixty year old man, was admitted to the medical service with a tentative diagnosis of myocardial infarction because of sudden onset of auricular fibrillation and hypotension. Significant in the history were cystoscopy and local therapy for transitional cell carcinoma of the bladder two years previously, diabetes mellitus, and intermittent claudication of the right lower extremity of two years’ duration. Physical examination revealed the patient to be cold and clammy with a temperature of 98.6”F. The pulse rate was 110 per minute and irregularly irregular, and the blood pressure was SO/O mm Hg. He was acutely ill and disoriented. The right femoral pulse was absent, and the toes of the right foot were cyanotic. The hematocrit was 52 per cent with a white blood count of 18,700/mm3. Urinalysis revealed 3+ albumin and 5 to 10 white blood cells per high power field. Blood urea nitrogen was 32 mg/lOO ml, creatinine 2.5 mg/lOO ml, and serum glucose I35 mg/IOO ml. Serial electrocardiograms and enzyme level studies did not confirm the impression of myocardial infarction. The arrhythmia was readily reversed. Within twentyfour hours of admission the patient had spiking fever, marked lower abdominal pain, distention, and nausea and vomiting with progressive paralytic ileus. On the second hospital day he complained of exquisite pain and tenderness in the perineum just anterior to the rectum. Barium enema and sigmoidoscopy revealed no abnormalities. Systemic antibiotic therapy, chloramphenicol and penicillin, was begun. Swelling, redness, and pain progressed to the posterior aspect of the scrotum and testicles. On the seventh hospital day, incision and unroofing of the greatly distended scrotal tissues were carried out with the patient under general anesthesia. There was no communication with the rectum or urethra. Cultures revealed mixed coliform organisms and Bacteroides. Continued ileus required intestinal intubation, with fluid and electrolyte replacement of 2 to 3 L daily. A large area of gangrenous scrotal skin required debridement. Because of continued ileus (Figure 5), the patient underwent exploratory laparotomy on the twenty-first hospital day to rule out mechanical obstruction or communication with the perineal infection. At operation, massively distended large and small bowel were seen with no point of obstruction. Over the ensuing three weeks, ileus gradually resolved as did minor gangrenous changes in the toes of the right foot. Clinical improvement correlated with control of the local scrotal gan-
The Amarlcen
Journal
of Surgery
Fournier’s Syndrome
Figure 2. Case 1. Scrotum and penis of patient with Fournler’s gangrene aHer extensive debridement of gangrenous tissue and unroofing of sinus tracts. Clamp points to denuded urethra. Figure 3. Case 1. Vigorous granuiatlan tissue response occurring two weeks after debridement of gangrenous scrotal tissue. Figure 4. Case 1. Wound closure using flaps of local scrotal tissue, with Penrose drains placed beneath the skin.
grene. Coverage of the exposed scrotal areas occurred spontaneously. Excluding the diminished renal function and diabetes, the patient has remained well for the past two years. Comments
Fournier’s syndrome may begin insidiously in an older debilitated patient or explosively in a young healthy male [l-5]. Regardless of the mode of onset, scrotal swelling and pain are the first local symptoms, followed by progressive necrosis of scrotal skin and subcutaneous tissues. Signs and symptoms of acute systemic illness may precede or accompany the local process. The remarkably malodorous purulence accompanying this process appears as a shaggy yellow-brown exudate. As in the cases described, the disease does not resolve with simple incision and drainage, but inevitably progresses to extensive loss of scrotal tissue. The patient often complains of considerable pain and becomes severely toxic. The gangrene involves the perineum and sometimes the penis in various degrees. Ominously, the infection may extend to the abdominal wall by burrowing under Scarpa’s fascia. Subcutaneous gas can then be palpated in the suprapubic area. As gangrene of the scrotum progresses, the testicles frequently are exposed and completely denuded. The cause of Fournier’s gangrene is unclear [1,2,6]. Scrotal gangrene may occur with systemic
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1975
Figure 5. Case iii. Abdominal roentgenogram showing dtstended bowel and M/tier-Abbott tube. Pattern is suggestive of unrelieved small bo wei obstruction.
diseases such as smallpox, measles, and diabetes. It may also be seen in urinary extravasation and trauma [6]. According to Dunaif [7], the most likely cause of Fournier’s gangrene appears to be spreading infection from periurethral glands. Infection may spread to the corpus spongiosum and
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Figure 6. Cross-section of penis and sagittal sect/on of genitalia, which show fascial planes involved in extension of synergistic scrotal gangrene. Arrows demonstrate potential spread of infection. Adapted from Tobin and Benjamin [g].
be temporarily contained by the tunica albuginea of the corpus. (Figure 6.) This layer encloses the penile corpora and separates them into three compartments. Buck’s fascia is the next obstacle to the spreading infection, but only rarely is the purulent material limited by this layer. After penetrating Buck’s fascia the infection spreads rapidly, following the dartos fascia along the penis and scrotum, and Colles’ fascia in the perineum. Because of these anatomic planes, the infection may proceed upward to the abdominal wall under Scarpa’s fascia [8,9]. Gangrene of the scrotal skin is due to an obliterating endarteritis secondary to the spread of microorganisms within the fatty subcutaneous tissue. The scrotal skin is nourished by branches from the internal pudendal artery and by the superficial and deep external pudendal branches of the femoral artery [lo]. Occlusion of these vessels leads to ischemic necrosis of the overlying skin [3,6,7]. Before antibiotics were available, Fournier’s gangrene was associated with high mortality. Gibson [2] in 1930 noted a mortality of 26.7 per cent. In a later series, Mair [I] in 1945 reported a mortality of 32.5 per cent. More recent studies [6,11] reveal a decreased mortality rate of 7 to 25 per cent. This improvement may be related to earlier recognition of the syndrome and aggressive antibiotic and surgical therapy. Many microorganisms have been obtained from the exudate in scrotal gangrene: Proteus, E. coli, Pseudomonas, Clostridia, Staphylococcus aureus, and beta-hemolytic Streptococcus [12]. Anaerobic
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Streptococcus almost always is found [12]. In mixed bacterial infection, however, microaerophilic Streptococcus may be difficult to culture [23]. This was the case in our patients. Only Klebsiella, E. coli, and Bacteroides were cultured; gram-positive cocci seen on Gram’s stain study did not grow on the usual culture media. To obtain maximal information, cultures and sensitivity studies using both aerobic and anaerobic culture media must be obtained. In addition, immediate Gram’s stain study is most helpful to demonstrate the characteristic combination of gram-positive cocci and gram-negative bacilli. Immediate examination of the smear after Gram’s staining is thus of more clinical utility for the prompt diagnosis of synergistic gangrene than are cultures. Because of the frequent simultaneous occurrence of anaerobic Streptococci with other microorganisms, Fournier’s gangrene should be viewed as a form of synergistic bacterial gangrene. When so considered, the clinical manifestations and the methods of treatment can be structured more logically. Other forms of synergistic bacterial gangrene resemble idiopathic scrotal gangrene [lo], including infection due to human bites [14], some forms of necrotizing fascitis [13,15], necrotizing cellulitis [16], and classic Meleney’s gangrene [I 7-191. All these processes have in common as the etiologic factor the synergy of aerobic and anaerobic organisms, which results in extensive tissue necrosis. The major features of synergistic bacterial gangrene include the lack of response to antibiotic therapy alone and the need for wide incision and drainage. These surgical infections progress inexorably until the malignant nature of the disease process is recognized and they are treated vigorously. As with all synergistic infections, wide incision and drainage is mandatory [13,14,16-191. Sinus tracts must be fully unroofed; tight packing of deep sinuses may produce an occlusive process and further the infection. Of all types of synergistic gangrene, only Meleney’s gangrene [18] requires radical debridement into normal tissue. The other varieties of synergistic gangrene, including Fournier’s, should be treated with wide incision and drainage and removal of necrotic skin and fat. The wounds should be dressed frequently, in the operating room using appropriate anesthesia. Adequate inspection of burrowing tracts and observation of the extent of infection require considerable manipulation of tissue. This cannot be accomplished unless pain is relieved; the affected scrotal tissues are exquisitely tender.
The American Jounal
of Surgery
Fournier’s Syndrome
In Fournier’s gangrene, the testicles and spermatic cords may be exposed or coated with dead tissue. A few authors [5,7] have described amputation of testicles. However, since the testicles are usually not gangrenous, orchidectomy is not indicated. The testicles almost always survive the scrotal infection since their blood supply, the spermatic arteries, is separate from that of the scrotum. With proper treatment, all necrotic tissue separates within two or three weeks. A vigorous granulation tissue response occurs, with the denuded testicles contributing to the production of granulation tissue. Since the scrotal skin is capable of rapid regeneration, remarkably large defects in this area can be covered from scrotal remnants [2,20]. Wound contraction is also prominent in this area [6]. Skin grafts or, rarely, pedicle flaps have been used to speed healing [7,20]. Broad spectrum antibiotics are required, preferably lincomycin or penicillin and chloramphenicol in high doses. If Pseudomonas is present, gentamycin should be used. Dakin’s solution has been used locally, delivered via catheters to the wound. This solution appears to help control infection and reduces the offensive odor [2,12]. Urinary drainage with urethral catheters was satisfactory in all these cases. Although suprapubic cystostomy has been recommended [12] in the past, it does not appear essential. Persistent adynamic ileus can become a major component of the clinical picture, as seen in two of our patients. One patient required laparotomy to rule out the presence of mechanical obstruction. This feature of Fournier’s gangrene has not been emphasized previously. The cause of the paralytic ileus is not clear. There may be two mechanisms involved in intestinal paralysis accompanying scrotal gangrene. Reflex sympathetic activity mediated by the splanchnic nerves can play a role. Bayliss and Starling [21] demonstrated in 1899 that sectioning of these nerves prevents paralytic ileus associated with laparotomy. Testicular trauma also reflexly inhibits small bowel peristalsis, and ileus due to testicular trauma can be prevented by prior sectioning of the splanchnic nerves [22]. It is possible that extensive scrotal inflammation might evoke this sympathetic reflex. Elevated levels of circulating catecholamines may also inhibit peristalsis and cause adynamic ileus [23], which also could be a contributing factor since circulatory failure also appeared in one of our patients. Management of protracted ileus with nasogastric or long tube suction, plus appropriate replace-
Volume 129, May 1975
ment of fluids and electrolytes, appears to be the best therapy. Particular emphasis must be placed on adequate potassium balance since hypokalemia can prolong the adynamic state. When mechanical obstruction or communication with an abdominal viscus has been ruled out, resolution of the scrotal process can be expected to correspond to resolution of the paralytic ileus. Although abdominal surgical intervention may seem necessary because of the protracted febrile course associated with abdominal pain, it is important to avoid laparotomy in these critically ill patients. This complex clinical entity, although infrequently encountered and of unknown cause, presents a diagnostic and therapeutic challenge. The manifestations and involvement of a variety of systems sometimes delay recognition. When therapy is hesitant or inadequate, extension of the necrotic process increases. When the entity is suspected, therefore, wide drainage and antibacterial therapy must be employed in a bold total manner. As described in this report, an appreciation of Fournier’s syndrome as a synergistic gangrene is most useful diagnostically and therapeutically. Summary Progressive spread of necrosis in the skin and subcutaneous tissues of the scrotum is the key feature of idiopathic scrotal gangrene. The disease may present initially as an acute abdomen, but laparotomy should be avoided. Usually an anaerobic Streptococcus is found, acting in synergism with aerobic, frequently gram-negative, bacilli. As in other synergistic gangrenes, wide debridement with drainage of all sinus tracts is required. Although the testicles are frequently bared, they are usually not necrotic and should not be amputated. Once the infection has resolved, a surprising amount of skin coverage, including coverage of the testicles, can often be obtained from the scrotal remnants. References I. Mair GB: Idiopathic gangrene of scrotum. Lancet 1: 464, 1945. 2. Gibson TE: Idiopathic gangrene of the scrotum: with report of a case and review of the literature. J Ural 23: 125. 1930. Kilby JO: Gangrene of scrotum and penis. Br J Surg 49: 619, 1962. Randall A: Idiopathic gangrene of the scrotum. J Ural 4: 219, 1920. Gerber MP, Peterson NE: Scrotal gangrene. Urology 1: 466, 1973. Campbell JC: Fournier’s gangrene. Br J &o/27: 106, 1955.
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7. Dunaif CB: Fournier’s gangrene. Plast Reconstr Surg 33: 84, 1964. 8. Gray TA: Gangrene of the genitalia as seen in advanced peri-urethral extravasation with phlegmon. J Ural 84: 741, 1960. 9. Tobin CE. Benjamin JA: Anatomical study and clinical consideration of the fasciae limiting urinary extravasation from the penile urethra. Surg Gynecol Obstet 79: 195, 1944. 10. Gregory IL: Fournier’s gangrene. Br J Ural 27: 116, 1955. 11. Thomas JE: Fournier’s gangrene of the penis and scrotum. J lJro175: 719. 1956. 12. Talarico RD: Fournier’s gangrene. Mod Treat 7: 1049. 1970. 13. Meade JW, Mueller CB: Necrotiiing infections of subcutaneous tissue and fascia. Ann Surg 168: 274, 1968. 14. Farmer CB, Mann RJ: Human bite infections of the hand. SouthMedJ59: 515, 1966. 15. Crosthwait RW Jr, Crosthwait RW, Jordan GL Jr: Necrotizing fascitis. J Trauma 4: 168. 1964. 16. Stone HH, Martin JD Jr: Synergistic necrotizing cellulitis. Ann Surg 175: 702, 1972.
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17. Grainger RW, MacKenzie DA, McLacklin AD: Progressive bacterial synergistic gangrene: chronic undermining ulcer of Meleney. Can J S&g 10: 439. 1967. 18. Melenev FL: Bacterial svneraism in disease processes with a confirmation of the synergistic bacterial etiology of a certain type of progressive gangrene of the abdominal wall. Ann Surg94: 961, 1931. 19. Meleney FL: A differential diagnosis between certain types of infectious gangrene of the skin with particular reference to haemolytic streptococcus gangrene and bacterial synergistic gangrene. Surg Gynecol Obstet 56: 847, 1933. 20. Moustafa MFH: Gangrene of the scrotum: an analysis of ten cases. Br J P&t Surg 20: 90, 1967. 21. Bayliss WM, Starling EH: The movements and innervation of the small intestine. J Physiot 24: 99, 1899. 22. Cannon WB, Murphy FT: Physiologic observations on experimentally produced ileus. JAMA 49: 840. 1907. 23. Landman MD, Longmire WP Jr: Neural and hormonal influences of peritonitis on paralytic ileus. Am Surg 33: 756, 1967.
The Amarlcan Journel 01 Surgery
Fournier’s gangrene [I] is a rare malignant surgical infection of the scrotum. Because its clinical presentation is protean, the syndrome is frequently misdiagnosed and inadequately treated. Although local manifestations eventually draw clinical attention to the proper diagnosis, presentation as systemic illness or an acute abdomen may be misleading. If not recognized and treated early in its course, this infection may be fatal. When the process is viewed as a specific type of synergistic gangrene, management and therapy can be planned more logically. We report three successfully treated cases of Fournier’s syndrome, illustrating various presentations, problems in management, and specific approaches to therapy. Case Reports Case I. The patient, a forty-seven year old chronic alcoholic male had a one week history of increasing scrotal pain and swelling accompanied by fever and chills. There had been no urethral discharge or instrumentation. On physical examination, the temperature was 103.8OF, pulse rate 110 per minute, and blood pressure 100/60 mm Hg. Examination revealed an acutely ill man with scleral icterus and hepatomegaly. The penis and scrotum were markedly edematous with bilateral inguinal tenderness and adenopathy. The hematocrit was 40 per cent, and white blood cell count 6,700/mm3. Total bilirubin was 4.8 mg, serum gluFrom the Department of Surgery, Case Western Reselw, University Medicine, University Hospitals of Cleveland. and Cleveland Veterans Administratfon Hospital. C@elsnd, Ohio. Present address and address for reprintrequests: Departmentof Plasticand Reconstructhre Surgery,MedicalColIeQs of Wisconsin, 8700 WestWisconsin Avenue, Mlhvaukee. Wisconsin 53??26.
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Volume 129, May 1975
tamic oxalacetic acid 125 units, and serum protein 2.3 mg/lOO cc. Results of urinalysis were normal. The patient was treated with ampicillin, 1 gm every six hours. However, scrotal swelling and tenderness persisted and progressed. The hematocrit fell to 28 per cent. On the seventh hospital day foul-smelling yellowbrown purulent material drained from the scrotal area, and gas was palpated in subcutaneous tissues of the scrotum and perineum. Distention of the large and small bowel progressed to marked paralytic ileus requiring intestinal suction. (Figure 1.) Sigmoidoscopy and barium enema did not reveal communication of the scrotal process with the colon. Immediate Gram’s stain study of the scrotal exudate revealed a mixed infection with both gram-positive cocci and gram-negative bacilli. Cultures later grew Klebsiella, Bacteroides, and Escherichia coli. At operation the necrotic scrotal tissue was widely debrided; sinus tracts near the urethra extending into the abdominal wall under Scarpa’s fascia were opened widely. (Figure 2.) The wounds were treated with fine mesh gauze moistened with Dakin’s solution. Systemic antibiotic therapy with lincomycin and chloramphenicol, 1 gm every six hours, was begun. Serial debridement was performed every two days for eight days. As necrotic scrotal tissue cleared, the paralytic ileus and signs of systemic illness resolved. The abnormal liver function test results returned to normal values by the twenty-first day. The wounds began to granulate, and after two more weeks all exposed areas had a healthy appearance. (Figure 3.) The remaining
scrotal skin was mobilized and sutured with chromic catgut.
(Figure 4.) Penrose drains were placed and re-
moved one week later. The wounds healed comoletelv: * “I no subsequent noted.
genitourinary
abnormalities
have been
591
Rudolph et al
The patient was returned to the operating room twice in the ensuing four days for debridement and dressing changes. His temperature returned to normal in the next two days, and signs of acute systemic illness resolved. Three weeks later the wounds were clean and granulating. The scrotal remnants were mobilized and sutured together to cover the exposed left testicle and cord.
Figure 1. Case 1. Abdominal roentgenogram marked dilatation of large and small bowel.
showing
Case II. The patient, a thirty-two year old man, was admitted with fever, chills, and scrotal swelling. One month prior to admission he had undergone urethral dilatation for stricture. On physical examination the temperature was 102’F, pulse rate 120 per minute, and blood pressure 104/80 mm Hg. The patient was acutely ill with marked swelling of the left scrotal area. The hematocrit was 26 per cent, and white blood cell count was 13,000/mm3 (87 per cent polymorphonuclear cells). An initial diagnosis of scrotal abscess was made; incision and drainage of the scrotum revealed foul-smelling purulent material. Immediate Gram’s stain study showed gram-positive cocci and gram-negative bacilli. Cultures later grew Klebsiella and Bacteroides. Urine culture was noncontributory. After dilatation of a stricture in the proximal urethra, a urethral catheter was inserted. Despite high doses of ampicillin, the patient’s condition did not improve and the temperature rose to 105°F. Within forty-eight hours after initial incision and drainage, an area of ascending cellulitis extended from the left hemiscrotum to the left inguinal and suprapubic areas. Extensive drainage was carried out through an incision from the base of the left scrotum to the left anterior superior iliac spine. The appearance of the scrotum was similar to that in the first case. Much of the necrotic left side of the scrotum was resected, but the testicle and spermatic cord were preserved. Dakin’s solution was used locally; lincomycin and chloramphenicol, 1 gm of each every six hours, were administered systemically.
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Case III. The patient, a sixty year old man, was admitted to the medical service with a tentative diagnosis of myocardial infarction because of sudden onset of auricular fibrillation and hypotension. Significant in the history were cystoscopy and local therapy for transitional cell carcinoma of the bladder two years previously, diabetes mellitus, and intermittent claudication of the right lower extremity of two years’ duration. Physical examination revealed the patient to be cold and clammy with a temperature of 98.6”F. The pulse rate was 110 per minute and irregularly irregular, and the blood pressure was SO/O mm Hg. He was acutely ill and disoriented. The right femoral pulse was absent, and the toes of the right foot were cyanotic. The hematocrit was 52 per cent with a white blood count of 18,700/mm3. Urinalysis revealed 3+ albumin and 5 to 10 white blood cells per high power field. Blood urea nitrogen was 32 mg/lOO ml, creatinine 2.5 mg/lOO ml, and serum glucose I35 mg/IOO ml. Serial electrocardiograms and enzyme level studies did not confirm the impression of myocardial infarction. The arrhythmia was readily reversed. Within twentyfour hours of admission the patient had spiking fever, marked lower abdominal pain, distention, and nausea and vomiting with progressive paralytic ileus. On the second hospital day he complained of exquisite pain and tenderness in the perineum just anterior to the rectum. Barium enema and sigmoidoscopy revealed no abnormalities. Systemic antibiotic therapy, chloramphenicol and penicillin, was begun. Swelling, redness, and pain progressed to the posterior aspect of the scrotum and testicles. On the seventh hospital day, incision and unroofing of the greatly distended scrotal tissues were carried out with the patient under general anesthesia. There was no communication with the rectum or urethra. Cultures revealed mixed coliform organisms and Bacteroides. Continued ileus required intestinal intubation, with fluid and electrolyte replacement of 2 to 3 L daily. A large area of gangrenous scrotal skin required debridement. Because of continued ileus (Figure 5), the patient underwent exploratory laparotomy on the twenty-first hospital day to rule out mechanical obstruction or communication with the perineal infection. At operation, massively distended large and small bowel were seen with no point of obstruction. Over the ensuing three weeks, ileus gradually resolved as did minor gangrenous changes in the toes of the right foot. Clinical improvement correlated with control of the local scrotal gan-
The Amarlcen
Journal
of Surgery
Fournier’s Syndrome
Figure 2. Case 1. Scrotum and penis of patient with Fournler’s gangrene aHer extensive debridement of gangrenous tissue and unroofing of sinus tracts. Clamp points to denuded urethra. Figure 3. Case 1. Vigorous granuiatlan tissue response occurring two weeks after debridement of gangrenous scrotal tissue. Figure 4. Case 1. Wound closure using flaps of local scrotal tissue, with Penrose drains placed beneath the skin.
grene. Coverage of the exposed scrotal areas occurred spontaneously. Excluding the diminished renal function and diabetes, the patient has remained well for the past two years. Comments
Fournier’s syndrome may begin insidiously in an older debilitated patient or explosively in a young healthy male [l-5]. Regardless of the mode of onset, scrotal swelling and pain are the first local symptoms, followed by progressive necrosis of scrotal skin and subcutaneous tissues. Signs and symptoms of acute systemic illness may precede or accompany the local process. The remarkably malodorous purulence accompanying this process appears as a shaggy yellow-brown exudate. As in the cases described, the disease does not resolve with simple incision and drainage, but inevitably progresses to extensive loss of scrotal tissue. The patient often complains of considerable pain and becomes severely toxic. The gangrene involves the perineum and sometimes the penis in various degrees. Ominously, the infection may extend to the abdominal wall by burrowing under Scarpa’s fascia. Subcutaneous gas can then be palpated in the suprapubic area. As gangrene of the scrotum progresses, the testicles frequently are exposed and completely denuded. The cause of Fournier’s gangrene is unclear [1,2,6]. Scrotal gangrene may occur with systemic
Volume
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May
1975
Figure 5. Case iii. Abdominal roentgenogram showing dtstended bowel and M/tier-Abbott tube. Pattern is suggestive of unrelieved small bo wei obstruction.
diseases such as smallpox, measles, and diabetes. It may also be seen in urinary extravasation and trauma [6]. According to Dunaif [7], the most likely cause of Fournier’s gangrene appears to be spreading infection from periurethral glands. Infection may spread to the corpus spongiosum and
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Figure 6. Cross-section of penis and sagittal sect/on of genitalia, which show fascial planes involved in extension of synergistic scrotal gangrene. Arrows demonstrate potential spread of infection. Adapted from Tobin and Benjamin [g].
be temporarily contained by the tunica albuginea of the corpus. (Figure 6.) This layer encloses the penile corpora and separates them into three compartments. Buck’s fascia is the next obstacle to the spreading infection, but only rarely is the purulent material limited by this layer. After penetrating Buck’s fascia the infection spreads rapidly, following the dartos fascia along the penis and scrotum, and Colles’ fascia in the perineum. Because of these anatomic planes, the infection may proceed upward to the abdominal wall under Scarpa’s fascia [8,9]. Gangrene of the scrotal skin is due to an obliterating endarteritis secondary to the spread of microorganisms within the fatty subcutaneous tissue. The scrotal skin is nourished by branches from the internal pudendal artery and by the superficial and deep external pudendal branches of the femoral artery [lo]. Occlusion of these vessels leads to ischemic necrosis of the overlying skin [3,6,7]. Before antibiotics were available, Fournier’s gangrene was associated with high mortality. Gibson [2] in 1930 noted a mortality of 26.7 per cent. In a later series, Mair [I] in 1945 reported a mortality of 32.5 per cent. More recent studies [6,11] reveal a decreased mortality rate of 7 to 25 per cent. This improvement may be related to earlier recognition of the syndrome and aggressive antibiotic and surgical therapy. Many microorganisms have been obtained from the exudate in scrotal gangrene: Proteus, E. coli, Pseudomonas, Clostridia, Staphylococcus aureus, and beta-hemolytic Streptococcus [12]. Anaerobic
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Streptococcus almost always is found [12]. In mixed bacterial infection, however, microaerophilic Streptococcus may be difficult to culture [23]. This was the case in our patients. Only Klebsiella, E. coli, and Bacteroides were cultured; gram-positive cocci seen on Gram’s stain study did not grow on the usual culture media. To obtain maximal information, cultures and sensitivity studies using both aerobic and anaerobic culture media must be obtained. In addition, immediate Gram’s stain study is most helpful to demonstrate the characteristic combination of gram-positive cocci and gram-negative bacilli. Immediate examination of the smear after Gram’s staining is thus of more clinical utility for the prompt diagnosis of synergistic gangrene than are cultures. Because of the frequent simultaneous occurrence of anaerobic Streptococci with other microorganisms, Fournier’s gangrene should be viewed as a form of synergistic bacterial gangrene. When so considered, the clinical manifestations and the methods of treatment can be structured more logically. Other forms of synergistic bacterial gangrene resemble idiopathic scrotal gangrene [lo], including infection due to human bites [14], some forms of necrotizing fascitis [13,15], necrotizing cellulitis [16], and classic Meleney’s gangrene [I 7-191. All these processes have in common as the etiologic factor the synergy of aerobic and anaerobic organisms, which results in extensive tissue necrosis. The major features of synergistic bacterial gangrene include the lack of response to antibiotic therapy alone and the need for wide incision and drainage. These surgical infections progress inexorably until the malignant nature of the disease process is recognized and they are treated vigorously. As with all synergistic infections, wide incision and drainage is mandatory [13,14,16-191. Sinus tracts must be fully unroofed; tight packing of deep sinuses may produce an occlusive process and further the infection. Of all types of synergistic gangrene, only Meleney’s gangrene [18] requires radical debridement into normal tissue. The other varieties of synergistic gangrene, including Fournier’s, should be treated with wide incision and drainage and removal of necrotic skin and fat. The wounds should be dressed frequently, in the operating room using appropriate anesthesia. Adequate inspection of burrowing tracts and observation of the extent of infection require considerable manipulation of tissue. This cannot be accomplished unless pain is relieved; the affected scrotal tissues are exquisitely tender.
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Fournier’s Syndrome
In Fournier’s gangrene, the testicles and spermatic cords may be exposed or coated with dead tissue. A few authors [5,7] have described amputation of testicles. However, since the testicles are usually not gangrenous, orchidectomy is not indicated. The testicles almost always survive the scrotal infection since their blood supply, the spermatic arteries, is separate from that of the scrotum. With proper treatment, all necrotic tissue separates within two or three weeks. A vigorous granulation tissue response occurs, with the denuded testicles contributing to the production of granulation tissue. Since the scrotal skin is capable of rapid regeneration, remarkably large defects in this area can be covered from scrotal remnants [2,20]. Wound contraction is also prominent in this area [6]. Skin grafts or, rarely, pedicle flaps have been used to speed healing [7,20]. Broad spectrum antibiotics are required, preferably lincomycin or penicillin and chloramphenicol in high doses. If Pseudomonas is present, gentamycin should be used. Dakin’s solution has been used locally, delivered via catheters to the wound. This solution appears to help control infection and reduces the offensive odor [2,12]. Urinary drainage with urethral catheters was satisfactory in all these cases. Although suprapubic cystostomy has been recommended [12] in the past, it does not appear essential. Persistent adynamic ileus can become a major component of the clinical picture, as seen in two of our patients. One patient required laparotomy to rule out the presence of mechanical obstruction. This feature of Fournier’s gangrene has not been emphasized previously. The cause of the paralytic ileus is not clear. There may be two mechanisms involved in intestinal paralysis accompanying scrotal gangrene. Reflex sympathetic activity mediated by the splanchnic nerves can play a role. Bayliss and Starling [21] demonstrated in 1899 that sectioning of these nerves prevents paralytic ileus associated with laparotomy. Testicular trauma also reflexly inhibits small bowel peristalsis, and ileus due to testicular trauma can be prevented by prior sectioning of the splanchnic nerves [22]. It is possible that extensive scrotal inflammation might evoke this sympathetic reflex. Elevated levels of circulating catecholamines may also inhibit peristalsis and cause adynamic ileus [23], which also could be a contributing factor since circulatory failure also appeared in one of our patients. Management of protracted ileus with nasogastric or long tube suction, plus appropriate replace-
Volume 129, May 1975
ment of fluids and electrolytes, appears to be the best therapy. Particular emphasis must be placed on adequate potassium balance since hypokalemia can prolong the adynamic state. When mechanical obstruction or communication with an abdominal viscus has been ruled out, resolution of the scrotal process can be expected to correspond to resolution of the paralytic ileus. Although abdominal surgical intervention may seem necessary because of the protracted febrile course associated with abdominal pain, it is important to avoid laparotomy in these critically ill patients. This complex clinical entity, although infrequently encountered and of unknown cause, presents a diagnostic and therapeutic challenge. The manifestations and involvement of a variety of systems sometimes delay recognition. When therapy is hesitant or inadequate, extension of the necrotic process increases. When the entity is suspected, therefore, wide drainage and antibacterial therapy must be employed in a bold total manner. As described in this report, an appreciation of Fournier’s syndrome as a synergistic gangrene is most useful diagnostically and therapeutically. Summary Progressive spread of necrosis in the skin and subcutaneous tissues of the scrotum is the key feature of idiopathic scrotal gangrene. The disease may present initially as an acute abdomen, but laparotomy should be avoided. Usually an anaerobic Streptococcus is found, acting in synergism with aerobic, frequently gram-negative, bacilli. As in other synergistic gangrenes, wide debridement with drainage of all sinus tracts is required. Although the testicles are frequently bared, they are usually not necrotic and should not be amputated. Once the infection has resolved, a surprising amount of skin coverage, including coverage of the testicles, can often be obtained from the scrotal remnants. References I. Mair GB: Idiopathic gangrene of scrotum. Lancet 1: 464, 1945. 2. Gibson TE: Idiopathic gangrene of the scrotum: with report of a case and review of the literature. J Ural 23: 125. 1930. Kilby JO: Gangrene of scrotum and penis. Br J Surg 49: 619, 1962. Randall A: Idiopathic gangrene of the scrotum. J Ural 4: 219, 1920. Gerber MP, Peterson NE: Scrotal gangrene. Urology 1: 466, 1973. Campbell JC: Fournier’s gangrene. Br J &o/27: 106, 1955.
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7. Dunaif CB: Fournier’s gangrene. Plast Reconstr Surg 33: 84, 1964. 8. Gray TA: Gangrene of the genitalia as seen in advanced peri-urethral extravasation with phlegmon. J Ural 84: 741, 1960. 9. Tobin CE. Benjamin JA: Anatomical study and clinical consideration of the fasciae limiting urinary extravasation from the penile urethra. Surg Gynecol Obstet 79: 195, 1944. 10. Gregory IL: Fournier’s gangrene. Br J Ural 27: 116, 1955. 11. Thomas JE: Fournier’s gangrene of the penis and scrotum. J lJro175: 719. 1956. 12. Talarico RD: Fournier’s gangrene. Mod Treat 7: 1049. 1970. 13. Meade JW, Mueller CB: Necrotiiing infections of subcutaneous tissue and fascia. Ann Surg 168: 274, 1968. 14. Farmer CB, Mann RJ: Human bite infections of the hand. SouthMedJ59: 515, 1966. 15. Crosthwait RW Jr, Crosthwait RW, Jordan GL Jr: Necrotizing fascitis. J Trauma 4: 168. 1964. 16. Stone HH, Martin JD Jr: Synergistic necrotizing cellulitis. Ann Surg 175: 702, 1972.
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17. Grainger RW, MacKenzie DA, McLacklin AD: Progressive bacterial synergistic gangrene: chronic undermining ulcer of Meleney. Can J S&g 10: 439. 1967. 18. Melenev FL: Bacterial svneraism in disease processes with a confirmation of the synergistic bacterial etiology of a certain type of progressive gangrene of the abdominal wall. Ann Surg94: 961, 1931. 19. Meleney FL: A differential diagnosis between certain types of infectious gangrene of the skin with particular reference to haemolytic streptococcus gangrene and bacterial synergistic gangrene. Surg Gynecol Obstet 56: 847, 1933. 20. Moustafa MFH: Gangrene of the scrotum: an analysis of ten cases. Br J P&t Surg 20: 90, 1967. 21. Bayliss WM, Starling EH: The movements and innervation of the small intestine. J Physiot 24: 99, 1899. 22. Cannon WB, Murphy FT: Physiologic observations on experimentally produced ileus. JAMA 49: 840. 1907. 23. Landman MD, Longmire WP Jr: Neural and hormonal influences of peritonitis on paralytic ileus. Am Surg 33: 756, 1967.
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