Chapter 6 6 Fournier’s Gangrene C.F.Heyns,P.D.Theron et al. 1983; Hejase et al. 1996). However, the incidence is 6.1 Definition and Historical Perspective 50 rising, most likely due to an increase in the mean age of 6.2 Etiology 50 the population, as well as increased numbers of pa- 6.3 Anatomy 52 tients on immunosuppressive therapy or suffering 6.4 Microbiology 53 from human immunodeficiency virus (HIV) infection, 6.5 Pathogenesis 54 especially in Africa (McKay and Waters 1994; Elem and 6.6 Ranjan 1995; Merino et al. 2001; Heyns and Fisher Clinical Presentation 55 2005). 6.7 Special Investigations 55 6.8 Management 55 6.8.1 Initial and Preoperative Management 56 6.2 6.8.2 Surgery 56 6.8.3 Postoperative Management 57 Etiology 6.8.4 Hyperbaric Oxygen 58 6.8.5 Wound Care 58 Anetiologicalfactororfactorscanbeidentifiedinmore 6.8.6 Reconstructive Surgery 59 than 90% of cases and should be actively sought, be- 6.9 Complications 59 cause it may determine the treatment and prognosis 6.10 Prognosis 59 (Smith et al. 1998; Santora and Rukstalis 2001). In ap- parently idiopathic cases, the cause may have been over- References 59 looked or obscured by the necrotizing disease process. Any process where a virulent, synergistic infection 6.1 gains access to the subcutaneous tissue of the perineum Definition and Historical Perspective may serve as the point of origin. The cause of infection may be from a urogenital, anorectal, cutaneous, or ret- Fournier’s gangrene is a synergistic polymicrobial nec- roperitoneal origin. The urogenital area is the most rotizing fasciitis of the perineum and genitalia. It can common etiologic site, where urethral stricture disease progress to a fulminant soft tissue infection that is at the top of the list (Edino et al. 2005). Knowledge of spreads rapidly along the fascial planes, causing necro- the anatomy of the perineum, urogenital area, and low- sis of the skin, subcutaneous soft tissue, and fascia, er abdomen is necessary to understand the etiology with associated systemic sepsis. If it is not diagnosed and pathogenesis of this fulminant infection. early and treated promptly, significant morbidity with The possible causes of Fournier’s gangrene are listed prolonged hospital stay and even mortality will ensue. in Table 6.1. Infection may originate in any of the listed In 1764, Baurienne described a fulminant gangrene areas, with extension to the fascial planes leading to a ofthemaleperineum.However,JeanAlfredFournier,a proliferating fasciitis (Jones et al. 1979; Karim 1984; French dermatologist and venereologist, became fa- Walker et al. 1984; Walther et al. 1987; Baskin et al. 1990; mous for this notorious condition when, in 1883, he de- Sengoku et al. 1990; Gaeta et al. 1991; Attah 1992; Paty scribed a series of five young men in whom gangrene of and Smith 1992; Theiss et al. 1995; Benizri et al. 1996; the genitalia occurred without any apparent etiologic Hejase et al. 1996; Fialkov et al. 1998; Corman et al. factor. As knowledge of the condition increased over the 1999; Eke 2000; Kilic et al. 2001; Ali 2004; Jeong 2004; years, it became clear that Fournier’s gangrene is most Yeniyol et al. 2004; Edino et al. 2005). commoninoldermen(peakincidenceinthe5th and 6th Although Fournier’s gangrene is predominantly a decades) and that most cases have an identifiable cause. condition of the older male, it may occur at any age, and Fortunately, it is a rare condition, with a reported in- approximately10%ofcasesoccurinfemales(Kilicet cidence of 1/7,500, and accounting for only 1%–2% of al. 2001; Quatan and Kirby 2004). Specific causes in urologic hospital admissions (Bejanga 1979; Bahlmann women include pudendal nerve block or episiotomy for 6.2 Etiology 51 Table 6.1. Causes of Fournier’s gangrene Table 6.2. Underlying disorders in patients with Fournier’s gan- grene Urogenital Urethral stricture Diabetes mellitus Indwelling transurethral catheter Chronic alcoholism Prolonged or neglected use of condom catheter Malnutrition Urethral calculi Obesity Urethritis Liver cirrhosis Transurethral surger y Poor personal hygiene Infection of periurethral glands and paraurethral abscess Immunosuppression: Urogenital tuberculosis Chronic steroid use Urethral cancer Organ transplantation Prostate biopsy Chemotherapy for malignancy Prostatic massage HIV/AIDS Prostate abscess Tuberculosis Insertion of penile prosthesis Syphilis Constriction ring device for management of ED Iatrogenic trauma bial infection (Table 6.2). Fournier’s gangrene is often a Cauterization of genital warts marker of an underlying disease such as diabetes melli- Circumcision tus, urogenital tuberculosis, syphilis, or HIV. Manipulation of longstanding paraphimosis Noniatrogenic trauma Diabetes mellitus is the most common associated Animal, insect, or human bite underlying systemic disease, affecting two-thirds of pa- Scrotal abscess tients with Fournier’s gangrene. Diabetic patients have Infected hydrocele a higher incidence of urinary tract infections, due to Hydrocelectomy Vasec tomy cystopathy with urinary stasis (Baskin et al. 1990). Hy- Balanitis perglycemia decreases cellular immunity by decreasing Phimosis phagocytic function. It retards chemotaxis of leuko- Anorectal cytes to the site of inflammation, neutrophil adhesion, Ischiorectal or perianal or intersphincteric abscess and intracellular oxidative destruction of pathogens. Rectal mucosal biopsy Wound healing is also retarded due to defective epithe- Banding of hemorrhoids Anal dilatation lialization and collagen deposition (Hejase et al. 1996; Cancer of sigmoid or rectum Nisbet and Thompson 2002). Apart from hyperglyce- Diverticulitis mia, diabetic patients also have microvascular disease, Rectal perforation by foreign body which contributes significantly to the pathogenesis. Al- Ischemic colitis though diabetes mellitus increases the risk for develop- Anal stenosis ment of Fournier’s gangrene, it does not increase the Cutaneous mortality (Baskin et al. 1990; Benizri et al. 1996; Hejase Hidradenitis suppurativa Folliculitis et al. 1996; Yeniyol et al. 2004). Scrotal pressure sore Chronic alcoholism, malnutrition, liver cirrhosis, Post-scrotal surgery wound infection poor personal hygiene, and personal neglect are quite Cellulitis of scrotum common in patients with Fournier’s gangrene (Benizri Pyoderma gangrenosum Femoral access for intravenous drug users et al. 1996; Hejase et al. 1996; Yeniyol et al. 2004). Other conditions causing depressed immunity that may pre- Retroperitoneal causes Psoas abscess dispose to the development of Fournier’s gangrene in- Perinephric abscess clude chronic steroid use, organ transplantation, che- Appendicitis and appendix abscess motherapy for malignancies such as leukemia, as well Pancreatitis with retroperitoneal fat necrosis as HIV infection (Paty and Smith 1992; Elem and Ran- Other jan 1995; Heyns and Fisher 2005). Inguinal hernia repair The rising incidence of HIV is paralleled by a rising Filariasis in endemic areas incidence of Fournier’s gangrene, especially in Africa. Strangulated Richter hernia Fournier’s gangrene may be the first presenting condi- tion in patients with HIV infection (McKay and Waters vaginal delivery, septic abortion, hysterectomy, and 1994; Elem and Ranjan 1995; Roca et al. 1998; Heyns Bartholin and vulval abscess (Roberts and Hester 1972; and Fisher 2005). Risk factors include a CD4 count un- Addison et al. 1984). der 400, chemotherapy for Kaposi’s sarcoma, and fem- A prominent feature of patients with Fournier’s gan- oral access for the administration of intravenous drugs. grene is that most of them have an underlying systemic HIV-positive patients with Fournier’s gangrene pre- disorder causing vascular disease or suppressed immu- sent at a younger age and have a wider spectrum of nity, which increases their susceptibility to polymicro- causative bacteria (McKay and Waters 1994). 52 6Fournier’sGangrene 6.3 theglansandproximallywiththesuspensoryligament Anatomy and crura of the penis. Camper’s fascia is the loose areolar fascial layer deep The pelvic outlet can be divided into anterior and pos- totheskinoftheabdominalwall,butsuperficialto terior triangles by drawing a line between the ischial Scarpa’sfascia.TogetherwithScarpa’sfasciaitiscon- tuberosities with the symphysis pubis and coccyx being tinuous with Colles’ fascia inferomedially. the apices (Fig. 6.1). Urogenital causes of Fournier’s Scarpa’s fascia lies deep to Camper’s fascia, covering gangrene lead to initial involvement of the anterior tri- the muscles of the anterior abdominal wall and thorax. angle, whereas anorectal causes primarily involve the It terminates at the level of the clavicles. posterior triangle. The perineal membrane lies deep to Colles’ fascia. It The five fascial planes that can be affected are: Col- is triangular in shape and lies between the pubic rami les’fascia,dartosfascia,Buck’sfascia,Scarpa’sfascia, from the symphysis pubis to the ischial tuberosities. It and Camper’s fascia. has a distinct posterior border, with the central perine- Colles’ fascia is the fascia of the anterior triangle of al tendon in the midline. Colles’ fascia terminates in the perineum. Laterally it is attached to the pubic rami this posterior border. and fascia lata, posteriorly it fuses with the perineal The central perineal tendon (or perineal body) lies membrane and perineal body, and anterosuperiorly between the anus and bulbar urethra. It serves as an at- it is continuous with Scarpa’s fascia (Smith et al. 1998). tachment for the various perineal muscles and helps