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html Diagnosis: Fournier's gangrene Discussion: Fournier's gangrene (FG) is an uncommon, rapidly progressive infection of the genital, perineal, and perianal regions. It is characterized by a synergistic necrotizing fasciitis leading to thrombosis of small subcutaneous vessels and development of gangrene.1 ean !lfred Fournier, a French venereologist, described this syndrome in 1""# as gangrene of the penis and scrotum in young healthy males, $hich progresses rapidly and $ithout apparent cause. %oday, the disease is not limited either to young individuals or to males, and the cause of the infection can usually be identified.& %here is no reported incidence' ho$ever, fe$er than ()) cases of FG have been described in the literature.#,* Females are less commonly affected than males. +tephens et al report that 1*, of their cases occurred in females.# -hildren are even less commonly affected, $ith only (. cases reported in the literature. FG can occur from the neonatal period to adolescence, $ith .., of the cases in children three months old or younger.&,( %he ultimate source of the invasive infection can often be localized to the colorectal or genitourinary tracts (%able 1). -ommon initiating causes include perianal, perirectal, and ischiorectal abscesses' fissures' urethral strictures $ith urine e/travasation' lo$er genitourinary (G0) tract anomalies' and chronic urinary tract infections.& FG has also been reported as a result of rectal and G0 trauma follo$ing instrumentation and surgical procedures.# +uperficial soft tissue in1uries of the perineum account for the remainder of the causes. In $omen, FG may occur from septic abortions, vulvar and 2artholin's gland abscesses. It has also been documented follo$ing procedures such as hysterectomy, pudendal nerve bloc3, and episiotomy.1 -auses in children include trauma, insect bites, circumcision, burns, perirectal diseases, and systemic infection.& 4ost patients $ho develop FG have underlying co5morbid disease, particularly diabetes mellitus. 6ther contributing factors include alcoholism, advanced age, malignancy, corticosteroid therapy, and malnutrition.1,& Interestingly, there have only been four reported cases of FG in association $ith 7I8 infection and !I9+. %he first case $as described in 1::1 by 4urphy et al and occurred in an 7I85positive male $ho in1ected intravenous drugs into his femoral veins.. %$o of the other cases resulted post5 operatively, the last in a patient receiving chemotherapy for ;aposi's sarcoma.<,",: 7I8 infection appeared to be the ma1or ris3 factor in each case. Clinical Presentation: 4ost patients see3 medical attention an average of five days after the onset of symptoms.& =atients usually complain of perineal pain and s$elling. >arly in the disease, the pain may be out of proportion to physical findings. %he s3in overlying the infection may range from normal to gangrenous and under represents the severity of the underlying disease. ! feculent odor characteristic of anaerobic infections may be present. -repitus is often present, but the absence of crepitus does not rule out FG.1 +ystemic findings include fever, tachycardia, dehydration, leu3ocytosis, thrombocytopenia, anemia, and hypocalcemia. ?eu3ocytosis, a common feature, may be absent in patients $ith underlying chronic illness.1,1) -hildren often appear nonto/ic and healthy despite

advanced infection and pyre/ia. !ll patients $ith fever or suspected sepsis re@uire a thorough genitalia e/amination to identify potential perineal sources of infection. Differential diagnosis: %he differential diagnosis of a s$ollen, painful scrotum includes testicular torsion, testicular fracture, and hematoma. Infectious causes include testicular abscess, epididymitis, and gas5containing scrotal abscess.1 Gastrointestinal disorders include inguinal or scrotal hernia $ith bo$el strangulation or scrotal emphysema secondary to bo$el perforation. ! rare cause of a s$ollen, painful scrotum is e/tension of subcutaneous emphysema from pulmonary barotrauma or pneumothora/.1# %he differential diagnosis in children includes cellulitis, balanitis, orchitis, epididymitis, torsion, hydrocele, strangulated hernia, benign scrotal edema, and various s3in diseases. ! convenient scheme is to divide the differential diagnosis into procedural or pathologic causes (table 1).1# Radiographic e aluation: %he radiographic evaluation of the patient $ith a painful, s$ollen scrotum $here the diagnosis in not obvious includes plain film, duple/ ultrasound, and -% scan of the pelvis. =lain radiographs can sho$ subcutaneous air. 0ltrasonography can help to differentiate the causes of acute scrotum especially $hen color 9oppler is utilized.1,& -omputed tomography can delineate the e/tent of disease spread, as $ell as help identify an intra5abdominal source, but is not mandatory to ma3e the diagnosis or initiate treatment.11 !nfectious agents: FG in adults is usually a polymicrobial infection and involves an average of four organisms.& %hese organisms result in an aggressive, virulent mi/ed infection that is rapidly progressive. >scherichia coli is the predominant aerobe $hile bacteroides is the predominant anaerobe. 6ther pathogens include proteus, enterococcus, anaerobic streptococci, and clostridium. In contrast, FG in children is most commonly caused by streptococci and staphylococci.( It should be noted that crepitus does not imply that clostridial gas gangrene is present.1 "anagement: FG is a true surgical emergency that is rapidly progressive and potentially lethal. 6nce FG is recognized, the emergency physician should prepare the patient for urgent surgical intervention $ith aggressive resuscitation and high dose parenteral antibiotics. Immediate urological consultation for debridement is re@uired. Aepeat trips to the operating room for debridement are often needed. Fluid and electrolyte deficits need to be aggressively replaced. %etanus immunization should be given if soft tissue in1ury is present. 2road spectrum antibiotics should be initiated. Initial choices include ampicillinBsulbactam, ticarcillinBclavulanate, piperacillinBtazobactam, or imipenem, or a penicillinase5resistant, synthetic penicillin plus clindamycin plus an antipseudomonal aminoglycoside.1* 7yperbaric o/ygen therapy has been used, but its role in treating non5clostridial infections is uncertain.* Indications for hyperbaric therapy includeC failure of ma/imal conventional management, evidence of -lostridial infection, and the presence of subcutaneous emphysema, myonecrosis or deep tissue involvement.&

+uprapubic catheterization is recommended if the G0 tract is the source of infection, especially if there is urethral trauma or stricture present.*,1) >ven $ith prompt surgical intervention mortality rates range from " to .<, and depend on the patient's age and underlying medical conditions.1 %he mortality rate in children is reported to be nine percent.( Clinical Pearls: 1. FG is a necrotizing soft tissue infection of the perineum or genitalia that is rapidly progressive, potentially lethal and is a true surgical emergency. &. 6nce FG is recognized, patient preparation for urgent surgical intervention is mandatory. #. !ggressive fluid resuscitation and broad spectrum antibiotics should be administered in the >9 to patients $ith FG. *. !ll patients $ith fever and possible sepsis should have a thorough genital e/amination to identify potential perianal sources of infection as FG may have serious underlying disease $ith minimal cutaneous physical findings. (. FG can affect all age groups and both se/es, and a predisposing condition, most commonly diabetes, is usually present. .. >ven $ith aggressive surgical debridement, the mortality rate is as high as .<,. Photographic criti#ue b$ "ichael "orris Figure 1 clearly demonstrates the patient's presenting physical findings (s$elling, discoloration, superficial ulceration). %he lighting techni@ue (high, upper left lighting angle) employed provides ade@uate, uniform illumination to sho$ the s$elling and discoloration of the scrotum. %he distracting elements in the image have been mas3ed $ith sheets and the photographer has included enough anatomical landmar3s so that the vie$er can readily identify the sub1ect matter. %he only improvements that can be suggested $ould be to move in closer so as to fill the frame $ith the sub1ect and to position the sub1ect in a more DstandardD anatomical vie$. -linical photographs of the genitalia are difficult, even in the best circumstances. %he patient's level of discomfort, both physically and emotionally, can ma3e the photographic e/perience trying for the patient and the photographer. Great care and sensitivity should be employed $hen ma3ing such photographs. %he use of a chaperone (of the patient's same se/) is strongly recommended and is helpful for patient positioning and draping. -lothing, sheets, dressings, and catheters can be distracting $hen included in the photograph, but are difficult to remove from the image. Ehenever possible, remove all such distracting elements from the field to be photographed to ma3e a DcleanerD image. R%F%R%&C%' 1. ?auc3s ++. Fournier's gangrene. +urg -lin F !m. 1::*' <*C 1##:51#(&.

&. =aty A, +mith !9. Gangrene and Fournier's gangrene. 0rol -lin F !m 1::&' 1:C1*:5 1.&. #. +tephens 2 , ?athrop -, Aice E%, Gruenberg -. Fournier's gangreneC historic (1<.*5 1:<") versus contemporary (1:<:51:"") differences in etiology and clinical importance. !m +urgeon 1::#' (:C1*:51(*. *. +alvino -, 7arford F , 9obrin =2. Fecrotizing infections of the perineum. +o 4ed 1::#' ".C:)"5:11. (. !dams A r., 4ata !, 8enable 99, -ul3in 9 , 2occhini ! r. Fournier's gangrene in children. 0rology 1::)' #(C*#:5**1. .. 4urphy 4, 2uc3ley 4, -orr , 8inayagamoorthy +, Grainger A, 4ulcahy F4. Fournier's gangrene of scrotum in a patient $ith !I9+. Genitourin 4ed 1::1' .<C##:5 #*1. <. Felson 4A, -artledge , 2arton +>, Gazzard 2G. Fournier's gangrene follo$ing hyfrecation in a male infected $ith the human immunodeficiency virus. Genitourin 4ed 1::&' ."C *)15*)&. ". 4c;ay %-, Eaters E2. Fournier's gangrene as the presenting sign of an undiagnosed human immunodeficiency virus infection. 0rol 1::*' 1(&C1((&51((*. :. 7ughes59avies ?%, 4urray =, +pittle 4. Fournier's gangreneC a hazard of chemotherapy in !I9+ (letter). -lin 6ncol A -oll Aadiol 1::1'#C&*1. 1). 2as3in ?+, -arroll =A, -attolica >8, 4c!ninch E. Fecrotizing soft tissue infections of the perineum and genitalia. 2r 0rol 1::)' .(C(&*5(&:. 11. !mendola 4!, -asillas , oseph A, !ntun A, Galindez 6. Fournier's gangreneC -% findings. !bdom Imaging 1::*' 1:C*<15*<*. 1&. +ussman + , +chiller A=, +hashi3umar 8?. Fournier's syndrome. Aeport of three cases and revie$ of the literature. !m 9is -hild 1:<"'1#&C11":511:1. 1#. Firman A, 7eiselman 9, ?loyd %, 4ardesich =. =neumoscrotum. !nn >merg 4ed 1::#' &&C1#(#51#(.. 1*. +anford =, Gilbert 9F, +ande 4!. %he +anford guide to antimicrobial therapy. 9allas, !ntimicrobial %herapy, Inc., 1::(, p #<. Table 1 Causes of Fournier's gangrene (enitourinar$ Pathological Infection %rauma 0rethral stricture =eriurethral (astrointestinal !norectal abscess Ischiorectal abscess =erianal fistula 7emorrhoids =erforated rectal carcinoma )bstetrical/($necological =ost5coital 8ulvar abscess 2artholin's gland abscess +eptic abortion

abscess >pididymitis =araphimosis +oft tissue in1uries =ost5coital Procedural =ost5operative %0A= %raumatic catheterization

Aectal perforation by foreign body =erforated sigmoid diverticulitis

!noscopyB+igmoidoscopy -olonoscopy =ost5operative !nastomotic dehiscence

7ysterectomy -ervicalB=udendal nerve bloc3 =ost5operative >pisiotomy =elvic e/enteration

Figure *egend Figure 1 %he patient's scrotum on arrival to the >9. Figure + =elvic radiograph sho$ing gas collection in left buttoc3 area as indicated by arro$s. ;ey $ordsC scrotum, edema, gangrene, genitalia, necrotizing fasciitis Photo 1