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On gross pathology, Fournier's gangrene is characterized by:
On gross pathology, Fournier's gangrene is characterized by:
*Subcutaneous emphysema
*Subcutaneous emphysema
*Swollen scrotal wall
*Edema
*Edema
*Erythema
*Erythema

Revision as of 19:48, 13 October 2016

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Steven C. Campbell, M.D., Ph.D.; Associate Editor(s)-in-Chief: Yamuna Kondapally, M.B.B.S[1]; Jesus Rosario Hernandez, M.D. [2]

Synonyms and keywords: Idiopathic gangrene of scrotum; Periurethral phlegmon; Streptococcal scrotal gangrene; Genito-perineal gangrene

Template:Search infobox

Fournier gangrene
ICD-10 N49.8 (ILDS N49.81), N76.8
ICD-9 608.83
DiseasesDB 31119
MeSH D018934

Overview

Fournier gangrene is a type of necrotizing infection (gangrene) of the perineal, genital or perianal regions usually affecting the male genitals but can also occur in female and children.[1] It is a fulminant form of necrotizing fasciitis. It was first described by Baurienne in 1764 and is named after a French venereologist, Jean-Alfred Fournier following five cases he presented in clinical lectures in 1883.[2][3]

Historical Perspective

  • Fournier's gangrene was first described by in 1764 by Baurienne.[2]
  • The detailed description of fournier's gangrene was given by Jean Alfred Fournier, who is a French venereologist in 1883.[3]

Classification

There is no classification system established for Fournier's gangrene. [4]

Pathophysiology

  • The transmission of pathogens occurs through the following routes:[5]

subcutaneous fat, nerves, arteries, and veins.

  • Superficial skin and deeper muscles are typically spared.
  • In late stages, lesions develop liquefactive necrosis at all tissue levels.

Pathogenesis

  • The pathogenesis of fournier's gangrene is the result of an imbalance between bacterial and host factors.[6][7][5]
  • Host factors: Decrease in host immunity provides favourable environment to initiate the infection
  • Bacterial factors: Virulence and synergism between multiple bacteria promotes rapid spread of infection
  • The aerobic and anaerobic bacteria produce exotoxins and enzymes like collagenase, heparinase, hyaluronidase etc which promote spread of infection.
  • The aerobic bacteria cause acceleration of coagulation by promoting platelet aggregation and complement fixation and the anaerobic bacteria produce collagenase and heparinase that promote the formation of clots leading to obliterative endarteritis.
  • The cutaneous and subcutaneous vascular necrosis develops leading to local ischemia and further bacterial proliferation.
  • The infection spreads from superficial (colles fascia) and deep fascial planes of genetalia to the overlying skin sparing the muscles. The infection then spreads from colles fascia to the penis and scrotum via Buck's and Dartos fascia or to the anterior abdominal wall via Scarpa's fascia or vice versa.
  • The inferior epigastric and deep circumflex iliac arteries (supply anterior abdominal wall), and external and internal pudendal artery supply the scrotal wall. Except internal pudendal artery, each of these vessels travels with in Camper's fascia and can therefore become thrombosed in the progression of fournier's gangrene.
  • The progression of infection to the perineal body, urogenital diaphragm and pubic rami is limited due to perineal fascia.[8]
  • Because of direct supply of blood from aorta, testicular involvement is limited in Fournier's gangrene.[9] However involvement of testis suggests retroperitoneal origin or spread of infection.[10]
  • Fournier's gangrene of the male genetalia spares testes, urethra and deep penile tissues while the skin sloughs off.[11]
  • Sepsis and multiorgan failure is the most common cause of death in Fournier's gangrene.

Gross pathology

On gross pathology, Fournier's gangrene is characterized by:

  • Subcutaneous emphysema
  • Swollen scrotal wall
  • Edema
  • Erythema
  • Bullae
  • Skin sloughing

Microscopic histopathological analysis

On microscopic histopathological analysis, the characteristic findings of Fournier's gangrene are:

  • Early stages
  • Late stages
  • Histology of excised skin shows full thickness, featureless, coagulative necrosis with no viable tissue remaining
    Histology of excised skin shows full thickness, featureless, coagulative necrosis with no viable tissue remaining
  • Histology of excised scrotal skin shows zones of neutrophil polymorph infiltration, with embedded foreign material possibly representing hair shafts
    Histology of excised scrotal skin shows zones of neutrophil polymorph infiltration, with embedded foreign material possibly representing hair shafts
  • Histology of excised skin shows bacterial colonies (cocci and bacilli) in the superficial zone
    Histology of excised skin shows bacterial colonies (cocci and bacilli) in the superficial zone

Causes

Fournier's gangrene is caused by mixed aerobic and anaerobic organisms which normally exist below the pelvic diaphragm in the perineum and genitalia.[4] Fournier's gangrene may be caused by the following organisms:[12]

Bacteria

Aerobic organisms Most common aerobic organisms are:[13]

Anaerobic organisms Most common anaerobic organisms are:

Other organisms

Idiopathic

Less than quarter of cases of fournier's gangrene are idiopathic.[1][17]

Differentiating Fournier gangrene from Other Diseases

Fournier's gangrene must be differentiated from other diseases that cause pain, swelling erythema, discharge and raised temperature such as:[18][5]

  • Scrotal abscess
  • Herpes simplex
  • Cellulitis
  • Strangulated hernia
  • Streptococcal necrotizing fasciitis
  • Gonococcal balanitis and edema
  • Vascular occlusion syndromes
  • Allergic vasculitis
  • Pyoderma gangrenosum
  • Necrolytic migratory erythema
  • Ecthyma gangrenosum
  • Warfarin necrosis
  • Polyarteritis nodosa

Epidemiology and Demographics

Incidence

Only 600 cases of Fournier gangrene were reported in the world literature in the ten years since 1996, with most patients in their 60s or 70s with other concurrent illnesses.[19] However, Fournier's gangrene is not a reportable illness, and the condition is not uncommon, especially among diabetic individuals. A similar infection in women has been occasionally described.[20]

In Turkey it was reported that 46% of patients had diabetes mellitus[21] whilst other studies have identified approximately a third of patients having either diabetes, alcoholism or malnutrition, and 10% having medical immunosuppression (chemotherapy, steroids, malignancy).[22]

Age

Fournier's gangrene affects individuals of all ages but commonly affects individuals older than 50 years of age.[5]

Gender

Men are more commonly affected with fournier's gangrene than women.[3]

Mortality

  • Mortality rate decreases with early aggressive treatment.
  • The mortality rate of Fournier's gangrene is between 20% to 80%. Higher mortality rates are found in daibetics, alcoholics and those with colorectal sources of infection.[23]

Risk Factors

Common risk factors in the development of fournier's gangrene are:[24][25][17]

  • Comorbid systemic disorders
  • Age>50 yrs
  • Male Gender
  • Diabetes mellitus
  • Alcohol misuse
  • Immunosupression
  • Chemotherapy
  • Chronic corticosteroid use
  • HIV
  • Leukemia
  • Liver disease
  • Debilitating illness
  • Malignancy
  • Cytotoxic drugs

The most common foci of fournier's gangrene include:[4][26]

Anorectal Genitourinary Dermatology Gynaecological
  • Trauma
  • Steroid enemas for radiation proctitis
  • Hemorrhoidectomy
  • Anal fissures excision
  • Diverticulitis
  • Colonic perforations
  • Ischiorectal, perirectal, or perianal abscesses
  • Appendicitis
  • Steroid enemas for radiation proctitis
  • Trauma
  • Cancer invasion to external genitalia
  • Hemipelvectomy
  • Epididymitis or orchitis
  • Penile artificial implant or a foreign body
  • Hydrocele aspiration
  • Genital piercing
  • Intracavernosal cocaine
  • Urethral catheterization or instrumentation
  • Penile implantsinsertion
    Prostatic biopsy
  • Vasectomy
  • Urethral strictures with urinary extravasation
  • Blunt perineal trauma
  • Intramuscular injections
  • Genital piercings
  • Scrotal furuncle
  • Perineal or pelvic surgery/inguinal herniography
  • Episiotomy wound
  • Infected bartholins gland
  • Septic abortion
  • Genital mutilation
  • Coital injury

Neonates and Children

  • Trauma[26]
  • Burns
  • Insect bites
  • Circumcision

Screening

According to the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Fournier's gangrene.

Natural History, Complications, and Prognosis

Natural history

  • If left untreated, the acute inflammatory changes spread quickly, accompanied by high fever and extreme weakness.[27][28]
  • The overlying skin becomes smooth, tense and shiny. Diffuse erythema without distinct borders is seen.
  • First 1 or 2 days, the lesions develop with progressive colour changes from red to purple to blue and then becomes frankly gangrenous, first turning black, then greenish yellow.
  • If the patient has survived, a line of demarcation between viable and necrotic tissue would become sharply defined from days 7 to 10.
  • Sloughing of necrotic skin would reveal the underlying pus and extensive liquefactive necrosis of subcutaneous tissues, which will be significantly more extensive than would be suspected with the overlying area of necrotic skin.
  • Metastatic abscesses and pulmonary distress may develop as well.

Complications

Common complications of Fournier's gangrene include:

  • Auto-amputation of the penis
  • Sepsis
  • Disseminated intravascular coagulation
  • Tetanus
  • Marjolin's ulcer
  • infertility (rare complication)

Prognosis

Depending on the underlying comorbidities, the prognosis of Fournier's gangrene varies. Few of the prognostic factors include:

  • Severe sepsis
  • Extent of necrosis
  • <3% of body surface area rarely die
  • ≥5% of body surface area have worse prognosis

Diagnosis

The diagnosis is primarily based on clinical findings.

Diagnostic Criteria

The Uludag Fournier's gangrene severity index

Physiologic Variables High Abnormal Values Normal Low Abnormal Values
+4 +3 +2 +1 0 +1 +2 +3 + 4
Temperature >41 39-40.0 38.5-39 36-38.4 34-35.9 32-33.9 30-31.9 <29.9
Heart Rate >180 140-179 110-139 70-109 55-69 40-54 <39
Respiratory Rate >50 35-49 25-34 12-24 10-11 6-9 <5
Serum Sodium (mmol/L) >180 160-179 155-159 150-154 130-149 120-129 111-119 <110
Serum Potassium (mmol/L) >7 6-6.9 5.5-5.9 3.5-5.4 3-3.4 2.5-2.9 <2.5
Serum Creatinine
(mg/100/ml*2 for acute renal failure) 		>3.5 2-3.4 1.5-1.9 0.6-1.4 <0.6
Hematocrit >60 50-59.9 46-49.9 30-45.9 20-29.9 <20
WBC (Total/mm*1000) >40 20-39.9 15-19.9 3-14.9 1-2.9 <1
Serum Bicarbonate (Venous,mmol/l) >52 41-51.9 32-40.9 22-31.9 18-21.9 15-17.9 <15
  • Score >10.5 indicates 96% probability of death[29]
  • Score ≤10.5 indicates 96% probability of survival

According to Loar and colleagues, the severity of Fournier's gangrene is:

  • Score ≥9 indicates 46% probability of death
  • Score <9 indicates 96% probability of survival


Laboratory risk indicator for necrotizing fasciitis (LRINEC) scoring system

  • LRINEC is a diagnostic scoring system used to distinguish necrotizing fasciitis from other soft tissue infections.[30][5]
  • It was first established by Wong et al in 2004.
  • Risk assessment of necrotizing faciitis using LRINEC score:
  • Low risk: ≤5
  • Intermediate risk: 6-7
  • High risk: ≥8
Variable Score
C reactive protein (mg/dL)

<150
>150

0
4

Total white blood cell count (/mm3)

<15
15-25
>25


0
1
2

Hemoglobin (g/dL)

<13.5
11-13.5
<11


0
1
2

Sodium (mmol/L)

≥135
<135


0
2

Creatinine (μmol/L)

<141
>141


0
2

Glucose (mmol/L)

<10
>10


0
1

History

Symptoms

The symptoms of Fournier's gangrene include:[5][31]

  • Pain and selling in the scrotum
  • Erythema
  • Discoloration of involved skin
  • Purulence or wound discharge
  • Pallor
  • Fever>38°C
  • Crepitation
  • Prostration
  • Fluctuance
  • Fetid odour

Physical examination

Gallery

Genitourinary system

Laboratory Findings

Laboratory findings consistent with the diagnosis of Fournier's gangrene include:

  • CBC with differential count
  • Culture of open wound or abscess
  • Disseminated intravascular coagulation panel
  • Coagulation studies(PT, aPTT, thrombin time etc)
  • Fibrinogen/fibrin degradation product levels
  • Blood culture
  • Urine culture
  • Arterial blood gas analysis
  • Electrolyte panel
  • Blood urea nitrogen (BUN)
  • Creatinine
  • Blood glucose levels

Imaging Findings

The diagnosis of Fournier's gangrene is based on clinical findings.The role of imaging includes:[18]

  • Diagnosis not established
  • Determine the extent of disease
  • Detect underlying cause

Radiography

  • On X-ray, Fournier's gangrene is characterized by:
  • Subcutaneous gas or soft tissue swelling (specific x-ray finding) seen extending from scrotum and perineum to the inguinal regions, anterior abdominal wall, and thighs.
  • Increase in the soft tissue thickness and opacity.
  • Plain x-ray is a poor screening study for Fournier's gangrene because:
  • Subcutaneous emphysema is an insensitive finding and is present in a minority of patients
  • In the early stages the findings are similar to cellulitis

Ultrasound

On ultrasound, Fournier's gangrene is characterized by:[5][32]

  • Thickened scrotal wall
  • Echogenic gas foci in scrotum pathognomonic-Seen as dirty shadowing
  • Testes and epididymi spared (due to their separate blood supply)
  • Reactive unilateral or bilateral hydroceles are present
  • Differentiate Fournier gangrene from inguinoscrotal incarcerated hernia (In inguinoscrotal incarcerated hernia gas is observed in the obstructed bowel lumen, away from the scrotal wall)

Computed tomography

The CT of Fournier's gangrene is characterized by:

  • Soft tissue stranding and fascial thickening
  • Soft tissue gas
  • The extent of disease can be assessed prior to surgery
  • A cause of infection may be apparent(e.g.perineal abscess, fistula)

Gallery of Imaging Findings

  • X ray of Fournier's gangrene [18]
    X ray of Fournier's gangrene [18]
  • Ultrasound of Fournier's gangrene [18]
    Ultrasound of Fournier's gangrene [18]
  • CT of Fournier's gangrene [18]
    CT of Fournier's gangrene [18]
  • CT of Fournier's gangrene with spontaneous perforation of rectal cancer [18]
    CT of Fournier's gangrene with spontaneous perforation of rectal cancer [18]

Other Diagnostic Studies

Treatment

Medical Therapy

Fournier gangrene is a urological emergency requiring intravenous antibiotics and debridement (surgical removal) of necrotic (dead) tissue. Despite such measures, the mortality rate overall is 40%, but 78% if sepsis is already present at the time of initial hospital admission.[21]

Antimicrobial Therapy

  • Fournier gangrene[33]
  • If caused by streptococcus species or clostridia
  • Polymicrobial
  • MRSA (methicillin resistant staphylococcus aureus) suspected

Surgery

Prevention

External links

References

  1. ↑ 1.0 1.1 Smith GL, Bunker CB, Dinneen MD (1998). "Fournier's gangrene". Br J Urol. 81 (3): 347–55. PMID 9523650.
  2. ↑ 2.0 2.1 Nathan B (1998). "Fournier's gangrene: a historical vignette". Can J Surg. 41 (1): 72. PMC 3950066. PMID 9492752.
  3. ↑ 3.0 3.1 3.2 Chennamsetty A, Khourdaji I, Burks F, Killinger KA (2015). "Contemporary diagnosis and management of Fournier's gangrene". Ther Adv Urol. 7 (4): 203–15. doi:10.1177/1756287215584740. PMC 4580094. PMID 26445600.
  4. ↑ 4.0 4.1 4.2 Eke N (2000). "Fournier's gangrene: a review of 1726 cases". Br J Surg. 87 (6): 718–28. doi:10.1046/j.1365-2168.2000.01497.x. PMID 10848848.
  5. ↑ 5.0 5.1 5.2 5.3 5.4 5.5 5.6 Mallikarjuna MN, Vijayakumar A, Patil VS, Shivswamy BS (2012). "Fournier's Gangrene: Current Practices". ISRN Surg. 2012: 942437. doi:10.5402/2012/942437. PMC 3518952. PMID 23251819.
  6. ↑ Morua AG, Lopez JA, Garcia JD, Montelongo RM, Guerra LS (2009). "Fournier's gangrene: our experience in 5 years, bibliographic review and assessment of the Fournier's gangrene severity index". Arch Esp Urol. 62 (7): 532–40. PMID 19815967.
  7. ↑ Shyam DC, Rapsang AG (2013). "Fournier's gangrene". Surgeon. 11 (4): 222–32. doi:10.1016/j.surge.2013.02.001. PMID 23578806.
  8. ↑ Katib A, Al-Adawi M, Dakkak B, Bakhsh A (2013). "A three-year review of the management of Fournier's gangrene presented in a single Saudi Arabian institute". Cent European J Urol. 66 (3): 331–4. doi:10.5173/ceju.2013.03.art22. PMC 3974467. PMID 24707378.
  9. ↑ Gupta A, Dalela D, Sankhwar SN, Goel MM, Kumar S, Goel A; et al. (2007). "Bilateral testicular gangrene: does it occur in Fournier's gangrene?". Int Urol Nephrol. 39 (3): 913–5. doi:10.1007/s11255-006-9126-1. PMID 17323114.
  10. ↑ Chawla SN, Gallop C, Mydlo JH (2003). "Fournier's gangrene: an analysis of repeated surgical debridement". Eur Urol. 43 (5): 572–5. PMID 12706005.
  11. ↑ Campos JA, Martos JA, Gutiérrez del Pozo R, Carretero P (1990). "Synchronous caverno-spongious thrombosis and Fournier's gangrene". Arch Esp Urol. 43 (4): 423–6. PMID 2383054.
  12. ↑ Thwaini A, Khan A, Malik A, Cherian J, Barua J, Shergill I, Mammen K (2006). "Fournier's gangrene and its emergency management". Postgrad Med J. 82 (970): 516–9. PMID 16891442.
  13. ↑ Paty R, Smith AD (1992). "Gangrene and Fournier's gangrene". Urol Clin North Am. 19 (1): 149–62. PMID 1736475.
  14. ↑ Yanar H, Taviloglu K, Ertekin C, Guloglu R, Zorba U, Cabioglu N; et al. (2006). "Fournier's gangrene: risk factors and strategies for management". World J Surg. 30 (9): 1750–4. doi:10.1007/s00268-005-0777-3. PMID 16927060.
  15. ↑ Jensen P, Zachariae C, Grønhøj Larsen F (2010). "Necrotizing soft tissue infection of the glans penis due to atypical Candida species complicated with Fournier's gangrene". Acta Derm Venereol. 90 (4): 431–2. doi:10.2340/00015555-0847. PMID 20574621.
  16. ↑ Tleyjeh IM, Routh J, Qutub MO, Lischer G, Liang KV, Baddour LM (2004). "Lactobacillus gasseri causing Fournier's gangrene". Scand J Infect Dis. 36 (6–7): 501–3. PMID 15307582.
  17. ↑ 17.0 17.1 Vick R, Carson CC (1999). "Fournier's disease". Urol Clin North Am. 26 (4): 841–9. PMID 10584624.
  18. ↑ 18.0 18.1 18.2 18.3 18.4 18.5 Fournier's gangrene https://radiopaedia.org/articles/fournier-gangrene (2016) Accessed on October 12, 2016
  19. ↑ Vaz I (2006). "Fournier gangrene". Trop Doct. 36 (4): 203–4. PMID 17034687.
  20. ↑ Herzog W (1987). "[Fournier gangrene--also in females?]". Zentralbl Chir. 112 (9): 564–76. PMID 2956804.
  21. ↑ 21.0 21.1 Yanar H, Taviloglu K, Ertekin C, Guloglu R, Zorba U, Cabioglu N, Baspinar I (2006). "Fournier's gangrene: risk factors and strategies for management". World J Surg. 30 (9): 1750–4. PMID 16927060.
  22. ↑ Tahmaz L, Erdemir F, Kibar Y, Cosar A, Yalcýn O (2006). "Fournier's gangrene: report of thirty-three cases and a review of the literature". Int J Urol. 13 (7): 960–7. PMID 16882063.
  23. ↑ Moslemi MK, Sadighi Gilani MA, Moslemi AA, Arabshahi A (2009). "Fournier gangrene presenting in a patient with undiagnosed rectal adenocarcinoma: a case report". Cases J. 2: 9136. doi:10.1186/1757-1626-2-9136. PMC 2803933. PMID 20062653.
  24. ↑ Clayton MD, Fowler JE, Sharifi R, Pearl RK (1990). "Causes, presentation and survival of fifty-seven patients with necrotizing fasciitis of the male genitalia". Surg Gynecol Obstet. 170 (1): 49–55. PMID 2294630.
  25. ↑ Morpurgo E, Galandiuk S (2002). "Fournier's gangrene". Surg Clin North Am. 82 (6): 1213–24. PMID 12516849.
  26. ↑ 26.0 26.1 Amendola MA, Casillas J, Joseph R, Antun R, Galindez O (1994). "Fournier's gangrene: CT findings". Abdom Imaging. 19 (5): 471–4. PMID 7950832.
  27. ↑ Morgan MS (2010). "Diagnosis and management of necrotising fasciitis: a multiparametric approach". J Hosp Infect. 75 (4): 249–57. doi:10.1016/j.jhin.2010.01.028. PMID 20542593.
  28. ↑ Ecker KW, Derouet H, Omlor G, Mast GJ (1993). "[Fournier's gangrene]". Chirurg. 64 (1): 58–62. PMID 8436051.
  29. ↑ Kabay S, Yucel M, Yaylak F, Algin MC, Hacioglu A, Kabay B; et al. (2008). "The clinical features of Fournier's gangrene and the predictivity of the Fournier's Gangrene Severity Index on the outcomes". Int Urol Nephrol. 40 (4): 997–1004. doi:10.1007/s11255-008-9401-4. PMID 18563618.
  30. ↑ Wong CH, Khin LW, Heng KS, Tan KC, Low CO (2004). "The LRINEC (Laboratory Risk Indicator for Necrotizing Fasciitis) score: a tool for distinguishing necrotizing fasciitis from other soft tissue infections". Crit Care Med. 32 (7): 1535–41. PMID 15241098.
  31. ↑ Yeniyol CO, Suelozgen T, Arslan M, Ayder AR (2004). "Fournier's gangrene: experience with 25 patients and use of Fournier's gangrene severity index score". Urology. 64 (2): 218–22. doi:10.1016/j.urology.2004.03.049. PMID 15302463.
  32. ↑ Rajan DK, Scharer KA (1998). "Radiology of Fournier's gangrene". AJR Am J Roentgenol. 170 (1): 163–8. doi:10.2214/ajr.170.1.9423625. PMID 9423625.
  33. ↑ Gilbert, David (2015). The Sanford guide to antimicrobial therapy. Sperryville, Va: Antimicrobial Therapy. ISBN 978-1930808843.

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