PICU Faculty Advocate Children's Hospital, United States
Introduction: Fournier’s Gangrene (FG) is a rare disorder of rapidly progressive necrotizing infection of the perineum and genitalia, with an incidence of 1.6 cases per 100,000. Mortality is reported to be as high as 20-40% likely due to indolent presentation and late diagnosis. Most cases are reported in adults. We describe a case of Fournier’s Gangrene in an adolescent that was successfully treated.
Description: A 17-year-old male with repaired myelomeningocele, paraplegia with sensory loss, multiple urethral reconstruction surgeries for neurogenic bladder, Mitrofanoff, and chronic indwelling penile foley catheter, presented to the emergency room with fever, nausea, vomiting and cellulitis in perianal area. Computerized tomography revealed a large pocket of fluid and gas within the perineum, consistent with FG. There was evidence of urethral tear, and the urethral catheter tip was in the pocket of fluid. There was an extension of the infection to the hip joints. He received fluid resuscitation, broad spectrum antibiotics and was placed on vasopressors for septic shock. He underwent an extensive debridement of the perianal, and pelvic area and hip joints. Fragments of pelvic bone were noted in the cavity. Cystoscopy revealed discontinuation of bladder neck with penile urethra. Despite urinary diversion via a catheter through the Mitrofanoff and bilateral percutaneous nephrostomies, he continued to leak urine through the open bladder neck into the pelvic cavity. Rectal wall involvement necessitated a colostomy. Postoperatively, inotropes were weaned off on day 2 and he was extubated on day 4. His infection was polymicrobial with Proteus mirabilis, Klebsiella pneumoniae, and Enterococcus faecalis. He subsequently needed multiple debridements and bladder neck repair followed by muscle flap coverage and wound closure.
Discussion: This patient’s admission FG Severity Index Score was 15, predicted mortality is more than 75% for a score >9. This patient survived initial septic shock. This case highlights the importance of prompt identification and management of FG which likely contributed to his survival. Lack of pain sensation may have led to delayed presentation in this case with resultant extensive tissue loss and long-term morbidity.
