Клініко-морфологічні прояви гангрени Фурньє: літературний огляд та власне спостереження

Abstract

Вивчення рідкісних захворювань різного ґенезу дає можливість удосконалити їх діагностику, лікування, прогноз та попередити виникнення ускладнень. Гангрена Фурньє – це специфічна форма некротизуючого фасциїту з прогресуючим некрозом тканин статевих органів і промежини внаслідок синергічної полімікробної інфекції. Патоморфологічні зміни при ГФ описані в незначній кількості публікацій, що відображають дані біопсій або післяопераційного матеріалу з наявністю патогномонічних та неспецифічних проявів. Наведено випадок летального випадку гангрени Фуріньє з патоморфологічною верифікацією. ГФ навіть у сучасних умовах при ранньому виявленні хвороби залишається захворюванням з високим рівнем летальності. Прогноз при ГФ, в першу чергу, залежить від поширеності процесу, етіології та термінів надання медичної допомоги. При діагностиці даного захворювання на ранній стадії необхідно звертати увагу на патогномонічні макроскопічні та мікроскопічні прояви хвороби, що допоможе вчасно постановити діагноз та, відповідно, надати невідкладну хірургічну допомогу.; The study of rare diseases of various geneses enables to improve their diagnosis, treatment and prognosis, preventing its complications. One of such disease is Fournier’s gangrene, which mortality rate is accounted for 31% to 80% depending on the area, localization of lesions and development of complications. Fournier’s gangrene (FG, Fournier’s disease, spontaneous gangrene of the scrotum, subfascial phlegmon and gangrene of the genitalia, epifascial necrosis, gangrenous inflammation of the scrotum, fulminant gangrene of the scrotum) is a specific form of necrotizing fasciitis with progressive necrosis of the tissues of genitalia and perineum as a result of synergistic polymicrobial infection. The etiology of the FG is unknown. The pathogen of the disease and the site of entry is managed to be identified in 95-97% of cases. If the cause of the disease is non-identifiable, then Fournier’s gangrene is considered idiopathic. There is a dearth of publication describing pathomorphological changes in FG, reporting about the findings of biopsy or postoperative material study. Early and late pathomorphological changes are distinguished according to the clinical course of the disease. We report a lethal case of Fournier's gangrene with pathomorphologic verification. The 54-year-old patient L. presented to the Surgical Unit of Central Clinical Hospital with the admission diagnosis of Fournier’s phlegmon, bedsore of the upper third of the femur, SIRS, intoxication. Based on the physical examination and results of the laboratory test a clinical diagnosis of Fournier's gangrene, fast-progressive form with the lesions of the scrotum and putrid lesions of soft tissue, intoxication, was made. The patient underwent urgent surgery on life indications: necrectomy of scrotum tissues and penis, sanation and drainage of abscess, necrectomy, sanation and drainage of bedsore. Epicystostomy was made with regard to the defect of the urethra. The bacteriological study of the postoperative material revealed Еscherichia coli and hemolytic Acinetobacter. Despite the surgery and conservative treatment the overall state of the patient was critical and he died within 24 days. Pathomorphological study of the autopsy material confirmed the clinical diagnosis: the underlying disease is phlegmon of the perineum, scrotum and penis. The underlying disease was complicated with marked parenchymal dystrophy of internal organs, pulmonary edema, brain edema, bedsores of the lumbosacral area, bacteriemia. Consequently, intoxication was the direct cause of death. Therefore, Fournier's gangrene remains a potentially life-threatening disease that is accompanied by toxemia and bacteremia with a high mortality rate. A widespread form of FG with fulminant and fast progressive clinical course, significant lesions of the scrotum and penis, was revealed in the deceased. The close relationship of the fascial spaces of the scrotum, penis, medial surfaces of the femur contribute to the rapid spread of infection to the adjacent anatomical areas. The patient was hospitalized in the full-scaled clinical stage of the FG, which corresponded to the advanced stage of pathomorphological manifestations, thus, with no difficulties in the diagnosis. However, apparently, combination of pathogens causing the disease and its antibiotic resistance played a key role in lethal outcome. To sum it up, to date, Fournier's gangrene is considered a disease with high level of lethality even at early detection of the disease. The prognosis for FG primarily depends on the prevalence of the process, etiology and terms of providing medical care. Noteworthy, pathognomonic macroscopic and microscopic manifestations are crucial in the early diagnosis of the disease, providing with prompt diagnosis and surgical care.