Fournier's gangrene in a woman with chronic neutropenia presenting at 12 weeks of pregnancy: a case report and literature review

Abstract

The management of Fournier's gangrene in pregnancy in a woman with chronic idiopathic neutropenia is described. A 36-year-old pregnant woman was admitted at 12 weeks of gestation with sepsis, high fever, severe anemia requiring transfusions, and a perianal necrotic area approximately 10 cm in diameter extending to the perineum, consistent with Fournier's gangrene. She required both surgery and medical therapy. Surgery included a laparoscopic defunctioning sigmoid loop colostomy and perineal debridement. The skin and mucous membranes of the perineum were cleansed daily using water irrigation, decontamination and disinfection, and the vagina and rectum were irrigated with antiseptic and antifungal solutions. The necrotic areas were removed. Granulocyte colony-stimulating factor and erythropoietin were administered to restore normal levels of white blood cells and hemoglobin. Vital parameters, hematological values, and clinical examination of the perineum showed progressive improvement during treatment, and complete restoration of the perineum was achieved. The patient successfully carried the pregnancy to term. In conclusion, key factors for success were treatment of the sepsis, loop colostomy, granulocyte growth factor therapy and cleansing of the anus and perineum.

Keywords: Chronic idiopathic neutropenia; Fournier's gangrene; Pregnancy; Sepsis.

Fig. 1

Physical examination on admission at the tertiary center. Physical examination revealed a large necrotic area around the anus, extending to the perineum, which was covered with semi-liquid feces. After cleansing, the lesion affected 5 cm of the rectal mucosa.

Fig. 2

The histological examination of an excisional biopsy from the anal mucosa and perianal ulcer revealed areas of ulcerated skin exhibiting epidermal hyperplasia, characterized by acanthosis and periulcerative papillomatosis. There was a notable chronic infiltration of lymphocytes and monocytes in the dermoepidermal region, alongside an acute response of granulocytes primarily located in the hypodermic area, which resembled an abscess.

Fig. 3

Histological examination of bone marrow biopsy. A-B) Haematoxylin and eosin staining: hypocellulated marrow by age; present all the asset series in various stages of maturation; mild degeneration of the stroma; C) Myeloperoxidase staining, 40×: slightly reduced mature granulopoiesis share; D) CD33 staining 40×: increase of granulopoiesis in various maturation phases in left shifting; E) E-cadherin staining, 40×: erythropoiesis present with a share of mature forms and presence of erythroid precursors: F) CD61 staining, 40×: megakaryocytes mostly of reduced volume with hypolobate nuclei and other mature elements with hypersegmented nuclei.

Fig. 4

Physical examination showed the complete healing of the lesions.

Fig. 5

Physical examination at 24 weeks of gestation. The patient experienced sudden-onset perineal cellulitis that extended to the groin, with perianal and vulvar lesions displaying signs of hyperkeratosis.

Fig. 6

Examination on the day of the cesarean section and a month later.

Supplementary Fig. 1

Evolution of the perineal cellulitis occurred during the 26th week of pregnancy, through the following weeks.

Supplementary Fig. 2

Evolution of the perineal cellulitis occurred during the 26th week of pregnancy, through the following weeks.

Supplementary Fig. 3

Evolution of the perineal cellulitis occurred during the 26th week of pregnancy, through the following weeks.