Work

Anatomical and Functional Damage in Severe Perianal Crohn's Disease

Year: 2024
Type: article
Study design: Case Report
Abstract: Case Presentation Two female patients with Crohn's disease, with onset at ages 18 and 14, are described. The first patient started with ileocolitis, progressing to rectal stenosis despite combined therapy with anti-TNF (azathioprine with infliximab and later, adalimumab). She developed perianal fistulizing Crohn's disease (pCD), with progressive severity, including deep perineal, perianal, vulvar, and intergluteal ulcers, as well as a rectovaginal fistula and multiple ano-genital subcutaneous fistulas. The second patient initially presented with pCD, which later progressed to ascending colitis up to the rectum. She used combined anti-TNF therapy (infliximab and adalimumab) at the onset, but discontinued medication due to poor adherence. There was worsening of the perianal disease, with ano-vulvar destruction. Due to difficulty accessing drugs with other mechanisms of action, low quality of life, and anatomical and functional damage, intestinal diversion was opted for both patients. The first patient accepted the proposed treatment, but there was no improvement in perianal involvement, requiring total proctocolectomy with amputation. After seven months of an open wound, a new intervention for skin resection from the buttocks, local dressings, and hyperbaric chamber therapy resulted in complete healing. After 10 years, new intergluteal ulcers developed, in addition to esophageal Crohn's disease and metastatic intermamary Crohn's disease. The second patient has not yet accepted the diversion or the amputation. Discussion pCD is a severe phenotype affecting 17-43% of patients, and it presents a challenging treatment manifestation. Management of pCD involves both clinical and surgical treatments. Initial surgical approaches are performed via proctological examination under anesthesia to control perineal sepsis with abscess drainage, ulcer and tract curettage, placement of drains, and opening of cavities. Multiple procedures are necessary. However, considering functional and anatomical damage, intestinal diversion can be proposed as a bridge to definitive treatment. In disease progression, lack of response to clinical treatment, and local anatomical failure, rectal amputation becomes necessary. One-fifth of patients with pCD undergo this surgery. Conclusion There is a tendency to delay surgical indication for severe pCD due to the mutilation associated with a permanent stoma. However, the morbidity and severity of the disease demand aggressive and resolutive actions, as irreversible anatomical and functional damage occurs. Publication History Article published online: 25 April 2025 © 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/) Thieme Revinter Publicações Ltda. Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil
Language: English
Subfield: Genetics
Open Access status: diamond
OpenAlex ID: W4409792192
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