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• W2019423927 abstract "Highly destructive perianal disease (HDPD) is a severe clinical manifestation of Crohn disease (CD) that is notoriously difficult to treat. It is diagnosed by visualization of deep perineal ulceration and tissue destruction associated with copious exudate (1). Historically, this disease process tends to be associated with a less favorable prognosis (2) and has been managed by either medical or surgical modalities alone with marginal success. Although attempts at combined therapy for treatment of HDPD have been made, there has been only modest clinical response with incomplete healing of the wounds reported in the literature (3). Here, we describe the first successful treatment of highly destructive perianal CD using combination biological and surgical therapy. CASE REPORT The patient presented at 14 years of age with a “little bump” around her anus and tenesmus. She believed the bump to be hemorrhoids and found temporary relief from an over-the-counter anti-inflammatory cream. She developed diarrhea, which she associated with stress from school. Three days before admission, she developed increasing pain in the perianal area as well as mucus and bloody drainage. She had an unremarkable medical history and no surgical history. The patient admitted to fatigue and poor appetite and had lost 14 pounds, putting her between the 25th and 50th percentile for her age (body mass index [BMI] 18.1). She had intermittent outbreaks of aphthous ulcerations in her mouth. Upon referral to the pediatric gastroenterologist, severe perianal changes were noted and a surgical consultation was obtained. Laboratory investigations revealed hemoglobin 12.0 g/dL, C-reactive protein 2.6 mg/L, albumin 3.3 g/dL, and erythrocyte sedimentation rate 88 mm/hour. She was admitted to the hospital and taken to the operating room where an examination under anesthesia was performed (Fig. 1). Deep ulceration of the entire perianal area was seen extending 5 cm around the anus and into the left labia. A laparoscopically assisted sigmoid colostomy was created. Biopsies of the perianal skin and her sigmoid colon were submitted for histological examination. The sigmoid colon demonstrated chronic active colitis with cryptitis, crypt abscess formation, and multiple microgranulomata diagnostic of CD (Fig. 2). The perianal skin biopsies exhibited inflamed granulation tissue with no definitive evidence of pyoderma gangrenosum.FIGURE 1: Severe perianal necrosis due to highly destructive perianal Crohn disease.FIGURE 2: Microgranuloma in colon biopsy (original magnification ×40).Following the diverting colostomy, infliximab 5 mg · kg−1 · dose−1 was given at 0, 2, and 6 weeks followed by every-8-week maintenance therapy. Concomitant 6-mercaptopurine at 1.5 mg · kg−1 · day−1 was administered as well. Silvadene ointment 1% was used to treat the perineal wound. By 3 months after her operation, the patient's BMI had increased from 18.1 to 20.6. She reported feeling well and having an increased appetite. Complete blood count and erythrocyte sedimentation rate had normalized. At this time, split thickness skin grafting to the perianal wound was begun and required several revisions (Fig. 3).FIGURE 3: Split thickness skin grafting applied to the perianal wound.At 1 year after presentation, the perineal wound was healed. Proctosigmoidoscopy revealed normal mucosa and mild anal stenosis, which responded to dilatation. The colostomy was reversed via an end-to-end hand-sewn anastomosis. Further dilatations were performed on subsequent office visits. One year following reversal and 2 years after initial presentation, the patient continues in remission on regularly scheduled infliximab therapy with concomitant 6-mercaptopurine. DISCUSSION Perianal CD (PCD) requiring treatment occurs in more than 40% of pediatric patients with CD (4,5). In adults, identifying PCD can be problematic because it is often misdiagnosed as hemorrhoids (6). Sangwan et al (7) suggest that the easy accessibility of the perianal area could explain why perianal disease is reported by patients instead of intestinal symptoms, which may predate it. However, it has been noted that PCD can appear as few as 2 weeks to as many as 12 years earlier than intestinal CD (7). PCD is most often found in patients with disease involving the colon, especially the left colon and rectum, rather than in those with only small bowel involvement (8). Patients with CD presenting in the perianal region carry a less favorable prognosis compared with those who present with either fibrostenotic or internally perforating disease (2). Numerous surgical treatments have been used to treat PCD. The use of a diverting colostomy to help heal perianal lesions by diverting the fecal stream has been debated. Zelas and Jagelman (9) reported a 95% rate of improvement, but Orkin and Telander (10) showed only a 20% improvement rate. In a series of 31 patients who underwent fecal diversion in a 27-year period, only 10% had the ileostomy or colostomy reversed (11). Dietrich and Schönfelder (12) suggest bowel resection after 2 to 4 unsuccessful local procedures and progressive perianal disease, but found that young age and anal symptoms at the onset of disease led to a poorer prognosis. In pediatric patients, Tolia (13) and Orkin and Telander (10) reported no improvement of perianal disease with resection. Studies regarding the role of surgical resection are difficult to interpret due to great variability across studies regarding clinical disease severity. Perianal disease alone is rarely an indication for resection. However, it has been noted by us and others that in patients with limited intestinal disease (eg, short segment of terminal ileum) and concomitant disease in the perineum, a resection of the diseased intestine leads to improvement of the perineum (12). HDPD was first described in pediatric patients by Markowitz et al (1) in 1995. This report described a condition presenting with deep perineal ulcers accompanied by destruction of perineal and perirectal tissue and copious exudate. Several cases of HDPD have been reported since (13,14), but prognosis remains poor. Markowitz had success in only 2 of his 6 patients with medical treatment alone and little success with local surgical treatment. Shetty et al (14) found no improvement with medical treatments and resorted to a diverting colostomy in their patients. Eradi et al (3) attempted infliximab treatment in 2 patients but found no perianal improvement. Both groups experienced greater success with surgical diversion. Successful skin grafting has been rarely reported with Eradi et al also reporting the ability to close perianal ulcers with skin grafting, although attempts to do so were unsuccessful at first (3). This case of HDPD is fairly unique in both approach and degree of successful clinical outcome. By using a combined medical and surgical approach initially, we were able to preserve the patient's continence. Infliximab is an approved standard treatment for fistulizing disease including that which involves the perianal skin (8). Attempts to treat HDPD with surgery and infliximab have been previously reported but with only modest clinical improvement and incomplete perianal healing (3). Our case marks the first completely successful treatment of HDPD using early and aggressive surgical intervention combined with the early use of infliximab. Infliximab has been used to treat other complications of CD as well. Jiang et al (15) used the drug in conjunction with a diverting ileostomy to clear a case of Fournier gangrene. Tan et al (16) found infliximab to be a highly effective treatment for pyoderma gangrenosum in patients with CD. It may be that complicated extraintestinal disease is best managed in a “top-down” manner with early use of biological therapy (17). CONCLUSIONS HDPD must be treated quickly and properly to prevent damage to the anal structures and the risk of incontinence. Our results suggest that patients with HDPD can show good prognosis if treated with early use of biological therapy in conjunction with appropriately aggressive surgical intervention." @default.
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• W2019423927 title "Combination Biological and Surgical Therapy in an Adolescent Presenting With Highly Destructive Perianal Crohn Disease" @default.
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