Perianal Disease Research Articles

Magnetic resonance imaging for suspected perianal Crohn's disease in children: a multi-reader agreement study.

We aimed to assess inter-radiologist agreement when interpreting pelvic MRI in children with newly diagnosed perianal Crohn's disease (CD). In this retrospective multi-reader study, we identified pediatric patients (< 18 years of age) who underwent a pelvic MRI examination for newly diagnosed perianal CD. Images were de-identified and uploaded to a cloud-based image platform for review by 13 fellowship-trained pediatric radiologists The reviewers assessed for the presence of a fistula and abscess, categorization of different imaging findings, and classification using the Parks and St James' University Hospital systems. Fleiss' kappa (κ) statistics and intra-class correlation coefficients (ICC) were used to measure inter-reader agreement, along with 95% confidence intervals (CI). Forty-six patients were included in our study (median age = 13.0 years [IQR: 10.5 to 16.0 years]); thirty-five (76.1%) were boys. Most imaging features showed fair agreement (κ = 0.21 to 0.35). There was moderate agreement for categorical fistula length (κ = 0.42 [95% CI: 0.32 to 0.53]), involvement of the genitalia (κ = 0.45 [95% CI: 0.26 to 0.63]), and presence of an abscess/collection (κ = 0.52 [95% CI: 0.31 to 0.73]). Maximum abscess/collection length had good agreement (ICC = 0.81 [95% CI: 0.41, 1.00]). There was an almost equal split (yes vs. no: 50.7% vs. 49.3%) regarding whether postcontrast T1-weighted images added value compared to T2-weighted images alone across all radiologists and examinations. Inter-radiologist agreement when interpreting pelvic MRI for perianal CD in children is fair for most imaging features, with fewer features demonstrating moderate or good agreement. Question Pelvic magnetic resonance imaging (MRI) is used for diagnosing and monitoring children with perianal Crohn's disease (CD). Limited information is known about inter-radiologist agreement. Findings Agreement between pediatric radiologists when interpreting MRI for perianal CD in children is only fair for most imaging features (κ = 0.21 to 0.35). Clinical relevance Understanding MRI inter-radiologist agreement is crucial to improve the reliability of pelvic MRI in children with perianal Crohn's disease since it may affect patient management (e.g., surgery); further radiologist education and improved imaging feature definitions may help improve inter-radiologist agreement.

Clinical trials for perianal Crohn's disease: A call for a revised approach.

Clinical trials for perianal Crohn's disease: A call for a revised approach.

Persistence and Efficacy of Ustekinumab in Crohn's Disease After Anti-TNF Failure: An Observational Study.

Anti-TNF treatment failure in Crohn's disease is common, and the literature on the selection of subsequent treatment is scant. Ustekinumab may be associated with high persistence rates and it appears to be effective in perianal disease. Primary objective: persistence, clinical, and biologic remission with ustekinumab. Persistence of the first biologic therapy, reasons for change of treatment, need for dose optimization, surgery, hospitalizations, and adverse events with ustekinumab. Retrospective, observational, single-center study from a prospective database of Crohn's disease adult patients receiving ustekinumab after failure of anti-TNF or vedolizumab. A sub-analysis was performed to evaluate ustekinumab persistence after the approval of risankizumab and upadacitinib. Mean duration with ustekinumab was 27.65months (SD 18.27) and persistence was 86.76%. Clinical remission was 40.63% at week 4, 54.35% at week 8, 54.9% at year 1, 76.92% at year 4, and 100% at year 5. Persistence with ustekinumab was longer than with anti-TNF: year 1, 93.2 vs 72.06%; year 2, 89.4 vs 45.59%; and year 3, 86.1 vs 30.88%. Just over one-third (36.76%) of patients required dose optimization. Nine (13.24%) patients stopped treatment due to primary non-response [1 (1.47%)], loss of response [5(7.35%)], and adverse events [3 (4.41%)]. Eleven (16.18%) patients needed surgery and hospitalization. After the approval of upadacitinib and risankizumab, ustekinumab persistence was 80.88%. Seven (70%) of the patients with perianal disease achieved clinical remission and 4 (40%) completed fistula healing. Ustekinumab may have better persistence as a second-line treatment compared to anti-TNF and may be effective in perianal disease.

Characteristics and long-term outcomes of children with perianal Crohn's disease.

The incidence of perianal lesions (PL) in children with Crohn's disease (CD) is higher in East Asia than in Western countries. Early intervention for PL is essential to prevent sphincter dysfunction and ostomy placement. In this study, we aimed to investigate the clinical features, treatment, and consequences of pediatric CD with PL. We retrospectively reviewed a cohort of children diagnosed with CD from 2010 to 2020 at a Japanese children's hospital. Demographics, treatments, and outcomes were evaluated and compared among subgroups. Among 112 pediatric patients with CD, 36 (32.1%) had experienced PL during the observational period. The median ages at diagnosis and follow-up periods were 131 and 70 months, respectively. Six (85.7%) patients in the very early-onset (VEO) group (CD diagnosed before 6 years old) and 24 (82.8%) in the older age group had PL upon diagnosis of CD (P= 0.851). Biologics were given to 94.4% of patients: infliximab (67.7%), adalimumab (58.8%), ustekinumab (44.1%), risankizumab (11.8%), and vedolizumab (5.9%). Biologics were introduced within 1 year in 89.5% and 40.0% of patients diagnosed in 2016-2020 and 2010-2016, respectively (P= 0.002). Seton was frequently used in the older age group (87.5 vs. 42.9%, P= 0.190). Ostomy was frequently required in the VEO group (42.9% vs. 0.0%, P= 0.006). Patients with VEO-CD and PL had a notably high risk of ostomy placement. The earlier introduction of biologics and surgical interventions reduced corticosteroids use and ostomy placement in pediatric CD patients with PL.

Differential Diagnosis Value of Neutrophil Gelatinase Associated Lipocalin as a Noninvasive Biomarker in Perianal Fistulizing Crohn's Disease.

Diagnosing perianal fistulizing Crohn's disease (pfCD) typically depends on costly and time-intensive endoscopic and radiographic procedures. Compelling evidence indicates that neutrophil gelatinase-associated lipocalin (NGAL) plays a role in the pathophysiology of Crohn's disease (CD) and may serve as a noninvasive biomarker for its diagnosis. This study aimed to evaluate NGAL's potential as a noninvasive diagnostic biomarker between pfCD and cryptoglandular (CG) perianal fistula, and its correlation with disease severity in pfCD. Serum, fecal, and fistula tissue samples were collected from 96 patients with pfCD and 97 patients with CG perianal fistula as controls. Serum NGAL levels were quantified through ELISA and fistula tissue NGAL levels were quantified through immunohistochemical staining, while pfCD disease severity was evaluated using the Crohn's Disease Activity Index (CDAI) and Perianal Disease Activity Index (PDAI). Additional laboratory parameters, including NGAL, fecal calprotectin (FC), C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR), were analyzed, and their correlations were assessed. Receiver operating characteristic (ROC) analysis was conducted to evaluate NGAL's diagnostic potential for pfCD. Levels of serum NGAL, FC, CRP, and ESR in patients with pfCD were significantly elevated compared to the control group (p < 0.001); Spearman correlation analysis indicated a positive correlation between serum NGAL and FC, CRP, ESR, CDAI, and PDAI scores. The area under the ROC curve (AUC) for serum NGAL in diagnosing pfCD was 0.927 (95% CI: 0.890-0.964). The AUC for FC in diagnosing pfCD were 0.887 (95% CI: 0.839-0.935). Additionally, serum and fistula tissue NGAL levels were positively correlated with disease complexity in pfCD according to the Montreal classification. These findings suggest that serum NGAL is associated with pfCD severity and may offer a promising noninvasive biomarker for diagnosing and assessing pfCD.

MULTI-OMICS CHARACTERIZATION OF PERIANAL FISTULIZING CROHN’S DISEASE IDENTIFY PATHOGENESIS AND NOVEL THERAPEUTIC TARGETS

Abstract INTRODUCTION The management of perianal fistulizing CD (PCD) is a major clinical challenge, where its underlying etiology remains elusive. MATERIALS &amp; METHODS We recruited patients with 1) PCD (n = 24); 2) CD without perianal disease (NPCD; n = 10); 3) idiopathic or cryptoglandular perianal fistulas (IPF; n = 29). Biopsies were taken from the fistula tracts, openings of the fistulas, and rectal mucosa during examination under anesthesia or colonoscopy. CyTOF, single cell RNA-sequencing (scRNA-seq), spatial transcriptomics, immunohistochemistry (IHC), ex vivo cell stimulation, and integrated analysis of published scRNA-seq datasets were performed. RESULTS CyTOF revealed a skewed mucosal immune landscape in PCD compared to NPCD and IPF. PCD expanded Th17 cells in the fistula tracts and IL17-producing CD8 T cells (Tc17) in the rectum. Altered exhaustion markers CD39 and CD127 were present in CD4 and CD8 T cells from PCD fistula tracts, fistula openings, and rectum. Regulatory B cells, with immunomodulatory function in the gut, were dramatically diminished in PCD compared to IPF. In addition, PCD fistula tracts, fistula openings, and rectum all exhibited substantially higher CD172+TREM1+ macrophages, which are associated with anti-TNF resistance in luminal CD. ScRNA-seq unraveled immune and non-immune cell compartments in PCD and IPF fistula tracts. PCD fistulas showed hyperactivated pathogenic pathways including interferon (IFN)G response, TNF signaling, and IL6-JAK-STAT3 in myeloid cells. Similarly, stromal cells from PCD fistulas exhibited higher activities in IFNG and TNF response, TNF signaling, and epithelial-mesenchymal transition (EMT). In addition to fistula tracts, luminal (rectal and ileal) cells from PCD patients also expressed greater levels of IFNG-responsive and EMT genes compared to those without perianal disease (Figure 1). Moreover, we showed that both fistula tracts and ileal mucosa from PCD patients harbored expanded IFNG+ Th17 cells with elevated expression of inflammatory mediators (Figure 2). Further analysis revealed cellular modules associated with anti-TNF therapy in PCD patients. The findings were independently validated using spatial transcriptomics, IHC, and ex vivo stimulation of PCD cells. CONCLUSION Using multi-omics analysis of perianal fistula tracts and luminal tissues from extensive patient cohorts, we revealed previously unknown immune, stromal, and epithelial cell landscapes of PCD. In addition to IL17 signaling, T cell exhaustion, pathogenic macrophages, and pro-inflammatory/remodeling fibroblasts, our data highlight the pathogenic role of hyperactivated IFNG signaling in both fistula tracts and luminal mucosa of patients with PCD. This study identified IFNG as a potential therapeutic target for PCD. Figure 1. IFN-γ signaling is a distinguishing feature of PCD. (A) Pathway rank plot of top pathways in PCD compared to IPF fistula. (B) Pathway rank plot of top upregulated pathways in rectum (active PCD vs inactive PCD), colon and terminal ileum (PCD vs NPCD). (C) Representative IHC image and quantification of IFN-γ and p-STAT1 staining of IPF and PCD fistula tracts. (D) UMAP plot comparing rectal epithelial cell clusters between healthy, active PCD, and inactive PCD (PCD-healed). (E) UMAP plot of single-cell module scores for IFNG response (left), TNF response (center), and EMT (right) pathways in PCD rectal epithelial cells. (F) Dot plot indicating transcription factory activity of STAT1, NFKB1, and NFKB2 in rectal epithelial cells clusters 1, 9, and 12 (as in D). (G) Bar plot of top transcription factors driving EMT gene expression in rectal epithelial cells. Figure 2. Pathogenic mononuclear phagocytes (MNP)-Th17 interaction underlies IFN-γ response in PCD. (A) Volcano plot highlighting select upregulated ligand-receptor signaling pairs. (B) Bar plots indicating IFNG senders significantly enriched in PCD. (C) Volcano plot of differentially expressed myeloid genes between PCD and IPF. (D) UMAP plot of NLRP3, AREG, SIRPA (CD172a), TREM1, TNF, and IL1B expression in myeloid cells. (E) UMAP plot of single-cell module scores for LPS signaling in IPF and PCD myeloid cells. (F) Volcano plot showing top enriched LR signaling pairs between MNPs and CD4 T cells in PCD.

IDENTIFYING CLINICAL CHARACTERISTICS AND PRESENTATION OF PEDIATRIC PATIENTS WITH INFLAMMATORY BOWEL DISEASE AND AUTISM SPECTRUM DISORDER

Abstract INTRODUCTION Autism Spectrum Disorder(ASD) is a neurodevelopmental condition that can affect communication and behavioral functioning in patients. Compared to those without ASD, patients with ASD experience higher rates of abdominal pain, nausea, and stooling abnormalities, common symptoms associated with inflammatory bowel disease (IBD). There is limited research on the impact of comorbid ASD on presenting symptoms and treatment of pediatric IBD. We evaluated whether clinical presentation and outcomes were different between pediatric patients with ASD and IBD and those with IBD alone. METHODS Patients with IBD diagnosed at Nationwide Children’s Hospital between 2012 and 2021 were identified using an internal database maintained by the IBD center. Those who also had a diagnosis of ASD were identified by using ICD-9 and -10 codes. Using propensity score matching, patients with IBD were matched 3:1 with patients with IBD and ASD by age at IBD diagnosis, race, sex, IBD type [Crohn’s disease (CD), ulcerative colitis (UC) and IBD-Unclassified], and IBD disease characteristics (perianal disease, CD behavior and phenotype, and UC disease extent). Time to diagnosis from symptom onset, clinical presentation, hospitalizations, family history, treatments, and one-year outcomes were compared between groups using chi-squared and Fisher exact tests where appropriate. Continuous variables were compared using student’s independent sample t-tests. RESULTS The study included 98 patients (73 with IBD, 25 with IBD and ASD). Patient demographics, IBD type, disease extent and disease phenotype did not differ between the cohorts. Patients with ASD and IBD were less likely to have diarrhea at diagnosis compared to those without ASD (32% vs 66%; p=0.0012). Other common IBD symptoms at diagnosis did not differ between groups. Patients with ASD and IBD were more likely to have seen gastroenterology for a different gastrointestinal complaint before subsequent presentation leading to IBD diagnosis when compared to patients without ASD (38 % vs 7.1 %; p=0.0005). Once IBD symptoms were reported to the provider, time to diagnosis of IBD did not differ significantly between the two groups(p=0.154). Patients with ASD were more likely to have a family history of IBD than those without ASD (67 % vs 25 %; p=0.0288). At diagnosis and one-year post, there were no differences in laboratory results, disease activity indices, treatments, hospitalizations, and repeat endoscopy rates to assess for mucosal healing. CONCLUSION While pediatric patients with ASD reported higher rates of gastrointestinal complaints, there were limited differences in the clinical presentation, laboratory values and one-year outcomes for patients with IBD and ASD when compared to those without ASD. In this single center cohort, we found that comorbid ASD did not result in a delay in IBD diagnosis.

CHARACTERIZING LATE-ONSET CROHN’S DISEASE: INSIGHTS FROM A RETROSPECTIVE COHORT STUDY

Abstract BACKGROUND Inflammatory bowel disease (IBD) most commonly has onset between the second and fourth decades of life; however, about 10-20% of individuals develop late-onset disease, frequently defined as being diagnosed at age greater than 60. Late-onset Crohn’s disease (LOCD) is less well characterized compared to adult-onset Crohn’s disease (AOCD). We compared a cohort of patients with LOCD to those with AOCD at the time of diagnosis to identify differences in disease characteristics and potential risk factors for late-onset disease. METHODS This retrospective cohort study utilized our institution’s electronic medical record (EMR) database to identify LOCD patients (diagnosed at age ≥ 60) with complete data. AOCD patients (age 20–40) were selected using a random number generator in a 3:1 ratio. Factors recorded at the time of initial diagnosis included age, BMI, race/ethnicity, history of current or prior smoking, disease location based on endoscopy and/or imaging, family history of IBD, presence of extraintestinal manifestations, history of appendectomy or tonsillectomy, and history of diverticulitis. In descriptive analysis, the two groups were compared using the Wilcoxon rank sum test for continuous variables and the chi-square or Fisher’s exact test for categorical variables. A p-value of &amp;lt; 0.05 was considered statistically significant. RESULTS A total of 40 LOCD patients (median age at diagnosis 64 years) and 120 AOCD patients (median age at diagnosis 28 years) were included in the analysis. The LOCD cohort had a higher percentage of white patients (80% vs. 55.8%, p=0.02), significantly higher BMI (median(IQR) 25(22-28.9) vs. 22.4(20.4-25), p&amp;lt; 0.01), and a greater proportion classified as obese or overweight (20% vs. 9.2% and 30% vs. 15.8%, respectively, p=0.03). There were statistically significant higher rates of prior diverticulitis and appendectomy/tonsillectomy in the LOCD cohort. A non-significant trend toward a higher proportion of isolated colonic disease was noted in the LOCD cohort (45% vs. 30%, p=0.10). Additionally, perianal disease was significantly less common in LOCD patients (5% vs. 22.5%, p=0.01). CONCLUSION Our results suggest that higher BMI and classification as obese or overweight, may be risk factors for LOCD. Additionally, there was a higher prevalence of white patients in the LOCD cohort, suggesting a potential propensity for the condition in this group. Even though differences were noted in diverticulitis and surgical history, the low numbers and change in surgical practice patterns over time limit interpretation. We found a trend towards higher isolated colonic disease and significantly lower rates of perianal disease in the LOCD cohort. These differences in disease location at the time of diagnosis have potential implications for therapy response and risk of disease progression. Table 1: Late-Onset vs Adult-Onset Crohn’s Disease

THE USE OF CHEWED OR CRUSHED UPADACITINIB AFTER BOWEL SURGERY IN PATIENTS WITH INFLAMMATORY BOWEL DISEASE

Abstract Upadacitinib, a small molecule therapy approved by the FDA for the treatment of ulcerative colitis and Crohn’s disease, is delivered via extended-release tablets. There is concern that absorption of extended-release medications may be impaired in patients after colectomy with ileostomies or ileal pouch-anal anastomoses (IPAAs) due to the longer disintegration time of these medications and shorter transit time in these patients with altered gastrointestinal anatomy. Anecdotally, several patients at our center have reported whole or partially digested upadacitinib pills in their ostomy effluent or ileal pouch stool output, which suggests some validity to these concerns. The aim of this case series is to describe the use of chewed or crushed upadacitinib to improve absorption in patients with IBD with ileostomies and IPAAs. We describe 5 patients with IBD who reported partially digested upadacitinib pills in their stool. All 5 patients had Crohn’s disease. Two of the patients had a colectomy and IPAA, both for medically refractory disease. Two of the patients had end ileostomies, 1 with colectomy for medically refractory disease, and the other had a diverting end ileostomy for perianal disease. Finally, 1 patient’s bowel remained in continuity and had a history of two ileocolic resections, with roughly 57cm ileum and 40.5cm colon removed in total. Of the 5 reported patients, 3 (2 with end ileostomies and 1 with IPAA) reported no longer seeing whole/undigested pills in their stool. The 3 patients who chewed their upadacitinib and no longer saw partially digested pills in their stool each demonstrated improvement in their Crohn’s disease. The first patient had objective evidence of endoscopic improvement (resolution of ileal ulcerations) after the intervention as well as clinical improvement (resolution of his abdominal pain). The second and third patients had improvement in their fistulizing disease, with both patients able to stop their antibiotics without recurrence of their symptoms and one patient having an exam under anesthesia confirming resolution of their fistula. Our case series highlights patients with IBD who underwent colectomy with ileostomies or IPAAs and observed whole/undigested upadacitinib in their stool. In these patients, we propose a new strategy of crushing/chewing their medication to improve absorption. Figure 1. Ileoscopy of patient with Crohn’s before (a) and 4 months after crushing upadacitinib pills, demonstrating endoscopic response with healing of ulceration in the distal 30cm of ileum.

SKIN TAG EXCISION IN CROHN’S DISEASE: FIRST DO NO HARM?

Abstract Traditionally, perianal skin tag excision has been contraindicated in patients with Crohn’s disease (CD) owing to risk of non-healing wounds. However, little data exists on this topic. We aimed to review our institutional experience with outcomes after perianal skin tag excision in patients with CD. We hypothesized that perianal skin tag excision in CD would be associated with non-healing wounds. We retrospectively reviewed adults with CD who underwent skin tag excision with or without limited excisional hemorrhoidectomy at our center between 2012 and 2024. Demographics, CD characteristics, medications, operative data, pathology, and postoperative outcomes were reviewed. Patients were selected for surgery based on surgeon discretion. Forty-five patients with CD underwent excision of skin tags (28, 62%) and/or limited excisional hemorrhoidectomy (20, 44%). Demographics, CD characteristics, and medications are detailed in Table 1. Short-term complications within 30 days occurred in 2 (4.4%), including fistula and wound dehiscence (one each). Pathology results were available for 42 (93%) patients; the majority were benign (33, 78.5%) but a small subset (2, 5%) had anal intraepithelial neoplasms (Table 2). Long-term complications occurred in 11 (24.4%) patients (Table 2), of which 9 (81.8%) were managed with conservative management or non-operative therapy. Complications included perianal fistula (4, 8.8%), anal stenosis (4, 8.8%), non-healing wounds (2, 4.4%) and anal fissure (1, 2.2%). Of these patients, seven (63.6%) later had complete wound healing. Almost half of these patients had penetrating disease with perianal involvement (5, 45.5%). At most recent follow-up, 41 (91.1%) patients had complete wound healing, and only one (2%) patient required proctectomy. Four patients (9%) had persistent non-healing wounds; 2 with history of perianal fistulae requiring surgery (one requiring diversion), 1 with fistula 4 years after skin tag excision, and 1 subsequently diagnosed with perianal hidradenitis suppurativa. Three (75%) of these patients had prior surgery for perianal disease (one each: fissure, abscess, and stricture), and all were on biologic therapy (infliximab, 3; adalimumab, 1). Perianal skin tag excision in carefully selected CD patients resulted in wound healing in the majority (91%) of patients and can be considered for lifestyle limiting symptoms. Pathology was typically benign but occasionally revealed actionable results, including neoplasia, and should encourage routine pathologic evaluation of specimens. The risk of long-term complications was low, but higher in the CD population compared to average, and the sequelae were mostly managed with non-operative therapy. Our data suggests that previous surgery for perianal Crohn’s may be a risk factor for non-healing wounds after perianal skin tag excision. Table 1. Baseline demographic data, preoperative Crohn’s characteristics and medication history. Table 2. Pathologic data, short- and long-term complications, and long term outcomes

Preoperative Optimization for Elective Surgery in Crohn's Disease: A Narrative Review.

Crohn's disease is a chronic inflammatory bowel disease and, despite an increase in the available drug treatments, many patients will still require surgery at some point in their disease course. Stricturing and penetrating phenotypes of Crohn's disease are less likely to respond to our current medical treatment and, therefore, surgical intervention may be required. This is most commonly elective, planned surgery, thereby affording the opportunity to optimize medications, nutritional and inflammatory status, and steroid use. Poor nutritional status and previous surgery increase the risk of postoperative complications. Preoperative optimization has three main goals: reduction of postoperative complications; reduction of reoperation rates; and reduction of postoperative recurrence rates. A literature search was completed using PubMed, Embase, and Ovid using the search term "preoperative optimization in Crohn's disease", and it included both adult and pediatric studies, excluding those for perianal Crohn's disease. In this narrative review, we examine the role of nutritional intervention, medical optimization pre and postoperatively, and the role of personalized prehabilitation in the reduction of postoperative complications. We demonstrate that these may all yield better postoperative outcomes for patients with Crohn's disease undergoing elective surgery, although the evidence is somewhat limited and there is a requirement for more prospective randomized controlled trials to implement their role into standard practice or guidelines.

24 Massive perianal hidradenitis suppurativa: aggressive surgical approach with curative intent

Abstract Aim Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease affecting areas rich in apocrine glands (for example groin, axillae), causing significant disability. Clinical manifestations include the formation of painful suppurative nodules, leading to recurrent abscesses extending deeply into the subcutaneous tissue, ultimately developing interconnecting sinus tracts, and resulting in hypertrophic scarring. This condition imposes substantial physical and psycho-social distress on patients, along with an economic burden due to the need for chronic care. Although the pathogenesis is not fully understood, HS is considered an androgen-dependent pathology associated with endocrine abnormalities, with risk factors such as smoking and obesity exacerbating its severity. Bacterial infection is viewed as a secondary phenomenon. This case-report aims to present a case of massive perianal HS and emphasize the efficacy of an aggressive surgical intervention with the intention of achieving a curative outcome. Methods We report the case of a 45-year-old Caucasian woman with a history of active smoking, alcoholism, and depression. The patient described recurrent perianal abscesses years ago, experiencing progressive symptoms with multiple episodes of abscesses that needed drainage in the Emergency Department. Due to the COVID-19 pandemic, regular follow-ups were interrupted for over a year, leading to clinical deterioration of the perianal disease. Upon emergency admission, the patient exhibited bilaterally encrusted perianal plaques extending to the buttocks, with multiple actively draining fistulas. Drainage, biopsies, and seton placement were performed. A differential diagnosis between HS and Crohn’s Disease was considered. Further investigations included imaging studies, colonoscopy, specific biopsies, and laboratory tests. Evaluation by Gastroenterology and Dermatology ruled out inflammatory bowel disease, and confirmed HS. Results Despite initial attempts at medical management by Dermatology, the patient’s deteriorating quality of life prompted a decisive surgical approach by Colorectal and Plastic Surgery. Bilateral excision of the affected perianal region was performed down to healthy subcutaneous tissue. Anal canal and introitus exploration revealed no identifiable tracts. Anal and vulvar margins were preserved to avoid tissue retraction. Primary closure was achieved in the posterior buttock and bilateral inguinal regions, leaving the central subcutaneous area exposed. Following local care for several weeks to promote granulation, Plastic Surgery considered a skin graft to cover the exposed region. Conclusions HS may manifest insidiously, needing an accurate differential diagnosis and interdisciplinary management and follow-up. Initial treatment involves antibiotics, topical antiseptics, or intralesional corticosteroids. Some medications (retinoids, anti-androgen therapy, immunotherapy, oral immunosuppressants) have shown positive effects on disease progression. However, for many cases of advanced disease, radical surgery may be the only curative option, as in the case presented. Early and extensive surgical excision is crucial for preventing complications and recurrence, ultimately improving the quality of life for these patients.

Prevalence, Clinical Features, and Extraintestinal Manifestations in Patients with Familial Inflammatory Bowel Diseases.

A positive family history is considered the strongest recognizable risk factor for developing inflammatory bowel diseases (IBD) and is present in 8-12% of cases. To determine the prevalence of familial IBD and also to compare the clinical characteristics and extraintestinal manifestations between familial and sporadic cases. We conducted a cross-sectional study based on retrospectively-prospectively collected data from a cohort of IBD patients followed in daily clinical practice. Patients with biopsy-confirmed familial and sporadic IBD aged 16-90years old were included in the study. A comprehensive questionnaire was used to collect data on each site visit. Logistic regression analysis and chi-squared test were used. In total, 265 patients with IBD were included. 12.1% had a first-degree relative with IBD present (16.4% in Crohn's disease and 5.7% in the ulcerative colitis group, P < 0.05). Familial IBD patients were mainly females, were more frequently diagnosed with Crohn's disease and had a higher risk for hospitalization due to an IBD flare (P < 0.05), to undergo a surgical procedure (P < 0.001), to require treatment with biologic agents (P < 0.05), to develop perianal disease (P < 0.05), and extraintestinal manifestations (P < 0.05), mainly spine joint complications, erythema nodosum, and anterior uveitis. In this study, a positive family history of IBD was present in 16.4% of Crohn's disease and 5.7% of ulcerative colitis patients and was associated with a greater risk for hospitalization, surgical procedures, occurrence of perianal disease, need for treatment with biologic agents, and development of extraintestinal manifestations.

Characteristics and surgical outcomes of small intestine-rectal fistulas in patients with Crohn’s disease

PurposeSmall intestine-rectal fistulas are a rare and complex complication in Crohn’s disease, posing significant diagnostic and management challenges. This study aims to investigate their distinctive features and evaluates surgical outcomes.MethodsWe conducted a retrospective analysis of Crohn’s disease patients with small intestine-rectal fistulas who underwent surgery from January 2019 to March 2023. Data on disease characteristics, postoperative quality of life, and functional outcomes were collected.ResultsA total of 92 patients were included, predominantly male (75%). The average time from Crohn’s disease diagnosis to small intestine-rectal fistula diagnosis was 5.18 years. Most fistulas originated in the ileum (84.79%), followed by the rectum (9.78%) and both sites (5.43%). Nearly half had perianal lesions (48.91%), with some also having entero-vesical fistulas (22.83%) and entero-vaginal fistulas (1.09%). Significant risk factors for the creation of temporary protective ileostomy included preoperative hemoglobin levels below 100 g/L, albumin levels below 35 g/L, and the presence of perianal disease. Fistulas originating from the rectum and rectal lesions of 3 cm or longer were significant risk factors for rectal resection. No small intestine-rectal fistula recurrence was observed over an average follow-up of 2.35 years. Patients without a permanent sigmoidostomy after small intestine-rectal fistula resection had better stool scores and improved quality of life, especially in emotional and social functioning.ConclusionsSmall intestine-rectal fistulas in Crohn’s disease have distinct characteristics. Surgical repair and resection effectively restore intestinal continuity and improve quality of life, particularly when anal function is preserved.

A180 THE IMPACT OF INTEGRATING PELVIC MRI AT DIAGNOSIS FOR EARLY DETECTION OF PERIANAL CROHN’S DISEASE IN PEDIATRICS

Abstract Background Perianal Crohn’s disease (CD) can be a severe, refractory manifestation of pediatric CD. It is underrecognized, particularly if asymptomatic, which has led to varied reported incidences (10-60%). Earlier detection by pelvic magnetic resonance imaging (pMR) may alter management and outcomes. Aims If performing pMR on newly diagnosed pediatric CD patients identifies asymptomatic perianal CD and leads to earlier biologic use, fewer hospitalizations, and less surgery. Methods New CD patients were prospectively enrolled into the Edmonton Pediatric Inflammatory Bowel Disease Clinic (EPIC) registry. This clinic has done baseline pMR since 2018. A retrospective review (2018-2023) collected pMR, perianal exam, symptoms, blood work, calprotectin, medications, and surgeries (perianal and intestinal). A blinded radiologist re-read pMRs using St. James and Parks criteria. Statistics included descriptive, Fisher’s exact test, multivariate logistic regression, and survival analysis. Results 139 patients were included (63% male, median age 13 [IQR 11-16]). Ninety-six patients (69%) had no pMR perianal disease (pMR-), despite 13/96 (14%) having perianal symptoms and 26/96 (27%) with inflamed (n=6)/ non-inflamed (n=14) tags or fissures (n=6). Of the 43 (31%) with pMR+ perianal disease, 27/43 (63%) were asymptomatic (ASx) and 17/43 (40%) had a normal exam. pMR+/ASx patients with perianal exam findings included inflamed (n=4)/ non-inflamed (n=5) tags, fissures (n=1), and draining fistula (n=1). pMR+/Sx had higher Parks and St James scores compared to pMR+/ASx. Compared to pMR-, pMR+/ASx and pMR+/Sx patients had a relative risk of 1.40 [95% CI 1.16-1.71] and 1.72 [95% CI 1.72-1.73] of requiring a biologic at 6 months, and a relative risk of 1.39 [95% CI 1.23-1.62] and 1.45 [95% CI 1.45-1.46] of requiring a biologic at 1 year. pMR+/Sx patients had an increased probability of CD surgery (p&amp;lt;0.002) but not hospitalization (p&amp;gt;0.05). There were no differences between pMR+/ASx and pMR- patients in surgery or hospitalization (p&amp;gt;0.05). Time to surgery was shorter in pMR+/Sx (p&amp;lt;0.01), but not different between pMR+/ASx and pMR- (p&amp;gt;0.05). Conclusions The majority of patients with perianal CD identified on pMR had no perianal symptoms and had a normal exam. This early identification of subclinical perianal disease led to increased and earlier biologic use, which may explain the lack of increased hospitalization and surgical rates compared to those without pMR+ perianal disease. Baseline imaging was also helpful for demonstrating the absence of deeper perianal disease in several patients who did have perianal symptoms and tags/fissures. Funding Agencies NACTRC, Stollery Children’s Hospital Foundation through WCHRI

A188 NATURAL HISTORY OF PERIANAL FISTULAS IN PATIENTS WITH ILEAL POUCH ANAL ANASTOMOSIS

Abstract Background The natural history of perianal fistulas (PAF) in patients with ileal pouch-anal anastomosis (IPAA) remains poorly understood. Perianal fistulas can significantly impact the quality of life and clinical outcomes in patients who have undergone IPAA. To date, there is limited data available on the long-term progression, treatment responses, and overall outcomes of PAF in this unique patient population. Aims The primary aim of this study is to report the clinical outcomes of PAF in patients with IPAA. Methods We performed a retrospective, observation cohort study between 2000 and 2024. We included adults (&amp;gt;17 years) with an IPAA who developed a perianal fistula and a minimum of 1 year follow-up after PAF diagnosis. PAF disease activity was categorized on an annual basis according to the following disease activity patterns: remission, minimally active, relapsing-remitting, and chronic persistent. We also determined medication utilization, medication effectiveness and major adverse fistula outcomes (MAFO): a composite of hospitalization, surgical drainage of PAF and fecal diversion. Results A total of 29 patients with an IPAA and PAF were identified: mean (SD) age 49.3 (13.2); 19 (65.5%) female, and 17 (63.0%) with complex fistulas radiologically at the time of diagnosis. After a mean (SD) follow-up of 9.7 years (7.4), 7 (25.0%) patients required fecal diversion, and 2 (7.1%) patients underwent pouch excision. Annual PAF disease patterns are reported in Figure 1. At the last date of follow-up, 26 (92.9%) patients achieved fistula remission. A total of 16 (55.2%) patients underwent treatment with an advanced therapy: 14 (87.5%) anti-TNF and 2 (12.5%) Risankizumab. After initiating a first advanced therapy, 10 (62.5%) patients achieved fistula remission at 12 months. Furthermore, 3 (18.8%) patients developed a MAFO after initiating their first advanced therapy: 3 (18.8%) underwent surgical drainage, 1 (6.3%) was hospitalized, and 1 (6.3%) underwent fecal diversion for PAF. Conclusions This study demonstrated that the majority of patients with PAF in the setting of IPAA achieved fistula remission clinically, particularly after treatment with advanced therapies. Despite the high remission rates, a substantial subset of patients still required fecal diversion and pouch excision. Annual perianal fistula disease patterns Funding Agencies None

Patterns and predictors of postoperative complications and recurrence after ileocecal resection for Crohn's disease: a national multicenter longitudinal study.

Crohn's disease (CD) negatively impacts quality of life, and a high percentage of patients will need surgery. Ileocecal resection (ICR) is the most common abdominal procedure performed in CD. Postoperative complications are frequent, and recurrence is common. The main objectives of this study were to analyze risk factors (RF) for anastomotic leakage (AL) and recurrence after ICR, as well as quality of life (QoL) in CD in a sample of the Spanish population. A prospective, multicenter, observational study was designed, including all types of hospitals in Spain from 2018 to 2021. Demographic and medical-surgical characteristics, postoperative complications, hospital types by annual ICR volume, and quality of life (IBDQ-9) up to 1year follow-up (OYF) were recorded and analyzed. A total of 386 ICRs were recorded. At 60days, there were 134 (36.4%) complications, 46 (11.91%) of which were major. A total of 23 (5.9%) AL were registered, and RF were Montreal A3 [OR 14.2, 95% CI (2.70-126), p = 0.005], Montreal p [OR 7.29, 95% CI (1.14-44.4), p = 0.029], and intensified adalimumab treatment [3.8, 95% CI (1.2-13.1), p = 0.026]. Recurrences at OYF were 67 (19%), and RF were history of neoplasia [OR 7.14, p = 0.01], Montreal B3 [OR 2.12, p = 0.02], and minimally invasive surgery (MIS) [OR 2.63, p = 0.02]. The mean difference in IBDQ-9 from baseline to 60days was +1.72 (n = 177, p < 0.001) and to OYF +2.12 (n = 140, p < 0.001). The AL rate was 5.9%. Montreal A3, perianal disease, and intensified adalimumab were associated with a higher rate of AL. The recurrence rate was 17.6% (OYF). Prior neoplasia, Montreal B3, and MIS were associated with higher risk of recurrence. ICR improves quality of life at 60days and is maintained 1year after surgery.

Adverse maternal outcomes after vaginal versus caesarean delivery in women with Crohn's disease and prior perianal surgery: a population-based study.

For pregnant women with perianal Crohn's disease (CD), major guidelines recommend delivery by caesarean section (c-section) for active disease, and that delivery mode be governed by obstetric indications for women with inactive disease. However, a significant gray zone exists between active versus inactive disease, such as for women who had a history of prior perianal surgery. We examined adverse maternal outcomes of vaginal delivery versus c-section in women with perianal CD and prior perianal surgeries. We used the Danish national registries to collect information on singleton live births from 1997 through 2022 by mothers with CD and previous perianal surgery. Adverse postpartum maternal outcomes included new perianal or intestinal surgery within 12 months, hospital diagnosed infection or outpatient antibiotic prescription within 6 months, and major haemorrhage within 3 days. Adjusted odds ratios (aOR) were estimated in logistic regression models. There were 704 deliveries by women with CD and prior perianal surgery, including 245 vaginal deliveries (34.8%) and 459 c-sections (65.2%). Women who delivered vaginally versus by c-section had less new perianal surgery (aOR 0.47, 95% CI, 0.24-0.93) and no significant differences in intestinal CD surgery (aOR 0.87, 95% CI, 0.43-1.75), hospital diagnosed infection (aOR 0.50, 95% CI, 0.18-1.38), outpatient antibiotic prescription (aOR 0.92, 95% CI, 0.65-1.28), or major hemorrhage (aOR 0.79, 95% CI, 0.39-1.59). Vaginal delivery was not associated with increased important adverse maternal outcomes in mothers with CD and prior perianal surgery.

Types, behaviour and therapeutic requirements of inflammatory pouch disorders: Results from the RESERVO study of GETECCU

Types, behaviour and therapeutic requirements of inflammatory pouch disorders: Results from the RESERVO study of GETECCU

Long-term outcomes of 'temporary' defunctioning in patients with severe perianal Crohn's disease.

This study aimed to analyse long-term outcomes in patients undergoing temporary faecal diversion for therapy-refractory Crohn's perianal fistulas. In this retrospective study, Crohn's patients who underwent defunctioning for perianal disease between 2012 and 2022 were included. The primary endpoints were successful ostomy reversal and proctectomy/proctocolectomy rates. Secondary endpoints were clinical fistula closure, development of proximal colonic disease recurrence, and the influence of medication, patient and disease characteristics on ostomy reversal. In total, 53 patients were included, 35 colostomies and 18 ileostomies. Previous L2 disease was more frequently seen in the ileostomy group (colostomy 29%, ileostomy 83%; P = 0.004). Clinical closure of the fistula was seen in 26%. 21% (11/53 patients) underwent an attempt at ostomy reversal during a median overall follow-up of 90.1 months (interquartile range 17.5-82.5) of which nine ostomies (colostomy 23%, ileostomy 6%; P = 0.244) were closed successfully. In 35%, a proctectomy/proctocolectomy was required to control ongoing perianal sepsis. Factors associated with ostomy reversal were fistula closure (P < 0.001) and L1/L3 disease (P = 0.043). In patients with ostomy reversal attempt (n = 11), successful reversal was associated with colostomy (P = 0.055) and use of anti-tumour necrosis factor (anti-TNF) (P = 0.055) despite being previously classified as anti-TNF refractory. Ostomy reversal rates are low, so defunctioning ostomies should be carefully considered because in most patients the ostomy will be permanent. In one-third of the patients, a proctectomy/proctocolectomy is required to treat ongoing perianal sepsis. If a patient is eligible for ostomy reversal, reversal should preferably be done under anti-TNF to optimize chances of success.