Perineal Disease Research Articles

Mo1341 Long-Term Quality of Life and Sexual Function After Ileal Pouch-Anal Anastomosis in Adults With Ulcerative Colitis or Familial Adenomatous Polyposis

Background: Previous studies have shown that diagnosis of Crohn's disease (CD) in childhood is associated with a different phenotype when compared to adults (higher risk for ileocolonic disease and fibrostenotic behavior), and may be associated with a more aggressive form of disease requiring more surgery. Those diagnosed at an older age have been reported to have an increased rate of isolated colonic disease and a milder course of disease requiring less surgery. Methods: A comprehensive medical chart review was done for 571 CD patients that were followed in a tertiary referral IBD clinic. Among the parameters that were recorded for each patient at specific time intervals were parameters of disease phenotype according to the Montreal Classification (A1 diagnosed ,16, n=88, A2 diagnosed 16-40, n=287, A3 diagnosed .40, n=77) as well as having surgery. Results: The study included 452 patients that had complete data in the charts. At 6 years from diagnosis and at last follow-up (median11 years), A3 had a higher rate of isolated colonic disease than A1 but not in comparison to A2. Perianal involvement was significantly less common in A3 than A1 or A2. Complicated disease behavior (B2/B3) was similar for all three groups at both time frames. Nonetheless, at 6 years and last follow-up, IBD-related abdominal surgery rates were significantly lower for A1 vs. A2 and vs A3. Conclusions: While there are some disease location differences that emerge with increasing age of presentation (more isolated colonic disease and less perineal disease) disease behavior over time is similar regardless of age at diagnosis and surgeries were least likely in those diagnosed prior to age 16. Our study did not corroborate a more aggressive course for CD that presents in the pediatric age group. Outcomes at 6 years from diagnosis

Mo1340 Younger Age At Diagnosis Does Not Predict a More Aggressive Course of Crohn's Disease

Background: Previous studies have shown that diagnosis of Crohn's disease (CD) in childhood is associated with a different phenotype when compared to adults (higher risk for ileocolonic disease and fibrostenotic behavior), and may be associated with a more aggressive form of disease requiring more surgery. Those diagnosed at an older age have been reported to have an increased rate of isolated colonic disease and a milder course of disease requiring less surgery. Methods: A comprehensive medical chart review was done for 571 CD patients that were followed in a tertiary referral IBD clinic. Among the parameters that were recorded for each patient at specific time intervals were parameters of disease phenotype according to the Montreal Classification (A1 diagnosed ,16, n=88, A2 diagnosed 16-40, n=287, A3 diagnosed .40, n=77) as well as having surgery. Results: The study included 452 patients that had complete data in the charts. At 6 years from diagnosis and at last follow-up (median11 years), A3 had a higher rate of isolated colonic disease than A1 but not in comparison to A2. Perianal involvement was significantly less common in A3 than A1 or A2. Complicated disease behavior (B2/B3) was similar for all three groups at both time frames. Nonetheless, at 6 years and last follow-up, IBD-related abdominal surgery rates were significantly lower for A1 vs. A2 and vs A3. Conclusions: While there are some disease location differences that emerge with increasing age of presentation (more isolated colonic disease and less perineal disease) disease behavior over time is similar regardless of age at diagnosis and surgeries were least likely in those diagnosed prior to age 16. Our study did not corroborate a more aggressive course for CD that presents in the pediatric age group. Outcomes at 6 years from diagnosis

Mo1342 A Concept Recognition Tool to Identify the Surgical Complications of Crohn's Disease in Electronic Health Records

Background: Previous studies have shown that diagnosis of Crohn's disease (CD) in childhood is associated with a different phenotype when compared to adults (higher risk for ileocolonic disease and fibrostenotic behavior), and may be associated with a more aggressive form of disease requiring more surgery. Those diagnosed at an older age have been reported to have an increased rate of isolated colonic disease and a milder course of disease requiring less surgery. Methods: A comprehensive medical chart review was done for 571 CD patients that were followed in a tertiary referral IBD clinic. Among the parameters that were recorded for each patient at specific time intervals were parameters of disease phenotype according to the Montreal Classification (A1 diagnosed ,16, n=88, A2 diagnosed 16-40, n=287, A3 diagnosed .40, n=77) as well as having surgery. Results: The study included 452 patients that had complete data in the charts. At 6 years from diagnosis and at last follow-up (median11 years), A3 had a higher rate of isolated colonic disease than A1 but not in comparison to A2. Perianal involvement was significantly less common in A3 than A1 or A2. Complicated disease behavior (B2/B3) was similar for all three groups at both time frames. Nonetheless, at 6 years and last follow-up, IBD-related abdominal surgery rates were significantly lower for A1 vs. A2 and vs A3. Conclusions: While there are some disease location differences that emerge with increasing age of presentation (more isolated colonic disease and less perineal disease) disease behavior over time is similar regardless of age at diagnosis and surgeries were least likely in those diagnosed prior to age 16. Our study did not corroborate a more aggressive course for CD that presents in the pediatric age group. Outcomes at 6 years from diagnosis

Outcome of end-to-end urethroplasty in post-traumatic stricture of posterior urethra.

To determine the outcome of delayed end-to-end anastomotic urethroplasty in blind post-traumatic stricture of posterior urethra at our setup. Case series. Department of Urology and Renal Transplantation, Quaid-e-Azam Medical College/Bahawal Victoria Hospital, Bahawalpur, from January 2009 to June 2011. Adult patients with completely obliterated post-traumatic stricture of posterior urethra ≤ 2 cm were included in the study. Patients with post-prostatectomy (TUR-P, TVP) stricture, stricture more than 2 cm in size or patients of stricture with neurogenic bladder and patients with any perineal disease were excluded from the study. Retrograde urethrogram and voiding cysto-urethrogram was done in every patient to assess stricture length and location. Stricture excision and delayed end-to-end anastomosis of urethra with spatulation was performed in every patient. Minimum followup period was 6 months and maximum 18 months. There were 26 cases with road traffic accident (indirect) and 14 had history of fall/direct trauma to perineum or urethra. Majority of the patients (57.5%) were between 16 to 30 years of age. Twelve (30.0%) patients developed complications postoperatively. Early complications of wound infection occurred in 01 (2.5%) patient. Late complications were seen in 11 (27.5%) patients i.e. stricture recurrence in 7 (17.5%), erectile dysfunction in 2 (5.0%), urethrocutaneous fistula and urinary incontinence in one patient (2.5%) each. Success rate was 70.0% initially and 87.5% overall. Delayed end-to-end anastomotic urethroplasty is an effective procedure for traumatic posterior urethral strictures with success rate of about 87.5%.

Reconstrucción de gran defecto perineal con colgajos musculocutáneos de grácilis. A propósito de un caso

Treatment of perineal oncologic disease is currently directly related to functional reconstruction. There are cases in which the spread of the disease is associated with large defects, thus the possibility of achieving adequate coverage becomes the determining factor for the resectability of certain lesions. We describe a clinical case study on a recurrence of squamous cell in situ carcinoma managed with a large posterior defect at resection and reconstructed with gracilis musculocutaneous flaps.

Topical tacrolimus 0.1% ointment for treatment of cutaneous Crohn’s Disease

BackgroundCutaneous Crohn’s Disease is a notoriously difficult condition to treat and causes significant morbidity, impacting heavily on quality of life. This is the first study in adults examining the effect of topical tacrolimus on the different cutaneous manifestations of Crohn’s Disease.MethodsThis open label observational study of 20 patients with heterogeneous forms of cutaneous Crohn’s disease used topical tacrolimus 0.1% ointment once daily to affected areas for 12 weeks with a maximal total dose of 90g. Therapy was stopped at 12 weeks to assess whether the condition relapsed. Thereafter relapsing patients optionally continued an open label extension of topical tacrolimus therapy and were observed for a total of 12 months.ResultsOf seventeen patients completing the twelve-week study, fifteen improved using a specifically designed physicians’ global severity scale. One patient cleared, four showed a pronounced improvement (51-75%) and ten demonstrated a mild (1-25%) or moderate improvement (25-50%) in twelve weeks. Over twelve months eleven patients remained in the study, nine of which improved, one cleared and one showed no change. Perineal disease responded better with two out of twelve clearing, four showing pronounced benefit and four mild to moderate improvement. Long-term application of 0.1% tacrolimus applied to broken skin and mucosa was safe and serum levels of tacrolimus were undetectable in all subjects throughout the study.Conclusion0.1% tacrolimus ointment was safe and effective in treating cutaneous manifestations of Crohn’s disease, particularly perineal disease and pyoderma gangrenosum, yet it seldom cleared the condition.Clinical trial registrationClinicalTrials.gov Protocol Registration System ID: 33000332

Incidence of Hidradenitis Suppurativa and Associated Factors: A Population-Based Study of Olmsted County, Minnesota

There are no population-based incidence studies of hidradenitis suppurativa (HS). Using the medical record linkage system of the Rochester Epidemiology Project, we sought to determine the incidence of the disease, as well as other associations and characteristics, among HS patients diagnosed in Olmsted County, Minnesota, between 1968 and 2008. Incidence was estimated using the decennial census data for the county. Logistic regression models were fit to evaluate associations between patient characteristics and disease severity. A total of 268 incident cases were identified, with an overall annual age- and sex-adjusted incidence of 6.0 per 100,000. Age-adjusted incidence was significantly higher in women compared with men (8.2 (95% confidence interval (CI), 7.0-9.3) vs. 3.8 (95% CI, 3.0-4.7). The highest incidence was among young women aged 20-29 years (18.4 per 100,000). The incidence has risen over the past four decades, particularly among women. Women were more likely to have axillary and upper anterior torso involvement, whereas men were more likely to have perineal or perianal disease. In addition, 54.9% (140/255) patients were obese; 70.2% were current or former smokers; 42.9% carried a diagnosis of depression; 36.2% carried a diagnosis of acne; and 6% had pilonidal disease. Smoking and gender were significantly associated with more severe disease.

Disintegrating perineal disease in patients with urethral stricture and HIV infection: a review of 12 cases

The aim of this study was to defne disintegrating perineal disease (DPD) and to determine whether the fulminating nature of the condition could be explained by urine and perineal swab microbiology or perineal histology. A retrospective study that included 12 male patients with urethral strictures and DPD was performed. DPD was defned as a chronic, destructive, purulent perineal infammation with multiple fstulae or sinuses of the perineum, scrotum or penoscrotal area, which continued for more than six weeks despite a patent urethra after direct vision internal urethrotomy (DVIU) or urinary diversion by means of suprapubic cystostomy. The median patient age was 43.5 years (range of 22-68 years). The patients all tested positive for human immunodefciency virus (HIV) infection. Their mean CD4 count was 340 cells/mm3 (range of 244-1 252 cells/mm3). Histology of the fstula tracts showed non-specifc infammation in 8 patients (66.7%), tuberculosis in 2 (16.7%), hydradenitis suppurativa in one (8.3), and squamous ...

Perineal Pyoderma Gangrenosum in a Patient with Inflammatory Bowel Disease

Purpose: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by necrotic, violaceous ulcers that follow an aggressive, painful, and debilitating course. Lesions usually affect the lower extremities, but often involve other cutaneous regions, such as peristomal tissues. Identifying atypical PG in unusual locations is difficult as the diagnosis is one of exclusion. The differential diagnosis for perineal ulcers includes hidradenitis suppurativa, ecthyma gangrenosum, herpes simplex, cutaneous Crohn's disease, cutaneous tuberculosis, cutaneous lymphoma, vasculitis, lichen planus, and autoimmune blistering conditions. We describe a 60-year-old man with ulcerative colitis (UC) without prior extraintestinal manifestations who was post-colectomy for dysplasia and presented with perineal dermatosis 5 months after ileostomy closure. His post-operative course was marked by frequent bowel movements with urgency, tenesmus and subsequent perineal irritation. Despite local therapy and oral antibiotics, his perineal irritation progressed to excoriations, and then exquisitely painful ulcerations. He was then admitted and evaluated by a multidisciplinary team including gastroenterology, dermatology, infectious diseases and colorectal surgery. Minimal enteric inflammation limited to the pouch was seen on endoscopy and histopathology. Perineal biopsy showed non-specific neutrophilic inflammatory infiltrate. Wound cultures grew Pseudomonas aeruginosa. He was placed on IV antibiotics for possible ecthyma gangrenosum. After five days of nonresponse, it was felt that his lesions represented PG despite the indolent perineal disease course. IV steroids were added with no effect, and IV cyclosporine was then started with dramatic improvement over the next 2 weeks and resolution after 2 months on oral cyclosporine. Interestingly, the patient's gastrointestinal symptoms also began to subside. An outpatient pouchoscopy for recurrent diarrhea with pain on defecation revealed deep stellate lesions in the distal pouch, and the patient's IBD diagnosis was changed to Crohn's disease. The PG remained quiescent. He was transitioned from cyclosporine to infliximab. Atypical presentations of PG are a diagnostic dilemma for clinicians, and a broad differential diagnosis must be entertained, especially in IBD patients. We report here the first case in the literature of perineal PG in an adult IBD patient.Figure: No Caption available.

Tuberculose pleural após uso de adalimumabe na doença de Crohn: relato de caso

O tratamento da doença de Crohn perineal é feito pela combinação da terapia medicamentosa e da cirúrgica. A terapia biológica é importante devido à eficácia clínica na indução e manutenção de remissão da doença. No entanto, devido à ação imunomoduladora e imunossupressora, o uso de biológicos como infliximabe e adalimumabe eleva o risco de infecções oportunistas. Relatou-se o caso de paciente feminino, 28 anos, diagnosticada com doença de Crohn perineal, em uso de azatioprina e adalimumabe. Há sete dias com tosse seca, febre vespertina e dispneia. Ao exame físico, febril, desidratada, diminuição do murmúrio vesicular nos campos médio e inferior do hemitoráx direito e cicatriz de fistulotomia anorretal sem sinais flogísticos. A radiografia de tórax mostrou derrame pleural em hemitórax direito, e a análise do líquido pleural constatou adenosina deaminase elevada, nível de glicose normal e citologia diferencial com 88% de monomorfonucleares. Foi estabelecido o diagnóstico de tuberculose pleural, e a paciente foi medicada com esquema tríplice (rifampicina, isoniazida e pirazinamida) por seis meses associada à prednisona 40 mg/dia, por um mês, com posterior desmame do corticoide. Atualmente, encontra-se assintomática e em uso de ciprofloxina 1 g/dia para a doença de Crohn perineal.

Combined approach with infliximab, surgery, and methotrexate in severe fistulizing anoperineal Crohnʼs disease: Results from a prospective study

Infliximab is the only medical therapy that has been proven to be effective in fistulizing Crohn's disease (CD), but the recurrence rate of fistulas is high despite maintenance therapy. The aim of this prospective study was to evaluate the short- and long-term efficacy of a combined schedule with infliximab, methotrexate, and sphincter-sparing surgery in patients with severe fistulizing anoperineal CD. From January 2006 to November 2007, all consecutive patients in three referral centers with severe fistulizing anoperineal CD were prospectively included after primary drainage. At inclusion, patients received three infliximab infusions at weeks 0, 2, and 6, and maintenance therapy with methotrexate. A second optimized surgical step consisting of at least removal of setons was performed between the second and the third infliximab infusions. Thirty-four CD patients (26 women; median age 38.5 years) with complex anoperineal fistula were enrolled (including 9 with recto-vaginal fistulas, and 10 with anorectal stenosis). At week 14 the response rate was 85% with 74% complete responders. At 1 year, 50% were still responders; luminal CD worsening was the major cause of relapse. Median Perineal Disease Activity Index (PDAI) and magnetic resonance imaging (MRI) scores significantly decreased from baseline to week 50. A combined approach with infliximab induction, two surgical sphincter-sparing steps and methotrexate is effective in achieving short-term response in severe fistulizing anoperineal CD. The best maintenance regimen remains to be determined.

Hermansky‐Pudlak Syndrome: Severe Colitis and Good Response to Infliximab

Hermansky‐Pudlak Syndrome: Severe Colitis and Good Response to Infliximab

Perineal extramammary Paget disease responsive to topical imiquimod

SummaryExtramammary Paget disease (EMPD) is a rare neoplasm that arises in skin rich in apocrine glands, such as the axillae and anogenital region and usually affects the elderly. In most cases, EMPD is an apocrine carcinoma in situ, but it can be associated with internal malignancy spreading to overlying skin. Surgical excision with margin control is the generally accepted standard of care. A 74‐year‐old woman presented with a 1 year history of a pruritic eczematous eruption in perineum which on biopsy was diagnosed as EMPD. Because of the location and extent of the tumor, any surgical approach would have been problematic. Imiquimod 5% cream applied three times weekly for 16 weeks induced complete resolution. Topical imiquimod appears to be a promising treatment option for EMPD, especially when surgery is a challenge, but only a few cases have been reported.

Perineal Crohn's Disease

There is a subset of patients with perianal Crohn's disease whose course is unusually severe. The hypotheses of this study are that these patients often have direct perineal involvement with Crohn's disease and that this involvement can be recognized clinically. A prospective database with data from 1989 to 2005 was examined for patients with perianal Crohn's disease. Patients were divided into those with and those without perineal involvement, defined by the presence of at least one of the following findings: spontaneous perineal ulceration; nonhealing, painless fissures; or waxy perineal edema. Presentation, treatment, and outcome of the two groups were compared. Seventy-two patients were identified, 19 with perineal involvement and 63 without. Perineal Crohn's disease was more often seen in women, presented at a younger age, and was associated with less small bowel and more colonic Crohn's. The chances of healing in patients with perineal Crohn's disease were lower (32 vs. 66 percent) and proctectomy was more likely (26 vs. 3.7 percent) than when the perineum was normal. Perineal involvement with Crohn's disease can be diagnosed on physical examination and confirmed by the finding of granulomas. When perineal involvement is suspected, surgery should be avoided and consideration given to medical therapy.

Endoscopic perineal approach to the presacral space: a feasibility study

Currently, pathologies from the presacral space are explored primarily by using transabdominal approaches. Major complications may occur, including bowel and rectal perforation, or bleeding. To avoid and reduce these potentially severe risks, a new surgical approach to the presacral space, which permits exploration through the perineum with minimal invasive techniques, had already been developed and is now further elaborated in a cadaver and clinical study. A prospective study was performed using four cadavers with no history of pelvic or perineal disease. A minimally invasive exploration of the presacral retroperitoneum was performed to examine a potential new anatomical surgical space. After positioning the patients in the prone or supine position, a 1-cm vertical median incision was made in the ano-coccygeal ligament. Entry to the presacral space was first established through blunt-finger and balloon dissection. A 30 degrees 10-mm laparoscope was inserted through a 12-mm trocar, and two additional 5-mm trocars were inserted to avoid injury to the sciatic nerve. A clinical pilot study was performed on three patients using this technique. Under direct vision, a wide dissected cavity was observed, with the rectum and mesorectum retracted ventrally. Access and manipulation of posterior pelvic organs were simplified. Placing cadavers in the jack-knife position provided superior accessibility to the presacral space when compared with a supine position. Clear exposure of the sacrum, mesorectum, ureters and bladder, prostate region, iliac vessels (with its branches), and lymph nodes was achieved. Endoscopic perineal approach to the presacral space was considered.

Myocutaneous flaps and proctocolectomy in severe perianal Crohn’s disease—a single stage procedure

Complex perianal wounds can be extremely difficult to treat and primary closure of these defects can be a challenge even for experienced surgeons. So far, myocutaneous flaps for wound closure after removal of malignant tumors are a well-accepted option, but there are only a few reports focusing on the primary closure of the perineal wound after proctocolectomy for Crohn's disease. We describe our experience with wide excision of the diseased perineum using a combined abdominoperineal two-team approach. We performed proctocolectomy with permanent ileostomy in five patients with longstanding extensive Crohn's disease. All five patients had fistulizing perineal Crohn's disease combined with Crohn's colitis. Each patient received at least one flap for primary wound closure, either a rectus abdominis myocutaneous flap or a gracilis flap. Indication for surgical intervention included anal or bowel stenosis, septic condition, fecal incontinence, or a combination of these features. One patient had a simultaneous adenocarcinoma of the sigmoid colon. Five patients underwent a total of seven flaps. Three months after surgery, complete healing was achieved in all patients; one patient suffered recurrence in the region of his right thigh. Mean follow up was 19.6 months (range-12-43 months). Myocutaneous flaps are a promising therapeutic option in patients with chronic perianal disease. With the transposition of well-vascularized tissue into the perineal defect, complete healing and control of sepsis can be achieved in the majority of patients.

A case report of Hailey–Hailey disease treated with alefacept (Amevive®)

Hailey-Hailey disease (chronic benign familial pemphigus) is a chronic, recurrent blistering disorder characterized clinically by erosions occurring primarily in intertriginous regions and histologically by suprabasilar acantholysis. We report a case of Hailey-Hailey disease initially unresponsive to multiple topical corticosteroids, tetracycline, dapsone, ciclosporin, isotretinoin, prednisone, methotrexate, topical ciclopirox, tazarotene cream, pimecrolimus cream and tacrolimus ointment. Partial response of this patient's perineal disease was achieved with Amevive 15 mg weekly for 12 weeks, intramuscularly. To our knowledge, this case represents the first such published report of successful treatment of Hailey-Hailey disease using alefacept.

Primary and metastatic perineal adenocarcinoma: aetiology and management

Objective: Adenocarcinoma of the perineum is unusual. This series of nine patients highlights the challenges of management.Results: Six male and three female patients aged 29–85 years presented to a single unit, five were T4 and three were T3 tumours. Pain and swelling in the perineum and perianal tissues remote from the anal canal occurred in six patients whilst three patients had chronic perineal fistulae, two of whom had longstanding Crohn's disease. In eight patients a primary adenocarcinoma was identified. One patient with fistulating perineal Crohn's disease had multifocal mucinous adenocarcinoma without evidence of a gastrointestinal primary. Radical multimodality treatment including long course chemo‐radiotherapy (CRT) and radical abdominoperineal resection was used in seven patients, four of whom required perineal reconstruction with myocutaneous flaps. Two patients had CRT without surgery, (one unresectable and one refused surgery). Negative resection margins were achieved in six out of seven patients. All seven patients undergoing resection were well palliated with relief of pain and currently four patients remain free of disease with only one patient developing further perineal disease remote from the surgical resection site.Conclusion: Perineal adenocarcinoma may be associated with an occult colorectal primary or chronic fistulae. Lower GI endoscopy and biopsies of nonhealing mucous producing fistulae should be undertaken to establish the diagnosis. Radical surgery often achieves local disease control.

Perineal metastatic Crohn's disease: a case report and review of the literature

Metastatic Crohn's disease is an uncommon complication of Crohn's disease defined as granulomatous inflammation not contiguous with inflammatory disease in bowel. We report on the presentation and management of a 36-year-old man, who had undergone panproctocolectomy 11 years ago, with complex fistulous disease in his perineum, which demonstrated granulomas histologically after resection. We review six similar case reports. Optimal treatment would appear to be by surgical debridement.

Primary and metastatic perineal adenocarcinoma: aetiology and management

Objective: Adenocarcinoma of the perineum is unusual. This series of nine patients highlights the challenges of management. Method: Six male and three female patients aged 29–85 years presented to a single unit, five were T4 and three were T3 tumours. Results: Pain and swelling in the perineum and perianal tissues remote from the anal canal occurred in six patients whilst three patients had chronic perineal fistulae, two of whom had longstanding Crohn's disease. In eight patients a primary adenocarcinoma was identified. One patient with fistulating perineal Crohn's disease had multifocal mucinous adenocarcinoma without evidence of a gastrointestinal primary. Radical multimodality treatment including long course chemo-radiotherapy (CRT) and radical abdominoperineal resection was used in seven patients, four of whom required perineal reconstruction with myocutaneous flaps. Two patients had CRT without surgery (one unresectable and one refused surgery). Negative resection margins were achieved in six of seven patients. All seven patients undergoing resection were well palliated with relief of pain and currently four patients remain free of disease with only one patient developing further perineal disease remote from the surgical resection site. Conclusion: Perineal adenocarcinoma may be associated with an occult colorectal primary or chronic fistulae. Lower GI endoscopy and biopsies of non-healing mucous producing fistulae should be undertaken to establish the diagnosis. Radical surgery often achieves local disease control.