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Desmoid tumor of the mesentery in a patient after restorative proctocolectomy as a result of familial adenomatous polyposis – case reports

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Krauze M., Nałęcz A., Dębski K., Dobosz M.

Polish Journal of Surgery

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2018

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vol. 90(3)

53-58

EN

Desmoid (desmoid tumor) is a cytologically benign fibrous tumor that originates from musculoskeletal structures of the entire body [1]. The term „desmoid”, first introduced by Muller in 1838, derives from the Greek word desmos, which means tendinous [2]. The etiopathogenesis of desmoid is not fully understood, most reports of publications regarding its treatment are based on individual case reports. The prognosis is good, and healing is achieved mainly through surgical excision of the lesion. The aim of the paper is to present a case of a 33-year-old woman with diagnosed familial polyposis in the colon, who has been identified with mesenteric desmoid.

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Familial Adenomatous Polyposis; 30-Year Observation of a Female Patient – Case Report

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Wnęk B., Strugała M., Łożyńska-Nelke A., Burdyński R., Krokowicz P.

Polish Journal of Surgery

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2015

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vol. 87

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issue 2

86-90

EN

Familial adenomatous polyposis (FAP) is a colorectal cancer syndrome caused by a germline mutation inherited in an autosomal-dominant pattern with a 100% penetrance. Our detailed case report presents a history of a 55-year-old FAP female patient who had been under constant clinical observation for 30 years. The disease was diagnosed at the age of 22. The patient underwent restorative proctocolectomy with ileal pouch-anal anastomosis (PRC-IPAA). During our follow-up extra-colonic manifestations occurred such as a desmoidtumour, fundic gland polyps in the stomach and duodenal polyps also in the periampullary region. Apart from disease-related symptoms the patient manifested other complications such as small bowel adhesive obstruction, benign breast tumours, uterine myomas, cholelithiasisand thyroid nodules. Our analysis of the above case presents advantages of a long-term medical observation of a FAP patient carried out by a specialist surgical medical centre

3

Sexual activity in patients after proctocolectomy with ileal pouch-anal anastomosis

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Kluska P., Dzika-Andrysiak K., Mik M., Zelga P., Włodarczyk M., Kujawski R., Dziki Ł., Dziki A., Trzciński R.

Polish Journal of Surgery

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2021

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vol. 93

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issue 1

19-24

EN

Introduction: Proctocolectomy with ileal pouch-anal anastomosis is the gold standard in the surgical treatment of patients with ulcerative colitis, familial adenomatous polyposis and other colorectal diseases requiring colectomy. The treatment consists in removing the large intestine and creating an intestinal reservoir from the last ileum loop and then anastomosing the intestinal reservoir with the anal canal. Like any surgical procedure, RPC-IPAA also carries the risk of complications, both early and late. Late postoperative complications include sexual dysfunction. Aim: The main goal of the following work is to assess the quality of life and sexual activity in patients having undergone the RPC-IPAA procedure at the General and Colorectal Surgery Clinic. Material and methods: The study group consisted of patients aged 19–79 who had been subjected to RPC-IPAA procedures at the General and Colorectal Surgery Clinic in years 2010–2019. The study was conducted on the basis of a survey consisting of 50 questions about the social and mental condition, medical history and previous treatment as well as the quality of sexual life before and after surgery. The scale used for the assessment of the quality of sex life consisted of 5 grades: very low, low, medium, high, very high. Thirty subjects (21 men and 9 women) took part in the survey. Ulcerative colitis (86.6%) was the most common reason for qualification for restorative proctectomy among the examined patients; less common reasons included familial adenomatous polyposis (13.3%) and synchronous colorectal cancer (3.3%). A vast majority of the surgeries had been performed after 10 years’ duration of ulcerative colitis, and the intestinal reservoir had been functioning for over a year at the time of the examination. In addition, the effect of taking steroids and the impact of early postoperative complications on the quality of sex life of patients was assessed. Results: High or very high sexual activity before surgery was reported by 46% of patients whereas low or very low quality was reported by 13%. The rest of the responders assessed their pre-operative sexual activity as average. After surgery, 23% of patients rated their sexual activity as high or very high while 36.6% of patients rated it as low or very low (P = 0.07). It was also noted that taking corticosteroids before surgery decreased the quality of sex life after surgery (P = 0.07 for activity, P = 0.04 for quality). None of the women surveyed used artificial moisturizing of intimate places during sex. Only 1 person stated that they started using artificial moisturization of intimate places after the procedure (P = 0.5). None of the men surveyed had used pharmacological agents to help them obtain an erection before surgery while as many as 33% of responders reported the need for their use after surgery (P = 0.008). Other postoperative sexual dysfunctions were also registered, such as dyspareunia (13.3%), sensory disorder within the intimate region, fecal incontinence, and urinary incontinence. Conclusions: To sum up, sexual activity and quality of sexual life deteriorated after RPC-IPAA in our patients.

4

Ileal-Pouch-Anal Anastomosis After Restorative Proctocolectomy in Patients With Ulcerative Colitis or Familial Adenomatous Polyposis - 11 Years of Experience

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Bednarz W., Olewiński R., Woldan J.

Polish Journal of Surgery

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2007

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vol. 79

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issue 5

362-367

EN

Restorative proctocolectomy is the "gold standard" in surgical treatment for ulcerative colitis and familial adenomatous polyposis. The two alternative techniques of ileal-pouch-anal anastomosis include handmade and double line stapled suture.The aim of the study was to analyze postoperative complications and functional results of the two types of anastomosis.Material and methods. The study group consisted of 108 patients operated between 1994 and 2005 for ulcerative colitis (n=97) or familial adenomatous polyposis (n=11). Stapled anastomosis was performed in 88 (81.5%) cases and hand-made suture was performed in 20 (18.5%) cases.Results. No significant differences between the two anastomotic techniques were found in terms of postoperative complications and late functional results.Conclusions. The low rate of complications and well-accepted functional outcomes prove that restorative proctocolectomy is a safe surgical procedure that can be offered to patients with ulcerative colitis or familial adenomatous polyposis. Double line stapled suture should be the preferred method of ilealpouch-anal anastomosis, however, the hand-made suture remains its valuable alternative and may be considered in selected cases.

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Transcriptional changes between uninflamed ulcerative colitis and familial adenomatous polyposis pouch mucosa can be attributed to an altered immune response

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Paziewska A., Horbacka K., Goryca K., Mikula M., Jarosz D., Dabrowska M., Krokowicz P., Karon J., Ostrowski J.

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2015

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vol. 62

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issue 1

69-75

EN

A total proctocolectomy with ileal pouch-anal anastomosis (IPAA) is considered the surgery of choice for definitive management of familial adenomatous polyposis (FAP) and some patients with ulcerative colitis (UC). However, this surgical treatment is often associated with pouchitis, a long-term complication that occurs mostly in UC patients. The purpose of this study was to better define the molecular background of pouchitis. A microarray-based survey was performed using pouch mucosal samples collected from 28 and 8 patients undergoing surgery for UC and FAP, respectively. There were 4,770 genes that significantly differentiated uninflamed from inflamed mucosal samples, and their functional features were represented mostly by metabolic and cell proliferation pathways. In contrast, functional analyses of aberrantly expressed genes between UC and FAP samples, irrespective of mucosal inflammation status, revealed multiple pathways and terms that were linked to changes in immune response. Interestingly, the comparison of uninflamed UC and FAP samples identified a set of 29 altered probe sets, including an inflammation-related transcript encoding a Charcot-Leyden crystal (CLC) protein. The most distinct changes in gene expression profiles differentiating uninflamed UC and FAP pouch mucosal samples were attributed to the Gene Ontology category innate immune response. Our study confirmed that alterations in immune responses can be found between patients who underwent surgery for UC and FAP, independent of the pouch inflammation status. This observation may be important when managing IPAA patients.

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Intestinal Pouch Complications in Patients Who Underwent Restorative Proctocolectomy for Ulcerative Colitis and Familial Adenomatous Polyposis in 1985-2008

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Burdyński R., Banasiewicz T., Marciniak R., Biczysko M., Szmeja J., Paszkowski J., Grochowalski M., Maik J., Majewski P., Krokowicz P., Drews M.

Polish Journal of Surgery

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2011

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vol. 83

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issue 3

161-170

EN

Restorative proctocolectomy is considered a surgical treatment of choice in ulcerative colitis (UC) and familial adenomatous polyposis (FAP).The aim of the study was to evaluate postoperative complications in patients who underwent surgery for familial adenomatous polyposis and ulcerative colitis, on the basis of a retrospective data analysis.Material and methods. Data of 138 patients after restorative proctocolectomy performed between 1985 and 2008 were collected at routine follow-up visits in 2004-2008. We evaluated the presence of pouchitis, the degree of ileal pouch mucosa atrophy, the presence of ileal pouch mucosal metaplasia, the presence of ileal pouch malignancies, the necessity for diverting ileostomy, the necessity for pouch resection, and severe faecal incontinence.Results. Complications were observed in 45 (32.4%) patients. Thirty-seven patients developed pouchitis (26.6%). Low-degree dysplasia, severe dysplasia or malignancies were observed in total in 20 patients (14.4%). Six (4.3%) operated patients developed other analysed complications.Conclusions. The most common complications of restorative proctocolectomy were dysplasia and pouchitis. The most common complication in patients operated for UC was pouchitis. The low observed incidence of intestinal pouchitis may be attributed to the implemented prophylaxis of inflammation. Dysplasia was the most common complication in patients undergoing proctocolectomy for FAP. Due to an increased risk of dysplastic lesions as compared with UC patients, careful endoscopic follow-up examinations are obligatory in this patient group. Other analysed complications were uncommon and were mostly a consequence of chronic pouchitis. Clinical symptoms of pouch-related problems were similar in both analysed groups.

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DNA Bank for Polish Patients with a Predisposition for Intestinal Polyposis

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Pławski A., Podralska M., Cichy W., Drews M., Krokowicz P., Słomski R.

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issue 10

465-473

EN

Intestinal polyposis syndromes include a group of diseases conditioned by the occurrence of hereditary mutations. The current paper presents a collection of DNA samples derived from persons from families with a diagnosed adenomatous polyposes which comprise: familial polyposis coli together with its recessive form, Turcot's syndrome, inherited mixed polyposis as well as persons with recognised hamartomatous polyposes: juvenile polyposis, Peutz-Jeghers syndrome, Cowden syndrome and Proteus syndrome.The aim of the study was to present current achievements associated with the establishment of the DNA Bank for intestinal polyposis.Material and methods. Investigations were conducted on DNA isolated from cells of the peripheral blood. The search for mutations in APC, MUTYH, PTEN, BMPR1A, SMAD4 and STK11 genes preconditioning the occurrence of individual diseases was performed employing PCR-SSCP, PCR-HD, DHPLC as well as RFLP techniques and DNA sequencing.Results. At the present time, the DNA Bank comprises the total of 1097 DNA samples derived from 449 families with intestinal polyposis of which 945 samples come from persons in whose families Familial Adenomatous Polyposis (FAP) occurred. In addition, the collected data also contain material for analyses derived from 25 families with Peutz-Jeghers syndrome and 20 families with juvenile polyposis as well as single cases with the Cowden syndrome, Proteus syndrome and desmoid tumors. The performed molecular investigations allowed identification of mutations ranging from 44 to 50%.Conclusions. With regard to the quantity of the material collected for analyses and the efficacy level of the employed molecular methods, the obtained results are in keeping with the results found in the literature from the field of genetics and medicine and do not differ from world standards. The collection of data and materials for investigations in the case of rare diseases allows qualitative, organisational and economic optimisation of the performed investigations.

Refine search results

Desmoid tumor of the mesentery in a patient after restorative proctocolectomy as a result of familial adenomatous polyposis – case reports

Familial Adenomatous Polyposis; 30-Year Observation of a Female Patient – Case Report

Sexual activity in patients after proctocolectomy with ileal pouch-anal anastomosis

Ileal-Pouch-Anal Anastomosis After Restorative Proctocolectomy in Patients With Ulcerative Colitis or Familial Adenomatous Polyposis - 11 Years of Experience

Transcriptional changes between uninflamed ulcerative colitis and familial adenomatous polyposis pouch mucosa can be attributed to an altered immune response

Intestinal Pouch Complications in Patients Who Underwent Restorative Proctocolectomy for Ulcerative Colitis and Familial Adenomatous Polyposis in 1985-2008

DNA Bank for Polish Patients with a Predisposition for Intestinal Polyposis