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Background: Dyskeratosis congenita (DKC), also known as Zinsser–Cole–Engman syndrome, is a progressive genetic disease with a triad of reticulate skin pigmentation, nail dystrophy, and leukoplakia. Approximately 8–10% of patients with DKC develop malignancies, and cases of colorectal cancer with DKC in young people have been reported previously. Case presentation: A 25-year-old man with DKC since approximately 10 years of age developed fever and lower abdominal discomfort. Diagnostic imaging revealed locally advanced rectal cancer with lymph node metastasis, direct invasion of the prostate, and pelvic abscess due to tumor microperforation (cT4bN2M0 cStage IIIC). Biopsy showed well to moderately differentiated ductal adenocarcinoma. Genetic testing was negative for RAS and BRAF gene mutations, and microsatellite instability (MSI) testing was also negative. After sigmoid colostomy, the patient was treated with total neoadjuvant therapy (TNT) with systemic chemotherapy (six courses of FOLFOX + panitumumab) followed by chemoradiation therapy (50.4 Gy with capecitabine). After TNT, the primary tumor and metastatic lymph nodes shrank. According to the findings of colonoscopy and magnetic resonance image (MRI), we diagnosed near complete response (near-CR) and decided to follow the patient without surgery by every 3 months re-evaluation. However, 5 months after TNT, tumor regrowth was detected on colonoscopy and imaging, and the patient underwent total pelvic exenteration. He developed paralytic ileus as a postoperative complication, and was discharged on the 38th postoperative day. Pathological examination revealed a residual tumor with invasion of the periprostatic tissue. There was no metastasis in the pararectal and lateral pelvic lymph nodes, but one extramural non-contiguous cancerous extension (tumor deposit) was observed (ypT4bN1cM0 ypStage IIIC). The patient has been free of recurrence for one year after surgery. Conclusions: DKC often develops into various tumors in the digestive system at an early age; therefore, appropriate surveillance may be required. In addition, considering that cancers in patients with DKC occur at a young age, fertility preservation and survivorship are also important, and adequate explanations and care should be provided to patients before and after treatment. [ABSTRACT FROM AUTHOR]

Background: The purpose of this study was to clarify the efficacy and safety of transanal minimally invasive surgery (TAMIS) for total pelvic exenteration (TPE) in advanced primary and recurrent pelvic malignancies. Methods: Using a prospectively collected database, we retrospectively analyzed the clinical, surgical, and pathological outcomes of TAMIS for TPE. Surgery was performed between September 2019 and April 2023. The median follow-up period was 22 months (2–45 months). Results: Fifteen consecutive patients were included in this analysis M:F = 14:1 and median (range) age was 63 (36–74). Their diagnoses were as follows: primary rectal cancer (n = 5; 33%), recurrent rectal cancer (n = 4; 27%), primary anorectal cancer (n = 5; 33%), and gastrointestinal stromal tumor (n = 1; 7%). Bladder-sparing TPE was selected for two patients (13%). In nine of 15 patients (60%) the anal sphincter could be successfully preserved, five patients (33%) required combined resection of the internal iliac vessels, and two (13%) required rectus muscle flap reconstruction. The median operative time was 723 min (561–1082), and the median intraoperative blood loss was 195 ml (30–1520). The Clavien–Dindo classifications of the postoperative complications were as follows: grade 0–2 (n = 11; 73%); 3a (n = 3; 20%); 3b (n = 1; 7%); and ≥ 4 (n = 0; 0%). No cases of conversion to laparotomy or mortality were observed. The pathological results demonstrated that R0 was achieved in 14 patients (93%). Conclusions: The short-term outcomes of this initial experience proved that this novel approach is feasible for TPE, with low blood loss, acceptable postoperative complications, and a satisfactory R0 resection rate. [ABSTRACT FROM AUTHOR]

Introduction: Empty pelvis syndrome (EPS) has been defined as a complications arising as a sequel of empty space created after extensive pelvic surgery involving perineal resection. However this definition has been heterogenous throughout the limited literature available. Hence, EPS is a significant yet under recognized complication vexing both patients and surgeons. Even till date, prevention and management of EPS remain a challenge. Various preventive strategies have been employed each with its own complications. Few small studies mentioned incidence of this dreaded complication in between 20 and 40%. But most of these studies quote vague evidence and especially only after TPE surgeries. To the best of our knowledge, incidence after APR and PE has never been mentioned in literature. Purpose: To assess the clinical burden of empty pelvis syndrome in patients undergoing abdominoperineal resection (APR), posterior exenteration (PE), or total pelvic exenteration (TPE) for low rectal cancers. Methods: This is a retrospective audit from a high-volume tertiary cancer center in India. Patients who underwent APR, PE, or TPE between the years 2013 to 2021 were screened and analyzed for incidence, presentation, and management of empty pelvic syndrome (EPS). Results: A total of 1224 patients' electronic medical records were screened for complications related to empty pelvis. The overall incidence of EPS was 95/1224 (7%) with 55/1024 (5%) in APR, 8/39 (20.5%) in PE, and 32/143 (21.9%) in TPE. The most common clinical presentation was small bowel obstruction 43/95 (45.2%) and most presented late, 56/95 (60%), i.e., after 30 days of surgery. Most of the patients who had EPS were managed conservatively 55/95 (57%). Conclusion: EPS is a significant clinical problem that can lead to major morbidity, especially after exenterative surgeries warranting effective preventive strategies. [ABSTRACT FROM AUTHOR]

Background: Total pelvic exenteration (TPE) with intent to achieve a pathological R0 resection is now considered as the only chance of a long-term survival for locally advanced rectal cancer (LARC) invading into adjacent organs. Lately, laparoscopic total pelvic exenteration (LTPE) is performed and achieved in several specialized centers and showed a promising application prospect. Although this is universally realized by surgeons, there are only few specialized centers to perform this complex surgery, due to concerns about the high morbidity and mortality. The techniques associated need to be disclosed and facilitated. Objective: The aim of this article is to introduce a fascial space priority approach for laparoscopic TPE step by step (with video). Methods: We describe here a fascial space priority approach for LTPE in highly selected patients with locally advanced rectal cancer. The main principle of this approach is that all of the pelvic organs are considered as a whole, the non-vascular spaces surrounding it are separated in the first place, the vascular pedicle and nerve pedicle of pelvic organs can be isolated and then transected precisely. Meanwhile, the associated key landmarks of this approach are disclosed (see the video). Results: The ureterohypogastric nerve fascia (UHGNF) and the vesicohypogastric fascia (VHGF) are two vital embryological planes on the lateral compartment of pelvis. The spaces on either side of them together with the retrorectal space, the space of Retzius, are all non-vascular spaces, and dissection of these spaces in LTPE surgery can be achieved simply and practicably. The ureter, the umbilical artery, the arcus tendinous fasciae pelvis (ATFP), piriformis and the puboprostatic ligament (PPL) are all important landmarks during surgery. Step-by-step illustration with precise anatomical landmarks in the present video may lead to less intraoperative blood loss and complications. Conclusions: LTPE with fascial space priority approach might be a standard surgical procedure for total pelvic exenteration with clear anatomy and reduced blood loss. [ABSTRACT FROM AUTHOR]

Copyright of Colo-Proctology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: Surgery for anal fistula cancer (AFC) associated with Crohn's disease usually entails extensive perineal wounds and dead space in the pelvis, which is often filled with a myocutaneous flap. However, use of a myocutaneous flap is invasive. We report a case of total pelvic exenteration (TPE) for AFC in which a myocutaneous flap was avoided by using an omental flap and negative pressure wound therapy (NPWT). Case presentation: The patient was a 47-year-old woman who had been treated for Crohn's disease involving the small and large intestine for 30 years and had repeatedly developed anal fistulas. She was referred with a diagnosis of AFC that had spread extensively in the pelvis. We performed laparoscopic TPE via a transperineal endoscopic approach. To prevent infection in the large skin defect and extensive pelvic dead space postoperatively, the perineal wound was reconstructed using an omental flap and NPWT. During 20 days of NPWT, the wound steadily decreased in size and closed on postoperative day (POD) 20. She was discharged without complications on POD 30. Discussion: NPWT is useful for preventing perineal wound infection and promoting granulation tissue formation. However, direct contact with the intestine may lead to intestinal perforation. In this case, the combination of an omental flap with NPWT effectively prevented surgical site infection. The flap filled the large pelvic dead space and physically separated the intestine from the polyurethane foam used for NPWT. Conclusion: NPWT and an omental flap may become an option when performing TPE. [ABSTRACT FROM AUTHOR]

Background: In Japan, Crohn's disease (CD)-related cancers occur most frequently in the anal canal. Many patients with advanced CD-related cancer require total pelvic exenteration (TPE) based on their medical history, and choosing the most effective method for urinary diversion is a major concern. We herein report the first case of CD-related cancer treatment with urinary diversion using a gastric conduit after TPE in Japan. Case presentation: A 51-year-old man with a 25 year history of CD was referred to our institution after having been diagnosed with fistulae between the rectum and urethra. Sigmoidoscopy revealed stenosis of the anal canal, and histological examination of this lesion led to a diagnosis of mucinous adenocarcinoma. Magnetic resonance imaging showed that the tumor had invaded the prostate and left internal obturator muscle, and TPE with left internal obturator muscle resection was planned. Urinary diversion was performed with a gastric conduit. The gastric conduit was created by trimming a gastric tube to a 1.5 cm width via stapled resection of the greater curvature, and the branches of the right gastroepiploic artery were preserved as feeding vessels. The ureters were raised from the mesentery on the right side of the ligament of Treitz. Ureterogastric anastomosis was performed using the Wallace technique, and the entire anastomosis was then retroperitonealized. The anastomotic site had a bleeding tendency, but hemostasis was obtained by proton pump inhibitor administration and discontinuation of enoxaparin, which had been administered to prevent venous thrombosis. No other major complications occurred, and the patient's quality of life was recovered 6 months after surgery. Conclusion: Urinary diversion using a gastric conduit is a feasible treatment option for patients with CD-related anorectal cancer requiring TPE. [ABSTRACT FROM AUTHOR]

Introduction: Total pelvic exenteration (TPE) for rectal cancers is associated with significant morbidity. We evaluated the complications related to urinary reconstruction following TPE and factors predicting urologic morbidity. Methods: Retrospective analysis of TPE patients with incontinent urinary diversions between August 2013 and January 2020. Results: One hundred TPE were performed with 96 ileal conduits (IC). Early complications occurred in 10 patients that included uretero-ileal leaks (5%), conduit-related complications (3%), and acute pyelonephritis (3%). Late complications were seen in 26% of patients with uretero-intestinal strictures in 11%. Mortality attributable to urinary complications was seen in 2%. No single factor, including prior radiation, recurrent disease, type of anastomosis, or blood loss, predicted development of urinary morbidity. Conclusion: Conduit urinary diversion following TPE is associated with high urinary morbidity rate but low mortality. It can be safely performed even after previous surgeries and radiation by a dedicated colorectal team. [ABSTRACT FROM AUTHOR]

Purpose: Perineal wound infection (PWI) rates are high after abdominoperineal resection (APR) and total pelvic exenteration (TPE). This study identified risk factors for PWI after surgery for anorectal tumors and examined the relationship between the surgical excision volume with the PWI degree.Methods: A retrospective review involving 135 patients who underwent surgical excision of anorectal tumors was performed. Superficial PWI included cellulitis and superficial dehiscence; deep PWI included major dehiscence, perineal abscess, and presacral abscess. The adjacent organ resection type was classified according to the dead space size formed by surgical excision.Results: Of the 135 patients, 119 underwent APR, and 16 underwent TPE. PWI occurred in 75 patients (superficial PWI, 44; deep PWI, 31). Adjacent organ resection was an independent risk factor for PWI. The cases with adjacent organ resection were classified into small-defect APR, large-defect APR, and TPE. Large-defect APR and TPE cases had significantly higher rates of deep PWI than APR cases without adjacent organ resection.Conclusions: Adjacent organ resection involving the removal of one or more organs and that involving wide-range muscle resection are strong risk factors for deep PWI. [ABSTRACT FROM AUTHOR]

Purpose: The clinical outcomes of patients who have undergone total pelvic exenteration (TPE) for locally advanced primary colorectal cancer have not been satisfactory. For the last 13 years, we have performed layer-oriented, en bloc resection of tumor for which TPE is indicated, in the hope of improving postoperative outcomes. The clinical outcomes of these cases were retrospectively analyzed. Methods: A total of 54 patients who underwent TPE from 1986 to 2013 were retrospectively analyzed. Since 2002, a layer-oriented removal for clinical T4 colorectal cancer, as in T3 or less invasive tumors removed by total mesorectal excision, was applied to 23 cases for which TPE was indicated. Postoperative mortality, morbidity, overall survival (OS), and disease-free survival (DFS) were evaluated. Results: On univariate analysis, good postoperative OS and DFS were associated with the layer-oriented operative maneuver, blood loss less than 2000 mL, negative nodal metastasis, and no preoperative radiation therapy. Male sex was the marginal determinant correlated with good OS and DFS. Depth of invasion to T3 was the marginal determinant correlated with good DFS. On multivariate analysis using the 4 factors identified on univariate analyses, the layer-oriented operative procedure was a significant determinant for both good OS and DFS, together with negative nodal metastases. Postoperative mortality and morbidity in the layer-oriented excision were acceptable. Conclusion: For primary colorectal cancers for which TPE is indicated, layer-oriented excision was a safe and effective procedure, and it may be recommended as one of the standard surgical approaches in TPE. [ABSTRACT FROM AUTHOR]

A vast metastatic tumor mass of recurrent rectal carcinoma in the intrapelvic organs is commonly considered unsuitable for total pelvic exenteration (TPE); first, because it is unlikely that it would improve the prognosis and health-related quality of life of the patient, and second, because of the difficulties involved in this surgical technique. However, by using a plastic surgery technique involving reconstruction by filling the pelvic dead space with pedicle myocutaneous flaps (PMF), primary wound closure and extensive resection of the perineum can be achieved, whereby postoperative metastasis may be prevented. We report herein the case of a 71-year-old man found to have local recurrence in the perineum with extensive invasion of the soft tissue as well as adjacent organs, 20 months after abdominal perineal resection for rectal carcinoma. TPE with extensive resection of the perineal soft tissue was performed, followed by perineal reconstruction and packing of the pelvic dead space with PMF, mainly constructed from the gracilis and sartorius muscles of both femurs. His postoperative course was uneventful and he has remained free of local recurrence and symptomatic perineal complaints for 1 year. In this report, we examine the effectiveness of using the gracilis muscle for PMF in intrapelvic and perineal reconstruction after TPE. [ABSTRACT FROM AUTHOR]

Multidisciplinary treatment was administered to a 32 year-old man with primary linitis plastica of the rectum, which was considered inoperable due to an extensive local spread at the first operation. Thirty KE of OK-432 was injected into the tumor per week (total 90-KE) and 5 KE of OK-432 was inoculated into the intracutaneous space per week (total 125 KE). Also methotrexete (MTX 50 mg) and 5-FU (500 mg) combined were given each week by intra-arterial infusion (total 20 courses). After this immuno-chemotherapy the CEA decreased from the pretreatment value of 12.7 ng/ml to 3.6 ng/ml and the tumor size was reduced. A total pelvic exenteration could then be performed almost curatively. As a result, this patient was able to return to the society with a better quality of life and has survived for 20 months since presentation. [ABSTRACT FROM AUTHOR]

Copyright of International Journal of Colorectal Disease is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Der Chirurg is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Primary malignant melanoma of vagina is a rare variant of melanoma and usually associated with a grave prognosis. Radical surgery is the only treatment option with reasonable loco regional control. A case of primary malignant melanoma involving whole of vagina infiltrating urethra and reaching up to vulva was treated by surgery and postoperative radiotherapy. The tumor was infiltrating bladder and rectum reaching the anal sphincter. Total pelvic exenteration was done to achieve tumor-free surgical margins. One year after treatment, patient is disease free. [ABSTRACT FROM AUTHOR]