Publications by authors named "Randolph Steinhagen"

22 Publications

  • Page 1 of 1

Better characterization of operation for ulcerative colitis through the National surgical quality improvement program: A 2-year audit of NSQIP-IBD.

Am J Surg 2021 01 12;221(1):174-182. Epub 2020 Jun 12.

University of California, San Diego School of Medicine, 9500 Gilman Drive, La Jolla, CA, 92093, USA. Electronic address:

Introduction: There is little consensus of quality measurements for restorative proctocolectomy with ileal pouch-anal anastomosis(RPC-IPAA) performed for ulcerative colitis(UC). The National Surgical Quality Improvement Program(NSQIP) cannot accurately classify RPC-IPAA staged approaches. We formed an IBD-surgery registry that added IBD-specific variables to NSQIP to study these staged approaches in greater detail.

Methods: We queried our validated database of IBD surgeries across 11 sites in the US from March 2017 to March 2019, containing general NSQIP and IBD-specific perioperative variables. We classified cases into delayed versus immediate pouch construction and looked for independent predictors of pouch delay and postoperative Clavien-Dindo complication severity.

Results: 430 patients received index surgery or completed pouches. Among completed pouches, 46(28%) and 118(72%) were immediate and delayed pouches, respectively. Significant predictors for delayed pouch surgery included higher UC surgery volume(p = 0.01) and absence of colonic dysplasia(p = 0.04). Delayed pouch formation did not significantly predict complication severity.

Conclusions: Our data allows improved classification of complex operations. Curating disease-specific variables allows for better analysis of predictors of delayed versus immediate pouch construction and postoperative complication severity.

Short Summary: We applied our previously validated novel NSIP-IBD database for classifying complex, multi-stage surgical approaches for UC to a degree that was not possible prior to our collaborative effort. From this, we describe predictive factors for delayed pouch formation in UC RPC-IPAA with the largest multicenter effort to date.
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http://dx.doi.org/10.1016/j.amjsurg.2020.05.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7736277PMC
January 2021

Presentation and Management of Giant Colonic Diverticula.

Am Surg 2019 Dec;85(12):e585-e587

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December 2019

Abdominal Emergencies in Inflammatory Bowel Disease.

Surg Clin North Am 2019 Dec 23;99(6):1141-1150. Epub 2019 Sep 23.

Division of Colon and Rectal Surgery, Department of Surgery, Icahn School of Medicine at Mount Sinai, Box 1259, One Gustave L. Levy Place, New York, NY 10029, USA. Electronic address:

Although improved medical therapies have been associated with decreased rates of emergent intestinal resection for inflammatory bowel disease, prompt diagnosis and management remain of utmost importance to ensure appropriate patient care with reduced morbidity and mortality. Emergent indications for surgery include toxic colitis, acute obstruction, perforation, acute abscess, or massive hemorrhage. Given this broad spectrum of emergent presentations, a multidisciplinary team including surgeons, gastroenterologists, radiologists, nutritional support services, and enterostomal therapists are required for optimal patient care and decision making. Management of each emergency should be individualized based on patient age, disease type and duration, and patient goals of care.
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http://dx.doi.org/10.1016/j.suc.2019.08.007DOI Listing
December 2019

The ACS National Surgical Quality Improvement Program-Inflammatory Bowel Disease Collaborative: Design, Implementation, and Validation of a Disease-specific Module.

Inflamm Bowel Dis 2019 10;25(11):1731-1739

Department of Surgery, Cleveland Clinic Florida, Weston, MA, USA.

Background: Surgery for inflammatory bowel disease (IBD) involves a complex interplay between disease, surgery, and medications, exposing patients to increased risk of postoperative complications. Surgical best practices have been largely based on single-institution results and meta-analyses, with multicenter clinical data lacking. The American College of Surgeons National Surgical Quality Improvement Project (ACS-NSQIP) has revolutionized the way in which large-volume surgical outcomes data have been collected. Our aim was to employ the ACS-NSQIP to collect disease-specific variables relevant to surgical outcomes in IBD.

Study Design: A collaborative of 13 high-volume IBD surgery centers was convened to collect 5 IBD-specific variables in NSQIP. Variables included biologic and immunomodulator medications usage, ileostomy utilization, ileal pouch anastomotic technique, and colonic dysplasia/neoplasia. A sample of the Surgical Clinical Reviewer collected data was validated by a colorectal surgeon at each institution, and kappa's agreement statistics generated.

Results: Over 1 year, data were collected on a total of 956 cases. Overall, 41.4% of patients had taken a biologic agent in the 60 days before surgery. The 2 most commonly performed procedures were laparoscopic ileocolic resections (159 cases) and subtotal colectomies (151 cases). Overall, 56.8% of cases employed an ileostomy, and 134 ileal pouches were constructed, of which 92.4% used stapled technique. A sample of 214 (22.4%) consecutive cases was validated from 8 institutions. All 5 novel variables were shown to be reliably collected, with excellent agreement for 4 variables (kappa ≥ 0.70) and very good agreement for the presence of colonic dysplasia (kappa = 0.68).

Conclusion: We report the results of the initial year of implementation of the first disease-specific collaborative within NSQIP. The selected variables were demonstrated to be reliably collected, and this collaborative will facilitate high-quality, large case-volume research specific to the IBD patient population.
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http://dx.doi.org/10.1093/ibd/izz044DOI Listing
October 2019

Endorectal Advancement Flaps for Perianal Fistulae in Crohn's Disease: Careful Patient Selection Leads to Optimal Outcomes.

J Gastrointest Surg 2019 11 12;23(11):2277-2284. Epub 2019 Apr 12.

UC San Diego Health System, San Diego, CA, USA.

Background: Anorectal fistulae resultant from Crohn's disease (CD) is a clinical challenge. The advent of immune therapy (IT) has altered the way in which fistulae have responded to treatment. Endorectal advancement flap (ERAF) is a surgical procedure that is used to treat complex fistulae. We have employed ERAF as our second stage treatment of choice in this patient population. Our aim was to determine the success of ERAF in treating perianal fistulas in patients with CD in an era of IT.

Methods: Multicenter retrospective review from 2007 to 2017 of all patients with CD and a perianal fistulae who underwent ERAF.

Results: Forty-one flaps were performed in 39 patients with perianal CD with an average follow-up of 797 days. There were no significant differences in patient demographics; however, all patients who were diverted at the time of surgery had successful healing. Of patients, 73.2% were on IT at an average of 380 days prior to surgery. The duration of single-agent therapy was associated with better healing rates (p = 0.03). The overall failure rate was 19.5% (n = 8). Six patients underwent secondary techniques for fistulae closure; five were successful. In combination with the patients who did not initially fail, the overall healing rate was 92.6%.

Conclusions: This study demonstrates several factors that may improve fistulae closure for CD patients. Patients who were diverted prior to surgery did not have a fistulae recurrence. Patients who were on IT longer prior to ERAF were more likely to achieve successful closure.
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http://dx.doi.org/10.1007/s11605-019-04205-0DOI Listing
November 2019

Case series: Incarcerated massive rectal prolapse successfully treated with Altemeier's procedure.

Int J Surg Case Rep 2018 5;51:309-312. Epub 2018 Sep 5.

Viet Duc University Hospital, 40 Trang Thi, Hoan Kiem, Hanoi, Viet Nam. Electronic address:

Introduction: Incarceration and necrosis of rectal prolapse is rare but when it occurs it requires urgent management. Perineal rectosigmoidectomy (Altemeier's procedure) may be a reasonable approach for the treatment of this condition. In some cases, a diverting stoma may be necessary.

Methods: We report two cases of incarcerated massive rectal prolapse, one of which also manifested tissue necrosis, that were successfully treated with perineal rectosigmoidectomy. In one case a diverting colostomy was required. Both patients recovered uneventfully.

Results: A literature review was performed to determine the optimal management of incarcerated and necrotic rectal prolapse, and to determine the indication for fecal diversion.

Conclusion: Perineal rectosigmoidectomy (Altemeier's procedure) can be utilized in emergency circumstances and, in our experience, the procedure was both safe and effective. The need for fecal diversion depends on the condition of the patient and the experience and judgement of the surgeon.
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http://dx.doi.org/10.1016/j.ijscr.2018.08.057DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6170932PMC
September 2018

Incidence and Factors Correlating With Incisional Hernia Following Open Bowel Resection in Patients With Inflammatory Bowel Disease: A Review of 1000 Patients.

Ann Surg 2018 03;267(3):532-536

The Department of Surgery, The Icahn School of Medicine at Mount Sinai, New York, NY.

Objective: The aim of this study was to identify the incidence and factors associated with the development of incisional hernia (IH) in patients with inflammatory bowel disease (IBD) undergoing open bowel resections.

Background: Predisposing factors for IH have not been well studied in patients with IBD undergoing open bowel resection. The role of duration of the disease, nutritional factors, anti-inflammatory treatment, previous operative procedures, wound infection, and other complicating factors remains unclear.

Methods: One thousand patients with ulcerative colitis and Crohn's disease were followed for a mean of 8 years after open bowel resection. The incidence of IH was recorded as well as correlating factors with the development of IH.

Results: The overall incidence of IH in this series was 20% (21% for ulcerative colitis and 20% for Crohn's disease). Statistically significant risk factors for development of IH were wound infection (HR 3.66, P <0.001), hypoalbuminemia (HR 2.02, P = 0.002), history of previous bowel resection (HR 1.60, P = 0.003), creation of ileostomy at the time of procedure (HR 1.53, P = 0.01), history of smoking (HR 1.52, P = 0.013), body mass index at surgery (1.036, P = 0.009), age at surgery (HR 1.021, P <0.001), and age at the onset of disease (HR 1.018, P <0.001).

Conclusions: Patients with IBD have a high incidence of incisional hernia after open bowel resection. Wound infection had the strongest correlation with the development of IH. The other factors were age at onset of IBD, age at surgery, body mass index, serum albumin, presence of ileostomy, previous surgical procedures, and history of smoking. Duration of disease, preoperative steroids, immunosuppressive therapy, and blood transfusion were not found to correlate with IH.
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http://dx.doi.org/10.1097/SLA.0000000000002120DOI Listing
March 2018

Outcome of incisional hernia repair in patients with Inflammatory Bowel Disease.

Am J Surg 2017 Sep 7;214(3):468-473. Epub 2017 Jun 7.

The Department of Surgery, The Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Background: Incisional Hernia (IH) repair in patients with Inflammatory Bowel Disease (IBD) has not been well studied.

Methods: Outcomes of 170 patients with IBD who underwent IH repair were included in the study.

Results: The incidence of recurrence after IH repair in IBD is 27%. Patients with Crohn's disease (CD) had larger defects at the time of repair, higher proportion of bowel resection and a longer postoperative stay when compared to Ulcerative colitis (UC). The only significant predictor of recurrence after IH repair was the number of previous bowel resections prior to hernia repair (HR 1.59, p < 0.01). Three cases (10%) of late onset enterocutaneous fistulas were identified in patients who underwent IH repair with synthetic mesh inlay.

Conclusion: Surgical repair results in a recurrence of IH in 27% of patients with IBD. The number of previous bowel resections is the only factor that correlates with development of recurrent IH in IBD.
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http://dx.doi.org/10.1016/j.amjsurg.2017.05.019DOI Listing
September 2017

Can laparoscopic surgery prevent incisional hernia in patients with Crohn's disease: a comparison study of 750 patients undergoing open and laparoscopic bowel resection.

Surg Endosc 2017 12 18;31(12):5201-5208. Epub 2017 May 18.

Department of Surgery, The Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, New York, NY, 10029, USA.

Background: Incisional hernia (IH) is a frequent occurrence following open surgery for Crohn's disease (CD). This study compares the IH rates of patients with CD undergoing open versus laparoscopic bowel resection.

Methods: Seven hundred and fifty patients with CD operated by the authors at the Mount Sinai Medical Center, New York, USA, were reviewed from a prospectively maintained surgical database. Five hundred patients with Crohn's disease undergoing open surgery were compared to 250 patients undergoing laparoscopic bowel resection.

Results: The mean duration of follow-up in the study population was 6.8 years. Patients undergoing open surgery had a significantly higher age at onset of disease, age at surgery, longer duration of disease, lower serum albumin, history of multiple previous resections, were more likely to be on steroids, needed more blood transfusions, and had an increased necessity for an ileostomy during resection. Nevertheless, the incidence of IH at 36 months was nearly identical in both groups (10.8 vs. 8.4% for open vs laparoscopic). 16% of the patients in the laparoscopic group (range: 7-20%) required conversion to open surgery. Patients undergoing laparoscopic resection that required conversion to open surgery had the highest IH rate at 18%. There was a significant correlation between IH and the length of the midline vertical extraction incision. Patients undergoing laparoscopic resection with intracorporeal anastomosis and small transverse or trocar site extraction incisions had no IH.

Conclusions: A marked decrease or complete elimination of IH in patients with CD undergoing bowel resection may be possible using advanced laparoscopic techniques that require intra-abdominal anastomosis and use of the smallest transverse extraction incisions.
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http://dx.doi.org/10.1007/s00464-017-5588-8DOI Listing
December 2017

Outcomes and cost of diverted versus undiverted restorative proctocolectomy.

J Gastrointest Surg 2014 May 14;18(5):995-1002. Epub 2014 Mar 14.

Division of Colorectal Surgery, Department of Surgery, Mount Sinai Medical Center, New York, NY, 10029, USA,

Background: Some observational studies suggest that diversion during restorative proctocolectomy mitigates the risk of anastomotic complications. However, diversion has its own costs and complications. The aim of this study was to compare the cost and outcomes of diverted to undiverted restorative proctocolectomy.

Methods: This study took advantage of a natural experiment within one surgical department to understand the clinical and financial implications of diversion during restorative proctocolectomy. For the last 10 years, two surgeons routinely diverted all patients undergoing restorative proctocolectomy, and two other surgeons routinely did not. The medical records of 288 consecutive restorative proctocolectomy patients were reviewed. Minimum follow-up time was 1 year, with an average of 4.7 years. Complications rates and costs of care were collected.

Results: There were no significant differences between rates of anastomotic leak, fistula, or hernias in diverted versus undiverted patients. The odds of having stricture (odds ratio (OR) = 17.08, P < 0.001) and small bowel obstruction (OR = 5.05, P = 0.02) were both significantly higher in diverted patients. The average cost per patient was $43,000 more in the routinely diverted patients.

Conclusion: Undiverted restorative proctocolectomy may be the highest value procedure with the most favorable outcomes at the lowest cost.
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http://dx.doi.org/10.1007/s11605-014-2479-3DOI Listing
May 2014

Colorectal surgery in cirrhotic patients.

ScientificWorldJournal 2014 15;2014:239293. Epub 2014 Jan 15.

Division of Colon and Rectal Surgery, Department of Surgery, Icahn School of Medicine at Mount Sinai, P.O. Box 1259, One Gustave L. Levy Place, New York, NY 10029, USA.

Patients with cirrhosis have a greater risk of morbidity and mortality following colorectal surgery. Therefore, preoperative medical optimization and risk assessment using criteria such as the MELD score are vital in preventing complications. Some risk factors include age, urgency of surgery, and ASA score. Postoperative morbidity and mortality are related to portal hypertension, ascites, infection, and anastomotic and stomal complications. This review highlights the assessment of risk and perioperative management of cirrhotic patients undergoing colorectal surgery.
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http://dx.doi.org/10.1155/2014/239293DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3914319PMC
December 2014

Current surgical management of ulcerative colitis.

Mt Sinai J Med 2009 Dec;76(6):606-12

Division of Colon and Rectal Surgery, Department of Surgery, Mount Sinai School of Medicine, New York, NY, USA.

Inflammatory bowel disease is divided into 2 major disease entities: Crohn's disease and ulcerative colitis. Ulcerative colitis is characterized by contiguous inflammation of the colorectal mucosa, always beginning in and involving the rectum and progressing for variable distances proximally within the colon. In ulcerative colitis, medical therapy, which is not curative, is directed at controlling symptoms and reducing the underlying inflammatory process. However, emergent or elective removal of the colon and rectum does cure the disease and also eliminates the possibility of developing a malignancy. Here we present the current surgical treatment of ulcerative colitis and issues in the management of ulcerative colitis. We discuss indications for surgical treatment, elective and emergent operative management, early and late complications of surgery, and functional results.
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http://dx.doi.org/10.1002/msj.20152DOI Listing
December 2009

Prospective analysis of clinician accuracy in the diagnosis of benign anal pathology: comparison across specialties and years of experience.

Dis Colon Rectum 2010 Jan;53(1):47-52

Division of Colon and Rectal Surgery, Department of Surgery, Mount Sinai Medical Center, New York, New York, USA.

Purpose: The majority of patients referred to a colorectal surgeon with anal complaints are told they have "hemorrhoids"; however, many of these patients have other anal pathology causing their symptoms. Therefore, we prospectively evaluated the diagnostic accuracy of physicians for common anal pathology, stratified by specialty and experience.

Methods: Seven common benign anal pathologic conditions were selected (prolapsed internal hemorrhoid, thrombosed external hemorrhoid, abscess, fissure, fistula, condyloma acuminata, and full-thickness rectal prolapse). Prospectively accrued subjects included attending physicians, fellows, residents, and medical students. Subjects were shown images and asked to provide a written diagnosis. We prospectively evaluated the overall diagnostic accuracy and stratified accuracy across specialties and years of clinical experience. Medical students were the control group.

Results: There were 198 physicians and 216 medical students. Overall diagnostic accuracy for physicians was 53.5% and for controls was 21.9% (P < .001). Surgeons had the highest overall accuracy at 70.4%, whereas all of the other groups had an accuracy of <50%. Physicians correctly identified condylomata and rectal prolapse most frequently and hemorrhoidal conditions least frequently. All 7 conditions were correctly identified by 4.1% of subjects and all of the conditions were incorrectly diagnosed by 20.2%. There was no correlation between years of experience and diagnostic accuracy (P = NS).

Conclusion: Diagnostic accuracy for common benign anal pathologic conditions was suboptimal across all clinical specialties. Although many specialties had a diagnostic accuracy that was significantly better than the control group, there was no association between years of experience and accuracy. Improved programs for physician education for these common conditions should be developed.
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http://dx.doi.org/10.1007/DCR.0b013e3181bbfc89DOI Listing
January 2010

Severity of inflammation as a predictor of colectomy in patients with chronic ulcerative colitis.

Dis Colon Rectum 2009 Feb;52(2):193-7

Department of Surgery, Mount Sinai School of Medicine, New York, New York, USA.

Purpose: We evaluated a large cohort of patients with longstanding ulcerative colitis in a colonoscopic surveillance program to determine predictors of colectomy.

Methods: We queried a retrospective database of patients who had symptoms of ulcerative colitis for seven years or more. Histologic inflammation in biopsies was graded on a validated four-point scale: absent, mild, moderate, severe. We performed a multivariate analysis of the inflammation scores and other variables to determine predictive factors for colectomy. Patients who underwent colectomy for neoplasia were censored at the time of surgery; those who did not undergo colectomy were censored at the time of last contact.

Results: A total of 561 patients were evaluated, with a median follow-up of 21.4 years since disease onset. A total of 97 patients (17.3 percent) underwent surgery; 25 (4.5 percent) for reasons other than dysplasia. These 25 constitute events for this analysis. For univariate analysis, mean inflammation (P < 0.001) and steroid use (P = 0.01) were predictors of colectomy. For multivariable proportional hazards analysis, mean inflammation (P < 0.001) and steroid use (P = 0.03) were predictors of colectomy, whereas salicylate use (P = 0.007) was protective.

Conclusions: Higher median inflammation scores and corticosteroid use were predictors of colectomy in this patient population. The overall rate of colectomy during a long period of follow-up was low (<1 percent per year).
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http://dx.doi.org/10.1007/DCR.0b013e31819ad456DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2753491PMC
February 2009

Collagen fistula plug for the treatment of anal fistulas.

Dis Colon Rectum 2008 Jun 11;51(6):838-43. Epub 2008 Mar 11.

Mount Sinai Medical Center, 5 E. 98th St., Box 1273, New York, NY 10029, USA.

Purpose: This study was designed to evaluate the efficacy of the Surgisis (Anal Fistula Plug) in multiple patients at our institution and present early clinical results along with notable clinical observations from our experience.

Methods: This was a prospective analysis of all patients who received the Anal Fistula Plug for treatment of anorectal fistulas between April 2006 and February 2007. All tracts were irrigated with peroxide, the plug was inserted in the tract, and buried at the internal opening with 2-0 vicryl and mucosal advancement flap. Statistical analysis was performed with Fisher's exact test.

Results: Forty-five patients were treated with the Anal Fistula Plug and one patient was lost to follow-up. There were 27 males and 17 females with average age of 44.1 years treated for simple (n = 24) or complex (n = 20) fistulas. Preliminary results indicated an 84 percent healing rate by 3 to 8 weeks postoperatively, which progressively declined from 72.7 percent at 8 weeks to 62.4 percent at 12 weeks and 54.6 percent at a median follow-up of 6.5 (range, 3-13) months. Long-term Anal Fistula Plug closure rate was significantly higher in patients with simple than complex fistulas (70.8 vs. 35 percent; P < 0.02) and with non-Crohn's disease vs. Crohn's disease (66.7 vs. 26.6 percent; P < 0.02). Patients with two successive plug placements had significantly lower closure rates than patients who underwent placement of the plug once (12.5 vs. 63.9 percent; P < 0.02). No significant difference in closure rates were found between patients with one vs. multiple fistula tracts. Postoperative complications included perianal abscess in five patients (3 Crohn's disease, 2 non-Crohn's disease).

Conclusions: Anal Fistula Plug is most successful in the treatment of simple anorectal fistulas but is associated with a high failure rate in complex fistula and particularly in patients with Crohn's disease. Repeat plug placement is associated with increased failure. Given the relatively low morbidity associated with the procedure, Anal Fistula Plug should be considered as a first-line treatment for patients with simple fistulas and as an alternative in selected patients with complex fistulas.
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http://dx.doi.org/10.1007/s10350-007-9191-2DOI Listing
June 2008

Cavernous, arteriovenous, and mixed hemangioma-lymphangioma of the rectosigmoid: rare causes of rectal bleeding--case series and review of the literature.

Int J Colorectal Dis 2008 Jul 11;23(7):653-8. Epub 2008 Mar 11.

Division of Colon and Rectal Surgery, Department of Surgery, Mount Sinai Medical Center, One Gustave L. Levy Place, Box 1259, New York, NY 10029, USA.

Introduction: Cavernous hemangiomas of the sigmoid colon and rectum are uncommon vascular malformations usually found in young adults with a long history of episodic and painless rectal bleeding. Alternatively, they may present with massive life-threatening hemorrhage.

Discussion: We report three cases of hemangioma of the rectosigmoid including one case of cavernous hemangioma, one case of arteriovenous hemangioma, and one case of hemangiolymphangiomatosis with emphasis on clinical presentation, radiologic, operative, and pathologic findings. Definitive treatment consists of complete resection with a sphincter-preserving procedure or abdominoperineal resection, based on extent of disease.

Conclusion: Therapy is typically delayed by several years in these patients due to erroneous diagnosis and failed treatment of hemorrhoids and inflammatory bowel disease. Relative to hemangiomas, lymphangiomas of the rectosigmoid are even more rare and when symptomatic, present with rectal bleeding and pelvic pain.
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http://dx.doi.org/10.1007/s00384-008-0466-4DOI Listing
July 2008

Acute ischemic proctitis: report of four cases.

Dis Colon Rectum 2007 Jul;50(7):1082-6

Division of Colon and Rectal Surgery, Department of Surgery, Mount Sinai Medical Center and Mount Sinai School of Medicine, New York, New York 10029, USA.

Acute ischemic proctitis is an extremely rare clinical entity. It is mainly described in patients with significant atherosclerotic and cardiac risk factors who present with lower gastrointestinal symptoms in the setting of hemodynamic instability. Previous reports of ischemic proctitis suggest that rectal resection is not necessary in the treatment of this disease. We present four cases of acute ischemic proctitis that required complete proctectomy. All patients had large vessel atherosclerosis with rectal bleeding and sepsis as the presenting signs and symptoms. Three of four patients underwent complete proctectomy as the initial procedure. The fourth patient underwent complete proctectomy five days after the initial intervention. Two of four patients survived and were ultimately discharged from the hospital. A third patient recovered from surgery but ultimately died of respiratory complications. Only the patient who was initially treated by subtotal proctectomy died as the result of the disease. Although ischemic necrosis of the rectum is rare, complete proctectomy may be necessary to save the patient's life.
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http://dx.doi.org/10.1007/s10350-006-0812-yDOI Listing
July 2007

Granulomatous enterocolitis associated with Hermansky-Pudlak syndrome.

Am J Gastroenterol 2006 Sep 18;101(9):2090-5. Epub 2006 Jul 18.

Department of Surgery, Mount Sinai Medical Center, New York, New York 10029, USA.

Background: Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder. It consists of a triad of tyrosinase-positive oculocutaneous albinism (Ty-pos OCA), bleeding diathesis resulting from platelet dysfunction, and systemic complications associated with accumulation of ceroid lipofuscin. Many patients are from a small area in northwestern Puerto Rico. HPS has been associated with granulomatous enterocolitis in up to 20% of affected patients. It is not known whether this granulomatous colitis is a part of the syndrome, or represents an independent but associated process, such as Crohn's disease. This colitis can be severe, and has been reported to be poorly responsive to medical therapies including sulfasalazine, mesalamine, steroids, and metronidazole.

Case Report: We report a series of four patients with refractory enterocolitis in the setting of HPS who were treated at Mount Sinai Hospital between 1998 and 2005. A trial of infliximab was attempted in all four, and produced a complete response in two.

Conclusions: Many phenotypic and pathologic similarities exist between granulomatous enterocolitis in HPS and Crohn's disease. However, it is unclear whether the granulomatous enterocolitis in HPS is because of ceroid deposition or reflects the coexistence of Crohn's disease and HPS. The occurrence of ileal involvement and perianal fistulization in our cases suggests that in at least some instances, HPS and Crohn's disease are truly associated.
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http://dx.doi.org/10.1111/j.1572-0241.2006.00733.xDOI Listing
September 2006

Carcinoid tumor and Crohn's ileitis.

Isr Med Assoc J 2005 Oct;7(10):674

Department of Surgery, Mount Sinai Hospital and Mount Sinai School of Medicine, New York, NY, USA.

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October 2005

Restorative proctocolectomy and ileal pouch-anal anastomosis for ulcerative colitis after liver transplant for primary sclerosing cholangitis: case report and review of literature.

Am Surg 2005 Apr;71(4):362-5

Division of Colon and Rectal Surgery, Department of Surgery, Mount Sinai School of Medicine, Mount Sinai Medical Center, New York, New York 10029, USA.

Primary sclerosing cholangitis (PSC) is present in 5 per cent of patients with ulcerative colitis (UC). Conversely, as many as 90 per cent of patients with PSC have been found to have UC. The accepted treatment for advanced PSC is orthotopic liver transplant, and the treatment of ulcerative colitis with concomitant PSC is restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA). A small number of studies have shown that there is an increased risk of pouchitis in UC patients with PSC after ileal pouch-anal anastomosis. We report a case of a 45-year-old male who underwent a two-stage restorative proctocolectomy with IPAA after previous orthotopic liver transplant for PSC. We have reviewed the available literature concerning restorative proctocolectomy after liver transplantation, giving special attention to postoperative complications and subsequent development of pouchitis. It is important to be aware of the possibility of increased risk for development of pouchitis and to follow these patients closely to prevent complications.
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April 2005

Reduction of a large incarcerated rectal prolapse by use of an elastic compression wrap.

Dis Colon Rectum 2005 Jun;48(6):1320-2

Department of Surgery, Mount Sinai School of Medicine, New York, New York, USA.

Reduction of a large rectal prolapse may be difficult because of significant edema that collects in the rectal tissues. If reduction is unsuccessful, an emergent laparotomy and internal reduction is required. A wide elastic wrap applied around the prolapsed rectum provides progressive compression, which reduces the amount of edema, allowing subsequent manual reduction. This novel technique is simple, safe, inexpensive, and can easily be performed in the emergency department setting. Manual reduction, by this or other described methods, should be attempted before emergent laparotomy for incarcerated rectal prolapse is performed.
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http://dx.doi.org/10.1007/s10350-004-0913-4DOI Listing
June 2005

Familial polyposis coli: clinical manifestations, evaluation, management and treatment.

Mt Sinai J Med 2004 Nov;71(6):384-91

Department of Surgery, Mount Sinai School of Medicine, One East 100th Street, New York, NY 10029, USA.

Familial adenomatous polyposis (FAP) is an autosomal dominant, hereditary colon cancer syndrome that is characterized by the presence of innumerable adenomatous polyps in the colon and rectum. Gardner's syndrome is a variant of FAP, which in addition to the colonic polyps, also presents extracolonic manifestations, including desmoid tumors, osteomas, epidermoid cysts, various soft tissue tumors, and a predisposition to thyroid and periampullary cancers. Mutations of the APC gene are thought to be responsible for the development of FAP, and the location of the mutation on the gene is thought to influence the nature of the extracolonic manifestations that a given patient might develop. Though patients are often asymptomatic, bleeding, diarrhea, abdominal pain and mucous discharge frequently occur. Diagnostic tools include genetic testing, endoscopy, and monitoring for extra-intestinal manifestations. Currently, surgery is the only effective means of preventing progression to colorectal carcinoma. Restorative proctocolectomy with ileal pouch anal anastomosis (RPC/IPAA) with mucosectomy is the preferred surgical procedure, since it attempts to eliminate all colorectal mucosa without the need for an ostomy. Periampullary carcinoma and intra-abdominal desmoid tumors are a significant cause of morbidity and mortality in these patients after colectomy. Frequent endoscopy is needed to prevent the former, while there is no definitive treatment available yet for the latter. The following article presents a case and reviews the evaluation, management and treatment of Gardner's syndrome.
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November 2004
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