FRONTIERS IN ENDOSCOPY, SERIES #60

Clinical Update on the Role of EUS in Pelvic Abscess

March 2020 • Volume XLIV, Issue 3
Gandhi Lanke,Douglas G. Adler

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The role of Endoscopic ultrasound (EUS) as a tool for drainage of pelvic abscess is emerging. With the advent of stenting and drainage catheters, EUS-guided drainage of pelvic abscess has become more feasible. The understanding of pelvic anatomy is key in the technical success of EUS-guided pelvic abscess drainage. Surgical drainage of pelvic abscess should be the last option when computer tomography (CT), ultrasound (US) or EUS fail to achieve the drainage of abscess. This review article focuses on the role of EUS in pelvic abscess drainage.

INTRODUCTION

EUS-guided drainage of pelvic abscess is evolving as an alternative to percutaneous or surgical methods of drainage. Pelvic abscess can occur as a result of diverticulitis, ischemic colitis, inflammatory bowel disease, perforated appendicitis, pelvic inflammatory disease, or secondary to post-surgical complications from low anterior rectal resection or total abdominal hysterectomy (among other surgeries).1-4 Pelvic abscess drainage poses challenge as they are surrounded by the bony pelvis, neurovascular structures, as well as the bladder, bowel, uterus, vagina, prostate and rectum. EUS guided drainage can be an alternative option when percutaneous methods of drainage are not amenable because of lack of adequate and safe window for access to the abscess.

Etiology and Pathogenesis

Pelvic abscesses are usually polymicrobial in etiology and contain a mixture of aerobic, anerobic and facultative microorganisms. The most common microorganisms isolated from pelvic abscess include aerobic gram-positive cocci (group B streptococci, enterococci, and staphylococcal species), aerobic gram-negative bacilli (Escherechia coli, pseudomonas, klebsiella pneumonia and proteus species), anaerobic grampositive cocci (peptococci and peptostreptococci species), anaerobic gram-negative bacilli (Bacteroides and Prevotella species) and grampositive facultative anaerobe (actinomyces species).5-7

The development of postoperative pelvic abscess is dependent on complex interaction between host defense mechanisms and the bacterial inoculum. In females, the ascending route of normal bacterial flora from the vagina and cervix plays an important role in the development of pelvic abscess.5 Microbial contamination of the surgical field also plays a key role in the post-operative development of pelvic abscess.6

Clinical Presentation and Diagnosis

The common symptoms include lower abdominal pain, with or without fever and chills, nausea and vomiting.6 Patients can also present with septic shock, altered mental status along with hypotension, tachycardia and tachypnea.6 On physical exam, the abdomen/pelvis is often tender and rigidity or guarding may be present when there is peritonitis.8 A fluctuant mass may be palpable.8 Laboratory findings include leukocytosis with left shift, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP).9 Routine blood cultures should be obtained before the administration of antibiotics. All patients should undergo a dedicated computed tomography (CT) or magnetic resonance imaging (MRI) of pelvis to determine the size, extent, proximity to Gastrointestinal (GI) lumen and to assess if it is uniloculated or multiloculated abscess before the endoscopic ultrasound (EUS).8,10 Transvaginal ultrasound may be useful in female patients with suspected tubo-ovarian abscess and history of pelvic inflammatory disease (PID).11

Management

Most authors agree that pelvic collections greater than 3 cm with suspected infection should be drained.8 Antibiotics in combination with drainage of pelvic abscess have led to decreased rates of surgical intervention, especially in patients with large pelvic abscess greater than 5cm.12 Pelvic abscess can be drained by various techniques like ultrasound (US), CT-guided percutaneous, surgical and EUS-guided drainage. The choice of drainage technique depends on the size, type of collection, location of the abscess, services available, and experience of the operator.8 US-guided technique is easy to perform, inexpensive and no radiation. US-guided drainage of the pelvic abscess is possible only when the abscess is within the reach of ultrasound probe.13 However, sometimes with US it becomes difficult to visualize deep collections in pelvis due to body habitus and the air acoustic barrier created by interposed small or large bowel.8 CTguided percutaneous drainage has better spatial resolution, better access to pelvic collections and better detection of adjacent structures.8 However, CT-guided drainage of pelvic abscesses can be challenging in deep pelvic collections because of surrounding anatomy and vascular structures.13 Also, CT-guided pelvic drainage can be more painful with transvaginal or trans gluteal route as the drainage catheters will be left in place.13 Unlike EUS-guided pelvic abscess drainage, there is no scope for transmural stent placement with CT-guided drainage, necessitating the placement of uncomfortable and potentially painful drainage catheter for long duration.13,14 EUS has several advantages when compared to other techniques which include drainage of pelvic abscess under real time sonographic guidance, intervening blood vessels and nerves can be avoided, provides access to abscess cavities which do not cause luminal compression and aids in establishing an alternative diagnosis in a small subset of patients.15

Procedure

Proper selection of the patient is very important as there is a high chance of therapeutic failure and risk of perforation whenever there is multiloculated pelvic abscess with size less than 4cm, located at the level of dentate line, immature wall without a rim, rectocele, J pouch, perianal abscess and location of the abscess 2 cm away from the EUS transducer.1,15 Prophylactic antibiotics should be administered if the patient is not already on antibiotics. To avoid the risk of bleeding during the procedure, laboratory abnormalities including coagulopathy and thrombocytopenia should be checked and corrected if abnormal before the procedure. The bladder should be empty, or foley catheter should be inserted before the procedure as there is a risk of bladder puncture and often missing the small pelvic fluid collections with distended bladder.16 Bowel preparation prior to the procedure can minimize contamination of the abscess with stool and aids in better visualization of the abscess.16 The procedure can be performed under conscious sedation or general anesthesia.1,13

Technique

A curved linear echoendoscope is used to locate the abscess and color doppler helps to identify and thus avoid any intervening vasculature. The linear echoendoscope has the advantage of visualization of the needle while performing the procedure. However, with the radial echoendoscope (which may be used to identify the target abscess), therapeutic intervention is not possible.17 The linear echoendoscopes available in USA include the FG 38UX (Pentax Precision Instruments Corp., Orangeburg, NY, USA), the EG 38UT (Pentax Precision Instruments Corp., Orangeburg, NY, USA), and the GF-UCT140/180 (Olympus Medical System Corporation, Center Valley, PA, USA). Both the EG 38UT and the GF-UCT 140 allow placement of a 10 F stent and they have working channels of 3.8 and 3.7 mm, respectively.15 With the FG 38X, it only permits placement of an 8.5 F stent and has a working channel of 3.2 mm.15 Once the abscess is located, a 19-guage fine needle aspiration (FNA) needle (Wilson-Cook, Winston-Salem, NC, USA) can be used to puncture the abscess cavity.1The abscess is then aspirated and, if desired, fluid can be sent to microbiology for culture and sensitivity to optimize the antibiotic treatment.17 Some practitioners skip this step as the fluid is usually polymicrobial in nature. Normal saline with 10-20 milliliter (ml) can be used to irrigate the FNA needle, if needed,as it can be clogged from mucosa or debris and to evacuate as much pus as possible.15 A 0.035” guide wire is then passed in to the abscess cavity under EUS and/or fluoroscopic guidance. Once the guide wire is in place, the transmural tract is sequentially dilated over the guide wire using a 5 F endoscopic retrograde cholangiopancreatography (ERCP) canula and a 6-15 mm over the guide wire biliary balloon dilator can also be used to further dilate the tract.15,17 Alternatively, a needle knife can also be used to puncture the abscess cavity after the FNA needle is withdrawn, followed by leaving a Teflon catheter in the abscess cavity after the metal part of the needle knife is withdrawn from the abscess cavity.17 Giovannini introduced a one-step drainage of pelvic abscess after the needle is withdrawn using the NWOA system (Cook Endoscopy® WinstonSalem, NC, USA). NWOA system consists of a 0.035-inch needle-wire suitable for cutting current, a 5.5F dilator and an 8.5 or 10 F stent preassembled on the same catheter.17

After the tract is dilated, straight or double pigtail stents are deployed in the abscess cavity under fluoroscopic guidance, usually 2 or more stents if plastic stents are used.15 After the deployment of first stent, an ERCP cannula preloaded with the guide wire is passed adjacent to the first stent into the abscess cavity under fluoroscopic guidance to deploy second stent. A 10 F transrectal catheter can be deployed adjacent to the stent especially when the pelvic abscess size is large to aid in better evacuation of the abscess.1 The transrectal catheter exits the anus and it can be secured to the gluteal region with the tape. The transrectal catheter can be irrigated with normal saline periodically until the aspirate is clear.1

Alternatively, fully covered self-expanding lumen-apposing metal stents (LAMS) can be used as tools for access and drainage of pelvic abscesses.18 LAMS (AXIOS and AXIOS- EC; Boston scientific, Marlborough, MA) are available in 3 sizes (lumen diameter x length; 10×10 mm, 10×15 mm, and 10×20 mm).19 The stent is available in electrocautery enhanced (hot) and nonelectrocautery enhanced (cold) versions. Under EUS guidance, the constrained stent is delivered in to the abscess cavity and the distal flange is deployed.20 The proximal flange is deployed under endoscopic ultrasound, or fluoroscopic guidance. Using a through-the scope balloon dilator, the stent lumen can be immediately dilated to the LAMS diameter if so desired.18 Dilation aids in immediate apposition of the tissues, maximizes initial drainage, and if required allows direct access for debridement.18 The enhanced-electrocautery LAMS can also be used to deliver the catheter in to the abscess cavity without prior dilation of the tract as it has the capability of application of electrocautery at the distal tip of the device introducer by means of cautery ring.21 Advantages include easy deployment system, a saddle-shaped design that decreases migration risk and decreases the risk of stent clogging with fecal matter. Also, a larger inner stent diameter allows better drainage of pelvic abscess without the need for drainage catheter and if needed allows direct endoscopic debridement by passage of standard gastroscope through the stent lumen.18,22,23 A representative EUS-guided pelvic abscess drainage is illustrated in Figure 1.

DISCUSSION

Results of studies that have evaluated outcomes of EUS-guided pelvic abscess drainage are shown in Table 1. Giovannini et al. evaluated the efficacy of EUS-guided transrectal aspiration and drainage of deep pelvic abscesses by using plastic stents (8.5-Fr, 10-Fr).24 In this study, 12 patients with perirectal or pelvic abscess were evaluated using EUS. Three patients had aspiration only and two of these developed recurrences requiring surgery. Nine patients underwent transrectal stent placement and one patient with an abscess>8cm experienced incomplete drainage requiring surgery. No major complications occurred in this study. Poincloux et al. evaluated the long-term outcome of 37 patients who underwent EUS-guided drainage of perirectal and perisigmoid abscesses via plastic stents or LAMS.13 Four patients underwent needle aspiration, plastic stent placement in 29 and LAMS placement in 4 patients. Technical success (ability to drain the pelvic abscess under EUS guidance) was 100%, clinical success (complete resolution of the abscess with symptom relief on follow up CT in 4 weeks) was 91.9% and longterm success (abscess resolution without the need for surgery and recurrence after 12 months) was 86.5% respectively. Five patients required reintervention via EUS within 14 days, 1 patient required surgery and 1 patient received supportive care because of persistent abscess. Stents were removed after complete resolution of the abscess. LAMS were removed within 6 weeks and plastic stents were removed 3-6 months after placement. Complications included perforation (1 patient) and stent migration (1 patient).

Puri et al. evaluated the efficacy of EUS-guided pelvic abscess drainage without fluoroscopy in 30 (4 prostatic, 7 perisigmoid and 19 perirectal abscesses) patients.25 Five patients underwent only aspiration (3 perisigmoid and 2 prostatic abscesses), aspiration with dilation in 6 (2 perirectal, 2 perisigmoid and 2 prostatic abscesses) and dilation with stent placement in 19 (17 perirectal and 2 perisigmoid abscesses) patients. Technical success (ability to drain the abscess under EUS guidance) was 100%, treatment success (symptom relief with complete resolution of the abscess on follow up EUS or CT in 1-2 weeks) was 93.3% with no recurrence (need for repeat EUS-guided drainage within 90 days after the stent removal) of abscess during 6-60 months follow up. Five (2 with perirectal abscess had migration of stent requiring repeat stent placement, 1 with prostatic abscess required re-aspiration and 2 underwent only aspiration with perisigmoid abscess required surgery) patients required re-intervention. Ramesh et al. evaluated the outcomes of EUSguided transrectal (TR-27) and trans colonic (TC11) drainage of abdomino-pelvic abscesses in 38 patients.26 Technical success (successful placement of stents or drainage catheters in the abscess cavity) was 100%, treatment success (resolution of abscess on follow up CT at 2 weeks with symptom improvement) was 70% (trans colonic) and 96.3% (transrectal) respectively. There were no procedural complications. Repeat EUS drainage was performed in 4 (TC cohort-1 diverticular

phlegmon, TR cohort- 3 diverticular phlegmon) patients because of worsening symptoms. Four patients (TC cohort-3, TR cohort-1) required surgery after failed EUS-guided drainage. Patients with diverticular abscess had poor outcomes (25 % vs 97%) compared to other (post-surgical, ischemic colitis, perforated appendix, endocarditis, trauma and IBD) causes. There was no recurrence of abscess in patients with treatment success after a median follow up of 3.36 years. Meylemans et al. evaluated the efficacy and safety of EUS-guided (23) and surgical transrectal drainage (23) of pelvic abscesses in 46 patients.27 The success rate (no need for additional treatment or intervention with surgical/EUS or radiological) of EUS-guided vs surgical transrectal drainage was 83% and 48% respectively. Anastomotic leak occurred in 3 cases of EUS-guided and 9 cases of surgical transrectal drainage of pelvic abscesses. The mean duration of drainage in EUS-guided vs surgical transrectal drainage was 42 and 13 days respectively. The total length of stay for EUSguided vs surgical transrectal drainage was 24 and 20 days respectively. The total duration of follow up for EUS-guided vs surgical transrectal drainage was 261 and 301 days respectively. Varadarajulu et al. evaluated the rate of recurrence, complications, technical and treatment success of EUS-guided pelvic abscess drainage in 25 patients.1 All patients with abscess size less than 8cm underwent placement of one or two 7F transrectal stents and for abscess size 8cm or more, a 10F drainage catheter is placed in addition to transrectal stents. The drainage catheter is removed if the abscess size decreased by more than 50% on follow up CT after 36 hours and the decision to discharge the patient from the hospital is made. All stents were retrieved by sigmoidoscopy after a follow up CT at 2 weeks showed complete resolution of abscess. Technical success (ability to drain the abscess under EUS guidance) and treatment success (symptom relief with complete resolution of the abscess on follow up CT at 2 weeks) was 100% and 96% respectively. One patient with perforated diverticulitis had persistent abscess requiring surgery. The mean duration of hospital stay was 3.2 days and after a mean follow up of 189 days there was no recurrence of abscess in all 24 patients.

Zator et al. evaluated the efficacy of single step EUS-guided lavage and instillation of gentamicin antibiotic for drainage of pelvic abscesses in 6 patients.28

All patients underwent EUS-guided aspiration, equal volume sterile saline lavage and gentamicin instillation (40mg/ml). Four patients had complete resolution of the abscess on follow up CT. One patient with diverticular abscess had recurrent diverticulitis without abscess requiring surgery, 1 patient with diverticular abscess had decreased size (7.3 to 3.4 cm) permitting planned surgery and 1 patient with crohn’s disease had decreased supra-levator abscess size (6.5 x 3.5 cm to 2.5 x 0.5 cm) required surgery for persistent sinus tracts with anal narrowing.

CONCLUSION

EUS-guided pelvic abscess drainage is safe and effective in experienced hands. Multidisciplinary care by different physicians including surgery, gynecology, radiology, oncology and interventional gastroenterology is key in the effective management of pelvic abscesses. Aspiration with stent placement and drainage catheter is more efficacious than aspiration with stent alone, especially in larger pelvic abscesses. Diverticular abscesses are more prone to recurrence and timely management can prevent complications. Use of LAMS should be individualized based on the indications. These devices have advantages including decreased migration risk, less risk of stent clogging with fecal matter and a larger inner stent diameter allows better drainage of pelvic abscess without the need for drainage catheter. Close follow up with repeat imaging after the procedure is essential to recognize early complications. Prompt recognition and early referral to interventional gastroenterology when indicated can prevent invasive surgery.

References
1. Varadarajulu S, Drelichman ER. Effectiveness of EUS in drainage of pelvic abscesses in 25 consecutive patients (with video). Gastrointestinal endoscopy. 2009;70(6):11211127.
2. Bang JY, Varadarajulu S. Endoscopic ultrasound-guided transrectal drainage of a pelvic abscess following total abdominal hysterectomy. Eur J Obstet Gynecol Reprod Biol. 2012;164(1):113-114.
3. Puri R, Eloubeidi MA, Sud R, Kumar M, Jain P. Endoscopic ultrasound-guided drainage of pelvic abscess without fluoroscopy guidance. Journal of gastroenterology and hepatology. 2010;25(8):1416-1419.
4. Granberg S, Gjelland K, Ekerhovd E. The management of pelvic abscess. Best Pract Res Clin Obstet Gynaecol. 2009;23(5):667-678.
5. Soper DE. Bacterial vaginosis and postoperative infections. Am J Obstet Gynecol. 1993;169(2 Pt 2):467-469.
6. Jaiyeoba O. Postoperative infections in obstetrics and gynecology. Clin Obstet Gynecol. 2012;55(4):904-913.
7. Hadithi M, Bruno MJ. Endoscopic ultrasound-guided drainage of pelvic abscess: A case series of 8 patients. World J Gastrointest Endosc. 2014;6(8):373-378.
8. Robert B, Yzet T, Regimbeau JM. Radiologic drainage of post-operative collections and abscesses. J Visc Surg. 2013;150(3 Suppl):S11-18.
9. Theisen J, Bartels H, Weiss W, Berger H, Stein HJ, Siewert JR. Current concepts of percutaneous abscess drainage in postoperative retention. Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract. 2005;9(2):280-283.
10. Nguyen TL, Soyer P, Barbe C, et al. Diagnostic value of diffusion-weighted magnetic resonance imaging in pelvic abscesses. J Comput Assist Tomogr. 2013;37(6):971-979.
11. Chappell CA, Wiesenfeld HC. Pathogenesis, diagnosis, and management of severe pelvic inflammatory disease and tuboovarian abscess. Clin Obstet Gynecol. 2012;55(4):893903.
12. To J, Aldape D, Frost A, Goldberg GL, Levie M, Chudnoff S. Image-guided drainage versus antibiotic-only treatment of pelvic abscesses: short-term and long-term outcomes. Fertil Steril. 2014;102(4):1155-1159.
13. Poincloux L, Caillol F, Allimant C, et al. Long-term outcome of endoscopic ultrasound-guided pelvic abscess drainage: a two-center series. Endoscopy. 2017;49(5):484490.
14. Harisinghani MG, Gervais DA, Maher MM, et al. Transgluteal approach for percutaneous drainage of deep pelvic abscesses: 154 cases. Radiology. 2003;228(3):701705.
15. Prasad GA, Varadarajulu S. Endoscopic ultrasoundguided abscess drainage. Gastrointest Endosc Clin N Am. 2012;22(2):281-290, ix.
16. Holt B, Varadarajulu S. Endoscopic ultrasound-guided pelvic abscess drainage (with video). Journal of hepatobiliary-pancreatic sciences. 2015;22(1):12-15.
17. Fernandez-Urien I, Vila JJ, Jimenez FJ. Endoscopic ultrasound-guided drainage of pelvic collections and abscesses. World J Gastrointest Endosc. 2010;2(6):223-227.
18. Mudireddy PR, Sethi A, Siddiqui AA, et al. EUS-guided drainage of postsurgical fluid collections using lumenapposing metal stents: a multicenter study. Gastrointestinal endoscopy. 2018;87(5):1256-1262.
19. Shah RJ, Shah JN, Waxman I, et al. Safety and efficacy of endoscopic ultrasound-guided drainage of pancreatic fluid collections with lumen-apposing covered self-expanding metal stents. Clinical gastroenterology and hepatology: the official clinical practice journal of the American Gastroenterological Association. 2015;13(4):747-752.
20. Fernandez-Urien I, Elosua A, Bernad B, Carrascosa J, Macias E. EUS-guided drainage of a pelvic abscess. VideoGIE. 2019;4(6):274-275.
21. Rinninella E, Kunda R, Dollhopf M, et al. EUS-guided drainage of pancreatic fluid collections using a novel lumen-apposing metal stent on an electrocautery-enhanced delivery system: a large retrospective study (with video). Gastrointestinal endoscopy. 2015;82(6):1039-1046.
22. Siddiqui AA, Kowalski TE, Loren DE, et al. Fully covered self-expanding metal stents versus lumen-apposing fully covered self-expanding metal stent versus plastic stents for endoscopic drainage of pancreatic walled-off necrosis: clinical outcomes and success. Gastrointestinal endoscopy. 2017;85(4):758-765.
23. Sharaiha RZ, Tyberg A, Khashab MA, et al. Endoscopic Therapy With Lumen-apposing Metal Stents Is Safe and Effective for Patients With Pancreatic Walled-off Necrosis. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association. 2016;14(12):1797-1803.
24. Giovannini M, Bories E, Moutardier V, et al. Drainage of deep pelvic abscesses using therapeutic echo endoscopy. Endoscopy. 2003;35(6):511-514.
25. Puri R, Choudhary NS, Kotecha H, et al. Endoscopic ultrasound-guided pelvic and prostatic abscess drainage: experience in 30 patients. Indian J Gastroenterol. 2014;33(5):410-413.
26. Ramesh J, Bang JY, Trevino J, Varadarajulu S. Comparison of outcomes between endoscopic ultrasound-guided transcolonic and transrectal drainage of abdominopelvic abscesses. Journal of gastroenterology and hepatology. 2013;28(4):620625.
27. Meylemans DVG, Oostenbrug LE, Bakker CM, Sosef MN, Stoot J, Belgers HJ. Endoscopic ultrasound guided versus surgical transrectal drainage of pelvic abscesses. Acta Chir Belg. 2018;118(3):181-187.
28. Zator Z, Klinge M, Schraut W, Tsung A, Khalid A. One step endoscopic ultrasound guided management of pelvic abscesses: a case series. Therap Adv Gastroenterol. 2018;11:1756284818785574.
29. Mukai S, Itoi T, Tsuchiya T, Tonozuka R, Sofuni A. EUS-guided pelvic abscess drainage with use of a biflanged metal stent through the perianal transgluteal route. Gastrointestinal endoscopy. 2016;84(6):1069-1070.
30. Trevino JM, Drelichman ER, Varadarajulu S. Modified technique for EUS-guided drainage of pelvic abscess (with video). Gastrointestinal endoscopy. 2008;68(6):1215-1219.
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A SPECIAL ARTICLE

Quality of Online Information About Irritable Bowel Syndrome

March 2020 • Volume XLIV, Issue 3
Louis Levine,Nabeel Akhtar,Lori-Ann Glasgow,Elizabeth Thompson,Sanam Razeghi,Ann Ouyang

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Currently there are no studies evaluating web-based resources providing patient information about irritable bowel syndrome (IBS), a common condition with complex pathophysiology.

Aims: to assess the reliance of patients on the internet for medical issues and the quality of commonly viewed websites about IBS.

Methods: 198 patients were surveyed about using the internet for medical information and the most popular websites identified by their web searches for “irritable bowel syndrome” were evaluated for validity, readability, and content.

Results: although a large portion of respondents rely on the internet for medical information, the majority of online resources for IBS are not easily readable. Four of 14 are written at an eighth grade reading level or below. Of these, only two have a Content Score of over 60 out of 93. This analysis supports clinicians in taking an active role to assist patients in finding online resources to improve their understanding.

Introduction

Irritable bowel syndrome (IBS) is a common functional gastrointestinal disorder with an overall prevalence of up to 20% in the United States. Most patients with IBS are managed by primary care providers. However, the syndrome remains a challenge to diagnose due to both the
heterogeneity of clinical presentations and a lack of definitive diagnostic testing. The complex pathophysiology of IBS and the limited time that health care providers have to spend with patients in a single clinic encounter make it difficult to fully educate all patients in the outpatient primary care setting. These factors may result in many patients not fully understanding the condition, making them more likely to turn to alternate sources, including the internet.

With increased awareness of IBS by the public, patients are also more commonly self-diagnosing IBS based on their own symptoms.2 In today’s technological age, more patients access and rely on the internet as a major resource when seeking medical information about themselves or others.3 Some studies also suggest that females use the internet more frequently than men for the purpose of obtaining health information.4-7 IBS is much more commonly diagnosed in women8, making it especially important for online information about IBS to be of high quality.

While recent studies have evaluated internetbased resources for other common medical conditions,9-12 there are no current studies that assess the quality of online information pertaining to IBS specifically. The purpose of our study was to identify the most commonly viewed online websites providing information about IBS and to assess their validity, readability, and relevance of content. We hypothesize that there are many online patient education resources on the topic of IBS and that these are of variable quality. With a thorough evaluation of current online information on IBS, providers taking care of patients with this syndrome can both be aware of and recommend the highest quality patient education materials to all patients using the internet as a major resource for improving their understanding of IBS.

METHODS

Patients visiting an outpatient gastroenterology clinic at an academic medical center were provided instructions to search “irritable bowel syndrome” on their smartphones or tablets and to voluntarily complete a survey to report their search results and answer a set of questions pertaining to demographics and internet use for medical information. Patients were asked to search using their own devices because online search engines customize search results based on an individual’s location and previous browsing history. All adult patients visiting the clinic were eligible for participation in the study. Specific exclusion criteria included age less than 18, lack of an Apple or Android device, or inability to properly complete the survey. The survey was cross-sectional in design and patients were recruited over a two-week period. Informed consent was obtained from each participant prior to beginning the survey. Study data were collected and managed using REDCap electronic data capture tools hosted at Penn State Health Milton S. Hershey Medical Center and Penn State College of Medicine.13 REDCap (Research Electronic Data Capture) is a secure, web-based application designed to support data capture for research studies, providing an intuitive interface for validated data entry, audit trails for tracking data manipulation and export procedures, automated export procedures for seamless data downloads to common statistical packages, and procedures for importing data from external sources. The study was approved by the researchers’ institutional review board.

Survey Instrument

The survey included five questions: age (organized by generation category; i.e. Millennial/Gen Y, Gen X, Baby Boomer, Mature), gender, highest level of education, and two questions about the use of and reliance on the internet for obtaining medical information. Finally, participants were asked to paste the link to their individual online search results for IBS. The complete survey instrument can be viewed in Figure 1. Results from the survey were tabulated and analyzed for trends.

Validity, Readability, and Content of Web-based Resources

Websites appearing on the first page of patient search results were noted. The 14 most frequently appearing websites from patient search results were individually scored by three researchers (L.L., N.A., L.G.) for validity, readability, and relevance of content. Results from each evaluator were averaged. Validity, as defined as the ability to trust an online resource’s information, was measured with the DISCERN (maximum score: 5) and Health on the Net (HON) tools. DISCERN is an online instrument supported by the National Health Services Executive Research and Development Programme that measures the reliability of medical information, including treatment options for conditions.14 It has three sections with 16 questions total. Though originally designed for print materials, DISCERN is viewed as an effective tool to measure the reliability of online medical information.15 The HON resource, a non-profit organization affiliated with the World Health Organization, is a measure of the quality, confidentiality, and transparency of websites.16 Online patient education websites can request certification by HON and, if specific criteria are met, this status can be indicated by an emblem displayed at the bottom of the website. A standardized tool was used to measure the readability of each online resource. The Flesch Reading Ease Score scale was originated by Rudolf Flesch and estimates the difficulty of reading passages in English.17 The Flesch-Kincaid Grade Level scale was adapted from Flesch’s original tool, initially used by the United States Navy.18 These scales measure readability based on word length, number of syllables, and sentence length. Flesch Reading Ease Scores typically span from 0 to 100, with lower scores indicating a greater degree of difficulty in reading by persons with a basic reading level. The Flesch-Kincaid Grade Level estimates the average school grade level matched to the Flesch Reading Ease Score. These tools are conveniently found in Microsoft Word; however, the calculable formulas for each of these scores is shown: Flesch Reading Ease Score = 206.835 – 1.015 x (words/sentences) – 84.6 x (syllables/words) Flesch-Kincaid Grade Level = 0.39 x (words/ sentences) + 11.8 x (syllables/words) – 15.59 Relevance of content was measured using a rubric developed by three of the researchers who specialize in the care of patients with functional gastrointestinal disorders (E.T., S.R., A.O.). The content scoring rubric was developed with reference to the Rome IV criteria.19 It contains a list of terms and phrases that are believed to be important for patients to know about IBS. The rubric includes pertinent IBS symptoms, disease mechanisms, differential diagnoses, components of the work-up, and management options. Items that were considered important received a score of 3, those that were felt to be useful but not critical information received a score of 2, and those that were factual but probably not important to patient understanding of the condition received a score of 1. The maximum score was 93. Each page’s patient education content about IBS was scored using this rubric. A sample of the Content Score rubric with point breakdown can be viewed in Figure 2

RESULTS

Trends in Internet Usage Among Survey Respondents 200 patients successfully completed the survey. Two were excluded due to age less than 18. Results of the survey, including demographic information, are displayed in Table 1 (N=198). 65% of the patients completing the survey were female. Nearly 40% of respondents did not have any educational degree beyond high school. When patients have questions regarding medical problems, 83% reported that they “always”, “often”, or “sometimes” use the internet, and 43% said that they use the internet as their main resource for obtaining medical information. A univariate logistic regression was performed (Table 2). This analysis reveals that there was a statistically significant (p <0.05) difference in the frequency of internet usage for medical information based on both age and gender. Compared to the 18-40 age group (Millennial/Gen Y), the age >70 group (Mature) relies on the internet for medical information significantly less frequently. Compared to males, females in the survey rely on the internet for medical information more frequently. Based on the regression results, there was no consistent statistically significant difference in frequency of internet use based on level of education.

Evaluation of Internet Resources

The 14 most commonly viewed websites on a search for IBS are shown in Table 3, along with their scores for validity, readability, and content for each website, sorted by the Content Score. Despite their presence on many patient search results, MedlinePlus and The Atlantic were excluded from evaluation because they were not formatted appropriately for analysis using the validated tools.

Results of Validity

7 of the 14 websites (50%) were certified by the Health on the Net (HON) foundation, as indicated by a visible emblem at the bottom of the page. Using the DISCERN rubric for validity, average scores for the websites ranged from 1.7 to 3.7 (out of 5). No website scored a 5 out of 5 using this rubric, primarily due to lack of detail about the exact sources of their information.

Results of Readability

No source evaluated was categorized as having “Easy” (>80) or “Fairly Easy” (70-79) readability according to the Flesch Reading Ease Score. Gastro. org, WebMD, the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), and Womenshealth.gov scored highest with >60 on the Flesch Reading Ease Score (“Standard”). All other sites were “Fairly difficult” (50-59), “Difficult” (30-49), or “Very confusing” (<29). Table 3 also shows the Flesch-Kincaid Grade Level for each website, which is the average educational grade level required to comprehend the information contained on the webpage.

Results of Content

Only 2 sources scored an average of >70 out of 93 for the content score (Wikipedia, Medicinenet). 6 sources scored between 60-69 (NIDDK, Emedicinehealth, Patient.info, MayoClinic, Medicalnewstoday, and GI.org). A comparison of Flesch Reading Ease Score and Content Score for the websites is shown in Figure 3, organized into groups based on the established Flesch Reading Ease Score categories. The Pearson’s correlation coefficient (r) between Flesch Reading Ease Score and Content Score was -0.5 (95% CI: -0.80 to 0.06), suggesting a moderate negative association between the two variables. Given the sample size of 14 websites, this result was not statistically significant at the p < 0.05 level (p = 0.0691). In general, the higher the Content Score of the resource, the more difficult it was to read (lower Flesch Reading Ease Score).

DISCUSSION

The results of this study reveal that almost 50% of patients frequently use the internet as their main educational resource for obtaining medical information, consistent with results from prior studies.20 The results support the published data that women rely on the internet as a resource for medical information more often than men.4-7 Further, trends from this study show that older generations (those aged >70) rely less on the internet as a resource for medical information than other age groups. This older age group reported that they use their health care provider most often when seeking medical information. Our study suggests that the internet is a more valuable tool for younger generations who grew up with access to the internet, highlighting the need to provide the highest quality online resources for this growing subset of patients. High quality medical information requires accuracy and depth of content, and the information should be understandable to the patient. When evaluating individual websites from an online search on IBS, the validity, readability, and relevance of content on the website could be measured. Most online IBS resources scored similarly on measurements of validity using the DISCERN scale. Half of the websites were certified by HON. Of those websites not certified by HON, some may in fact meet HON criteria due to their association with major medical associations (e.g., the National Institute of Diabetes and Digestive and Kidney Diseases, the American Gastroenterological Association, and the American Society of Colon and Rectal Surgeons). As shown in Table 3, the highest scoring resources for readability include Gastro. org, WebMD, NIDDK, and Womenshealth. gov. The highest scoring resources for relevance of content include Wikipedia, Medicinenet, NIDDK, and Emedicinehealth. Figure 3 also shows a moderate negative association between Flesch Reading Ease Score and Content Score, indicating that, in general, as websites add more pertinent content about IBS to their education materials, the readability for the average patient declines. Though the correlation was not statistically significant at the level of p < 0.05 because of the sample size of 14 websites, investigating this relationship further with additional websites about IBS may be an avenue for additional research. Creators of online patient education materials should be cognizant of this balance and design websites that are easily readable by the public but still contain the most important content.

This evaluation of popular websites reveals that most online resources providing information about IBS have poor readability, particularly Wikipedia. Given that only 4 out of 14 websites are written at the grade level recommended by the American Medical Association for patient education resources (6th-8th grade),21 there is likely poor patient comprehension of most online resources about IBS. For example, despite having the highest Content Score, Wikipedia would not be recommended as an educational source for patients due to the very advanced grade level (“16th grade”, i.e. a graduate-level degree) required to comprehend the information. Most websites did not indicate if content was directed towards health care professionals or patients. Recognizing that patients frequently use the internet for medical questions unanswered in a clinic visit, health care providers can take a proactive role in directing patients to online resources that are valid, readable, and relevant. Figure 3 serves as a tool that providers can use when managing patients with IBS, by referring patients to higher quality and appropriate online patient education material. Ideally, this would be a resource at the very topright of Figure 3 (high readability, high content). In practice, designing such a website would be difficult. However, because each patient has a different level of education and reliance on the internet for seeking medical information, providers can use Figure 3 to tailor their recommendations for online resources about IBS based on the patient’s unique needs and background. For example, a highly educated patient may appreciate a site that is slightly less readable with more detailed content. Further research is needed to determine if tailoring internet resources for patients has a beneficial impact on patients’ understanding of their disease. This study is the only thorough evaluation of online information about IBS, an exceedingly common diagnosis in the United States. The study confirms what other studies have discovered about trends in internet usage for medical information. There are limitations to the study, including the Hawthorne effect: most patients completed the brief survey in the presence of a researcher, which may have influenced the way they responded to the questions about internet usage. Additionally, the DISCERN score and Content Scores were determined by three of the study’s researchers. However, these tools were designed to be objective to limit bias. The data presented indicate that most current online information about IBS is of variable quality. Primary care physicians managing IBS patients should use these results to take an active role in directing patients to credible online resources that have key disease information written in an easily readable format. Doing so may be able to increase patient understanding of disease, improve the doctor-patient relationship and, ultimately, improve health outcomes for patients.

Acknowledgements

The research team would like to acknowledge Vernon M. Chinchilli and Sandeep Pradhan for their contribution to the statistical analysis of the data. The project described was supported by the National Center for Advancing Translational Sciences, National Institutes of Health, through Grant UL1 TR002014 and Grant UL1 TR00045. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.

References

1. Canavan C, West J, Card T. The epidemiology of irritable bowel syndrome. Clin Epidemiol. 2014;6:71.

2. Van den Houte K, Carbone F, Pannemans J, et al. Prevalence and impact of self-reported irritable bowel symptoms in the general population. United European Gastroenterol J. 2019;7.2:307-15.

3. Stevenson FA, Kerr C, Murray E, Nazareth I. Information from the Internet and the doctor-patient relationship: the patient perspective–a qualitative study. BMC Fam Pract. 2007;8.1:47.

4. Basch CH, MacLean SA, Romero RA, Ethan D. Health Information Seeking Behavior Among College Students. J Community Health. 2018;43.6:1094-9.

5. Bidmon S, Terlutter R. Gender differences in searching for health information on the internet and the virtual patient-physician relationship in Germany: exploratory results on how men and women differ and why. J Med Internet Res. 2015;17.6:e156.

6. Fox S. The social life of health information 2011. Washington, DC: Pew Internet & American Life Project; 2011.

7. Rice RE. Influences, usage, and outcomes of Internet health information searching: multivariate results from the Pew surveys. Int J Med Inform. 2006;75.1:8-28.

8. Lovell RM, Ford AC. Effect of gender on prevalence of irritable bowel syndrome in the community: systematic review and meta-analysis. Am J Gastroenterol. 2012;107.7:991.

9. van der Marel S, Duijvestein M, Hardwick J, et al. Quality of web-based information on inflammatory bowel diseases. Inflamm Bowel Dis. 2009;15.12:18916.

10. Zhang D, Schumacher C, Harris MB, Bono CM. The quality and readability of information available on the internet regarding lumbar fusion. Global Spine J. 2016;29.02:133-e8.

11. Saeed F, Anderson I. Evaluating the quality and readability of Internet information on meningiomas. World Neurosurg. 2017;97:312-6.

12. Lovett J, Gordon C, Patton S, Chen CX. Online Information on Dysmenorrhea: An Evaluation of Readability, Credibility, Quality, and Usability. J Clin Nurs. 2019.

13. Paul A. Harris, Robert Taylor, Robert Thielke, Jonathon Payne, Nathaniel Gonzalez, Jose G. Conde, “Research electronic data capture (REDCap) – A metadata-driven methodology and workflow process for providing translational research informatics support,” J Biomed Inform. 2009 Apr;42(2):377-81.

14. Charnock D, Shepperd S, Needham G, Gann R. DISCERN: an instrument for judging the quality of written consumer health information on treatment choices. J Epidemiol Community Health. 1999;53.2:105-11.

15. Charnock D, Shepperd S. Learning to DISCERN online: applying an appraisal tool to health websites in a workshop setting. Health Educ Res. 2004;19.4:440-46.

16. Health on the Net Foundation. [http://www.hon.ch]. Last accessed July 11, 2019.

17. Flesch R. How to write plain English: A book for lawyers and consumers. New York, NY: Harper & Row; 1979.

18. Kincaid JP, Fishburne Jr RP, Rogers RL, Chissom BS. Derivation of new readability formulas (automated readability index, fog count and flesch reading ease formula) for navy enlisted personnel. Institute for Simulation and Training 1975:56. [https://stars.library.ucf.edu/istlibrary/56]

19. Drossman DA, Hasler WL. Rome IV—functional GI disorders: disorders of gut-brain interaction. Gastroenterology. 2016;150.6:1257-61.

20. Diaz JA, Griffith RA, Ng JJ, Reinert SE, Friedmann PD, Moulton AW. Patients’ use of the Internet for medical information. J Gen Intern Med. 2002;17.3:180-5. 21. Weiss B. Health literacy. American Medical Association (2003).

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BOOK REVIEWS

Diagnosis and Management Guide for Anorectal Disease: A Clinical Reference

February 2020 • Volume XLIV, Issue 2
Justin C. Wheeler

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Editor: Waqar Qureshi, MD

Publisher: SLACK Incorporated Publication

Year: 2020

ISBN-13: 978-1-63091-492-9 (paperback)

List Price: $119.95

Diagnosis and Management Guide for Anorectal Disease: A Clinical Reference, edited by Dr. Waqar Qureshi, was written to help address comprehensive training deficiencies in management of anorectal disorders in gastroenterology fellowship programs. Dr. Qureshi is Professor in the section of Gastroenterology and Hepatology at Baylor College of Medicine in Houston, TX, where he established an anorectal clinic and served as Chief of Endoscopy. The book was written by 28 contributing authors, most of which are gastroenterologists or surgeons working in the United States. Management of a broad spectrum of nonsurgical anorectal disease is covered, as well as underlying disease processes and management strategies of surgical topics for non-surgeons. This book was written for gastroenterology fellows and practicing gastroenterologists. Pediatric-specific topics are not addressed.

The succinct and easy to navigate 213page book is divided into four sections, with 17 total chapters. The sections are:
1) Anatomy and Examination
2) Benign Anorectal Conditions
3) Benign Soft Tissue, Perianal, Perineal, and Complicated Conditions, and
4) Neoplasms. Chapter topics include anatomy and physiology of the pelvic floor, hemorrhoids, anal fissures, anal pruritus, pilonidal disease, anorectal IBD management, defecations disorders, and anal carcinoma, among others.

Each chapter begins with bulleted key-points, and are broken into easy-to-read subsections such as pathophysiology, symptoms, diagnosis, and evidence-based treatment strategies. Chapters end with a comprehensive list of references. The book is written concisely and includes numerous tables, diagrams, color pictures, and treatment algorithms. Clinical management strategies for both common and rare conditions are covered, including frequently used medications and procedures. A particularly welcome inclusion are ideas on how to address real-world challenges facing clinicians, ranging from costly medications to minimizing side-effects of interventions. As the first comprehensive volume covering both conservative treatment and surgical strategies for anorectal diseases, this book would serve well as a desk reference for any trainee or practicing gastroenterology clinician. Inclusion of pediatricspecific topics may broaden the applicability of this well-written book.

Justin C. Wheeler, MD, FAAP

Assistant Professor of Pediatrics,

Division of Pediatric Gastroenterology

University of Utah

Primary Children’s Hospital

Salt Lake City, Utah

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A CASE REPORT

Gastric Varices from Metastatic Ovarian Cancer with Splenic Involvement

February 2020 • Volume XLIV, Issue 2
Allyce Caines,Ghassan Allo,Yousuf Siddiqui

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Left-sided portal hypertension (LSPH), also known as splenoportal hypertension, is a rare but life-threatening cause of upper gastrointestinal bleeding. LSPH often occurs in non-cirrhotic patients as a consequence of splenic vein obstruction. We present a case of isolated gastric varices due to mass effect on the splenic vein and likely tumor thrombus due to metastatic ovarian cancer.

INTRODUCTION

Portal hypertension refers to increased pressures in the hepatic portal system. This in turn leads to complications such as variceal hemorrhage from gastric or esophageal varices, ascites and hepatic encephalopathy. Upper gastrointestinal bleeding secondary to portal hypertension in the form of variceal hemorrhage is a recognized lifethreatening cause of gastrointestinal bleeding. In patients with gastrointestinal bleeding due to portal hypertension, bleeding from gastric varices is the cause in 5%-10% of patients.1 Increased resistance to portal flow due to a stiff, cirrhotic liver is often the cause of portal hypertension. However, portal hypertension can also be caused by isolated obstruction of the splenic vein, which is often referred to left sided portal hypertension (LSPH). LSPH accounts for less than 5% of all patient with portal hypertension1. Most of the reported cases of splenic vein obstruction have been the result of malignancy involving the spleen. Pancreatic disorders, including pancreatic carcinoma, acute and chronic pancreatitis, cysts, and pseudocysts which may block the splenic vein via thrombus formation or mass effect, are the most common causes of LSPH. These patients often have no known prior liver disease and have no evidence of cirrhosis on presentation. Bleeding can be catastrophic due to high portal pressures. In these cases, it is important to consider causes of left sided portal hypertension and the available treatment options.

Case Report

A 59 year-old female with a past medical history of metastatic ovarian cancer and obstructive uropathy, secondary to extrinsic compression, presented to her primary gynecologic oncologist with complaints of fatigue, lightheadedness and melena for one week. On laboratory evaluation, she was found to have a hemoglobin of 5.7 g/dL prompting emergency evaluation. Four years prior she was diagnosed with stage IIIC serous carcinoma of the ovary, at which time she underwent total abdominal hysterectomy, omentectomy, pelvic lymphadenectomy, tumor debulking and subtotal colectomy with diverting ileostomy followed by chemotherapy. She remained disease free for approximately nine months when imaging showed disease recurrence. She developed significant disease progression involving the splenic hilum, retroperitoneal lymph nodes and pelvis in addition to bilateral hydronephrosis. On presentation to the emergency department she was hemodynamically stable with unremarkable physical exam. An episode of hematemesis consisting of 500cc of bright red blood occurred in the emergency department with repeat hemoglobin of 4.5 g/dL. Endoscopy revealed type 1 isolated gastric varices (IGV1). She received a total of 6 units of packed red blood cells. The patient was then transferred for consideration of balloon retrograde transvenous obliteration (BRTO). Repeat endoscopy confirmed the findings of type 1 isolated gastric varices with red wale signs (Figure 2). BRTO, splenectomy, gastric vessel ligation and cyanoacrylate injection were discussed as potential therapeutic options. The patient had no further episodes of bleeding and her hemoglobin remained stable. Magnetic resonance imaging (MRI) revealed a hypovascular mass in the splenic hilum with minimal central enhancement concerning for metastatic disease. Areas of hypovascular nodularity around the lesion extending into part of the splenic vein at the hilum and branches were suggestive of tumor thrombus. Liver lesions concerning for metastatic disease were also present. On MRI the hepatic vein, celiac artery, hepatic artery, portal vein and superior mesenteric vein were patent. The splenic artery appeared tortuous and the splenic vein was engorged. The patient was not a candidate for BRTO due to the lack of portosystemic collaterals. She underwent splenic artery embolization to decrease inflow to the spleen and splenectomy the following day. Pathology revealed a 4.4 x 3.7 x 3.1 cm ill-defined mass located within the splenic hilum (Figure 1A), which consisted of poorly differentiated adenocarcinoma (Figure 1B1C). Immunohistochemically, the adenocarcinoma was positive for cytokeratin AE1/AE3, CK7, PAX-8 and estrogen receptor (Figure 1D). The adenocarcinoma was negative for CK20. The morphology and immunohistochemistry were consistent with metastatic adenocarcinoma of müllerian origin, from the patient’s known ovarian adenocarcinoma.

Discussion

LSPH, also known as splenoportal or sinistral hypertension is a rare, but life-threatening cause of upper gastrointestinal bleeding.2 LSPH often occurs in non-cirrhotic patients as a consequence of splenic vein obstruction. Pancreatic disorders, including pancreatic carcinoma, acute and chronic pancreatitis, cysts, and pseudocysts which may block the splenic vein via thrombus formation or mass effect, are the most common causes of LSPH.3 To our knowledge there is only one other case of LSPH with bleeding gastric varices secondary to metastatic ovarian cancer published by Wallace et al in 2004.4 In our case, mass effect on the splenic vein as well as likely tumor thrombus caused splenic venous outflow obstruction. Obstruction of the splenic vein results in venous hypertension in collateral pathways that carry splenic arterial blood via the short gastric, coronary, and gastroepiploic veins to the superior mesenteric and portal veins. In the gastric wall veins of the fundus, blood flow and pressure increase, and submucosal structures consequently dilate, producing gastric varices.4,3 Risk factors for gastric variceal hemorrhage include the size of fundal varices (as there is a linear relationship between size of varices and risk of variceal hemorrhage), endoscopic presence of variceal red spots, and Child-Pugh class in patients with cirrhosis.5 Gastric varices are less frequent than esophageal varices and are present in 5%33% of patients with portal hypertension. The reported incidence of bleeding from gastric varices is approximately 25% in two years, with a higher bleeding incidence for fundal varices.6 Compared to esophageal varices, gastric varices are larger, more extensive, and lie deeper in the submucosa. As a result, standard endoscopic treatments for esophageal varices, including band ligation and sclerotherapy are largely ineffective for gastric varices.7 Management of gastric varices is dependent on the etiology, presence of collaterals and the available treatment modalities. LSPH, for example, has distinct therapeutic management options that are not appropriate for the management of generalized portal hypertension. Currently the gold standard for treatment of fundal (IGV1) varices as a result of splenic vein thrombosis is splenectomy. Surgical removal of the spleen decreases venous outflow through collateral circulations and decompresses IGV to prevent future bleeding.8 Additional therapies have been used to control gastric variceal bleeding and prevent re-bleeding from occurring. These include band ligation and endoscopic sclerotherapy (frequently by cyanoacrylate glue injection). The American Association for the Study of Liver Diseases (AASLD) Society guidelines recommend endoscopic variceal sclerotherapy in patients who bleed from gastric fundal varices otherwise when available, endoscopic variceal ligation is an option. AASLD also recommends that transjugular intrahepatic portosystemic shunt (TIPS) should be considered in patients in whom hemorrhage from fundal varices cannot be controlled or in whom bleeding recurs despite combined pharmacological and endoscopic therapy.5 Left-sided portal hypertension should be considered in the presence of gastrointestinal bleeding with normal liver function and unexplained splenomegaly.1 Isolated gastric varices type 1 should immediately raise the clinician’s suspicion for splenic vein obstruction. Although a rare cause, in patients with prior malignancy or without evidence of pancreatic pathology, malignancy should remain on the differential as a cause of splenic vein obstruction.

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INTRODUCTION: DISPATCHES FROM THE GUILD CONFERENCE

Dispatches from the GUILD Conference 2020

February 2020 • Volume XLIV, Issue 2
Uma Mahadevan

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Welcome to the Fourth annual Dispatches from GUILD series! The Gastrointestinal Updates-Inflammatory Bowel DiseaseLiver Disease (GUILD) Conference is an annual CME conference held in Maui, Hawaii every February (GUILD 2020: February 16-19). This meeting offers a cutting edge update in gastroenterology by world class speakers in a setting conducive to learning and interaction with peers. Our topics this year include 2 days of IBD updates, a day of hepatology and a day of obesity related topics. GUILD also recognizes the role played by nurse practitioners and physician assistants in the care of IBD patients and introduced an IBD boot camp in 2019. Scholarships are awarded to 10 NP/PA’s and 10 Gastroenterology fellows to attend the meeting and receive daily mentoring and networking.
To share our learning with the gastroenterology community at large, we are happy to continue our series beginning with the following article, “Prevention in Inflammatory Bowel Disease: An Updated Review”. We look forward to providing informative and educational articles covering IBD, Hepatology and special topics in GI in Practical Gastroenterology over the following months

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DISPATCHES FROM THE GUILD CONFERENCE, SERIES #27

Prevention in Inflammatory Bowel Disease: An Updated Review

February 2020 • Volume XLIV, Issue 2
Millie D. Long

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Patients with inflammatory bowel disease (IBD) are subject to complications from the disease itself and also from the immunosuppressive therapies used for treatment. To optimize the care of patients with IBD, providers need to consider primary, secondary and tertiary prevention. Primary prevention is employed to prevent a disease or complication from developing, such as immunizations. Secondary prevention detects a disease early to prevent disability, such as through screening programs. Tertiary prevention employs measures to reduce the impact of long-term disease and disability. This review highlights methods of prevention that can be utilized in patients with IBD via partnership between the primary care and gastroenterology provider.

INTRODUCTION

Inflammatory bowel disease (IBD), including both Crohn’s disease (CD) and ulcerative colitis (UC), causes inflammation and ulceration in the gastrointestinal tract. This inflammation results in considerable morbidity for patients, including symptoms such as diarrhea, abdominal pain, rectal bleeding, and weight loss. The prevalence of IBD is increasing in the United States (US) and worldwide. It is estimated that 2.2 million Americans will be living with IBD by 2025.1,2 With this growing population, there is an increasing need for effective and safe therapies for management of inflammation. Currently, there are a large number of classes of agents available for treatment of IBD, with varying relationships between safety and efficacy. Immunosuppressive agents used in the treatment of IBD include corticosteroids, biologic agents [anti-tumor necrosis factor alpha (anti-TNF), antiintegrin therapies, anti IL-12/23 inhibitors, janus kinase (JAK) inhibitors, and immunomodulators such as thiopurines or methotrexate]. As we target goals of mucosal healing in our IBD population using immunosuppressive agents, we must also use a patient-centered approach to prevent downstream complications, either of IBD itself, or of the therapies used for treatment of IBD. To do so, a partnership must exist between gastroenterology and primary care providers to target primary, secondary and tertiary prevention for our patients. This article will discuss current guidelines and recommendations for prevention specific to patients with IBD

Primary Prevention

Primary prevention is defined as prevention of disease development. Examples of primary preventive efforts include immunizations against infectious diseases or educational interventions on regular exercise. Primary preventive efforts can occur at an individual level or at a population level. One example of a population level primary preventive effort is fluoridation of the water supply to prevent dental caries. As compared to the agematched general population, patients with IBD are at increased risk for vaccine-preventable illnesses, such as influenza,5 pneumococcal pneumonia,6 and shingles.7 Inactivated vaccines are safe to administer in patients with IBD, regardless of immunosuppression status. Therefore, adherence to vaccination guidelines of inactivated vaccines can reduce these infectious complications. Annual influenza vaccination is indicated in all patients with IBD, pneumococcal vaccination series is indicated in all immunosuppressed patients with IBD and herpes zoster vaccination is indicated in all patients with IBD ≥ 50 years of age. Due to increased risk of shingles in younger IBD populations,7 consideration can be given for earlier vaccination while on certain high risk therapies. In particular, tofacitinib is associated with an increased risk of shingles, particularly at higher doses.8 However, more data are needed on safety, efficacy and durability in younger populations prior to a definitive recommendation. The human papillomavirus (HPV) vaccine has been shown to protect against specific serotypes of HPV linked to cervical dysplasia and genital warts. This vaccine has been studied specifically in IBD populations. The HPV vaccine has demonstrated both efficacy and safety.9 Another method of primary prevention in IBD patients is sunscreen use to prevent skin cancer. Patients with IBD have increased risks of both non-melanoma (NMSC) and melanoma skin cancer.10, 11 Evidence has demonstrated that thiopurines specifically increase the risk of NMSC and that the mechanism of action is associated with photosensitivity to ultraviolet-A light.12 Therefore, this is a potentially preventable complication of therapy through broad-spectrum sunscreen use. Finally, weight bearing exercise and calcium/ vitamin D supplementation, when appropriate, can prevent downstream osteoporotic fracture in these patients who may require recurrent courses of corticosteroids during their lifetimes with IBD. Table 1 shows a summary of primary preventive measures recommended in IBD patients.

Secondary Prevention

Secondary prevention is defined as detecting a disease early to prevent disability; such as through screening programs. Therefore, there are a number of important opportunities for secondary prevention in IBD patients. For example, patients with IBD are known to have an increased risk of melanoma, regardless of immunosuppressive therapy. Individuals on anti tumor necrosis factor alpha (anti-TNF) agents have nearly a two-fold further increased risk of developing melanoma.10 Therefore, a dermatology skin screening program is recommended in all patients with IBD.3 Prior studies have suggested an increased risk of abnormal Pap smear and/or cervical dysplasia in patients with IBD on immunosuppression.13 Therefore, annual cervical cancer screening is recommended in patients with IBD on immunosuppression.3 Patients with IBD who have longstanding colonic inflammation (> 10 years of duration) have an increased risk of developing colorectal dysplasia and cancer. Therefore, patients with longstanding colonic inflammation should undergo routine colonoscopy starting at 8-10 years of colonic disease duration, with subsequent colonoscopy intervals based on the results (often every 1-3 years). From a bone health perspective, all individuals (regardless of gender) with ≥ 3 months duration of corticosteroid use are at risk for osteopenia or osteoporosis. Therefore, screening with DEXA scan for these individuals and in women age ≥ 65 years is recommended. Subsequent DEXA screening can be determined based on the initial results. Finally, depression and anxiety are common in IBD patients.14 Earlier recognition and treatment of depression and anxiety can improve quality of life in patients with IBD. Screening for depression and anxiety is recommended in all patients with IBD.3 Table 2 describes currently recommended secondary preventive efforts in IBD patients.

Tertiary Prevention

Tertiary prevention refers to utilization of measures to reduce the impact of long-term disease and disability. In CD, ongoing inflammation can lead to development of strictures, which may cause obstruction and require bowel resection surgery. Additionally, inflammation can progress to fistulizing disease, including abnormal connections between the bowel and other organs. These fistulas can result in abscesses and other complications ultimately often requiring surgery. In UC, ongoing inflammation can increase the risk of colon cancer and dysplasia. Additionally, disease can extend from only left-sided involvement to pan-colonic involvement over time. Therefore, by intervening early and treating inflammation, with a goal of mucosal healing, we can potentially prevent these morbid and potentially life-threatening, complications of IBD. The paradigm in IBD management has shifted to one of a “treat to target” approach15. After initiation of medical therapy for the treatment of IBD, guidelines recommend subsequent reassessment to ensure that both symptoms and mucosal inflammation are improved.16,17 This dual method of reassessment is important, as symptoms do not always correlate with ongoing inflammation. This standard of reassessing a current therapy is also important in post-operative CD, where early evaluation with colonoscopy in the first 6-12 months after a resection, with alteration of medical therapies based on this, has been shown to improve long-term endoscopic outcomes.18 Therefore, by optimizing therapies to improve mucosal healing, we may be able to impact the long-term disability associated with irreversible bowel damage in IBD.

CONCLUSION

Management of patients with IBD can be difficult. IBD itself can be associated with a number of complications for patients, including ongoing chronic bowel symptoms and structural bowel damage, as well as extra-intestinal manifestations of IBD. These extra-intestinal manifestations
can include significant joint symptoms, skin manifestations, anemia, and kidney stones. While there are a number of effective therapeutic agents for the treatment of IBD and these extraintestinal complications, many of these therapies are themselves immunosuppressive. Therefore, the drugs themselves can result in therapy-related complications. These may include infectious, malignant, or idiopathic complications. As the patient may present to the primary care provider or the gastroenterologist for evaluation of symptoms or complications, it becomes very important for the entire care team to collaborate on diagnostic and management plans for individual patients with IBD. Importantly, prior studies have demonstrated that primary care physicians may not be comfortable addressing preventive care in IBD patients on immunosuppression.3 However, the gastroenterologist may assume that all preventive activities are occurring in primary care. The gastroenterologist may also not be comfortable addressing all of an IBD patient’s preventive health needs. In fact, many patients with IBD consider their gastroenterologist to be their primary care provider. Therefore, a collaboration between primary care and gastroenterology is needed to ensure appropriate adherence to preventive health recommendations in patients with IBD. Each IBD patient should have regular evaluation with both a primary care and gastroenterology provider. Through this partnership, an individualized plan for preventive medicine can be developed for each patient with IBD. This proactive approach of addressing primary, secondary and tertiary prevention in IBD patients can ultimately help to reduce infectious, malignant and long-term disease-related complications. There is an old African proverb stating, “It takes a village to raise a child.” This reflects the emphasis that African cultures place on family and community. In fact, this community of support is also needed for each patient with IBD. By sharing the burden of the complete care of the IBD patient, a care team can deliver evidencebased, patient-centered care. Figure 1 shows the integral components of a care team for a patient with IBD. Through collaboration, a care plan can be implemented for each IBD patient addressing his or her individual needs and goals. By focusing on implementing the three forms of prevention: primary, secondary, and tertiary, we can improve the lives of our patients with IBD.

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THE MICROBIOME AND DISEASE, SERIES #8

The Relationship Between Parkinson’s Disease and the Microbiome

February 2020 • Volume XLIV, Issue 2
Skylar Steinberg,Sabine Hazan,Jordan Daniels

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Parkinson’s Disease (PD) is a central nervous system movement disorder characterized by the formation of spherical protein deposits in the brain (Lewy bodies) and the development of spindle-like Lewy neurites in the body of the affected neuron. These start in the medulla oblongata and spread in a predictable pattern, resulting in a gradual drop in dopamine levels which causes tremors, rigidity and a progressive loss in mobility and body functions. In the later stages of the disease, behavioral and cognitive issues become prevalent.1

It has been known that in addition to prominent tremors and motor symptoms associated with Parkinson’s disease, up to 75% of patients display gastrointestinal abnormalities as well.2 These symptoms often precede the appearance of motor symptoms by many years, prompting speculation on the role of gut bacteria and the disease. While there are treatments for PD, no cure exists. Recent research examining the gut microbiota and its possible connection to PD offer potential new approaches to treatment. While this research is still in the early stages, it offers a glimmer of hope to Parkinson’s patients.

A study by Sampson et al. investigated the alterations of bacteria in the gut, dysbiosis, and motor deficits in Parkinson’s disease in mice. The team conducted three experiments to test this relationship, assessing bacterial microbiome and motor function in mice and how different microbiota affect symptoms. They first showed that gnotobiotic mice (mice lacking their natural gut microbiome) accumulated less alpha-synuclein in their brains, the primary protein component of Lewy bodies, and as such moved more freely. This provided a model for how the environment and gut flora may play a role in PD development as well as other neurodegenerative disorders. In the second experiment, Sampson’s team examined whether imbalances in short-chain fatty acids (SCFAs) created in gut could be associated with activated immune responses in the brain. They discovered that germ-free mice treated with microbially produced SCFAs had higher levels of neuroinflammation, which is linked to the malfunction of neurons through the activation of microglia. The third experiment treated mice with fecal transplants using donor stool from human patients with and without Parkinson’s disease. Mice that received stool from patients with Parkinson’s developed deficits in motor function.3 Future study by this team will focus on identification of specific organisms in the gut associated with the motor deficits. This could lead to alteration of the microbiome as a treatment for Parkinson’s disease.

However, researchers in Finland have discovered decreased abundance of the Prevotellaceae family of bacteria in the gut microbiome of patients with PD compared to healthy controls. Prevotellaceae is normal in the human gut in varying amounts, however it was discovered that the mean abundance of Prevotellaceae in the feces of PD patients was reduced by 77.6% compared to healthy individuals.4 Although PD patients display less Prevotellaceae, some controls had low levels as well, indicating that this cannot be the sole explanation of PD. This demonstration of how bacterial populations may influence disease has important implications for future research.

Another study by Tetz et al. demonstrated a significant correlation of gut bacteria with Parkinson’s disease. The human GI tract is home to bacteria, archaea, fungi, and viruses, including bacteriophages, the last of which are a type of virus that infects, replicates within, and destroys bacteria. This study showed that drug-naïve patients with PD had a 10-fold decrease in Lactococcus species (lactic acid bacteria) compared with healthy controls. It was noted that an increase in lytic bacteriophages was accompanied by a decrease of Lactococcus bacteria, indicating that a depletion of Lactococcus in patients with PD could be caused by lytic phages.4 A fourth way that gut bacteria could be implicated in Parkinson’s disease is via the enteric nervous system (ENS). A study by Liu et al. demonstrated that a truncal vagotomy, in which the trunk of the vagus nerve is removed where it enters the stomach from the esophagus, was related to a reduced risk for PD.5 Therefore, changes gut microbiota composition could cause alterations in the intestinal barrier function and permeability implicating both the immune system and the ENS, resulting in the development of PD symptoms. A study by Hill-Burns et al. sought to find microbial causes of Parkinson’s disease, partly by interrogating 39 potential confounders.6 Of these 39, the test results of eight indicated potential involvement. Once these confounders were taken into consideration, the microbiome sequencing of 197 patients with Parkinson’s disease and 130 controls were compared using three metrics. The team discovered several dysbiotic features of the PD microbiome, including elevated levels of Akkermansia, Lactobacillus, and Bifidobacterium and reduced levels of Lachnospiraceae. This study represents the largest to date of the microbiome in Parkinson’s patients.

It is important to recognize the pivotal impact of microbiome research, since it shows that pathology in the gut can impact neurological diseases. Microbiome research and its relationship with Parkinson’s disease is only in its infancy. Further research will hopefully identify new bacterial markers that contribute to the development of PD and guide new treatments. Although PD is likely a multicausal disease and the microbiome is not fully responsible, it is impossible to ignore the impact the gut microbiome could have on our future knowledge around Parkinson’s Disease.

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NUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #195

Mobility and Motility: Constipation Impairs Enteral Feeding in Disabled and Immobile Patients

February 2020 • Volume XLIV, Issue 2
Stephen M. Borowitz

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At least a third of children and adults with neurodevelopmental disabilities with or without limited mobility are significantly undernourished. The incidence and severity of malnutrition increases with the duration and severity of disability. Nutritional support in children and adults with a variety of neurodevelopmental disabilities can result in weight gain, increased muscle mass, improved peripheral circulation, better wound healing, fewer and less severe decubitus ulcers, less irritability and spasticity, and fewer hospitalizations, all of which result in an improved sense of well-being and an improved quality of life. Chronic constipation is a commonly unrecognized contributor to feeding intolerance among children and adults with neurodevelopmental disabilities and/or limited mobility. This article will provide the clinician with tools to recognize and treat what can be a very debilitating condition.

W.R. is a 24-year-old young man who was born extremely prematurely and as a result, has a number of chronic complications including quadriparetic spastic cerebral palsy, cortical blindness, profound global developmental delay with intellectual disability, a chronic seizure disorder, chronic respiratory difficulties that are likely the result of subclinical aspiration, and chronic feeding difficulties for which he had a gastrostomy placed during infancy. He also has a long history of chronic constipation. He has been hospitalized on at least three occasions this past year with acute respiratory illnesses that appear to be related to aspiration events and there are concerns that he is more prone to aspiration because of chronic, inadequately treated constipation. On examination he has the stigmata of severe quadriparetic cerebral palsy and appears quite malnourished with minimal subcutaneous tissue and muscle mass. His height is 152 cm and his weight is 45.8 kg (BMI = 14.49 kg/M2), which is well below the first percentile. It is certainly not surprising W.R. suffers from chronic constipation given his profound spastic cerebral palsy, chronic under nutrition, and lack of mobility. Nearly all children and adults with this constellation of symptoms will have difficulties with constipation. The constipation in this setting is multifactorial, in part due to a lack of mobility, and possibly due to the chronic ingestion of a liquid diet lacking fiber (although data on use of fiber is inconclusive). Spasticity may be a major contributor as well. When patients with spasticity strain to defecate, they often paradoxically contract their pelvic floor and external sphincter making the defecation process inefficient and ineffective. In addition to this, when these patients are ill or in pain, their spasticity often worsens making defecation even less efficient and effective. Nearly all patients like this require some sort of chronic laxative regimen. Laxatives and stool softeners alone are often insufficient to produce regular bowel movements in this population. Clinicians may need to resort to regular use of stimulant suppositories or even large volume enemas to produce regular bowel movements. In some patients with these problems, cecostomy placement to administer antegrade enemas daily can result in a major improvement in their quality of life. It is also quite possible and in fact quite probable that W.R.’s difficulties with constipation are contributing to his recurrent pulmonary difficulties. Chronic constipation can slow gastric emptying and exacerbate or even precipitate the symptoms of GE reflux. As such, in many effected individuals, regulation of their bowel habit improves their tolerance of tube feedings with less bloating, gagging, retching, vomiting and/or signs/ symptoms of GE reflux.

INTRODUCTION

Studies suggest at least 1/3 of children and adults with neurodevelopmental disabilities with or without limited mobility are significantly undernourished, and not surprisingly, the incidence and severity of malnutrition increases with the duration and severity of disability. Historically, this state of malnutrition was considered to be part of the diseases they are suffering from, however a number of studies have demonstrated that nutritional support in children and adults with a variety of neurodevelopmental disabilities can result in weight gain, increased muscle mass, improved peripheral circulation, better wound healing, fewer and less severe decubitus ulcers, less irritability and spasticity, and fewer hospitalizations, all of which are associated with an improved sense of well-being and an improved quality of life.1 As many as 90% of children and adults with significant disabilities experience gastrointestinal difficulties including, but not limited to, dysphagia, aspiration during swallowing, gastroesophageal reflux, poor gastric emptying, and chronic constipation, any or all of which may interfere with the ability to ingest adequate nutrition2 (Table 1). As oral or enteral intake diminishes and nutritional status deteriorates, gastrointestinal symptoms may worsen, further compromising the patient’s ability to ingest adequate calories resulting in a vicious and self-perpetuating downward spiral. Studies have demonstrated that malnutrition in and of itself can produce feeding intolerance. Nutritional restitution can improve gastric motility and lessen the severity of gastroesophageal reflux,3,4 in addition to improving gastric compliance and lessening early satiety.5 In some cases, the feeding intolerance associated with worsening malnutrition is a result of superior mesenteric artery syndrome in which the third portion of the duodenum is compressed due to narrowing between the superior mesenteric artery and the abdominal aorta6 (see Figure 1). In many cases, in undernourished or malnourished individuals, nutritional restoration can improve feeding tolerance. Nutritional repletion, either via a jejunal tube or parenterally, is the treatment of choice for superior mesenteric artery syndrome.

Constipation

Another less commonly recognized contributor to feeding intolerance among children and adults with neurodevelopmental disabilities and/ or limited mobility is chronic constipation. As many as two-thirds of children and adults with disabilities and/or limited mobility suffer from chronic constipation. The severity of constipation in this population is often underestimated and its significance on their quality of life is frequently unrecognized or discounted by health care professionals (Table 2). Abdominal cramping, bloating, and perianal pain due to fissures and/or perineal skin breakdown can be quite debilitating. Moreover, chronic constipation increases the risk of recurrent urinary infections, worsens vesicoureteral reflux, and diminishes enteral feeding tolerance by delaying gastric emptying and producing early satiety.9 Numerous studies have demonstrated that otherwise healthy children and adults with chronic constipation have delayed gastric emptying that improves with effective management of the constipation.10 In healthy adults, voluntary suppression of defecation significantly slows gastric emptying,11 and moreover, intermittent painless rectal distension significantly slows gastric emptying and small bowel motility.12 The mechanism of the effects of rectal distension on gastric emptying is unclear but likely reflects a combination of both humoral and neural effects.10,12 Chronic constipation can cause chronic or recurrent vomiting and exacerbate or even precipitate the symptoms of gastroesophageal reflux and once the constipation is adequately treated, the vomiting and symptoms of reflux may abate.

Many factors contribute to the high prevalence of chronic constipation in children and adults with neurodevelopmental disabilities and/or limited mobility. While it is commonly assumed that inadequate intake of dietary fiber and a lack of sufficient fluid intake are major contributors, there is remarkably little evidence this is the case.14 In contrast, there is good evidence that undernutrition slows colonic motility14 and that diminished physical mobility slows gastrointestinal motility, and as a result, constipation and fecal impaction are common complications of prolonged immobility.15 Spasticity and/or dystonia are often significant contributors to chronic constipation as spasticity and dystonia can disrupt normal defecation dynamics. In healthy individuals, rectal distension triggers the recto-inhibitory reflex and cues the individual to the urge to defecate after which he or she increases intra-abdominal pressure by taking a breath, closing their glottis, pushing downward with the diaphragm and tensing the lower abdominal muscles while simultaneously relaxing the muscles of the pelvic floor and the external anal sphincter. Individuals with spasticity or dystonia will often paradoxically contract the pelvic floor muscles and external sphincter while they are straining making the process of defecation extremely inefficient, ineffective, and more painful. Appropriate positioning during defecation may help mitigate these involuntary and counter-productive behaviors. If possible, have the person sit on the toilet. If there is a tendency for their buttocks to slip through the toilet seat, use a seat insert so they do not need to work to suspend themselves above the toilet bowl. While they are sitting, their knees should be flexed and at or above the level of their hips and their feet should be flat on the floor. Often it is necessary to place a step stool beneath their feet so they can achieve the appropriate posture. If the person is unable to sit on the toilet to defecate, have them lie left side down (e.g., the position we usually recommend when administering enemas), knees flexed at or above the level of the hips, and put something immobile beneath their feet to push against like the footboard of the bed.

Assessment of Constipation

Given how often children and adults with neurodevelopmental disabilities and/or limited mobility suffer from constipation, early identification and aggressive management of constipation is warranted. When eliciting a history, it is important not only to ask about the frequency of bowel movements, but whether there is any bleeding with the passage of bowel movements and also about the size, caliber and consistency of the bowel movements (Table 3). If the bowel movements are long and slender “snakes”, or if they pass small bowel movements throughout the day, this suggests the patient is experiencing anismus (failing to relax the pelvic floor muscles and external sphincter during attempted defecation) and is not completely relaxing his or her pelvic floor and external sphincter while straining and thus their defecation process is relatively inefficient/ ineffective.16 In most cases, anismus is the result of the patient experiencing perianal pain with defecation, however, as mentioned above, patients with spasticity or dystonia frequently paradoxically contract their pelvic floor and external sphincter while straining. Often the best way to determine if the patient is experiencing anismus is to ask about their posture while they are trying to defecate. If their buttocks are clenched and/or their legs are stiff and/or trembling, it is quite likely they are not relaxing their pelvic floor and external sphincter while straining. During the physical exam, it is important to try and determine if there is a fecal impaction. In some patients it is relatively easy to feel a large mass of stool in the descending and/or sigmoid colon. A digital rectal exam may prove useful not only to determine if there is a large amount of firm stool in the rectum, but also to evaluate perianal sensation, anal tone, and the presence of anal fissures or hemorrhoids. If the diagnosis of constipation is unclear based on the history and physical examination, an abdominal radiograph or transabdominal ultrasonography may be helpful in assessing the amount of stool in the colon.17 Even with a careful history and exam and abdominal imaging, it can be difficult at times to determine if a child or adult with developmental disabilities and/or limited mobility is truly suffering from constipation, and if that is the case, it is reasonable to treat them empirically.

Treatment of Constipation

Once the diagnosis of chronic constipation has been established, aggressive treatment should commence. Initial therapy should be aimed at eliminating a fecal impaction, as there is evidence that treatment outcomes of chronic constipation are better if patents undergo some form of disimpaction procedure before they commence daily laxative therapy.18,19 High doses of polyethylene glycol given over several days appear to be as effective as a series of enemas in eliminating impactions.19 The usual regimen for oral/enteral disimpaction is 1 g/kg of polyethylene glycol mixed in 8–12 ounces of fluid given three or four times daily for two or three days until the patient develops watery diarrhea. After disimpaction, some form of bowel regimen should be prescribed to prevent recurrence of the constipation and to produce soft bowel movements ideally every day or every other day.17 It is probably more important that the patient does not have to strain, and most importantly does not experience pain with defecation, than it is how often the patient is passing bowel movements. While additional fiber and additional fluid are often prescribed, these are rarely sufficient to assure children and adults with neurodevelopmental disabilities and/or limited mobility are regularly passing soft bowel movements without difficulty or pain, and may worsen gas, bloating and abdominal cramping, and further increase the colonic stool burden. Hence, some form of laxative regimen is almost always required.17 The most commonly prescribed laxatives are polyethylene glycol 3350, magnesium hydroxide, and lactulose, all of which are osmotic stool softeners. While there are no large comparative studies, most of the available evidence and experience suggest that provided they are given in sufficient doses, these agents are all equally effective; hence, the choice of the agent should be based on patient or family preference, cost, ease of administration, and potential side effects. The most common side effect of all of these agents is diarrhea, however lactulose often produces flatulence, distension and bloating. At higher doses magnesium can produce nausea and there are reports of hypermagnesemia when magnesium containing laxatives are given in very high doses and/or if the patient suffers from renal insufficiency.20 While docusate sodium is often prescribed, what little evidence there is suggests that in the doses typically prescribed, this agent is not a very effective stool softener.21 Given that many (if not most), children and adults with neurodevelopmental disabilities and/ or limited mobility who suffer from chronic constipation have disordered intestinal motility,1,2,17 treatment with osmotic stool softeners is often not sufficient to produce regular soft bowel movements without simultaneously causing fecal leakage or seepage. In this group of patients, the use of a stimulant laxative alone or in combination with osmotic stool softeners can be very effective. The most commonly prescribed stimulant laxatives are sennosides or bisacodyl (See Table 4 for different laxative preparations). Regardless of the laxative regimen that is prescribed, it is important to explain to the patient/ family that these agents will almost certainly need to be used chronically, and it is also very important to reassure them that there is no evidence that the chronic use of any of these agents results in dependency or increases the risk of colon cancer .22 In cases that don’t respond adequately to oral laxative therapy, some patients/families opt for regular use of suppositories or saline enemas. Another option is a Malone antegrade continence enema (MACE) procedure or a percutaneous cecostomy (Figure 2). With both of these procedures, there is a surgically constructed conduit from the skin into the proximal colon that allows the administration of antegrade colonic irrigations/enemas.17,23 With either a MACE procedure or percutaneous cecostomy, flushes of between 500 and 1000mL of water containing 17g or polyethylene glycol or 5 ml of glycerin soap are typically administered once or twice daily. For some patients, these procedures can substantially improve their quality of life.24,25 See Table 4 for treatment options for severe constipation in those with disabilities.

CONCLUSION

A large number of children and adults with neurodevelopmental disabilities, with or without limited mobility, are undernourished and suffer from chronic gastrointestinal difficulties including feeding intolerance and chronic constipation. While it is not always recognized, chronic constipation can clearly worsen feeding intolerance in this group of individuals. Not only will this worsen their nutritional status, but it may worsen their gastrointestinal symptoms, further compromising their ability to ingest adequate calories and producing a vicious downward spiral. Given how often children and adults with neurodevelopmental disabilities and/or limited mobility suffer from constipation and the potential impact of the constipation on their quality of life, as well as the potential for impairing their ability to tolerate enteral feedings, early identification and aggressive management of constipation is not only appropriate, but the right thing to do.

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FRONTIERS IN ENDOSCOPY, SERIES #59

Solid Pseudopapillary Tumors of the Pancreas: A Rare but Important Clinical Entity

February 2020 • Volume XLIV, Issue 2
Douglas G. Adler,Malan Shiralkar

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A 45-year-old man presented with 3 months of progressive left upper quadrant abdominal pain and a sensation of abdominal fullness. Complete blood count and comprehensive metabolic blood panel were normal. A CT scan showed a large solid pancreatic mass in the tail of the pancreas. Endoscopic ultrasound (EUS) was performed at 7.5MHz with Doppler. EUS revealed a hypoechoic, heterogeneous, 7×6 cm solid mass lesion in the pancreatic tail with scattered hyperechogenic foci consistent with some peripheral calcifications. (Figure 1). EUS guided core biopsy was performed with a 22 gauge needle. The pathology report revealed loosely clustered to discohesive monomorphic cells with eccentric nuclei and fine chromatin. There were hyalinized stromal fragments lined by monomorphic, bland-appearing, polygonal cells that appeared to be falling off the underlying stromal core. Immunohistochemical stains were used to aid in the evaluation of the pathology specimen. The cells of interest (associated with the hyalinized stromal core) are positive for B-catenin (nuclear), showed patchy positive staining for CD56. Tissue samples were mostly negative for Cam 5.2 and synaptophysin. The histology and immunohistochemistry together were supportive of a diagnosis of a solid pseudopapillary tumor (SPT). (Figures 2-5) The patient was referred to surgery and underwent a distal pancreatectomy and splenectomy. This confirmed the diagnosis of SPT. All resected lymph nodes were free of disease. He has done well thereafter.

Incidence and Demographics

SPT of the pancreas is a rare tumor that was first described by Virginia Frantz in 1959.1 SPT has previously been called many other names including solid and papillary epithelial neoplasm, papillary cystic neoplasm, solid and cystic papillary epithelial neoplasm, solid and cystic acinar cell tumor, low grade papillary neoplasm, Hamoudi tumor, and Frantz tumor.2 In 2010, the World Health Organization designated the name of this tumor as solid pseudopapillary tumor.2 SPT is a rare neoplasm, accounting for 1-2% of all exocrine pancreatic neoplasms and 0.17-2.7% of nonneuroendocrine tumors of the pancreas.3,4,5 Since 2000, there has been a seven fold increase in SPT, which has largely been attributed to more frequent and improved imaging tests as well as increased clinical awareness of this entity.3 SPT has a marked female predominance, affecting females around ten times more often than males.3,4,5,6 More than ninety percent of reported cases of SPT affect females in their second decade with a mean age of diagnosis around 22.5 years.5 Men are generally found to be diagnosed at a later age than women.14 The tumor has been found to have a higher prevalence in Asian and Black populations.

Symptoms

SPTs are often asymptomatic and are often incidentally noted.4,5 In patients who are symptomatic, the most common symptom is abdominal pain or mass with a reported incidence of 63% of symptomatic cases.5 Other less common symptoms include nausea, vomiting, fever, weight loss, jaundice, and early satiety. Acute abdomen can occur in the setting of tumor rupture.7 SPTs are not typically associated with exocrine or endocrine pancreatic insufficiency.3

Tumor Characteristics

SPTs can occur in any part of the pancreas; however, the most common site of the tumor in adults is the pancreatic tail, followed by the pancreatic head, then the body.3,5 SPTs are often large in size with a mean size of 7.5cm at time of diagnosis.5 There is significant variability in appearance of the tumor due to varying degrees of cystic degeneration within the tumor. There are often solid, cystic, and pseudopapillary components within the tumor.5 Smaller tumors tend to be more solid but less well circumscribed while larger tumors tend to have more cystic degeneration, hemorrhage, and necrosis with a pseudocapsule and variegated cut surface.5 Cyst formation tends to be more centrally located while solid components are usually found on the periphery.3 SPTs appear well circumscribed, but do not have a fibrous capsule and microscopically neoplastic cells infiltrate surrounding pancreatic parenchyma entrapping acinar cells and islets.8 Primary SPT can occur outside of the pancreas when there is presence of ectopic pancreatic tissue, but is extremely rare.3 The most common sites that this occurs include the ovary, mesocolon, and omentum.

Imaging

On both CT imaging and MR imaging, SPTs appear to be well circumscribed, encapsulated, and heterogeneous with hemorrhagic and cystic degeneration. MR imaging is more sensitive than CT imaging in evaluating intratumoral hemorrhage, cystic degeneration, and presence of capsule.14 Transabdominal ultrasound imaging often shows a heterogeneous solid and cystic mass with occasional calcifications.9 Findings of pancreatic duct dilation and vessel invasion are suggestive of more aggressive tumor.12 Other than surgery, the best diagnostic and imaging test is endoscopic ultrasound (EUS) with fine needle aspiration (FNA) or fine needle biopsy (FNB). On EUS examination, the mass will appear as a wellcircumscribed, hypoechogenic, heterogeneous tumor with solid and cystic components with calcifications.10 EUS has a reported sensitivity of 91-95% and specificity of 92-95% in the diagnosis of SPT3,11 Given concern for seeding of tumor cells to the peritoneum with laparoscopic biopsy, it is not recommended or routinely performed.

Pathology

On cytological examination, cells are typically bland, uniform, round to oval with eccentrically located nuclei, moderate cytoplasm, and finely dispersed chromatin.8,12 Metachromatic hyaline globules can be found in the cytoplasm.8 Clear myxoid material surrounds papillae.13 Large, cytoplasmic vacuoles can be helpful in differentiating SPTs from other pancreatic tumors.12 Clusters of foamy macrophages, multinucleated giant cells, cholesterol, and necrotic debris are occasionally seen. Histologically, the classic finding of a SPT is presence of pseudopapillary areas with fibrovascular stalks or rosette-like structures secondary to poor cohesion of the malignant cells. Immunohistochemically, most SPTs demonstrate intense nuclear localization of B-catenin and loss of membrane expression of E-cadherin with disruption of the activated Wnt pathway.8 SPTs are typically positive for vimentin, alpha-1-antitrypsin, alpha-1-antichymotrypsin, and neuron specific enolase. SPTs are typically negative for chromogranin A, epithelial membrane antigen, and cytokeratin.7 The presence of the CTNNB1 molecular marker in conjunction with the lack of KRAS, GNAS, RNF43 and LOH on chromosome 18 is helpful in making the diagnosis of SPT.7 Histiogenesis and the cell of origin of SPT have yet to be definitively identified. Given the female predominance of SPTs and the increased estrogen receptors associated with SPT, a proposed theory is that female sex hormones may play a role in tumorigenesis.3 In vitro studies have shown an increased proliferation of SPTs with estrogen.

Differential Diagnosis

The differential diagnosis of SPT is broad due to its variable appearance. The differential includes pancreatic neuroendocrine neoplasms, acinar cell cancer, mucinous or serous cystic neoplasms, and lymphoma. Pancreatic neuroendocrine neoplasms can often have similar histologic features as a SPT. The presence of pseudopapillae, hyaline globules, foamy histiocytes, and grooving of the nucleus are more consistent with the diagnosis of SPT. Alternatively, the appearance of speckled chromatin would favor a neuroendocrine tumor.12 Acinar cell carcinoma of the pancreas can be differentiated from SPTs by the presence of irregular nuclei with maintained cytoplasmic polarity and clusters of cohesive clusters of cells with acinar formation.12 SPT can be sometimes confused with serous cystadenoma on cross sectional imaging; however, serous cystadenomas are typically well circumscribed nodules with cystic spaces lined by cuboidal cells with clear, glycogen containing cytoplasm.12 EUS is often helpful in distinguishing SPT from serous cystadenoma, either by visualization alone or with the addition of FNA or FNB. Additionally, serous cystadenomas sometimes contain a central scar with a radiating pattern.

Prognosis

SPTs typically carry a good prognosis as they have low malignant potential. There is a low likelihood of metastases and vascular invasion. Only 10-15% of patients will develop metastases.15 The most common site of metastasis is the liver.5 Peritoneal metastases are less frequently seen and are thought to be a result of trauma or rupture of tumor.5 Lymph node metastases occur infrequently with less than 10 reported cases.5 There are even few reports of pulmonary metastases.11 Rarely does tumor locally invade into the vasculature, stomach, duodenum, or spleen.5 The overall prognosis of SPT even in the presence of metastases is excellent. The reported five-year survival rate is greater than 95% and is highest in patients with SPT limited to the pancreas.5 The reported mortality from this tumor is reported as less than 2%.15 Male patients and elderly patients tend to have a worse prognosis.3,4 Compared to female patients, males have a twofold higher incidence of metastases and a threefold higher incidence of death.

Treatment

Surgical resection of the tumor, with as much sparing of normal pancreatic tissue, is the treatment of choice for SPTs and is often curative.17 The type of surgery is dictated by the location and size of the tumor. Tumor invasion to the portal vein or superior mesenteric artery is not considered a contraindication for surgical resection, but may limit complete resection.14 Surgical procedures utilized include pancreatoduodenenectomy, distal pancreatectomy, middle pancreatectomy, duodenum-preserving pancreatic head resection, spleen preserving distal pancreatectomy and local resection.3 More invasive surgical techniques include synchronous portal-superior mesenteric vein or adjacent organ resection if there is evidence of local invasion.3 Due to increased risk of dissemination, higher recurrence rate, and development of pancreatic fistula, tumor enucleation is not recommended.3

Unfortunately, SPT can be unresectable if there is evidence of large vessel invasion. In patients with unresectable tumors due to size or location, neoadjuvant chemotherapy and radiation has been utilized to decrease the size to create conditions favorable for potential resection.3 Long-term survival is thought to be improved after resection of metastatic SPT. In patients with liver metastases, synchronous or metasynchronous surgical resection can be performed.3 Recent studies have shown that a one centimeter margin in hepatic metastasis resection is considered curative. In patients with diffuse hepatic metastases, which are not amenable to resection, liver transplantation (both orthotopic liver transplant and living donor transplant) has been performed in the past, as removal of liver metastases in addition to removal of primary SPT can potentially be curative.18 Diffuse growth pattern, extensive necrosis, high mitotic rate, and sarcomatoid areas within tumor have been associated with more aggressive tumor behavior.19 Patients with lymph node metastases, tumors larger than 8cm, cellular atypia, capsule invasion, lymphovascular invasion, perineural invasion, and peripancreatic fat tissue invasion were found to have worse outcomes following surgery.20 The reported incidence of tumor recurrence is around 1.9-6%.

The role of chemotherapy and radiation is unclear at this time. Described chemotherapy regimens have included 5-fluorouracil, doxorubicin, and streptozocin or interferon, cisplatin, and topotecan or gemcitabine-based chemotherapy. Long term follow up is suggested following surgical resection. Given the rarity of this tumor, there does not currently exist guidelines or a current consensus regarding the interval, length, or modality of follow up. Due to the low rate of recurrence of this tumor, no risk factors have been associated with recurrence. A proposed further therapy for SPT includes selective estrogen receptor modulators as in vitro studies have shown the influence of estrogen on tumor proliferation.

CONCLUSION

SPTs are rare pancreatic tumors but are increasingly being identified due to more frequent and better cross sectional abdominal imaging. They typically occur predominantly in a younger female population. Our patient was notable for his age and male sex. SPTs are usually asymptomatic and incidentally noted on imaging, but commonly associated symptoms include abdominal discomfort as experienced by our patient. Luckily, SPTs typically carry a good prognosis as they have low malignant potential with low likelihood of metastases to liver, peritoneum, lungs, and rarely lymph nodes. The reported five-year survival rate of SPT is greater than 95%. Treatment of SPTs includes surgical resection of the mass with as much preservation of the pancreas as possible and in some cases, neoadjuvant chemotherapy may be indicated.

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FROM THE LITERATURE

Surgical vs. Endoscopic Resection of Large, Nonmalignant Colorectal Polyps

January 2020 • Volume XLIV, Issue 1

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To determine nationally representative estimates and to identify predictors of in-hospital mortality and morbidity after surgery for nonmalignant colorectal polyps, data was analyzed from a national inpatient sample for 2005 to 2014. All discharges for adult patients undergoing surgery for nonmalignant colorectal polyps were identified. Rates of in-hospital mortality and postoperative wounds, infections, urinary, pulmonary, gastrointestinal or cardiovascular adverse effects were calculated. Multivariable logistic regression using survey-weighted data was used to identify variables associated with postoperative mortality and morbidity. An estimated 262,843 surgeries for nonmalignant colorectal polyps were analyzed. In-hospital mortality was 0.8% and morbidity was 25.3%. Postoperative mortality was associated with open surgical technique (vs. laparoscopic), older age, black race, Medicaid use and burden of comorbidities. Female sex and private insurance were associated with lower risk. Patients developing a postoperative adverse event had a 106% increase in mean hospital length of stay and 91% increase in mean hospitalization cost. It was concluded that surgery for nonmalignant colorectal polyps is associated with almost 1% mortality and common morbidity. Risk vs. benefit discussion for clinicians and patients was indicated, and although confounding by patient selection, cannot be excluded, the risk associated with surgery support consideration of endoscopic resection as a potentially less invasive therapeutic option.

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