bluuebunny/crossref_metadata_2025 huggingface dataset:
2030 A Unique Case of Appendiceal Orifice Bleeding
Authors: Valadez David , Camero Alfredo , Hackman Sarah , Penninti Pranav , Echavarria Juan
Published: October 2019

INTRODUCTION:

Appendiceal orifice bleeding is an uncommon etiology of lower gastrointestinal bleeding and is often attributed to various appendix pathologies. We report a case of appendiceal orifice bleeding in a woman with fibrous obliteration of the appendix.

CASE DESCRIPTION/METHODS:

A 51-year-old female with fibromyalgia and chronic abdominal pain underwent outpatient colonoscopy to evaluate several months of intermittent hematochezia. Hemoglobin four months prior to procedure was 12.3 g/dL. Colonoscopy revealed fresh blood in the cecum and right colon. After irrigation, active oozing from the appendiceal orifice was seen. There was no blood in the terminal ileum. She was also found to have a non-bleeding rectal AVM, which was cauterized. Hemoglobin the day of procedure was 11.8. She had normal vitals and physical exam. A CT scan obtained three days later revealed a normal appendix and GI tract. She was referred to general surgery and underwent laparoscopic appendectomy three months later. She reported intermittent hematochezia two months after her surgery but did not pursue further work up. Her next follow up encounter was 15 months after her surgery and no further hematochezia was reported. Pathology analysis of her appendix revealed fibrous obliteration with foci of neural hyperplasia and entrapped residual neuroendocrine nests. There was no evidence of cellular atypia, mitotic activity, or neoplasm.

DISCUSSION:

Available literature on appendiceal orifice bleeding is sparse and mainly compromised of isolated cases. Most case reports involve acute, inpatient situations and abnormal CT findings, so it is important to underscore the decision to pursue a CT scan despite our patient’s chronic and mild presentation. The recurrent hematochezia two months after surgery is difficult to explain, but it did resolve by the time she re-established care and could be unrelated. Pathology findings did not include previously noted etiologies of appendiceal orifice bleeding (erosions, diverticula, malignancy). However, fibrous obliteration of the appendix is a rare finding and occurs when spindle cell proliferation replaces the appendiceal lumen. Fibrous obliteration of the appendix is also termed neurogenic appendicitis or appendiceal neuroma. There does not appear to be any previous associations of documented appendiceal orifice bleeding and fibrous obliteration of the appendix. Our case highlights the need for further investigation into these two rare entities.
2031 Metastatic Neuroendocrine Tumor of Unknown Primary Presenting as Acute Gastrointestinal Bleed With Hemorrhagic Shock
Authors: Mankoo Ravinder , Malik Lybia , Habib Mustafa , Smith Zachary , Kuwajima Vanessa , Ibdah Jamal A. , Tahan Veysel
Published: October 2019

INTRODUCTION:

Neuroendocrine tumors (NETs) are often localized, however up to 50% of cases can be metastatic, with 13% having an unknown primary . Most NETs are identified during work up of related symptoms or functional syndrome (i.e. carcinoid) or found incidentally. We present a case of metastatic NET of unknown primary origin presenting as acute hemorrhagic shock from gastrointestinal bleeding.

CASE DESCRIPTION/METHODS:

A 21-year-old male presented with hematochezia, abdominal pain and nausea and was admitted to the ICU for hemorrhagic shock with a drop in hemoglobin from 14.5 to 8.2. EGD showed 15 mm, necrotic, oozing duodenal ulcer without visible blood vessel, treated with Hemospray. Recurrent episode of bleeding necessitated IR-guided GDA embolization. CT imaging initially identified, and later MRI confirmed three complex hepatic lesions in both right and left lobes of the liver . IR-guided liver biopsy showed low-grade, well-differentiated metastatic NET, Ki-67 1%. Gastrin and chromogranin A levels were elevated at 166 pg/mL and 223 ng/mL respectively. Otreotide scan found enhancement in the liver consistent with metastatic NET, without identification of a primary site. He was started on octreotide infusions. Additional work-with CT chest and pelvis, repeat EGD/EUS and colonoscopy were negative for primary tumor. He underwent laparotomy with left lateral segment partial hepatectomy, microwave ablation of the remaining liver lesion and cholecystectomy.

DISCUSSION:

NETs are typically indolent cancers involving the small intestine, appendix, colon/rectum, pancreas or lung. Common sites of metastasis include the liver, peritoneum, lymph nodes and lungs. The majority of metastatic NETs have an identifiable primary site, but it is important to recognize that many can have an unknown origin. Understanding the primary site of NETs is an important prognostic factor in overall survival. In general, metastatic disease is associated with a poorer prognosis, particularly in the setting of unknown primary origin. This case illustrates that patients with NETs can have a variable initial presentation, including acute GI bleeding from peptic ulcer disease. In addition, this case shows that despite appropriate diagnostic testing including biochemical markers, anatomic and functional imaging studies, endoscopic procedures and tissue biopsies, the primary site of metastatic NETs can go unidentified, which can effect definitive treatment options.
2035 Small Cell Carcinoma of Rectum: A Rare Cause of Hematochezia
Authors: Wieckowska Joanna , Patel Kruti , Minaudo Mark
Published: October 2019

INTRODUCTION:

Small cell carcinoma (SmCC) is a subtype of neuroendocrine tumor that has commonly been associated with lung malignancy. However, it has been seen in <1% of colorectal malignancies. Majority of neuroendocrine tumors are asymptomatic and non-functioning, but about 75% have metastatic disease by the time of diagnosis. We present a case of SmCC of the rectum in a patient who presented with intermittent hematochezia.

CASE DESCRIPTION/METHODS:

A 65-year-old female with a medical history of Diabetes Mellitus Type II, Hypertension, and Hyperlipidemia, was referred for endoscopic evaluation of intermittent hematochezia ongoing for several months with the following associated symptoms: change in bowel habits, constipation, perianal discomfort increased upon defecation, urinary frequency, and dysuria. She was not on anticoagulation or NSAIDs. She had no personal or family history of GI malignancy or IBD, and she denied tobacco, alcohol, or illicit drug use. Screening colonoscopy ten years ago had been negative for any abnormalities. Laboratory data was unremarkable for anemia or liver disease, but did reveal a mildly elevated CEA 4.8 ng/mL. Subsequent colonoscopy revealed large ulcerated rectal mass at 15 cm with luminal narrowing concerning for rectal malignancy. Follow up imaging with CT of the abdomen and pelvis with contrast showed a large rectal mass with multiple enlarged peri-rectal lymph nodes, 1.5 cm right adrenal nodule, 3 mm non-calcified nodule of left lower lobe and multiple sub-centimeter hypodense lesions in left liver lobe. Biopsy with histological staining suggested neoplastic cells consistent with rectal small cell carcinoma. Patient was treated with neoadjuvant chemotherapy and radiation therapy followed by abdominoperineal resection. Ultimately, she could not tolerate adjuvant therapy and enrolled in hospice.

DISCUSSION:

To date, fewer than 1000 cases of GI SmCC have been reported with prevalence of 0.1% to 1% of all GI tumors. Seventy- five percent of patients present with metastasis at the time of diagnosis. Six-month survival rate is 58%, and five-year survival is 6%. Clinical presentation generally mimics the usual presentation of colorectal malignancies. Workup is also similar. However, there is no standard treatment approach for SmCC as none has been developed due to low incidence. Given the paucity of literature, cases should be reported to influence future prospective studies to establish standard diagnostic and treatment regimens and to improve the overall prognosis.
2036 A Rare Cause of Obscure Gastrointestinal Bleeding
Authors: Som Aniruddh , Nath Anand , Lee Zone-En
Published: October 2019

INTRODUCTION:

Serrated adenomas are most commonly detected in the colon and characterized by the saw-tooth-shaped infoldings of the mucosa and crypt epithelium. It is a precursor precancerous lesion accounting for 10–30% of all colorectal carcinomas. Serrated adenoma in the small intestine have been reported much less frequently and are localized predominantly in the duodenum, with jejunal adenomas being rarer (< 10 cases). We hereby describe a case of recurrent melena in the setting of a jejunal serrated adenoma.

CASE DESCRIPTION/METHODS:

A 63-year-old male with a past medical history of CAD, ischemic cardiomyopathy with ejection fraction 10-15%, paroxysmal A Fib, PE on warfarin presented to the hospital in acute decompensated heart failure. After further workup and evaluation, the patient underwent LVAD placement. The hospital course was complicated by respiratory failure requiring intubation and cardiogenic shock requiring inotropes. He then had an episode of melena with a fall in hemoglobin from 9.4 to 7.0 gm/dl on anticoagulation. He underwent push enteroscopy that revealed a pedunculated jejunal polyp with active bleeding noted in the proximal jejunum. This was resected, clipped and the site was tattoed leading to stabilization of his hemoglobin.10 days later the patient had a repeat episode of melena after resumption of anticoagulation. Repeat push enteroscopy showed visible bleeding vessel adjacent to jejunal tattoo treated with epinephrine injection and bicap cautery. The pathology report was consistent with serrated adenomatous polyp of the jejunum. The patient recovered well after the repeat procedure and is doing well after resumption of anticoagulation.

DISCUSSION:

The small bowel accounts for 75% of the length of the alimentary tract yet small intestinal serrated adenomas are extremely rare. They are challenging to diagnose because of their nonspecific presenting symptoms. Barium GI series or CT scans are not sensitive in detecting lesions smaller than 4 cm. Enteroscopy is an ideal tool in evaluating these cases with obscure GI bleeds. Although the morphology of small bowel serrated adenoma resembles that of traditional adenomas of the colon, the biomolecular alterations and biological behaviors indicate a distinct subtype with more aggressiveness and higher malignant potential. Hence they need therapeutic resection either endoscopically or surgically based on an assessment of their size and location. Given the paucity of data, currently, there is no consensus on surveillance interval.
2037 Intestinal Spirochetosis: An Unexpected Cause of Massive Gastrointestinal Bleed
Authors: Bilal Muhammad , Umar Anam , Chang Eric Y. , Abe Temidayo , Simien Melvin
Published: October 2019

INTRODUCTION:

Human intestinal spirochetosis (HIS) is a condition characterized by the presence of spirochetal microorganisms attached to the apical cell membrane of the colorectal epithelium leading to chronic diarrhea. The invasive form of this disease is more often seen in HIV patients or homosexual individuals. Here, we present a rare case of HIS presenting as lower gastrointestinal (GI) bleed and abdominal pain in the absence of diarrhea.

CASE DESCRIPTION/METHODS:

A thirty-year-old male with a history of peptic ulcer disease (PUD) and HIV (CD4 >250) presented to the emergency department with a two-days duration of severe, generalized abdominal pain and bleeding per rectum. On presentation, the patient was hypotensive (mean arterial pressure < 65) and tachycardic (heart rate 120). Physical examination revealed generalized abdominal tenderness with normal bowel sounds but no rebound tenderness or guarding. Labs were significant for anemia (Hemoglobin 6.5 g/dl; baseline >14 g/dl). The rest of his labs including serum chemistry, white blood cells, amylase, lipase levels, were within normal limits. Due to the patient’s significant history of PUD and concern for rapid GI bleed, aggressive fluid resuscitation and proton pump inhibitor infusion were initiated. He also received two units of packed red blood cells with good toleration. CT scan of the abdomen/pelvis did not reveal any acute intraabdominal pathology. Despite the achievement of hemodynamic stability, the patient continued to have bright red blood per rectum. Extensive infectious workup (including blood cultures and stool tests such as stool culture, ova, and parasites) were negative. Esophagogastroduodenoscopy was performed and revealed well healed PUD without any signs of bleeding. Colonoscopy, however, revealed pan-colonic ulcers, most pronounced in the descending colon. Histopathology revealed spirochetal microorganisms attached all the way from the ascending colon to the rectum consistent with invasive HIS. Metronidazole was initiated with significant clinical improvement and he was subsequently discharged home with appropriate outpatient follow up.

DISCUSSION:

Most cases of HIS are asymptomatic and often diagnosed during screening/surveillance colonoscopy. Thus, data regarding symptomatic patients is scant. The few cases describing affected patients generally associate infection with chronic watery diarrhea, acute appendicitis or intermittent hematochezia. Metronidazole is an effective treatment of choice in symptomatic patients.
2041 Successful Treatment of Ulcerative Colitis and Seronegative Inflammatory Spondyloarthritis Using Concomitant Vedolizumab and Tofacitinib: A Case Report
Authors: Lee Joy A. , Magavi Pooja R. , Konijeti Gauree G.
Published: October 2019

INTRODUCTION:

Inflammatory bowel disease (IBD) encompasses ulcerative colitis (UC) and Crohn’s disease, two chronic digestive diseases that fall on a clinical spectrum. While they overlap in some ways, many of their defining characteristics are distinct. The management of IBD continues to evolve. Combination therapy has emerged as a strategy to achieve remission through various mechanisms. The use of multiple agents with distinct molecular targets may be the key to helping patients achieve steroid-free remission.

CASE DESCRIPTION/METHODS:

55-year-old female with pan-UC and seronegative HLA B27 inflammatory spondyloarthritis, both with partial response to adalimumab, presented with abdominal pain, diarrhea, and rectal bleeding. C-reactive protein and tissue transglutaminase IgA were normal. Stool testing was negative for pathogens. Colonoscopy revealed moderate, active pan-UC (Mayo 2) without dysplasia. The patient was switched to infliximab, with inadequate response. Given the limited efficacy of anti-TNF therapy, vedolizumab was initiated. Despite vedolizumab dose escalation to q5weeks, she continued to have intermittent abdominal pain, loose stools, and arthralgias. Fecal calprotectin was < 100 ug/g. Vedolizumab trough level was > 40 ug/mL without anti-drug antibodies. Subsequent add-on trials of mesalamine and methotrexate were unsuccessful. Repeat colonoscopy showed mild, diffuse colonic erythema without erosions (Mayo 1), with active colitis in the proximal and distal colon and inactive colitis in the rectum. Despite steroid-free response, the patient’s abdominal and arthritic symptoms persisted. Thus, tofacitinib (5 mg twice daily) was added, with resolution of all symptoms. Colonoscopy after 6 months on combination therapy showed normal colonic mucosa (Mayo 0) and inactive colitis.

DISCUSSION:

Although the response to treatments for moderate-to-severe UC remains low, combining newer therapies with differing mechanisms of action may improve remission rates, particularly in refractory patients. However, cost and the risk of adverse reactions must be determined before initiation. Vedolizumab, a gut-specific anti-integrin with a favorable safety profile, led to improvement in our patient’s UC, but not her arthritis. The addition of tofacitinib, a janus kinase inhibitor, led to resolution of arthralgias and remission of UC. Randomized trials evaluating combination therapy in IBD may help guide future management of patients with refractory UC.
2052 Crohn's Disease and Rare Renal Extra-Intestinal Manifestations: Two Case Reports
Authors: Simmelink Andrew , Spence Linsey , Hanson John
Published: October 2019

INTRODUCTION:

Crohn's Disease (CD) diagnosis is usually based on the presence of gastrointestinal symptoms and findings. Extra-intestinal manifestations (EIMs) occur in up to 50% of patients with CD. We present two cases of rare renal EIM associated with CD activity.

CASE DESCRIPTION/METHODS:

Case 1: 31 yo white male w/ ileal-colonic CD diagnosed in 2014 presents with 6 weeks of diarrhea, abdominal pain, fatigue, and frothy urine. 3 years prior he developed biopsy proven acute interstitial nephritis from mesalamine which resolved with cessation and steroid taper. He is not on any medications. Laboratory analysis reveals: BUN 40 mg/dL, Cr 6.06 mg/dL, GFR of 13 mL/min, CRP 4 mg/dL, urinalysis with 25 RBCs and no leukocytes, urine protein/creatinine ratio (Pr/Cr) of 1.6, and negative serologies for HIV, HCV, HBV, ANA, ANCA. Complement levels were normal. Renal biopsy shows tubular interstitial nephritis (TIN) with lymphocytic T-cell infiltrate with interstitial fibrosis and tubular atrophy. Random colon biopsies show mild active pan-colitis with non-necrotizing granulomas and normal terminal ileum. He is treated with a slow prednisone taper and adalimumab, but does not have renal recovery resulting in a kidney transplant. Immunosuppression includes tacrolimus, mycophenolate mofetil, and prednisone. Follow-up CD monitoring reveals no symptoms and negative fecal calprotectin. Case 2: 21 yo healthy white female presents with 3 months of watery diarrhea, abdominal pain, and fevers up to 103 F. She is on no medications. Laboratory analysis reveals: Hgb 8.4 g/dL, normal BUN, and a Cr of 1.8 rising to 2.6 mg/dL. Urinalysis with 4 RBCs, 39 leukocytes with negative culture, positive urine eosinophils, Pr/Cr 0.36, CRP of 17 mg/dL, and negative serologies as in case 1. Colonoscopy reveals moderate pan-colitis with non-necrotizing granulomas. Renal biopsy reveals granulomatous interstitial nephritis. She improves with a prolonged steroid taper but has persistent but mild colitis on evaluation. She has no renal recovery but does not require dialysis.

DISCUSSION:

Renal EIMs of CD are rare. Most commonly they include nephrolithiasis in 28% CD, medication adverse effects, amyloidosis, as well as CD activity correlated TIN and glomerulonephritis. There is no standard treatment for TIN, but a prolonged steroid taper, MMF, cyclosporine, and anti-TNF therapy may be attempted for CD and renal recovery. Both cases reveal renal recovery may not occur with significant immunosuppression and transplant may be required.
2056 CMV Colitis Superimposed on Ischemic Colitis in an Immunocompetent Individual
Authors: Surapaneni Balarama K. , Choksi Vivek , Sorathia Sufian , Patel Nehal , Yella Prashanth R. , Priyadarshni Shivani , Kasmin Franklin
Published: October 2019

INTRODUCTION:

Cytomegalovirus (CMV) infections are more commonly seen in individuals with immunosuppressive diseases. We present a unique case of CMV colitis superimposed as ischemic colitis in an immunocompetent individual.

CASE DESCRIPTION/METHODS:

A 68-year-old man with history of PE (on Xarelto), pneumothorax & COPD presented with 10 weeks of diarrhea, associated with chills, lower abdominal discomfort, poor appetite, and weight loss of 15 pounds. Rectal exam showed dark stool and FOBT was positive Lab data showed elevated ESR and CRP. All stool studies were negative. CT Abdomen reported colonic findings suggestive of colitis with areas of active inflammation. Colonoscopy showed inflammation characterized by congestion (edema), erosions, granularity & ulcerations in a continuous and circumferential pattern from the sigmoid colon to the terminal ileum with sparing of mid & distal sigmoid colon and the rectum (Figures 1 and 2). Preliminary biopsies were suspicious for Crohns Disease. Patient was started on PO Prednisone 60 mgs daily & Pentasa TID and discharged for close follow up with GI. He kept on failing outpatient treatment, and was re-admitted twice with worsening of symptoms. Repeat CT scans showed worsening diffuse colitis. C Diff was checked, which was –ve. He was re-started on IV Abx, IV Steroids and supportive care. His symptoms continued to worsen, so Colorectal Surgery was consulted and, he underwent open subtotal colectomy. Pathology Slides from the Surgical Specimen were diagnostic for Ischemic looking bowel, and showed no features of IBD. So, decision was made to send the slides for second opinion. Meanwhile, he continued to deteriorate, so he was taken for emergent exploratory laparotomy and was found to have diffusely ischemic and distended bowel. Confirmation of pathology was not consistent with IBD. It showed Ischemic ileocolitis with possible superimposed CMV colitis with CMV inclusion bodies (Figure 3). IV Ganciclovir was initiated, steroids were discontinued. He began to improve, diet was advanced and was discharged with GI follow up.

DISCUSSION:

Suspicion of CMV colitis should be investigated by endoscopic evidence. The typical findings such as owl’s eye inclusion bodies on histology is specific for CMV. This is an unusual case of CMV associated ischemic colitis in an immunocompetent individual. This case report also should sound an alarm to GI physicians on thinking about CMV colitis infections even in immunocompetent individuals.
2071 Rare Case of Ulcerative Colitis With Autoimmune Hemolytic Anemia
Authors: Manoharan Suganya , Aloreidi Khalil , Gujjula Srilaxmi , Vachhani Akshaykumar , Reddy Madhavi , Etienne Denzil
Published: October 2019

INTRODUCTION:

Inflammatory Bowel disease (IBD) encompasses Ulcerative colitis (UC) and Crohn’s disease. UC is a chronic inflammatory process causing inflammation limited to the mucosal layer of the intestine. Anemia can occur due to multiple reasons in UC - iron deficiency anemia, anemia of chronic disease and rarely autoimmune hemolytic anemia. Here, we present an UC patient with rare manifestation of autoimmune hemolytic anemia (AIHA).

CASE DESCRIPTION/METHODS:

Report: 42-year-old male with significant past medical history of UC was admitted because of diarrheal episodes, jaundice for one week. He discontinued mesalamine two months prior to presentation. His vital signs were unremarkable and physical exam were was significant for pallor. Pertinent labs include hemoglobin: 5.7 g/dl, WBC count: 11.8 k/mm3, MCV: 113 fl, RDW 20.8%., Total Bilirubin: 4.1 mg/dl with direct of 2.7 mg/dl, LDH 592 U/L, and Haptoglobin was 8 mg/dl and reticulocyte count: 14.3%. On further studies cold agglutinin were found to be positive. Peripheral smear showed significant polychromasia, anisopoikilocytosis, macrocytes, mild agglutinated RBC. Stool studies were negative as well. Patient was transfused two units of blood. Colonoscopy with biopsy confirmed active colitis throughout cecum, ascending colon, transverse colon, sigmoid colon and rectum. Colonoscopy didn’t reveal any bleeding. Subsequently, patient was started on mesalamine, which lead to improvement of his symptoms. Hemoglobin level remained stable. Patient was discharged on maintenance dose and to follow up with hematology and gastroenterology as outpatient.

DISCUSSION:

AIHA is a rare complication of UC. It occurs in less than 1 % of UC cases. It is associated with acute flare up of UC. AIHA is caused by presence of warm agglutinins present in the blood that’s mostly IgG which reacts with the proteins on RBC membrane at particular temperature and causing hemolysis. There are several mechanisms causing AIHA in UC, IgG autoantibodies targeted against the RBC, modification of RBC when they pass through the colon and absorption of the antibodies from the colitis lesions. The treatment includes steroids and mesalamine. For resistant cases, immunotherapy and splenectomy can be considered and the last resort being colectomy. It is important to keep AIHA as a differential for secondary causes of anemia in patient with UC. Further studies are needed for the pathogenesis and treatment of the same.
2078 Can Dosing Intervals of Adalimumab Be Increased in Crohn’s Disease?
Authors: Euers Lindsay , Glassner Kerri , Wilhite Amanda , Abraham Bincy
Published: October 2019

INTRODUCTION:

Adalimumab is a recombinant monoclonal antibody that binds to human tumor necrosis factor alpha (TNF-a), thereby interfering with TNF-α receptor sites and cytokine-driven inflammation. There is limited data on de-escalation of adalimumab dosing to intervals greater than every other week (EOW) in Crohn’s disease. We present a case of a young man with Crohn’s who remained in clinical remission on adalimumab de-escalation dosing.

CASE DESCRIPTION/METHODS:

A 23-year-old man with Crohn’s disease presented to clinic in September 2018 in remission on adalimumab 40 mg every 7 weeks. He initially presented in January 2011 with bloody diarrhea, fever, anemia, leukocytosis and elevated ESR. Colonoscopy revealed a normal small bowel with pancolitis. He started oral budesonide and mesalamine, however due to lack of improvement he required escalation to biologic therapy with infliximab. Several months after initiation, he developed an infusion reaction and was switched to adalimumab. After induction, he went into clinical remission. At each yearly follow up his dosing interval was increased, until 2018 when he was on every 7 week dosing. In October 2018, inflammatory markers were normal and adalimumab level was low but detectable at 1.1 without drug antibodies, so dosing was increased to every 5 weeks. Colonoscopy in February 2019 revealed moderate inflammation in the descending, transverse and ascending colon with a total SES-CD score of 12. He is scheduled to have a repeat adalimumab level drawn before his next infusion.

DISCUSSION:

Recommended maintenance dosing for adalimumab is 40 mg EOW. Drug levels can vary from one patient to the next. In a retrospective cohort study Van Steenbergen et al. identified 40 patients with Crohn’s who de-escalated adalimumab to every 3 week dosing. At a median follow-up of 24 months, 65% of patients maintained clinical response, and 35% needed dose escalation back to EOW because of relapse, low drug levels, or both1. Our case is the first of a patient maintained in clinical remission, with detectable drug level and no antibody formation on adalimumab at intervals greater than 3 weeks. Given the economic burden of biologic therapy, prolonging the dosing interval of therapy is of interest. More studies are needed to investigate the effects of de-escalation in Crohn’s disease.
2080 The Curious Case of the Belly Button: Penetrating Crohn’s Disease Presenting as Umbilical Drainage
Authors: Steinhauser Christina M. , Singla Manish
Published: October 2019

INTRODUCTION:

Crohn’s disease is an inflammatory condition that can present with fistulous communications with extraluminal structures. The presence of urachal remnants that do not communicate with the skin account for 50% of urachal malformations and are generally asymptomatic and benign.

CASE DESCRIPTION/METHODS:

We present a case of an active duty soldier, with no previous medical history, found to have Crohn’s disease presenting as an enterourachocutaneous fistula. The patient presented to primary care with persistent umbilical drainage that did not resolve with antibiotics given. Computed tomography showed a mass on the dome of the bladder with surrounding inflammation but no extravasation of contrast outside of the bladder during cystography. Ileocolonoscopy revealed a narrowed ileocecal valve with biopsies consistent with active Crohn’s disease. Magnetic resonance enterography showed a fistulous communication from the terminal ileum to a urachal cyst which drained to the skin via the umbilicus. After multi-disciplinary discussion with urology and colorectal surgery, patient will undergo planned cyst, fistula, and terminal ileal resection.

DISCUSSION:

This case represents a rare presentation of Crohn’s disease. The true prevalence of urachal malformations is unclear; a large number remain undiagnosed until noted on imaging when asymptomatic. Enterourachal fistulas are rare, with only 13 cases reported; cutaneous communication has occurred in only 4 of the 13 reported cases. Cutaneous fistulas are more commonly seen in penetrating Crohn’s Disease given proximity of the bowel to the abdominal wall. In our case, the involved segment of bowel created a cutaneous fistula by way of a patent urachal remnant. This case highlights the ability of Crohn’s disease to mimic more benign conditions. A diagnosis of Crohn’s disease should be considered in patients with umbilical drainage that fails to resolve with antibiotics.
2098 Management of Severe Ulcerative Colitis in a Patient With Familial Dysautonomia
Authors: Hine Ashley M. , Ramprasad Chethan , Barnes Erin , Kaufmann Horacio , Chang Shannon , Malter Lisa
Published: October 2019

INTRODUCTION:

Familial dysautonomia (FD) is a progressive neurogenetic disease with carrier rate as high as 1 in 18 persons in European Jews of Polish origin. Clinical hallmarks include cardiovascular instability, spinal deformities, renal dysfunction, alacrima, ataxia, and impaired nociception. Physical or emotional stress may elicit autonomic crises characterized by hypertension and vomiting. Despite profound sensory deficits, GI perturbations are frequently reported by FD patients. While the incidence of inflammatory bowel disease (IBD) and FD is unknown, concurrence is underreported given increased frequency of both diseases in Ashkenazi Jews.

CASE DESCRIPTION/METHODS:

We report a 33-year-old female with FD and ulcerative colitis who presented with one week of abdominal pain and bloody diarrhea. She had been maintained on balsalazide. Colonoscopy one year prior revealed endoscopic and histologic remission. On physical examination, her abdomen was tender in the lower quadrants. A CT scan revealed pancolitis. Stool studies resulted negative. Her CRP was 58.4 mg/L and albumin was 2.4 g/dL. A flexible sigmoidoscopy noted Mayo endoscopic score 3 in the rectum and CMV staining was negative. The patient was started on IV steroids. Her hospital course was complicated by ileus, parainfluenza infection, and MSSA bacteremia with a pacemaker lead vegetation, requiring extraction. Lack of optimal clinical response to treatment on hospital day five led to consideration of alternative treatments with careful attention to her underlying FD. A subtotal colectomy with end ileostomy was unfavorable due to concern for volume loss. Infliximab and cyclosporine were opposed due to infection risk and later exhibiting possible nephrotoxicity. During this discussion the patient improved enough to be transitioned to oral steroids with a plan to initiate vedolizumab as an outpatient. On recent colonoscopy she had achieved mucosal healing.

DISCUSSION:

This is the first case of UC in a FD patient reported. Given myriad GI symptoms in the later diagnosis it can be hard to distinguish disease-related from treatment-related events. Due to the gut-specificity of vedolizumab, infection risk is considerably reduced compared to that of other biologics and is the most favorable option in the setting of underlying FD. This case highlights the difficulty encountered when treating IBD in the setting of systemic illness and underscores the need to carefully consider management options to enhance patient outcomes.
2106 Granulomatous Lung Disease in a Patient With IBD: What Is It?
Authors: Padial Doble Ian C. , Torres Esther
Published: October 2019

INTRODUCTION:

When a patient with inflammatory bowel disease (IBD) presents with respiratory complaints the differential diagnosis includes many conditions unrelated to IBD. Extra-intestinal manifestations (EIM) involving the lungs are relatively uncommon. Biologics for IBD can also have secondary effects including pulmonary toxicity and infectious complications.

CASE DESCRIPTION/METHODS:

A 48 y/o female nurse with history of bronchial asthma and hypertension was diagnosed with ulcerative pancolitis in 2005. In 2017, she was started on adalilumab (ADA) after repeated use of steroids and intolerance to azathioprine. ADA was discontinued after one year due to multiple subcutaneous nodules and fever that cleared on drug discontinuation. Differential diagnosis included erythema nodosum and hidradenitis. She was found with (+) quantiferon despite negative tuberculin (TB) skin test and chest x ray and was treated for latent TB with isoniazid for one year (04/18-04/19). Four months later (08/18), she was started on infliximab (IFX) with good response. On 01/19, an incidental left pleural effusion was found and she underwent thoracentesis. In 03/19, she developed night sweats and pleuritic chest pain. Chest x-ray demonstrated a large right pleural effusion. Bronchoscopy and pleural biopsy showed necrotizing granulomas negative for AFB and GMS. IFX was discontinued and she was started on high dose steroids with little improvement. Chest CT preformed on 05/19 demonstrated bilateral effusions and multiple upper lobe and centrilobar noncalcified opacities with tree in bud appearance. Bronchoscopy with lung biopsy showed multiple noncaseating granulomas negative for AFB and GMS.

DISCUSSION:

The differential diagnosis in this patient includes lung involvement of IBD, vasculitis, infections, drug reaction and concurrent primary sarcoidosis among others. Vasculitis and infections have been excluded, and the lack of improvement after IFX discontinuation and steroids suggests IFX is not the cause. However, several cases of sarcoidosis associated to anti-TNF drugs have been reported and drug-induced sarcoidosis remains a high probability. Pulmonary EIM is also still a possibility in spite of clinical remission of UC.This case report demonstrates the importance of remaining aware of possible rare EIM of IBD and also of other rarer complications in this new age of biologics. Early diagnosis and effective treatment are important to prevent complications and improve outcomes for these patients.
2115 Novel Use of a Bare Metal Self Expanding Stent for Biliary Drainage Following Hepatic Cystectomy
Authors: Anderson Jeremy , Truscello David , St. Michel David , Sebastian Eli
Published: October 2019

INTRODUCTION:

Bile leak following a partial hepatectomy or cystectomy is not uncommon. Perihepatic collections following surgery are largely managed in two ways: Image-guided percutaneous drainage or endoscopic retrograde cholangiopancreatography (ERCP). Intervention during ERCP may include sphincterotomy with placement of a plastic 7 or 10 Fr stent. While metal stents have been indicated in cases of biliary stenosis, no cases to date have presented the use of a covered self-expanding metal stent (cSEMS) for maximizing drainage. We present the case of a 78 yo female with polycystic liver disease who had a cSEMS placed after the formation of a perihepatic bile collection following a liver cyst resection.

CASE DESCRIPTION/METHODS:

This is a 78 year old female with a history of polycystic liver disease (PLD). Patient had previous resection of large liver cyst approximately one year earlier. She developed recurrence of a large liver cyst requiring surgical intervention and underwent laparoscopic liver cyst resection of the right lobe. Within one week of surgery, increased biliary drainage prompted a return to the hospital. Gastroenterology was consulted and took the patient for ERCP. A 10 mm covered self-expanding metal stent was placed in the common bile duct under direct visualization of the sphincter in conjunction with fluoroscopy imaging. This was done instead of Fr stents with the expectation that a large metal stent would afford improved biliary drainage and divert away from parenchymal leakage. The patient did well following the procedure and was discharged with percutaneous drain in place. Follow up CT of the abdomen and pelvis was performed 5 weeks following placement of biliary stent. The study demonstrated that the drain and stent were in good position. There was an interval decrease in size of perihepatic collection.

DISCUSSION:

A bile leak is often found due to increased output or change in fluid output from a surgical drain as was seen in this patient. If endoscopic intervention is undertaken, current practice involves placement of plastic stents. Literature demonstrates the use of cSEMS in patients who present with biliary stricture secondary to malignancy. Comparisons of plastic versus metal stents in this population show migration rates were lower in the metal stent arm. While there was no comparison of their drainage capabilities, they do demonstrate the feasibility of cSEMS placement in other patient populations where plastic stent placement has been standard.
2124 Piling Gold in the Prostate
Authors: Hanjar Abrahim , Kumar Manoj , Fahmawi Yazan , Mizrahi Meir
Published: October 2019

INTRODUCTION:

Precisely steered radiation beams to tumors attenuates the radiation to contiguous tissues. Image-guided radiation therapy which uses implantable radiographic markers, or fiducial markers (FMs), serving as reference points to accurately delineate tumor location. This is particularly important in the prostate, a moving target whose positioning depends on the dynamics of its neighboring bladder and rectum. Traditionally, implantation of FMs into the prostate has been an invasive surgical procedure with potential risks. With the evolution of advanced endoscopic ultrasound (EUS), FMs placement under EUS guidance is rapidly gaining interest as a novel therapeutic technique. Below is a case series discussing our experience with this procedure.

CASE DESCRIPTION/METHODS:

Case Series: Technique- Conscious sedation was used. Transrectal EUS was performed by using a curvilinear-array echoendoscope. The base and the apex of the prostate were identified as a hypoechoic, round structure with respect to rectum and seminal vesicle location. Using a 22-gauge needle, 4 fiducials were placed in a 4-quadrant fashion (bilateral base and apex) with at least 1 cm of distance between the fiducials. 1st case: 60-year-old AAM with prostate cancer was referred for fiducial placement for stereotactic body radiation therapy (SBRT). FMs were placed under EUS guidance. Patient completed his radiation treatment and did not report any procedure related complications during follow ups. 2nd Case: 73-year-old male on active surveillance for low risk prostate cancer for several years was elected for SBRT due to recent elevation of prostate specific antigen (PSA). FMs were placed under EUS guidance. Patient completed the SBRT successfully. Post treatment, patient had episode of acute prostatitis and cystitis managed with Analpram and Flomax and was linked as unrelated to FMs placement. 3rdcase: 53-year-old male with prostate adenocarcinoma underwent EUS guided FMs placement. He completed SBRT successfully. On follow-up visits he reported some burning on urination that was controlled with Pyridium, minimal bowel symptoms of increased frequency of bowel movements in the morning and generalized fatigue. These symptoms were not speculated to be induced by the FMs placement.

DISCUSSION:

Discussion: EUS-guided placement of FMs for prostate radiation treatment is an achievable alternative to transperineal or transrectal US approaches. Larger studies can contribute to additional outcome data.
2125 Overcoming the Challenge of Full Thickness Resection of Gastric Lesions Using Colonic Full Thickness Resection Device (FTRD): Novel Technique
Authors: Fahmawi Yazan , Kumar Manoj , Mizrahi Meir
Published: October 2019

INTRODUCTION:

Colonic FTRD (over the scope full thickness resection device) is safe, feasible and approved for colorectal lesions. However, it’s role for upper gastrointestinal (UGI) lesions resection need to be further studied. Colonic FTRD system’s cap diameter is 21 mm which makes it very challenging to insert through the mouth for UGI lesions. We present a case of endoscopic full thickness resection (eFTR) of stomach lesion using colonic FTRD using a novel technique for upper esophagus dilation to assist in the insertion of colonic FTRD through the mouth.

CASE DESCRIPTION/METHODS:

Case Presentation A 49-year-old female was referred to us for endoscopic resection of submucosal lesion suspected for gastric stromal tumor (GIST) prior to bariatric gastric bypass surgery. EGD revealed the lesion in the gastric body. Endoscopic ultrasound (EUS) confirmed it as well demarcated hypoechoic lesion measuring 0.8 cm × 0.8 cm arising from the intersection of submucosal layer and muscularis layer without adjacent lymphadenopathy. FTRD under general anesthesia was planned. eFTR technique: To assist the insertion of colonic FTRD, upper esophagus was dilated with savory dilator size 51 French for 1 minute, then with 55 French for 1 minute and then with 60 French for 5 minutes. Pediatric colonoscope installed with FTRD was gently advanced into the stomach lumen. Circumferential marking of the lesion was done using FTRD marking probe. Favorable positioning was achieved. FTRD grasper was used to grasp and mobilize the tissue into the cap. Clip was applied and snare was hooked to high frequency generator performing high frequency snare resection. Endoscope was removed along with resection specimen. Histopathology revealed fibrotic nodule with complete resection (R0). Endoscopy at 3-month revealed no recurrence.

DISCUSSION:

UGI FTRD is not yet approved in the US. Our case illustrates that colonic FTRD can be used for UGI lesion. Our novel technique of serial esophageal dilation before insertion of larger size FTRD is unique. Similar technique can be used for insertion of future UGI FTRD system as well as its cap diameter is still larger at 19.5 mm which can still be challenging in patients with small upper GI caliber. Since muscularis propria was missing in resected tissue, it opens up a question if device is only capable of eFTR in lower GI tract (thin GI wall as compared to upper GI wall) or if it can achieve similar results with respect to true “full thickness resection” as eFTR in lower GI tract.
2132 Perivesicular Abscess Drainage With Axios Stent
Authors: Mohamed Abdalla , Cavanagh Yana , Estifan Elias , Grossman Matthew
Published: October 2019

INTRODUCTION:

Pelvic abscesses can occur as sequelae of surgery and are associated with significant morbidity and mortality. The treatment of choice for these abscesses is immediate initiation of broad-spectrum antibiotics and drainage of the collection. We propose that lumen-apposing self-expanding metal stents (LAMS) are a safe treatment option for drainage of pelvic abscesses. LAMS were originally designed for transmural pancreatic fluid collection drainage however have been successfully used for drainage of various extra-luminal fluid collections.

CASE DESCRIPTION/METHODS:

The patient is an obese 34-year-old female with a PMHx of IDDM, and HTN who had recently underwent ex-lap for a large non-malignant pelvic mass. On POD #5, the patient developed a fever and repeat CT revealed a multiloculated pelvic abscess measuring 5.8 × 4.2 cm. IR was unable to drain the collection in this location between bladder and rectum. Rectal EUS was performed and revealed a septated fluid collection viewed best using 7.5 mHz ultrasound at 10 cm from the anal verge. A 2 cm margin was observed between the abscess and the urinary bladder. A 2 mm margin was seen between the rectal wall and the abscess wall. A 19-gauge non-core needle was used to access the fluid collection which appeared purulent. A 15 mm AXIOS stent was then deployed using the same technique employed with pseudocyst drainage. Drainage was successful. 9 days after placement, the stent was removed using a cold snare. No bleeding resulted from stent pull. The defect was then clipped closed with a 15 mm over the scope clip. The patient tolerated the procedure well, experienced complete resolution of the abscess.

DISCUSSION:

Manvar et al suggest that EUS-guided LAMS drainage of pelvic abscesses is more efficacious and has a more favorable safety profile when compared to EUS-guided drainage with catheters and plastic stents. It is an efficient and safe alternative for the management of intra-abdominal and pelvic collections. This case demonstrated the novel use of a lumen-apposing self-expanding metal stent for peri-rectal abscess drainage. Although more evidence is needed for its off-label use, we conclude that it should be strongly considered as a viable treatment option if not the initial treatment modality for collections located in the pelvis.
2136 The Migrating and Perforating Biliary Stent
Authors: Bui Rosa , Sohail Muhammad Adnan , Ashley Christopher
Published: October 2019

INTRODUCTION:

Although there are high success rates with biliary endoprostheses, there is also accumulating evidence of short and long term complications including: sepsis, migration, perforation, fistula formation and bowel obstruction. This case report presents successful nonsurgical intervention of a rare but grave complication following biliary stent placement.

CASE DESCRIPTION/METHODS:

A 78 year old male with choledocholithiasis underwent an ERCP with incomplete stone removal, necessitating a 10Fr x 5 cm double-pigtail stent. Repeat ERCP at 6 weeks and an abdominal x-ray suggested distal migration of the stent. Despite multiple laxatives, a followup CT scan confirmed stent retention localized to the IC valve. Although he remained asymptomatic, colonoscopy was pursued and unexpectedly revealed one end of the stent penetrating terminal ileum mucosa, with the other end transecting the serosal surface and perforating through the cecum. The stent was removed with a snare and hemostatic clips were placed to close the defect. At 6 months, he continues to do well without any complications.

DISCUSSION:

Stent migration occurs in up to 10% of cases, with less than 1% risk of perforation. Short stents tend to travel proximally while longer stents tend to travel distally causing abdominal pain or bowel obstruction. Stent length (>7 cm), benign strictures, sphincter of Oddi dysfunction, post-sphincterotomy and papillary stenosis have all been associated with higher rates of distal migration. Most stents will pass uneventfully into excreta. Rarely, bowel perforation can occur. The duodenum is the most reported site of perforation due to its fixed setting. However, there have been close to a dozen cases of colonic perforation. Both stent design and patient’s anatomy can be obvious risk factors. Straight plastic stents have been associated with higher risk as compared to metal or pigtail stents. Anatomic aberrancies such as abdominal hernias, extensive adhesions and colonic diverticulae are associated with higher risk. Our case highlights bizarre stent migration/perforation with a theoretically lower risk stent, in an asymptomatic patient without known risk factors. Despite this precarious scenario, endoscopic intervention without surgery was sufficient. The collection of case reports in the literature, including our own, suggests the need for guidelines outlining appropriate measures (e.g. followup intervals for repeat imaging) and guidelines outlining management of biliary endoprostheses complications.