Charles Lombard

An elusive cause of severe gastrointestinal bleeding.

A 53-year old Filipino-American man with a history of recurrent episodes of rectal bleeding was initially evaluated in the emergency department with chief complaint of painless passage of dark red blood per rectum. He was in good health until 4 months prior to admission, at which time he was evaluated in the emergency room for hematochezia and hemoglobin of 7.5 g/dl. His past history was otherwise significant for hyperlipidemia and hypertension. He had an appendectomy 10 years prior to admission. He had been a smoker and alcoholic but had quit both 10 years previously. He worked as a caregiver in an assisted living facility. Endoscopy revealed long-segment Barrett’s esophagus and a small hiatal hernia but no active bleeding. Although fresh blood was noted throughout the colon and terminal ileum by colonoscopy, no active source of bleeding was identified. Gastrointestinal bleeding continued and was treated with intravenous fluids and 10 units of packed red cells. A second colonoscopy performed for persistent bleeding did not reveal any blood in the colon. A CT scan, Meckel’s scan (Fig. 1), and an abdominal angiogram, were all negative. Eventually his bleeding stopped, he became hemodynamically stable and was discharged. A week later, capsule endoscopy was normal except for delayed passage into the cecum. Despite such an extensive evaluation, the cause of this massive GI bleed remained elusive. Two months after the initial episode, he was re-evaluated in the emergency department for an abrupt episode of bloody diarrhea and hemoglobin of 8.4 g/dl. He required intravenous fluids and blood resuscitation with proton pump inhibition. The bleeding stopped spontaneously with hemoglobin stabilizing at 9.7 g/dl. Endoscopy again showed long-segment Barrett’s esophagus. A trans-anal double balloon enteroscopy revealed old blood in the distal 100 cm of ileum but no active lesions in the ileum or colon. He was discharged without a definitive diagnosis. He was doing well until one day prior to the current admission when he had a sudden onset of diarrhea along with dark bleeding per rectum. He denied any abdominal pain, nausea or vomiting, weight loss, or changes in appetite. On examination, he appeared anicteric and afebrile but in mild distress. His pulse was 132/min, respiration 20/min, blood pressure 120/83 mmHg without orthostatic signs; oxygen saturation by peripheral pulse oximetry was 100 % while breathing room air. Physical examination revealed a soft, non-tender abdomen with positive bowel sounds. There was no organomegaly or masses. Hemoglobin was 8.3 g/dl; comprehensive metabolic panel was normal. He was stabilized with intravenous fluids and two units of packed RBCs. Urgent abdominal multidetector CT angiography revealed active hemorrhage from a small bowel loop in the left lower quadrant (Fig. 2). Because repeat angiography was negative, no radiological intervention was possible. Because of the inconclusive evaluation up to that point he underwent laparoscopic exploration. At laparoscopy, adhesions were found in the right lower quadrant at the site of his previous open appendectomy. A Meckel’s diverticulum was identified in the jejunum S. Kommareddy A. K. Roorda P. Legha C. Lombard G. Triadafilopoulos El Camino Hospital, Mountain View, CA, USA

Multiple Rectal Polyps in a Young Woman with Constipation

A 27-year-old Asian-American woman presented for evaluation of long-standing constipation. Her first severe episode of constipation occurred at age 7 and resolved with colonic lavage. Colonoscopy at that time was normal. She then experienced complete resolution of her symptoms from ages 7 to 17, when she started again experiencing defecatory difficulties with infrequent and hard stools, difficulties with evacuation, and a sensation of incomplete rectal emptying. On presentation in July 2009, she was dependent on two Senna pills per day; without them, she would not have a bowel movement for up to 1 week. She also reported episodes of rectal bleeding or blood in toilet paper, and abdominal bloating and pain that were relieved with bowel evacuation, as well as continuous and severe straining at evacuation with a feeling of incomplete evacuation. She did not, however, use finger manipulations to facilitate defecation. Her past medical and surgical history was negative and she was using no other medications except for oral contraceptives and multivitamins. She was single, worked as a program manager, lived with her boyfriend, and exercised 3–4 times/week. She did not smoke cigarettes, and drank wine socially. She was vaccinated against human papillomavirus (HPV). Family history was significant for her mother having lung cancer and her father with diabetes mellitus; she had one healthy sister. Upon further review of systems, she reported 8 lbs of weight gain, general fatigue, headaches, and mild anxiety. Her physical examination was normal except for small external hemorrhoids. Complete blood count, comprehensive chemistry panel, and thyroid function tests were all normal. On initial assessment of her constipation, a lactulose breath test was negative. A colon transit study using Sitzmarks was normal. An anorectal motility and compliance study showed a normal anal sphincter length of 5 cm, a normal internal anal sphincter pressure of 72 mmHg, and a slightly decreased external anal sphincter pressure of 91 mmHg. Rectal sensation was impaired since she barely sensed a rectal balloon filled with 50 cc of water. The anorectal inhibitory reflex was present and elicited with 30 cc of balloon distention, thereby excluding Hirschsprung’s disease. Rectal compliance was normal, but she was unable to expel an empty rectal balloon, which was suggestive of pelvic floor dysfunction. Colonoscopy revealed multiple inflammatory polyps in the rectal vault extending circumferentially to the anorectal junction. Biopsies of the polyps were performed and were associated with significant oozing that required control with application of hemostatic clips (Fig. 1). Pathologic examination of the polyp biopsies revealed six separate fragments of rectal mucosa. Five of these fragments showed polypoid hyperplastic mucosa with areas of ulceration, consistent with rectal prolapse. One of these fragments showed an area of moderate squamous epithelial dysplasia (AIN 2). No evidence of an invasive lesion was identified (Fig. 2). Despite her previous vaccination history, HPV immunohistochemistry was positive. She was then treated with polyethylene glycol twice daily for the next 3 months with some improvement of her G. Triadafilopoulos (&) Division of Gastroenterology and Hepatology, Stanford University Medical Center, Alway Building, Room M 211, 300 Pasteur Drive, MC: 5187, Stanford, CA 94305-5187, USA e-mail: vagt@stanford.edu

Gastritis Cystica Polyposa: A Rare Cause of Abdominal Pain and Early Satiety Treated with Endoscopic Resection

A 51-year-old Korean–American woman with a history of untreated Helicobacter (H.) pylori infection was referred for an evaluation of left upper quadrant abdominal pain and early satiety. She denied associated symptoms, such as nausea, vomiting, or a change in bowel habits. She had experienced left lower quadrant pain in the past but more recently had been having left upper quadrant discomfort with fullness and early satiety. She denied the use of tobacco or alcohol and had no family history of gastric malignancy. She was diagnosed with mild iron-deficiency anemia (serum hemoglobin: 11.2 g/dl, ferritin: 11 ng/ml, negative fecal occult blood test) approximately 1 year prior. A subsequent colonoscopy was normal. She did not take iron supplements. A follow-up CBC revealed hemoglobin of 12 g/dl and hematocrit of 36.9%. A computed tomography (CT) scan with contrast performed for an evaluation of abdominal pain revealed a 2.3-cm polypoid intraluminal gastric mass arising from the greater curvature of the proximal stomach (Fig. 1a, b). An upper endoscopy was requested to further evaluate the mass lesion and to exclude possible malignancy. Endoscopic examination identified a 3 × 3 cm partly pedunculated polypoid lesion with normal overlying mucosa in the gastric fundus, approximately 3 cm from the gastroesophageal junction (Fig. 2a). Endoscopic ultrasound examination revealed a hypoechoic submucosal mass without evidence of deeper invasion into the muscularis propria (Fig. 2b). The base of the lesion was injected with 5 ml of 1:10,000 epinephrine enabling complete resection using snare electrocautery (Fig. 3b). A non-bleeding submucosal vessel was seen in the mucosal defect and was closed with endoclips (Fig. 3a). Histopathology of the polypoid mass revealed large dilated epithelial spaces with proliferating small antral-type glands; microscopic examination revealed a disorderly array of smooth muscle with chronic inflammation (Fig. 4a), consistent with gastritis cystica polyposa. Evidence of H. pylori infection was also noted (Fig. 4b). The patient was seen for a follow-up visit 3 weeks later reporting no complications. Importantly, she noted complete recovery from her left-sided abdominal pain and fullness and remains asymptomatic. A follow-up endoscopy performed 3 months later revealed a well-healed scar at the site of resection; endoscopic biopsies showed no recurrence of the lesion.

Sun Should Not Rise and Set on a Case of Acute Intestinal Obstruction

A 45-year-old woman with a history of total proctocolectomy and end ileostomy for perforated ulcerative colitis 10 years before was evaluated in the Emergency Department for a 10-h history of abdominal pain, lack of ileostomy output, and severe, recalcitrant nausea and vomiting. Her pain was of sudden onset, moderate to severe in intensity, periumbilical, non-radiating, and associated with progressive abdominal distention. She reported several similar episodes of pain and vomiting that had been selflimited over the past several months and for which she had sought no medical attention. She denied any other symptoms. She did not take any medications and denied drug, tobacco, or alcohol use. On examination, she was afebrile with normal vital signs. Cardiopulmonary examination was normal. Her abdomen was distended, with a well-healed mid-abdominal incision, diffusely tender, with active bowel sounds, but without organomegaly or masses. Her ileostomy bag was empty, and the right lower quadrant stoma was patent to finger examination. Her total peripheral leukocyte count (WBC) was 12.1 with neutrophilic pleocytosis; hemoglobin was 12.7. Comprehensive chemistry panel was normal. A plain abdominal film was unremarkable (Fig. 1). She refused a computed tomography (CT) scan due to fear of radiation exposure. Because of ongoing symptoms, she was admitted to the inpatient medicine service for observation, where she received with intravenous (IV) fluids, nasogastric tube decompression, narcotic analgesics, and anti-emetics. Eight hours later, she reported no improvement, with constant and severe abdominal pain, distention, and tenderness. Although she refused further studies, several hours later a CT of abdomen and pelvis with intravenous contrast revealed small bowel dilation measuring up to 4.5 cm in diameter (Fig. 2a) with mesenteric edema, free fluid, and many small bowel loops exhibiting wall thickening. Dilated loops converged with abrupt transitions in the location of the mesentery (Fig. 2b). There was evidence of a previous colectomy with a right lower abdominal enterostomy and decompression of the distal small bowel. These findings were compatible with a high-grade small bowel closed-loop obstruction possibly due to internal herniation. Importantly, the areas of small bowel wall thickening raised concern for ischemia (Fig. 3). A calcified gallstone was also seen, without features of cholecystitis. Surgical evaluation—by now 24 h after the onset of symptoms— revealed hypotension (BP: 99/50) without tachycardia or fever. There was marked abdominal tenderness, guarding and rebound in both lower quadrants, suggestive of peritonitis. An emergency laparotomy revealed an ischemic small bowel segment secondary to internal herniation from band adhesions causing closed-loop obstruction and hemorrhagic ascites. After lysis of adhesions, a segmental bowel resection was performed, followed by primary jejuno& George Triadafilopoulos vagt@stanford.edu

When It Rains It Pours: Evolving, Complicated Small Intestinal Crohn’s Disease

A 44-year old white male was evaluated in the Emergency Department in September 2010 due to mid-abdominal pain of several days’ duration associated with abdominal distention, bloating, nausea, vomiting, and mild fever. He related a history of untreated Crohn’s disease (CD) for 10 years that was minimally symptomatic except for occasional episodes of non-bloody diarrhea and intermittent right lower quadrant abdominal discomfort that required no medical therapy. An abdominal computed tomography (CT) scan (Fig. 1) revealed terminal ileitis with intestinal obstruction. There was no family history of inflammatory bowel disease or malignancy. Examination of the abdomen revealed no palpable masses, organomegaly or tenderness and bowel sounds were normoactive. He was admitted to the hospital, quickly responding to antiemetics, nasogastric tube decompression, intravenous fluids, and corticosteroids. A tapering prednisone regimen was given along with oral 6-mercaptopurine (MP). A subsequent abdominal CT with oral contrast showed a ‘‘chain-of-lakes’’ appearance of the terminal ileum, dilated small bowel, and partial intestinal obstruction due to CD. In November 2010, an ileo-colonoscopy revealed terminal ileitis with stricture formation that was balloon dilated to 12 mm without complications (Fig. 2). The colon was macroscopically normal. Biopsies revealed terminal ileal inflammation with hyperplastic changes (Fig. 3). Colonic biopsies were normal. The patient improved prednisone was discontinued, and he was maintained on oral 6-MP 75 mg/day. In January 2011, he was maintaining his weight and appetite, only reporting rare mild non-bloody diarrhea and right lower quadrant discomfort, unrelated to meals. On follow-up examination in July 2012, his body mass index was 30, his blood pressure 145/97, and the patient complained of stress-related diarrhea and mild cramps. Abdominal examination was significant for mild right lower quadrant fullness. He decided to lose weight and to not proceed with a repeat ileo-colonoscopy or switch to biologic therapy for CD given his clinical response and the related risks. In July 2013, he was well but reported a self-limited episode of nausea and vomiting. He was then lost to follow-up. In September 2015, he was evaluated in the Emergency Department again with clinical features of partial small bowel obstruction, confirmed by abdominal CT with oral contrast (Fig. 4). Since the episode resolved quickly and he was generally well, he decided against repeat endoscopic dilation and only continued 6-MP 100 mg/day. Nevertheless, in January 2016, he returned with abdominal pain and nausea, but this time was noted to have an erythematous skin discoloration acme to the left of the umbilicus with an underlying tender mass. CT of the abdomen revealed a & George Triadafilopoulos vagt@stanford.edu

Double-Edged Sword: Esophageal and Colonic Crohn's Disease.

A 50-year-old woman of northern European ancestry diagnosed with ulcerative colitis (UC) 15 years previous was admitted to the hospital due to a 2-week history of severe dysphagia, odynophagia, and cramping, diffuse abdominal pain associated with diarrhea. She described painful swallowing, mostly to solids but occasionally to liquids, centered in the upper mid-chest that felt like ‘‘swallowing swords.’’ She limited her intake only to liquids and broth; for 2 days before admission, she was unable to tolerate anything by mouth, including her own saliva. In conjunction with her esophageal symptoms, she noted cramp-like, diffuse, moderately severe abdominal pain, worsening watery, non-bloody diarrhea occurring up to six times per day and at night, urgency, and ‘‘shearing’’ anal pain with defecation. She denied any fever or chills, but reported a 7-kg weight loss during the past month. Although her primary physician initiated treatment with ciprofloxacin and metronidazole for suspected worsening colitis and referred her to a gastroenterologist, given the intensity of her odynophagia, she visited the Emergency Department for further treatment. Her past medical history was significant for the diagnosis of UC that had been until recently maintained in remission with the use of oral mesalamine, 1.2 g per day. She also had a history of heartburn, treated with pantoprazole, and seizure disorder that had been inactive without therapy for several years. Her last colonoscopy performed 6 years prior to this episode was interpreted as showing mild UC. Upon admission to the hospital, she was afebrile, tachycardic to 112 per min, with normal blood pressure and respirations. Examination was significant only for diffuse abdominal tenderness, hyperactive bowel sounds, and brown occult blood-positive stool. She had no oral or perianal abnormalities. Admission laboratory examination included WBC = 10.8, Hgb = 11.6, and platelets = 502; a comprehensive chemistry panel was normal. Urinalysis was notable for bacteriuria and pyuria, suggestive of a urinary tract infection. Tests for tuberculosis and human immunodeficiency virus (HIV) infection were negative. A chest X-ray was normal. A computed tomography (CT) scan of the abdomen and pelvis revealed colonic wall thickening, mostly in the splenic flexure and sigmoid colon, associated with numerous, mildly enlarged mesenteric and peri-aortic lymph nodes and multiple small liver cysts (Fig. 1). An urgent upper endoscopy showed a deep, linear, 3-cm-long non-bleeding ulcer in the proximal esophagus (Fig. 2); the remaining upper endoscopic examination was normal. Colonoscopy including terminal ileoscopy revealed a normal terminal ileum but extensive inflammation, nodularity, ulcerations, and friability of the colon, starting at the level of the rectosigmoid and extending in a scattered fashion to the hepatic flexure, with the most severe inflammatory changes in the distal transverse and descending colon (Fig. 3). Histologic examination of the biopsies revealed ulcerative esophagitis (Fig. 4) and severe colitis without dysplasia starting at the hepatic flexure and extending distally with microgranulomas at the splenic flexure (Fig. 4). The terminal ileum and cecum were histologically normal. Stool cultures were negative for bacterial, & George Triadafilopoulos vagt@stanford.edu

“War and Peace” with Barrett’s Esophagus

Although the patient recalls heartburn and acid indigestion since his teenage years, he experiences increasingly frequent belching, heartburn and acid regurgitation. He reports no dysphagia, gastrointestinal bleeding or weight loss. He also reports longstanding symptoms of irritable bowel syndrome with erratic bowels, mostly constipation and bloating, which is treated with fiber and a healthy diet. A partial thyroidectomy is performed for a thyroid nodule, but there is no thyroid cancer and replacement therapy is not needed. He remains very active, athletic, and otherwise healthy, and is followed carefully by his internist. He is not a smoker, a social alcohol user, or a coffee drinker. Initially he uses antacids as needed, then H2 receptor antagonists (H2RAs) and eventually is started on proton pump-inhibitor (PPI) therapy. His physical examination is normal. By the end of the decade, his symptoms occur daily and disturb his sleep. Given the chronicity, frequency, and increasing severity of his reflux symptoms, an endoscopy is performed. There is no esophagitis, but a C2,M3 (Prague C and M Classification) Barrett’s esophagus, 3 cm sliding hiatal hernia, and normal stomach and duodenum are found. There is no dysplasia.