Clinical Challenges and Images in GI

Grace Elta and Robert J. Fantana, Section Editors

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A Woman Who Faints When She Eats and DrinksAndrea E. Wagner and David A. Katzka

Division of Gastroenterology The Mayo Clinic Rochester, Minnesota

Question: A 48-year-old woman presentedwith a 10-year history of intermittent liquiddysphagia that progressed to solid food dyspha-gia 2 years ago. Symptoms would occur severaltimes per week, last several seconds, and resolveabruptly. Patient reported symptoms were moresignificant with eating and drinking quickly oringesting large boluses. She reported 8 episodesof syncope associated with dysphagia over thepast 2 years. During an episode, she would ex-perience a sense of midsternal food sticking andlose consciousness within 5–10 seconds. Shedenied chest pain, convulsions, visual changes,or incontinence. She only reported syncope with

ysphagia, but had some minor orthostatic symptoms. She was taking loratadine (Claritin).Her physical examination was unremarkable with a resting heart rate of 61, blood pressure 105/77, and negative orthostatic blood

ressure measurements. A recent upper endoscopy with distal esophageal biopsies was normal. A treadmill stress test showed sinusradycardia with a ventricular rate of 43 and the resting electrocardiogram was normal. A transthoracic echocardiogram wasemarkable for mild left atrial enlargement. An autonomic reflex screen was negative. Esophageal evaluation included a normalarium esophagram including transit of a barium tablet and normal esophageal manometry. A Holter monitor was performedFigure A). With the monitor in place, upon ingesting a cold carbonated beverage, the patient developed presyncopal symptomsFigure A).

What is the diagnosis? How should the patient be managed?Look on page 780 for the answer and see the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information

on submitting your favorite image to Clinical Challenges and Images in GI.Conflicts of interest: The authors disclose no conflicts.© 2011 by the AGA Institute0016-5085/$36.00doi:10.1053/j.gastro.2011.02.068

Clinical Challenges and Images in GI

An Unusual Case of Peritonitis in an Intravenous Drug UserNeil Johns, David Cooper, and John Terrace

Department of Surgery, Queen Margaret Hospital, Fife, Scotland, United Kingdom

Question: A 24-year-old man was admitted complaining of acute abdominal pain of 48 hours’ duration. He also described chills,igors, and extreme nausea. He had no significant past medical history other than active intravenous drug use. He recalled injectingeroin in his groin 48 hours before admission. He denied having ever smoked the drug. He was fully conscious, had a pulse of 138eats per minute, blood pressure of 95/64, and temperature of 37.7°C. Positive abdominal findings were marked peritonitis andigidity. There was also soft tissue swelling of the right thigh. Peripheral blood showed: white cell count, 25.1; neutrophils, 21.34;

D– dimer, 4483; sodium,124;

ymphocytes, 2.44; hemoglobin, 20.1; prothrombin time, 19; activated partial thromboplastin time, 51;

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Clinical Challenges and Images in GI, continued

potassium, 6.2; urea, 9.0; cre-atinine, 249; amylase, 8; C-re-active protein, 46; and albu-min, 25. Abdominalcomputed tomography (Fig-ure A, arrow) showed markedsoft tissue thickeningthroughout the mesenteryand gross ascites. Celluliticchanges were noted in theright groin without localizedcollection (Figure B, arrow).Limited peritoneal fluid wasobtained via ultrasound guid-ance owing to its viscous con-sistency. At laparotomy, therewas copious, clear, thick, mu-cus-like fluid within the ab-dominal wall. The same wasencountered on opening theperitoneum, reminiscent ofpseudomyxoma peritonei. Thesmall bowel looked normal,but small and large bowel mes-enteries were markedly edema-tous and full of similar mucus(Figure C, arrow).

What is the diagnosis?Look on page 780 for the answer and see the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on

ubmitting your favorite image to Clinical Challenges and Images in GI.Acknowledgements: The authors thank Mr Symon Macpherson, Dr Ben Shippey, and Dr Craig Ferguson for their help, support, and guidance in theproduction of this case report.Conflicts of interest: The authors disclose no conflicts.© 2011 by the AGA Institute0016-5085/$36.00doi:10.1053/j.gastro.2011.02.076

Clinical Challenges and Images in GI

Polypoid Rectal Mass in a 21-Year-Old Woman With HematocheziaRajiv Sharma and Benedict Maliakkal

University of Rochester Medical Center, Department of Gastroenterology and Hepatology, Rochester, New York

Question: A 21-year-old woman presented to the emergency room with dizziness, left lower quadrant abdominal pain, andhematochezia for 3 months. Laboratory tests showed iron-deficiency anemia. Physical examination was benign except for mildtenderness in left lower quadrant. Esophagogastroduodenoscopy was normal. Colonoscopy showed 5 � 3 cm-pedunculated,polypoid mass 15-cm from the anal verge (Figure A). Magnetic resonance imaging of the pelvis (Figure B) showed that the mass waslimited to the rectum without any pelvic or ovarian connections. Pathology slides are shown (Figure C–D).

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Answers to the Clinical Chal-lenges and Images in GI Ques-tion: Image 1 (page 435): SwallowSyncopeA Holter monitor (Figure B) revealed short epi-sodes of high-grade second degree atrioventricu-lar block characterized by isolated “p” waves(arrows) with absent “QRS” complexes whiledrinking the cold carbonated beverage and thiswas associated with presyncopal symptoms. Thediagnosis of swallow syncope was made and thepatient was referred to cardiology and under-went placement of a permanent pacemaker. Fol-low-up several months later revealed no subsequent episodes.

The pathophysiology of swallow syncope is not entirely known. These events have been categorized as reflex-mediated syncopalepisodes and result in temporary cerebral hypoperfusion. They are presumed to result from a dysfunction of the afferent– efferentreflex arc. The afferent neural impulses from the upper gastrointestinal tract lead to increased vagal tone and the efferent portionextends from the vagus to the cardiac plexus, where the sinoatrial node and atrioventricular node are innervated, thus allowing forslowed or blocked conduction.1,2 A review of the literature in 2006 described 63 case reports of swallow syncope, of which 24 hadno underlying esophageal disease to explain the events. However, esophageal spasm, malignancy, diverticulum, stricture, large hiatalhernia, and gastroesophageal reflux disease have been reported as underlying diseases in individuals with swallow syncope. It occurspredominantly in men.3 Patient signs and symptoms include lightheadedness, dizziness, or passing out with swallowing. Usually,symptoms occur quickly after swallowing without warning. The temperature and consistency of the precipitating food or liquidbolus can vary.2 Diagnosis should include a complete evaluation of the esophagus, including an esophagram. A cardiovascularexamination including an electrocardiogram, Holter monitor, and echocardiogram are used to rule out a cardiovascular cause forsyncope. To evaluate for autonomic disorders, a head-up tilt test and carotid sinus massage can be completed. Treatment of anyunderlying disease is important; however, in the absence of a clearly identifiable esophageal cause, permanent pacemaker placementis effective. Review of the literature found this to be the most common treatment.3

References1. Calkins H, Zipes DP. Hypotension and syncope. In: Libby P, Bonow RO, Mann DL, et al, eds. Braunwald’s heart disease: a

textbook of cardiovascular medicine. 8th ed. St Louis: W. B. Saunders Company, 2007.2. Cherukuri S, Gardner G. Deglutition syncope. Otolaryngol Head Neck Surg 2004;130:145–147.3. Omi W, Murata Y, Yaegashi T, et al. Swallow syncope, a case report and review of the literature. Cardiology 2006;105:75–79.

For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).

Answer to the Clinical Challenges and Images in GIQuestion: Image 2 (page 435): Primary Anthrax Perito-nitisPeritoneal fluid exhibited large numbers of pus cells and large Gram-positivebacilli. Culture on blood agar revealed typical soft peak nonhemolytic medusahead colonies, confirmed as Bacillus anthracis by the Specialist Pathogens Refer-ence Unit (Figure D). The patient was treated postoperatively in the intensive careunit with intravenous anthrax immune globulin according to Centers for DiseaseControl and Prevention (CDC) protocol. On day 4 he underwent relaparotomyand exploration of thigh wound that showed no evidence of tissue to debride. Onday 7, he died of multiple organ failure.

Anthrax is a rod-shaped, Gram-positive, aerobic bacterium, primarily infectingwild and domesticated herbivorous mammals.1 These animals ingest or inhalethe soilborne spores of B anthracis while grazing, allowing entry into the gastrointestinal tract.2,3 Principally a disease of this group,

nthrax also has the ability to affect humans.2,3 The modes of transmission have been shown to be by direct injection, inhalation,nd cutaneous inoculation of the spores.2,3 Anthrax has a variable clinical presentation depending on site of entry. To date, thereave been a growing number of cases arising in intravenous drug users, especially in Scotland.

We believe we have described the first case of injection-related anthrax leading to anthrax peritonitis resulting in septic shock. Thisase is unusual in several respects. The patient presented with an acute abdomen and then went on to develop systemic involvement.

few similar cases have been reported in the literature1 and these conclude that the source of the gastrointestinal anthrax was from

he ingestion of contaminated meat.1 Our hypothe sis is that anthrax spores were in troduced into the bloodstream after injection

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of contaminated heroin. It is possible that there may have been direct seeding of the peritoneal cavity owing to the patient directlyinjecting it via the groin. It is unlikely it could have been inhaled because there was no history consistent with heroin smoking.

Anthrax infection is not a common differential diagnosis for an acute abdomen in a young male. It is imperative that suspectedcases are discussed with microbiology/infectious disease colleagues as soon as possible to ensure appropriate samples such as tissuebiopsy, wound or sterile site fluid aspirates are sent immediately for Gram-staining, culture, and polymerase chain reaction testing.Early appropriate antibiotics are paramount.

References1. Kanafani ZA, Ghossain A, Sharara AI, et al. Endemic gastrointestinal anthrax in 1960s Lebanon: clinical manifestations and

surgical findings. Emerg Infect Dis J 2003;9:520 –525.2. Ramsay CN, Stirling A, Smith J, et al. An outbreak of infection with Bacillus anthracis in injecting drug users in Scotland. Rapid

ommunications Eurosurveillance 2010;15:14.3. Booth MG, Hood J, Brooks TJ, et al; Health Protection Scotland Anthrax Clinical Network. Anthrax infection in drug users.

ancet 2010;375:1345–1346.

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Answer to the Clinical Challenges and Images in GIQuestion: Image 3 (page 436): Benign Primary RectalTeratomaA colorectal surgeon was consulted. The mass was partially resected by flexiblesigmoidoscopy. The remainder of the mass was removed with transanal micro-scopic surgery (TEMS). Figure A Shows the teratoma with protruding hair. FigureC shows hyaline cartilage (asterisk), respiratory epithelium (arrow). Figure D showskin and appendages. There were no malignant elements. Patient has beensymptomatic on follow-up.

Teratomas are germ cell tumors that arise from totipotential cells and areommonly composed of multiple cell types derived from �1 of the 3 germ layers:ctoderm, mesoderm, and endoderm. Teratomas range from benign, well-differ-ntiated mature lesions that are cystic, to immature malignant solid tumors.

ature teratomas may contain, hair, teeth, fat, skin, muscle, neural, and endo-rine tissue, but most commonly squamous tissue, and may undergo malignantransformation.1

It is hypothesized that primordial germ cells enter the rectum as they migratefrom the dorsal mesentery of the hindgut through the mesenchyme toward thegonadal ridge. Extragonadal teratoma may involve the retroperitoneum, medias-tinum, and the sacrococcygeal region. Teratoma of the gastrointestinal tract israre and few cases arising from the rectum have so far been reported.1,2

When a teratoma is found in the rectum, it is important to differentiatebetween a primary rectal teratoma and a teratoma arising primarily from anadjacent organ and eroding into the rectal lumen. A rectal teratoma should bethoroughly worked up by imaging to rule out an ovarian source before calling itprimary.2

Most teratomas are pedunculated and benign, and therefore amenable toendoscopic removal. TEMS is particularly advantageous for lesions of the middleand upper rectum when transanal excision is difficult and may be difficult to reach with conventional instrumentation and offersa preferable alternative to transabdominal surgery.3

References1. Terada T. Mature teratoma of the rectum. Gastrointestinal Endosc 2010;71:1068 –1069.2. Basu S, Kumar M, Shukla VK. Ovarian dermoid eroding into rectum and masquerading as recurrent rectal teratoma. Open

Access Surgery 2010;3:5–7.3. Zacharakis E, Freilich S, Rekhraj S, et al. Transanal endoscopic microsurgery for rectal tumors: the St. Mary’s experience. Am J

Surg 2007;194:694 – 698.

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