Congenital intrahepatic shunt. a case report and a brief review of literature

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ination was normal except slight swelling and discomfort of the involved joints.Standard tests for rheumatological disorders were negative, initially and subsequently. Serological tests for syphilis, HIV, hepatitis A, hepatitis B and cryoglobulinemia were also negative initially and remained negative throughout the period of follow– up. Available tests for other liver diseases were also negative. Complete blood picture and liver chemistry were normal initially and at all subsequent follow– up visits. Hepatitis C virus antibody test was positive and quantitative estimation of hepatitis viral RNA revealed 550,000 viral equivalents / mL. Patient declined liver biopsy, but insisted for anti–HCV therapy. He was treated with interferon alpha 2b, 300 million units three times weekly, and responded by complete and sustained relief of joint symptoms. Hepatitis C viral RNA levels progressively decreased and were undetectable after six month of therapy and one year after stopping the therapy. HCV arthropathy should be considered in the differential diagnosis of polyarthritis of undetermined etiology, even in absence of clinical and / or biochemical evidence of liver disease.

AJG – Vol. 97, No. 9, Suppl., 2002

Introduction: Congenital intrahepatic porto–systemic shunt is a rare cause of hepatic encephalopathy in the absence of cirrhosis. However unlike other forms of congenital disease, it presents most commonly in the middle and the older population of patients. Case: We are presenting a case of a 70 year old lady who presented with multiple episodes of confusion and gait disturbence with negative neurological work up. She was subsequently found to have on Doppler ultrasound examination (Fig 1A&1B) a large intrahepatic shunt which was later confirmed on hepatic portocavogram to be between the middle portal vein and right hepatic vein (Fig 2).In addition she was also found to have a cholecystogastric fistula secondary to recurrent cholecystitis.

419 ZOLLINGER–ELLISON PHENOTYPE IN THE ABSENCE OF GASTRINOMA AND HYPERGASTRINEMIA Anish A. Desai, M.D., James E. McGuigan, M.D. and Peter Draganov, M.D.*. Department of Medicine, University of Florida, Gainesville, FL. Background: The Zollinger–Ellison syndrome is characterized by greatly increased gastric acid secretion, ulcer disease and gastrin–releasing islet cell tumors (gastrinomas). Patients with this syndrome frequently, but not invariably, have multiple ulcers, often beyond the first portion of the duodenum, and at least 30% have acid–induced diarrhea. Case report: A 48 year– old white male presented with abdominal pain, nausea, vomiting, and 3– 4 years of diarrhea. His pain was epigastric, dull, constant and radiated to his back. The diarrhea was profuse and watery. He had been treated for H. pylori in the past. Although lansoprazole (30 mg/day) provided some relief, his symptoms continued to prevent him from working. Serum amylase, lipase, LFTs, electrolytes, CBC, and stool studies were normal. CT with thin cuts of the pancreas did not detect tumor in the pancreas or elsewhere in the abdomen. EGD showed prominent gastric folds as well as multiple erosions and ulcers in the first and second portions of the duodenum. Gastric biopsies demonstrated parietal cell hyperplasia, without evidence of H. pylori. Fasting serum gastrin was normal (37 pg/ml). Basal acid output was markedly elevated at 50 meq/hr (nl males 0.1–3.8 meq/hr). Endoscopic ultrasound failed to demonstrate tumor in the pancreas or in adjoining structures. Octreotide scintigraphy was negative for gastrinoma. An intravenous secretin stimulation test showed no significant stimulation of gastrin release which is not consistent with gastrinoma. The patient was treated with pantoprazole (120 mg/day) with clinical improvement. Discussion: A small number of cases of gastric acid hypersecretion with non– gastrin secreting islet cell tumors have been described in the literature. A yet to be isolated, non– gastrin peptide from these tumors was shown to stimulate gastric acid secretion. The above case is unique in that this patient demonstrated consequences of striking gastric acid hypersecretion without elevated serum gastrin or evidence of an islet cell tumor. Possible explanations include a non– gastrin acid stimulant released from an unidentified tumor. Alternatively, the massive gastric acid hypersecretion and clinical manifestations characteristic for the Zollinger–Ellison syndrome may be consequent to as yet undefined dysregulation of gastric acid secretion. This case demonstrates the importance of recognition that classical manifestations of the Zollinger–Ellison syndrome can occur in the absence of gastrinoma. 420 CONGENITAL INTRAHEPATIC SHUNT. A CASE REPORT AND A BRIEF REVIEW OF LITERATURE Sreenu Ada, M.D.*, Jayanta Choudhury, M.D. and David Talenti, M.D. Medicine, Guthrie Clinic, Sayre, PA; Medicine, Guthrie Clinic, Sayre, PA and Gastroenterology, Guthrie Clinic, Sayre, PA.

Fig 1a

Fig 1b

Fig 2 Discussion: This form of shunt in absence of liver disease is thought to be a high flow communication between omphalo–mesenteric venous system and right horn of sinus venosus secondary to absence of sinusoid formation. The other reported causes of this form of shunt are hepatic trauma and secondary to a liver biopsy. The reason for this anomaly to present in middle or older age groups is thought to be at least in part due to higher sensitivity of the brain in the older age group to hepatic metabolites. Angiography is the gold standard for confirming the diagnosis although the initial test of choice is doppler ultrasonography and in some cases computed tomography. The management involves closure of the shunt either percutaneously with steel coil embolization or direct surgical ligation. Conclusion: Although congenital intrahepatic shunt is a rare cause of hepatic encephalopathy, it is a potentially correctable cause in selected

AJG – September, Suppl., 2002

cases.This should always be considered in the list of diagnostic possibilities in elderly patients with recurrent attacks of confusion.

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423 CHRONIC APPENDICITIS PRESENTING AS CROHNS DISEASE Mike L. Anderson, M.D. and Fernando Ramos, M.D.*. Gastroenterology, Tripler Army Medical Center, Honolulu, HI.

421 OCCULT GASTROINTESTINAL INFECTION MIMICKING ADULT–ONSET STILL’S DISEASE Olaitan A. Adeniji, M.D. and Jack A. Di Palma, M.D.*. Division of Gstroenterology, University of South Alabama College of Medicine, Mobile, AL. Purpose: The diagnosis of adult– onset Still’s disease is based on clinical criteria. Exclusion of other conditions is essential for accurate diagnosis. This case illustrates the need for systematic evaluation for infection, including the gastrointestinal tract. Case Report: A 56 –year– old white male had one–year history of recurrent fevers, arthritis, myalgias, rash, diaphoresis and headaches. He specifically reported no episode of abdominal pain, diarrhea or hematochezia. Infectious disease and rheumatologic evaluation led to the diagnosis of adult– onset Still’s disease. He was placed on prednisone, methotrexate, chloroquine, azulfidine and rofecoxib without much response. The patient subsequently developed pneumaturia and fecaluria. Endoscopic and radiologic imaging revealed colovesical fistula and abscess with probable diverticular source. A quinolone and metronidazole was started with prompt improvement however fecaluria recurred two weeks later. The patient underwent sigmoid colectomy and closure of colovesical fistula. Pathologic specimens confirmed diverticulitis and marked acute inflammation with granulation tissue and foreign body–type giant cell reaction to fecal material of plant origin. All anti–inflammatory drugs were discontinued and the patient had complete resolution of his symptoms. He continues to be asymptomatic one year after the surgery. Conclusion: Abdominal imaging should be considered when evaluating patients with suspected Still’s disease, even if symptoms referable to the digestive system are lacking.

Purpose: Recurrent and chronic appendicitis are often overlooked in the differential diagnosis of chronic abdominal pain. The symptoms described in these entities overlap with numerous other more common conditions resulting in frequent misdiagnosis. Colonoscopy provided a diagnosis in this case which was not considered after CT evaluation. Despite an accuracy described as near 98%1, CT may result in misdiagnosis if not followed by colonoscopy. Case Presentation: This is a 40 year– old male with a 17 year history of recurrent abdominal pain accompanied by diarrhea and anorexia lasting 3 to 4 days occurring every 2 months on average. The patient presented after 24 hours of his usual abdominal pain with a new component of sharp right upper quadrant pain. He denied any history of hematochezia or melena. Previous workup included included flexible sigmoidoscopy and HIDA scan which were normal. Physical exam showed fever with tenderness to palpation in the lower abdomen. Labs revealed a white blood cell count of 10.6, and hemoglobin of 12.8. Right upper quadrant ultrasound was read as normal. Acute abdominal series showed diffuse small bowel dilation to 4cm with several areas of air–fluid levels. Abdominal/pelvis CT scan showed thickening of terminal ileum with fat stranding and multiple fluid collections. The patient responded well to a course of methylprednisolone (40mg IV Q6 hrs). Colonoscopy three days later revealed a bulging appendix with pus at the orifice. Given this finding the patient was diagnosed with chronic appendicitis. Conclusions: Our submission represents a case of chronic appendicitis with symptoms and CT findings mimicking Crohn’s disease. 1. Rao PM, Rhea JT, Novelline RA, et al. Helical CT technique for the diagnosis of appendicitis: Prospective evaluation of a focused appendix CT examination. Radiology 1997; 202(1): 139 – 44.

422 INFARCTED APPENDIX EPIPLOICA CLINICALLY MIMICKING ACUTE APPENDICITIS Olaitan A. Adeniji, M.D. and Jorge L. Herrera, M.D.*. Division of Gastroenterology, University of South Alabama College of Medicine, Mobile, AL. Purpose: Acute appendicitis is the most common cause of right lower quadrant pain in men. We present a case of infarcted ascending colon epiploic appendix presenting clinically as acute appendicitis. Case Report: A 36 –year– old white male presented to the emergency department with one– day history of severe right lower quadrant pain. The pain was crampy, continuous and associated with nausea and anorexia. He also reported episodes of chills and generalized malaise. He had no vomiting or diarrhea. His physical examination was normal except a soft non– distended abdomen with tenderness and guarding in the right lower quadrant (McBurney’s point). His rectal examination was normal. Laboratory results revealed an elevated white blood cell count (11.5 cells/mL), normal hemoglobin and normal serum electrolytes. An abdominal plain radiograph was also normal. The patient was clinically diagnosed as acute appendicitis and a laparotomy revealed normal appearing appendix. Further exploration of the abdomen revealed an infarcted epiploic appendix of the ascending colon. The epiploic appendix was resected and the patient had a stable postoperative period with resolution of his symptoms. Conclusion: Infarcted appendix epiploica should be considered in patients presenting with symptoms of acute appendicitis in whom the appendix is normal on laparotomy.

424 ESOPHAGEAL INTRAMURAL PSEUDODIVERTICULOSIS Leka Gajula, M.D., Arturo Baez, M.D. and Gottumukkala S. Raju, M.D., FACG*. Internal Medicine, University of Texas Medical Branch, Galveston, TX. Introduction: Intramural Pseudodiverticulosis (IP) of the esophagus is very rare. We describe a case of IP and present a complete review of the literature of this topic. Case Report: A 50 year old female with AIDS underwent an ERCP for suspected choledocholithiasis – recurrent biliary colics, abnormal LFTs, dilated bile duct & cholelithiasis. She also had heartburn and intermittent dysphagia. Other medical problems include hypertension, diabetes mellitus, coronary artery disease, & chronic renal insufficiency. Intubation of the esophagus with a sideveiwing endoscope was limited as the lumen was plugged with creamish white material. A forward viewing endoscope was used to clear the lumen and exclude esophageal stenosis. On clearing the esophagus of the whitish material, extensive intramural pseudodiverticulosis was noted, which was later confirmed by a barium swallow. Gastroesophageal reflux was noted on barium swallow. Esophageal biopsies revealed acute erosive esophagitis with abundant invasive fungal pseudo– hyphae and yeasts consistent with candidial esophagitis. A CT scan of the chest demonstrated diffuse esophageal thickening without mediastinal disease. She made a remarkable improvement on treatment with Fluconazole and Omeprazole. She had undergone biliary sphincterotomy for ampullary stenosis and subsequently cholecystectomy was performed.