七院聯合 CPC 93 年 11 月 03 日

1. 七院聯合CPC93年11月03日 鄧景升醫師/李春銘主治 三軍總醫院 小兒部

2. Chief complaints • Progressive abdominal pain, occasional vomiting, diarrhea and intermittent high fever for six days

3. Present illness • A two- year- old boy was in good condition without any complaint of abdominal symptoms before presentation. • He was referred to our pediatric clinic with a six- day history of progressive abdominal pain and intermittent high fever. • Vomiting and diarrhea were occasionally accompanied with abdominal pain. • Appetite decreased.

4. Physical examinations • BT: 39’C • Abdomen: palpable mass on RUQ of abdomen

5. Laboratory data Initial laboratory data • WBC: 16.2 X 109/ l • Neutrophil: 83% • ESR: 82 mm/h • CRP: 8.8 mg/dl • PPD skin test: negative • Biochemical data: normal range • Blood culture: pending result • Stool culture: pending result

6. Hospital course • After admission, KUB and abdominal ultrasound were arranged. • The abdominal ultrasound revealed a target lesion on the RUQ of the abdomen  the presumptive diagnosis of intussusception was made at that time. • Barium enema reduction was attempted but failed to find intussusceptum. • CT of abdomen was performed. • The patient was placed at bowel rest, and intravenous fluid were administration. • A blood culture and stool culture were obtained, and empirical antibiotic therapy was initiated

7. Image studies • KUB: non specific gas pattern • Abdominal sonography: a target lesion on the RUQ of the abdomen • Barium enema: A radiopaque filling defect with moth- eaten margin in the ascending colon near the hepatic flexure region.

8. Image studies • CT of the abdomen: circumferential thickening of the intestinal wall in the ascending colon. The length of the thickened segment was about 7 cm, and the wall thickenness was about 2 cm

9. Questions to be asked

10. Questions: (1) • What was the pattern of abdominal pain? Location ? • physical examination? • Vital sign?, BP, RR, PR? • Weight loss ? • Other signs of physical examination? Chest ?abdomen ? Bowel sound? Rigidity? Soft ? Locate the site of maximal pain? Tenderness? Pattern of palpable mass ?, etc. • Was there any other enlarged lymph nodes on physical examination ? • History? • Did he have history of foreign travel ? Did he live with froeign servant? • Was there family history of cancer ? • Have the patient drunk unpasteurized milk? • Did he have history of contact with a case of tuberculosis, a parent ? or other family ?

11. Questions: (2) • Lab • Stool routine? Stool pattern?Bloody stool ? Examination of stool specimens showed ova or parasites? • CBC-H, LDH, uric acid level ? • Chest X-ray ? • Was there any other findings on CT and barium enema ? Another abnormal finding on other sites ? skip lesions ? fissures, sinus tracts, fistulas ? Hepatosplenomegaly? • Did CT scanning show any other lymphadenopathy in this patient ? • colonoscopic examination ?

12. What are the patient’s problems ??

13. Palpable mass on RUQ of abdomen Abdominal ultrasound revealed a target lesion on the RUQ Barium enema: a radiopaque filling defect with moth- eaten margin in the ascending colon near the hepatic flexure region. CT of the abdomen: circumferential thickening of the intestinal wall in the ascending colon. Abdominal pain High fever Vomiting Diarrhea Leucocytosis Elevation of ESR and CRP Minor Problems Major Problems

14. Abdmonial mass Target lesion high fever ? ? location Filling defect Thickened bowel wall

16. Targetlesion • A targetlesion at the ultrasound scan indicating thickened bowel wall • On ultrasonography encircling thickening of the colonic wall (targetsign). Journal of Korean Medical Science. 15(4):371-9, 2000 Aug.

17. Target sign Gastroenterology ClinicsVolume 31 • Number 3 • September 2002 • Neoplasms bowel wall thickening  target sign or “pseudokidney” sign • Focal inflammatory or ischemic masses. • Perforating diverticulitis • Ischemic colitis : "target lesion"

18. fillingdefect • A lesion protruding into the lumen appear as a radiolucent fillingdefectin the barium pool • Mass lesion • Ischemic colitis • Amoebiasis– amoeboma

19. CT Diagnosis of the Abnormal Bowel Wall Radiographics. 2002;22:1093-1107 Thickened bowel wall • idiopathic inflammatory bowel diseases, • infectious diseases, • radiation damage. • malignancy.

20. Physiological  Spasm  Distended bladder Malignant  Annular carcinoma  Scirrhous carcinoma Lymphoma Diverticular disease Muscle thickening  Pericolic abscess Superimposed malignancy Ischaemia Radiation colitis Inflammatory bowel disease Ulcerative colitis  Crohn’s disease  Tuberculosis  Lymphogranuloma venereum Amoebiasis Extrinsic disease  Intra-abdominal masses  Metastatic carcinoma  Endometriosis  Pelvic lipomatosis  Cholecystitis  Pancreatitis Miscellaneous  Postoperative anastomosis  Trauma  Hirschsprung’s disease Causes of large-bowel strictures

21. Hepatic: Hepatoblastoma Hepatocellular carcinomaRhabdomyosarcoma (rare)Angiosarcoma (very rare) Renal Wilms' tumor Renal cell carcinoma(rare) Lymphoma (very rare) Adrenal Neuroblastoma  Adrenal cortical carcinoma (rare) Gastrointestinal Lymphoma Carcinoid (appendix) Teratoma Carcinoma (very rare) Lymphatic Lymphoma Other Teratoma Neuroblastoma, sympathetic chain Sarcoma Pancreatoblastoma (very rare) Malignant abdominal masses (nonneonatal)

22. Teratoma • The sacrococcygeal region: most site. • most commonly in infants, at birth, • females Carcinoid Tumors • usually occur in the appendix in children • outside the appendix (ileojejunum, colon) commonly metastasize • carcinoid syndrome  episodic intestinal hypermotility and diarrhea, vasomotor disturbances (flushing) (75% to 90%) , bronchoconstriction (wheezing) (25%), and right-sided heart failure • Barium enema: single or multiple filling defects in the distended ileum • Carcinoid tumors rarely occur in the large intestine  X

23. LYMPHOGRANULOMA VENEREUM • a systemic sexually transmitted disease • Chlamydia trachomatis causes lymphogranulomavenereum. A chronicproctitis is complicated by fistula formation, extensive fibrosis, and eventual stricture formation.X

24. CT OF THE COLONGrainger & Allison's Diagnostic Radiology: A Textbook of Medical Imaging, 4th ed • Three basic patterns have been described in benign disease: • (i) a homogeneous ring of bowel wall >4 mm thick; • (ii) a double halo with alternating layers of density; • (iii) target sign

25. Carcinoma • Diagnosis: family history, endoscopic findings, gastrointestinal bleeding, or obstruction. • Symptoms are nonspecific abdominal pain, an abdominal mass • carcinoma does not follow a circumferential pattern • Rare in children, in early adulthood • Scirrhous carcinoma may infiltrate diffusely to present as a relatively smooth stricture. This type of tumour is much more likely to be metastatic from the stomach or breast than a primary lesion X

26. Abdmonial mass Target lesion High fever Amebic colitis with ameboma Infectious colitis (parasitic, viral) Intestinal tuberculosis Pericolic abscess Inflammatory bowel disease(ulcerative colitis and Crohn’s disease ) Lymphoma Ischemic colitis Filling defect Thickened bowel wall colon

27. PPD skin test(Gershon: Krugman's Infectious Diseases of Children, 11th ed ) • A negativetuberculinskintestnever rules out tuberculosis in a child. • The most common causes of false-negative : incubation of viral infections incubation of bacterial infections; overwhelming tuberculosis; recent administration of live viral vaccines; severe malnutrition; diseases and drugs causing anergy; extremes of age (newborns and the elderly) • False-negative TST : severe tuberculosis disease soon after infection, those with immunosuppressive illnesses, malnutrition, or other severe infections. (The Lancet Infectious Diseases Volume 3 • Number 10 • October 2003)

28. The Lancet Infectious Diseases Volume 3 • Number 10 • October 2003 • Extra-pulmonary tuberculosis disease is more common in children than adults, 25% of infants and young children less than 4 years of age • But extra-pulmonary manifestations of tuberculosis, such as gastrointestinal or renal, are rare in children because of long incubation periods required following haematogenous dissemination to manifest as disease. • The rate of false-negative TST in children with tuberculosis who are infected with HIV, is unknown, but it is certainly higher than 10% and is dependent on the degree of immunosuppression (ie, CD4 counts).

29. Intestinal tuberculosis • pain, diarrhea or constipation, and weight loss with low-grade fever. • primary bovine origin from drinking unpasteurized milk, the chest radiograph being normal. Question ? • Ulcerative, hypertrophic or mixed forms are described. • The ulcers tend to be large and circumferential with a shaggy edge, • The hypertrophic form presents with an inflammatory mass and stenosis of the bowel lumen. • palpable mass may be noted • commonest in the ileocaecal region, but may be seen in any part of the gastrointestinal tract

30. Intestinal tuberculosis • a conical caecum with a patulous ileocaecal valve and a dilated terminal ileum • transverse ulceration, ulcerated stricture sharply demarcated from normal bowel. • Utrasound and CT : ascites, peritoneal involvement, and lymphadenopathy. • Caseous lymph nodes with a hypoechoic centre on ultrasound, or peripheral enhancement on CT, with ascites and a thickened bowel wall are highly suggestive of tuberculosis. • caseous epithelioid granulomas (CGs) are characteristic of tuberculosis.

31. Intestinal tuberculosis • The diagnosis of extrapulmonary TB: collecting specimens for AFB stain and culture and sometimes for pathologic diagnosis. • PCR: rapid diagnosis of tuberculosis. • PCR does not seem to be markedly sensitive for intestinaltuberculosis. Kim et al. found that the PCR was positive in only 30% of fresh biopsy specimens.

32. LYMPHOMA • Usual < 3y/o • Lymphoma is the most common malignancy of the gastrointestinal tract in children • Primary lymphoma of the colon is rare and non-Hodgkin’s in type. • stomach, distal ileum, cecum, or appendix and may present as crampy abdominal pain, vomiting, distention, or a palpable abdominal mass. • Mild fever. • The caecum or rectum are usually involved, as these sites contain the most lymphoid tissue. • The most common location was ileum

33. LYMPHOMA • A large polypoid mass or annular lesion is typical • Annular infiltration involving a long segment of >5 cm, with deep fissuring, cavitation, and a large extraluminal component, are features that suggest lymphoma. •  X

34. Diverticular disease • Diverticular disease is recognized radiologically from its two main components — the muscular abnormality and the diverticula. • commonest in sigmoid lesions, rare in caecal lesions • there is left-sided abdominal pain, tenderness, and some fever. • The presence of diverticular disease : thickening of the muscle wall, and the presence of extramural gas bubbles or pockets of fluid within the diverticula. X • Diverticular disease is a common abnormality in the elderly  X • interdigitating folds

35. Pericolic abscess • Perforation of a diverticulum  an inflammatory reaction in and around the wall of the colon, segmental narrowing • Plain radiographs : localized ileus, a soft-tissue mass or, rarely, gas within the abscess. • A water-soluble contrast enema: tracking from a ruptured diverticulum • CT: wall thickening >4 mm of homogeneous density, and the presence of gas or contrast medium in the diverticula outpouches. • fine stranding, a fluid collection with gas bubbles or a fluid level is diagnostic of an abscess. • the ‘saw-tooth’ pattern ( fibrosis), which may be ‘draped’ around the site of the abscess.

36. Inflammatory bowel diseases • onset at 15–25 yr of age and a second smaller peak at 50–80 yr of age. X • Diarrhoea, fever and malaise are common to both. • Abdominal pain and a tender mass are more typical of Crohn’s disease, • chronic diarrhea of greater than 4 weeks' duration.

37. Ulcerative colitis Radiographic imaging of inflammatory bowel diseaseGastroenterology Clinics Volume 31 • Number 1 • March 2002 • Double- contrast barium enema • fillwith barium creating the granular --typical of ulcerative colitis--X • Deeper ulcerscollar-button ulcers– X • rectal bleeding -- ulcerative colitis • In advanced disease • ulcerative colitis is characterized by a pancolitis with diffuse ulceration, or absent haustral folds, thumbprinting, and narrowing or shortening of the colon, most commonly in the rectosigmoid • Computed tomography • CT: mural thickening • the mean wall thickness is 8 mm, (normal colon is only 2 to 3 mm) • inhomogeneous enhancement of the colonic wall • This enhancement results in a classic target or double halosign

38. Crohn's disease • a chronic idiopathic granulomatous process characterized by transmural inflammation of the bowel, often associated with the development offissures, sinus tracts, fistulas, and abscesses  question ? • Large anal skin tags (1–3 cm diameter) or perianal fistulas  question? • discontinuous skip lesions between areas of uninvolved bowel question ? • most terminal ileum and proximal colon, isolated colonic disease in 20% to 27% • may involve the gastrointestinal tract anywhere from the mouth to the anus • chronic phase: circumferential thickening of the bowel wall, irreversible strictures in the small bowel or colon

39. Crohn's disease Barium studies • the small bowel is involved in up to 80% of casesquestion? • The earliest radiographic findings of Crohn's disease in the small bowel or colon are aphthous ulcers (collections of barium surrounded by radiolucent halos) • development of a cobblestonemost commonly occurs in the small bowel .

40. Crohn's diseaseComputed tomography • the most common : mural thickening • Average wall thickness of 11 to 13 mm in the small bowel or colon • Mural thickening is most common in the terminal ileum: thickness of 2 cm • fistulas and sinus tracts 20% to 40% of cases • Colonoscopy with biopsy in establishing a diagnosis.

41. ISCHAEMIC COLITIS • the systemic manifestations of cardiac arrhythmia, myocardial infarction, and congestive heart failure • abdominal pain and rectal bleeding of sudden onset. • The mucosa  oedema thumbprinting • mosaic’ pattern • The splenic flexure and the descending colon are the commonest sites (watershed between the superior and inferior mesenteric arteries).X • The rectum and ascending colon are rarely involved • Ischaemia is usually segmental, involving about a 19-cm length.

42. Thumbprinting Mosaic pattern in early ischaemic colitis

43. Amebic colitis • Amebic colitis affects all age groups, but its incidence is strikingly high in children 1–5 yr of age. • a granular mucosa • The radiological features of invasive amoebiasis include a segmental or diffuse colitis, with a granular or ulcerated mucosa. • Aphthoid ulceration may be seen, and amoeboma formation occurs in about 10% of cases. • inflammatory granulation masses cause an irregular stricture • luminal narrowing on a barium-enema examination  ameboma. • Tender, palpable, abdominal mass.

44. Amebic colitis • they are often multiple and are usually found at the flexures and the caecum. • Ameboma results from the formation of annular colonic granulation tissue at a single site or multiple sites, usually in the cecum or ascending colon. An ameboma may mimic carcinoma of the colon

45. Amebic colitis • history of cramping abdominal pain, weight loss, and watery or bloody diarrhea. • Infection with Entamoeba histolytica may be asymptomatic or may cause dysentery • The insidious onset and variable signs and symptoms make diagnosis difficult, with fever and grossly bloody stool absent in most cases.

46. luminal narrowing (arrow) on a barium-enema examination in a patient with ameboma.

47. INFECTIOUS COLITIS • Salmonella, Shigella and Campylobacter may all present with a localized or diffuse colitis, with a granular or ulcerated mucosa. • marked ileus • profuse, watery diarrhea, rectal bleeding, and edematous mucosa in the rectum

48. PSEUDOMEMBRANOUS (ANTIBIOTIC-ASSOCIATED) COLITIS • Broad-spectrum antibiotics or chemotherapy may predispose to an overgrowth of the Gram-positive Clostridium difficile • diarrhoea, pyrexia, and leukocytosis. • Plain radiographs may show a generalized ileus. • CT and ultrasound may detect ascites and a thickened colonic wall. • Nodular haustration • stool specimen for the C. difficile toxin

49. VIRAL COLITIS • ileocecal area, in immunocompetent persons. • Cytomegalovirus (CMV) causes a vasculitis with a thick wall, lymphadenopathy and large ulcers that may bleed, and is typically ileocolic in distribution X • Tissue staining for cytomegalovirus • CT will show a thickened bowel wall, mesenteric lymphadenopathy, and often ascites. • The herpes simplex virus is associated with a proctitis and multiple more superficial ulcers. X

50. PARASITIC COLITIS • In trichuriasis (鞭蟲病)small coiled worms may be seen on the mucosal surface.X • Strongyloides stercoralis may simulate ulcerative colitis. X • In Chagas’ disease a megacolon results from the neurotoxic effect of the protozoon Trypanosoma cruzi. • In schistosomiasisova are deposited in the submucosa of the large bowel. The inflammatory response results in the formation of numerous polyps. Fibrosis may later cause stricture formation and calcification may be visible in the bowel wall.X