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Diseases of the Gallbladder and Bile Ducts 4.13 Angela D

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Diseases of the Gallbladder and Bile Ducts 4.13 Angela D Chapter Diseases of the Gallbladder and Bile Ducts 4.13 Angela D. Levy, Charles A. Rohrmann Contents Introduction Introduction . 509 Over the past decade, technological advances in ultra- Part I:Gallbladder . 509 Inflammatory Diseases . 509 sound, computed tomography (CT), and magnetic reso- Emphysematous Cholecystitis . 509 nance imaging (MR) have expanded the application and Xanthogranulomatous Cholecystitis . 510 versatility of these techniques to more accurately iden- Tumor-like Lesions . 511 tify and characterize diseases of the gallbladder and bile Adenomyomatous Hyperplasia . 511 ducts. Precise definition of the underlying pathologic Cholesterol Polyps . 513 process and extent of disease is now feasible with non- Neoplastic Diseases . 513 invasive imaging techniques. In this chapter, the radio- Gallbladder Adenomas . 513 Gallbladder Carcinoma . 514 logic features of the diseases of the gallbladder and bile ducts will be discussed with pathologic correlation. Part II:Bile Ducts . 516 Congenital Disorders . 516 Caroli Disease . 516 Choledochal Cyst . 517 Part I: Gallbladder Choledochocele . 519 Biliary Diverticulum . 520 Inflammatory Diseases Inflammatory Disorders . 520 Acute Pyogenic Cholangitis . 520 Emphysematous Cholecystitis Primary Sclerosing Cholangitis . 520 Secondary Sclerosing Cholangitis . 523 Incidence and Clinical Features Recurrent Pyogenic Cholangitis . 523 AIDS-Related Cholangiopathy . 524 Ischemic Cholangiopathy . 525 Emphysematous cholecystitis is a rare variant of acute Neoplastic Diseases . 526 cholecystitis characterized by the presence of gas in the Adenomas and Papillomatosis . 526 gallbladder wall, lumen, or pericholecystic tissues. The Biliary Adenocarcinoma . 527 majority of patients are between 50 and 70 years of age Metastases . 527 and have underlying chronic medical conditions such Lymphoma . 530 as diabetes mellitus or atherosclerotic peripheral vascu- References . 530 lar disease [1]. Emphysematous cholecystitis is more common in men. Patients may present with signs and symptoms of acute cholecystitis or vague, insidious symptoms. Vascular compromise of the cystic artery is thought to play a role in the pathogenesis of gas forma- tion [2]. Clostridium welchii and Escherichia coli are or- The opinions and assertions contained herein are the ganisms that are commonly isolated from the infected private views of the author, and are not to be construed gallbladders. as official, or as reflecting the view of the Department of the Army or Defense. Radiologic Features The radiologic diagnosis of emphysematous cholecys- titis is made by the identification of gas within the gall- bladder lumen, wall, or pericholecystic tissues. Abdom- 510 Angela D. Levy, Charles A. Rohrmann der”because it resembles the appearance of effervescent bubbles rising in a champagne glass [4, 5]. Careful at- tention to the appearance of suspected intramural and intraluminal gas should be made because other highly reflective entities such as calcification in the gallbladder wall, a contracted gallbladder with stones, or adenom- yomatous hyperplasia may be mistaken for intramural or intraluminal gas. CT is the most sensitive and specific imaging modal- ity for identifying gas within the gallbladder lumen or wall (Fig.2).The presence of pneumoperitoneum,pneu- mobilia, and portal venous gas are readily identified as well. Fig. 1. Emphysematous cholecystitis in a 49-year-old diabetic man with a history of pain and fever. Longitudinal ultrasound image of Xanthogranulomatous Cholecystitis the gallbladder shows a diffusely echogenic gallbladder wall due to intramural gas (arrowheads). Intraluminal gas (arrows) is shown as echogenic reflectors within the gallbladder lumen Incidence and Clinical Features Xanthogranulomatous cholecystitis is an uncommon form of chronic cholecystitis characterized by the pres- inal radiography may demonstrate clear demarcation of ences of xanthomatous histiocytes, chronic inflamma- the gallbladder by intramural gas collections or may tory cells, and scarring in the gallbladder wall [6]. The show hyperlucency in the right upper quadrant. inflammatory process produces a tumor-like appear- The most common sonographic feature of intramu- ance in the gallbladder that may simulate malignancy ral gas is highly echogenic reflectors within the gall- radiologically and pathologically [7]. bladder wall producing low-level posterior acoustic Xanthogranulomatous cholecystitis is most fre- shadowing and reverberation artifact. The echogenic quently observed in women between the ages of 60 and reflectors in the gallbladder wall may change position 70 years [6]. Patients usually present to medical atten- and configuration when the position of the patient is tion with complaints of right upper quadrant pain, nau- changed. Intramural gas may also appear as a highly re- sea, and vomiting. A positive Murphy sign may be flective gallbladder wall [3]. Less commonly, real-time present on physical examination. A tender, palpable, scanning will show gas in the lumen of the gallbladder right upper quadrant mass is found in less than 50% of as a band of highly reflective echoes or small, nonshad- patients [8]. Complications such as perforation, abscess owing echogenic foci rising up from the dependent por- formation, fistulous tracts, and extension of the inflam- tions of the gallbladder lumen (Fig. 1). The latter ap- matory process to adjacent organs are present in 32% of pearance has been termed the “effervescent gallblad- cases [9]. Fig.2A,B.Emphysematous cholecystitis. A Unenhanced CT in a 56- the gallbladder. There are inflammatory changes in the adjacent year-old man who presented with right upper quadrant pain and fat. B Opened cholecystectomy specimen from a different patient fever shows mural (arrow) and intraluminal (asterisk) gas within shows a black, necrotic gallbladder mucosa Chapter 4.13 Diseases of the Gallbladder and Bile Ducts 511 Pathologic Features The pathogenesis of xanthogranulomatous cholecys- titis is poorly understood. It has been postulated that the inflammatory process is the result of intravasated bile into the gallbladder wall. Bile may enter the gall- bladder wall through mucosal ulceration or rupture of Rokitansky-Aschoff sinuses when there is cystic duct obstruction and increased intraluminal pressure within the gallbladder [6]. A poorly-defined, infiltrating, yellow nodular mass is typically identified on gross inspection of the gallblad- der. Lithiasis is frequently present. Histologically, foamy histiocytes, lymphocytes, plasma cells, polymorphonu- clear leukocytes, fibroblasts, and foreign body giant cells characterize the inflammatory process (Fig. 3). Histiocytes may contain bile or ceroid pigment. Bands of collagen and cholesterol clefts may be present in the gallbladder wall [10]. Radiologic Features Focal or diffuse gallbladder wall thickening is the most prominent imaging feature of xanthogranulomatous cholecystitis. Foci of xanthogranulomatous inflamma- tion may appear as hypoechoic bands or nodules on ultrasound, or low attenuation nodules on CT (Fig. 3) [11, 12]. Other features that may be identified on sonog- raphy and CT include disruption of the gallbladder wall, indistinct liver margin, pericholecystic fluid, hepatodu- odenal ligament adenopathy, and cholelithiasis. CT more effectively demonstrates adjacent organ involve- ment and extension of the inflammatory process into the adjacent fat planes than sonography. It is well known that xanthogranulomatous cholecystitis may co- exist with gallbladder and biliary malignancies and the imaging features overlap with those of gallbladder car- cinoma [6]. Therefore, the preoperative distinction between these xanthogranulomatous cholecystitis and gallbladder carcinoma is virtually impossible. Fig. 3A–C. Xanthogranulomatous cholecystitis. A Longitudinal Tumor-like Lesions ultrasound image in a 55-year-old man with pain, fever, and leuko- cytosis shows marked gallbladder wall thickening with prominent Adenomyomatous Hyperplasia hypoechoic nodules (arrows). The gallbladder lumen is com- pressed (asterisk) and there is a shadowing stone near the neck of the gallbladder (arrowhead). B Intravenous contrast-enhanced CT Incidence and Clinical Features scan in the same patient shows hypoattenuating nodular areas in the thickened gallbladder wall. There is hypoattenuation in the ad- jacent liver parenchyma (arrow). C Photomicrograph (original Adenomyomatous hyperplasia is a common benign magnification, x2; hematoxylin-eosin stain) shows a thick, fibrotic condition found in 9% of cholecystectomy specimens gallbladder wall with a xanthogranulomatous lesion (arrows) con- [13]. Epithelial and smooth muscle proliferation of the taining foamy histiocytes, bile pigment, and inflammatory cells gallbladder wall may result in localized, segmental, or diffuse disease. Throughout the medial literature a va- riety of names have been applied to this condition in- cluding adenomyomatosis, adenomyoma, diverticular 512 Angela D. Levy, Charles A. Rohrmann Pathologic Features Adenomyomatous hyperplasia is histologically charac- terized by epithelial and smooth muscle proliferation. Normal epithelial structures may invaginate into the gallbladder wall, subserosa, and serosa. The epithelial invaginations (intramural diverticula) may contain in- spissated bile, mucus, or stones. Hyperplastic smooth muscle cells accompany the epithelial invaginations (Fig. 4). Inflammatory and fibrotic changes may accom- pany the proliferative process [14]. There are three variants
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