Cystic Disease of the Liver and Biliary Tract Gut: First Published As 10.1136/Gut.32.Suppl.S116 on 1 September 1991

S116 GutSupplement, 1991, S116-S122 Cystic disease of the liver and biliary tract Gut: first published as 10.1136/gut.32.Suppl.S116 on 1 September 1991. Downloaded from A Forbes, I M Murray-Lyon Abstract ances, aspiration for microbiological and cyto- The widespread availability of ultrasound logical examination is warranted. Several reports imaging has led to more frequent recognition - (for example,5 and our own unpublished obser- of cystic disease affecting the liver and biliary vations) of needle diagnosis of unsuspected hy- tract. There is a wide range ofpossible causes. datid disease, and even therapy by ultrasound Cystic disease of infective origin is usually guided transcutaneous injection of sclerosant,67 caused by an Echinococcal species, or as the indicate that ifthe transhepatic route is taken the sequel of a treated amoebic or pyogenic risk ofmorbidity is low. abscess. The clinical and radiological features Distinction of abscess from cyst is relatively are often then distinctive and will not be dwelt simple if an abscess has viscous echo dense upon in this review, except in respect of their contents with a thick wall and densely com- contribution to the differential diagnosis of pressed surrounding hepatic parenchyma. Per- non-infective disorders. The principal non- cutaneous aspiration allows confirmation of the infective cysts can be conveniently divided diagnosis, provides material for microbiological between the simple cyst, the polycystic syn- examination, and may be of major therapeutic dromes (usually with coexistent renal disease), benefit. Positive blood cultures or amoebic Caroli's syndrome, and choledochal cysts. serology may, however, render aspiration super- The overlap between constituent members of fluous, given that small single abscesses can be these groups, and the association of cystic effectively managed with systemic antimicro- disease with hepatic fibrosis (especially with bials alone. Open surgical drainage remains congenital hepatic fibrosis) has attracted con- necessary for optimal results in some patients siderable attention, and it has been suggested with chronic multiloculated abscesses. that they may all be considered to belong Cystic lesions in continuity with the biliary to a hepatobiliary fibrocystic continuum. In tree or with vascular structures may be identified addition there are a variety ofcystic neoplasms by ultrasound (the latter particularly so with and a miscellany ofunusual forms. Doppler probes), but it will usually be necessary to obtain contrast studies (by ERCP or angio- http://gut.bmj.com/ graphy) for complete diagnosis. In this context Investigation and diagnosis computed tomography and MR images of the Large cystic lesions may be recognised clinically, hepatobiliary region do not appear at present to but in most cases will be found at ultrasound offer many advantages over high resolution sono- examination undertaken because of symptoms, graphic examination. The interested reader is or laboratory screening tests suggestive ofhepato- referred to one ofa number ofillustrated reviews biliary disease; cysts are not infrequently an in the radiological literature. i2I48 on October 1, 2021 by guest. Protected copyright. incidental finding when scans are done for un- Liver biopsy tends to be avoided in the related clinical problems. investigation of cystic disorders, a stance cer- An accurate differential diagnosis will often be tainly justified when there is suspicion ofhydatid possible from ultrasonography alone.'2 Most disease, and probably so when imaging suggests commonly, one or more clearly defined echo- choledochal cyst, but the recognition of under- lucent spaces are identified within the liver lying fibrosis is then less likely, and the clinician substance. When these have thin walls and is denied potentially important prognostic reduce the signal from more distant structures, information. the most likely diagnosis is of simple cyst and further investigation is usually unnecessary. It is suggested, however, that magnetic resonance SIMPLE HEPATIC CYSTS (MR) imaging may be particularly helpful in Benign non-parasitic cysts of the liver, once doubtful cases.34 Thicker walled single or mul- considered rare because they are generally small tiple cysts with a multilayer or laminated margin and asymptomatic, are more commonly shown are likely to be hydatid, and the presence of by modem imaging (prevalence between 0 19 daughter cysts within the lesion is virtually and 2.5%),2 more so in women (female:male ratio pathognomonic. A supportive history and posi- 1.5),2 more often in the right lobe, and probably tive serology will usually allow confirmation of ofcongenital origin. The cysts are lined by biliary Gastrointestinal Unit, this diagnosis. Aspiration ofpresumptivehydatid columnar epithelium, but it is relatively unusual Charing Cross Hospital, cysts is contra-indicated because ofthe perceived for the fluid contents to be bile. If the typical London high risk ofanaphylaxis if cyst contents spill into appearances at sonography described above are A Forbes I M Murray-Lyon the peritoneum, and there is usually no need to seen, no further investigation or treatment is consider if disease is suspec- The of Correspondence to: aspiration hydatid required. sensitivity aspiration cytology Dr I M Murray-Lyon, ted. The hazard has perhaps been over empha- in potentially neoplastic cysts with no definite Gastrointestinal Unit, Charing Cross Hospital, London sised, however, and ifhydatid disease is unlikely solid component is low, but may be improved by W6 8RF. and cysts have inconclusive sonographic appear- assay ofcyst contents for tumour markers such as Cysticdisease ofthe liverand biliary tract S117 carcinoembryonic antigen.'0 Other than from disease may, however, occur in the absence of serial scans begun at the time ofinfection it is not significant renal involvement. The cysts are lined clear that postinfective cysts - longterm sequelae by biliary type epithelium which retains a func- Gut: first published as 10.1136/gut.32.Suppl.S116 on 1 September 1991. Downloaded from of unaspirated abscesses - can accurately be tional response to secretin.9 Their pathogenesis distinguished from congenital benign cysts.'8 (akin to that of simple cysts) is considered to All benign cysts appear subject to a variety of reflect failed involution of excess intralobular complications. Bleeding into or infection of the bile ducts during embryological develop- cyst contents may be responsible for the onset of ment.3031 Liver biopsy shows portal tract fibrosis pain or other symptoms; enlargement of cysts and numerous cystically dilated channels; the also occurs without obvious cause, and may then hepatic parenchyma is otherwise normal but it is lead to the presence of a painful swelling," not unusual to find Von Meyenburg complexes obstructive jaundice'2 or intraperitoneal perfora- in non-cystic parts ofthe liver (see below). Overt tion.'3 Although surgical intervention may congenital hepatic fibrosis (see below) certainly become necessary, in the absence of peritonitis occurs32 but is unusual. or severe intraabdominal bleeding, conservative Apparently spontaneous infection of hepatic management is probably to be preferred,'4 15 cysts is described, usually with gut derived with'2 or without'6 percutaneous aspiration. organisms, and prompt recognition and drainage Recurrent symptoms may be prevented by in- greatly reduces the mortality which may exceed ternaldrainage ofthe cyst'7 or bythe instillation of 75% with antibiotic therapy alone.33 Mechanical sclerosant,'891 the latter course in our view to be cholestasis caused by the cysts is rare,3' but a followed only after very careful direct cholangio- common topic ofcase reports: it remains unclear graphy has excluded communication with the why the pressure within the cysts should rise biliary tree, if biliary sclerosis is to be avoided. sufficiently to cause obstruction, but hormonal Although there does not seem to be a major responsiveness29 may be of relevance. Relief of problem with alcohol injection in analogous biliary obstruction may be satisfactorily pro- management of hydatid cysts7 longterm follow vided by percutaneous aspiration.3' In advanced up data are sparse. Surgical deroofing of cysts disease portal hypertension responsible for without drainage ofthe residual cavity is strongly ascites and variceal haemorrhage can become recommended20 ifsurgery is thought indicated. problematic34: good results may be obtained Concernthatsimplecystsrepresentapremalig- from portosystemic anastomosis. In the majority nant state has been generated by a series of of cases, however, management will be that of reports of neoplasia in apparently preexisting chronic renal failure with consideration of renal benign cysts. The tumours are, however, of transplantation. If there are problems related to disparate histological type - for example, muco- the sheer size of the liver or coexistent sympto- epidermoid carcinoma,2' multifocal papillary matic portal hypertension, then many units will cystadenocarcinoma,22 squamous cell car- now take the opportunity to carry out double cinoma23 - and these may represent coincidental organ grafts from the same donor: good results associations rather than complications. The are reported, but disappointingly King's was not http://gut.bmj.com/ observation that foci of cystadenoma and cysta- the first unit to publish specifically on this denocarcinoma (the most common malignancy topic.35 described with simple cysts) coexisted

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