Beninfield, SJ

Krige, JEJ

Shaw, JM

English

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The South African Gastroenterology Review • March 2007 8Ciliated hepatic foregut cysts (CHFC) are rare congenitalcystic lesions of the liver. CHFC are usually asymptomatic butmay present with vague abdominal symptoms. CHFC areclinically important because of the possibility of malignanttransformation1 and the diagnostic difficulties CHFC pose. Wereport the details of a patient with a large, symptomaticCHFC.Case ReportA 50-year-old woman presented with a 4-month history ofright upper quadrant pain. The pain fluctuated in severity andwas controlled with oral analgesic medication. There was noassociated jaundice, rigors or weight loss. Apart from minimalright upper quadrant tenderness, examination wasunremarkable. Full blood count, liver and renal function testsand clotting profile were normal. The CA 19-9 serum levelwas 27 IU/l (normal 0-37 IU/l). An abdominal ultrasound scan(US) reported a 50-mm diameter proximal main bile duct withno intra-hepatic biliary dilatation or gallstones, suggesting atype I choledochal cyst. Computed tomography (Figure 2)and magnetic resonance imaging (Figure 3,4) confirmed acystic mass in the porta hepatis. An MRCP was performedand is shown in Figure 5. Included in the differential diagnosiswas a type I choledochal cyst, a Phrygian cap of the gallbladder, biliary cystadenoma, duplicate gallbladder hydatidCASE REPORTCiliated hepatic foregut cyst:a rare cystic liver lesion JM Shaw, JEJ Krige, SJ BeninfieldDepartments of Surgery and Radiology , University of Cape Town Health Sciences Faculty and Surgical Gastroenterology Unit,Groote Schuur Hospital, Cape Town, South Africa.Correspondence:JM ShawDepartment of SurgeryUniversity of Cape Town Health Sciences FacultyObservatory 7925Cape Town, South AfricaTel: -27-21-404 3072Fax: -27-21-448 0981E-mail: jshaw@curie.uct.ac.zaFigure 2:Figure 3:The South African Gastroenterology Review • March 2007 9cyst.and duplication cyst.At laparotomy an 8 cm isolated cyst in segment five of theliver was found, displacing the normal gallbladder from itsbed. The common bile duct was normal macroscopically andintra-operative cholangiography demonstrated a normal intra-and extra-hepatic biliary system. The cyst had nocommunication with the biliary system. A retrogradecholecystectomy was performed and the cyst was excisedafter aspirating its contents. The cyst contained thick greenfluid yielding histiocytes on cytology, and the followingchemistry: bilirubin 22 µmol/l (normal 5-19), cholesterol 2.4mmol/l and amylase 422 IU/l. The patient made an uneventfulpost-operative recovery and was discharged on day 4.Pathology of the specimen showed a fibrous cyst walllined by pseudostratified ciliated columnar epithelium and afocus of thickened basement membrane reminiscent ofrespiratory epithelium. No evidence of malignancy waspresent.DiscussionIntrahepatic ciliated foregut cysts are rare, with only 67 casesreported since first described as a congenital malformationby Friederich in 1857.2 The majority (55) of these cases havebeen reported in the last 25 years as imaging techniqueshave improved. CHFC are thought to arise from theembryologic foregut, as do bronchial and oesophageal cysts,but do not contain cartilage.3 Most cases have been reportedin adults, and rarely exceed 4cm in diameter. The averageage of presentation is 52 years, but ranges from neonates tothe ninth decade. Forty percent of the reported cases havebeen found incidentally on imaging studies, 26% incidentallyat autopsy, 6% incidentally at surgery and 22% presented withabdominal symptoms. CHFC are most frequently locatedsuperficially in the median segments of the liver (segments 4,5, 8), are rarely multi-loculated or septated and are mostlyasymptomatic.4 Although sludge-like bile and viscid mucoidcontent5 of the cyst have been described, as in our patient, nocommunication with the biliary tree could be demonstrated inany reported cases.The imaging appearances of CHFC’s are variable andappear as anechoic or hypoechoic cysts on ultrasound. CTfindings are of a non-enhancing, rounded lesion of varyingdensity depending on the contents of the cyst which caninclude calcium crystals and cholesterol.6 Typical MRI featuresare a hyperintense cyst on T2-weighted images and avariable appearance on T1-weighted images.7Pathologically, the cysts are typically single/solitary andunilocular, and have four layers: an outer fibrous rim, a layerof smooth muscle (often incomplete), subepithelial connectivetissue and a lining of ciliated, pseudo-stratified, mucin-secreting columnar epithelium.8The differential diagnosis includes any cystic lesion of theliver, but also noncystic lesions if the cysts contain dense fluidor harbour malignant change.The management of CHFC is not well established due tothe rarity of this finding. Diagnostic uncertainty ormisdiagnosis frequently results in surgical exploration, as inour case. Of concern are reports of CHFC harbouringCASE REPORTFigure 4: Figure 5:Figure 1: Intra-operative photograph demonstrating (1)Ciliated hepatic foregut cyst, (2) gallbladder dissected off bedand attached by cystic duct,  (3) duodenumThe South African Gastroenterology Review • March 2007 10squamous cell carcinoma (4.4% in reported cases)1,9,10, asCHFC was previously thought to be a benign condition. Thisfact may mandate exploration of all CHFC’s diagnosed pre-operatively. There is also a single report of portal veincompression secondary to the mass-effect of the CHFC.11In conclusion, we report a rare case of a large 8 cm CHFC.The location in segment 5 of the liver and the absence ofbiliary communication are features consistent with other casereports. Due to the risk of malignant transformation andpotential confusion with other benign and non-benignconditions, surgical resection is warranted.References:1. Vick DJ, Goodman ZD, Ishak KG. Squamous cell carcinoma arisingin a ciliated hepatic foregut cyst. Arch Pathol Lab Med 1999;123:1115-1117.2. Friederich N. Cyst emit Flimmerepithel in der Leber. Arch PatholAnat 1857; 11:466-4693. Chatelain D, Chailley-Heu B, Terris B, Molas G, Le Cae A, Vilgrain V,Belghiti J, Degott C, Flejou JF. The ciliated hepatic foregut cyst, anunusual bronchiolar foregut malformation: a histological,histochemical and immunohistochemical study of seven cases.Human Pathol 2000;31:241-2464. Wheeler DA, Edmondson HA. Ciliated hepatic foregut cyst. Am JSurg Path 1984; 8:467-4705. Vick DJ, Goodman ZD, Deavers MT, Cain J, Ishak KG. Ciliatedhepatic foregut cyst. A study of six cases and review of theliterature. Am J Surg Path 1999; 23:671-6776. Kimura A, Makuuchi M, Takayasu K, Sakomoto M, Hirohashi S.Ciliated hepatic foregut cyst with solid tumour appearance on CT. JComput Assist Tomogr 1990; 14:1016-10187. Fang SH, Dong DJ, Zhang SZ. Imaging features of ciliated hepaticforegut cysts.World J Gastroenterol 2005; 27:4287-42898. Stringer MD, Jones MO, Woodley H, Wyatt J. Ciliated hepatic foregutcyst. J Pediatr Surg 2006; 41:1180-11839. Furlanetto A, Dei Tos AP. Squamous cell carcinoma arising in aciliated hepatic foregut cyst. Virchows Arch 2002; 441:296-29810. De lajarte-Thirourard AS, Rioux-leclercq N, Boudjema K, gandon Y,Ramee MP, Turlin B. Squamous cell carcinoma arising in a hepaticforegut cyst. Pathol Res Pract 2002;198:697-70011. Harty MP, Hebra A, Ruchelli ED, Schnaufer L. Ciliated hepaticforegut cyst causing portal hypertension in an adolescent. Am JRoentgenol 1998; 170:688-690CASE REPORT

Ciliated hepatic foregut cyst: a rare cystic liver lesion

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Ciliated hepatic foregut cyst: a rare cystic liver lesion

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