Congenital Cystic Disease of the Liver and Biliary System

Longmire, William P.; Mandiola, Sergio; Gordon, H. Earl

doi:10.1097/00000658-197110000-00014

Cited by 138 publications

(42 citation statements)

References 5 publications

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“…As noted by Longmire et al [3], the cyst may be fusi form or localized in only one segment of the extrahepatic system. Therefore, in 7 of our patients the dilatation was fusiform, and in 2 the cyst involved only the hepatic hilum.…”

Section: Anatomical Featuresmentioning

confidence: 81%

“…Anatomical study was based upon precise analysis of the routine intraoperative cholan giography. As described by Alonso-Lej et al [2] and Longmire et al [3], cysts were classified into 2 forms according to their shape: spher ical or fusiform. As emphasized by several authors, special attention was drawn to the involvement of intrahepatic ducts [4][5][6][7] and abnor mal arrangement of the pancreatico-biliary system [8][9][10].…”

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Cystic Dilatation of the Common Bile Duct

Saubier¹,

Gouillat²,

Samaniego³

et al. 1985

Dig Surg

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The records of 13 adult patients operated on for a choledochal cyst were reviewed. The cyst was saccular in 6 patients and fusiform in 7. A fusiform enlargement of the main intrahepatic ducts was associated in 5 cases. In 5 patients the pancreatico-biliary ductal union occurred at a high position, forming an abnormally long common terminal duct. 11 patients underwent an excision of the cyst with Roux-en-Y hepaticojejunostomy. Microscopically, the wall of the cyst consisted of fibrous tissue usually devoid of epithelium and muscle layer. Recovery was uneventfull with good late results in most patients. A precise knowledge of its radiological features is necessary to recognize this anomaly and manage it adequately.

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Section: Anatomical Featuresmentioning

confidence: 81%

Section: Methodsmentioning

confidence: 99%

Cystic Dilatation of the Common Bile Duct

Saubier¹,

Gouillat²,

Samaniego³

et al. 1985

Dig Surg

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“…Congenital cysts may develop at any level of the entire intra-and extrahepatic biliary tree [1,3]. However, intrahepatic cysts are believed to be the result of failure of communication between intralobular bile ducts with the interlobular ducts which are a product of the ingrowing extrahepatic biliary tree.…”

Section: Discussionmentioning

confidence: 99%

“…The lining consists of cuboidal or cylindrical epithelial cells. The lining of a big cyst is flat or completely absent [1,2].…”

Section: Discussionmentioning

confidence: 99%

Congenital Multilocular Cysts of the Liver

Al-Idressi

1992

Ann Saudi Med

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Liver cysts are etiologically classified into parasitic and non-parasitic cysts. The latter are differentiated into neoplastic, inflammatory, traumatic, and congenital [11]. The congenital, particularly solitary cysts, are distinctly rare. Their clinical presentation frequently poses a diagnostic and therapeutic challenge. A case of congenital multilocular liver cysts is reported. Review of relevant literature has been done, computed tomographic (CT) findings have been described and problem pertaining to differential diagnosis have been discussed. Case ReportA 53-year-old Saudi female, mother of nine healthy children presented with the complaint of intermittent stabbing pain in the right hypochondrium with pyrexia of three days' duration. The pain radiated to the back and right shoulder. There was a history of similar attacks of pain since adolescence.On examination, the right upper abdomen was found to be tender. Two separate masses were felt in the epigastrium and right hypochondrium. Blood count showed leukocytosis of 12 × 10 3 with 7% bands, 68 polys, 17% lymphs, 13% monos, 2% eosinophils and 1% atypical lymphocytes. Hemoglobin was 12 g/dl. Total bilirubin was 12.1 μmol/L, total protein 70 g/L, albumin 24 g/L, GOT 104 IU/L, GPT 79 IU/L, LDH 231 IU/L, GGTP 249 IU/L. Hepatitis B surface antigen (HBsAg) and Casoni's test were negative.Ultrasound imaging and CT scan revealed a slightly enlarged liver with irregular outlines. Several hepatic cysts of varying attenuations and a single splenic cyst were reported. The largest cystic space measured 8.3 × 6.8 cm. Pancreas and kidneys were free (Figure 1).Exploratory laparotomy was performed with the tentative diagnosis of multiple hydatid cysts. The liver was found to be studded with multiple cysts of varying sizes. The smallest was 2 and the largest was 80 mm in diameter. The nature of aspirated fluid varied from cyst to cyst with watery in some, and turbid or purulent in others. The bacteriological and cytological examination did not reveal any bacteria, ameba or malignant cells. All cysts were tense and firm in consistency. Two large cysts, one in each lobe, were deroofed and filled with omentum. The remaining cysts were aspirated. The histology of the resected cyst walls revealed fibrous tissue without any epithelial lining. The underlying liver tissue showed marked inflammatory changes with liver cell necrosis and hemorrhage. There was no evidence of hydatid scolices or ameba in the tissue (Figure 2). Post-operatively, the liver function tests returned to normal. The pyrexia and pain disappeared and the patient was discharged 13 days following laparotomy.

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“…Polycystic liver disease (PLD) is asymptomatic in most cases and is compatible with long-term survival [1]. However, cases that developed unusual symptoms such as jaundice and dyspnea were reported [2, 3].…”

Section: Introductionmentioning

confidence: 99%

Successful Left Trisegmentectomy for Polycystic Liver Disease Accompanied by Jaundice

Endo

Tanabe²,

Tanaka³

et al. 2001

Dig Surg

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Background: We report a case of severe polycystic liver disease (PLD) with jaundice in a 57-year-old woman who underwent successful left trisegmentectomy. Method: She was admitted for the first time in February 1992 to our hospital with a 7-year history of PLD, and became jaundiced in June 1995. Because normal liver parenchyma was confirmed mainly to the posterior segment, left trisegmentectomy was performed. Results: No postoperative complication occurred. The serum bilirubin level decreased promptly after the operation, but postoperative endoscopic retrograde cholangiography showed that the root of the posterior hepatic duct remained thin. Thus, the elimination of jaundice was presumed to have been caused by a decrease of intra-abdominal and peripheral biliary pressure, since a large volume of tissue had been removed from the peritoneal cavity. She has since remained well without any symptoms. Conclusion: This procedure is useful for severe PLD, because it can be performed safely and the symptoms disappear dramatically. However, further follow-up is needed to determine the long-term effects of this procedure, because the remaining liver has shown some increase in size.

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Congenital Cystic Disease of the Liver and Biliary System

Cited by 138 publications

References 5 publications

Cystic Dilatation of the Common Bile Duct

Cystic Dilatation of the Common Bile Duct

Congenital Multilocular Cysts of the Liver

Successful Left Trisegmentectomy for Polycystic Liver Disease Accompanied by Jaundice

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