Biliary atresia and the polysplenia syndrome: Its impact on final outcome

Vázquez, Juan José; Gutiérrez, Juan Carlos López; Gámez, M; López‐Santamaría, Manuel; Murcia, J; Larrauri, J; Díaz, María Eugenia López-Jacoiste; Jara, Paloma; Tovar, Juan A.

doi:10.1016/0022-3468(95)90062-4

Cited by 75 publications

(57 citation statements)

References 4 publications

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“…9,[21][22][23] The present study shows the same trend in long-term results. Similarly, the unfavorable prognostic value of the polysplenia syndrome, 20,24 which was not found in some studies, 21,25 seems confirmed by our long-term results; in the present series, only 1 patient with this syndrome survived with a native liver for more than 20 years.…”

Section: Discussionsupporting

confidence: 85%

Outcome in adulthood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liver

et al. 2005

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To define the long-term prognosis of children undergoing the Kasai operation for biliary atresia, a retrospective study was undertaken comprising 271 patients operated between 1968 and 1983. Twenty years after surgery, 63 (23%) were alive with their native liver. Serum bilirubin was normal in 21 of these patients, 12 also had normal serum aminotransferase and ␥-glutamyltransferase activities, all but 2 had signs of cirrhosis, 44 had signs of portal hypertension, 19 had late bacterial cholangitis, and 6 had gallstones. Seven female patients gave birth to 9 children, and 3 male patients fathered 6 children. After age 20, 2 patients died of liver failure and 14 underwent or are awaiting liver transplantation. Twenty-year survival with native liver was significantly better in children with biliary atresia restricted to the hepatic ducts or with cysts at the porta hepatis. In conclusion, in the long term, less than 18% of infants with biliary atresia who are treated with corrective surgery may avoid liver transplantation, but even these patients require assiduous lifelong care. (HEPATOLOGY 2005;41:366-371.)

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Section: Discussionsupporting

confidence: 85%

Outcome in adulthood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liver

et al. 2005

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“…In specialized institutions where both operations are performed, reported overall survival in the subgroup of BA patients who underwent a Kasai operation followed by LT if needed ranges from 66% to 100%. 6,[15][16][17][18] This wide range of results does not take into account untreated patients and patients directed to de novo LT. This national study is the first study of the overall prognosis of BA including all patients with BA from the time of diagnosis, since both the Kasai operation and LT became widely available: 5-and 10-year overall survival rates are 70% and 68%, respectively.…”

Section: Discussionmentioning

confidence: 99%

“…[4][5][6][7][8][9] Age at Kasai operation and anatomical pattern of atresia were confirmed as prognostic factors of Kasai operation. 16,[21][22][23][24] The impact of polysplenia is more controversial, 16,18,21 probably because of its rarity, which requires large numbers of patients for statistical analysis.…”

Section: Discussionmentioning

confidence: 99%

Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996

et al. 1999

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Since the sequential treatment of Kasai operation with or without liver transplantation became available, the overall prognosis of biliary atresia remains unclear. This study examined the prognostic factors from diagnosis. All patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed. Actuarial survival rates were calculated for survival with native liver, survival after liver transplantation, and overall survival. Potential prognostic factors were analyzed using the logrank test and the Cox model. A total of 472 patients were identified. Ten-year overall survival was 68%. Independent prognostic factors for overall survival were (S ‫؍‬ 10-year rates) performance of Kasai operation (performed: S ‫؍‬ 69%; not performed: S ‫؍‬ 50%), age at Kasai operation (I45 days: S ‫؍‬ 80%; G45

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“…[6][7][8] However, when left lateral segment grafts are used, the results have been variable (Table 1). In this situation, we believe an important factor is the success of the hepatic venous anastomosis, which is dependent on the length and direction of the graft left hepatic vein and the recipient hepatic venous cuff.…”

Section: Discussionmentioning

confidence: 99%

“…If the graft left hepatic vein can be fashioned to produce a transverse orifice, it may be anastomosed successfully to the inferiorly facing recipient hepatic venous cloaca. 6,9 However, if the graft left hepatic vein is facing posteromedially, it can cause 2 potential problems 10 : (1) venous outflow obstruction may result from kinking caused by misalignment of the posteromedially facing graft left hepatic vein and the inferiorly facing recipient hepatic venous cloaca, and (2) antigravity biliary drainage and stasis may occur when the graft is rotated in an attempt to avoid such hepatic venous misalignment.…”

Section: Discussionmentioning

confidence: 99%

Living related liver transplantation in biliary atresia with absent inferior vena cava

et al. 2001

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The success of the triangulation technique for hepatic venous anastomosis in left lateral segment liver transplantation has led to standardization of this procedure. We report a case of syndromic biliary atresia with absent inferior vena cava in which we constructed a neo cava to implant a living related left lateral segment graft by using the triangulation technique. (Liver Transpl 2001;7:376-377.)

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Biliary atresia and the polysplenia syndrome: Its impact on final outcome

Cited by 75 publications

References 4 publications

Outcome in adulthood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liver

Outcome in adulthood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liver

Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996

Living related liver transplantation in biliary atresia with absent inferior vena cava

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