Pediatric liver transplantation for biliary atresia: results of primary grafts in 328 recipients1

Vo, Hanh D.; Evrard, V.; Vinh, Ha; Sokal, Étienne; Janssen, M.; Otté, Jean-Bernard; Reding, Raymond

doi:10.1097/01.tp.0000062570.83203.a3

Cited by 105 publications

(30 citation statements)

References 17 publications

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“…Despite HPE being performed in a timely fashion, liver transplantation (LT) is ultimately required for the majority of patients during childhood 4, 5 . Although single center 6-8 and multi-center 9-11 characterizations of the health status and medical outcomes in patients with BA who have undergone LT are available, the long-term outcomes in older children with BA living with their native livers have not been examined in detail in large patient populations, and are limited to small single-center retrospective experiences 12-18 . Progression to chronic liver disease was avoided in only 11% of 244 ten-year survivors of HPE performed at King's College Hospital over the 12 year period of 1979 to 1991 19 .…”

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confidence: 99%

Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with Their Native Livers: Results from a North American Multicenter Consortium

Haber

Magee

et al. 2014

The Journal of Pediatrics

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Objectives To examine the medical status of children with biliary atresia (BA) with their native livers after hepatic portoenterostomy (HPE) surgery. Study design The Childhood Liver Disease Research and Education Network (ChiLDREN) database was utilized to examine subjects with BA living with their native livers 5 or more years after HPE and to describe the prevalence of subjects with BA with an “ideal” outcome, defined as no clinical evidence of chronic liver disease, normal liver biochemical indices (aspartate aminotransferase, alanine aminotransferase, gamma glutamyl transpeptidase, platelet count, total bilirubin, International Normalized Ratio, and albumin) and normal Health-Related Quality of Life (HRQOL) 5 or more years after HPE. Results Children with BA (n=219; 43% male) with median age 9.7 years were studied. Median age at HPE was 56 (range 7-125) days. Median age- and sex-adjusted height and weight Z-scores at 5 year follow-up were 0.487 (interquartile range [IQR]: -0.27 to 1.02) and 0.00 (IQR: -0.74 to 0.70), respectively. During the 12 preceding months, cholangitis and bone fractures occurred in 17% and 5.5%, respectively. HRQOL was reported normal by 53% of patients. However, only 1.8% met the study definition of “ideal” outcome. Individual tests of liver synthetic function (TB, Alb, and INR) were normal in 75%, 85% and 73% of the study cohort. Conclusion Cholangitis and fractures in long-term survivors underscore the importance of ongoing medical surveillance. Over 98% of this North American cohort of subjects with BA living with native livers 5 or more years after HPE have clinical or biochemical evidence of chronic liver disease.

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confidence: 99%

Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with Their Native Livers: Results from a North American Multicenter Consortium

Haber

Magee

et al. 2014

The Journal of Pediatrics

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“…Bowel perforation is a noteworthy complication that occurs after LT in BA patients [8–11]. The incidence of this complication after pediatric LT is reported to be 6.4–20% [17, 19–23].…”

Section: Discussionmentioning

confidence: 99%

“…However, LT remains the ultimate surgery for BA and two-third of BA patients will require LT due to the progression of chronic liver disease [5, 7]. Many reports that have investigated the outcomes of LT for BA have evaluated the patients in childhood [8–11]. More than 50 years have passed since Kasai introduced KPE in 1959 [3]; thus in some cases, patients reached adulthood with their native liver [12, 13].…”

Section: Introductionmentioning

confidence: 99%

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Bowel perforation after liver transplantation for biliary atresia: a retrospective study of care in the transition from children to adulthood

et al. 2016

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PurposeWe evaluated the outcomes of liver transplantation (LT) in pediatric and adult patients with biliary atresia (BA). We focused on bowel perforation after LT (BPLT) as the most common surgical complication and analyzed its risk factors.MethodsThis was a retrospective analysis of 70 BA patients who underwent LT. The patients were divided into three groups according to the timing of LT: within the first year of age (Group A), between 1 and 12 years of age (Group B), and after 12 years of age (Group C). The outcomes of LT and the clinical presentations of BPLT were compared. The surgical variables of patients with and without BPLT were analyzed to assess the risk factors.ResultsThe timing of LT did not affect patient survival. The incidence of BPLT was significantly higher in Group C. In Group C, BPLT progressed to severe peritonitis. No cases of BPLT-associated mortality were observed. A multivariate analysis revealed that a prolonged operative time for LT was an independent risk factor (p = 0.03).ConclusionThe clinical course after transplantation was complicated after adolescence. BPLT should be strongly suspected and relaparotomy should be performed in a timely manner for patients undergoing LT after adolescence.

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“…Portal vein complications following liver transplantation are relatively uncommon, with an incidence of only 1% to 6% (12). They may result from the use of faulty surgical techniques, the excessive length of the interposed vascular graft, vessel misalignment, hypercoagulable states, previous portal vein surgery or previous portal vein thrombosis (9).…”

Section: Postoperative Evaluationmentioning

confidence: 99%

Multi-Slice Spiral CT of Living-Related Liver Transplantation in Children: Pictorial Essay

2004

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In pediatric living-related liver transplantation, preoperative evaluation of the recipient is important for surgical planning, while the accurate diagnosis of postoperative complications is essential for graft salvage. Multiplanar and three-dimensional imaging using multi-slice spiral CT can be used for preoperative vascular imaging, as well as for evaluating postoperative complications. In this essay, we describe the usefulness of multi-slice CT, combined with a variety of different reconstruction techniques, for the preoperative evaluation of transplant recipients. In addition, we demonstrate the multi-slice CT findings of postoperative complications, including vascular stenosis or thrombosis, bile duct leak or stricture, and extrahepatic fluid collection.

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Pediatric liver transplantation for biliary atresia: results of primary grafts in 328 recipients1

Abstract: Best results in children undergoing transplantation beyond 6 years indicate the importance of performing a KHPE as the first therapeutic step in BA; innovative surgical techniques, particularly LR donor graft, allowed successful transplantation in infants with early failure of KHPE.

Cited by 105 publications

References 17 publications

Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with Their Native Livers: Results from a North American Multicenter Consortium

Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with Their Native Livers: Results from a North American Multicenter Consortium

Bowel perforation after liver transplantation for biliary atresia: a retrospective study of care in the transition from children to adulthood

Multi-Slice Spiral CT of Living-Related Liver Transplantation in Children: Pictorial Essay

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