Biliary atresia in England and Wales: results of centralization and new benchmark

Davenport, Mark; Ong, Evelyn; Sharif, Khalid; Alizai, Naved; McClean, Patricia; Hadžić, Nedim; Kelly, Déirdre

doi:10.1016/j.jpedsurg.2011.04.013

Cited by 231 publications

(220 citation statements)

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“…Centralization of these high-complex and low-volume procedures could potentially lead to further improvement of surgical technique and perioperative care and might have a beneficial effect on postoperative outcomes and survival as shown for other high complex (pediatric) surgical procedures [34][35][36]. Further research is necessary, since the effects of caseload and organization of care on outcomes for patients with hepatoblastoma have not yet been investigated.…”

Section: Discussionmentioning

confidence: 99%

Surgical treatment of childhood hepatoblastoma in the Netherlands (1990–2013)

et al. 2016

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Background Achievement of complete surgical resection plays a key role in the successful treatment of children with hepatoblastoma. The aim of this study is to assess the surgical outcomes after partial liver resections for hepatoblastoma, focusing on postoperative complications, resection margins, 30-day mortality, and long-term survival. Method Chart reviews were carried out on all patients treated for hepatoblastoma in the Netherlands between 1990 and 2013. Results A total of 103 patients were included, of whom 94 underwent surgery. Partial hepatectomy was performed in 76 patients and 18 patients received a liver transplant as a primary procedure. In 42 of 73 (58 %) patients, one or more complications were reported. In 3 patients, information regarding complications was not available. Hemorrhage necessitating blood transfusion occurred in 33 (45 %) patients and 9 (12 %) patients developed biliary complications, of whom 8 needed one or more additional surgical interventions. Overall, 5-year disease-specific survival was 82, 92 % in the group of patients who underwent partial hepatectomy, and 77 % in the group of patients who underwent liver transplantation. Conclusions Partial hepatectomy after chemotherapy in children with hepatoblastoma offers good chances of survival. This type of major surgery is associated with a high rate of surgical complications (58 %), which is not detrimental to survival.

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Section: Discussionmentioning

confidence: 99%

Surgical treatment of childhood hepatoblastoma in the Netherlands (1990–2013)

et al. 2016

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“…Surgical treatment consists of the Kasai hepatoportoenterostomy (KPE) (5). Adequate drainage of bile is achieved in 38% to 75% of cases (2,3,6,7). Studies with almost exclusively term BA patients showed that early KPE is associated with better transplant-free survival; a KPE after 60 days of age has a negative effect on transplant-free survival (2,(8)(9)(10).…”

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confidence: 99%

Preterm Infants With Biliary Atresia

Wessel

Boere²,

Hulzebos

et al. 2017

J. pediatr. gastroenterol. nutr.

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Objectives: Biliary atresia (BA) occurs in 0.54 of 10.000 of overall live births in the Netherlands. BA has an unfavorable prognosis: <40% of patients are cleared of jaundice after Kasai portoenterostomy (KPE), 4-year transplant-free survival rate is 46% and the 4-year survival rate is ∼75%. Little is known on difficulties in diagnosis and the outcome of BA in preterm infants. We aimed to analyze the incidence and outcome of BA in preterm infants in the Netherlands. Methods: Retrospective study including Dutch preterm infants treated for BA. Parameters included gestational age, congenital anomalies, age at KPE, days between first symptoms, and KPE and referral interval (first hospital to KPE). Outcome parameters were clearance of jaundice (COJ) and (transplant-free) survival. Data are presented as medians (ranges). Results: Included 28 preterm infants (13 boys/15 girls) between March 1988 and December 2015. The incidence of BA was 1.06 of 10.000 preterm live births. Gestational age was 34.8 (27.3–36.9) weeks. Congenital anomalies were present in 11 of 28 (39%) infants. Time between first symptoms and KPE was 57 (9–138) days. Referral interval was 28 (8–86) days. Age at KPE was 70 (35–145) days. COJ was achieved in 23% of cases. Four-year transplant-free survival rate was 21%. Four-year overall survival was 61%. Conclusions: BA has a higher incidence in the preterm population compared to the overall BA population. The outcome of BA in preterm infants is poor, regarding COJ and (transplant-free) survival. We speculate that timely recognition of BA-related signs and symptoms in preterm infants will improve prognosis.

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“…age at surgery, macroscopic appearance of the bile ducts, microscopic analysis of the resected specimen, and liver histopathology. 2,9 The extent of histological abnormality (degree of fibrosis) at the time of surgery may indicate a poorer prognosis, but this finding has not been consistent. The degree of portal hypertension at the time of the procedure is correlated with a shorter time to requiring liver transplantation, reflecting liver pathophysiology in a more functional way.…”

Section: Resultsmentioning

confidence: 97%

Solving difficult hepatobiliary problems in children

Millar

2012

S Afr Med J

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Biliary atresia in England and Wales: results of centralization and new benchmark

Cited by 231 publications

References 18 publications

Surgical treatment of childhood hepatoblastoma in the Netherlands (1990–2013)

Surgical treatment of childhood hepatoblastoma in the Netherlands (1990–2013)

Preterm Infants With Biliary Atresia

Solving difficult hepatobiliary problems in children

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