Percutaneous Treatment of Biliary Strictures After Pediatric Liver Transplantation.

BACKGROUND Biliary strictures (BS) are frequent after pediatric liver transplantation (LTx) and in spite of ongoing progress, they remain a significant cause of morbidity. In children, the majority of reconstruction is hepatico-jejunal anastomosis (HJA). The aim of this study was to analyze our experience in percutaneous transhepatic treatment of BS. MATERIAL AND METHODS Between 1998 and 2014, 589 (269 living donor) pediatric LTx were performed in our institution. We retrospectively reviewed clinical data of patients with HJA who developed BS and who underwent percutaneous transhepatic biliary drainage (PTBD). RESULTS Out of 400 patients with HJA, 35 patients developed BS. There were 27 cases (77%) of anastomotic BS (ABS) and 8 cases (23%) of multilevel BS (MBS). Ninety-two PTBD sessions (2.5 per patient) were performed, with successful outcomes in 20 cases (57%). Fifteen patients, after failed PTBD, underwent surgery which was successful in 11 cases. Overall good outcomes were achieved in 31 cases (88.5%). The most common complication of PTBD was cholangitis which occurred in 5.4% of the cases. We did not find any risk factors for PTBD failure, except for treatment occurring before 2007. CONCLUSIONS Percutaneous treatment is effective and safe in BS and is recommended as a first-line approach. The majority of patients in our study required multiple interventions, however, the overall risk of complications was low. Surgery is essential in selected cases and always should be considered if PTBD fails.

Endoscopic treatment in biliary strictures after pediatric liver transplantation.

BACKGROUND AND AIM: The aim of the study was to assess efficacy and safety of endoscopic treatment in BS after pediatric LTx. METHODS: We retrospectively reviewed data of patients with DDA who developed BS and underwent ERCP. RESULTS: Of 189 transplanted patients with DDA, strictures developed in 30 (16%). In this subgroup, the median age at LTx was 14.7 (1.5-17.6) and follow-up period was 3.9 (1.3-11.3). ABS were in 76% and NABS in combination with ABS in 24% of patients. Overall, 95 ERCP sessions (3.0 per patient) were performed with successful outcome in 22 (73%) cases. Duration of treatment was 9.1 (1.8-24.1) months. Five patients underwent surgical revision and three patients retransplantation (10%). Risk factors of endoscopy failure were HCV or HBV infection, prolonged CIT and treatment before 2007. The most common complications after ERCP were cholangitis (8.2%) and pancreatitis (4.2%). There were worse overall prognosis and higher risk of post-ERCP complications in NABS. CONCLUSIONS: ERCP is safe and effective in the majority of patients with post-transplant duct-to-duct BS, and it is currently recommended as the first-line treatment.

The limited prognostic value of liver histology in children with biliary atresia.

UNLABELLED: Background and rationale for the study. The aim of the study was to determine the prognostic value of histopathological findings with special care to the severity of liver fibrosis at the moment of hepatoportoenterostomy (HPE) in children with biliary atresia (BA). We performed analysis of 142 wedge liver biopsies taken at the time of HPE. All patients were operated by the same surgical team between 1995 and 2007. According to the outcome 6 months after HPE patients were divided into prognostic groups: group 1-bilirubin level < 2 mg% (n = 65), group 2-bilirubin level > 2 mg% (n = 77). Liver biopsies were re-evaluated according to the extended histopathological protocol and then were compared between the prognostic groups. Survival with native liver (SNL) estimates were performed in regard to severity of liver fibrosis. RESULTS: Survival with native liver estimates after 2, 5 and 10 years in patients after successful operation were 96%, 91%, 75% vs. 30%, 11%, and 5% if operation failed (p < 0.001). There was no difference between groups in the following variables: fibrosis (p = 0.69), portal inflammation (p = 0.99), lobular inflammation (p = 0.95), cholangiolitis (p = 0.23), accumulation of bile pigments (zone 1:p = 0.49; zone 2:p = 0.51; zone 3:p = 0.48), bile plugs in canaliculi (p = 0.12), bile plugs in ducts (p = 0.32), bilirubinostasis in hepatocytes (p = 0.45), bile ductular proliferation (p = 0.59), ductal plate malformation (p = 0.12), focal necrosis (p = 0.44), giant cell transformation (p = 0.45), haematopoesis (p = 0.52), ductopenia (p = 0.46), microabscesses (p = 0.49), ballooning of hepatocytes (p = 0.08). The actuarial 5/10-year SNL was not dependent on severity of liver fibrosis (log-rank test p = 0.84). The severity of fibrosis corresponded neither with the age at HPE nor with the laboratory findings before operation but increased the risk of portal hypertension. CONCLUSION: Liver histology at the time of HPE is of limited value in prognosis making in BA.

Clinical and molecular characteristics of two transaldolase-deficient patients.

UNLABELLED: Transaldolase (TALDO) deficiency is a rare metabolic disease in the pentose phosphate pathway, which manifests as a severe, early-onset multisystem disease. The body fluids of affected patients contain increased polyol concentrations and seven-carbon chain carbohydrates. We report the molecular and clinical findings in two recently diagnosed transaldolase-deficient children, both presented at birth. During infancy, they presented thin skin with a network of visible vessels, spider telangiectasias and multiple haemangiomas. Such unusual skin changes are characteristic of liver damage. Later, the patients developed rapidly progressive nodular liver fibrosis, tubulopathy and severe clotting disturbances. The clinical features of these patients were in line with previously studied patients with transaldolase deficiency. The diagnosis was established by detecting high concentrations of erythritol, ribitol, arabitol, sedoheptitol, perseitol, sedoheptulose and sedoheptulose-7-phosphate in the urine. Detection was made by gas chromatography and liquid chromatography-tandem mass spectrometry and then confirmed by molecular analysis of the TALDO gene. CONCLUSION: Transaldolase deficiency, a rare early-onset multisystem disease, should be considered by neonatologists, paediatricians, hepatologists and nephrologists in the differential diagnosis of patients presenting hepatosplenomegaly, thrombocytopenia, anaemia, bleeding diathesis, liver failure and tubulopathy.