The efficacy of surgical shunts to treat severe portal hypertension after a Kasai procedure for biliary atresia.

BACKGROUND: To assess the outcome of patients with biliary atresia (BA) who underwent a surgical shunt (SS) for severe portal hypertension (PH) following a Kasai procedure. METHODS: We collected and analyzed the data and outcomes of patients with BA who underwent SS for severe PH following a Kasai procedure between 1974 and 2014, focusing on complications related to the procedure, overall survival (OS), and transplant-free survival (TFS). RESULTS: SS was performed at a median age of 5.5 years [2-13.5] in 38 patients. Conjugated bilirubin level (cBL) was ≤20 µmol/l in 24 patients at time of SS. Median follow-up was 15 years [1-32]. OS at 5 and 10 years was 91% and 87% respectively. TFS at 5 and 10 years was 84% and 70% respectively. Long-term complications included hepatic encephalopathy in 9 patients, and hepatopulmonary syndrome in 3. At last follow-up, 10/14 patients without LT and 18/ 24 who had a delayed LT at a median delay of 11 years [1.5-22] were alive. CONCLUSION: Surgical shunt for severe portal hypertension in biliary atresia may delay the need for liver transplantation. However complications are indications for transplantation. LEVEL OF EVIDENCE: Type of study: Therapeutic. Level of evidence III.

Twin-to-twin transfusion syndrome: perinatal outcome and recipient heart disease according to treatment strategy.

AIM: The aims of the study were to compare perinatal outcome and assess recipient cardiac disease according to treatment strategy (amnioreduction (AR), laser or selective feticide). METHODS: We retrospectively reviewed 81 consecutive cases of twin-to-twin transfusion syndrome diagnosed before 28 weeks between 1993 and 2007. RESULTS: Although fetuses treated by laser were younger at diagnosis (median 20.4 vs. 22.4 weeks, P = 0.01), they were significantly older at birth (median 33.6 vs. 28.5 weeks, P = 0.004) than those treated by AR. Neonatal morbidity was globally lower after laser than AR, and cardiac insufficiency tended to be less frequent (31% vs. 57%, P = 0.09). There was a trend towards increased perinatal survival after laser treatment (68% vs. 49%, P = 0.1). Heart failure was the cause of death in half (23/46) of the recipients. Fetal heart failure leading to death was 2.7 times more frequent after AR than after laser (n = 11 vs. n = 4), and all four neonatal cardiac deaths occurred after AR. Compared with laser, selective feticide did not further improve the outcome. CONCLUSIONS: Heart failure was an important cause of perinatal morbidity and death. However, laser therapy resulted in a longer diagnosis-delivery interval and lower global neonatal morbidity than AR, with a trend towards increased perinatal survival. Improved outcome after laser treatment compared with AR might be related to its impact on recipient heart disease.

[Rupture of the renal artery and renal parenchyma in a pregnant woman with vascular form of Ehlers-Danlos syndrome]. / Rupture de l'artère rénale et du parenchyme rénal chez une patiente enceinte, atteinte du Syndrome de Ehlers-Danlos de type vasculaire.

A case of intraparenchymal renal haemorrhage associated with renal artery dissection, occurring at the end of pregnancy in a woman with a vascular form of Ehlers-Danlos syndrome is reported for the first time. This cases illustrates the potential risk of this syndrome and the importance of multidisciplinary management to ensure an appropriate diagnostic and therapeutic strategy. Magnetic resonance imaging is useful in this setting to elucidate complex cases of renal colic in pregnant women presenting an increased risk of ischaemic or haemorrhagic disorders.