Liver and systemic hemodynamics in cirrhotic children : contributions to the physiopathology and to the surgical algorithm in pediatric liver transplantation

Liver and systemic hemodynamics are altered in cirrhotic adults. This work attempts to determine if these hemodynamic parameters are perturbed in cirrhotic children as in adults. The characterization of these hemodynamic disturbances could contribute to a better understanding of the hepatic and systemic physiopathology in pediatric cirrhosis. They also could assist surgeons in the establishment of a surgical algorithm for the pediatric liver transplant procedure.(MED - Sciences médicales) -- UCL, 201

Meso-Rex shunt for immediate portal revascularization in pediatric liver transplantation: First report.

Rivera J, Fusaro F, de Magnée C, Clapuyt P, Reding R. Meso-Rex shunt for immediate portal revascularization in pediatric liver transplantation: First report. Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S. Abstract:  We describe the case of a 13-month-old girl transplanted for biliary atresia with PV hypoplasia. She received the left liver lobe of her mother and presented intraoperative portal thrombosis. Because of technical reasons, the opportunity to have conventional PV reconstruction using the donor left PV stump was lost. Immediate conversion to a meso-Rex shunt, using the recipient jugular vein as a bridge between the superior mesenteric vein and the graft Rex recessus, allowed excellent portal revascularization of the transplant. We suggest that synchronous meso-Rex shunt may constitute a valid alternative to truncal PV anastomosis during pediatric LT

Preoperative Tracheoscopy for Esophageal Atresia and/or Congenital Esoaerian Fistula: Experience at Cliniques Universitaires Saint Luc in Brussels

Introduction: Congenital septal anomalies between the trachea and the esophagus are rare conditions. It seemed to us interesting to recall the contribution of tracheoscopy in the diagnostic and therapeutic strategy of esophageal atresia and congenital esoaerian fistulas. Patients and methods: This is a retrospective study between June 1994 and June 2014 of children who underwent a tracheoscopy, at the Saint-Luc University Clinics in Brussels, the diagnostic set of esophageal atresia (EA) or a congenital esoaerian fistula was suspected. Results: A total of 43 children with esophageal atresia or congenital esoaerian fistula underwent tracheoscopy. Before the tracheoscopy, the diagnosis of the anatomical type of atresia of the esophagus and esoaerian fistula was as follows: type C, 34 (79.1%); type A, 4 (9.3%); type E, 5 (11.6%). After performing the tracheoscopy, the diagnosis was changed as follows: type C, 34 (79.1%); type A, 3 (7%); type E, 4 (9.3%); type B, 1 (2.3%), a patient with a tracheoesophageal laryngo cleft (2.3%). Tracheoscopy also made it possible to find 2 cases of tracheomalacia, 2 cases with 3 bronchial tubes and one case associating a diverticulum of the trachea. No complications were correlated with the performance of the tracheoscopy. Conclusion: Our study confirms the benefits of tracheoscopy in the laden price of esophageal atresia or congenital esoaerian fistula

Complications after conservative treatment of blunt liver trauma in children.

This series fully validates conservative approach of BLT in hemodynamically stable children. Complications of such approach are uncommon; many can be successfully treated using minimally invasive techniques with very satisfying results

Vanishing gastroschisis visualized by antenatal ultrasound: a case report and review of literature.

We report a case of vanishing gastroschisis visualized by antenatal ultrasound with a 7-year long term follow-up. Currently, the child is still dependent on daily parenteral nutrition with no signs of hepatotoxicity. To our knowledge, it's the fourth case with a long-term follow-up. Vanishing gastroschisis is a rare complication of gastroschisis. However, physicians should be aware of it because its prognosis is worse than classical gastroschisis. When a vanishing gastroschisis is visualized or suspected by antenatal ultrasound, prenatal counseling is required with explanations about the risk of short bowel syndrome, the need of parenteral nutrition and related complications (inflammatory colitis, sepsis, liver failure and organ transplant). Mortality rate was initially around 93%, and dropped to 27% after the years 2000 (versus 10% for classical gastroschisis). After birth, all children will require surgery, and sometimes autologous gastro-intestinal reconstruction. Most survivors (68%) could be taken off the TPN. Unfortunately, long-term outcomes for children with vanishing gastroschisis are still missing in current literature

Impact of pre-transplant liver hemodynamics and portal reconstruction techniques on post-transplant portal vein complications in pediatric liver transplantation : a retrospective analysis in 197 recipients

Portal vein (PV) complications are the most frequent vascular complications in pediatric liver transplant (LT). We hypothesized that pre-LT liver hemodynamic parameters and PV reconstruction technique could predict the risk of PV complications post-LT

Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II.

Progressive familial intrahepatic cholestasis (PFIC) can cause intense pruritus that is refractory to medical therapy. Surgical biliary diversion techniques, including partial internal biliary diversion (PIBD), have been developed over the years to relieve pruritus without requiring liver transplantation. No clinical or genetic features can currently predict postoperative pruritus response. We present three PFIC type 2 (PIFC 2) patients who underwent transient endoscopic nasobiliary drainage (NBD) prior to PIBD surgery. Two patients repeatedly responded to NBD and presented with complete pruritus resolution after subsequent PIBD. NBD failed technically in the third patient, and PIBD was partially successful. Mild post-endoscopic biological pancreatitis occurred in 2/6 NBD procedures and resolved spontaneously. The only adverse effect observed within 7 years post-PIBD was very mild transient osmotic diarrhea.Conclusion: Our limited data suggest that NBD is a safe and effective way to predict pruritus response before performing permanent biliary diversion surgery in PFIC patients.What is Known:• Surgical biliary diversion techniques have been developed to relieve intractable pruritus in progressive familial intrahepatic cholestasis (PFIC).• No clinical or genetic features can currently predict pruritus response to surgery.What is New: • Our data suggest that nasobiliary drainage could be a safe and effective tool to predict pruritus response to biliary diversion and avoid unnecessary surgery in PFIC patients

Recovery of graft steatosis and protein-losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child.

Nicastro E, Stephenne X, Smets F, Fusaro F, de Magnée C, Reding R, Sokal EM. Recovery of graft steatosis and protein-losing enteropathy after biliary diversion in a PFIC 1 liver transplanted child. Abstract:  PFIC 1 is a genetic disorder characterized by hepatic and gastrointestinal disease, often requiring LT during childhood. Extrahepatic symptoms, such as diarrhea and malabsorption, do not improve or may be aggravated after LT, as graft steatosis or steatohepatitis as consequences of the interaction between transplanted liver and native bowel. We describe a patient with PFIC 1 who presented with cholestasis in infancy, who developed intractable pruritus and liver fibrosis. The child underwent living donor LT at 3.6 yr of age, and he early developed severe refractory diarrhea, secondary malabsorption with protein-losing enteropathy, and an early fatty liver disease trough graft steatohepatitis. As the response to cholestyramine was unsatisfactory, we decided to perform an EBD by using the jejunal loop used for the cholangiojejunostomy. Diarrhea resolved rapidly after surgery. He remained well after six months following biliary diversion, with normal stool output and no protein loss. We documented a dramatic improvement of graft steatosis at histology as well as normalization of liver function test. EBD can be considered a valuable treatment option to avoid organ disfunction and loss in PFIC 1 transplanted patients who develop graft steatohepatitis