Frédéric Gauthier

Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening

BACKGROUND. Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists and if this effect persists with extended follow-up. In this study we examined the relationship between increased age at surgery and its results in adolescence. METHODS. All patients with biliary atresia who were living in France and born between 1986 and 2002 were included. Median follow-up in survivors was 7 years. RESULTS. Included in the study were 743 patients with biliary atresia, 695 of whom underwent a Kasai operation; 2-, 5-, 10-, and 15-year survival rates with native liver were 57.1%, 37.9%, 32.4%, and 28.5%, respectively. Median age at Kasai operation was 60 days and was stable over the study period. Whatever the follow-up (2, 5, 10, or 15 years), survival rates with native liver decreased when age at surgery increased (≤30, 31–45, 46–60, 61–75, and 76–90 days). Accordingly, we estimated that if every patient with biliary atresia underwent the Kasai operation before 46 days of age, 5.7% of all liver transplantations performed annually in France in patients younger than 16 years could be spared. CONCLUSIONS. Increased age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. These findings indicate a rational basis for biliary atresia screening to reduce the need for liver transplantations in infancy and childhood.

Laparoscopic Left Lateral Sectionectomy in Living Donors: Safety and Reproducibility of the Technique in a Single Center

Background Data and Objective:Left lateral sectionectomy for liver transplantation in children performed through laparoscopy is an innovative procedure that was developed by considering our acquired experience in both laparoscopic liver resection and graft harvesting in living donors. The main goal was to minimize donor morbidity while preserving the abdominal wall. Herein, we report the technical feasibility and reproducibility, and compared it with open liver resection (OLR). Methods:Sixteen successive donors underwent a laparoscopic liver resection (LLR) from 2001 to 2005. They were compared with 14 other donors who underwent a standard open liver resection (OLR) during a first period (1998–2004). First, this report describes the technical features of laparoscopic resection. Second, perioperative morbidity and graft characteristics were compared according to the use or not of the laparoscopic approach. Results:Laparoscopic harvesting was successfully performed in 15 of 16 cases in an intention-to-treat basis. One conversion was required to ensure the quality of the laparoscopic repair of a left portal vein injury occurring during the pedicle dissection. No specific complication related to laparoscopy was observed. As compared with OLR, the operation was longer (320 ± 67 vs. 244 ± 55 minutes, P < 0.005). The blood loss was significantly lower in the LLR group (18.7 ± 44.2 vs. 199.2 ± 185.4 mL, P < 0.005). The morbidity rate was similar in both groups (18.7% in LLR vs. 35.7% in OLR). One donor in the LLR group experienced a bile leak treated by redo laparoscopy. Grafts were anatomically similar irrespective of the use of laparoscopy. The duration of hospital stay and use of self-infused morphine pump was not different between the 2 groups. Conclusion:Left lateral section harvesting by laparoscopy is a safe and reproducible procedure, allowing to obtain similar grafts as compared with laparotomy and can therefore be recommended to transplant centers that have previous experience in laparoscopic liver resection.

Management of patients with biliary atresia in France: Results of a decentralized policy 1986-2002†‡§

This study analyzed the results of the decentralized management of biliary atresia (BA) in France, where an improved collaboration between centers has been promoted since 1997. Results were compared to those obtained in England and Wales, where BA patients have been centralized in three designated centers since 1999. According to their birth dates, BA patients were divided into two cohorts: cohort A, with patients born between 1986 and 1996, had 472 patients; and cohort B, with patients born between 1997 and 2002, had 271 patients. Survival rates were calculated according to the Kaplan‐Meier method and compared by using the log rank test and the Cox model. Four‐year overall BA patient survival was 73.6% (95% CI 69.5%‐77.7%) and 87.1% (CI 82.6%‐91.6%) in cohorts A and B, respectively (P < .001). Median age at time of the Kasai operation was 61 and 57 days in cohorts A and B, respectively (NS). Four‐year survival with native liver after the Kasai operation was 40.1% and 42.7% in cohorts A and B, respectively (NS): 33.9% (cohort A) and 33.4% (cohort B) in the centers with two or fewer caseloads a year, 30.9% (cohort A) and 44.5% (cohort B) in the centers with 3‐5 cases/year, 47.8% (cohort A) and 47.7% (cohort B) in the center with more than 20 caseloads a year. In cohorts A and B, 74 (15.7%) and 19 (7%) patients, respectively, died without liver transplantation (LT). Four‐year survival after LT was 75.1% and 88.8% in cohorts A and B, respectively (P = .006). In conclusion, BA patients currently have the same chance of survival in France as in England and Wales. The early success rate of the Kasai operation remains inferior in the centers with limited caseloads in France, leading to a greater need for LTs in infancy and early childhood. (HEPATOLOGY 2006;44:75–84.)

Biliary complications after transplantation in children: Role of imaging modalites

Among a series of 140 liver transplantations in children, 21 biliary complications (BC) (15%) are reported. BC were identified from 2 days to 3 months after LT. Positive US findings were present in 20 cases. Cholangiography was obtained by opacification of a surgical drain in 3 cases, per-operatively in 3 and by PTC in 15. Drainage was placed in 9 and ballon dilatation performed in 2. Causes of BC include hepatic artery (HA) thrombosis in 7, HA stenosis in 1, anastomosis stricture in 7, anastomosis kink in 3, mucocele of cystic duct remnant in 2 and sludge in 1. Treatment was surgical in all, but 2 cases were treated percutaneously. There is a great difference in severity of prognosis between complications secondary to HA thrombosis and isolated BC. Role of US in diagnosis and of PTC and interventional radiology in treatment are emphasized.

Radiological treatment of common bile duct lithiasis in infancy

Abstractthe authors report a series of 10 infants aged from 20 days to 11 months, presenting with CBD lithiasis, explored and cured by radiological procedure. US showed BD dilatation in 9 cases, sludge in the GB in 5 and in the CBD in 2. Histological findings of cholangitis were present in 4 infants. PTC was performed by GB puncture in 6 and BD puncture in 4. There was evidence of a filling defect in CBD but no anatomical anomaly. Blackish concretions were removed through a side-holes catheter or pushed in the duodenum by washing with saline. An external drainage was left a few days to allow control cholangiogram. Three infants underwent subsequent surgery but no residual lithiasis was found. No recurrence has occurred with a follow-up ranging from 10 months to 7 years. Mechanisms of this entity are discussed.

Outcome of central hepatectomy for hepatoblastomas

BACKGROUND/PURPOSE Central hepatoblastomas (CHBL) involving liver segments (IV + V) or (IV + V + VIII) are in contact with the portal bifurcation. Their resection may be achieved by central hepatectomy (CH) with thin resection margins on both sides of the liver pedicle, by extended right or left hepatectomy with thin resection margins on one side, or by liver transplantation with thick free margins. The aim of this study is to assess the operative and postoperative outcome of CH for hepatoblastoma. METHODS This was a retrospective monocentric study of 9 patients who underwent CH for CHBL between 1996 and 2008. RESULTS The operative time was 4 hours 50 minutes (2 hours 20 minutes to 7 hours), vascular clamping lasted 30 minutes (0-90 minutes), and the amount of blood cell transfusion was 250 mL (0-1800 mL). Two patients had biliary leakage requiring percutaneous drainage. Median follow-up time was 27 months (14-120 months). All of 8 nonmetastatic patients are alive and disease-free; 1 metastatic patient died of recurrent metastases at last follow-up. Although 3 of 9 patients had surgical margins less than 1 mm, none, including the patients who died from metastases, had local recurrence. CONCLUSIONS Our study demonstrates the feasibility of CH for CHBL without operative mortality or local recurrence. Central hepatectomy is an alternative to extensive liver resections in selected patients.

Variability of clinical presentation of hepatic artery thrombosis in pediatric liver transplantation: Role of imaging modalities

Among a series of 90 pediatric liver transplantations, 9 cases of hepatic artery thrombosis (HAT) in 8 patients are reported. All cases were diagnosed in the first two weeks and confirmed angiographically and surgically. Clinical presentation was often unreliable with only 2 cases showing the typical pattern of massive hepatic necrosis. Five cases presented with biliary complications and in 2 cases, HAT was discovered fortuitously on duplex sonography. The role of imaging modalities is emphasized. Duplex sonography is the best non-invasive screening method and we recommend a routine daily examination in the first 2 weeks. CT is the most useful method to assess the extension of liver infarction. PTC remains necessary to evaluate biliary complications and to plant the best therapeutic approach in this devastating event. Two patients died, 2 had to be retransplanted (one patient twice) and are doing well, 2 are on a waiting list for re-transplantation and one underwent an emergency surgical arterial desobstruction and is completely asymptomatic.

Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children

BACKGROUND Liver nodules have been reported after portal systemic shunt surgery (PSSS) in animal experiments or in humans with liver cirrhosis. The aim of our study was to assess the incidence of liver nodules after surgery for extrahepatic portal vein obstruction (EHPVO) in children without associated liver disease. METHODS We retrospectively reviewed the charts of 45 children who had surgery from 1979 to 2005 for EHPVO in our institution, consisting of 38 PSSS and 7 portal reperfusion procedures (PRPs). We assessed the presence of liver nodules on ultrasonography. RESULTS Of 45 patients, 7 (15%) had liver nodules during a median of 80 months of follow-up. All the nodules occurred after PSSS. Five nodules were subjected to biopsy; we found 2 liver cell adenomas and 3 focal nodular hyperplasias. CONCLUSIONS In this study, liver nodules occurred in 18% of cases after PSSS for EHPVO in children and not after PRP. As many children have undergone PSSS throughout the world, the presence of liver nodules should be considered during the follow-up of those patients.

Prenatal and postnatal Ciliated Hepatic Foregut Cysts in infants.

PURPOSE Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC. METHODS We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis. RESULTS Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery. Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia. CONCLUSION In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.

SFCP CO-74 - Chirurgie des métastases hépatiques des néphroblastomes : expérience monocentrique

Introduction Les metastases hepatiques de nephroblastome (MHN) sont rares et leur prise en charge est specialisee. Materiel et methodes Etude monocentrique retrospective (1993–2010) incluant les patients (pts) ayant une chirurgie hepatique pour MHN. Resultats Dix pts ont ete identifies : 4 pts avec metastases synchrones, 6 pts metachrones (delai moyen de rechute de 23 mois [1–123]). Toutes touchaient le lobe hepatique droit. Elles etaient multiples 4 fois et unique 6 fois. Une atteinte pulmonaire etait associee chez 5 pts (dont les 4 synchrones). Localement, dans 40% des cas le stade de la tumeur etait I, II dans 40%, III dans 20%. Elle etait a droite chez 6 pts. Le geste chirurgical etait une chirurgie reglee pour 6 pts et une resection atypique pour 4. Un patient a necessite la pose d’un packing. Il n’y a pas eu de mortalite peroperatoire. La resection a ete microscopiquement complete pour 8 patients. Une chirurgie pulmonaire a ete faite chez 4 pts. 3 patients ont presente une rechute hepatique avec un delai moyen de 5 mois. Parmi eux, 2 sont decedes. La survie globale est de 80% dans cette serie. Conclusion Le pronostic des enfants ayant une resection complete des MHN persistant apres chimiotherapie est bon. Une prise en charge chirurgicale agressive est necessaire.