Biliary drainage surgery before or after 3 months of life versus primary liver transplantation in children with biliary atresia: comparative cohort study.

BACKGROUND: Early biliary drainage surgery (BDS; Kasai) is associated with longer transplant-free survival in biliary atresia. However, evidence is lacking on whether an age limit can be established at which liver transplantation should be performed as first-line treatment for children with a delayed diagnosis of biliary atresia. The aim of the current study was to compare the outcome of a large cohort of children with biliary atresia who underwent BDS after 90 days of life with those who underwent early BDS (before 90 days) and those who did not receive BDS and were directly referred for primary liver transplantation. METHODS: All patients with biliary atresia treated at Bicêtre, Paris-Saclay University Hospital between 1995 and 2017 were analysed in this STROBE-compliant study. Three groups were defined: BDS before 90 days of life (early BDS); BDS after 90 days of life (late BDS); and patients without BDS who were referred for primary liver transplantation (no BDS). Patient characteristics, overall survival, and native liver survival were compared. RESULTS: Of 424 children with biliary atresia, 69 patients (16 per cent) were older than 90 days when they underwent BDS. Twenty-five patients had no BDS and were referred for primary liver transplantation (6 per cent). The main reason for not performing BDS was manifest portal hypertension (18/25). Two- and 5-year transplant-free survival were significantly higher in patients with late BDS compared with no BDS (53.5 versus 12.0 per cent respectively for 2-year data and 30.4 versus 4.0 per cent respectively for 5-year data, P < 0.001). Five- and 10-year overall survival did not differ between early BDS (92 and 91 per cent respectively), late BDS (88 and 83 per cent respectively) and no BDS (80 and 80 per cent respectively, P = 0.061). CONCLUSION: Age alone should not routinely be considered a contraindication to BDS in patients older than 90 days. Liver transplantation in infancy (less than 12 months) could be avoided in 88 per cent of cases with late diagnosis of biliary atresia and is delayed significantly even when BDS is performed after 3 months. Overall survival is at least equal to patients who are referred for primary liver transplantation.

Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication. (3) Evaluate the impact of preventive fundoplication on gastro-intestinal outcomes in children with a CDH patch repair; METHODS: This prospective multi-institutional cohort study (French CDH Registry) included CDH neonates born in France between January 1st, 2010-December 31st, 2018. Patch CDH was defined as need for synthetic patch or muscle flap repair. Main outcome measures included need for curative fundoplication, tube feed supplementation, failure to thrive, and oral aversion. RESULTS: Of 762 CDH neonates included, 81 underwent fundoplication (10.6%), either preventive or curative. Median follow-up was 3.0 years (IQR: 1.0-5.0). (1) Preventive fundoplication is considered in only 31% of centers. The rates of both curative fundoplication (9% vs 3%, p = 0.01) and overall fundoplication (20% vs 3%, p < 0.0001) are higher in centers that perform preventive fundoplication compared to those that do not. (2) Predictive factors for preventive fundoplication were: prenatal diagnosis (p = 0.006), intra-thoracic liver (p = 0.005), fetal tracheal occlusion (p = 0.002), CDH-grade C-D (p < 0.0001), patch repair (p < 0.0001). After CDH repair, 8% (n = 51) required curative fundoplication (median age: 101 days), for which a patch repair was the only independent predictive factors identified upon multivariate analysis. (3) In neonates with patch CDH, preventive fundoplication did not decrease the need for curative fundoplication (15% vs 11%, p = 0.53), and was associated with higher rates of failure to thrive (discharge: 81% vs 51%, p = 0.03; 6-months: 81% vs 45%, p = 0.008), tube feeds (6-months: 50% vs 21%, p = 0.02; 2-years: 65% vs 26%, p = 0.004), and oral aversion (6-months: 67% vs 37%, p = 0.02; 1-year: 71% vs 40%, p = 0.03). CONCLUSIONS: Children undergoing a CDH patch repair are at high risk of requiring a curative fundoplication. However, preventive fundoplication during a patch repair does not decrease the need for curative fundoplication and is associated with worse gastro-intestinal outcomes in children. LEVEL OF EVIDENCE: II - Prospective Study.

Esophageal Atresia and Respiratory Morbidity.

BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children. METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value <.10 in univariate analyses were retained in logistic regression models. RESULTS: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft. CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for >50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies.

Antenatal Management of Bronchopulmonary Sequestration by Intrafetal Vascular Laser Ablation under Ultrasound Control: Narrative Review of the Literature and Report of Three Cases.

OBJECTIVE: The objective of this study is to assess the effectiveness and safety of intrafetal vascular laser ablation (VLA) for fetuses with bronchopulmonary sequestration (BPS) with hydrops. METHODS: First, we present 3 cases of fetuses with BPS and hydrops treated by VLA. Second, we aimed to conduct a narrative review to identify all reported cases of fetuses with BPS treated by intrafetal VLA. RESULTS: The review of the literature identified 41 fetuses treated by VLA for BPS with hydrops. The median gestational age of the VLA was 27+0 weeks' gestation [25+0-31+0] with an associated procedure at the same time in 43% of the cases (pleuroamniotic shunt, thoracentesis, and amniodrainage). A second procedure was required in 25% of cases for residual flow in the feeding vessel. No stillbirth or neonatal death was reported. The complications reported were a fetal thoracic hematoma complicated by fetal anemia and 4 preterm deliveries with a rate of 9%. CONCLUSION: VLA of the feeding vessel can be an effective treatment but is not without complications. In cases demonstrating cardiac output failure, intrafetal VLA should be considered as a treatment for BPS.

Liver metastasis at diagnosis in children with nephroblastoma enrolled in SIOP2001 protocol: A French multicentric study.

BACKGROUND: Liver metastases are rare in children with Wilms tumor (WT), and their impact on the outcome is unclear. PATIENTS AND METHODS: The French cohort of patients with WT presenting liver metastases at diagnosis and enrolled in the International Society of Pediatric Oncology (SIOP) 2001 study was reviewed. RESULTS: From 2002 to 2012, 906 French patients were enrolled in the SIOP2001 trial. Among them, 131 (14%) presented with stage IV WT and 18 (1.9%) had liver metastases at diagnosis. Isolated liver metastases were displayed in four of them. After preoperative chemotherapy, persistent liver disease was reported in 14/18 patients, and 13 of them underwent metastasectomy after nephrectomy. In resected liver lesions, the same histology of the primary tumor was reported for three patients, blastemal cells without anaplasia were identified in one patient with DA-WT, and post-chemotherapy necrosis/fibrosis was identified for the other 10 patients. For the four patients who had liver and lung surgery, both sites had nonviable cells with post-chemotherapy necrosis/fibrosis. Six patients had hepatic radiotherapy. Sixteen patients achieved primary complete remission and were alive at the last follow-up (median follow-up: 6.4 years). The only two deceased patients presented diffuse anaplasia histology. The five-year EFS and OS were 83% (60%-94%) and 88% (66%-97%), respectively. CONCLUSION: Liver involvement does not appear to be an adverse prognostic factor in metastatic WT. The role of hepatic surgery and radiotherapy remains unclear, and should be carefully considered in case of persistent liver metastases, according to histology and radiological response to other metastatic sites.

Management of focal nodular hyperplasia of the liver: Experience of 50 pediatric patients in a tertiary center.

BACKGROUND: Focal nodular hyperplasia (FNH) is a rare benign hepatic lesion in children. No management guidelines for pediatric population exist because of limited evidence. OBJECTIVE: To review the experience of a large tertiary liver center, providing additional clinical data to help formulate management guidelines for FNH in the pediatric population. METHODS: We analyzed data of children <18 years diagnosed with FNH from 1996 to 2018 at our hospital, detailing management and long-term clinical outcome. RESULTS: 50 patients were identified. The median age was 10 years old (range 0.75-15.5 years old). The mean diameter of FNH was 5.9 cm (±3.1 cm). 10 patients had multiple lesions. First-line management: watchful waiting with serial checks (n = 37), surgery (n = 13). Of the watchful waiting patients, 10 required eventual second-line surgery. After a median follow-up of 4.7 years (range 0.5-20 years), 46 patients were asymptomatic, with no significant difference in clinical outcome (p = 0.962) between the two first-line management approaches. Lesions demonstrated growth in 13 cases: 5 of these required second-line surgery. In these patients, there was no significant difference in clinical outcome (p = 0.188) compared to nonoperative patients. Considering all surgically treated patients, there was no significant difference between first-line and second-line surgery for clinical outcome (p = 0.846), hospital stay (p = 0.410), complications (p = 0.510) and severe complications (p = 0.385). CONCLUSIONS: Our data support the hypothesis that watchful waiting is a safe initial approach to pediatric FNH management in patients with no major symptoms or complications. Surgery should be reserved for patients with diagnostic doubt, persistent symptoms and/or biological or significant anatomical abnormalities. FNH growth alone should not be considered as an indication for surgery. TYPE OF STUDY: Therapeutic study. LEVEL OF EVIDENCE: Level III.

Predictors of the Performance of Early Antireflux Surgery in Esophageal Atresia.

OBJECTIVE: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia. STUDY DESIGN: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia. RESULTS: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001). CONCLUSIONS: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia.

Congenital diaphragmatic hernia has a better prognosis when associated with a hernia sac.

OBJECTIVE: To evaluate neonatal mortality and morbidity up to 6 months in neonates with congenital diaphragmatic hernia (CDH) with or without a hernia sac. METHODS: Seventy-two cases of isolated CDH were included in a retrospective single-center study between January 2010 and December 2016. Hernia sac was defined at the time of surgery or at postmortem examination if the neonate died before surgery. RESULTS: Seventeen newborns (23.6%) had a hernia sac. Survival at 6 months was significantly greater for isolated CDH with a hernia sac: 100% versus 63.6% (P = .003). High-frequency oscillatory ventilation was used significantly more in the no hernia sac group (P = .04). At surgery, the need for patch repair was significantly lower in the hernia sac group: 12% versus 50% (P = .005). The prenatal observed/expected lung-to-head ratio was significantly higher in the hernia sac group than in the no hernia sac group: 49.7% versus 38.6% (P < .05). CONCLUSION: The presence of a hernia sac in CDH is associated with better outcome, especially survival at 6 months. If the presence of a hernia sac is recognized as a particular entity, which carries a good prognosis, it is necessary to be able to diagnose it prenatally, especially in the era of prenatal fetal surgery.

Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort.

INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. RESULTS: Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). CONCLUSION: About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. LEVELS OF EVIDENCE: Level III retrospective comparative treatment study.

Hepatic Portocholecystostomy: 97 Cases From a Single Institution.

OBJECTIVES: Approximately 20% of cases of biliary atresia do not involve the gallbladder, the cystic duct, and the common bile duct. In these cases, a hepatoportocholecystostomy (HPC) may be performed instead of the classical hepatoportoenterostomy (HPE). METHODS: We reviewed our cohort to investigate the efficacy of HPC and the associated surgical complications and clinical problems. RESULTS: From 1984 to 2009, 97 patients underwent HPC in our institution. In the first 6 months of the postoperative period no patient presented with bacterial cholangitis. Nine patients had bile leakage, and 1 patient had a gallbladder obstruction. For these 10 patients, HPC was modified in HPE. Jaundice clearance was maintained after HPE, but 4 of the patients presented 1 or more cholangitis episodes. After 6 months, there were no cases of cholangitis recorded, 2 patients presented bile leakage and 4 patients experienced gallbladder obstruction. After 5 years of follow-up, 46% of the patients were jaundice free with their native liver and 29.4% were transplanted. CONCLUSIONS: In patients with biliary atresia with patent extrahepatic bile ducts, hepatoportocholecystomy is a good surgical technique that can prevent cholangitis. These results are counterbalanced by specific surgical complications that need to be known and looked for in the postoperative period.

Sepsis risk factors in infants with congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly and remains among the most challenging ICU-managed disease. Beside severe pulmonary hypertension, lung hypoplasia and major abdominal surgery, infective complications remain major determinants of outcome. However, the specific incidence of sepsis as well as associated risk factors is unknown. METHODS: This prospective, 4-year observational study took place in the pediatric intensive care and neonatal medicine department of the Paris South University Hospitals (Le Kremlin-Bicêtre, France), CDH national referral center and involved 62 neonates with CDH. MAIN RESULTS: During their ICU stay, 28 patients (45%) developed 38 sepsis episodes. Ventilator-associated pneumonia (VAP: 23/38; 31.9 VAP per 1000 days of mechanical ventilation) and central line-associated blood stream infections (CLABSI: 5/38; 5.5 per 1000 line days) were the most frequently encountered infections. Multivariate analysis showed that gestational age at birth and intra-thoracic position of liver were significantly associated with the occurrence of sepsis. Infected patients had longer duration of mechanical and noninvasive ventilation (16.2 and 5.8 days, respectively), longer delay to first feeding (1.2 days) and a longer length of stay in ICU (23 days), but there was no difference in mortality. CONCLUSIONS: Healthcare-associated infections, and more specifically VAP, are the main infective threat in children with CDH. Sepsis has a significant impact on the duration of ventilator support and ICU length of stay but does not impact mortality. Low gestational age and intra-thoracic localization of the liver are two independent risk factors associated with sepsis.

Results from the French National Esophageal Atresia register: one-year outcome.

BACKGROUND: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA). METHODS: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database. RESULTS: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007). CONCLUSIONS: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

Esophageal atresia: data from a national cohort.

PURPOSE: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan. METHODS: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report. RESULTS: Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted. CONCLUSIONS: Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.

Prenatal diagnosis of congenital mesoblastic nephroma.

A case of prenatal diagnosis of congenital mesoblastic nephroma by magnetic resonance is described.

Nitric oxide activity through guanylate cyclase and phosphodiesterase modulation is impaired in fetal lambs with congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypertension and death. Administration of nitric oxide (NO) alone remains ineffective in CDH cases. We investigated in near full-term lambs with and without CDH the role of guanylate cyclase (GC), the enzyme activated by NO in increasing cyclic 3'-5'-guanylosine monophosphate, and the role of phosphodiesterase (PDE) 5, the enzyme-degrading cyclic 3'-5'-guanylosine monophosphate. METHODS: Congenital diaphragmatic hernia was surgically created in fetal lambs at 85 days of gestation. Pulmonary hemodynamics were assessed by means of pressure and blood flow catheters (135 days). In vitro, we tested drugs on rings of isolated pulmonary vessels. RESULTS: In vivo, sodium nitroprusside, a direct NO donor, and methyl-2(4-aminophenyl)-1,2-dihydro-1-oxo-7-(2-pyridinylmethoxy)-4-(3,4,5 trimethoxyphenyl)-3-isoquinoline carboxylate sulfate (T-1032) and Zaprinast, both PDE 5 blockers, reduced pulmonary vascular resistance in CDH and non-CDH animals. The activation of GC by sodium nitroprusside and the inhibition of PDE 5 by T-1032 were less effective in CDH animals. In vitro, the stimulation of GC by 3(5'hydroxymethyl-2'furyl)-1-benzyl indazole (YC-1) (a benzyl indazole derivative) and the inhibition of PDE 5 by T-1032 were less effective in pulmonary vascular rings from CDH animals. The YC-1-induced vasodilation in rings from CDH animals was higher when associated with the PDE 5 inhibitor T-1032. CONCLUSIONS: Guanylate cyclase and PDE 5 play a role in controlling pulmonary vascular tone in fetal lambs with or without CDH. Both enzymes seem to be impaired in fetal lambs with CDH.

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.

Duhamel operation vs neonatal transanal endorectal pull-through procedure for Hirschsprung disease: which are the changes for pathologists?

BACKGROUND: The aim of this study was to evaluate whether performing definitive surgery for Hirschsprung disease (HD) in neonatal period with a transanal endorectal pull-through (TEPT) procedure had modified our diagnostic relevance, particularly during intraoperative frozen sections (IOFS), compared to classic Duhamel (DH) surgery performed in older children. METHODS: We collected pathologic data for 47 children who underwent surgery for neonatal nontotal HD over a 5-year period. RESULTS: Twenty-nine patients underwent TEPT and 18 the DH operation. Mean age at operation was 19 days for TEPT and 4 months for DH operation. The mean number of IOFS was 2.6 for TEPT and 2.4 for DH operation. Gross examination could be fully completed in all TEPT cases, but was incomplete in 5 DH cases. The average total lengths of bowel, and aganglionic, transitional, and ganglionic segments were 12.3, 7.3, 3, and 2 cm for TEPT, and 17.6, 9.3, 3.5, and 4.8 cm for DH operation, respectively. Discordance between IOFS and paraffin-section analysis occurred in 5 cases (3 TEPT and 2 DH operation). CONCLUSION: When TEPT was used, the gross examination and sampling was more accurate, leading to a clearer pathology report. The TEPT procedure facilitates the work of the pathologist without modifying the results of IOFS, if some precautions are taken.

Retroperitoneal laparoscopic versus open pyeloplasty in children.

PURPOSE: The indications for laparoscopy in pediatric urology are expanding and yet the advantages over open surgery remain unclear. We compared the results of retroperitoneal laparoscopic vs open pyeloplasty for pyeloureteral junction obstruction in children. MATERIALS AND METHODS: A total of 22 children with a mean age of 88 months (range 25 to 192) underwent laparoscopic dismembered pyeloplasty via the retroperitoneal approach. An additional 17 children with a mean age of 103 months (range 37 to 206) underwent similar procedures via open surgery through a flank incision. We retrospectively analyzed and compared operative time, the use of analgesics (acetaminophen or morphine derivatives) and hospital stay. RESULTS: The 2 groups were similar in mean age and weight at surgery. Mean operative time was significantly shorter in the open surgery vs the laparoscopy group (96 minutes, range 50 to 150 vs 219, range 140 to 310, p <0.0001). Mean postoperative use of acetaminophen (1.9 vs 3.22 days, p = 0.03) and morphine derivatives (1.9 vs 3.06 days, p not significant) was less in the laparoscopy group. Mean hospital stay was shorter in the laparoscopy group than in the open surgery group (2.4 days, range 1 to 5 vs 5, range 3 to 7, p <0.0001). Mean followup was 21 (range 12 to 51) and 24 months (range 12 to 60) in the open and laparoscopy groups, respectively. CONCLUSIONS: The operative time of laparoscopic pyeloplasty remains significantly longer than that of the open procedure in children. The main advantage of the laparoscopic approach is that it significantly decreases hospital stay compared with that after an open procedure. Although in our study analgesic use was less after laparoscopy, our results should be confirmed by a prospective, randomized study.

Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center.

The aim of this study was to review our experience in orthotopic liver transplantation (OLT) for biliary atresia (BA) in children and analyze the survival and prognostic factors, and long-term outcome. We reviewed 332 OLTs performed in 280 children between the years 1986 and 2000. Univariate and multivariate analysis were performed on patient and graft survivals according to recipients' and donors' characteristics as well as intraoperative data. The long-term outcome among the 80 children living at 10 years after OLT was studied according to growth, immunosuppressive therapy, and liver and renal functions. Liver graft status was eventually documented by liver biopsy. Status of rehabilitation was assessed by reviewing school performance and employment. Overall patient survival rates at 1, 5, and 10 years were 85, 82, and 82%, respectively, and the corresponding overall graft survival rates were 77, 73, and 71%. In the multivariate analysis, we identified 4 independent prognostic factors: polysplenia syndrome (P = .03), United Network for Organ Sharing (UNOS) status (P = .05), donor's age (P = .01), and perioperative surgical complications (P = .03). At 10 years after transplant, 80 children were alive and had normal growth rates. Liver histology was abnormal in 73% of these long-term survivors, mainly due to chronic rejection and centrilobular fibrosis. A total of 63 of the 80 children attended normal school and in 55 children (69%) school performance was not delayed. In conclusion, we discovered that a good long-term survival could be achieved after liver transplantation for BA, with a 82% survival rate at 10 years with normal scholastic studies in the majority of recipients.