Search

Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

Introduction Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition. Material and Methods A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given. Results According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt. Conclusions Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers.

Objective The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only “curative” technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO. Material and Methods A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time. Results Of the 73 children with EHPVO, 39 were operated (12 posttransplant and 27 idiopathic). The median age at surgery was 9.36 years (range, 1.60–17.42 years). The MPS was the technique of choice; it was offered in 21 patients but eventually could be performed in only 18 (9 posttransplant and 9 idiopathic). The results of MPS were better in idiopathic (just one thrombosis successfully converted into mesocaval bypass). In the MPS after LT (n = 9), six shunts are permeable, two became thrombotic (one patient requiring retransplantation), and one late thrombosis occurred and the patient died eventually because of gastrointestinal bleeding. In the remaining patients (21 out of the 39), MPS was not performed because of intrahepatic cavernoma, portal vein hypoplasia, or insufficient length of vascular graft (especially in left lateral segment graft LT, since the portal vein is usually located in the right upper quadrant). They underwent 10 distal splenorenal shunts (DSRS) (1 posttransplant and 9 idiopathic), 5 proximal splenorenal shunts (PSRS) (1/4), 6 mesocaval shunts (1/5), and 1 modified Sugiura procedure. The results with DSRS have been favorable (one thrombosis, converted into mesocaval bypass). In the PSRS no thrombosis was identified and in the mesocaval shunt one early thrombosis occurred. Posttransplantation group had higher risk of shunt thrombosis, regardless of the surgical technique (chi-square, 0.021). The total increase of platelets after 6 months was not different in MPS as compared with other surgical techniques (analysis of variance, 0.110). Conclusions The MPS is the technique of choice in EHPVO for idiopathic thrombosis as well as secondary to LT. Not all cases are favorable for MPS, so the surgeon must consider the possibility of alternative techniques for EHPVO. The results in terms of shunt patency are much better in idiopathic cavernoma that posttransplant patients.

Aim of the Study Severe lung hypoplasia and persistent pulmonary hypertension are the main determining factors of survival in infants with congenital diaphragmatic hernia (CDH). The oxygenation index (ratio of delivered oxygen and its arterial level) closely reflects lung function. Single-institution studies have reported that best oxygenation index on day 1 of life (BOI-d1) is the most reliable postnatal predictor of survival in CDH. The aim of this study was to evaluate the predictive value of BOI-d1 in four disparate high volume centers in Europe. Methods A retrospective, multicenter study of infants with CDH born between 2000 and 2009 in four European tertiary institutions was conducted. Ethical approval was obtained from institutional review boards. Centers no. 1 and. 4 offered extracorporeal membrane oxygenation (ECMO), whereas center no. 3 offered fetal endoluminal tracheal occlusion (FETO) in fetuses defined as poor prognosis (lung-to-head ratio [LHR] ≤ 1.0 and “liver-up” position). Prenatal LHR and perinatal variables, including gestational age, birth weight, defect side, liver position, BOI-d1, and patch requirement, were analyzed. Receiver operating characteristic curves were used to determine cutoff values for continuous variables. Comparison was made between survivors and nonsurvivors using univariate analysis and logistic regression analysis, p Results A total of 235 infants (center no. 1, n = 29; no. 2, n = 64; no. 3, n = 113; and no. 4, n = 29) were included. One infant required (2%) ECMO and 66 (28%) had FETO. LHR was available in 83 patients (36%). Overall survival (discharge from hospital) and 28-day survival were 67.6% (n = 159) and 72.3% (n = 170), respectively. Univariate analysis showed that significant categorical predictors of 28-day survival were liver-down position (p 1 (p = 0.003), and primary repair (p = 0.02) but not defect side (p = 0.83). Area under the receiver operating characteristic (AUROC) curve for continuous variables; gestational age, birth weight, and BOI-d1 were 0.70, 0.68, and 0.88, respectively. AUROC for BOI-d1 (28-day survival) was 0.91 and had sensitivities (73 and 91%) and specificities (92 and 80%) for cutoffs of 40 and 82, respectively. Conclusion This multicenter study showed, that except from the defect side, all the prenatal variables studied have predictive value but the most useful is BOI-d1. This is simple to calculate and represents an excellent marker for lung function and a reliable early postnatal predictor of survival.

Introduction Severe tracheobronchial injuries (TBI) in children are usually traumatic or iatrogenic. However, they can also be caused by mediastinal infections that lead to critical situations. We herein report our experience in the treatment of these lesions. Methods A retrospective study was conducted for patients treated at our center from 2008 to 2014. TBI was diagnosed by imaging studies and bronchoscopy. Treatment was initially conservative (drainage of air and secretions, mechanical ventilation with minimal pressures, and an early extubation) with a limited use of surgical procedures whenever necessary. Results A total of 10 patients (7 males and 3 females) with a median age of 7.5 years (range, 3–17 years) suffered TBI. The mechanism was traumatic in six (three accidental and three iatrogenic) and mediastinal infection in four (three mycotic and one bacterial abscesses). All traumatic cases responded to conservative measures, except one iatrogenic lesion, which was surgically repaired. There were no complications or residual damages. Two patients with mediastinal infection presented with sudden cardiorespiratory arrest, one with hemoptysis caused by an arteriotracheal fistula and the other because of carinal rupture. Both died before any therapeutic measures could be taken. The other two patients were treated, one with previous extracorporeal membrane oxygenation support, underwent arterial embolization, but ultimately died, and the other one survived, but required esophagectomy and creation of a thoracostome for secondary wound closure of the bronchocutaneous fistula. Conclusion Conservative treatment with gentle respiratory support suffices in most traumatic cases of TBI. Infectious abscesses with involvement of adjacent structures sometimes require complex surgery and are life-threatening.

Aim The aim of the study is to present views of pediatric surgeons on the optimal approach to inguinal hernias in children. Methods A questionnaire was submitted to participants of EUPSA-BAPS 2012 and it was returned by 187 responders (85% senior surgeons) from 46 (27 European) countries. Main Results Approximately 80% of the responders work in centers treating more than 100 cases/year. Approximately 79% of the responders routinely perform laparoscopy for other conditions, but of these, only 22% of the responders routinely perform laparoscopic inguinal hernia repair. Open approach is preferred by 83% of the responders. Of these, 79% of the responders favor unilateral approach, 12% of the responders contralateral, and 9% of the responders contralateral laparoscopic exploration via hernial sac. Considered advantages are the less risk of recurrence (66%), less abdominal organ injury (31%), less vas/vessel injury (25%), and speed (16%). Laparoscopic approach is preferred by 17% of the respondents, of whom 58% perform laparoscopy at all ages, 15% only in younger than 1-year-old infants. Approximately 81% of the responders offer laparoscopy to both sexes, and 17% only in girls. Only 15% would do laparoscopy after incarceration. Purse string is the most popular (58%), then laparoscopic-guided percutaneous closure (28%), “flip-flap” (6%), or other techniques (8%). Proficiency is achieved after 50 (14%), 25 (48%), 10 (32%), or 5 (6%) laparoscopic hernia repairs. Considered advantages are less metachronous contralateral hernias (57%), better cosmesis (41%), easier technique (34%), less vas/vessel injury (31%), and less postoperative pain (22%). A total of 50 responders are planning to start laparoscopic hernia repairs. Conclusions Most pediatric surgeons favor open unilateral inguinal hernia repair. The role of laparoscopy in inguinal hernias is still controversial and yet to be accepted even amongst pediatric surgeons expert in laparoscopy.

Introduction Ganglioneuroma (GN) is a benign, differentiated variety of neurogenic tumor. It is often asymptomatic and may be diagnosed by serendipity. Surgical removal is the treatment of choice. However, it has been suggested that postoperative complications and sequelae might outweigh the benefits of this approach. The purpose of the present study was to examine these issues in a large experience of neural tumors. Methods Patients treated between 1992 and 2012 were retrospectively reviewed. Modern imaging, measurement of catecholamine metabolite excretion and metaiodobenzylguanidine were used for workup. Surgical treatment aimed at complete resection. Complications and sequelae were recorded. Literature was searched for regrowth or malignant transformation of GN. Results Of 227 patients with neural tumors, 24 were GN patients (12 abdominal, 11 thoracic and 1 cervical with 8 dumbbell extensions). Six children were symptomatic (three with abdominal pain and mass, one with stridor or dysphonia, and one each with anisocoria and opsomyoclonus). However, 18 (75%) were asymptomatic and the diagnosis was incidental. Several tumors were large and involved more than one body space. There were no neurologic symptoms in eight cases with dumbbell extension. Complete resection was achieved in 20 children (83%) whereas gross residual was left in four. Postoperative complications were: Horner syndrome (3 patients), mild scoliosis (1 patient), adhesive bowel obstruction (1 patient) and acute urinary retention (1 patient). There was no evidence of either regrowth or malignant behavior in residual masses left in place after follow-up of 84 (1–194) months. Conclusions There were a limited number of general minor complications in this series that did not include cases of regrowth or malignant transformation. However, these unfavorable events were occasionally reported in the literature. Since diagnosis of GN cannot be ascertained before removal of the mass, this should remain the aim of the treatment, although limiting the chances of complications to a minimum even if incomplete resection is the price to pay. Nonoperative attitudes should not be recommended in all cases, but they are certainly justified in some.

Background/Aim Patients with esophageal atresia and tracheo-esophageal fistula (EA-TEF) have chronic respiratory tract disease and deficient tracheal innervation. This study tests the hypothesis in rats with EA-TEF that deficient lung innervation could be one of the causes of respiratory disease. Material and Methods Pregnant rats were treated with either 2 mg/kg i.p. adriamycin or vehicle on E7, E8 and E9. Lungs and tracheas were retrieved on E15, E18 and E21 (term: E22). Innervation was examined by regular (PGP 9.5 and GDNF) and whole-mount (PGP 9.5 and α-actin) immunohistochemistry. PGP 9.5 and GDNF mRNA were measured by real-time, quantitative RT-PCR and the levels of PGP 9.5 protein by immunoblot. Embryonic lung primordia harvested on E13 were cultured for 72 h and airway peristalsis was assessed under an inverted microscope. PGP 9.5 expression was then examined in explants by whole-mount immunohistochemistry and RT-PCR. Values were compared with non-parametric tests. Results Neural networks were present in both EA-TEF and control fetuses on E15, E18 and E21, but the neural network was obviously disorganized in whole-mount immunohistochemistry of EA-TEF. The pan-neural marker PGP 9.5 protein was increased at term whereas the neural chemo-attractant GDNF protein was unchanged. PGP 9.5 mRNA significantly increased from subnormal levels on E15 to very increased ones on E18 compared with controls. GDNF mRNA levels followed the same trend. Airway peristalsis of explanted embryonal lungs was similar in both groups. The neural networks were underdeveloped in these primordia, as assessed by whole-mount PGP 9.5 immunohistochemistry and RT-PCR. Conclusions The development of respiratory tract innervation in adriamycin-induced EA-TEF was delayed and abnormally controlled in rats compared with controls. However, these deficiencies were apparently compensated for at term and had no effect on airway peristalsis. The possible significance of innervation anomalies for respiratory sequelae in EA-TEF patients deserves further investigation.

Aim of the study Cystic fibrosis (CF) is a multisystemic disease, with some patients developing end-stage liver disease (ESLD), requiring liver transplantation (LT). These children usually present with severe mutations of the CFTR gene. Almost 100% of patients with severe mutations develop exocrine pancreatic insufficiency, leading later to endocrine insufficiency. Immunosuppression accelerates the development of insulin-dependent diabetes (IDD) in transplanted children with CF. Our aims were: 1) to analyze our experience with CF-related ESLD children who received LT, and the relationship to the development of IDD; 2) to report our preliminary results with en bloc liver-pancreas transplantation (CLPT). Methods 9 children (6M/3F) with CF and ESLD underwent LT between 1993 and 2010; median age and weight were 12.3 years (range: 5.4–17.0) and 36.7 kg (range: 14.2–58.5), respectively. 4 patients received a whole graft, 4 had reduced grafts (1 split) and 1 underwent CLPT. Immunosuppression followed the protocols at the time of transplantation. Results Liver function was restored in all patients and none of them needed re-transplantation. Median follow-up was 105 months (range: 4–206). 1 child died of respiratory failure at 23 months after transplantation while awaiting pulmonary transplantation. Survival (Kaplan-Meier) at 105 months was 87.5%. 4 children already had IDD before transplantation and 3 developed diabetes immediately after transplantation. 2 had not developed IDD at the end of the study: the youngest at the time of LT (5.4 years, follow-up 7.1 years) and the girl who had had CLPT and who recovered normal exocrine and endocrine pancreatic function after transplantation. Conclusions LT is a realistic option to treat CF-related ESLD children. IDD is common in these patients. En bloc liver-pancreas transplantation is an appealing option, since it simultaneously restores exocrine function and prevents IDD. This procedure has clear technical advantages over simultaneous isolated liver and pancreas transplantation.

Background/aim Surgery for neural tumors occasionally involves opening of the neural canal and/or costovertebral resection that may be followed by radiotherapy. Spinal deformities (SD) may occur in these cases. This study investigated the incidence, surgical indications and results of repair of late SD in a large series of patients with neural tumors. Methods A retrospective review of neural tumors treated between 1991 and 2009 at our institution identified children with SD who subsequently required spinal surgery. SD was meaÂ-sured using Cobb's angle. Instrumentation of the spine was indicated in patients with a Cobb's angle of more than 60° or rapidly progressing SD. The operation was adapted to the location, type and degree of SD. Patients were followed up and the remaining deviations were measured annually. Results Out of a series of 188 patients with neural tumors, 5 of 173 patients with neuroblastomas/ganglioneuromas (NB/GN) and 3 of 15 patients with PNET/Askin tumors developed SD over time. NB/GN children had intraspinal tumors requiring laminotomy (n=3) or laminectomy (n=2). PNET/Askin tumor patients had undergone largescale rib and partial vertebral body resection and radiotherapy. Scoliosis or kyphosis were located in the thoracic spine in 4, in the thoracolumbar spine in 3 and in the cervicothoracic spine in 1. The median preoperative Cobb's angle was 65° (50°-90°) prior to spinal surgery performed 2.9 (0.4-11) years after tumor removal. 3 growth bars, 2 vertical expandable prosthetic titanium ribs, 1 pediatric ISOLA device and anterior instrumentation were used. Arthrodesis was necessary in 3 patients. Median post-instrumentation deformity after 3.1 (0.2-12.2) years was 40° (20°-50°), giving an improvement of 38.4%. Conclusions With increasing numbers of children surviving cancer, attention must focus on sequelae. A sizeable number of survivors of NB/GN and PNET/Askin tumors develop serious SD requiring surgical correction. Pediatric surgeons, patients and relatives must be aware of these possible sequelae.

Aim Aim of the study was to review our experience in the management of liver tumors in children over the last 15 years. Patients and methods A cohort of 78 children with liver tumors managed in our institution between 1991 and 2006 was retrospectively reviewed. There were 45 males and 33 females with a mean age of 32 +/- 41 months at diagnosis. Most tumors were malignant (n = 57); the most frequently occurring tumor was hepatoblastoma (n = 47), followed by hepatocarcinoma (n = 5), sarcoma (n = 4), and lymphoma (n = 1). Vascular tumors (n = 12) predominated among the benign tumors followed by mesenchymal hamartoma (n = 4), focal nodular hyperplasia (n = 3), adenoma (n = 1), and inflammatory pseudotumor (n = 1). We reviewed the epidemiologic features, clinical presentation, diagnosis, treatment and outcomes. We employed MRI and angio-CT for SIOPEL PRETEXT staging and selected the management accordingly for malignant tumors. We analyzed the long-term survival using Kaplan-Meier curves. Results Benign tumors had an excellent outcome with both medical or surgical management. Of the malignant tumors 4 were PRETEXT I and were treated by left lateral segmentectomy with 100 % survival; 20 were PRETEXT II (12 left and 8 right lobe) and were treated by lobectomy of the corresponding side, except for 1 case which required OLT (90 % survival); 9 children had PRETEXT III tumors requiring trisegmentectomy or extended lobectomies with OLT in 1 case (77.7 % survival). Fourteen children had PRETEXT IV tumors: 10 received OLT and 9 of them are still alive (64.2 % survival). Overall survival was 80.8 %, and actuarial survival at 6 years was 82.2 %. Other malignant tumors had variable results. Conclusions Outcomes have improved much in the last years. Surgical removal is necessary in most cases. Transplantation is a very useful adjunct. Treatment of these tumors should be concentrated in centers with expertise.

The management of children receiving small bowel grafts involves potentially life-threatening complications that affect their nutritional status. The aim of this paper was to define these factors and their influence on nutritional outcome.Patients with intestinal failure (IF) who received an isolated small bowel transplantation (SBT) or small bowel/liver transplantation (SBLT) at our hospital during the last 6 years were reviewed for weight Z-score, biochemical nutritional parameters, total parenteral nutrition (TPN) weaning, catheter-related sepsis, rejection and steroid treatment.Twenty patients, 11 females and 9 males, received a SBT or a SBLT and survived the postoperative period; in the present study we only included 11 children with follow-up periods longer than 1 year. Seven males and 4 females with a mean age of 4.5 years (range, 1 to 20 years) received 6 SBLT and 5 SBT. Nine (82%) were weaned from TPN to an amino-acid or peptide enteral formula during the first 6 months after surgery. During the first year there was a significant increase in total protein from 5.11 +/- 1.8 mg/dl to 6.1 +/- 1.5 mg/dl (p0.05) and an increase in albumin from 3.8 +/- 0.9 mg/dl to 4.5 +/- 1.1 mg/dl (p0.05). There was an increase in weight Z-score in 9 patients (82%) during the first year. Mean Z-score improved from - 2.6 +/- 1 at transplant to - 1.0 +/- 0.6 (p0.05) after 1 year. Three patients (27.2%) had at least one rejection period, which was treated with steroids alone or in combination. Mean weight Z-score 1 year after surgery was - 0.9 +/- 0.6 for patients without rejection and - 1.24 +/- 0.8 for those with at least one rejection episode treated with steroids (p0.1). Four patients (36%) had at least one catheter-related sepsis episode. Mean weight Z-score 1 year after surgery was - 1.01 +/- 0.6 for patients without catheter-related sepsis and - 1.24 +/- 0.8 for those with at least one catheter-related sepsis episode (p0.1).There was a significant improvement in weight Z-score and biochemical nutritional parameters 1 year after receiving a small bowel graft. No influence of steroids or catheter-related sepsis on children's nutritional status was noted 1 year after surgery, although this point will need further evaluation.

But: Une connaissance precise de l'anatomie complexe et souvent inattendue des enfants siamois est necessitee pour prevoir la strategie chirurgicale appropriee a leur separation. Les progres recents des techniques d'imagerie permettent un bilan pour planifier la strategie de separation. Materiel et Methodes: Depuis 1990, nous avons traite 8 couples d'enfants siamois: 1 acardiaque-anencephalique, 1 heterophage epigastrique, 2 omphalopages, 2 omphalo-thoracopages, 1 ischiopage tetrapus, et 1 parapage tripus. Le premier couple etait separe immediatement apres la naissance et etait exclu. L'echographie et les radiographies simples de l'abdomen sont utilises chez tous les autres, une angiographie chez 5, un scanner chez 4 et une IRM chez 4. Le scanner helicoidal et la resonance magnetique nucleaire (MRA) etaient utilises chez 3 couples. Resultats: Deux couples d'omphalopages et un couple d'omphalothoracopages etaient separes des la naissance car l'un des deux presentait un dommage cerebral; une exstrophie cloacale et un large shunt arteriel chez l'autre. Ils ont des etudes barytees (3 couples), une urographie intraveineuse (3 couples), une angiographie (2 couples), un CT (2 couples) et une IRM (1 couple). 3 des 6 bebes ont survecus apres la separation. Dans le second couple d'omphalothoracopages, l'echographie et MRA montrerent que l'etendue du systeme cardiovasculaire commun empechait la separation. Dans les deux autres couples, ischiopages et parapages, l'etendue des organes en commun a ete analyse par un scanner helicoidal et par une IRM. Les 4 enfants sont vivants apres la separation. Conclusion: L'imagerie conventionnelle contribue seulement modestement a preparer la separation. En contraste, les techniques avancees d'imagerie sont seulement efficaces pour depister les fusions completes qui doivent etre reconnues au moment du planning de la strategie de separation.

AIM Aim of the study was assess the results of the treatment of High-Risk Hepatoblastoma (HRH) in a tertiary center where all liver surgery facilities, including pediatric transplantation (LT), are available. METHODS 91 primary liver tumors treated between 1991 and 2009 were retrospectively reviewed. HRHs as defined by the SIOP criteria (PRETEXT IV or any stage with venous involvement, extrahepatic disease, tumor rupture and

Sepsis in short-bowel syndrome (SBS) is in part due to bacterial translocation (BT). Parenteral nutrition (PN) is often necessary in SBS and promotes BT. The aim of this study was to asses the effect of the presence or absence of ileocecal valve (ICV) on BT in parenterally-fed rats with massive intestinal resection. Sixty-five adult Wistar rats underwent central venous cannulations and were randomly assigned to one of five groups receiving for ten days five treatment regimes: Sham (n = 17) standard rat chow + i.v. saline. PN (n = 17) fasting + PN. Res-Sham (n = 10) standard rat chow + i.v. saline + 80% gut resection. Res-PN (n = 11) fasting, PN + 80% gut resection. Res-ICV-PN (n = 10) fasting, PN + 80% gut resection including ICV. At the end of the experiment they were euthanized and mesenteric lymph nodes (MLN), spleen and peripheral and portal blood specimens were recovered and cultured. BT was found in 47% of PN animals, 91% of Res-PN rats, 100% of Res-Sham group and 60% of Res-ICV-PN animals, but not in Sham ones. 97% of BT+ animals had positive cultures in MLN and/or portal blood, whereas germs beyond liver were detected in 30% of Res-Sham, 37% of PN, 50% of Res-PN and 0% of Res-ICV-PN rats. The present study confirms that both massive intestinal resection and PN promote BT. In addition, it shows that animals deprived of ICV have lower incidence of BT in this setting than those with it and that the germs do not reach in them peripheral blood in the same proportions as in ICV-intact animals. These results suggest that the presence of an intact ICV favor BT in parenterally-fed rats with massive intestinal resection.

Introduction Several variant techniques have been developed as alternatives to whole liver transplantation to improve size matching, timing, or simply to increase the pool of donors. The aim of this study was to assess the requirements of these techniques and their outcomes in a pediatric transplant program. Patients and method A retrospective analysis of children on the waiting list in the last 4 years was carried out. Data of patients who died while on the waiting list (WL) were recorded. Transplanted patients were divided according to the type of graft: whole liver, split, living donor and reduced liver. The analyzed outcome variables were: age, weight, UNOS status, cause of liver failure, complications and graft and patient survival. Comparisons between types of graft were performed by using Kaplan-Meier, log-rank, chi (2) and Kruskal-Wallis tests. Results During the period studied, 116 children were listed for liver transplantation. Of these 116 children, nine (7.7 %) died after a mean period of 40.5 (5-175) days waiting for a suitable graft. Their median age at inclusion was 214 (35-1607) days, and median weight was 7.2 (12.3-3.6) kg. The cause of liver failure in this group was: 1 hemochromatosis, 1 hepatoblastoma, 2 biliary atresia, 2 acute liver failure, 2 primary non-function (PNF) and 1 chronic rejection. Liver transplantation was performed in 103 children: 25 (24 %) whole livers, 17 (16.5 %) split, 29 (28 %) living donor, 32 (31 %) reduced and 4 remain on the waiting list. Recipient age and weight were significantly lower in those receiving split and living donor than in those who given whole livers. Patient and graft survival were similar in all groups although there was a trend to lower graft survival in patients receiving whole livers. More than 50 % of patients with UNOS status I received a split graft and 5/6 children with hepatoblastoma underwent living donor transplantation. There were no differences in the rate of acute vascular complications, but long-term biliary complications were more frequent in split and living donor grafts. Conclusions As long as the goal of zero mortality for children on the waiting list is not achieved, variant techniques will be necessary in pediatric liver transplantation programs. Split and living donor were employed mostly to treat younger children and particularly those with a higher UNOS status. Children with tumors were treated mainly with living donor grafts. Variant techniques, which are absolutely necessary in a pediatric program, need to be improved in order to avoid long-term biliary complications.

Antecedents: Le nombre reduit de donneurs est un facteur limitant critique pour l'extension des programmes de transplantation pulmonaire pediatrique. Nous rapportons ci-dessous les details de la technique de la transplantation lobaire ainsi que les donnees de la monitorisation perioperatoire dans le but de demontrer que la fonction du parenchyme greffe peut etre suffisante. Methodes: Le lobe inferieur gauche de 10 porcs adultes hybrides Large-White (15-20 kg) fut greffe chez 10 porcelets de la meme race (5-7 kg) et une heure apres, le poumon droit fut exclu par clampage. Les 5 premiers animaux furent etudies du point de vue de l'anatomie du montage et de l'efficacite de l'instrumentation (groupe A) tandis que chez les 5 autres on a etudie de facon seriee les donnees hemodynamiques et la fonction pulmonaire durant une periode de 6 heures. Resultats: Le lobe pulmonaire inferieur gauche s'est revele tres approprie pour la transplantation chez un animal de moindre taille. Le calibre reduit de la bronche est particulierement apte pour une anastomose telescopee. Le clampage auriculaire gauche fut tres bien tolere pendant les anastomoses veineuses. L'anti-aggregation pre-operatoire et l'heparinisation post-operatoire avec valeurs ACT de plus de 200 secondes ont evite la thrombose auriculaire gauche dans tous les cas. Apres exclusion pulmonaire droite, les changements hemodynamiques ont consiste en une augmentation de la resistance vasculaire pulmonaire ainsi qu'en une reduction du debit cardiaque. La mecanique pulmonaire changea aussi dans le sens d'une augmentation progressive de la resistance de la voie aerienne avec une chute de la compliance. Conclusion: Le porcelet tolere bien la transplantation d'un lobe pulmonaire provenant d'un donneur adulte. Bien qu'il s'agisse d'un modele particulierement adapte a l'entrainement pour ce type de transplantation chez l'enfant, son application clinique pose encore des problemes.

Nous rapportons notre experience sur un modele experimental de Defauts du Tube Neural (DTN) induit par l'action de l'acide retinoique chez le foetus de rat. Le produit teratogene dilue dans de l'huile d'olive fut administre en une seule dose (125 mg/kg de poids) par voie intra-gastrique a des rats gravides (n = 31) au 10eme jour de la grossesse, et les foetus furent recuperes par cesarienne au 20eme jour. Des rats controles (n=9) furent traites identiquement avec de l'huile d'olive pure. Nous avons pu etudier 201 foetus experimentaux et 82 foetus controles. n y avait des DTN chez 36.3% des foetus traites, dont les plus frequents etaient des dysraphies sacrees, accompagnees parfois d'une malformation d'Arnold Chiari ou d'une augmentation du volume de l'encephale. Il y eut aussi de nombreuses malformations cranio-faciales (78.1%), caudales (80%), ano-rectales (31.4%) et des membres (89.5%). Ce modele experimental d'induction de DTN est relativement facile et economique et peut permettre d'approfondir la connaissance de la biologie du foetus porteur de cette malformation.

A 4-year-old boy with neurofibromatosis was operated on for abdominal tumor and a malignant Schwannoma adhering to the bladder was completely resected. Four months later he died with multiple liver metastases. The authors comment on the main features of this rate tumor and on the problems of malignancy associated with von Recklinghausen's disease.

Nasal regurgitation of milk and choking after feeding were observed in a 1,450 g newborn boy. A nasogastric tube was inserted and several episodes of aspiration pneumonia occurred after every interruption of gavage. Weight gain was very slow. At the age of 7 months, cineradiographic studies depicted cricopharyngeal spasm and passage of the barium into the nasopharynx and larynx. Pharyngo-oesophageal manometry showed incoordination, high cricopharyngeal pressure and incomplete relaxation of the muscle. Extended lower oesophageal pH-metering revealed severe gastro-oesophageal reflux. After cricopharyngeal myotomy and fundoplication the patient recovered, x-ray findings improved, and so did manometry except for a persistent incoordination. Functional studies are mandatory for diagnosis of this complex clinical pattern. The need for ruling out gastrooesophageal reflux before myotomy in order to prevent subsequent severe aspiration, is pointed out.

Traumatic arteriovenous fistula is rare in children. We describe one case resulting from a small glass-fragment stab wound in the left leg. There was an associated false aneurysm of the anterior tibial artery. The patient was cured after multiple ligation and excision of the aneurysm.