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L'invagination intestinale aiguë (IIA) correspond à la pénétration d'un segment intestinal d'amont dans un segment d'aval. Les invaginations iléocoliques sont les formes les plus fréquentes et nécessitent une prise en charge thérapeutique rapide. Elles sont le plus souvent idiopathiques et fréquemment associées à un contexte d'infection virale. Elles surviennent majoritairement dans la 1re année de vie et représentent la cause principale de syndrome occlusif de l'enfant de moins de 2 ans. Le diagnostic est le plus souvent évoqué devant une symptomatologie clinique très évocatrice. Ainsi, des douleurs abdominales évoluant par crises avec accès de pâleur, associées à des vomissements, des rectorragies ou des signes neurologiques doivent faire évoquer le diagnostic chez le jeune enfant. Le praticien ne doit pas méconnaître la possibilité d'une cause sous-jacente, qui peut être présente chez le grand enfant notamment. L'échographie est l'examen de référence pour le diagnostic et aussi pour rechercher une cause secondaire, apprécier le retentissement digestif et rechercher des signes de gravité. Le traitement est le plus souvent radiologique avec une réduction de l'invagination à l'aide d'un lavement pneumatique, hydrostatique ou échoguidé en fonction des équipes. Ces lavements sont réalisés en l'absence de signes de gravités cliniques ou échographiques et sont le traitement de première intention avec un succès de l'ordre de 90 %. En cas d'échec ou en présence d'une forme secondaire, une prise en charge chirurgicale est nécessaire. [ABSTRACT FROM AUTHOR]

Summary: Giant omphalocele is associated to morbidity and mortality because of the strain the reintegrated herniated mass places on the hemodynamic equilibrium and breathing functions of affected infants. Currently, care management consists in a reintegration in one time or progressive reintegration. We report here a multicenter retrospective study about alternative management by VAC® therapy for giant omphaloceles. The study included three patients (1 girl, 2 boys) presenting with giant omphaloceles, born at full term in three different University Hospitals (prenatal diagnosis, normal karyotype). VAC® therapy was implemented at different times according to the cases (at Day 11, Month 1 and Month 5 after birth). The initial pressure applied was −10 mmHg progressively increased to −50 mmHg. A middle size VAC GranuFoam Silver® Dressing was used in all cases. Wound healing occurred at Month 4 for the first case, Month 6 and Month 8 for the other two. VAC® therapy is a good alternative for the care management of giant omphaloceles with more advantages especially when using prosthetic material. We also aimed at refining the most adapted indications in these specific situations, and finally we envisioned a harmonization of care for these children. [Copyright &y& Elsevier]

Objectives: Short Bowel Syndrome (SBS), secondary to various underlying diseases, is one of the main causes of intestinal failure in children. Surgical management by serial transverse enteroplasty (STEP), is feasible in selected cases, but results on long-term follow-up are scarce. The aim of this study was to report long-term outcomes of the STEP procedure in children with SBS., Methods: We performed a multicenter national retrospective study reviewing medical charts of children who underwent a STEP technique between 2000 and 2022 in 6 university hospitals. Collected data included demographics, SBS history, surgical procedures, nutritional support (enteral or parenteral), STEP management, complications, and outcomes (nutritional support, digestive symptoms, growth)., Results: STEP was performed in 36 SBS, resulting from 14 gastroschisis, 10 intestinal atresia, 8 necrotizing enterocolitis (NEC), 2 midgut volvulus, and 2 intestinal Hirschsprung disease. Median age at first STEP was 10.8 months [4.5; 63.8]. Bowel length significantly increased (47 vs 70 cm, p < 0.01), with a median gain of 16 cm [11; 25] or 34%. At surgery, 33 children had parenteral nutrition (PN). A second STEP was performed in 11 patients, at a median age of 4.2 years [2.8; 6.8]. One patient underwent a third STEP. Two children required intestinal transplantation. Median follow-up was 7 years [4.4;11.4]. Out of 33 children requiring PN before surgery, 14 children were weaned off PN. The remaining 19 children still required PN, but their dependency decreased by 19%. Out of 17 children presenting severe intestinal dilations and bacterial overgrowth, 12 became asymptomatic. Out of 20 with chronic obstructive symptoms, 8 patients still required supplementary PN whereas 12 achieved complete enteral autonomy, including 3 requiring supplementary enteral support., Conclusion: STEP technique remains a surgical option in the management of these children, enabling a decrease in PN dependency, resulting in weaning off PN in some cases, as well as an improvement of clinical symptoms., Type of Study: Retrospective multicenter observational study., Level of Evidence: Level 3., Competing Interests: Conflict of interest The authors declare that they have no conflicts of interest., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Isolated fallopian tube torsion (IFTT) is very rare gynecological emergency in pediatric population. Our objective is to assess treatment options and discuss outcome of a cohort of IFTT with a focus on the association between IFTT and hydrosalpinx (HSX)., Methods: A retrospective review was conducted. Pediatric patients with IFTT operated in the same center were included., Results: Seventeen girls (aged: 11-16 years) were managed for acute abdominal pain between 2008 and 2018, with intraoperative diagnosis of IFTT. All patients underwent laparoscopic exploration, with laparoscopically fallopian tube detorsion in all patients. Based on the association of IFTT with HSX after fallopian tube detorsion, patients were divided into 2 groups: group 1 (IFTT without HSX; 12 girls) and group 2 (IFTT with HSX; 5 girls). During the same surgery, complementary surgical procedures were done. In group 1: salpingectomies (4), partial salpingectomies (2) and cystectomies (6) were done. In group 2: salpingectomy (1), salpingotomy (1), and cyst ablation (1). The treatment was called conservative when the tube was preserved.Follow-up was uneventful in group 1. In group 2, for all patients with initial fallopian tube preservation, further surgical procedures were necessary (1-4 surgeries/patient), and, finally, another 3 patients required salpingectomy., Conclusions: Conservative treatment with tube preservation of IFTT without HSX appeared to be beneficial compared to those with HSX, with no recurrence of torsion or symptoms during the follow-up. However, the same conservative treatment was not sufficiently effective for IFTT with HSX and required further procedures due to recurrence of torsion., Level of Evidence: IV., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background Data: EA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years., Methods: A multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed., Results: Thirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group., Conclusions: Long gap strongly influenced digestive morbidity at age 6 years., Competing Interests: Conflict of interest All the authors declare that they have no conflict of interest., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure., Study Design: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P < 0.20 in univariate analyses were retained in a logistic regression model., Results: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year ( P < 0.05). Neither EA type nor surgical treatment was associated with growth failure., Conclusion: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential of interest., (Copyright © 2022 Depoortere, Lapillonne, Sfeir, Bonnard, Gelas, Panait, Rabattu, Guignot, Lamireau, Irtan, Habonimana, Breton, Fouquet, Allal, Elbaz, Talon, Ranke, Abely, Michel, Lirussi Borgnon, Buisson, Schmitt, Lardy, Petit, Chaussy, Borderon, Levard, Cremillieux, Tolg, Breaud, Jaby, Grossos, De Vries, Arnould, Pelatan, Geiss, Laplace, Kyheng, Nicolas, Aumar and Gottrand.)

Introduction: With advances in surgical and neonatal care, the survival of patients with oesophageal atresia (OA) has improved over time. Whereas a number of OA-related conditions (delayed primary anastomosis, anastomotic stricture and oesophageal dysmotility) may have an impact on feeding development and although children with OA experience several oral aversive events, paediatric feeding disorders (PFD) remain poorly described in this population. The primary aim of our study was to describe PFD in children born with OA, using a standardised scale. The secondary aim was to determine conditions associated with PFD., Methods: The Feeding Disorders in Children with Oesophageal Atresia Study is a national cohort study based on the OA registry from the French National Network. Parents of children born with OA between 2013 and 2016 in one of the 22 participating centres were asked to complete the French version of the Montreal Children's Hospital Feeding Scale., Results: Of the 248 eligible children, 145 children, with a median age of 2.3 years (Q1-Q3 1.8-2.9, min-max 1.1-4.0 years), were included. Sixty-one children (42%) developed PFD; 13% were tube-fed (n=19). Almost 40% of children with PFD failed to thrive (n=23). The presence of chronic respiratory symptoms was associated with the development of PFD. Ten children with PFD (16%) had no other condition or OA-related complication., Conclusion: PFD are common in children with OA, and there is no typical profile of patients at risk of PFD. Therefore, all children with OA require a systematic screening for PFD that could improve the care and outcomes of patients, especially in terms of growth., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

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., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Objective: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula., Study Design: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life., Results: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01)., Conclusions: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: The authors present a multicenter retrospective series of different benign rib lesions in children operated on using thoracoscopy., Materials and Methods: Between 2005 and 2015, 17 rib resections were performed thoracoscopically, in four French departments of pediatric surgery. Of these 17 cases, 13 exostoses, 2 endochondromas, 1 synostosis, and 1 Cyriax's syndrome were noted. Inclusion criteria were benign tumors or rib anomalies such as synostosis, in children younger than the age of 18 years, and thoracoscopy. Open surgery and malignant tumors were excluded. Thoracoscopy was put forward using one optical port as well as one or two operative ports., Results: Ten patients presented with chest pain, dyspnea, or unexplained cough. Six tumors were incidentally diagnosed. One patient presented with a chest wall deformation. Single-lung ventilation was required in 2 cases. In 1 case of endochondroma, a segmental rib resection was performed, leaving a part of the periosteum and the intercostal vessels and nerve. In this case, rib resection was performed using an endoscopic shaver drill system. As for the other cases, a simple resection of the tumor or of the bridge between two ribs (synostosis) was performed. In these cases, a Codman Kerrison laminectomy rongeur was used. There was no complication during or after surgery. Nevertheless, 2 years after surgery, pain did not disappear in 1 case, probably due to a definitive intercostal nerve damage., Conclusion: Benign rib lesions in children are rare. Thoracoscopy may be offered to reduce the functional deleterious consequences of an open surgery. It may be put forward especially in case of hereditary multiple exostoses where redo procedures may be required.

Objective: Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA., Study Design: We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year., Results: Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P < .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P < .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P < .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P < .001)., Conclusion: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of laparoscopic splenectomy (LS) and can lead to bowel ischemia or portal hypertension. In childhood, this complication is reported in 5-10 % of the cases whereas it can be up to 50 % in adult population. Our aim was to evaluate PVT incidence after LS and associated risks factors. A retrospective chart review identified 37 children who underwent elective LS from 2005 to 2013. The main indications were spherocytosis or sickle cell disease. Median age and weight were respectively 7.4 years and 25.1 kg. Thromboembolic prophylaxis was not routinely given. Duration of surgery was 129 min and hospital length of stay 4 days. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases. Post-operative course was uneventful in all but one patient. She was a 17 year-old girl previously operated for an ovarian tumor with hyperandrogenism. Histopathology revealed a splenic lymphoma. At day 4, a systematic USS showed a PVT extending in the portal branches. Therapeutic low molecular weight heparin was used and then transitioned to fluindione for 3 months. Follow-up USS performed at 1 and 4 months demonstrated complete resolution of the PVT. PVT after pediatric LS is a rare event in our series. Clinician should be cautious in oncologic cases and if very large spleen or if thrombocythemia >650.10(9)/L is present. If detected early, PVT can be treated efficiently. We therefore recommend a systematic USS during the first postoperative week.

Background: Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES., Methods: Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome., Results: Over 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27)., Conclusions: CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

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., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Transrectal small bowel evisceration (TSBE) is a rare entity that can occur after traumatic injuries. It has been described after impalement, sexual assault, blunt abdominal trauma, and swimming pool drain suction. The authors encountered such a case in a 4-year-old by following a crush abdominal injury. A laparotomy was performed. The eviscerated bowel was pulled inside the abdominal cavity, revealing the rectal perforation. A portion of devascularized ileum was resected, a primary bowel anastomosis was performed, and the longitudinal rectal laceration was repaired with a two-layered closure. There was no fecal soilage, and no protective colostomy was performed. The mortality and morbidity of TSBE are usually related with the severity and type of trauma. The mesenteric laceration and concomitant injuries can cause significant bleeding leading to a hemorrhagic shock and multiorgan failure., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Most of the pediatric LT grafts consist in a LLS. Liver graft size matching is one of the major factors determining a successful outcome. The aim of our study was to assess whether anthropometric parameters can be used to estimate the LLS weight. A total of 122 donors (48F/74M) from two transplantation centers were retrospectively reviewed. Eighteen were living related donors (LRLT) and 104 DDs. The donor age was 28.2 yr (range 15-63). The BW and height were, respectively, 70.1 kg (range 45-111) and 172.7 cm (range 152-197). The WLW (n = 66) was 1462 g (range 921-2340), and the liver-to-BW ratio was 2% (range 1.45-2.8%). The LLS graft weight was 313 g (range: 183-537 g). The ratio between LLS and BW was 0.452% (range 0.27-0.74). The LLS represented 22.3% of the WLW with a large variability (range 15.4-31.3%). None of the developed models (linear, nonlinear, or multiple) was clinically usable. The LLS weight is highly variable and is not predictable using simple anthropometric variables. When available, we propose that ultrasonographic estimation of the liver volume should be performed when a liver splitting is considered., (© 2011 John Wiley & Sons A/S.)

Segmental liver grafts from DCD in pediatric LT have been safely used even in acute liver failure situations. Furthermore, despite the risk of antibody-mediated acute rejection, some studies have also demonstrated the safety of ABO incompatible LT in infants. The use of such grafts can be beneficial by reducing the time on the transplant waiting list but they are more susceptible to initial dysfunction and there is a lack of enthusiasm to consider their use especially for an emergency LT as a life-saving procedure. In this short article, we describe the use and successful outcome in a neonate with fulminant acute liver failure secondary to neonatal hemochromatosis who received an ABO-incompatible reduced-size DCD graft., (© 2011 John Wiley & Sons A/S.)

Liver transplantation (LT) for very small recipients is challenging but in experienced centres, good results can be achieved. Despite the risk of antibody-mediated acute rejection, some studies have demonstrated the safety of ABO incompatible liver transplantation (ILT) in children and particularly in infants. The aim of our study was to describe the outcome of liver transplantation in infants <5 kg and the safety of using ILT in this group. All LT performed between 1991 and 2010 in children <5 kg were reviewed. Twenty-nine patients were included, five of whom had an ILT. Acute liver failure was encountered in 20 cases. The recipient age and weight at transplantation were respectively 63 days (range: 14-268 days) and 4 kg (range: 2.4-5 kg). The graft-to-recipient ratio was 6.1% (range 2.3-9%). An aortic conduit and delayed abdominal closure were used respectively in 76% and 81% of the procedures. The ABO compatible liver transplantation (CLT) and ILT groups were similar regarding recipient's demographics, graft types or technical transplantation data. The one- and five-yr patient and graft survival were respectively 62%, 62% and 62%, 57.9% with a median follow-up of 95 months. Vascular complications occurred in six cases (21.4%) and biliary complications were encountered in five patients (17%). Acute and chronic rejection developed respectively in 37% and 26% of the recipients. The five patients undergoing ILT are all alive without graft lost after a median follow-up of 34 months (range 7-55 months). When compared with the CLT group, no significant differences were found regarding patient or graft survival, vascular or biliary complications and rejection rates. In our experience, ILT in small infants has short and long term outcomes comparable to ABO-compatible grafts and excellent results can be achieved with a standard immunosuppressive protocol. To avoid mortality on the waiting list for neonatal recipients, ABO-incompatible liver grafts can be used safely., (© 2011 John Wiley & Sons A/S.)

Background/aims: The aim of this study was to analyze the technique of reconstruction, prior to liver transplantation, of donor replaced right hepatic arteries and to study its impact on graft outcome., Methodology: Two groups of liver grafts were retrospectively examined. The first group (group 1) did not require any arterial reconstruction prior to liver transplantation (n=507). The second one (group 2) included grafts with right replaced hepatic arteries which required reconstruction (n=75). Patients' data were analyzed to evaluate the effect of the arterial reconstruction on the incidence of posttransplant mortality, arterial and biliary complications on one hand and patient and graft survivals on the other. We also made a comprehensive literature search to analyze the different approaches described to reconstruct such variation., Results: The two groups showed no statistical sig nificant difference for early posttransplant mortality and arterial complications. Hepatic arterial thrombosis occurred in two cases in the group 2 (2.6%) and in four cases in group 1 (0.7%); p=0.17. Patient and graft survivals over the whole follow-up period were not significantly different between the two groups. The analysis of the literature found few publications dealing with reconstruction of right replaced hepatic arteries, though because various techniques were reported and the samples of patients were small, a preferred method could not clearly be identified., Conclusions: Graft arterial reconstruction of a right replaced hepatic artery using a safe and rigorous technique does not enhance the risk of arterial complications or graft loss and the technique using the GDA stump could be recommended for routine use.

There are some evidence to suggest that careful antenatal monitoring, scheduled preterm delivery and immediate abdominal wall closure may reduce gastroschisis morbidity. We hypothesised that the advantages of a scheduled preterm delivery balance possible complications related to prematurity. A retrospective study was performed including all cases of gastroschisis born between 1990 and 2004 (n = 69). Cases were categorised in two groups. Group 1 contained gastroschisis cases born between 1990 and 1997. Group 2 contained cases occurring since 1997, when a new management pathway for gastroschisis was established: weekly evaluation of the foetal gut by ultrasound (>28 weeks), corticosteroids, and delivery by scheduled caesarean section at 35 weeks (before if evidence of bowel compromise was present). The primary endpoints of this study were the initiation of oral feeding and the number of re-operation for intestinal obstruction. There was a significantly faster initiation of oral feeding (P < 0.0001), however, duration of parenteral nutrition (34 vs. 38 days) and hospital discharge (53 vs. 58.5 days) was not reduced. There was no complication due to prematurity in group 2. Postoperative outcome was improved with less need for muscular stretching or prosthetic patch and less re-operation for intestinal obstruction (P < 0.05). Scheduled and elective preterm delivery facilitates surgical procedure and shortens the time to first feeding. A delivery at 35 weeks (preferring vaginal delivery) seems to be a good compromise between risks related to prematurity and complications related to intestinal peel.

Purpose: We evaluated the long-term consequences of complete fetal UUO on renal hemodynamics and anatomy., Materials and Methods: A total of 26 fetal lambs underwent surgical UUO at 90 days of gestation and 14 twin matched animals served as controls. Synchronous bilateral ARBF was measured using mean transit time technology. Standard anatomical analysis, including evaluation of RPV, was performed in the kidneys. Measurements were done 10, 20 and 40 days following UUO in groups 1 to 3, respectively and in 1-month-old lambs in group 4., Results: All obstructed kidneys underwent typical hydronephrotic transformations with a progressive decrease in parenchymal volume compared to that of contralateral and control kidneys. ARBF in obstructed kidneys was significantly decreased compared to their contralateral counterparts in all groups (p <0.01). Comparing ARBF to RPV showed that the decrease in ARBF was proportional to the loss of parenchymal volume in fetal obstructed kidneys but it remained significant in lambs (p <0.05)., Conclusions: Complete UUO alters ARBF, while vascularization of the remaining renal parenchyma is maintained in fetuses. Profound impairment of the renal arterial supply observed in lambs may be due to physiological changes linked to birth.

Purpose: We analyzed the dynamics of the renal tissue response to experimental fetal urinary flow impairment concerning renal morphology, extracellular matrix composition, regulators of connective tissue degradation and PAX2 protein expression., Materials and Methods: A total of 26 fetal lambs underwent surgical unilateral ureteral obstruction at 90 days of gestation and 14 twin matched animals served as controls. Kidneys were harvested 10, 20 and 40 days after the prior procedure in groups 1 to 3, respectively and in 1-month-old lambs (group 4). Morphological analysis was done using light microscopy. Picrosirius red staining was used to evaluate the area occupied by extracellular matrix components. Collagen I, III and IV, alpha-smooth muscle actin, MMP-1, 2 and 9, TIMP-1 and 2 and PAX2 protein were assessed using immunochemistry., Results: All obstructed kidneys were hydronephrotic without dysplasia. Hypoplasia resulting from a decreased NGG was observed. The inflammatory response to obstruction was poor in fetal obstructed kidneys. From 10 days after obstruction interstitial fibrosis was noted and confirmed by an increase in picrosirius red staining. In obstructed kidneys immunochemistry showed an increase in collagen deposition beginning from the papillae and extending through the whole parenchyma. Aberrant interstitial collagen IV deposition was observed. The increase in alpha-smooth muscle actin staining was mainly localized in the blastema and interstitial cells in obstructed kidneys. MMP and TIMP immunostaining was mainly present in tubules throughout the whole nephrogenic period and persisted in mature kidneys. Beginning from 20 days after obstruction a progressive increase in MMP and TIMP expression was noted. This was associated with ectopic expression in the medullary tubules. PAX2 protein was highly expressed in the nephrogenic zone, decreasing progressively to being markedly decreased in control lamb kidneys. No difference was found in PAX2 expression during the fetal period when comparing unobstructed and obstructed kidneys, it but remained strongly expressed in the dilated collecting ducts of obstructed lambs., Conclusions: Complete unilateral ureteral obstruction performed in fetal lambs at 90 days of gestation led to pure hydronephrotic transformation, hypoplasia and a marked increase in connective tissue deposition. Inflammatory infiltrates and PAX2 dysregulation were not seen as having a decisive role in these modifications.

Purpose: The majority of colorectal carcinomas diagnosed are adenocarcinomas. Squamous-cell carcinoma is a rare pathologic curiosity. Since 1943, only 18 cases have been described in the medical literature. The aim of this study was to report retrospectively six new cases and to review the medical literature., Patients: Of the 6 cases, 4 were females, and age ranged from 43 to 93 years. Tumors were located 7 to 12 (mean, 8.5) cm from the anal verge. Five patients underwent surgical resection. Intraoperative radiotherapy was performed in one case. One patient was treated only by external beam radiotherapy. In two cases neoadjuvant combination of external beam radiotherapy and chemotherapy and in one case neoadjuvant contact x-ray treatment were performed. This treatment was followed by external beam radiotherapy in two cases and by chemotherapy in two cases, in patients with lymph node involvement., Results: The clinical tumor response to radiotherapy was almost complete for the patient who did not undergo surgery. One tumor was sterilized by preoperative radiation. Three patients were alive without recurrence at 6 months, 2 years, and 16 years., Conclusion: The etiopathogenicity of squamous-cell carcinoma of the rectum is discussed. The prognosis of these tumors seems to be worse than that for adenocarcinoma because of a delayed diagnosis. Surgical resection seems to be the most important treatment. Chemotherapy and especially radiotherapy may have some indications.