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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.

Congenital adrenal hyperplasia (CAH) is the commonest cause of virilization in genetically female children. Although no consensus has been reached concerning the indications, surgical management of these patients has improved considerably over the last twenty years. The authors describe the main genitoplasty techniques currently used and report their results, although they are difficult to interpret in view of the small number of published series.

The very large number of surgical techniques described and the inadequate long-term results published for each of them illustrate the difficulty of objective evaluation of hypospadias surgery [9]. Although the majority of surgeons define a satisfactory result as a straight penis without excess skin, regular scars, an apical urethral meatus with a "good" urinary stream, evaluation of these criteria remains very subjective due to the lack of precise, reliable, and standardized methods. The surgeon's criteria of success of this surgery differ significantly from the patient satisfaction criteria [53]. The complication rate reported in the literature therefore varies from 5% to 70% depending on the technique used and the surgeon's honesty.

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.