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Background The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. Methods Babies with EA from January 2005 – December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. Results Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/− trachea-esophageal atresia (EA +/− TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. Conclusion We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/− TEF. Level of evidence Prognosis study – level IV. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: Following a Soave pull-through for Hirschsprung disease (HD), some children struggle with obstructive symptoms. We hypothesized that these symptoms could result from a functional obstruction of the pull through caused by the Soave cuff, and that cuff resection might improve bowel emptying. Methods: We reviewed patients referred to our center from 2008 to 2012 with obstructive problems following a Soave pull-through for HD (CCHMC IRB # 2011–2019). Only patients with an obstructing Soave cuff were analyzed. Patients with other reasons for obstruction (anastomotic stricture, transition zone, aganglionic segment) were excluded. Results: Thirty-six patients underwent reoperation at our center for obstructive symptoms after an initial Soave pull-through. Seventeen of these patients had a Soave cuff only as the potential source of obstruction. Pre-operative symptoms included enterocolitis (10), constipation (6), and failure to thrive (1). Nine patients (53%) required irrigations to manage distension or enterocolitis pre-operatively. 14/17 patients (82%) had a palpable cuff on rectal exam. Eight patients (47%) had radiographic evidence of a cuff demonstrated by distal narrowing (4) or a prominent presacral space (4). Four children (23%) underwent excision of the cuff only. Thirteen (76%) had removal of the cuff and proximally dilated colon [(average length 7.2cm) (12 performed transanally, and five needed laparotomy as well.)] Post-operatively, episodes of enterocolitis were reduced to zero, and need for irrigation to treat distension was reduced by 50%. Nine patients have voluntary bowel movements, and five are clean on enemas. 3/6 patients with pre-operative constipation or impaction now empty without enemas. (Follow up 1–17months, mean 7months.) Conclusions: Recurrent enterocolitis, constipation, or failure to thrive can indicate a functional obstruction due to a Soave cuff when no other pathologic cause exists. Physical exam or contrast enema can identify a problematic cuff. Reoperation with cuff resection can dramatically improve bowel emptying. [Copyright &y& Elsevier]

Abstract: Purpose: Surgical options previously described by us as part of a bowel management program for the treatment of soiling and fecal incontinence include (1) resection of a megarectosigmoid to reduce a patient’s laxative requirement or (2) a Malone appendicostomy in patients who require enemas. We have found that some patients may benefit from both procedures. Methods: We reviewed 18 fecally incontinent patients with structural or functional disorders of the anorectosigmoid (16 ARM, 1 spina bifida, and 1 SCT) who underwent both procedures. Results: Of 18 patients, the enema regimen prior to resection had an average volume of 681ml of saline (Range 400–1000ml) and 60ml (Range 48–117ml) of additives (glycerine, castile soap and/or phosphate). Following the colon resection, the average volume of saline and additives was 335ml (Range 130–650ml) and 25ml (Range 0–60ml), respectively, a 50% reduction for both (P<0.01). The time for enema administration and evacuation was reduced by 25%, and the enemas were more effective, rendering the patients clean in 18 of 18 cases (follow-up was 3months to 21years). 2 patients later demonstrated that they could be managed with laxatives alone. Conclusion: In patients with poor continence potential and a megarectosigmoid, combining a colon resection with a Malone appendicostomy can make the enema more effective. In some rare cases we found the resection may allow for a better response to laxatives. [Copyright &y& Elsevier]

Abstract: Purpose: Swenson’s procedure for Hirschsprung disease (HD) was thought to disturb fecal, urinary, and ejaculatory functions leading to other approaches including the Soave and Duhamel techniques. Given our Center’s experience with a full-thickness rectal dissection for anorectal malformations, and using the new transanal concept, we chose to apply these ideas to the primary treatment of HD, and describe technical aspects and impact on fecal, urinary, and sexual function. Methods: We reviewed our series of HD patients who underwent a transanal, Swenson-like rectosigmoid dissection, assessing for postoperative stricture, anastomotic leak, enterocolitis, and long-term results for bowel, urinary, and sexual function. Results: Of 67 patients, 28 had a transanal resection, 5 had transanal plus laparoscopy, and 34 had transanal plus laparotomy, of those, 28 patients had a leveling colostomy prior to referral. The average length of resection was 27cm±12.7cm. Mean follow-up was 17.2months (range 1–96months). 44 patients were at least three years old at follow-up and were assessed for urinary and fecal continence; all (100%) had voluntary bowel movements and urinary continence. Enterocolitis occurred in 9 patients (14%) and constipation (requiring laxatives) occurred in 21 (32%). Of 24 male patients, 21 (88%) reported the occurrence of spontaneous erections post-operatively. Conclusion: Our data support the fact that a modification of Swenson’s original transabdominal dissection concept using the recently described transanal approach is an excellent technique for Hirschsprung, and produces excellent long-term outcomes for fecal and urinary continence, and seems to preserve erectile function. [Copyright &y& Elsevier]

Abstract: Introduction: Rectal atresia/stenosis is a rare disorder in the spectrum of anorectal malformations and is particularly associated with a presacral mass. These patients are born with a normal anal canal but have a stricture or complete atresia located a few centimeters proximal to the dentate line. We present a surgical technique for the management of these patients, as well as their unique clinical concerns and outcomes. Methods: We reviewed the records of 14 patients with rectal atresia and 3 with rectal stenosis. We describe a novel technique that we have developed for the preservation of the anterior dentate line that was performed in the last 13 patients. Results: Rectal atresia/stenosis was associated with a presacral mass in 5 patients (29%). Definitive repair was completed using a circular rectorectal anastomosis in the first 4 patients and an anterior dentate line sparing procedure in the last 13. All patients older than 3 years have demonstrated the ability to have voluntary bowel movements. Conclusion: With the largest reported series of rectal atresia/stenosis, we have demonstrated a safe and effective technique for repair. Preoperative evaluation must be thorough because a significant number of these patients will have an associated presacral mass. [Copyright &y& Elsevier]

Abstract: Trauma is a leading cause of death and disability in children. The following contribution discusses the important anatomical and physiological differences between children and adults. Important aspects of the recognition and management of thoracic and abdominal injuries in children are discussed. [Copyright &y& Elsevier]

Abstract: Background/Purpose: It is important to establish a physiologic range of gastric emptying (GE) in children. Gastric emptying time measured with 99Tc-labeled solid meal (GETc) is the gold standard. Large-scale studies with GETc are ideal but not feasible because of radiation exposure. The 13C-labeled octanoic acid breath test (GE13C) does not involve radiation and is suitable for large studies. The aim of this study was to validate GE13C with GETc and to explore whether GE13C could be used to establish a physiologic range of GE in children. Method: Twenty-five healthy children underwent simultaneous GETc and GE13C using a standardized solid test meal. The time taken for the stomach to empty half its content (T½13C) and solid lag phase (lag13C) for GE13C were computed from the fractional excretion of 13C in expired air. T½13C and lag13C were compared with corresponding values obtained by GETc. Result: Correlation coefficient between T½13C and T½Tc was 0.69 (P < .01; r = 0.92 if 3 outliers were omitted). Correlation coefficient between lag13C and lagTc was 0.39 (P < .05). There was good agreement between the methods by the Bland Altman method. Conclusion: There is good agreement between GE13C and GETc. GE13C can be safely used to establish the reference range of GE in healthy children. [Copyright &y& Elsevier]

Abstract: Menkes disease is a rare disorder of copper metabolism. Massive bladder diverticula are a characteristic urological abnormality associated with this condition. In this case report, we describe excision of such diverticula with good postoperative results in terms of bladder function. There are only three previous instances reported of excision of these diverticula, and the postoperative outcome has not been described previously. [Copyright &y& Elsevier]

Abstract: Presence of an associated azygos lobe may cause problems during thoracotomy on the right side for esophageal atresia. Awareness of the anomaly allows its recognition and appropriate management. [Copyright &y& Elsevier]

An anticancer, entirely carbohydrate conjugate, Globo H-polysaccharide A1 (Globo H-PS A1), was chemically prepared and immunologically evaluated in C57BL/6 mice. Tumor associated carbohydrate antigen Globo H hexasaccharide was synthesized in an overall 7.8% yield employing a convergent [3 + 3] strategy that revealed an anomeric aminooxy group used for conjugation to oxidized PS A1 via an oxime linkage. Globo H-PS A1, formulated with adjuvants monophosphoryl lipid A and TiterMax® Gold. After immunization an antigen specific immune response was observed in ELISA with anti-Globo H IgG/IgM antibodies. Specificity of the corresponding antibodies was determined by FACS showing cell surface binding to Globo H-positive cancer cell lines MCF-7 and OVCAR-5. The anti-Globo H antibodies also exhibited complement-dependent cellular cytotoxicity against MCF-7 and OVCAR-5 cells., Competing Interests: The authors declare no conflict of interest., (This journal is © The Royal Society of Chemistry.)

Sortilin is a type I membrane glycoprotein belonging to the vacuolar protein sorting 10 protein (Vps10p) family of sorting receptors and is most abundantly expressed in the central nervous system. Sortilin has emerged as a key player in the regulation of neuronal viability and has been implicated as a possible therapeutic target in a range of disorders. Here, the identification of AF40431, the first reported small-molecule ligand of sortilin, is reported. Crystals of the sortilin-AF40431 complex were obtained by co-crystallization and the structure of the complex was solved to 2.7 Å resolution. AF40431 is bound in the neurotensin-binding site of sortilin, with the leucine moiety of AF40431 mimicking the binding mode of the C-terminal leucine of neurotensin and the 4-methylumbelliferone moiety of AF40431 forming π-stacking with a phenylalanine.

The identification of the novel, selective, orally bioavailable Sortilin inhibitor AF38469 is described. Structure-activity relationships and syntheses are reported, along with an X-ray crystal structure of the sortilin-AF38469 protein-inhibitor complex., (Copyright © 2013 Elsevier Ltd. All rights reserved.)