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Background Although serial transverse enteroplasty (STEP) improves function of dilated short bowel, a significant proportion of patients require repeat surgery. To address underlying reasons for unsuccessful STEP, we compared small intestinal mucosal characteristics between initial and repeat STEP procedures in children with short bowel syndrome (SBS). Methods Fifteen SBS children, who underwent 13 first and 7 repeat STEP procedures with full thickness small bowel samples at median age 1.5 years (IQR 0.7–3.7) were included. The specimens were analyzed histologically for mucosal morphology, inflammation and muscular thickness. Mucosal proliferation and apoptosis was analyzed with MIB1 and Tunel immunohistochemistry. Results Median small bowel length increased 42% by initial STEP and 13% by repeat STEP (p = 0.05), while enteral caloric intake increased from 6% to 36% (p = 0.07) during 14 (12-42) months between the procedures. Abnormal mucosal inflammation was frequently observed both at initial (69%) and additional STEP (86%, p = 0.52) surgery. Villus height, crypt depth, enterocyte proliferation and apoptosis as well as muscular thickness were comparable at first and repeat STEP (p > 0.05 for all). Patients, who required repeat STEP tended to be younger (p = 0.057) with less apoptotic crypt cells (p = 0.031) at first STEP. Absence of ileocecal valve associated with increased intraepithelial leukocyte count and reduced crypt cell proliferation index (p Conclusions No adaptive mucosal hyperplasia or muscular alterations occurred between first and repeat STEP. Persistent inflammation and lacking mucosal growth may contribute to continuing bowel dysfunction in SBS children, who require repeat STEP procedure, especially after removal of the ileocecal valve. Level of evidence Level IV, retrospective study.

Background and objectives Sarcopenia, defined as reduced muscle mass, is typically assessed by CT scans, which are infrequently performed in children. Using MRI to measure sarcopenia, we determined the association with postoperative complications after colectomy for ulcerative colitis (UC). Methods Clinical and preoperative MRI data for 13–18-year-old UC patients who underwent colectomy were retrospectively reviewed. Bilateral paraspinous muscle area (PSMA) and psoas muscle area (PMA) at L3 vertebra were measured and averaged. Composite complications were infection, wound dehiscence, postoperative leak/abscess, prolonged ileus, pulmonary embolism, venous thromboembolism, or readmission. Results Twenty-nine patients with average age 15.9±1.36years and weight 61.5±19.8kg had a preoperative MRI. The 18/29(62%) with complications had significantly reduced PSMA (4.71±1.44 vs 5.64±1.38cm2, p=0.04) and PMA (7.16±2.60 vs 8.93±2.44, p=0.04). When stratified and compared to highest PSMA, patients with lowest PSMA had increased complication rates (88% vs 29%, p=0.04). There were no differences in age, BMI, albumin, CRP, ESR, or preoperative steroid or anti-TNFα use. Odds of complication in the lowest tertile were 25.0-fold higher than the highest tertile (p=0.04, 95% CI=1.2–520.73). Conclusion This is the first study to show low PSMA on MRI is associated with complications and increased hospital stay after colectomy in children with UC. Levels of evidence Level III retrospective comparative study.

Fecal incontinence is a socially debilitating problem for many children. We hypothesized that in selected patients with medically-refractory encopresis, placement of an appendicostomy or cecostomy tube for administration of antegrade continence enemas (ACE) would improve quality of life (QOL).We reviewed all patients with encopresis who underwent appendicostomy or cecostomy placement from 2003 to 2014 at our institution. We contacted subjects' parents by phone and administered 3 surveys: a survey reflecting current stooling habits, a disease-specific QOL survey, and the PedsQL™ QOL survey. QOL surveys were completed twice by parents, once reflecting pre-operative QOL, then again reflecting current QOL. Pre-procedure and post-procedure scores were compared by paired t-test.Ten patients underwent appendicostomy/cecostomy for encopresis. Eight completed phone surveys. All procedures were performed laparoscopically. All patients experienced fecal soiling pre-operatively, whereas 5/8 surveyed patients (63%) noted complete resolution of soiling post-procedure (p0.01). General and disease-specific QOL improved from pre-procedure to post-procedure in the following domains: social habits, physical activity, ability to spend the night elsewhere, feeling, and overall QOL (p0.05). PedsQL™ scores improved significantly in physical functioning, social functioning, and overall functioning (p0.05).Antegrade continence enemas significantly improve quality of life in patients with medically-refractory encopresis, likely related to resolution of soiling.4.

Purpose Chronic obstructive defecation can occur in patients with Hirschsprung Disease (HD) and internal anal sphincter (IAS) achalasia. Injection of Botulinum Toxin (BoTox) into the IAS can temporarily relieve obstructive defecation, but can be challenging when performed by tactile sense alone. We compared results of BoTox injections with and without ultrasound (US) guidance. Methods We retrospectively reviewed BoTox injections into the IAS for obstructive defecation over 5years. Analyzed outcomes included short-term improvement, defined as resolution of enterocolitis, new ability to spontaneously defecate, and/or normalization of bowel movement frequency 2weeks post-operatively, as well as requirement of more definitive surgical therapy (myotomy/myomectomy, colectomy, colostomy, cecostomy/appendicostomy, and/or sacral nerve stimulator implantation). Outcomes were compared using t-test and Fisher's Exact test, with significance defined as p Results Twelve patients who underwent BoTox injection were included, including 5 patients who underwent injections both with and without ultrasound. Ten underwent an ultrasound-guided injection (13 injection procedures), 5 of whom had HD. Seven underwent an injection without ultrasound guidance (17 injection procedures), 5 of whom had HD. Procedures performed with US resulted in greater short-term improvement (76% versus 65% without ultrasound) and less requirement of a definitive procedure for obstructive defecation (p Conclusions US-guided BoTox injection is safe and effective for obstructive defecation, and may decrease the need for a definitive operation. Level of Evidence III.

Purpose Previous methods of distraction enterogenesis have relied upon blind-ending intestinal segments or transmural device fixation, requiring multiple operations and potential bowel injury. We hypothesized that using a novel attachment would allow reversible device coupling to the luminal bowel surface, achieving effective endoluminal distraction. Methods A telescopic hydraulic device was designed with latex balloon attachments covered with high-friction mesh and a dilating fenestrated elastic mask (DFM attachment), allowing mesh-to-mucosa contact only with inflation. Yorkshire pigs underwent jejunal Roux-en-Y limb creation and device placement via jejunostomy. Devices underwent 3cycles of balloon inflation and hydraulic extension/retraction per day for 7days and then explanted and studied for efficacy. Results DFM attachment allowed reversible, high-strength endoluminal coupling without tissue injury or reduction in bowel perfusion. After 7day implant, distracted bowel achieved a 44±2% increase in length vs. fed, nondistracted bowel, corresponding to a gain of 7.1±0.3cm. Distracted bowel demonstrated increased epithelial cell proliferation vs. control bowel. Attachment sites demonstrated villus flattening, increased crypt depth, thicker muscularis mucosa, and unchanged muscularis propria thickness vs. control. Conclusion Novel high-strength, reversible attachments enabled fully endoluminal distraction enterogenesis, achieving length gains comparable to open surgical techniques. This approach may allow development of clinically applicable technology for SBS treatment.

Objective To identify the optimal clinical criteria to diagnose Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung disease (HSCR). Background HAEC is the most common life-threatening complication in HSCR patients, yet the diagnostic criteria for HAEC remain unclear. The consensus-based HAEC scoring system was not validated using patient data, thereby making its diagnostic accuracy uncertain. Methods From 2009 to 2015, consecutive children with HSCR underwent retrospective evaluation of their medical records, and questionnaire-directed parent interviews to identify treatment of suspected HAEC episodes and the 16 clinical criteria in the HAEC score. Logistic regression modeling was employed to identify criteria predicting suspected HAEC episodes. Results One hundred sixteen HSCR patients met inclusion criteria, 43 patients (37.1%) were treated for at least one suspected HAEC episode. An HAEC score of 4 maximized the sum of sensitivity (83.7%) and specificity (98.6%) while the previously established cut-off score of 10 showed lower sensitivity (41.9%) with perfect specificity. Multivariable analysis identified four criteria utilized to create a new HAEC Risk score with performance characteristics similar to the HAEC score cutoff of 4. Conclusion When using the HAEC score, a cutoff of 4 should be used rather than 10, which under-diagnosed patients with HAEC. Alternatively, the new HAEC Risk score could be employed. Level of Evidence Diagnostic Study, Level 3.

Hydrocolpos presenting at birth in a neonate with a cloacal malformation may lead to massive distension, with compression of adjacent structures. At times, the hydrocolpos requires urgent drainage. Additionally, these neonates need a divided colostomy to divert their fecal stream and prevent genitourinary contamination. We present a novel approach by which these two procedures can be performed as a single operation guided by a combination of cystoscopy, colposcopy, and laparoscopy. This provides a minimally invasive technique with excellent outcome and potentially superior visualization of the necessary intra-abdominal structures.

Objective Hirschsprung-associated enterocolitis (HAEC) is one of the most troublesome problems encountered after a pullthrough. We hypothesized that prophylactic administration of probiotics after a pullthrough procedure would decrease the incidence of HAEC. Study Design A prospective, double-blind, placebo-controlled, randomized trial was conducted at 2 children's hospitals. Infants undergoing pullthrough were randomized to probiotic or placebo for a period of 3months post-pullthrough. Primary outcome was incidence of post-operative HAEC. Other outcomes included severity of HAEC by clinical grade, number of HAEC episodes and extent of aganglionosis. Pearson Chi Square analysis, as well as logistic regression, was used for statistical analysis. Results Sixty-two patients were recruited (Sites: A=40; B=22). One was lost to follow up and one immediate post-op death was not included in final analysis. Probiotics were administered to 32 patients. Distribution of placebo/probiotics was equal between sites (P=0.858). Mean age at pullthrough was 6.5±8.1(±SD) months. The incidence of HAEC was 28.3%. The incidence of HAEC was not statistically different between probiotic and placebo study groups. Conclusions Incidence of HAEC was not reduced with prophylactic probiotics. Future studies are needed to better determine the etiology and possible ways of preventing this complex condition.

Purpose Short bowel syndrome (SBS) is a highly morbid condition primarily because of parenteral nutrition (PN)–associated complications. Bowel lengthening via serial transverse enteroplasty (STEP) has become standard of care. While initial STEPs have resulted in weaning from PN, outcomes of repeated STEPs (ReSTEPs) are not well described. We investigated outcomes of initial STEP compared to ReSTEP procedures. Methods This retrospective review of STEPs included 17 children and a total of 24 procedures. Demographics, complications, hospital readmission rates, postoperative costs, and PN weaning were analyzed. Results Neither patient-specific data nor the etiology of SBS was predictive of requiring a ReSTEP. PN weaning was more likely in the year following a first STEP (18% wean rate vs. 0% for ReSTEP, p >.05). No ReSTEP patients reached enteral autonomy. Enteral nutrition (%EN) increases were greater after first STEP compared to ReSTEP (26.0% vs. 4.7%, p =0.03). This trend was true for bowel length as well, where first STEPs resulted in a 51% increase in bowel length compared to a 20% increase after in ReSTEP ( p =0.02). Conclusions ReSTEPs failed to result in significant PN weaning, with no ReSTEP patients achieving enteral autonomy during follow-up. Given its higher costs, smaller bowel length gains, and limited ability to produce enteral autonomy, surgeons should carefully consider performing ReSTEP procedures. Level of evidence Level III.

The purpose of this study was to define morbidity from gastrojejunostomy tube (GJT) placement in children.A retrospective single-center 5-year review of GJT placement in children was performed. Age, weight, prior surgery, indication, type of GJT, and complications (GJT replacement, wound complications, and perforation) were recorded. Logistic regression for morbidity was performed.142 children underwent 394 GJT placements at a median age of 2.7years (range 5 weeks-18years). The most common indications were failure to thrive (62%) and reflux (25%). Among the 296 GJT replacements, the most common reason was tube dislodgement (30%). Risk factors for replacement, which occurred at a median interval of 12 weeks (range 2days-2.4years), were peristomal complaint (OR=5.4, p=0.02) and prior GJT replacement (OR=1.8, p=0.03). In all, 7 (5%) jejunal perforations occurred at a median of 3 days (range 0-21 days) from GJT placement. Patients with perforation had a median weight of 4.6kg (range 3-11.2kg) and age of 3.9months (range 8 weeks-2.1years). Lower weight (p0.01) and younger age (p=0.02) predicted perforation, with those weighing less than 6kg (OR=51.9, p0.001) or younger than 6months (OR=28.6, p0.01) at highest risk.GJT placement has a significant risk of recurrent dislodgement and the highest risk of perforation in children weighing less than 6kg or younger than 6months. Alternate feeding options should be strongly considered in this vulnerable population.

Biliary tract duplication cysts with heterotopic gastric mucosa are rare congenital anomalies, with our case representing only the fourth reported case in the literature. An 8-year-old girl with several months of abdominal pain was found to have a complex cystic mass communicating with the biliary system via the common hepatic duct. Intraoperatively, inflammation caused by the cystic mass was found to have resulted in a Mirizzi-like syndrome, with a nearly complete obstruction at the confluence of the left and right hepatic ducts. Histopathologic examination of the biliary mass revealed it to be a duplication cyst lined by heterotopic gastric mucosa with secondary ulceration and fibrosis. Biliary duplication cysts are a rare but important process that should be considered in a child with a mass in the portal triad and biliary obstruction.

Objective The aim of this study was to review evidence-based data addressing key clinical questions regarding parenteral nutrition–associated cholestasis (PNAC) and parenteral nutrition–associated liver disease (PNALD) in children. Data Source Data were obtained from PubMed, Medicine databases of the English literature (up to October 2010), and the Cochrane Database of Systematic Reviews. Study Selection The review of PNAC/PNALD has been divided into 4 areas to simplify one's understanding of the current knowledge regarding the pathogenesis and treatment of this disease: (1) nonnutrient risk factors associated with PNAC, (2) PNAC and lipid emulsions, (3) nutritional (nonlipid) considerations in the prevention of PNAC, and (4) supplemental medications in the prevention and treatment of PNAC. Results The data for each topic area relevant to the clinical practice of pediatric surgery were reviewed, evaluated, graded, and summarized. Conclusions Although the conditions of PNAC and PNALD have been well recognized for more than 30 years, only a few concrete associations and treatment protocols have been established.

Objective There is lack of data relating to the research interests and funding of pediatric surgeons within the United States and Canada. These data may be helpful in promoting basic and clinical research among pediatric surgeons. Methods The American Pediatric Surgical Association (APSA) Outcomes and Clinical Trials Committee developed and administered an online survey via e-mail to the APSA membership to help characterize research activities and funding. The survey was available for completion during December of 2009. The survey contained 10 items with a drop-down menu for multiple choice answers and required 5 to 10 minutes to complete. Results based on research interests as well as funding sources were compiled and analyzed. Results A total of 275 members, which comprises 27.4% of the APSA membership, completed the survey. Of the respondents, 177 (64%) described being in an academic practice, 44 (16%) in an academically associated private practice, 9 (3.3%) in a private solo practice, 17 (6.2%) in private group practice, and 3 (1%) in the military. A total of 189 (68.7%) respondents stated that they participated in formal research. Respondents also categorized their research interests, and the following were the most common subjects of study (decreasing order of frequency): appendicitis, trauma and critical care, outcomes, minimally invasive surgery, and congenital diaphragmatic hernia. Of those participating in research, 64.5% stated that they have no formal financial support. Of those supported through the National Institutes of Health, funding grants achieved were as follows: R01 (n = 29), K08 (n = 9), K23 (n = 2), and U01 (n = 8). Conclusions Research activities are common among APSA members and encompass a wide range of pediatric surgery topics. Strikingly, the overall financial support of these efforts is limited, predominantly supported by the surgeons themselves. Funded respondents attained grants through Public Health Service grants, departmental grants, or private institutions.

Background Proper nutrition for children with short bowel syndrome (SBS) is challenging. An underappreciated SBS cohort is the group of adolescents in which nutritional demand is markedly increased. We identified several children that previously had weaned off parenteral nutrition but needed re-initiation of parenteral nutrition or increased enteral nutritional supplementation (NS) during adolescence. This study aims to identify characteristics of adolescent SBS patients to better understand their nutritional status. Methods A single institution, retrospective review of SBS patients over 10 years was done. Patients were analyzed for NS need, remaining bowel length, height, weight, and essential nutrient levels. Results Sixteen adolescent SBS patients were identified for analysis. Of these, ten did not require increased NS upon reaching adolescence (group A), and six required increased NS during adolescence (group B). Group B patients required a mean increase in NS of 29.6 kcal/kg per day. Group B patients had significantly shorter remaining bowel length. NS resulted in minimal height increase, but more substantial gains in weight and pubertal development. Conclusions Adolescents with SBS may require NS beyond what can be provided enterally. These patients are at risk for nutritional deficiencies, and an aggressive approach to monitoring and supplementing these deficiencies is warranted, especially during this critical period of growth.

We describe a child who presented with a traumatic hepatic artery pseudoaneurysm and arterioportal fistula, which were subsequently managed with an endovascular stent graft and coil embolization using flow control with balloon remodeling. This case demonstrates a rarely seen condition in the pediatric population and a novel management strategy, which should be considered in the management of this complex injury.

Two infants are described who presented in the neonatal period with a direct hyperbilirubinemia. This was initially presumed to be because of the diagnosis of gastroschisis and the prolonged use of parenteral nutrition. However, both infants were eventually found to have an associated choledochal cyst. The cases are a novel association not previously reported and should heighten the awareness that anatomical causes of a direct hyperbilirubinemia need to be ruled out in all neonates.

Background Infants with severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) have a high morbidity and mortality. We hypothesized that placement of an abdominal wall silo and staged abdominal wall closure may reduce problems associated with decreased abdominal domain in CDH. Methods We performed a retrospective review and identified 7 CDH patients requiring ECMO who had a silastic abdominal wall silo between 2003 and 2006. Variables analyzed included survival, ECMO duration, duration of silo, time to discharge, and long-term outcome. Results Predicted mean survival for the entire cohort using the published CDH Study Group equation was 47% (range, 9%-86%). All 7 patients (100%) survived. Extracorporeal membrane oxygenation duration averaged 15 days (range, 5-19 days). Four of the patients (58%) were repaired with a silo on ECMO, and 3 (42%) had their repair after ECMO. The abdominal wall defect was closed at a mean of 21 days (range, 4-41 days). Hospital stay after silo placement averaged 54 days (range, 20-170 days) with no infections or wound complications. Conclusions Abdominal wall silo placement in infants with CDH requiring ECMO appears to be an effective strategy for decreased abdominal domain. Further studies are warranted to determine the efficacy of such a strategy for these high-risk CDH patients.

Purpose The serial transverse enteroplasty procedure (STEP) procedure is a successful and safe approach to lengthen small bowel in patients with short bowel syndrome (SBS). However, postlengthening dilatation may occur, which can lead to bacterial overgrowth and malabsorption. We addressed this problem by reperforming the STEP in 2 patients. Methods Two infants underwent the STEP procedure at 3 days and at 4 months of life for SBS secondary to intestinal atresia and gastroschisis. The patients' small bowel lengths were 20 and 32 cm before and 25 and 52 cm after the initial STEP. Nine and 8.5 months afterward, the patients developed dilatation of the small bowel and feeding intolerance. A second STEP procedure was undertaken with additional transverse firings of staplers between previously lengthened segments and tapering of redundant blind-ending portions of bowel. Results At operation, the bowel lengths were 45 and 62 cm. The second STEP left the patients with 61 and 73 cm of small bowel with a normalization of intestinal diameter. The first patient is doing well 5 months after surgery, and the second child tolerated increased enteral intake but died 1 year later from total parenteral nutrition-related liver failure. Conclusions This is the first report of a successful application of a second STEP procedure to further lengthen small bowel in SBS patients. Reapplication of the procedure requires careful stapling and a removal of small blind-ending segments to avoid further stasis.

Purpose The transanal endorectal pull-through (TERPT) is becoming the most popular procedure in the treatment of Hirschsprung disease (HD), but overstretching of the anal sphincters remains a critical issue that may impact the continence. This study examined the long-term outcome of TERPT versus conventional transabdominal (ABD) pull-through for HD. Methods Records of 41 patients more than 3 years old who underwent a pull-through for HD (TERPT, n=20; ABD, n=21) were reviewed, and their families were thoroughly interviewed and scored via a 15-item post–pull-through long-term outcome questionnaire. Patients were operated on between the years 1995 and 2003. During this time, our group transitioned from the ABD to the TERPT technique. Total scoring ranged from 0 to 40: 0 to 10, excellent; 11 to 20 good; 21 to 30 fair; 31 to 40 poor. A 2-tailed Student t test, analysis of covariance, as well as logistic and linear regression were used to analyze the collected data with confidence interval higher than 95%. Results Overall scores were similar. However, continence score was significantly better in the ABD group, and the stool pattern score was better in the TERPT group. A significant difference in age at interview between the 2 groups was noted; we therefore reanalyzed the data controlling for age, and this showed that age did not significantly affect the long-term scoring outcome between groups. Conclusion Our long-term study showed significantly better (2-fold) results regarding the continence score for the abdominal approach compared with the transanal pull-through. The stool pattern and enterocolitis scores were somewhat better for the TERPT group. These findings raise an important issue about the current surgical management of HD; however, more cases will need to be studied before a definitive conclusion can be drawn.

Purpose Children with Hirschsprung disease (HD) who have a history of enterocolitis (HAEC) have a shift in colonic microbiota, many of which are necessary for short chain fatty acid (SCFA) production. As SCFAs play a critical role in colonic mucosal preservation, we hypothesized that fecal SCFA composition is altered in children with HAEC. Methods A multicenter study enrolled 18 HD children, abstracting for history of feeding, antibiotic/probiotic use, and enterocolitis symptoms. HAEC status was determined per Pastor et al. criteria (12). Fresh feces were collected for microbial community analysis via 16S sequencing as well as SCFA analysis by gas chromatography–mass spectrometry. Results Nine patients had a history of HAEC, and nine had never had HAEC. Fecal samples from HAEC children showed a 4-fold decline in total SCFA concentration vs. non-HAEC HD patients. We then compared the relative composition of individual SCFAs and found reduced acetate and increased butyrate in HAEC children. Finally, we measured relative abundance of SCFA-producing fecal microbiota. Interestingly, 10 of 12 butyrate-producing genera as well as 3 of 4 acetate-producing genera demonstrated multi-fold expansion. Conclusion Children with HAEC history have reduced fecal SCFAs and altered SCFA profile. These findings suggest a complex interplay between the colonic metabolome and changes in microbiota, which may influence the pathogenesis of HAEC.

Purpose Laparoscopy has recently been used to treat intussusception that cannot be reduced radiologically. The effectiveness and practical nature of this approach has been questioned. Methods This study retrospectively analyzed the authors' experience with this laparoscopic approach and compared this to the conventional open laparotomy procedure. Results Sixteen patients were treated via laparoscopy, with 2 of these requiring conversions to an open procedure (12.5%). Twenty-five patients underwent an open reduction. Operative time was not significantly different ( P = .698) between the laparoscopic (49.56 ± 26.40 minutes) and open groups (45.00 ± 24.74). Length of stay, however, was significantly reduced ( P = .005) in the laparoscopic group (3.00 ± 1.31 days) compared to the open group (4.52 ± 1.98). Total hospital charges were lower in the laparoscopic group ($8171 ± 2595) compared to the open group ($11,672 ± 5466); this difference was not significant ( P = .088). There were no significant differences in intra- or postoperative complication rates ( P = .637) between the 2 approaches. Conclusions Although there remains a group who will require a conversion to an open procedure, the laparoscopic approach should be considered a safe and effective option for all children who do not respond to a radiological reduction.

The aim of this study was to review outcomes after surgical treatment of total colonic Hirschsprung's disease (TCH).Twenty-five records of patients with TCH treated between 1974 and 2002 were reviewed. Follow-up data were collected using a standardized questionnaire. Objective functional outcome was assessed using a scoring system.Twenty patients had aganglionosis of the colon and distal ileum, 5 of whom had a more extensive condition. One of these 5 patients underwent an endorectal pull-through (ERPT), 1 underwent intestinal transplantation, and 3 died. Four of the remaining 20 patients underwent a primary ERPT, 16 received a stoma as neonates followed by ERPT in 12, and a Martin-Duhamel procedure or Swenson's operation in 3 (median age, 10.5 months); 1 remains with an ostomy. Postoperative complications included enterocolitis (55%), anal stricture (25%), and perineal excoriation (20%). Mean follow-up were 17.5 years (+/-11.1 years). Eighty-nine percent were free of recurrent enterocolitis. Frequency of bowel movements is 1 to 5 per day in 82% of the patients, 18% have 6 or more bowel movements per day. Occasional soiling is noted in 40% (one third of those requiring nighttime diapers). Overall functional outcome was good in 83%. Those patients with the longest follow-up periods had the best stooling scores (P = .04).Surgical treatment of TCH is associated with a number of complications including recurrent enterocolitis and anal strictures. Long-term outcome is quite favorable.

Background The Stretta procedure is an endoluminal antireflux procedure using radio frequency to induce collagen tissue contraction, remodeling, and modulation of lower esophageal sphincter physiology in an effort to treat gastroesophageal reflux disease (GERD). Although Stretta has been widely reported in the adult GERD literature as a viable initial surgical option, similar use in children has not been reported. The authors present the first report of Stretta as the initial antireflux procedure in children with GERD, evaluating indications, safety, and efficacy. Method The charts of 8 children (aged 11-16 years) who received Stretta between January 2003 and September 2003 were retrospectively reviewed under an Institutional Review Board protocol. All patients had documented GERD preoperatively. Three children required concomitant feeding tube placement (percutaneous gastrostomy tube, group A). Five children with isolated severe GERD refractory to aggressive medical therapy received Stretta only (group B). Results Stretta was successfully completed in all 8 children. In group A, 1 child developed a postoperative aspiration, which was successfully treated. All 3 children had resolution of their GERD symptoms (ie, feeding intolerance, emesis) and were able to tolerate full enteral nutrition post-Stretta. In group B, 3 of 5 children are currently off medications and asymptomatic on short-term follow-up (6-15 months). Of the remaining 2, 1 experienced symptomatic relief immediately postprocedure, but symptoms recurred off medications. Stretta was deemed unsuccessful in the remaining patient, and Nissen fundoplication was subsequently performed without difficulty. Conclusions Stretta can be safely and successfully used as the initial antireflux procedure for children with GERD. Concomitant Stretta with PEG is an attractive option in children with preexisting GERD who require long-term feeding access. Longer follow-up and a larger patient population are needed to better confirm the safety and efficacy of Stretta presented in this report.

Background Hirschsprung's disease (HD) patients after pull-through (PT) may have recalcitrant constipation or recurrent enterocolitis (EC). Posterior myotomy/myectomy (POMM) are possible options for these problems. This study analyzed the outcome of POMM in HD patients post-PT. Methods Records of 348 HD patients were reviewed, and 32 were found to have undergone a POMM post-PT (1981 to 2002). Outcomes after this procedure were assessed. Statistics used linear and logistic regression. Results Of the 32 patients, 29 had complete records for analysis. Of those with pure constipation (12), 6 had aganglionosis post-PT. Of those with recurrent EC (17) only 1 had aganglionosis post-PT. POMM was performed at a mean of 3.1 years post-PT (11 myotomy, 18 myectomy). Average follow-up was 8.6 years (range, 0.7 to 21). Type of POMM had no correlation with overall functional outcome ( P = .44). Of those with chronic constipation, 60% had good results after POMM; the remainder required a redo-PT or colostomy. Interestingly, most patients with retained aganglionosis and chronic constipation did not respond after POMM (83%). Of those with recurrent EC, 75% became free of symptoms; none of the patients not responding have required redo-PT. Conclusions POMM to treat chronic constipation or recurrent EC in patients with HD post-PT is moderately successful. Because of the unsuccessful outcome with POMM in patients with a combination of constipation and aganglionosis, one should defer to a redo-PT in this group.

Background/purpose Over the last 4 years, the authors changed their management of acute nonperforated appendicitis from emergent surgery within the first 2 to 6 hours of admission to initiation of antibiotic therapy with operation within 24 hours of admission in those seen in the late evening or early morning. They examined, therefore, whether a delay in operation for acute appendicitis would affect outcome measures of patient morbidity and resource use. Methods The medical records of 126 patients with acute appendicitis occurring between 1998 and 2001 were retrospectively reviewed. Incidence of perforation at surgery, length of stay (LOS), hospital charges, operating time, and complications as a function of duration between emergency room (ER) triage and operation (ER-OR) or admission and operation (Admit-OR) were analyzed by Student's t test, and regression analysis with P less than .05 considered significant. Results Thirty-eight children (26%) were operated on within 6 hours of ER triage, whereas the remaining 88 children (74%) were operated on between 6 and 24 hours from ER triage. No significant difference was noted in perforation rate, LOS, costs, or operative time, nor were substantial changes in complications noted between those with an ER-OR ≤6 hours and greater than 6 hours. Likewise, no significant differences in these outcome measures were noted for Admit-OR greater than 6 when compared with ≤6 hours. Only costs with ER-OR greater than 12 hours and LOS with Admit-OR greater than 6 hours were significantly (without Bonferroni correction) different than ≤ 6 hours. Multivariable linear regression analysis identified only LOS as a significant predictor of time to OR. Conclusions In children with acute appendicitis, delaying surgery until the daytime hours did not significantly affect operating time, perforation rate, or complications. Delayed management allows greater efficiency and effective use of physician and hospital resources, including decreased resident involvement in operations during the night.

Purpose Recurrent gastroesophageal reflux disease (GERD) after gastroesophageal surgery is a troublesome problem. Reoperative surgery often is complicated by adhesions and recurrence. Radiofrequency ablation or energy delivery (RF or the Stretta procedure) is a new method for treating GERD. This study is the first report describing the use of the Stretta procedure in pediatric patients. Methods Six patients who underwent previous gastroesophageal surgery presented with recurrent GERD. Medical records were reviewed and the severity of reflux graded using a modified scoring system. All underwent RF and were graded for GERD at 6 months postprocedure. Results Mean operating time was 80 ± 12 minutes. Mean age at initial operation was 12 ± 4 years and for the RF, 18.0 ± 3.4 years. All patients were discharged as outpatients. Early complications occurred in one child with self-resolving acute gastric distension. Five of 6 patients were completely asymptomatic at 3 months after the procedure, and 3 stopped anti secretory agents. One patient was improved but still symptomatic and needed a redo fundoplication. Another required a repeat application of RF 10 months after the initial one. Mean GERD score pre-Stretta was 5.2 ± 1.0, which improved to 1.6 ± 1.9 at 6 months postprocedure ( P Conclusions Use of RF treatment of the lower esophageal sphincter is a potentially successful modality to treat recurrent GERD in children. Long-term follow-up is required.

The aim of this study was to utilize clinical outcome methodology through multivariable analysis of perioperative factors to predict a successful Kasai-portoenterostomy (PE).Records of 81 patients treated for biliary atresia (BA) were reviewed. Outcome was defined as successful if the patient was alive and had no liver transplant (LT). To predict future successful or failed PE, patients were categorized at 6 months post-PE into 2 groups: Success: direct bilirubin (DB) less than 2.0 mg/dL; Failure: DB greater than 2 mg/dL, or the patient was listed/had undergone LT, or had died. Groups were analyzed for positive or negative predictive values (PPV, NPV) at 2 and 5 years after PE. Cox regression was used to determine risk factors for PE.PE was successful in 38% and failed in 62%. PPV of future success was 96% at 2 years post-PE and 95% at 5 years post-PE, NPV of failure was 76% and 74%, respectively. Bridging liver fibrosis at the time of PE and postoperative cholangitic episodes were interdependent risk factors for a failed PE (P.05). Other covariates showed no significant relationship for PE outcome.Classifying of patients 6 months postoperatively allowed us to determine a successful PE outcome. Bridging liver fibrosis at the time of the Kasai, and the increased number of postoperative cholangitic episodes were predictive of a poor PE outcome.

Background/Purpose: Total parenteral nutrition (TPN) induces epithelial cell (EC) apoptosis. Keratinocyte Growth Factor (KGF) increases EC-growth; however, little is known of its effect on apoptosis. This study aims to determine if mRNA expression of Bcl-2 proteins (major mediators of epithelial cell apoptosis) is altered with TPN, and if KGF-administration influences Bcl-2 family expression. Methods: C57BL/6J mice (n = 6 per group) received oral feeding (control), TPN (TPN), or TPN plus intravenous KGF daily (TPN + KGF). After 7 days, intestine was harvested and EC isolated. Apoptosis was identified using flow cytometry. EC mRNA expression of Bcl-2 family members was measured by reverse transcriptase polymerase chain reaction; Bcl-2 protein level was measured by immunoblot analysis. Results: EC apoptotic rates were: control, 14.4% ± 5.1%; TPN, 29.4% ± 11.3%; KGF, 17.2% ± 5.6%. Pro-apoptotic Bcl-2 proteins changed minimally with TPN or KGF; however, the antiapoptotic protein Bcl-2 changed significantly: control, 0.78 ± 0.24; TPN, 0.10 ± 0.13; KGF, 0.76 ± 0.36. EC Bcl-2 protein levels were: control, 0.16 ± 0.13; TPN 0.18 ± 0.16; and TPN + KGF 0.47 ± 0.19. Conclusions: TPN-induced apoptosis decreased Bcl-2 mRNA expression. KGF decreased EC apoptosis and increased Bcl-2 expression. Modalities to increase endogenous KGF, or KGF-administration may have benefit in patients on TPN. J Pediatr Surg 38:92-96. Copyright 2003, Elsevier Science (USA). All rights reserved.

Background/Purpose: Although thoracoscopy in children is a well-accepted technique, there are limitations to this approach. Small lung nodules or those not located on the pleural surface often require an open thoracotomy. The purpose of this report is to evaluate the utility of computed tomography (CT) guided needle localization of lung nodules in children followed by thoracoscopic resection. Methods: From 1997 to 2000, 13 thoracoscopic procedures were performed on 12 children who presented with small pulmonary nodules (

This study examined predictors of achieving enteral autonomy among pediatric short bowel syndrome (SBS) patients remaining on parenteral nutrition (PN) beyond one year.A retrospective single-institution study of 171 pediatric SBS patients (defined as ≥50% small bowel (SB) loss or ≥60 days of PN with onset before 6 weeks of age) was performed. Multivariate Cox proportional hazards analysis was conducted, with subgroup analysis of patients on PN for ≥1 year (n=59). Primary outcome was successful wean from PN.Over a follow-up of 4.1±4.8 years, 64.3% of children weaned from PN. Mortality was 15.2%. Presence of ≥10% expected SB length (hazard ratio [HR] 6.48, p=0.002) or an ileocecal valve (ICV; HR, 2.86, p0.001) predicted PN weaning. Of those on PN ≥1 year, the wean rate was 50.8%, and ICV no longer predicted weaning (p=0.153). Predictors among those on PN ≥1 year were: ≥10% expected SB length (HR, 8.27, p=0.010), intestinal atresia (HR, 4.26, p=0.011), and necrotizing enterocolitis (NEC, HR, 2.84, p=0.025).SBS children on PN ≥1 year continue to wean from PN, and those with ≥10% of predicted SB length, NEC, or atresia are more likely to do so. These findings may help direct management and advice for these challenging patients.