Late Diagnosis of Hirschsprung's Disease: Definition and Implication on Core Outcomes.

INTRODUCTION: Late diagnosis of Hirschsprung's disease (LDHD) may carry a poor prognosis. Its definition remains unclear and its implication on HD-related core outcomes has not been fully reported. METHODS: A single-center 20-year series was reviewed to include HD with follow-up of 1 year or more post pull-through (PT) and aged 5 years or older. We investigated six core outcomes derived from NETS1HD study by comparing the groups dichotomized by four time points using age at diagnosis (44-week gestation, 6 months, 1 year, and 3 years). Following establishment of definition of LDHD, the outcomes and complications were compared with timely diagnosis of HD (TDHD). RESULTS: Forty-nine out of eighty-six HD were included. The definition of LDHD was found to be HD diagnosed at 1 year of age or later because 3/6 core outcomes were significantly worse than TDHD. Nine patients (18%) had LDHD-median age at diagnosis 42 months (12-89) and PT performed at 57 months (12-103), mostly Soave (73%); a covering stoma was performed in 7/9, significantly higher than TDHD in 10/40 (p = 0.001). LDHD was associated with increased unplanned surgery (78% vs. 30%, p = 0.019), fecal incontinence (100% vs. 62%, p = 0.01), and permanent stoma (33% vs. 5%, p = 0.037). Major complications (56% vs. 20%, p = 0.043) and redo PT (33% vs. 5%, p = 0.037) were also higher in LDHD. CONCLUSION: LDHD could be defined as HD diagnosis at or over 1 year of age. LDHD was associated with more preoperative stoma, major postoperative complications, unplanned reoperation, and worse HD-related core outcomes.

A Critical Analysis of Rectal Biopsy to Exclude Hirschsprung's Disease.

INTRODUCTION: Most Hirschsprung's disease (HD) are diagnosed in young children with increased risk ("red flag"). Older children (>6 months) require open rectal biopsy (ORB) with its own impact on risk and resources. We investigated if "red flag", age, and sex used in combination could exclude HD. MATERIALS AND METHODS: "Red flags" are risk factors associated with HD, including neonatal bowel obstruction, genetic association, failure of passage of meconium in <48 hours, infantile constipation, distension with vomiting, or family history. All rectal biopsies (2015-2018) were reviewed for indications, methods, and histopathological findings. Logistic regression analysis was adopted to assess predictive value of "red flag," age, and sex (p < 0.05* was significant). RESULTS: A total of 187 children underwent 84 suction rectal biopsies and 113 ORBs (n = 197 in total). Final histopathological diagnoses were non-HD (n = 154) and HD (n = 43). Total 78% of rectal biopsies were non-HD, of which 63% by ORB. Non-HD was associated with absence of "red flag" (49 vs. 16%*), increased age at biopsy (22 months vs. 28 days*), >6 months old (62 vs. 30%*), and female gender (54 vs. 16%*), compared with HD. In the absence of "red flag," 7/82 (9%) had HD (negative predictive value = 91%). Logistic regression analysis found absent "red flag" predicted non-HD biopsy with odds ratio 4.77 (1.38, 16.47), corrected for age and sex. CONCLUSION: Negative rectal biopsy rate for HD is very high. The majority required ORB. Although "red flag" and gender, but not age, have strong predictive values, it is inadequate for excluding HD. This study supports the need for alternative strategies in excluding HD.

Therapeutic strategies for stricturing Crohn's disease in childhood: a systematic review.

PURPOSE: Childhood stricturing Crohn's disease (CD) has significant morbidity. Interventions including resection, stricturoplasty and endoscopic balloon dilatation (EBD) are often required. Optimal intervention modality and timing, and use of adjuvant medical therapies, remains unclear. We aim to review the therapies used in paediatric stricturing CD. METHODS: A systematic review in accordance with PRISMA was performed (PROSPERO: CRD42020164464). Demographics, stricture features, interventions and outcomes were extracted. RESULTS: Fourteen studies were selected, including 177 patients (183 strictures). Strictures presented at 40.6 months (range 14-108) following CD diagnosis. Medical therapy was used in 142 patients for an average of 20.4 months (2-36), with a complete response in 11 (8%). Interventions were undertaken in 138 patients: 53 (38%) resections, 39 (28%) stricturoplasties, and 17 (12%) EBD. Complications occurred in 11% of resections, versus 15% stricturoplasties, versus 6% EBD (p = 0.223). At a median follow-up of 1.9 years (interquartile range 1.2-2.4) pooled stricture recurrence was 22%. Resection had 9% recurrence, versus 38% stricturoplasty, versus 47% EBD (p < 0.001). CONCLUSIONS: Resection is associated with a low incidence of recurrence and complications. There remains a paucity of evidence regarding adjuvant medical therapy and the role of EBD. We propose a minimum reported dataset for interventions in paediatric stricturing CD.

Feeding Jejunostomy: Is It a Safe Route in Pediatric Patients? Single Institution Experience.

INTRODUCTION: Impossibility to place a gastrostomy and failed gastroesophageal reflux surgery with unsafe swallow are the main indications to Feeding Jejunostomy (FJ) in children. The aim of this study is to quantify the incidence of complications associated with FJ. MATERIALS AND METHODS: A retrospective review of patients who had surgically inserted FJ between January 2009 and August 2013 at our institution was conducted. Data were obtained from medical records, operative notes, and radiology database, focusing on complications. RESULTS: A total of 19 patients, average age 39.6 months (3-168 months), were treated during the study period. Indications to FJ were gastroesophageal reflux disease (GERD) associated with unsafe swallow in 12, esophageal atresia in 5, and foregut dysmotility in 2. Seventeen FJ were inserted via laparotomy and 2 were laparoscopically assisted. In all cases, a serosal tunnel on the antimesenteric border was fashioned. No intraoperative complications were recorded. Tube dislodgement/blockage occurred on an average of 0.48 times per month in 18 out of 19 patients. The average radiation dose received for tube reinsertion/manipulation was 3.316 mSv/year/patient (0-10.66). Major postoperative complications occurred in 7 out of 19. After an average follow-up of 21 months, two have abandoned the use of FJ due to poor tolerance and three have fully weaned off. Two patients died due to unrelated causes. CONCLUSION: FJ, as an alternative means for enteral feeding, may require multiple readmissions and exposure to radiological procedures. The high risk of severe complications should be considered when offering this procedure.

Can common serum biomarkers predict complicated appendicitis in children?

PURPOSE: As appendicitis in children can be managed differently according to the severity of the disease, we investigated whether commonly used serum biomarkers on admission could distinguish between simple and complicated appendicitis. METHODS: Admission white blood cell (WBC), neutrophil (NEU), and C-reactive protein (CRP) levels were analysed by ROC curve, and Kruskal-Wallis and contingency tests. Patients were divided according to age and histology [normal appendix (NA), simple appendicitis (SA), complicated appendicitis (CA)]. RESULTS: Of 1197 children (NA = 186, SA = 685, CA = 326), 7% were <5 years, 55% 5-12, 38% 13-17. CA patients had higher CRP and WBC levels than NA and SA (p < 0.0001). NEU levels were lower in NA compared to SA or CA (p < 0.0001), but were similar between SA and CA (p = 0.6). CA patients had higher CRP and WBC levels than SA patients in 5-12- (p < 0.0001) and 13-17-year groups (p = 0.0075, p = 0.005), but not in <5-year group (p = 0.72, p = 0.81). We found CRP >40 mg/L in 58% CA and 37% SA (p < 0.0001), and WBC >15 × 109/L in 58% CA and 43% SA (p < 0.0001). CONCLUSIONS: Admission CRP and WBC levels may help the clinician predict complicated appendicitis in children older than 5 years of age. Early distinction of appendicitis severity using these tests may guide caregivers in the preoperative decision-making process.

Learning Curves in Pediatric Minimally Invasive Surgery: A Systematic Review of the Literature and a Framework for Reporting.

AIMS: There exists a learning curve (LC) with the adoption of any minimally invasive surgical (MIS) technique with implications for training, implementation, and evaluation. A standardized approach to describing and analyzing LCs in pediatric MIS is lacking. We sought to determine how pediatric MIS LCs are quantified and present a framework for reporting. METHODS: Systematic search of MEDLINE and EMBASE 1985-October 2015 for articles describing MIS in the pediatric population and presenting formal analysis of the LC. Articles screened by two independent reviewers. RESULTS: Twenty-nine articles (n = 17 general abdominal/thoracic, n = 12 urological) from an 18-year period (1997-2015) were included representing 3345 procedures (n = 3116 laparoscopic, n = 10 thoracoscopic, n = 219 robotic). Seven (24%) were prospective, three multicenter. Twenty-two (76%) presented data pertaining to >1 operating surgeon. Operative time was the most commonly employed surrogate of proficiency (n = 26 [90%] studies). Twenty (69%) described >1 LC outcome measure. Sixteen additional measures were described, including conversion (n = 12 studies); blood loss (n = 4 studies); complications (n = 10 studies); and postoperative outcomes (n = 14 studies). Three studies assessed impact of LC on trainees and one considered economic impact. LCs were presented in tabular form (n = 14 studies) and graphically (n = 19). Eleven (38%) studies undertook statistical appraisal utilizing comparative statistics (n = 8 studies) and regression analysis (n = 4 studies). CONCLUSIONS: Multiple outcome measures of proficiency are employed in reporting pediatric MIS experience and analysis of LCs is inconsistent. A standardized multioutcome approach to reporting should be encouraged. In addition, attempts should be made to quantify the impact on trainee involvement. We present an idealized framework for reporting.

The value of contrast studies in the evaluation of bowel strictures after necrotising enterocolitis.

PURPOSE: Strictures of the bowel are a frequent complication post-necrotising enterocolitis (NEC). Contrast studies are routinely performed prior to stoma closure following NEC. The aim of this study was to evaluate the ability of these studies to detect strictures and also directly compare them to operative and histological findings. METHODS: Two hundred and fourteen neonates who had a diagnosis of NEC (Bell stage 2 or greater) in a single unit (2007-2011) were analysed. Their case notes, radiology, and histology were reviewed. RESULTS: One hundred and sixteen neonates underwent an emergency laparotomy and 77 had stomas fashioned. Sixty-six patients had a contrast study prior to stoma closure (distal loopogram 18, contrast enema 37, both studies 11). Colonic strictures were reported in 18 patients and small bowel strictures were reported in two patients. Fourteen of these colonic strictures were confirmed at operation and on histology but three colonic strictures were missed on contrast studies; one patient had had both contrast studies and the other two only a distal loopogram. Two small bowel strictures reported were confirmed and an additional small bowel stricture missed on distal loopogram was also detected at the time of operation. The incidence of post-op strictures was 19 out of 68 patients (27.9 %) and 16 (84.2 %) of these strictures were found in the colon. Contrast enemas had a much higher sensitivity for detecting post-NEC colonic strictures than distal loopograms; 93 versus 50 %, respectively; however, they are more likely to give a false positive result and therefore their specificity is lower; 88 versus 95 %, respectively. CONCLUSION: Colon is the commonest site for post-NEC stricture and contrast enema is the study of choice for detecting these strictures prior to stoma closure.

Motion Analysis-Based Skills Training and Assessment in Pediatric Laparoscopy: Construct, Concurrent, and Content Validity for the eoSim Simulator.

PURPOSE: To validate the eoSim(®) (eoSurgical Ltd., Edinburgh, Scotland, United Kingdom) simulator for pediatric laparoscopy. MATERIALS AND METHODS: Participants were stratified according to their pediatric laparoscopy expertise. Three tasks were tested on the Pediatric Laparoscopic Surgery (PLS) and adapted eoSim simulators. Skill assessment was undertaken using motion analysis software for eoSim tasks and an existing validated scoring system for PLS tasks. Content validity was determined using Likert scale graded feedback responses. Construct validity was evaluated by investigating the respective abilities of the eoSim and PLS assessment tools to differentiate levels of experience. Concurrent validity was investigated by assessing the relationship between PLS and eoSim task completion times. RESULTS: In total, 28 participants (8 experts, 7 intermediates, and 13 novices) were recruited. Content validity results were comparable or more favorable for the eoSim. Construct validity for motion analysis parameters was established for instrument path length (objects transfer, P = .025; suturing, P = .012), speed (suturing, P = .034), acceleration (suturing, P = .048), and smoothness (suturing, P < .001). For all tasks, there were significant differences between level of experience groups for eoSim task completion times and PLS scores (P = .038 to < .001). Significant relationships were found between eoSim and PLS task completion times for the precision cutting and suturing tasks (ρ = 0.298 and ρ = 0.435, respectively). CONCLUSIONS: This study demonstrates validity of the adapted eoSim simulator for training in pediatric laparoscopy. Future work should focus on implementing and evaluating the proficiency-based training curriculum that is proposed using construct validity-derived metrics.

Postprandial hyperinsulinaemic hypoglycaemia secondary to a congenital portosystemic shunt.

BACKGROUND: Portosystemic shunts (PSS) are abnormal vascular connections between the portal vein or its tributaries and the systemic vein that allow mesenteric blood to reach the systemic circulation without first passing through the liver. PSS can be associated with various syndromes and can lead to serious complications. We report a rare case of a child with PSS and recurrent hypoglycaemia. CASE: A 20-month-old girl with Down's syndrome presented with recurrent hypoglycaemic episodes. She had multiple anomalies including a ventricular septal defect, oesophageal atresia and tracheo-esophageal fistula, gastro-oesophageal reflux, and conjugated hyperbilirubinaemia. The initial investigations suggested hyperinsulinaemic hypoglycaemia (HH). She did not respond to diazoxide. An oral glucose tolerance test suggested postprandial HH. Further vascular imaging showed a side-to-side portocaval shunt (Abernethy malformation) with relative hypoperfusion of the liver. Hypoglycaemia resolved following surgical closure of the portocaval shunt. CONCLUSION: PSS can rarely be associated with HH, possibly due to lack of insulin degradation in the liver. Surgical closure of the shunt resolves the hypoglycaemia.

Dysbiosis anticipating necrotizing enterocolitis in very premature infants.

BACKGROUND: Necrotizing enterocolitis (NEC) is a devastating inflammatory bowel disease of premature infants speculatively associated with infection. Suspected NEC can be indistinguishable from sepsis, and in established cases an infant may die within hours of diagnosis. Present treatment is supportive. A means of presymptomatic diagnosis is urgently needed. We aimed to identify microbial signatures in the gastrointestinal microbiota preceding NEC diagnosis in premature infants. METHODS: Fecal samples and clinical data were collected from a 2-year cohort of 369 premature neonates. Next-generation sequencing of 16S ribosomal RNA gene regions was used to characterize the microbiota of prediagnosis fecal samples from 12 neonates with NEC, 8 with suspected NEC, and 44 controls. Logistic regression was used to determine clinical characteristics and operational taxonomic units (OTUs) discriminating cases from controls. Samples were cultured and isolates identified using matrix-assisted laser desorption/ionization-time of flight. Clostridial isolates were typed and toxin genes detected. RESULTS: A clostridial OTU was overabundant in prediagnosis samples from infants with established NEC (P = .006). Culture confirmed the presence of Clostridium perfringens type A. Fluorescent amplified fragment-length polymorphism typing established that no isolates were identical. Prediagnosis samples from NEC infants not carrying profuse C. perfringens revealed an overabundance of a Klebsiella OTU (P = .049). Prolonged continuous positive airway pressure (CPAP) therapy with supplemental oxygen was also associated with increased NEC risk. CONCLUSIONS: Two fecal microbiota signatures (Clostridium and Klebsiella OTUs) and need for prolonged CPAP oxygen signal increased risk of NEC in presymptomatic infants. These biomarkers will assist development of a screening tool to allow very early diagnosis of NEC. Clinical Trials Registration. NCT01102738.

Simulation in the early management of gastroschisis.

AIM: Our aim was to design, create, and validate a simulator model and simulation scenario for the early management of gastroschisis. METHODS: Candidates of varying surgical experience had 1 attempt on an abdominal wall defect simulator and were scored for 4 different aspects: resuscitation of the neonate, application of a silo by both a global rating scale and a procedure-specific checklist, and nontechnical skills (scored by Non-Technical Skills scale). Surgical trainees subsequently received a focused teaching module on the resuscitative management and the surgical decision-making process, including bowel protection methods. Trainees then had a second attempt, which was objectively analyzed for improvement. RESULTS: Candidates attempted the simulation and were assessed, looking for construct validity. There was a statistically significant difference between candidate experience levels for all aspects of the simulation (resuscitation, global rating scale, procedure-specific checklist, and nontechnical skills) calculated using analysis of variance. Feedback forms gave us face validity, with a mean adjusted score of 8.3/10 for realism. After teaching the module, there was a statistically significant improvement (P < 0.05) of 20% for technical skills and 10% for nontechnical skills, which is comparable with similar controlled studies. CONCLUSIONS: We showed that creating and running a simulation scenario for the early management of gastroschisis is a feasible and useful tool for training and assessment. The simulation may also be able to discriminate between experience levels and could be used as a teaching aid to improve a surgeon's technical and nontechnical skills.

The incidence of intraabdominal abscess formation following laparoscopic appendicectomy in children: a systematic review and meta-analysis.

AIMS: Recent systematic reviews have suggested an increased incidence of intraabdominal abscess (IAA) formation following laparoscopic appendicectomy (LA) compared with the open approach (OA). As the majority of these analyses have focused on appendicectomy in adults, our aim was to review the evidence base for pediatric patients. SUBJECTS AND METHODS: We performed a comprehensive review of relevant studies published between 1990 and 2012. Specific inclusion and exclusion criteria were used to identify studies that investigated the incidence of IAA following LA and OA in pediatric patients. The primary outcome measure in the present meta-analysis was IAA formation, and secondary outcomes included wound infection (WI) and incidence of postoperative small bowel obstruction (SBO). RESULTS: Sixty-six studies with a total of 22,060 pediatric patients were included: 56.5% OA and 43.5% LA. There was no overall difference in the incidence of IAA formation: 2.7% for OA (333/12,460) versus 2.9% for LA (282/9600) (P=.25). However, OA patients had a higher incidence of wound infection: 3.7% for OA (337/9228) versus 2.2% for LA (183/8154) (P<.001). Moreover, the incidence of SBO was lower in patients undergoing LA: 0.4% LA (86/5767) versus 1.5% (29/6840) (P<.001). CONCLUSIONS: The IAA incidence is comparable in LA versus OA in pediatric patients. LA confers a significantly lower risk of other postoperative complications, including WI and SBO.

Varicocele surgery: 10 years of experience in two pediatric surgical centers.

AIM: The study was designed to compare recurrence rates and complications after laparoscopic versus open varicocele surgery in children. SUBJECTS AND METHODS: A retrospective case-note review of all varicocele surgery over a 10-year period (April 1999-March 2009) in two pediatric surgical centers was performed. Multivariate analysis using logistic regression was performed using SPSS Statistics version 18 (SPSS Inc., Chicago, IL). RESULTS: Thirty-seven patients had varicocele surgery during the study period. The median age at surgery was 14 years (range, 11-16 years). Most children had left-sided Grade 2 varicocele. Twenty-five (68%) primary procedures were laparoscopic (17 artery-sparing), and 12 (32%) procedures were open (9 artery-sparing). Six (16%) children had recurrence, and 6 (16%) had postoperative hydrocele. Recurrence rates after laparoscopic (16%) and open (17%) surgery were similar. Increasing age significantly decreased recurrence (odds ratio, 0.373; 95% confidence interval 0.161-0.862; P = .021). Although laparoscopy was associated with higher rates of postoperative hydrocele (odds ratio, 2.817; 95% confidence interval, 0.035-3.595; P = .380) and artery-sparing ligation was associated with higher rates of recurrence (odds ratio, 2.667; 95% confidence interval, 0.022-4.235; P = .787), these associations were not statistically significant. CONCLUSIONS: The best results of varicocele surgery in terms of recurrence and postoperative hydrocele were achieved by open mass ligation; however, larger prospective studies are warranted.

Comparison of intraabdominal abscess formation after laparoscopic and open appendicectomies in children.

AIM: Although laparoscopic appendicectomy (LA) is an accepted alternative to the open appendicectomy (OA) approach, it has been suggested that there is a higher incidence of intraabdominal abscesses (IAAs). Our aim was to determine the incidence of IAA in 3 pediatric surgical centers routinely practicing both techniques. METHODS: Data were collected retrospectively for pediatric patients undergoing LA or OA over an 8-year period. Analysis included IAA formation, appendicitis complexity, radiologic/histologic investigations, grade of surgeon, and wound infection. MAIN RESULTS: A total of 1267 appendicectomies were performed (514 LAs and 753 OAs). There was no difference between the incidences of IAA (LA, 3.9% [19/491] vs OA, 3.9% [28/714]; P = 1.0). The incidence of IAA was increased in those with complicated appendicitis (34/375 [9.1%] vs 13/830 [1.6%]; P ≤ .0001). There was an increased proportion of those with complicated appendicitis in the LA group (182/491 [37.1%] vs 193/714 [27.0%]; P = .0002). Surgical trainees were more likely to be the primary surgeon in the OA group (79% vs 63%; P = .0001), although the incidence of IAA did not correlate with grade of surgeon. There was no significant difference in incidence of wound infection between groups (LA, 4.6% [8/173] vs OA, 2.5% [18/377]; P = .93). CONCLUSION: This large retrospective study shows that the technique of appendicectomy does not appear to affect the incidence of IAAs. Patients with complicated appendicitis are more likely to develop an IAA regardless of technique.

Isolated gallbladder perforation following blunt abdominal trauma in a six-year-old child.

Isolated perforation of the gallbladder secondary to blunt trauma is rare. Furthermore, only a few cases exist in the infant age group. It has vague symptoms and interpretation of the radiology imaging is challenging. Diagnosis is usually made at operation. We report the case of a six-year-old boy who fell on to the handlebars of his scooter, sustaining an isolated gallbladder perforation. The authors highlight the importance of interpreting the volume of intraperitoneal fluid and early diagnostic laparoscopy.

Gene expression profiling in male genital lichen sclerosus.

Male genital lichen sclerosus (MGLSc) has a bimodal distribution in boys and men. It is associated with squamous cell carcinoma (SCC). The pathogenesis of MGLSc is unknown. HPV and autoimmune mechanisms have been mooted. Anti extracellular matrix protein (ECM)1 antibodies have been identified in women with GLSc. The gene expression pattern of LSc is unknown. Using DNA microarrays we studied differences in gene expression in healthy and diseased prepuces obtained at circumcision in adult males with MGLSc (n = 4), paediatric LSc (n = 2) and normal healthy paediatric foreskin (n = 4). In adult samples 51 genes with significantly increased expression and 87 genes with significantly reduced expression were identified; paediatric samples revealed 190 genes with significantly increased expression and 148 genes with significantly reduced expression. Concordance of expression profiles between adult and paediatric samples indicates the same disease process. Functional analysis revealed increased expression in the adult and child MGSLc samples in the immune response/cellular defence gene ontology (GO) category and reduced expression in other categories including genes related to squamous cancer. No specific HPV, autoimmune or squamous carcinogenesis-associated gene expression patterns were found. ECM1 and CABLES1 expression were significantly reduced in paediatric and adult samples respectively.

Does thoracoscopic congenital diaphragmatic hernia repair cause a significant intraoperative acidosis when compared to an open abdominal approach?

PURPOSE: Thoracoscopic congenital diaphragmatic hernia (CDH) repair is increasingly reported. A significant intraoperative acidosis secondary to the pneumocarbia, as well as an increased recurrence rate, are possible concerns. Our aim was to review our early experience of the technique. METHODS: A prospective and retrospective data collection was carried out on all patients undergoing either an open or thoracoscopic CDH repair for a 4-year period. Preoperative blood gas values were identified at various stages of the operative procedure. A pH of 7.2 was considered to be a significant acidosis. The duration of surgery, complications, and recurrence rates were also recorded. Data were analyzed using the Mann-Whitney U test, and a P value of .05 or less was considered significant. RESULTS: Twenty-two patients were included. One death occurred before surgery. Twelve patients underwent thoracoscopic repair (8 neonatal), and 9 underwent open repair (8 neonatal). There were 9 left-sided defects in the thoracoscopic group and 9 in the open group. Operative time was longer in the thoracoscopic group compared to the open group (median, 135 vs 93.5 minutes; P = .02). Neonates undergoing thoracoscopic repair were heavier compared to the open group (median, 3.9 vs 2.9 kg; P = .05), and their preoperative requirements for ventilation and inotropes were comparable. However, the association between those patients who required preoperative inotropes and those who required a patch repair was statistically significant P = .03. Two patients in each group developed an intraoperative acidosis. A further patient in the thoracoscopic group had a severe acidosis present at the beginning of surgery. There was no statistical difference in pH values or recurrence rate between the 2 groups. All recurrences were in patients requiring patch repairs. No postoperative mortality occurred. CONCLUSIONS: We present our early experience of thoracoscopic CDH repair. Our results from thoracoscopic repair appear similar to the open procedure performed over the same period. No clear difference in intraoperative pH or recurrence rate has been demonstrated in our series. There is a need for a multicenter prospective study to establish the longer term outcome of this technique.

Percutaneous endoscopic gastrostomy placement in a human immunodeficiency virus-positive pediatric population leads to an increase in minor complications.

AIM: The development of effective multiple drug regimens for treating human immunodeficiency virus (HIV) are associated with nonadherence in children. HIV-positive children also have a higher incidence of malnutrition. Placement of a percutaneous endoscopic gastrostomy (PEG) is a potential solution. Primary outcome was to determine the complications of PEG placement in a pediatric HIV-positive population. MATERIALS AND METHODS: A 10 year retrospective data analysis was carried out on all HIV-positive children undergoing insertion of a PEG at two institutions. Parameters examined included infections, leakage, displacement, reasons for removal, total time in situ, HIV stage, CD4 count, and serological investigation. Data were compared against published data for PEG insertion in pediatric oncology patients and other comparable pediatric series using Fisher's exact test. RESULTS: Eighteen children were identified, with a median age 35 months and follow-up of 62 months. The majority of patients had advanced disease (Stage C; 65%). Fifty percent of PEGs were inserted for feeding supplementation and all were used for the administration of medications. Sixty-one percent experienced a minor complication; 5/18 (27.7%) experienced peristomal infection; 2/18 (11.1%) experienced either bleeding, leakage, or excessive granulation; and 1/18 (5.6%) experienced dislodgement. Stage of HIV did not affect the incidence of bleeding or infection: 5/11(Stage C) versus 2/7(Stage B) (P = .3). There was no significant difference for major complications when compared with any series though comparison with a large pediatric series revealed a significant difference for minor complications 11/18 versus 27/120 (P = .0003). CONCLUSIONS: There is a low rate of serious complications with PEG insertion in our patients, and the rate is comparable to that seen in pediatric oncology patients. The minor complication rate is, however, higher than a nonimmune compromised population; and careful follow-up for these patients is recommended so that the appropriate therapy can be promptly initiated.

Health-related quality of life in children with achalasia.

AIMS: To assess self-reported QoL in children with achalasia aged 5-18 and compare this with both disease and healthy control children in a prospective study. METHODS: All children diagnosed with achalasia at one hospital were asked to participate in this study by completing the self-report module of the validated PedsQL™ generic QoL assessment. All children attending a tertiary paediatric gastroenterology clinic from February 2009 to May 2009 with chronic constipation or inflammatory bowel disease were asked to participate in this study as disease controls. The PedsQL™ considers physical, emotional, social and school domains and is scored from 0-100. Healthy children were also recruited from the same site. Groups were compared using Analysis of Variance with Tukey's post-hoc test. RESULTS: One hundred and sixty one children completed the assessment (90 (56%) male, mean age 11.3 yrs ± 3.4 years) including 17 children with achalasia, 44 with chronic constipation, 59 with inflammatory bowel disease and 41 healthy children. QoL was significantly lower in the achalasia group compared to both children with IBD (73 vs. 82, p = 0.035) and healthy children (73 vs. 84, p = 0.005), and was comparable to that of children with chronic constipation (73 vs. 74, p = 0.99). CONCLUSION: Children with achalasia report a significantly lower QoL compared to children with inflammatory bowel disease and healthy children.

Surgery in children with Crohn disease refractory to medical therapy.

OBJECTIVE: The aim of this study was to evaluate the results of surgery in children with Crohn disease (CD) not responding to medical therapy and establish whether surgery improves growth and nutrition. PATIENTS AND METHODS: Children with CD diagnosed between 1998 and 2008 were reviewed. Relapse was defined by Harvey-Bradshaw index >5. Data, reported as median (range), were compared by Fisher exact test and repeated-measures ANOVA. RESULTS: One hundred forty-one children, ages 12.7 years (3.5-16.8), were identified; 27 (19%) required surgery 14.5 months (1.1-61.8) after diagnosis. Twenty-one had elective surgery (19 isolated ileocaecal disease and stricture, 2 diffuse disease of ileum); 6 had emergency surgery (3 peritonitis, 2 haemorrhage, 1 perforation). Surgery included 18 ileocaecal resection and end-to-end anastomosis, 5 stoma formation, 2 left hemicolectomy and end-to-end anastomosis, and 2 stricturoplasty. Follow-up was 2.5 years (1-9.4). Growth and nutrition improved by 6 and 12 months after surgery, with a significant increase in weight z score (P < 0.0001), height z score (P < 0.0001), albumin (30 [13-36] vs 39 [30-46] vs 40 [33-45], P < 0.0001), and haemoglobin [10 (6.8-13.2) vs 11.7 (8.2-13.7) vs 12.0 (9.3-14.7), P < 0.0001]. All patients of the received azathioprine (2-2.5 mg · kg⁻¹ · day⁻¹) after surgery. Fifteen patients (55%) relapsed with a modified Harvey-Bradshaw index of 8 (6-11) within 11.5 months (4.2-33.4). Of these, 5 patients (18%) relapsed within 1 year. Five patients (18%) had further surgery (2 anastomotic strictures, 2 diseased stoma, and 1 enterocutaneous fistula). CONCLUSIONS: Growth and nutrition following surgery for CD improve, but there is a high relapse rate. Despite this, the improved growth and nutrition before relapse may be beneficial during puberty and justify surgery in children not responding to medications.