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In 1981, Currarino et al described a triad of findings that consist of partial sacral dysgenesis, presacral mass (anterior meningocele, enteric cyst, or presacral teratoma) and anorectal malformation. Currarino syndrome exhibits variable expressivity and the clinical presentation tends to vary with the age of the subject such as spinal anomaly detected in the fetus, imperforate anus in the newborn, and intractable constipation or neurologic symptoms in the infant and older child. At any age, meningitis can be the presenting symptom and imaging is required for proper investigation. Meningitis, sepsis, urinary tract infections, and, rarely, malignant transformation of a teratoma are serious potential complications. This pictorial review describes the imaging findings, clinical history, surgical interventions, and genetic background in 5 children with this syndrome who presented in our hospital in the interval of 1 year.

The etiology of infantile hypertrophic pyloric stenosis (HPS) is not fully understood. The objective of this study was to determine whether formula-feeding is associated with increased incidence.This case-control study included HPS cases and controls admitted between 1992 and 2012. Demographic data including feeding method were collected from patient charts and analyzed.We identified 882 HPS cases and 955 controls. The highest incidence of HPS presentation was in summer (P=0.0028). Infants with HPS were more likely to have been exclusively formula-fed, have a family history of HPS, and be male compared to infants in the control group (P0.001); they were also more likely to live in rural areas, although not significantly so. After adjusting for family history, sex, place of residence, and season of presentation, exclusively formula-fed infants were 1.36 times more likely to develop HPS compared with exclusively breastfed infants (RR 1.36, 95% CI 1.18-1.57, P0.005).Formula-feeding is associated with significantly increased risk of HPS. Further investigation may help to determine the components of formula that simulate hypertrophy of the pylorus muscle, or the components of breast milk that are protective, as well as other influencing factors.3b.

The Kwak Thyroid Imaging Reporting and Data System (Kwak-TI-RADS) guideline (2011) and American College of Radiology Thyroid Imaging Reporting and Data System (ACR TI-RADS) guideline (2017) were developed as ultrasound (US) risk stratification tools for detecting thyroid malignancy in adults. The purpose of this study was to investigate the inter-rater reliability and diagnostic performance of the ACR TI-RADS guideline in the pediatric population and compare it to the Kwak guideline. This retrospective study comprised 75 children who underwent thyroid US at a tertiary-level pediatric hospital. Three pediatric radiologists and one pediatric radiology fellow graded the US findings using the Kwak-TI-RADS and ACR TI-RADS guidelines. We assessed reliability of radiologists’ ratings using percentage inter-rater agreement, and intra-class correlation coefficients (ICC2,1). We assessed area-under-the-receiver-operating-characteristic curve (AUROCC) to compare the discriminative diagnostic ability of the Kwak-TI-RADS and ACR TI-RADS scoring systems against histopathology/cytology, or stability on US over a 2-year follow-up period for cases without tissue diagnosis. The inter-rater agreement was significantly better for the ACR TI-RADS level compared to the Kwak-TI-RADS level (P

After definitive repair of esophageal atresia (EA) and tracheoesophageal fistula (TEF), a chest tube (CT) may be left in place to diagnose and conservatively treat a postoperative anastomotic leak, although its necessity is controversial. The purpose of this study was to determine if the use of a CT decreases rates of early postoperative complications after EA and TEF repair. A retrospective chart review was performed to identify all patients with EA and TEF who underwent repair between 1985 and 2012. Univariate analysis was performed to compare patients who did or did not have a CT in terms of preoperative characteristics and postoperative outcomes. One hundred twenty neonates were included; 69 had a CT inserted intraoperatively, while 51 did not. The two groups were similar in terms of patient characteristics. There was no statistically significant difference between the groups in terms of length of hospital stay (31 ± 12 vs. 36 ± 16 days, p = 0.5), or complication rates (13% vs. 12%, p = 0.9) for those with or without CT, respectively. The use of a CT does not alter early postoperative complications after EA/TEF repair.

This paper examines narratives from young men seeking non-medical circumcision. Non-medical circumcision remains controversial, yet there are young men seeking elective circumcision. At our centre, young men 15 to 17 seeking elective circumcision were asked to write short pieces to reflect their understanding and reason(s) for wanting circumcision. Fourteen youth participated; four changed their minds about the procedure. Findings are described and evaluated within the medico-legal description of informed consent, and, from a narrative ethics perspective explicitly concerned with giving voice to patients. Persons are presumed capable of making their own health care decisions, yet, with youth, there may be a concern that an irrevocable decision may be coerced, or impulsive. Narrative reflections provide a means by which (i) misinformation/misunderstanding can be resolved (ii) coercion can be minimized and (iii) impulsivity mitigated. This paper describes our experience with narrative reflection and aims to facilitate informed decision-making for elective procedures among youth, and other potentially vulnerable populations.

Background Postoperative complication from the ileoanal anastomosis (J-pouch) procedure for surgical management of refractory ulcerative colitis (UC) increases the risk of pouch dysfunction. The purpose of this study was to determine if histologic inflammatory activity of the rectal margin is an independent predictor of complication after controlling for other variables. Methods A retrospective chart review was performed to identify all pediatric patients with UC who underwent a J-pouch procedure between 1995 and 2014. Univariate and multivariate regression analyses were performed on the following variables: age at surgery, body mass index, comorbidities, time between colectomy and pouch, mucosectomy, protective ileostomy status, length of pouch, and histologic inflammatory activity in the intestinal epithelium of the rectal margin. Results Nineteen complicated and 23 uncomplicated cases were included. Histologic inflammatory activity was significantly higher among the complicated group (9.3±3.1 vs. 4.1±3.1, p=0.02). No significant difference was found regarding other variables. In a multivariate regression, histologic inflammation of the rectal margin remained significantly associated with complication (p=0.04) after adjusting for other factors. Conclusion After controlling for potential confounders, histologic inflammatory activity at the rectal margin was found to be a significant predictor of postoperative complication in the J-pouch procedure for refractory UC. Level of evidence: 2b.

Background Several comparative studies are published evaluating both the open and the minimally invasive approaches for congenital lung lesions with inconsistent results. Our objective was to compare both procedures using systematic review and meta-analysis methodology. Methods All publications describing both interventions were reviewed. The statistical analysis was performed using RevMan 5 software (Cochrane library). Main Results No randomized trials were identified. Six retrospective studies were identified and were included in this study. There was no significant difference in overall complication rates between both techniques. Lengths of hospital stay as well as days with chest tube in place were longer with the open approach. There was no difference in the duration of surgery. Postoperative pain management was heterogeneous between studies. No study looked at long-term follow-up. Subgroup analysis for congenital cystic adenomatoid malformation of the lung was done. Conclusions Our results suggest no differences between thoracotomy versus thoracoscopy for congenital lung lesions with respect to overall complications and the duration of surgery. However, length of hospital stay and days with chest tube in place were longer after the open approach. Thoracoscopic resection is a safe and feasible alternative to open resection of congenital lung lesions in experienced hands.

Fowler and Stephens showed that by dividing the spermatic vessels a high intra-abdominal testis could be placed in the scrotum. Testicular atrophy is a potential complication of this technique. We conducted a systematic review to determine whether single or 2-stage Fowler-Stephens orchiopexy results in better testicular viability.We searched electronic databases, clinical trial registries and gray literature. We included reports describing boys younger than 18 years with a primary outcome of "testicular viability and position." We performed a meta-analysis using random effects models. Heterogeneity was assessed using forest plot and I(2) statistic.We identified 1,807 citations and included 61 articles. Single stage Fowler-Stephens orchiopexy was discussed in 9 articles, a 2-stage procedure in 36 and both approaches in 16. There were no randomized controlled trials, and most studies were cohort or case series. The pooled estimate of success rates was 80% for single stage Fowler-Stephens orchiopexy (95% CI 75 to 86) and 85% for 2-stage Fowler-Stephens orchiopexy (95% CI 81 to 90). The pooled odds ratio of single stage vs 2-stage Fowler-Stephens orchiopexy was 2.0 (95% CI 1.1 to 3.9) favoring the 2-stage procedure. There was no difference in the success rate between laparoscopic and open techniques in either single or 2-stage Fowler-Stephens orchiopexy. There was no evidence of asymmetry on the funnel plot. There were no complications reported with single stage, while ileus, hematoma and infection were the most common complications with 2-stage Fowler-Stephens orchiopexy.Both techniques have a fairly high success rate but 2-stage Fowler-Stephens orchiopexy appears to carry a higher rate of success than the single stage approach (85% vs 80%, OR 2 in favor of 2-stage). Laparoscopic and open techniques had the same success rate. However, the level of evidence of the studies was low, and a study of a more robust design, such as a randomized controlled trial, should be performed.

The objectives of this study were to describe the effects of an alternate payment plan (APP) on clinical surgical practice--at eight surgical divisions of Children's Hospital of Eastern Ontario--and to emphasize the important role of corporate indicators (CIs). To do this, we analyzed CIs comparing two years before the implementation of the APP with years one and two and years three and four post-implementation. The number of in-hospital consultations decreased in division two comparing pre-APP encounters with years one and two and years three and four post-APP. There was no difference between years one and two and three and four post-APP. Encounters in outpatient clinics increased in divisions four and seven. Division six had a decrease in encounters comparing pre-APP with years one and two and years three and four post-APP. There was no difference between years one and two and three and four post-APP. Encounters for same-day surgery increased in division six after the implantation of the APP; division two had a decrease comparing pre-APP with years one and two and years three and four post-APP. No difference was seen between years one and two and three and four post-APP. For in-patient surgery, only division eight had a significant decrease in encounters comparing pre-APP with years one and two and years three and four post-APP. There was no difference between years one and two and three and four post-APP. This study demonstrates that the APP has had little influence on patterns of clinical practice in our institution, which is the sole pediatric referral centre for the region. Since CIs are produced on a yearly basis by the institution and physicians have no influence on the data, CIs may play a role in replacing shadow billing as a way of measuring healthcare service delivery in an APP setting on academic institutions.

The J-pouch is a surgical procedure offered to children with refractory ulcerative colitis (UC) who have undergone subtotal colectomy to reconstruct a reservoir function with ileo-anal anastomosis. Unfortunately, post-operative complications may occur and can compromise the pouch function. We assessed rectal histopathology to determine whether severity of inflammation in the rectum prior to the creation of the J-Pouch was associated with post-operative complications. We retrospectively reviewed the histopathology of all J-pouch procedure specimens from paediatric patients during the period 2000-2013 using an objective grading system that assesses the chronicity and activity of the UC disease. We analysed the parameters for association with the post-operative complications. A classification tree algorithm was generated to predict the risk of complication based on histopathological parameters. A total of 28 paediatric patients were identified, among whom 10 developed post-operative complications (35%). The activity score at the recto-anal margin was higher among the patients with post-operative complications (mean 7.3±3.1 versus 4.8±3.1; p=0.04). The involvement of more than 5% colonic crypts with epithelial neutrophilic infiltration at the recto-anal margin was found to be an independent parameter that would stratify the patients into low-risk or high-risk group for developing complications (17% versus 64%; p=0.04). An association between UC disease activity at the recto-anal margin and post-operative J-pouch complications was determined. Potentially, this association suggests that a histopathological assessment of the recto-anal transitional zone may have value in guiding the surgeon on the risk of post-operative complications.

Gastrostomy tube (G-tube) removal has been suggested at 3 months following successful renal transplantation (Tx). We noticed a delay in the removal of G-tubes in our pediatric patients following Tx and therefore conducted a cross-sectional single center survey on all renal transplant recipients to assess the causes of delayed G-tube removal. In total, 17 of 23 patients completed the survey, including all patients with G-tubes (n = 10) and seven patients without G-tubes. Median age at Tx of gastrostomy patients was significantly lower than that of patients without a G-tube (median 3.0 yr, range 1.2-4.7 yr vs. median 14 yr, range 6-17 yr, p < 0.0001) and significantly younger than in previous studies on gastrostomies. At the time of the survey, only three of 10 patients had their G-tube removed at 22, 41 and 61 months after Tx at the ages of 3, 5.5 and 9 yr, respectively. The median age at recent follow up of the remaining seven patients was 4.7 yr (range 2.6-8.75 yr). The most important reason for continued use was fluid intake and medication. Results of our survey showed appropriate concern regarding the risk of prolonged use of the gastrostomy. Caregivers felt that benefits of the gastrostomy in the post-transplant period outweighed the risks. Caregivers felt pressured towards removal by the physicians. Our findings support the decision to remove gastrostomies based on individual patient needs and total fluid intake rather than a fixed time following successful Tx.

Purpose: The aim of this study was to describe a new technique for the repair of high and intermediate imperforate anus. Methods: From 1989 to 1999, 22 children with high and intermediate imperforate anus (17 boys, 5 girls) were operated on with a combination of a posterior sagittal and 3-flap perineal anoplasty. Long-term clinical follow up (to a maximum of 10 years) was done in all patients as well as a recent phone interview with a questionnaire regarding bowel function and degree of satisfaction with the result of the surgical correction. A continence ratio (CR, patient score/maximum possible score) was obtained by a blinded interviewer. Results: A continence survey was obtained in 19 patients. The average CR was 0.68. The CR for high anomalies was 0.62 and for intermediate anomalies was 0.78 (0.84 for girls and 0.64 for boys). Patients with sacral anomalies had a CR of 0.58. Two patients with Trisomy 21 had associated Hirschsprung's disease and were excluded from analysis. Conclusions: Advantages of this combined surgical approach are excellent anatomic exposure, the ability to limit rectal mobilization to a minimum, reduction of the incidence of mucosal prolapse, the new skin-lined anal canal may assist attainment of continence by providing a "sensory warning zone," and, finally, the cosmetic appearance is satisfactory. J Pediatr Surg 36:711-714. Copyright © 2001 by W.B. Saunders Company.

Background/Purpose: With modern chemotherapeutic protocols and advances in medical care, the outcome of intraabdominal non-Hodgkin's lymphoma (NHL) in children can be excellent for limited disease. Advanced disease, however, is associated with increased tumor aggression and requires more rigorous adjuvant therapy. Hence, complications early in the course of the disease process or its management often lead to a poor outcome. Perforation of the gastrointestinal tract, either iatrogenic, tumor related or chemotherapeutically induced is one such complication and may result significant morbidity and mortality. Methods: The authors reviewed their experience with this disease, and present two cases of children with abdominal NHL, which poignantly demonstrate these points. Results and a review of the literature are then discussed. Results: Fifteen cases of abdominal NHL were examined with an overall mortality rate of 40%. This increased to 100% in the presence of perforation. In two cases, inadvertent entry into the bowel occurred at the time of laparotomy for tumor biopsy. In the first case, intestinal wall was included in the biopsy specimen; in the second, laparotomy unmasked an already sealed-off perforation secondary to tumor invasion. Sepsis ensued in both cases. In the first, this resulted in repeated delays in chemotherapy, and the child succumbed to the disease. In the second, chemotherapy was continued, and although the small bowel leak was controlled, the initial insult hampered marrow recovery and host defenses, resulting in fatal sepsis. Conclusions: These data and other reported cases in the literature indicate that intestinal perforation associated with abdominal lymphomas in children portends an extremely poor prognosis. All attempts to avoid this complication should be made, including avoiding direct tumor biopsy whenever posible.

Background The optimal method to achieve analgesia after inguinal hernia repair in children is unknown. This study compared the analgesic efficacy, adverse events, and the costs associated with supplementation of local anesthesia (infiltration of the wound) with either intravenous ketorolac or caudal analgesia in children having inguinal hernia repair. Methods With parental consent and institutional review board approval, children aged 2-6 yr having elective, outpatient inguinal hernia repair were studied in this randomized, single-blinded investigation. Anesthesia was induced by inhalation with nitrous oxide and halothane or intravenously with propofol. Anesthesia was maintained with nitrous oxide and halothane. Patients were randomly assigned to receive caudal analgesia (1 ml/kg 0.20% bupivacaine with 1/200,000 epinephrine) or intravenous ketorolac (1 mg/kg) immediately after induction of anesthesia. Both groups received field blocks with 0.25% bupivacaine administered by the surgeon under direct vision during operation. Patients were assessed for 24 h. In-hospital pain was assessed using a behavior-based pain score. Parents assessed pain with a visual linear analog pain scale with anchors of 0 (no pain) and 100 (worst pain imaginable). Results The authors studied 164 children, with 84 patients in the ketorolac group. The groups had similar demographic data. In-hospital analgesic requirements and pain scores were almost identical in both groups. Pain at home was significantly less in the ketorolac group, with visual linear analog pain scale scores of 10 (0-80) compared with 20 (0-80) (median [range]) for ketorolac versus caudal (P = 0.002 by the Mann-Whitney U test). The ketorolac group also had a lower incidence of vomiting, ambulated more rapidly, and micturated earlier (P < 0.05). Conclusion The use of intravenous ketorolac to supplement local anesthesia infiltrated by the surgeon during pediatric inguinal hernia repair is superior to supplementation with caudal analgesia.

We present a histochemical study of resected colon from a 13-year-old boy diagnosed with rectal ectasia. Laminar preparations and sectioned tissue of rectum were assayed histochemically for nitric oxide (NO) synthase activity by reducing nitro blue tetrazolium salt in the presence of the cofactor NADPH. Tissue preparations displayed intensely labelled neurons and fibers throughout the gut wall. Laminar preparations of Meissner's plexus showed a hyperplasia of ganglia and NO-related neurons throughout the length of the resected rectum compared with normal bowel. Sectioned tissue of the Auerbach's plexus demonstrated a normal number of ganglia and NO-related neurons. As well, the ectatic bowel showed a proliferation of nerve fibers in keeping with the degree of circular smooth muscle hypertrophy. This proliferation may represent a reactive phenomenon secondary to the functional obstruction. The NO histochemical technique may form the basis of further investigations in defining the cause of this functional obstruction.

The most common risk factors for the development of necrotizing enterocolitis (NEC) are prematurity and enteral feeding. Most models of NEC involve ischemic insult resulting in generalized necrosis, different from the classical ileocecal predilection of NEC. This anatomic predisposition is explained by dysmotility of immature gut, leading to bacterial overgrowth in the terminal ileum and colon. Infant formula containing lactose as the sole carbohydrate source overwhelms partially developed lactase activity, allowing enteric bacteria to ferment excess carbohydrate to short-chain fatty acids, decreasing intraluminal gut pH and predisposing to mucosal injury. Impaired clearance of intraluminal contents exacerbates this effect. In the present study the authors used a model of NEC, originally developed in rabbits and based on analysis of intestinal contents of NEC babies, modified and adapted here to neonatal piglets, the gastrointestinal tract of which more closely resembles the human neonate.Piglets3 days old and 2 weeks old were laparotomized. Loops created from the distal ileum to the proximal colon were injected with isoosmolar acidified casein solution or 0.9% saline. Segments were harvested 3 hours later, sectioned for HE, and graded from 0 (intact villi) to 4 (transmural necrosis).Acidified casein-induced damage included areas of necrosis, submucosal edema, inflammatory cell infiltrate, and lymphatic distension. In younger animals, lesions were more pronounced (3.25 +/- 0.13 for the3-day-old v 2.43 +/- 0.14 for the 2-week-old piglets; P.005).The authors believe that this piglet NEC model most closely approximates human NEC because it incorporates two of the most common risk factors: dysmotility (by creating intestinal loops) and enteral feeding (by intraluminal injection of acidified casein).

Inflammatory cloacogenic polyp (ICP) is a rare lesion arising in the region of the anorectal transitional zone. It is likely caused by occult internal prolapse. Most cases reported in the literature are in the adult population. This is a report of ICP in four children. Awareness of this entity in children is important because of both the propensity for recurrence or persistence of the polyps if the underlying etiology is not corrected, as well as the long-term implications of internal intussusception: procidentia, descending perineum syndrome, and ultimately, incontinence.

Background/Purpose There is considerable controversy regarding optimal mode and timing of delivery for fetuses with gastroschisis. Our objectives were to describe the variation in institutional approach regarding these factors, and to evaluate the effect of timing of delivery on outcomes in fetuses with gastroschesis. Methods Members of the maternal–fetal medicine community across Canada were surveyed regarding their personal and institutional approach of delivery. Data from the Canadian Pediatric Surgery Network (CAPSnet) were analyzed. Results The survey showed significant variability in delivery approach between institutions, although no center routinely performs cesarean section. Infants delivered vaginally (VD) were categorized into three groups: Group 1, VD n =114); Group 2, VD 36–37weeks ( n =218); and Group 3, VD ≥38weeks ( n =75). Score of Neonatal Acute Physiology, complication rates, length of time on total parenteral nutrition (TPN), and length of hospital stay (LOS) were higher in Group 1; bowel matting was greater in Group 3. There were no differences between the groups regarding other complications. Conclusions Our data suggest that preterm delivery was associated with more complications, longer time on TPN, and longer LOS; delivery ≥38weeks was associated with increased bowel matting. These outcomes should be considered when determining institutional protocol.

The purpose of this study was to analyze the factors that affect the longevity of central venous catheters.Comprehensive clinical data recorded during insertion and removal of totally implantable devices (TID) and tunneled lines (TL) from October 1988 to January 2009 were analyzed. Univariate and multivariate Cox proportional hazards regression models were used to identify clinical factors that predict catheter longevity.Information was available for 1167 central venous catheter insertions in 858 patients, 648 TID and 509 TL. Univariate analysis detected longer device longevity in the following: TID longer than TL (P.0001), catheter tip in the superior vena cava (SVC)/right atrial junction (P.0001), and right side greater than left (P = .002). Shorter device longevity was observed in lines used for total parenteral nutrition (P.0001) and young age (P.0001). Multivariate model detected the following: hazard of removal for TID is 0.304 that of TL (P.0001) and SVC is 0.525 that of other locations (P = .0005). Hazard decreases by 5.4% for every 1-year increase in patient age (P.0004).Multiple confounding factors were encountered. However, the single most important factor in catheter longevity that is influenced by the surgeon is tip location in the SVC/right atrial junction.

Pediatric inguinal and scrotal surgeries for inguinal hernia, cryptorchidism and hydrocele are common and usually involve the excision of a hernia sac. Groups at many centers send hernia sacs for pathological analysis to identify occult disease as well as structures that may have been erroneously resected. We hypothesized that, since the incidence of significant findings is low and the associated health care costs are significant, the routine pathological analysis of inguinal hernia sacs is unnecessary.After receiving institutional review board approval we retrospectively reviewed pathology reports at our institution of patients who underwent surgery with an inguinal hernia sac sent for pathological analysis from January 2000 to September 2009. The primary outcome was to determine the incidence of clinically significant structures in hernia sac specimens. The secondary outcome was to evaluate the costs associated with analyzing these specimens.A total of 2,287 boys and 441 girls underwent some form of inguinal or scrotal surgery during the study. In the 2,287 boys a total of 2,657 hernia sac specimens were analyzed, of which 2 (0.08%) contained clusters of epididymal-like tubules. Most unexpected findings were likely clinically insignificant, including mesothelial proliferation in 5.6% of cases, genital duct remnants in 0.8%, lipoma in 0.23% and adrenocortical rests in 0.04%. The average cost of analyzing hernia sac specimens at our institution was approximately $7,100 Canadian annually.Routine analysis of inguinal hernia sacs is unnecessary and costly, and should be reserved for cases in which resection of important structures such as the vas deferens is suspected.

Erosion of the skin over a totally implanted vascular access device (TIVAD) is a rare event that may lead to life-threatening sequelae. From 1994 to 2007, we reviewed the medical records and central line database of 960 central line insertions for the complication of skin erosion over the TIVAD. Outcome measures included age, gender, and nutritional status, number of days until complication, insertion site, and attending surgeon. A total of 540 of the 960 central lines were TIVAD. Skin erosion occurred in 9 patients for an incidence of 1.67%. Average age at insertion was 51 months (range 25-116.5 months). The average catheter duration use in days was 335 with a range of 39-1575 days. Malnutrition defined as BMI5% or a decrease in BMI percentiles occurred in 2 and 4 patients, respectively, and contributed to the thinning of the subcutaneous fat. Skin erosion over TIVAD is a rare complication. Most cases can be prevented by inserting the device in a subfacial location in the very young child or in the child with expected weight loss. Furthermore, the device should be placed at a fair distance from the skin incision to prevent early skin erosion through the wound.

Purpose The aim of this study is to determine if there has been a true, absolute, or apparent relative increase in congenital diaphragmatic hernia (CDH) survival for the last 2 decades. Method All neonatal Bochdalek CDH patients admitted to an Ontario pediatric surgical hospital during the period when significant improvements in CDH survival was reported (from January 1, 1992, to December 31, 1999) were analyzed. Patient characteristics were assessed for CDH population homogeneity and differences between institutional and vital statistics-based population survival outcomes. SAS 9.1 (SAS Institute, Cary, NC) was used for analysis. Result Of 198 cohorts, demographic parameters including birth weight, gestational age, Apgar scores, sex, and associated congenital anomalies did not change significantly. Preoperative survival was 149 (75.2%) of 198, whereas postoperative survival was 133 (89.3%) of 149, and overall institutional survival was 133 (67.2%) of 198. Comparison of institution and population-based mortality (n = 65 vs 96) during the period yielded 32% of CDH deaths unaccounted for by institutions. Yearly analysis of hidden mortality consistently showed a significantly lower mortality in institution-based reporting than population. Conclusion A hidden mortality exists for institutionally reported CDH survival rates. Careful interpretation of research findings and more comprehensive population-based tools are needed for reliable counseling and evaluation of current and future treatments.

Technically expedient repair of CDH defects is desirable. With increasing trend toward thoracoscopic repair, herein we examine physiologic predictors for the need for patch closure (PC) versus primary closure. All neonates who underwent surgical repair of CDH defects in a geographically defined region between 1992 and 2002 were included (n = 210). Two groups of patients were compared, primary repair (PR) versus PC. The 25th quartile was used as a cut off point for continuous variables. Univariate and multivariate logistic regression were performed. One hundred and fifty neonates underwent open PR (71.43%) versus 28.57% had PC. On univariate analyses the following variables were significantly associated with the need for PC: prenatal diagnosis, birth weight2.7 kg, gestational age37 weeks, APGAR at 5 min6, immediate postnatal PCO(2)34, Immediate oxygen saturation93%, use of Nitric oxide and the need for high frequency oscillation (HFO). On multivariate analyses, only a PCO(2)34 and the need for HFO were significantly associated with PC. Neonates with an initial PCO(2)34 or need HFO pre-operatively should be excluded from attempts to repair the CDH thoracoscopically based on their higher potential need for PC with its entailed technical difficulty and increased operative time.

Side-to-side, functional end-to-end stapled anastomosis (SS-EESA) is a frequently employed technique to re-establish continuity following bowel resection. We describe, for the first time in children, two cases of an important complication of this form of bowel anastomosis. Patient 1 had resection of a jejunal lymphangioma and formation of an SS-EESA at the age of 3 years. By the age of 7 years he was demonstrating symptoms consistent with malabsorption, which was confirmed by hydrogen breath testing. An upper GI contrast study indicated a segmental dilatation of the distal small bowel. Elective laparotomy revealed partial volvulus of a greatly dilated SS-EESA. Patient 2 had undergone bowel resection as a neonate for ileal atresia, with end-to-end anastomosis. An anastomotic stricture developed at two months of age that was resected with formation of an SS-EESA. Multiple ensuing episodes of partial small bowel obstruction were managed non-operatively until, at 5 years of age, she presented with complete bowel obstruction. At operation, volvulus of a hugely dilated SS-EESA was found. Intraoperative cultures of the succus entericus were consistent with bacterial overgrowth. Both patients were successfully treated with resection of the SS-EESA and primary anastomosis. SS-EESA can be complicated by bacterial overgrowth, massive dilatation and volvulus. In patients with SS-EESA who present with recurrent obstructive symptoms, this complication should be considered.

A retrospective chart review of 32 patients who underwent laparoscopic interval appendectomy at the Children's Hospital of Eastern Ontario between May 1999 and December 2003 was performed. The age range was 0.5 to 18 years (mean, 11.8 years; median, 11.5 years). The interval time from the acute episode to the laparoscopic interval appendectomy ranged from 1 to 16 weeks (median, 6 weeks). The initial presentations were 11 patients with appendiceal abscess, 9 with appendiceal masses/phlegmons, and 12 patients with an acute but resolving clinical picture with ultrasonographic evidence of appendicitis. There were no wound infections or recurrent intra-abdominal abscesses. The average length of stay was 1.38 days, ranging from same-day discharge (1 patient) to a three-night stay (2 patients). There were no complications related to the laparoscopic technique, confirming reports that laparoscopic interval appendectomy is a technically safe procedure. Pathologic analysis of the appendices demonstrated acute or subacute changes in 14 patients (interval time = 7.9 weeks), chronic changes in 8 (interval time = 8.1 weeks), both acute and chronic changes in 5 (interval time = 8.2 weeks), and no pathologic diagnosis in 6 (interval time = 4.28 weeks). These findings support the need for interval appendectomy, and suggest that laparoscopy is a safe alternative to open appendectomy.

A 16-year-old girl presented with hematochezia and abdominal pain. The patient developed a small bowel obstruction that required a laparotomy, which showed a segment of grossly infarcted small bowel with 3 perforations, localized abscesses, and creeping fat. Resection and an end-to-end anastomosis were performed. Pathologic examination showed underlying mesenteric venous thrombosis with no evidence of Crohn's disease. Hematology workup showed no evidence of a congenitally inherent hypercoagulable state. The use of oral contraceptives was the only predisposing factor identified.

Wilms' Tumor is the most common malignant neoplasm of the urinary tract in children. Since 1969, the National Wilms' Tumor Study Group (NWTSG) has contributed to improving the clinical management and outcome of children affected by Wilms' Tumor. We have managed our patients according to NWTSG protocols and report our results herein.Retrospective chart review of consecutive patients presenting at the Children's Hospital of Eastern Ontario (CHEO) with a diagnosis of Wilms' Tumor between April 1990 and March 2001.Forty patients with Wilms' Tumor (18 M/22 F) were diagnosed at CHEO during this interval. Mean age at diagnosis was 28.5 months. The most common presenting feature was a palpable abdominal mass in 85%. An overgrowth syndrome was noted in 10%. Metastatic disease was present at diagnosis in 20%, with the lungs (75%) the most common site of involvement. Distribution of clinical stage: I = 40%, II = 20%, III = 20%, IV = 15%, V = 5%. Favorable histology (FH) was diagnosed in 82.5%, anaplasia in 12.5% and clear cell sarcoma (CCSK) in 5%. Nephrogenic rests were present in 43% (perilobar (PL) = 20%, intralobar (IL) = 18%, PL + IL = 5%). Recurrence of disease occurred in 10%, with lungs (100%) the most frequent site of relapse. Survival was significantly greater in stage I disease and in those patients with FH. Patient 4-year survival data: stage I (FH), 100%; stage II (FH), 80%; stage III (FH), 100%, stage IV (FH), 67%; stages I-IV (CCSK), 100%; stage V, 0%. Overall 4-year survival rates of patients with FH were 92% and of all patients in the study was 86%.The presentation of Wilms' Tumor at our institution mirrors that described in the literature. Importantly, by following NWTSG protocols we have achieved an outcome of overall 4-year patient survival comparable to the "gold standard". This demonstrates the utility of the NWTSG protocols as regards patient management of this relatively uncommon disease.

The most difficult aspect of the surgical treatment of chest wall tumors is reconstruction of the large residual defect. Materials that have been used include Marlex, Goretex, Vicryl, bone, metal, and fascia. The authors' successful experience with dehydrated human dura (Tutoplast) for moderate-size defects is described. A large Askin's tumor in a 13-year-old boy required resection of the right posterior aspect of the 9th to 11th ribs and the transverse process of T-10. The 12− × 12-cm thoracic defect was closed with dura. Partial soft-tissue coverage was obtained with the latissimus dorsi muscle. Although a scoliosis secondary to paraspinal muscle resection has developed, the chest wall is stable, without evidence of a flail chest, at 18 months of follow-up. A 6-year-old girl underwent left anterior chest wall resection of three ribs for an epithelioid sarcoma. Human dura and a myocutaneous flap were used for reconstruction, with good functional and cosmetic results at 16 months of follow-up. Dura is simple to use, has low antigenicity, and in experimental studies appears to be incorporated into the tissues, acting as a collagen matrix. For moderate-size chest wall defects, it appears to be an excellent alternative to synthetic prosthesis.

A three-month-old infant presented to the emergency department with a 24 h history of irritability and lesion prolapsing intermittently per anus. The child had a six-week history of blood and mucus per rectum. Digital rectal examination and sigmoidoscopy revealed what appeared to be a large mobile polypoid lesion arising from the rectum. Plans for excision were made. Sigmoidoscopy performed the following morning failed to demonstrate any lesion in the rectum or sigmoid. Barium enema demonstrated a sessile ‘apple core type’ lesion in the descending colon. Segmental resection of the descending colon with end-to-end anastomosis was performed. Pathological examination of the resected bowel demonstrated features consistent with an inflammatory fibroid polyp. This case appears to be unique because of the young age of the patient, its unusual location and its presentation as an intermittent colocolic intussusception.

Laparoscopic-assisted stoma creation is becoming a more popular procedure in the adult surgical population. Our institution has previously reported the use of the laparoscopic assisted colostomy (LAC) in children. In this paper, we describe an important complication in a 4-year-old patient, with trisomy 21, who underwent a laparoscopic-assisted loop ileostomy (LAI) and, subsequently, developed a volvulus of the cecum around the loop ileostomy. We suggest that when LAI is performed, cecal mobility should be carefully evaluated. In the absence of adequate cecal peritoneal attachments, an additional procedure to fix the cecum should be considered to prevent a potential volvulus around the stoma.

A left upper quadrant fetal abdominal mass was detected at 24 weeks gestation. The mass was again confirmed in a postnatal ultrasound. Pathological analysis of the excised mass demonstrated an intraabdominal lung sequestration with Stocker type II congenital cystic adenomatoid malformation (CCAM). The sonographic characteristic of these lesions are those of a homogeneous echogenic mass with variable shape passing through or arising from the diaphragm. Surgical excision is recommended because of the uncertainty of the preoperative diagnosis and the possibility of malignant changes in CCAM.

The purpose of this study was to compare the effect of local anaesthesia (LA) with that of caudal anaesthesia (CA) on postoperative care of children undergoing inguinal hernia repair. This was a randomized, single-blind investigation of 202 children aged 1-13 yr. Anaesthesia was induced with N2O/O2 and halothane or propofol and maintained with N2O/O2/halothane. Local anaesthesia included ilioinguinal and iliohypogastric nerve block plus subcutaneous injection by the surgeon of up to 0.3 ml.kg-1 bupivacaine 0.25% with 5 micrograms.kg-1 adrenaline. The dose for caudal anaesthesia was 1 ml.kg-1 up to 20 ml bupivacaine 0.2% with 5 micrograms.kg-1 adrenaline. Postoperative pain was assessed with mCHEOPS in the anaesthesia recovery room, with postoperative usage of opioid and acetaminophen in the hospital, and with parental assessment of pain with a VAS. Vomiting, time to first ambulation and first urination were recorded. The postoperative pain scores and opioid usage were similar; however, the LA-group required more acetaminophen in the Day Care Surgical Unit. The incidence of vomiting and the times to first ambulation and first urination were similar. The LA-patients had a shorter recovery room stay (40 +/- 9 vs 45 +/- 15 min, P < 0.02). The postoperative stay was prolonged in the CA group (176 +/- 32 vs 165 +/- 26 min, P = 0.02). We conclude that LA and CA have similar effects on postoperative care with only slight differences.

Although multifactorial in etiology, prematurity and feeding are two of the most common risk factors associated with necrotizing enterocolitis (NEC). To understand the pathogenesis of NEC, the complex interaction between intestinal contents and clearing mechanisms in the immature human gut must be elucidated. Nitric oxide (NO) is a proposed mediator of nonadrenergic noncholinergic neural inhibition, causing relaxation in the gut. In addition to its role as a neuroeffector substance, studies suggest that endogenous formation of NO maintains intestinal mucosal integrity, protecting the gut from blood-borne toxins and tissue-destructive mediators. Thus, NO has a dual role in both gut smooth muscle relaxation and mucosal protection. Because two of the primary risk factors in the development of NEC are prematurity (as it relates to gut dysmotility) and enteral feeding (as it relates to mucosal damage by intraluminal substrate), the authors chose to investigate the role of NO in the pathogenesis of NEC induced by intraluminal injection of acidified casein solution in neonatal piglets.Having confirmed the consistent induction of NEC both macroscopically and histologically with this model (n = 32), the following were undertaken. Neonatal piglets (3 days old) were laparotomized, and intestinal loops were created from the terminal ileum to the proximal colon. The loops were injected with acidified casein solution and separated by saline-injected control loops. When the abdomen was closed, a continuous peripheral intravenous infusion of L-arginine, an NO synthase substrate (600 mg/kg/h [n = 6]), or N-omega-nitro-L-arginine methyl ester (L-NAME), an NO synthase inhibitor (20 mg/kg/h [n = 6]), was begun. Gut segments were harvested 3 hours later and processed for evaluation of the extent of necrosis.Macroscopically, the L-NAME-treated group showed areas of hemorrhagic necrosis in the NEC-induced loops. The L-arginine-treated group had greatly diminished or virtually absent lesions. HE-stained sections were graded microscopically, using a scale from 0 to 4, ranging from intact villi (grade 0) to transmural necrosis (grade 4). In the untreated NEC group, intestinal damage in the acidified casein loops was exhibited by areas of necrosis (extending, in some cases, transmurally), submucosal edema, and inflammatory cell infiltrate (average grade, 3.5). In the L-NAME-treated group, the intestinal damage was similar to that of the NEC-induced group (average grade, 3.5), but also presented with marked hemorrhagic congestion. In the L-arginine group, NEC-induced tissue damage was greatly attenuated, with necrosis limited primarily to the villus tips (average grade, 1). Nevertheless, inflammatory cell infiltrate and mild submucosal edema were still present.Continuous intravenous infusion with the NO synthase substrate L-arginine markedly attenuates intestinal injury in this neonatal piglet model of NEC. Intravenous administration of the NO synthase inhibitor L-NAME causes hemorrhagic congestion of the gut wall. Based on these findings, the authors propose that treatment with the amino acid L-arginine should be considered as a potential therapeutic modality for NEC.

Deformities of the chest wall, breast, shoulder girdle, and spine are well-documented sequelae of major thoracotomies that transect muscies, divide major motor nerves, resect ribs, or cause them to fuse. These deformities are probably aggravated by the growth process. This is why we make a plea for the routine use, in infants and children, of a muscle-sparing thoracotomy that will minimize these sequelae without sacrificing exposure. Major (lateral) thoracotomy by this technique involves these steps: (1) creating a transverse incision below the tip of the scapula, or a vertical axillary incision; (2) elevating the skin flaps to expose the muscles; (3) retracting the latissimus dorsi posteriorly; (4) retracting the serratus anterior and scapula superiorly; (5) disinserting the lower origins of the serratus if required only; (6) opening the desired intercostal space; (7) reapproximating the ribs without crowding, using a pericostal suture that is passed along the inferior rib subperiosteally, to avoid any compression on the neurovascular bundle; and (8) allowing the muscles to fall back into place, reattaching the serratus insertions as indicated. Lessened postoperative pain and improved respiratory function are additional benefits.

A case of radial artery hypoplasia in a girl with the VATER syndrome is reported. This finding should alert surgeons dealing with vascular access for dialysis and suggests the possibility of intrauterine ischemia in the etiology of upper limb anomalies in these patients.

The outcome of malignant sacro-coccygeal teratomas (SCT) is well described in the literature, but there are no reports on long-term follow-up of patients with benign SCT. The present series is a retrospective study of 49 cases of SCT treated in our institution during the past 27 years. Ten patients died; 6 deaths were related to malignant SCT. One child died of medulloblastoma at 2 years of age, 1 of intraoperative blood loss, 1 of preoperative bleeding, and 1 died prenatally. One patient is still under chemotherapy 2.5 years after resection of a yolk-sac tumour. Of the remaining 38 patients, 13 could not be reached and 25 were available for follow-up (66%). The mean follow-up was 10.1 years (1–27 years). Despite the extreme disturbances of the pelvic musculature, as regularly noticed intraoperatively, all patients available for follow-up were continent for stools and urine. Adequate assessment of rectal evaluation was possible in 21 patients: 10 of the 21 (47.6%) had a significant degree of constipation and 5 of them also had documented episodes of urinary tract infections. In this particular group the mean follow-up was 9.5 years (1–24 years). The absence of incontinence in these patients suggests either the return to normal function of the peri-rectal muscular components or, more probably, a more important role of the external sphincter in the mecaanism of continence. The increased frequency of significant constipation in this group of patients has not been previously emphasized, and suggests the need for longer follow-up.

This is the case report of a 4-year-old white boy who was diagnosed as having acute lymphoblastic leukemia (ALL) in November 1985. While in remission and on maintenance chemotherapy, he developed a primary Epstein-Barr virus (EBV) respiratory infection in October 1986. On October 27, 1986 a plain abdominal radiograph taken for abdominal distention showed free air. At celiotomy, multiple nodules were noted to stud the small bowel. Central necrosis of these nodules with perforations were present in the distal small bowel. Resections and end-to-end anastomoses were performed. Three days later the patient again had a similar acute abdominal episode. At reexploration, similar lesions in the liver, kidney, duodenum, proximal jejunum, and colon were found. Liver biopsy as well as intestinal resections and end-to-end anastomoses were performed, along with a loop ileostomy. Polymorphic B-cell lymphoma positive for EBV was found in the specimens. After cessation of chemotherapy and institution of abdominal radiotherapy, the hepatic and renal lesions were seen to resolve on computed tomography scan. The patient's course was complicated by the development of cervical and mediastinal abscesses that were drained, and E coli sepsis accompanied by chronic diarrhea requiring intravenous hyperalimentation. By January 1988, he appeared to be recovering. His ileostomy was closed in March 1988. Despite cessation of chemotherapy since October 1986, the patient is now well and in complete remission.

We reviewed empyema management at our center since video-assisted thoracoscopic surgery (VATS) was introduced. Between 1991 and 2003, 58 patients (34 male, 24 female) ages 1 day to 17 years (median, 6 years) were identified. The median interval from the onset of symptoms to presentation was 7 days; 31% of patients received inpatient care at another hospital, and 24% were from northern communities, including the Arctic. Symptoms included fever (100%), cough (66%), shortness of breath (46%), chest pain (38%), and abdominal pain (17%). Most patients (96%) had pneumonia. The most common organisms were Streptococcus pneumoniae (22%), group A streptococcus (8%), and Mycobacterium tuberculosis (3%). No organisms were identified in 31% of patients. One empyema was trauma-related and one was due to complications of esophageal stricturoplasty. All patients received intravenous antibiotics (median, 17 days). Three patients had thoracentesis, 17 had chest tubes, 3 had thoracentesis and chest tubes, 26 had VATS, 6 had thoracotomy, and 3 had minithoracotomy. Median operative times were 86 minutes for VATS, 90 minutes for thoracotomy, and 75 minutes for minithoracotomy. Median total length of stay (LOS) was 15 days for VATS, 20.5 days for thoracotomy, and 21 days for minithoracotomy. The median preoperative LOS was longer for thoracotomy (10 days) than for VATS (5 days) or minithoracotomy (1 day). The median postoperative LOS was 14 days for VATS, 9.5 days for thoracotomy, and 8 days for minithoracotomy. Our experience shows that VATS is a safe and effective approach to managing children with empyema. The shorter preoperative LOS for VATS is likely due to earlier surgical referral and intervention using less invasive surgical procedures.

During the past 20 years, 23 patients (7 males, 16 females) were operated on for thyroid carcinoma in our institution. The average age was 13.6 years (range, 22 months to 27 years). Our series includes papillary carcinoma in 11, follicular carcinoma in four, and medullary thyroid carcinoma in eight patients. Follow-up ranged from 8 months to 20.3 years, with an average of 7.5 years for well-differentiated carcinomas and 4.3 years for medullary thyroid carcinomas. All patients are presently alive with no evidence of progressive disease. Patients with papillary and follicular carcinomas underwent partial thyroidectomy; those with medullary carcinoma underwent total thyroidectomy. Serious complications included three permanent hypoparathyroidism and two tracheostomies, all after secondary neck explorations. The overall results observed in our series of patients seem to support the current conservative approach to well-differentiated thyroid carcinoma, reserving total thyroidectomy for medullary cancer of the thyroid. A more aggressive search for familial medullary carcinoma through use of pentagastrin stimulation leads to early detection and more effective therapy.

During the past 10 years, 26 cases of blunt pancreatic trauma were diagnosed in our institution. In 42.3% (11/26) the accident was bicycle-related. Seventy-three percent of patients were seen within 48 hours of injury. The most frequent clinical presentations included abdominal pain, tenderness and vomiting. Diagnosis of pancreatic injury was suggested by hyperamylasemia in most cases. Associated trauma was seen in seven patients (26.9%) and it was intraabdominal in four (15.3%). Computerized tomography (CT) scan is the single most useful radiologic investigation in evaluating pancreatic trauma. Ultrasound, although less accurate than CT scan in determining the severity of the initial injury, is useful in the evaluation and treatment of pancreatic pseudocysts. Pancreatic pseudocysts developed in ten patients. Spontaneous resolution occurred in five (50%). In three patients, percutaneous external drainage (PED) was successful in treating pancreatic pseudocysts without complications or recurrence at 11, 19, and 31 months. PED is a suitable form of treatment in selected cases of pancreatic pseudocysts. Results in children are better than in the adult population, probably due to the absence of primary pancreatic pathology. We believe that PED should be considered the primary therapeutic procedure for traumatic pancreatic collections prior to more invasive surgical treatment, when there is no evidence of pancreatic duct transection on CT scan.

Poor postoperative continence in patients operated for high imperforate anus may result from an inadequate bowel pult-through behind the puborectal muscle. Between 1979 and 1983, we used the anterior perineal approach for reoperations in five such cases (3 males, 2 females). In all of them, preoperative continence was socially unacceptable. Each time it was possible to identify a partially or totally missed puborectal muscle. Although some complications occurred postoperatively, all patients acquired a socially acceptable level of continence with a mean follow-up of 4.5 years. Preoperative and postoperative rectal manometries are presented. The anterior perineal approach not only provides adequate exposure of the urethra and puborectal muscle, but also has the advantage of operating through an area that has not been previously dissected.