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Background: Biliary atresia (BA) is typically treated by Kasai portoenterostomy (KPE), and there is a relationship between age at surgery and outcome. We hypothesize that previous abdominal surgery (PAS) for associated congenital intestinal conditions could be used to identify BA earlier, perhaps improving prognosis., Methods: A retrospective case note review was performed of all BA patients at a single centre from 1999 to 2016. Demographics and clinical outcome data were collected. Additional data on laparotomy, parenteral nutrition, and referral were collected from patients who underwent PAS. Data are median (range)., Main Results: Two-hundred-and-fifty-seven children were reviewed. Of these, 16 (6.2%) underwent PAS on day 3 (0-23), during which 5 atretic gallbladders were noted. Gallbladder appearance was not referenced in the operation notes of 8 infants. Jaundice and acholic stools were noted at 4 (0-56) days and 21 (0-60) days, respectively. Age at KPE was comparable between PAS and the other patients (50 vs. 51days; P=0.78), but native liver survival was significantly lower after PAS (p<0.0001). Mortality rate was higher in PAS patients (25% vs. 4.5%; P=0.0007). Survival was unaffected by early referral of patients on finding an atretic gallbladder at surgery., Conclusion: About 6% of infants have already undergone abdominal surgery for biliary atresia associated intestinal anomalies. Routine gallbladder examination at time of laparotomy could have aided earlier diagnosis and treatment of biliary atresia in up to 80% of patients in this cohort. However, our data suggest that clinical outcome is poorer in biliary patients who undergo prior abdominal surgery and is not improved by earlier referral., Level of Evidence: Prognostic study: Level III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Congenital diaphragmatic hernia (CDH) is a lethal birth defect, which occurs in 1:2000-3000 live births. Bilateral CDH is a rare form (1%), with a high mortality. This study presents the outcomes of the largest cohort of bilateral CDH patients., Methods: The records of patients with bilateral CDH from the Congenital Diaphragmatic Hernia Registry born between 1995 and 2015 were retrospectively analyzed to identify parameters associated with mortality., Results: Eighty patients with a bilateral CDH were identified. Overall mortality was 74% (n=59). Apgar scores at 1 and 5min were statistically lower in the non-survivors compared to the survivors (median 3.0 and 5.0, versus 6.5 and 8.0, respectively, p<0.001). All survivors were repaired (n=21), compared to 22% of the non-survivors (n=17). The type of repair was equally divided in the survivors (52% primary versus 48% patch), while non-survivors were mainly patch repaired (82% versus 12%). Nineteen were treated with extracorporeal membrane oxygenation (ECMO) (24%), only three of them survived. When calculating the risk on mortality for the patients who lived until repair, ECMO had an adjusted odds ratio for mortality of 10.8 (95% CI: 2.0-57.7) and patch repair 5.2 (95% CI: 0.8-34.9)., Conclusions: The treatment of bilateral CDH patients remains challenging with a high mortality rate. Lower Apgar-scores, ECMO (probably as a surrogate for the severity of disease), and patch repair were negatively associated with outcome., Level of Evidence: Level IV study., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Introduction: Megacystis microcolon intestinal hypoperistalsis (MMIHS) is a rare disorder characterized by distended nonobstructed bladder, microcolon, and decreased intestinal peristalsis. MMIHS has a particularly poor prognosis; however, when appropriately managed, survival can be prolonged., Study Design: A systematic review (1996-2016) was performed with the key words "megacystis microcolon intestinal hypoperistalsis syndrome." In addition, a case series of four patients is presented as well as algorithms for the diagnosis and treatment of MMIHS., Results: 135 patients with MMIHS were identified in the literature. 73% (88/121) of the patients were female, 65% underwent diagnostic biopsy (64/99), and 63% (66/106) were identified with prenatal imaging. The majority of patients were treated with TPN as well as gastrostomy or ileostomy and CIC, however 15% (18/116) received multivisceral or intestinal transplant, and 30% (22/73) had a vesicostomy. The survival rate was 57% (68/121)., Conclusion: Appropriate management of MMIHS patients is crucial. An enlarged, acontractile bladder in a child with bowel motility problems should be considered diagnostic. Bladder distension can be managed with CIC or vesicostomy in addition to prophylactic antibiotics if frequent urinary tract infections are present. These patients often require gastrostomy or ileostomy as well as total parenteral nutrition. This management has led to significant improvement in survival rates., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aim of Study: We examined variables associated with survival for oesophageal atresia between 1996 and 2014., Methods: Possible explanatory variables: birth weight, gestation, cardiac anomalies (any or major), renal anomalies (any or severe), primary anastomosis, leak, secondary oesophageal surgery, tracheomalacia, aortopexy, tracheostomy, gastrostomy, fundoplication, karyotype, neurological status. Variables were assessed with logistic regression and a new model assessed with Kaplan-Meier graphs., Results: 104/120 (87%) babies survived. Median gestation 37weeks, 4 (3%) born before 28weeks. Mean birth weight 2.3 (SD 0.7) kg, 17 (14%) less than 1500g. Frequency (%) of explanatory variables: Major cardiac anomaly 21 (18%), any cardiac anomaly 48 (40%), severe renal anomaly 10 (8%), any renal anomaly 25 (21%), primary anastomosis 105 (88%), anastomotic leak 16 (13%), symptomatic tracheomalacia 28 (23%), aortopexy 17 (14%), tracheostomy 12 (10%), neurological anomaly 7 (6%), fundoplication 15 (13%), gastrostomy 30 (25%), secondary oesophageal surgery 8 (7%), abnormal karyotype 6 (5%). Multivariate analysis showed only renal (OR 0.04, 0.007 0.2) p=0.001, cardiac (OR 0.1, 0.002 0.6) p=0.01 and a primary anastomosis (OR 12.2, 1.8 81.6) p=0.01 (R2=0.48), or major cardiac (OR 0.04, 0.007 0.29) p=0.001 and severe renal anomalies (OR 0.009, 0.001 0.12) p<0.001 alone were significant (R2=0.57)., Conclusions: Survival is dependent on cardiac and renal anomalies. Birth weight is not significant. We propose a new classification system: 1: neither severe renal nor major cardiac anomaly, 2: either severe renal or major cardiac anomaly, 3: severe renal and major cardiac anomaly., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan., Methods: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan., Results: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n=11) and colon (n=5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively., Conclusions: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Gastroschisis may be complicated by intestinal atresia, necrosis, and/or perforation. In the absence of an urgent indication, intestinal procedures are often delayed to allow for bowel recovery. This practice has not been evaluated., Methods: We queried a prospective Canadian database of all patients with gastroschisis born between 2005 and 2011. Patients with intestinal atresia who underwent an intestinal operation during the first 21 days of life (EARLY GROUP) were compared with those who underwent operations later (LATE GROUP)., Results: Of 629 gastroschisis patients, 78 (12.4%) had intestinal complications; 27 patients (4.3%) had intestinal operations for atresia without necrosis or perforation - 14 EARLY and 13 LATE. Baseline clinical parameters were similar between the two groups. There was a decreased incidence of the following complications in the EARLY group but none reached statistical significance: post-operative bowel obstruction (28.6% vs. 61.5%, p = 0.1); line sepsis (14.3% vs. 30.8 %, p = 0.4); and wound infection (14.3% vs. 46.1%, p = 0.1). Earlier tolerance of enteral feeding in the EARLY group was manifested by younger age at first enteral feeding (14.8 + 2.6 vs. 44.7 + 7.4 days, p = 0.002) and higher tolerance of enteral feeding at 28 days of life [less patients exclusively on TPN (28.6% vs. 61.5%, p = 0.06), and more patients on more than 50 cc kg(-1)day(-1) of enteral feeding (42.9% vs. 7.7%, p = 0.08)]., Conclusions: Early intestinal operations in patients with gastroschisis and intestinal atresia are not associated with increased complications, and allow patients to receive and tolerate enteral feeding earlier., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH., Methods: All patients recorded in the CDH Study Group registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A-D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac., Results: A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, (p<0.0001; p for trend <0.0001). GA, BW, prevalence of associated anomalies, particularly CVM, number of abnormal organ systems, and prevalence of sac were significantly different between the groups, with a significant reduction of GA, BW, and prevalence of sac. There was an increase in prevalence of associated anomalies, liver in the chest, and number of abnormal systems as the defect size increased., Conclusion: Defect size is directly correlated with mortality rate, prevalence of other anomalies (particularly CVM), and number of abnormal systems, and inversely with GA, BW, and prevalence of hernia sac. The defect size may be a marker for the magnitude of developmental abnormality, thereby explaining its relationship with the outcome., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: A major determinant of survival in patients with congenital diaphragmatic hernia (CDH) is severity of pulmonary hypoplasia. This study addresses the comparative effectiveness of prenatal methods of lung assessment in predicting mortality, extracorporeal membrane oxygenation (ECMO), and ventilator dependency., Methods: We retrospectively reviewed all patients born with isolated CDH between 2004 and 2008. Lung-to-head ratio (LHR) and observed-to-expected LHR (OELHR) were obtained from prenatal ultrasounds. Percent-predicted lung volume (PPLV) was obtained from fetal MRI (fMRI). Postnatal data included in-hospital mortality, need for ECMO, and ventilator dependency at day-of-life 30., Results: Thirty-seven patients underwent 81 prenatal ultrasounds, while 26 of this sub-cohort underwent fMRI. Gestational age during imaging study was associated with LHR (p=0.02), but not OELHR (p=0.12) or PPLV (p=0.72). PPLV, min-LHR, and min-OELHR were each associated with mortality (p=0.03, p=0.02, p=0.01), ECMO (p<0.01, p<0.01, p=0.03), and ventilator dependency (p<0.01, p<0.01, p=0.02). For each outcome, PPLV was a more discriminative measure, based on Akaike's information criterion. Using longitudinal analysis techniques for patients with multiple ultrasounds, OELHR remained associated with mortality (p=0.04), ECMO (p=0.03), and ventilator dependency (p=0.02), while LHR was associated with ECMO (p=0.01) and ventilator dependency (p=0.02) but not mortality (p=0.06)., Conclusion: When assessing fetuses with CDH, OELHR and PPLV may be most helpful for counseling regarding postnatal outcomes., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: The study aim was to determine outcomes of children with congenital heart disease who underwent laparoscopic procedures., Methods: A single-institution, institutional review board-approved, retrospective review was conducted including children younger than 5 years with congenital heart disease who underwent laparoscopic or open abdominal procedures. Patient demographics, operative details, complications, and 30-day mortality were examined., Results: Over 10 years, 111 children with congenital heart disease underwent 121 laparoscopic procedures. Median age was 2.5 months, with 87% being infants. Laparoscopic gastrostomy was the most common procedure (101). There was no intraoperative hemodynamic instability, median operative time was 70 minutes, postoperative complications were low (5%), and all children were alive at 30 days. Only 8 patients required conversion from laparoscopic to open, all secondary to technical issues, not hemodynamic instability. There were 42 children with cardiac disease who underwent 45 open procedures during the study period. There were no significant differences between patient demographics, type of procedure, operative time, complications, or 30-day mortality comparing the open and laparoscopic groups., Conclusion: In this review, there were no major contraindications to performing laparoscopic procedures in children with congenital heart disease, and we conclude that it is reasonably safe to perform laparoscopic surgery on these children., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Little is known about associated anomalies in fetuses with gastroschisis (GS) who experience an "atypical perinatal event," defined as spontaneous abortion, stillbirth, termination, or death within 24 hours of birth., Purpose: This study aims to compare associated malformation rates in an atypical perinatal event cohort vs newborns with GS surviving longer than 24 hours., Methods: A national prospective GS database was analyzed for cases with an atypical perinatal event. Associated anomaly rates were compared between this cohort and babies surviving longer than 24 hours., Results: Twenty-three atypical perinatal events (2 spontaneous abortions, 7 stillbirths, 11 terminations, and 3 deaths within 24 hours) were identified from 529 total GS cases. Autopsies in 14 (61%) of 23 identified at least 1 anomaly (excluding intestinal, patent ductus arteriosus, and undescended testicle) in 11 (78.6%) and a "lethal" anomaly in 4 (36%). The associated anomaly rate in newborns surviving longer than 24 hours was 7.3% (37/506; P < .0001). The anomalies in the atypical perinatal event cohort were musculoskeletal (35%), cardiac, central nervous system, pulmonary, and genitourinary (12% each). Among survivors, the most common anomalies were cardiac (38%), genitourinary (32%), musculoskeletal (16%), and central nervous system (8%)., Conclusion: Rates of associated anomalies are significantly higher in fetuses experiencing atypical perinatal events and may represent the "hidden mortality" of GS., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Sanjad-Sakati syndrome is a rare autosomal recessive disorder mainly occurring in the Arab Peninsula. This condition is associated with metabolic and septic complications starting in the neonatal period. Chronic intestinal pseudoobstruction owing to visceral myopathy is a rare disabling condition. We report a rare concurrence of Sanjad-Sakati syndrome and chronic intestinal pseudoobstruction in a Saudi child complicated by intestinal failure, sepsis, and early mortality., (Copyright 2010 Elsevier Inc. All rights reserved.)

Background: Gastroschisis is a rare congenital anomaly, the improved surgical management of which has contributed to a survival rate greater than 90%. Development of an accurate risk stratification system to help identify the subset of patients at greatest risk for death may lead to further improvements in outcome., Methods: Infants with gastroschisis were identified from 16 years of the National Inpatient Sample database and the Kids' Inpatient Database using the International Classification of Diseases, Ninth Revision, Clinical Modification procedure code 54.71 (repair of gastroschisis) and an age of less than 8 days. Logistic regression analysis determined which coexisting diagnoses were significantly associated with death. Odds ratios from the logistic regression model were simplified and used as weighting factors to create an additive index. The index was validated using the 2003 Kids' Inpatient Database data set., Results: Intestinal atresia, necrotizing enterocolitis, rare cardiac anomalies, and lung hypoplasia were strongly associated with death and used to create a scoring system with a potential range of 0 to 10. Every point increase on the scale of gastroschisis risk stratification index is associated with a 95% relative increase in the likelihood of death., Conclusion: We have developed a novel index, which is superior to previous classification systems in identifying patients with gastroschisis who are at highest risk for death.

Background: Children with multisystem involvement including congenital heart defect (CHD) are a very salient problem. The purpose of this study was to evaluate the incidence of CHD associated with malformations of other systems and to assess the modalities of treatment and perioperative mortality among patients referred to the department of pediatric cardiac surgery., Methods: The medical records of 1856 children were reviewed retrospectively from 1997 to 2002 to establish CHD and types of associated malformations. The connections between CHD and other lesions were investigated. Furthermore, the influence of patient and perioperative variables on mortality risk was scrutinized. Univariate and multivariate analyses were used., Results: Eighty-four children (4.53%) had CHD and associated malformations. The malformations of digestive (35.7%), urinary (22.4%), and nervous (14.3%) systems were the most frequently observed associated defects. No relation was found between CHD and concomitant lesions. The results of multivariate logistic regression showed significant influence of patient age, primary cardiac procedure, and CHD type on mortality (ca 19%) in children with multiorgan lesions., Conclusions: The treatment of children with CHD and associated multiple lesions is connected with higher mortality risk. The following factors: younger age, urgency of surgical procedure, and primary surgical procedure had negative impact on patient's outcome. However, these risks in certain cases are inevitable. The cardiac procedure preceding the surgical operation may improve the overall effect of treatment because of circulatory stabilization, provided that the condition of the patient does not preclude any intervention at all.

Background/purpose: The aim of this study was to analyze the outcome of giant omphalocele repaired in the neonatal period., Methods: Twelve consecutive (1997-2004) neonates with giant omphalocele (defect >6 cm with liver herniation) were reviewed. A silo of Prolene mesh (Ethicon) was attached to the fascia and the defect was closed without opening the amniotic sac after sequential reduction. In 2 neonates with ruptured omphalocele a plastic sheet was inserted below the mesh. Data are reported as median and range., Results: Gestational age was 38 weeks (range, 32-40 weeks) and birth weight was 2.9 kg (range, 1.0-3.1 kg). The final closure was achieved at 26 days (range, 16-62 days). Three neonates (25%) died before final closure (causes: ruptured omphalocele, lung hypoplasia, cardiac anomalies, and intestinal failure). In the 9 surviving neonates, mechanical ventilation was required for 8 days (range, 2-20 days), hospital stay was 42 days (range, 23-73 days), and full enteral feeding was achieved on day 12 (range, 4-53 days). Complications included wound infection in 5 neonates and midgut volvulus in 1. Prophylactic Ladd's procedure was performed laparoscopically at a later stage in 4 children. At laparoscopy, intraperitoneal adhesions were minimal and the central liver did not preclude the operation. The 9 survivors are all well after 46 months (range, 12-67 months)., Conclusions: Giant omphalocele can be safely repaired in the neonatal period without opening the amniotic sac. Intestinal malrotation should be excluded and Ladd's procedure can be performed laparoscopically at a later stage.

Purpose: The authors developed a clinical pathway for optimal management after antenatal diagnosis of gastroschisis. This is the outcomes analysis of our first 30 consecutive patients., Method: Antenatal counseling was provided for all families with in-utero diagnosis of gastroschisis. Bowel dilatation, thickness, motility, amniotic fluid volume, and fetal development were followed by ultrasonography every 4 weeks. Babies were delivered by cesarean section between 36 and 38 weeks gestation if the lungs were mature or earlier for bowel complications. Gastroschisis repair was scheduled 90 minutes after birth. Primary repair was attempted in all through the abdominal wall defect without an additional incision, resulting in an umbilicus with no abdominal scar., Results: Primary repair was achieved in 83%. Babies needed assisted ventilation for 3 days, reached full feeds by 19 days, and were discharged by 24 days (all medians). There were 3 (10%) deaths, all after staged repair., Conclusions: Our new protocol of both scheduled elective cesarean section and early gastroschisis repair resulted in a higher proportion of primary repair, shorter duration of mechanical ventilation, earlier full feeds, and shorter length of stay. There was no increase in mortality or morbidity. The primary-repair babies had no mortality and had excellent cosmesis.

Background/purpose: Treatment of long gap esophageal atresia (EA) is still a major challenge. Gastric transposition and colon interposition are the 2 most popular choices for esophageal replacement, but there is general agreement that the child's own esophagus is the best. The aim of the study was to critically evaluate the feasibility and outcome of primary repair of long gap EA with or without tracheoesophageal fistula (TEF) by direct esophago-esophageal anastomosis as the only technique., Methods: Seventy-one neonates with EA+/-TEF were considered. Nineteen cases were classified as long gap (> or =3 cm). All infants underwent either primary or shortly delayed repair. In the latter group, a gastrostomy was performed along with an x-ray evaluation of the gap a few days before surgery (mean age, 46.4 days). To avoid disruptive anastomotic force, all infants were kept paralyzed and mechanically ventilated for an additional 6 days after esophageal anastomosis. Before starting feeding, postoperative esophagogram was done on day 7. Endoscopy was done routinely, starting 1 month after surgery; pH monitoring was conventionally performed at 1 year of age or even earlier, should gastroesophageal reflux disease (GERD) be suspected. Follow-up ranged from 11 months to 7 years., Results: In all 19 long gap EA infants an esophago-esophageal anastomosis was performed. Six of them (31%) required an anterior esophageal flap to bridge residual gap. Complications included minor anastomotic leak in 2 cases and anastomotic stricture (<5mm) in 12 (80%) cases, which were treated with an average of 5 dilatations (1 of which with resection of the stricture). GERD occurred in 8 cases (53.3%), of which, 3 required fundoplication. None of the patients had esophageal swallowing difficulties or persistent dysphagia. Two children experienced food aversion. Mean hospital stay was 66.2 (22 to 230) days. There were 4 deaths (very low birth weight, 1; associated anomalies, 1; and late sepsis, 2)., Conclusions: Considering heat gap determination remains imprecise, it seems possible to conclude that in a well-established tertiary care level referral center: (1) long gap EA could be treated successfully with primary repair and anastomosis; (2) strictures and GER represent the most frequent postoperative problem, but additional procedures required seem "acceptable" to maintain the patient's own esophagus and avoid replacement; (3) esophageal substitution in long gap EA should be reserved for cases in which a previous attempt of esophageal reconstruction failed.

Background: Duodenal atresia and stenosis is a frequent cause of congenital, intestinal obstruction. Current operative techniques and contemporary neonatal critical care result in a 5% morbidity and mortality rate, with late complications not uncommon, but unknown to short-term follow-up., Methods: A retrospective review of patients with duodenal anomalies was performed from 1972 to 2001 at a tertiary, children's hospital to identify late morbidity and mortality., Results: Duodenal atresia or stenosis was identified in 169 patients. Twenty children required additional abdominal operations after their initial repair with average follow-up of 6 years (range, 1 month to 18 years) including fundoplication (13), operation for complicated peptic ulcer disease (4), and adhesiolysis (4). Sixteen children underwent revision of their initial repair: tapering duodenoplasty or duodenal plication (7), conversion of duodenojejunostomy to duodenoduodenostomy (3), redo duodenojejunostomy (3), redo duodenoduodenostomy (2), and conversion of gastrojejunostomy to duodenoduodenostomy (1). There were 10 late deaths (range, 3 months to 14 years) attributable to complex cardiac malformations (5), central nervous system bleeding (1), pneumonia (1), anastomotic leak (1), and multisystem organ failure (2)., Conclusions: Late complications occur in 12% of patients with congenital duodenal anomalies, and the associated late mortality rate is 6%, which is low but not negligible. Follow-up of these patients into adulthood is recommended to identify and address these late occurrences.

Background: This study evaluated the complications of colostomy and its closure in infants and children., Methods: One hundred forty-six colostomies were performed in 86 neonates, 23 infants, and 37 children older than 1 year. These children underwent colostomies for anorectal malformation (84), Hirschsprung's disease (47), and other miscellaneous (15) conditions like colonic atresia, volvulus, rectal tuberculosis, traumatic rectal perforation, and intestinal obstruction caused by ascariasis., Results: Of these, 17 (11.6%) had early complications, and 80 (69.8%) had stomal complications. Three patients died, but only 1 death was directly related to colostomy. Colostomy prolapse, peristomal excoriation, and malnutrition were the major complications. The complications were not dependant on the children's age or primary indication. Sigmoid colostomy had a lower malnutrition rate than transverse colostomy (34.9% v 16.9% P =.009). Among the 56 children who underwent colostomy closure, major complications include death (1.8%), anastomotic leak (7.1%), and wound infection (12.6%)., Conclusions: A divided sigmoid colostomy should be performed whenever possible. Proper stomal care, regular nutritional assessment, and early closure of the colostomy would minimize morbidity and mortality of colostomy and its closure.

Background/purpose: The purpose of this analysis was to investigate outcomes in newborns with esophageal atresia (EA) or tracheoesophageal fistula (TEF) with respect to prognostic classifications and complications., Methods: Charts of all 144 infants with EA/TEF treated at British Columbia Children's Hospital (BCCH) from 1984 to 2000 were reviewed. Patient demographics, frequency of associated anomalies, and details of management and outcomes were examined., Results: Applying the Waterston prognostic classification to our patient population, survival rate was 100% for class A, 100% for class B, and 80% for class C. The Montreal classification survival rate was 92% for class I and 71% for class II (P =.08). Using the Spitz classification, survival rate was 99% for type I, 84% for type II, and 43% for type III (P <.05). The Bremen classification survival rate was 95% "without complications" and 71% "with complications." Complications included stricture (52%), gastroesophageal reflux (31%), anastomotic leakage (8%), recurrent fistula (8%), and pneumonia (6%). Seventeen patients underwent fundoplication for gastroesophageal reflux, 16 pre-1992 and one post-1992., Conclusions: Comparing the major prognostic classifications, the Spitz classification scheme was found to be most applicable. In our institution, the trend in management of gastroesophageal reflux after repair of EA/TEF has moved away from fundoplication toward medical management.

Background/purpose: The literature lacks a concise description of the anorectal anomalies encountered in pygopagus twins., Methods: This is a retrospective literature review of 34 pygopagus twins highlighting their anorectal malformations., Results: Overall pygopagus twins represent 17% of all conjoined twins. Live-birth pygopagus twins are more commonly female (86%), whereas stillborns are commonly male (80%). Half of the twins had nonfused rectums, and half had fused rectums. The nonfused had 2 rectums (80%) or one rectum and one rectovaginal fistula (20%). The fused had high (46%) or low (54%) rectal junctions. All reported living male pygopagus twins have had nonfused rectums. All can be managed applying the principles of posterior sagittal anorectoplasty., Conclusions: In general, live-born male pygopagus twins are likely to survive with a nonfused rectum and good bowel function, whereas live-born female pygopagus twins have a good chance of surviving with a fused rectum (high or low), which requires appropriate operative reconstruction., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Purpose: The aim of this study was to investigate contemporary patterns of presentation and outcome in newborns with esophageal atresia with or without tracheoesophageal fistula (OA) using modern prognostic criteria to appraise survival., Methods: Over a 12-year period (1986 through 1997), 134 patients with OA were admitted to a single institution. Patient demographics, the presence of cardiac defects, other associated abnormalities (VACTERL), surgical intervention, and patient outcome were recorded. To identify and evaluate changes in the pattern of clinical presentation, frequency of associated anomalies and outcome, patients were analyzed during 4 consecutive time periods, 1986 to 1988, 1989 to 1991, 1992 to 1994, and 1995 to 1997., Results: A primary repair or delayed primary repair was performed in 113 (84%) patients, with a staged procedure in 19 (14%). Two babies with trisomy 18 did not undergo surgery. Thirty-eight newborns (28%) had a major cardiac malformation (excluding patent ductus arteriosus, unless needing ligation), and 25 (19%) had recognized VACTERL associations. There was a significant increase in the proportion of infants with major cardiac defects diagnosed over the study period, 5 of 34 patients between 1986 and 1988 to 19 of 41 patients between 1995 and 1997 (chi(2) test, P <.001), but the incidence of VACTERL associations remained unchanged. Overall survival rate was 86% in those who underwent surgery. The relative risk of mortality in patients with major cardiac disease and VACTERL associations was 3.47 (95% CI; 1.51 to 7.96) and 2.54 (95% CI; 1.14 to 4.86), respectively. Birth weight was significantly higher in infants who survived (2.68 kg) compared with those who died (2.16 kg, P =.003). Thirty percent of infants with more than one system abnormality died compared with 8% of infants with 1, system abnormality (P =.004)., Conclusions: This study has found a significant increase in the frequency of cardiac abnormalities encountered in a cohort of OA patients during the period under review. Cardiac disease and multiple abnormalities carried a substantial increased risk of mortality. In the era of the Spitz classification, the phenotypic presentation is important to accurately assess caseload severity and prognosis., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The form and function of the heart are the final result of an integration of cells, tissues, and extracellular material. The extracellular matrix (ECM) is a complex array of different molecular components, and it plays an important role for the transfer of mechanical force in both contraction and relaxation phases in the cardiac cycle. ECM plays also a significant role in the development of the heart. The aim of this study was to evaluate the expression of important ECM components in the heart of rats with induced CDH to test the hypothesis that an alteration of ECM may contribute to the cardiac maldevelopment, which recently has been identified as a contributive factor for the high mortality rate in babies with congenital diaphragmatic hernia (CDH)., Methods: CDH model was induced in pregnant rats after administration of 100 mg of nitrofen on day 9.5 of gestation (term, 22 days). In control animals the same dose of olive oil was given without nitrofen. Cesarean section was performed on day 21 of gestation. The fetuses were divided into 2 groups: normal control (n = 10) and nitrofen-induced CDH (n = 10). Reverse transcription polymerase chain reaction (RT-PCR) was performed to evaluate the relative amount of tropoelastin and alpha1 (I) procollagen mRNA expression. Elastin protein content was measured using enzyme-linked immunosorbent assay (ELISA)., Results: There was a reduction in tropoelastin mRNA (P <.05) and procollagen mRNA (P <.05) in CDH compared with controls. The cardiac alpha-elastin content also was reduced in CDH (P <.01)., Conclusions: The reduced cardiac tropoelastin and procollagen gene expression and the reduced alpha-elastin content indicate that the heart in CDH structurally is immature. The reduced production of cardiac ECM may contribute to a contractile dysfunction, which makes the heart unable to respond to the hemodynamic load accompanying persistent pulmonary hypertension (PPH)., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The aim of this study was to review all available studies reported in the English-language literature from 1975 through 1998, and by meta-analysis assess the importance of prenatal diagnosis, associated malformations, side of hernia, timing of surgery, and study population on mortality rates in patients with congenital diaphragmatic hernia (CDH)., Methods: One-hundred-two studies were identified, and 51 studies (2,980 patients) fulfilled the prespecified inclusion criteria. Studies were grouped according to study population into: (I) fetuses diagnosed prenatally; (II) neonates admitted to a treatment center; and (III) population-based studies., Results: Pooled total mortality rate was significantly higher in category I than in category III (75.6% v 58.2%, P < .001). Pooled hidden postnatal mortality rate (deaths before admittance to a treatment center) in population-based studies was 34.9%. Prenatally diagnosed patients in both category II and III had significantly higher mortality rates than those diagnosed postnatally. Mortality rates were significantly higher among CDH infants with associated major malformations compared with isolated CDH in all 3 categories. An increased mortality rate in right-sided CDH was found in category II and III., Conclusions: Prenatal diagnosis of CDH, presence of associated major malformations, and the study population have a major influence on mortality rate. The very high mortality rate in studies of fetuses with a prenatal diagnosis of CDH should be taken into account in prenatal counselling.

Background/purpose: The authors present their experience and results in the treatment of infants with imperforate anus over a 10-year period. Differences between these and previously published western results are noted and discussed., Methods: One hundred eight patients with imperforate anus were treated from June 1988 to July 1998. Of these patients, 66 were boys and 42 were girls. Associated anomalies include congenital heart disease, anomalies of bone and cartilage, and Down's syndrome. Thirty-five patients with a low lesion received a limited posterior sagittal anorectoplasty. Seventy-one patients had a high lesion and received 3-staged operations including colostomy, posterior sagittal anorectoplasty, and takedown of colostomy. All patients underwent follow-up by the author. Postoperative anorectal function was evaluated based on the following criteria: ability to have voluntary bowel movement, soiling, and constipation. The duration of follow-up ranges from 6 months to 10 years., Results: One patient died of multiple congenital anomalies after colostomy. One patient died of hyaline membranous disease. All except 2 patients had voluntary bowel movement. Three patients had soiling, and 19 suffered from constipation after operation. The constipation improved with medical treatment and time. Four patients who received the first operation at another hospital (3 underwent posterior sagittal anorectoplasty and 1 had cutback anoplasty) had problems with soiling. In these patients, soiling improved after redo posterior sagittal anorectoplasty., Conclusions: Utilizing the posterior sagittal operation described by Peña, most patients were continent and able to have voluntary bowel movements. Constipation occurred in a substantial number of patients with high-type lesions, but few of these patients needed medication or enemas. There were significantly fewer sacral and urogenital anomalies than have been reported in most western series. This may explain the excellent results.

Background/purpose: Although biliary atresia (BA) is rarely associated with other congenital anomalies, the presence of a distinct subgroup of patients with accompanying structural anomalies such as situs inversus, polysplenia, or portal vein anomalies has been postulated. The authors present 7 patients with this association., Methods: Of 87 patients with BA treated in the past 19 years, 7 (8.0%) have had multiple congenital structural anomalies., Results: These anomalies included situs inversus in 4, polysplenia in 5, preduodenal portal vein in 5, absent portal vein in 1, absent inferior vena cava in 2, malrotation of the intestine in 5, and congenital heart disease in 3 patients. In these 7 patients, hepatic portoenterostomy (HPE) was performed at the age from 63 to 158 days with an average of 92 days. The porta hepatis was abnormal in position in 1 patient. The connective tissue at the porta hepatis was diminished in 6 patients. Histologically, liver fibrosis was mild in 2 and moderate in 5 patients. Bile excretion was good initially in all patients but gradually diminished in 5 patients. Five patients had multiple episodes of cholangitis, followed by sepsis, liver failure, or cardiac failure and subsequently died at the age from 2 months to 6 years. Of the other 2 patients who underwent HPE recently, 1 is doing well and the other has had one episode of cholangitis., Conclusions: BA in association with other congenital structural anomalies may have a poor prognosis. These patients have poor bile secretion after HPE mainly because of delayed operation.

A longitudinal study identified 987 foetal uropathies over a 13-year period. There were 147 deaths. Forty infants died as a result of a lethal uropathy in the presence of associated congenital anomalies. Sixty-six infants with an isolated uropathy died. There were 4 cot deaths, 2 obstetric related deaths, and 34 deaths caused by associated congenital anomalies. There was 1 termination of pregnancy following a false-positive diagnosis of uropathy. Of the 147 deaths, 34 occurred postnatally, 20 within 24 hours. Twenty-nine infants were spontaneously aborted. There were 78 terminations of pregnancy, 43% occurring after 24 weeks of gestation. There was complete concordance in antenatal and postnatal diagnoses in 113 (77%) cases and incomplete concordance in 19 (13%) cases. There were 14 false-negative diagnoses (9.5%). The relative frequency of different lethal congenital uropathies is detailed. Accurate in utero diagnosis of foetal uropathy and hence prediction of outcome is possible. The relatively late gestational age at time of diagnosis remains a constraint when fatal malformations would otherwise prompt termination of pregnancy.

Up to 25% of babies with biliary atresia (BA) bear associated malformations that most often cluster in the polysplenia syndrome (PS). This article examines the impact of associated PS on the final outcome of the authors' BA patients and the necessary modifications of surgical technique during orthotopic liver transplantation (OLT) in these patients. The authors studied 88 consecutive infants with biliary atresia operated upon during the 10-year period between 1984 and 1993. Biliary drainage was provided by a Roux-en-Y hepaticojejunostomy. OLT was performed in 38. Eleven infants (12%) had three or more components of PS: malrotation (11), preduodenal portal vein (11), polysplenia (10), situs inversus (4), absent inferior vena cava (3), cardiac defects (2), and anomalous hepatic artery supply (2). Bile drainage was achieved in 7/11 infants (63% versus 48% for regular BA patients) 5 of whom (45% versus 31%) are jaundice-free with a mean follow-up of 105 months. Three patients received transplants (2 from living-related donor) and are alive and well with normal liver function after a mean follow-up of 35 months. Overall survival was 72% in the BA plus PS and 66% in the BA group (P = not significant [NS]). Results show that long-term bile drainage in these PS patients can be achieved at least as often as in other BA patients (45% versus 31%) (P = NS) and indicate that Kasai operation remains the best initial choice. When OLT becomes necessary, attention to vascular anatomy is required to determine the feasibility of reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

This review encompasses 50 years (1942 through 1991) and 69 newborns (43 boys, 26 girls). Half the babies were premature (weighing less than 2.5 kg), and about one third had other anomalies. The procedures used in this series were late primary anastomosis (17), gastric tube reconstruction (16), staging esophagostomy and gastrostomy (13), gastric pull-up (13), early primary anastomosis (4), and colon replacement (3). Four neonates received no treatment. The most common repair in the 1940s and 1950s was the gastric pull-up; the gastric tube was the most popular in the 1960s and 1970s. Delayed primary anastomosis has been the operation of choice since the 1980s. Over the last decade, it has become apparent that primary repair is successful in three quarters of such infants if the wait is 3 months and/or the newborn weight has at least doubled. This repair appears to provide the best functional result, unless there is an anastomotic stricture. Before the 1970s, the survival rate was below 40%, but since the 1980s the rate has more than doubled, to 90% in our series, regardless of the type of repair used.

The general concept of the association of congenital diaphragmatic hernia (CDH) with other anomalies has been well described. This study is aimed at assessing the distribution of the associated anomalies (AA) by organ system, their influence on prognosis, and the practical signs that should prompt a diagnostic search. One hundred and sixty-six high-risk patients with CDH (symptomatic within the first 6 hours of life) were treated in this institution in the past decade. Sixty-five patients (39.2%) were found to have one or more AA, and 101 had isolated CDH. Of patients with anomalies, cardiac (excluding patent foramen ovale and patent ductus arteriosus) was the most frequent type of AA (63%). Hypoplastic heart syndrome was the most common defect. Many patients had multiple AA. For purposes of analysis, the patients were divided into three groups: isolated CDH, cardiac anomalies, and all other anomalies. The groups were compared with respect to several common clinical and laboratory variables, as well as survival. The frequency and timing of antenatal diagnosis were also noted. The analysis led to the following conclusions. (1) AA are present in more than one third of high-risk patients with CDH; in this group, cardiac lesions predominate. (2) High-risk CDH infants with AA have significantly lower APGAR scores and a lower BPDPO2 (best postductal PO2 before ECMO or surgery) than those with isolated CDH. This is even more evident in the group with cardiac AA. In such patients, a careful search for an undetected AA, especially cardiac, is warranted.(ABSTRACT TRUNCATED AT 250 WORDS)

We evaluated why and how life support was withheld or withdrawn in surgical neonates. During the study period, January 1988 through December 1991, 529 neonates were admitted, 52 of whom died (10%). Twenty-eight deaths were due to the underlying disease. The other 24 patients died because treatment was withheld or withdrawn. In 15 of 24 (group A, mean stay 9.2 +/- 9.1 days) treatment was initially started but later withdrawn (13/15) because of the severity of congenital anomalies alone (7/12) or congenital anomalies associated with chromosomal anomalies (5/12). In 9 of 24 (group B, mean stay 20.3 +/- 17.3 days) treatment was withdrawn because of serious complications. In all cases often lengthy discussions have led the doctors and nurses together with the parents to chose unanimously for withdrawal of treatment. Mechanical ventilation was the intervention most frequently withdrawn (10/15 group A, 9/9 group B). Vasoactive and other drugs were withheld in 5 patients of group A. Sedatives and analgetics were administrated as supportive care permitting the child to die in a humane way, 17 in the lap of a parent and 7 in the lap of a nurse. We conclude that life-sustaining care is withheld or withdrawn relatively frequently from patients at our ICU. Such decisions are ethical ones, taken in the light of professional and technical expertise. Evaluation of withholding or withdrawal of treatment is difficult but necessary to evolve appropriate decision-making procedures and to formulate humane standards of intensive care.(ABSTRACT TRUNCATED AT 250 WORDS)

In children with symptoms secondary to malrotation of the intestine, a retrospective statistical study was undertaken to identify factors associated with an increased risk of mortality. Between 1964 and 1989, laparatomy was performed on 182 children. For study purposes, the children were divided into three groups. Group I included 71 patients with an obstruction only in the duodenum. The remaining 111 children, all of whom had midgut volvulus, were further divided; those without gut necrosis (79) into group II, and those with necrosis (32) into group III. One child each died in groups I and II, and 15 in group III. In all children we evaluated the relationship between mortality and age at presentation, presence of associated serious abnormalities, time from onset of symptoms to surgery, and the presence of necrotic bowel. For group III, we considered the influence of percentage of bowel resected on mortality. In these 182 children the factors associated with an increased risk of mortality were presence of necrosis (P < .0001), presence of other abnormalities (P = .0008), and younger age (P = .0084). Time from onset of symptoms to surgery was not associated with statistically increased risk of mortality. The 1% mortality noted in children without intestinal necrosis (group I and II) was related to associated abnormality. For group III the estimated probability of survival ranged from .999 for patients with 10% of intestinal necrosis to .351 for whose with 75% of intestinal necrosis, assuming the best prognostic conditions (patient older than 3 months with no associated serious abnormalities).

From 1960 through 1980, 14 patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) with two or more other associated anomalies of VATER have been seen. Seventy percent of patients were preterm or low in birth weight. In addition to the esophageal abnormality, vertebral anomalies were found in 7 patients, anorectal anomalies in 11, limb anomalies in 9 and renal anomalies in 9 patients. Three patients had associated duodenal atresia and 4 had cardiac anomaly. Eight patients are dead, 5 are living, and 1 is lost to follow-up. Six of the eight deaths occurred during 1960 to 1970. Preliminary gastrostomy was done in all patients. Primary esophageal repair was carried out in 9 patients--5 died and 4 survived. Three patients had delayed esophageal repair and two of these patients survived. Cervical esophagostomy was done in one patient with EA without TEF after unsuccessful repeated bouginage for elongation of the upper esophageal pouch. Three of the survivors showed growth retardation--below 5th percentile. Aggressive nutritional support and management of chronic problems related to the anomalies are important.

The surgical experience in 30 infants with the VATER association of multiple congenital anomalies is detailed. These infants can be satisfactorily managed by a thorough multisystem (cardiac, renal, digestive, orthopedic) evaluation, well planned operative therapy, and sophisticated neonatal intensive care. Survival was achieved in 77% of patients. Satisfactory growth and development and few longterm complications have been observed in this complicated group of infants. These data indicate that infants with the VATER association can lead reasonably normal lives following aggressive operative treatment and supportive care.

Ninety-seven newborns with esophageal atresia and distal tracheoesophageal fistula (EA-TEF) were treated between 1979 and 1985 inclusive; there were 54 boys and 43 girls. Their weights ranged from 800 to 4,000 g (average, 2.5 kg). They included: 28 neonates with cardiac defects (most common: patent ductus arteriosus [PDA], ventricular septal defect and atrial septal defect [VSD-ASD]), of whom 18 survived (64%); 17 babies with other gastrointestinal anomalies (imperforate anus, duodenal atresia), of whom 12 survived (70%); 12 patients with skeletal malformations (digital, vertebral), of whom 11 survived (91%); 8 newborns with genitourinary abnormalities (hypospadias, undescended testis), of whom 6 survived (75%); and 16 infants with other congenital lesions (trisomy 18, lung agenesis-hypoplasia), of whom 3 survived (18%). Forty-six infants (average, 2.7 kg) had no other anomalies and all survived. As the number of systems with defects increased, both the weight of the baby and survival rate decreased. From this entire series of 97 newborns with EA-TEF, 81 (83%) survived (average, 2.3 kg). Sixteen babies died (average, 1.9 kg); 11 had defects incompatible with life. Eleven of the 16 were never operated on (seven patients with trisomy died within 5 days, and four patients with complex cardiac defects died within 3 weeks). Four of the 16 who were operated on died between 3 months and 2 years from chest problems and one newborn died in the operating room. We concluded that (1) newborns who have the common type of EA-TEF will almost certainly survive if there are no other anomalies; (2) the most frequently associated congenital defects are cardiovascular (28%), gastrointestinal (17%), skeletal (12%), and genitourinary (8%); (3) as the number of systems with defects increases, the weight of the baby and its survival rate fall; and (4) 11% will have trisomy and/or complex cardiac defects with no survival.

Because of the devastating consequences of midgut volvulus as a result of malrotation, we reviewed the charts of 70 consecutive children to define the spectrum of presentation. Although 27 patients (39%) had presenting symptoms within the first ten days of life, 35 (50%) were older than 2 months of age. In general, the older children had a longer course of vague, antecedent symptoms such as intermittent, nonbilious vomiting and chronic abdominal pain. Associated congenital anomalies were common, with 32 patients (46%) presenting with 56 anomalies, the most prevalent of which were intestinal atresia, imperforate anus, duodenal web, and cardiac and orthopedic anomalies. Upper gastrointestinal (GI) series revealed the diagnosis in 29 cases (41%), as did contrast enema in 24 (34%). It is important to note that volvulus, intestinal gangrene, and mortality occurred regardless of age or chronicity of symptoms. Fifteen patients (21%) were discovered serendipitously while being evaluated and treated for seemingly unrelated conditions. No morbidity of mortality occurred in those patients who underwent subsequent semielective Ladd's procedure. The majority of morbidity and all seven mortalities occurred in patients with volvulus and intestinal necrosis. This study emphasizes the need for consideration of Ladd's procedure for children of all ages. In addition, due to the broad range of initial symptoms, a high index of suspicion is required in evaluating children with possible malrotation. Because it remains impossible to predict which patients will have catastrophic complications (based on age or type of presentation), we urge that even incidentally discovered patients with intestinal malrotation undergo Ladd's procedure.

Recently, interest has been expressed in the correction of diaphragmatic hernia in utero as a solution to the problem of high mortality from this condition. Before such a procedure can be adopted in the human, studies aimed at establishing the contribution such advanced techniques would make in reducing mortality are required. Between 1973 and 1982, there were 36 cases of congenital diaphragmatic hernia among 75,512 births--an incidence of 1 in 2097 births. there were 11 (31%) stillbirths and 25 (69%) livebirths. the liveborn patients were divided into two groups: group A included 15 patients who died prior to transfer to the referral center; group B included ten neonates who arrived at the referral center and were operated upon. Twenty (56%) cases of diaphragmatic hernia (11 stillbirths and 9 liveborns who did not live long enough to be transferred to the referral center) were found to have lethal nonpulmonary associated anomalies at autopsy. In view of the high association of lethal anomalies it appears that fetal surgery would have a limited role in reducing mortality in congenital diaphragmatic hernia.