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Background: Long term outcomes of gastric transposition (GT) for complex esophageal atresia (EA) are poorly reported. We aimed to perform comprehensive long term follow up of adults who had been treated with GT for EA as children., Methods: Consecutive patients who underwent GT for EA in childhood aged >18 years old were identified alongside age matched patients who had primary repair (PR). Type of EA, comorbidities and details of surgery were recorded. Telephone interviews included medical history, current symptoms - including gastrointestinal symptom rating scale (GSRS), morbidity and health related quality of life (HRQoL) using gastrointestinal quality of life index (GIQLI)., Results: 32 participants were interviewed in each group (mean age 29 years). BMI (19.9 ± 3.5) was significantly lower (p = 0.0006) in GT group. 6/32 (19%) still required supplementary feeding. Adult morbidity included anastomotic stricture (34%), chronic respiratory disease (28%), dumping symptoms (25%), anemia (47%) and depression (19%). 3 patients required major revision surgery. Participants in both groups report regular upper gastrointestinal symptoms (GSRS: GT = 2.1, PR = 2.0) and were more symptomatic than the normal population (1.4) but not statistically different from each other. HRQoL (GIQLI = 113) was lower than after PR (122) but not significantly different (p = 0.29) and the normal population (125). 23% of GT participants had higher than normal HRQoL., Conclusions: GT for EA is associated with significant morbidity and symptoms, including issues previously unreported in adulthood such as mental health problems. This mandates long term follow up and quality transition of these patients into adult care., Type of Study: Retrospective study., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Three developments which have contributed to the declining necessity for esophageal replacement are improvement in the management of esophageal atresia, prevention of caustic injuries to the esophagus, and early antireflux surgery for intractable gastro-esophageal reflux. Despite these advances, replacement of the esophagus may still be necessary. The two most commonly used procedures for replacing the esophagus are colonic interposition and gastric transposition. Experience with 236 gastric transposition operations reveals a mortality of 2.5%, leak rate of 12%, and stricture of 20%. The follow-up shows a satisfaction of over 90%. New methods of overcoming the need for esophageal replacement are in progress with tissue engineering with a scaffold to produce a tubular graft to bridge the gap in the continuity of the esophagus., (Copyright © 2014. Published by Elsevier Inc.)

Purpose: Various pull-through techniques, both open and laparoscopic, have been performed for Hirschsprung disease. Our study compares open and laparoscopic Duhamel pull-through., Methods: After ethical approval, we reviewed all children (n = 181) with Hirschsprung disease admitted to our institution between 1999 and 2009. We excluded total colonic aganglionosis (n = 14), previous pull-through done elsewhere (n = 33), or follow-up performed abroad (n = 58). Open and laparoscopic pull-through were done in the same period according to surgeon preference. Data were analyzed using χ(2) or Mann-Whitney U test., Results: Seventy-six children had a Duhamel pull-through for rectosigmoid aganglionosis. Operative time, time to full feeds, and length of hospital stay were similar in each group. OPEN (N = 41): Fifteen children (37%) required 33 further procedures. Fourteen had procedures for persistent constipation, including redo Duhamel (n = 2), stoma formation (n = 2), spur division (n = 2), and dilatation/stretch/Botox/rectal biopsy/manual evacuation (n = 23). Three children had other procedures (adhesiolysis [n = 2] and incisional hernia repair [n = 1]). LAPAROSCOPIC (N = 35): Fourteen children (40%) required 30 further procedures. Eleven had procedures for persistent constipation, including redo Duhamel (n = 1), stoma formation (n = 4), spur division (n = 9), and dilatation/stretch/rectal biopsy (n = 8). Three children had other procedures (adhesiolysis [n = 1] and incisional hernia repair [n = 2]). There were 4 conversions., Conclusion: Open and laparoscopic Duhamel pull-through have similar outcomes. We show that the techniques have comparable operative times and hospital stay., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Details of the modern history of paediatric surgery in the United Kingdom with particular emphasis on the 3 main training centres in England in the 1960s to 1970s are discussed. The genesis of the National Health Service and of the British Association of Paediatric Surgeons and their influence on the establishment and sitting of regional centres, education and training, and centralization of rare conditions is highlighted., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Aim: The aim the study was to review our experience and determine the effectiveness of peritoneovenous shunts in the management of persistent ascites., Methods: A retrospective review of all patients who had a peritoneovenous shunt inserted for persistent ascites in our institution over 32 years (1977-2010) was performed., Results: Thirty-one peritoneovenous shunts were inserted into 25 patients. Records from 22 were available. Median age was 34 months (range, 1 month-12 years), with median duration of ascites of 1.75 months (range, 3 weeks-2.5 years). Sixteen had previous abdominal surgery, whereas one had previous thoracic surgery. Other underlying pathologies included hepatitis (n = 2), lymphohistiocytosis, lymphatic hypoplasia, and carcinomatosis. One was regarded as idiopathic. Previous management consisted of paracentesis (n = 15), dietary modification (n = 11), diuretics (n = 9), and total parenteral nutrition (TPN) (n = 4). One underwent fetal drainage of ascites. No intraoperative complications occurred. Eight (36%) had postoperative complications, including shunt occlusion (n = 2), pulmonary edema (n = 2), infection (n = 2), and wound leakage (n = 1). One developed a varix following shunt removal. The ascites resolved after shunting in 20 (91%) of the 22 children. Four died from their underlying pathology. In children with malignancy, the shunt allowed prompt resumption in treatment schedule., Conclusion: This represents the largest series of children receiving peritoneovenous shunt for persistent ascites. It is a safe and effective treatment which should be considered early., (Copyright © 2011. Published by Elsevier Inc.)

Purpose: The aim of the study was to evaluate the surgical outcome of esophageal atresia (EA) without fistula for 24 years at a single tertiary center for pediatric surgery., Methods: The study used a retrospective chart review of infants diagnosed with EA without fistula between 1981 and 2005., Results: Of 33 patients with EA without fistula, 31 charts were available. Mean birth weight was 2327 g (range, 905-3390 g), and 71% were male. Most common associated anomalies were cardiac (n = 6; 19%) and renal (n = 5; 16%), followed by vertebral (n = 4; 13%) and anorectal (n = 2; 7%). The median initial esophageal gap was 5 vertebral bodies. Six had a primary repair, and 25 patients had esophageal replacement at a median age of 7 months. This involved gastric transposition in 20 (1 followed failed jejunal interposition), colonic interposition in 5, jejunal interposition in 1 (after a failed colonic), and repair at another center in 1. With a median review of 9 years, 21 patients had long-term sequelae with the need for multiple further surgical procedures including an antireflux procedure in 5. One patient died., Conclusions: Management of EA without fistula remains challenging. Most patients required staged treatment that included esophageal replacement. The frequency of late complications indicates the need for programmed long-term review.

In the Hunterian Museum at the Royal College of Surgeons of England in London, there are a series of fetal specimens of varying gestational ages collected by John Hunter (1728-1793). Among this collection is a fetus demonstrating a gastroschisis anomaly that had gone unrecognized for almost 200 years.

Aim: An enormous amount of information about pediatric surgical conditions is available on the World Wide Web (www). Our aim was to ascertain how many parents accessed the www and how useful they found the exercise., Method: Over a 2-month period, all parents attending the surgical outpatient clinics were asked to complete a questionnaire regarding Internet use in seeking more information about their child's condition. Parents were able to tick more than one option to the questions., Results: A total of 271 questionnaires were collected and analyzed. There were 53% of responders who had accessed the www. Of this group, 93% used a computer at home, with 60% using the Internet daily. The most popular search engine used was Google (75%). There were 90% who used their child's condition as keyword(s), with 21% using their child's symptoms. The most popular information sought is as follows: Ninety-four percent found the Internet useful. Of this group, 18% considered the information too technical, 18% too distressing, and 2% insufficiently specific. Of the group who did not find the Internet useful (6%), 50% found little or no information, 38% too much information, and 13% thought the details were too technical. Only 25% discussed their findings with their surgeons. Of those who did not, most found that the information was already covered by the surgeons or was irrelevant., Conclusion: The Internet is a useful educational tool in teaching parents about their child's condition. Parental use of the Internet is already widespread and may need to be specifically addressed during consultation. The best way to ensure that parents have access to quality and accurate information about their child's condition on the www, hence providing support, is to provide it ourselves.

A brief resume of the highlights in the history of oesophageal atresia is presented. This is followed by research into the etiology, ontogeny and embryology, and microbiological studies. A revised classification of risk factors with consequent survival statistics is presented. Lessons learned in the management of the condition over a 40-year period are reported with particular emphasis on the management of the preterm infant with associated severe respiratory distress, right-side aortic arch, upper pouch fistula, 'long-gap' atresia, and the use of gastrostomy and intercostals drains. The incidence and treatment of early and late complications is discussed.

Purpose: The Spitz classification of oesophageal atresia (OA) based on the birth weight and the presence of a major cardiac anomaly was proposed 1994. Advances in neonatal care have led us to question if these outcome figures are still valid. We tested the hypothesis that the outcome of neonates with OA has improved during the last decade., Methods: The records of all neonates (n = 188) born with OA and treated in a single institution between 1993 and 2004 were reviewed and compared with data from the original Spitz study. Data were obtained on the birth weight, presence of a major cardiac anomaly, and survival. Differences in survival were compared using the Yates-corrected chi2 test., Results: In the early period, 326 neonates survived (87.6%) compared with 174 (91.5%) in the most recent decade (P = .10). Based on Spitz classification, the outcome comparing both periods was the following: group I, 97% (283/293) and 98.5% (130/132) (P = .44); group II, 59% (41/70) and 82% (41/50) (P = .01); group III, 22% (2/9) and 50% (3/6) (P = .57), respectively., Conclusions: The Spitz classification remains valid. It may be of use when counselling parents and in comparing outcome among centres. In our centre, the overall survival of neonates with OA has not significantly changed in the recent decade. The improvement in survival of neonates in group II, however, demonstrated the recent advances in neonatal, paediatric surgical, and cardiac care.

Background: Retching, an early component of the emetic reflex, is a common and distressing symptom in children after Nissen fundoplication. Alimemazine (trimeprazine, Vallergan; Castlemead, Herts, UK) is a phenothiazine derivative histamine(1) antagonist, which anecdotally relieves the retching symptoms., Material and Methods: A prospective, double-blind, randomized, crossover, placebo-controlled study of 15 neurologically impaired children with retching after Nissen fundoplication over a period of 1 year (December 2002-December 2003). Patients were randomly allocated to receive 1 week each of alimemazine and placebo with crossover. A diary was maintained of retching episodes 1 week before, during, and 1 week after the trial. Dosage of alimemazine used was 0.25 mg/kg 3 times a day (maximum, 2.5 mg per dose). Statistical analysis was done using a paired Student's t test, where P value of less than .05 was considered significant. Results are presented as mean +/- SD., Results: Twelve parents completed the diaries (9 open, 3 laparoscopic Nissen fundoplication). Median age of the child was 36 months (8-180 months), median duration of retching was 4.5 months (1-52 months), and mean number of retching episodes per week was 60 +/- 29.40. Mean number of retching episodes with alimemazine was 10.42 +/- 9.48 vs 47.67 +/- 27.79 with a placebo (P < .0001). No adverse effects were reported in those cases that completed the study., Conclusion: At low dose, alimemazine (Vallergan) is a safe and effective drug in the management of retching after Nissen fundoplication.

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.

Background/purpose: The aim of this study was to review the management of the gastrointestinal tract in cloacal exstrophy and to assess the effect of spinal dysraphism on nutritional outcome., Methods: Twenty-two patients with cloacal exstrophy were reviewed retrospectively. Gastrointestinal and spinal anomalies were documented. The need for nutritional supplementation was recorded. Weights at 1 and 5 years of age were used to assess growth in childhood., Results: Ten patients were treated initially with ileostomy, 7 with colostomy, and 3 without stoma. Two patients died before surgical reconstruction. Total parenteral nutrition (TPN) was utilized in 8 infants for a median of 10 days (range, 5 to 200). Three patients had complications with colostomies requiring conversion to ileostomy. Patients with an ileostomy required more nutritional supplementation compared with patients with a colostomy (50% v 28%). High-output stoma losses were more common in patients with an ileostomy (40% v 14%). Fourteen patients (67%) with spinal dysraphism had a higher incidence of failure to thrive in the first year of life (69% v 26%) and multiple episodes of enteritis (40% v 25%). Two neonates with duodenal atresia and small bowel deletion died within the first month of life. One patient with short bowel syndrome died of TPN-associated liver disease at 6 months of age. There were no other deaths., Conclusions: The gastrointestinal tract contributes significantly to the morbidity and mortality in cloacal exstrophy. Nutritional supplementation is more frequently required in patients with an ileostomy. Stoma complications were higher in those with a colostomy. Morbidity is high in patients with spinal dysraphism.

Background: Gastroenteric fistula occurs in 2% to 3% of patients undergoing percutaneous endoscopic gastrostomy (PEG). The authors identified 12 children with this major complication., Methods: A retrospective case note review was performed., Results: Over a 5-year period, the complication rate for gastroenteric fistula was 3.5% (12 of 343 procedures). Presentation ranged from 1 day to 25 months post-PEG insertion. Eight patients presented with acute intestinal obstruction. The gastroenteric fistulous tract involved the posterior wall of the stomach in all cases. The plain abdominal x-ray was useful in establishing the diagnosis of the gastroenteric fistula., Conclusions: Patients with gastroenteric fistula as a complication of PEG insertion can remain asymptomatic for prolonged periods. It often is difficult to make the diagnosis. A plain abdominal x-ray is a useful diagnostic modality.

Purpose: The purpose of this study was to analyze the outcome in 173 children (104 M, 69 F) undergoing transposition since 1981., Methods: The most common indications for esophageal replacement included failed repair of different varieties of esophageal atresia (127), caustic injury (23), and peptic strictures (8). A total of 81% of the patients were referred from other hospitals (50% from other countries). Age at operation ranged from 7 days to 17 years. The gastric transposition was performed using blunt mediastinal dissection only in 90 patients with an additional 81 patients undergoing lateral thoracotomy. The retrosternal position was used in 2 patients., Results: There were no graft failures including those who had previously failed gastric tube or Scharlilike operations. Anastomotic leakage occurred in 12% (all but 1 resolved spontaneously). Anastomotic stricture requiring dilatation occurred in 19.6%. Half of these patients had previous caustic esophageal injury. There were 9 deaths in the group (5.2%). One death occurred intraoperatively, 5 in the early postoperative period, and there were 3 late deaths. In more than 90% of our patients, the outcome was considered good to excellent in terms of absence of swallowing difficulties or other gastrointestinal symptoms. Many, however, preferred to eat small frequent meals. Poor outcome was particularly associated with multiple previous attempts at esophageal salvage. There was no deterioration in the function of the gastric transposition in those patients followed up for more than 10 years., Conclusions: Gastric transposition for esophageal substitution is an acceptable procedure. It is attended by 5% mortality and a 12% leak rate. A total of 19.6% of the patients needed anastomotic dilatation for stricture. In the long term, good function has been maintained. Gastric transposition compares favorably with alternative methods of esophageal replacement.

Purpose: The aim of this study was to review the outcome and complications after resection of hepatoblastoma treated over 2 decades in our institution., Methods: Clinical, radiologic, and pathologic data were reviewed retrospectively, focusing on the outcome and complications., Results: Between January 1978 and December 2002, 56 children were treated for hepatoblastoma. The age range was 0.08 to 8.74 years (median, 1 year). The right lobe was involved in 48%, the left lobe in 22%, and in 29% the main bulk of the tumour was centrally located. Surgical procedures included the following: hemihepatectomy in 62%, trisegmentectomy in 18%, extended hemihepatectomy in 16%, and liver transplantation and laparotomy in one patient each. Intraoperative complications occurred in 5(9%)--rupture of the tumour (1), haemorrhage from the contralateral lobe (1), a defect in the left hepatic duct (1), cardiac arrest from tumour embolus (1), and bleeding from the inferior vena cava (1). The mean blood loss was 280 mL (50 to 2,000 mL). Postoperative complications occurred in 12 patients (22%) including subphrenic abscess (3), adhesion obstruction (2), ischaemic stenosis of the bile duct (1), abdominal wound dehiscence (1), pyloric obstruction (1), and pleural effusion (2). Fifteen patients died, 14 as a result of tumour recurrence (mortality rate, 27%).

The authors report experience with 2 neonates with the unusual combination of intrathoracic stomach and Marfan's syndrome.

Purpose: The aim was to determine the effects of early and late endotoxemia on neonatal cardiac and renal mitochondrial energetics., Methods: Suckling rats received intraperitoneal 300 microgram/kg lipopolysaccharide; controls received saline. Heart and kidney mitochondria were isolated after 2 hours (early) or 6 hours (late sepsis). State 3 (maximum mitochondrial flux) and 4 O(2) consumption and complex I activity were measured. Results, expressed as mean +/- SEM normalized to citrate synthase (CS), were compared using paired t tests., Results: Mortality rate was zero within 2 hours, 2.7% between 2 and 6 hours of endotoxemia, and 100% 6 to 8 hours; therefore, we consider that 2 hours and 6 hours represent early and late endotoxemia, respectively. Endotoxic heart mitochondria had unaltered O(2) consumption at 2 hours but significantly decreased state 3 after 6 hours, resulting in significantly decreased respiratory control ratio. Complex I activity, which could affect O(2) consumption, was decreased significantly at 6 hours (9.8 +/- 0.6 mU/U CS; n = 15) versus controls (11.3 +/- 0.8, n = 15; P =.04), but not at 2 hours. There were no differences in these measurements at either 2 hours or 6 hours in kidney mitochondria., Conclusions: The respiratory chain is affected late in endotoxemia. Neither early nor late endotoxemia affects oxidative function of kidney mitochondria., (Copyright 2003 Elsevier Inc. All rights reserved.)

Background/purpose: The secreted glycoprotein, Sonic hedgehog (Shh) plays an important patterning role in the development of many organ systems. The authors aimed to study the temporal and spatial pattern of expression of Shh and its receptor Ptc1 during the development of the anterior foregut and to test the hypothesis that the Shh expression pattern is disturbed during the development of oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) in Adriamycin-treated mouse embryos., Methods: Saline and Adriamycin-treated (4 mg/kg) CBA/Ca embryos were harvested between embryonic days (E) 10.5 and 12.5, and Shh and Ptc1 expression was studied by whole-mount and section in situ hybridisation using digoxygenin-labelled riboprobes., Results: At E10.5, saline-treated embryos had an undivided foregut in which the ventrally placed prospective tracheal epithelium was positive for Shh, whereas the dorsal part was negative. At E11.5, this pattern had reversed with the separated trachea becoming negative and the oesophagus gaining expression of Shh. Ptc1 was expressed in the mesoderm adjacent to Shh expressing endoderm at both stages. Affected Adriamycin-treated embryos had an undivided foregut at E11.5, the epithelium of which showed diffuse Shh staining that lacked the dorso-ventral patterning seen in controls., Conclusions: The reversal in the dorso-ventral pattern of Shh expression during the narrow embryologic window in which tracheo-oesophageal separation is initiated suggests that Shh may play an important role in this process. Transient disturbance of this pattern may underlie the abnormal organogenesis in the Adriamycin model., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Background/purpose: Sepsis is an important cause of neonatal mortality. The aim of the study was to investigate the metabolism of endotoxic neonatal rats and the potential beneficial effect of glutamine., Methods: Suckling rats received intraperitoneal saline (control; C), endotoxin (300 microg/g LPS; E), saline+glutamine (2 mmol/g; CG), endotoxin+glutamine (EG), saline+leucine (2 mmol/g; CL) or endotoxin+leucine (EL). Sepsis score (0-8) and rectal temperature were monitored. Hypothermia was defined as rectal temperature less than 32 degrees C. Oxygen consumption (VO2, mL/kg/h), a determinant of heat production, was measured by indirect calorimetry. Data (mean +/- SEM) were compared by analysis of variance (ANOVA), paired t test or Fisher's Exact test., Results: Endotoxic (E) rats had significantly lower VO2 than C rats from 90 minutes postinjection to the end of the experiment, 210 minutes (VO2 from 150 to 210 minutes: C 671 +/- 45; E 429 +/- 36, P <.0004; n = 8; paired t test). VO2 of CL or CG rats was elevated between 90 and 210 minutes compared with control, but significantly (P <.01) only in the L group (C 706 +/- 31; CG 871 +/- 63; CL 984 +/- 31; n = 7-9, ANOVA). VO2 was significantly higher (P <.05) in EG rats than E rats (E 460 +/- 29; EG 654 +/- 68; n = 9-10). In the EL group, VO2 was raised but was not significantly different from E (E 460 +/- 29; EL 637 +/- 52; n = 8-10). EG rats were significantly less hypothermic between 90 and 210 minutes (58 of 132 measurements) compared with E (95 of 147; P =.0007, Fisher's Exact test), whereas the EL group were similarly hypothermic (74 of 120) to E (P =.7). Sepsis score was significantly lower in the EG group than both E and EL groups (E 4.9 +/- 0.3; EG 3.6 +/- 0.3; EL 5.0 +/- 0.3; n = 40; P <.01; ANOVA)., Conclusions: Neonatal endotoxaemia lowers VO2, heat production, and body temperature. Glutamine and leucine both cause nutrient-induced thermogenesis in control animals and restore VO2 of endotoxic animals. Glutamine additionally increases rectal temperature, reduces incidence of hypothermia, and improves clinical signs of endotoxic rats. This suggests that glutamine may be beneficial for nutrition in neonatal sepsis., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Purpose: The aim of this study was to review the surgical complications of pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI)., Methods: A retrospective review was conducted of patients undergoing pancreatectomy for PHHI in one institution over the past 13 years., Results: The records of 48 patients were reviewed; the age at operation ranged from 10 days to 30 months (median, 8 weeks). Weight at operation ranged from 1.97 to 11.4 kg (median, 5.2 kg). There were no deaths. Intraoperative complications comprised bleeding in 7, (major in 2), splenic injury in one, bile duct injury in 2 (1 oversewn, 1 choledochoduodenostomy), and 1 small bowel injury. Postoperatively, 5 children underwent choledochoduodenostomy: 2 for biliary leak and 3 for delayed bile duct stricture. Other postoperative complications included wound infection (n = 3), prolonged ileus (n = 1) and adhesion obstruction (n = 1), and wound leakage (n = 1). Renal failure developed in one child owing to acute tubular necrosis. Nine patients required further pancreatic resection because of continued hypoglycaemia. Three patients continued to require medication for hyperinsulinism despite surgery, 20 required insulin, and 13 required pancreatic enzyme replacement at the time of the last review., Conclusions: Pancreatectomy resulted in resolution of hyperinsulinism in 45 of 48 patients. Sixteen patients required no further surgery or medication. Pancreatectomy for PHHI may be associated with major intra and postoperative morbidity., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Background/purpose: Secondary organ damage to the lungs is an important consequence of intestinal ischaemia reperfusion (IIR) injury. Moderate hypothermia ameliorates gut necrosis and liver energy failure after IIR but potential beneficial effects on lung neutrophil infiltration after reperfusion of ischaemic bowel have not been investigated., Methods: Adult Sprague-Dawley rats underwent 60 minutes intestinal ischaemia followed by 120 minutes of reperfusion. The animals were maintained at either normothermia (36 degrees to 38 degrees C) or moderate hypothermia (30 degrees to 32 degrees C). Four groups were studied: (A) sham normothermia; (B) IIR normothermia; (C) sham hypothermia; and (D) IIR hypothermia. Lungs and terminal ileum were removed for measurement of myeloperoxidase activity (a marker of neutrophil infiltration). Results are expressed as milliunits per milligrams protein, mean +/- SEM, and one-way analysis of variance (ANOVA) with Tukey post-test was used for group comparisons., Results: Lungs: IIR at normothermia significantly increased lung neutrophil infiltration assessed by myeloperoxidase activity compared with sham-operated controls (normothermia sham 4.6 +/- 1.0, n = 8; normothermia IIR 37.7 +/- 13.8, n = 8; P =.011). Moderate hypothermia during IIR significantly attenuated lung neutrophil infiltration (7.2 +/- 2.1, n = 9) compared with normothermia IIR (P =.016) such that myeloperoxidase activity was similar to that found in sham normothermia (4.6 +/- 1.0, n = 8) and sham hypothermia (3.1 +/- 1.3, n = 8). Intestine: Gut myeloperoxidase activity was 0.9 +/- 0.5 in sham normothermia (n = 9) and 2.3 +/- 0.6 after normothermic IIR (n = 8). After IIR at hypothermia gut myeloperoxidase activity (0.5 +/- 0.2; n = 8) was significantly less than normothermic IIR (P =.035) and higher than sham hypothermia (0.2 +/- 0.1, n = 9; P =.01)., Conclusions: These results indicate that moderate hypothermia may prevent damage to another distant organ, ie the lungs, by preventing recruitment of neutrophils. This may be of benefit in decreasing distal organ damage in diseases in which intestinal ischaemia-reperfusion is implicated in the pathogenesis., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Unlabelled: The absorption of carbon dioxide (CO2) used for positive pressure pneumoperitoneum may lead to an increased CO2 load. CO2 elimination during laparoscopy has not been investigated previously in paediatrics. The aim of this study was to characterise the pattern of CO2 elimination during laparoscopic surgery in infants and children., Methods: Twenty children undergoing laparoscopy and 19 children undergoing laparotomy for elective abdominal operations were studied. Pneumoperitoneum was achieved using insufflation of unheated CO2. CO2 elimination (metabolically produced + absorbed; milliliters per kilogram per minute) was measured minute by minute during the operation by indirect calorimetry. End-tidal CO2 (kPa) was recorded every 10 minutes. The above variables were assessed before CO2 insufflation, during pneumoperitoneum, and after desufflation., Results: Before insufflation, CO2 elimination was 4.6 +/- 0.3 ml/kg/min and increased after 15 minutes of pneumoperitoneum to 5.2 +/- 0.3 (P <.001). Post desufflation, CO2 elimination decreased toward preinsufflation values, but did not return to baseline by the end of operation (5.8 +/- 0.3; P <.001). End-tidal CO2 was 4.7 +/- 0.2 preinsufflation, peaked at 1 hour (5.3 +/- 0.2; P <.001) and subsequently decreased in response to ventilatory adjustments. The total amount of CO2 insufflated was positively correlated with patient age (r2 = 0.27; P <.01). CO2 elimination was age related, as indicated by multilevel model analysis and by negative correlations between maximum increase in CO2 elimination and both age (r2 = 0.27; P <.01) and weight (r2 = 0.29; P <.01). These data suggest that the younger or smaller the child, the larger the increase in CO2 elimination. Seven patients (35%) responded to desufflation with a sharp transient increase in CO2 elimination, which did not appear to be related to patient age, length of pneumoperitoneum, abdominal pressure, or type of operation., Conclusions: During pneumoperitoneum, younger children absorb proportionately more CO2 than older individuals. The short-lived increase in CO2 elimination postdesufflation may be related to an increase in venous return from the lower limbs after release of the abdominal pressure. These findings suggest that small children warrant close monitoring during laparoscopy and during the immediate postoperative period., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Background/purpose: A small proportion of infants born with oesophageal atresia in which the gap between the 2 ends of the oesophagus is too great for an end-to-end anastomosis will require oesophageal replacement. Since 1981 the author's procedure of choice for oesophageal replacement has been gastric transposition. The long-term functional outcome appears to be satisfactory, but the quality of life of these patients has not been investigated formally. This report assesses the health-related quality of life (QOL) of 2 groups of patients born with oesophageal atresia who have undergone gastric transposition., Methods: The study group comprised 28 patients aged 2 to 22 years who resided in England. Group 1 (n = 13), comprised patients who had undergone cervical oesophagostomy and gastrostomy without attempt at oesophageal anastomosis; group 2 (n = 15), comprised patients who had undergone previous attempts at reconstruction or replacement. QOL was assessed using modified versions of the Gastrointestinal Quality Of Life Index (GIQLI)., Results: QOL scores based on patients' responses showed no significant differences between the groups (124 v 119). However, the disease-specific symptom scores showed that patients in group 1 experienced fewer symptoms compared with those in group 2. Additionally, based on parental responses, patients in group 1 had higher QOL scores than those in group 2. QOL scores for patients aged 2 to 4 years (n = 5) did not differ between the groups (81 v 92, not significant)., Conclusions: The quality of life for patients with oesophageal atresia undergoing gastric transposition was generally unimpaired by any side effects of gastric transposition. Patients undergoing gastric transposition as a primary procedure experienced fewer disease-specific symptoms in the medium term compared with patients who had undergone previous unsuccessful attempts at reconstruction or replacement of their oesophagus., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Background/purpose: The heart and kidney are both affected in sepsis-related multiple organ failure. Both utilize fatty acid substrates during the neonatal period, and impairment of oxidative metabolism during sepsis could lead to bioenergetic failure. The enzyme carnitine palmitoyl transferase I (CPT I) is important in the control of fat oxidation in the neonatal period. The aim of this study was to determine the effects of sepsis on neonatal cardiac and renal CPT I., Methods: Suckling rats received 300 microgram/kg lipopolysaccharide intraperitoneally. Mitochondria were isolated from the heart and kidney after 2 hours. CPT I and II activity were measured radiochemically. Protein levels of M- and L- isoforms of CPT I, both of which are present in heart, were determined by Western blotting., Results: CPT I activity was decreased significantly in the heart but not in the kidney by endotoxemia, whereas CPT II activity was the same in each organ. To investigate the mechanism of this decrease, we carried out Western blotting of the CPT I isoforms in heart mitochondria. Neither M- nor L- isoform was decreased in amount. To determine whether free-radical attack could directly inhibit CPT I activity, control heart mitochondria were incubated with free-radical generating systems. Although hydrogen peroxide had no effect on CPT I activity, the reactive oxygen species nitric oxide, superoxide, and peroxynitrite, all of which are generated in the heart during sepsis, significantly inhibited CPT I activity., Conclusions: The activity of CPT I, a rate-controlling step of fat oxidation, is significantly impaired in heart but not in kidney during neonatal sepsis. This may be caused by direct attack by free radicals, suggesting that antioxidant strategies could be of use in preventing sepsis-related cardiac damage., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Purpose: This study investigated the roles of moderate hypothermia and extraluminal oxygenated perfluorcarbon (PFC) on intestinal metabolism after ischemia-reperfusion., Methods: A model of 30-minute intestinal ischemia followed by 60 minutes of reperfusion was used. The animals were maintained at either normothermia (36.5 to 37.5 degrees C) or moderate hypothermia (31 to 32 degrees C). Four groups of adult rats were studied (n = 8 per group): (A) sham at normothermia, (B) ischemia-reperfusion at normothermia, (C) ischemia-reperfusion at hypothermia and, (D) ischemia-reperfusion with extraluminal oxygenated PFC perfusion during ischemia at normothermia. Intestinal phosphocreatine, ATP and lactate levels were measured. Histologic changes in the intestine were evaluated., Results: Intestinal ischemia-reperfusion at normothermia caused a marked reduction in phosphocreatine and ATP with an increase in lactate. Moderate hypothermia exerted beneficial effects by attenuating the depletion of high-energy phosphates and the elevation of lactate. Extraluminal PFC perfusion during ischemia failed to produce a protective effect on high-energy phosphates, although it reduced lactate accumulation. Moderate hypothermia significantly decreased the degree of mucosal damage., Conclusions: Whole-body moderate hypothermia protects the small intestine from reperfusion injury as measured both biochemically and histologically. Extraluminal oxygenated PFC administration during ischemia did not protect the intestine from reperfusion injury in this model., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Background/purpose: Thomsen-Friedenreich cryptantigen activation (TCA) exposes neonates with necrotizing enterocolitis NEC to the risk of hemolysis after transfusion of blood products. The authors aimed to determine the prevalence of TCA in neonates with NEC and to correlate TCA with severity of disease and outcome., Methods: One hundred four neonates with NEC were tested for TCA on admission. Patients with TCA requiring transfusion were given packed red cells, low-titer anti-T fresh frozen plasma, and washed platelets to avoid hemolysis., Results: Twenty-three infants had TCA, and 96% of these had stage III disease. The incidence of TCA was significantly higher in infants with stage III disease compared with those with stage II (30% v 4%; P <.01). A total of 91% of infants with TCA required laparotomy compared with 81% of those with no activation. At laparotomy, widespread disease was more common in the TCA group (71% v 55%). TCA did not significantly increase mortality rate (TCA, 39% v no TCA, 28%); this may reflect the transfusion policy of our unit., Conclusions: Twenty-two percent of neonates with NEC referred to our unit had TCA. There is an association between TCA and advanced NEC. Screening of neonates with advanced NEC for TCA is advised to identify those at risk of hematologic complications., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Background/purpose: The well-established Adriamycin rat model of oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) complements recently described mouse genetic models in which loss of function mutations in foregut patterning genes, such as Nkx2.1 (Ttf 1), lead to OA/TOF. The authors aimed to integrate the 2 systems by adapting the Adriamycin model to the mouse to study molecular aspects of tracheo-oesophageal development., Methods: Pregnant CBA/Ca mice were injected intraperitoneally with 4 mg/kg of Adriamycin on embryonic days 7.5 and 8.5. Embryos and fetuses of various gestational ages were subjected to morphologic or histologic examination. Sections were stained with H & E or processed for immunohistochemistry using an antibody specific for Nkx2.1., Results: Tracheo-oesophageal malformations were observed in 47% of Adriamycin-treated embryos. Early foregut development was similar in Adriamycin-exposed and control embryos but, by E11.5, many treated embryos had an undivided oesophago-trachea, which gave rise to the lung buds and a fistula to the stomach. The fistula originated from the dorsal aspect of the undivided tube and was negative for Nkx2.1, or showed only transient Nkx2.1 expression, compared to the strongly positive bronchi ventrally., Conclusions: The Adriamycin model of OA is adaptable to the mouse. In the absence of tracheo-oesophageal separation, the dorsal fistula retains its nonrespiratory commitment suggesting that dorsoventral patterning of foregut development is undisturbed by Adriamycin exposure.

Background/purpose: In neurologically impaired children, retching and recurrent vomiting are common after Nissen fundoplication. The aim of this study was to identify whether there are preoperative factors that predict their occurrence., Methods: Twenty neurologically impaired children (8 boys, 12 girls; age range, 3 months to 8 years) were studied prospectively by taking a detailed history of behaviors and symptoms associated with feeding before and after Nissen fundoplication for gastroesophageal reflux., Results: Preoperatively, children could be classified into 2 groups. Children in group A had symptoms suggestive of only gastroesophageal reflux (effortless "vomiting" or regurgitation), whereas children in group B exhibited one or more features associated with activation of the emetic reflex (pallor, sweating, retching, forceful vomiting). Postoperatively 0 of 8 in group A retched compared with 8 of 12 in group B (P <.005, Fishers Exact test)., Conclusions: Children at high risk of retching, and ultimately vomiting, after antireflux surgery may be identified clinically preoperatively. They have symptoms that are specifically caused by activation of the emetic reflex rather than to gastroesophageal reflux. In these cases, antireflux surgery could be considered inappropriate and hence be avoided., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The aim of this study was to assess long-term educational attainment in adolescence of former infants who required major neonatal surgery., Methods: The study is an extension of a prospective longitudinal study begun in 1983 on 30 full-term newborns requiring major neonatal surgery, a matched healthy control group, and full-term infants requiring neonatal intensive care for medical reasons. Educational attainment levels were obtained from the results of compulsory national curriculum examinations taken at age 11 years in 3 core academic subjects, English, Mathematics, and Science and teachers' assessments of current academic performance. Additionally, follow-up data on health were obtained from family general practitioners and parents., Results: Seventy percent of the original surgical group, 48% of the original control group, and 77% of the original medical group underwent follow-up. The participants were aged between 11 and 13 years. After adjustment for social factors (gender, social group, and mother's age), the surgical group was significantly behind on all measures of educational attainment (English, P < .0005; Mathematics, P < .02; Science, P < .0005; academic performance, P = .004), compared with the control group and medical group who did not differ from each other. Independent predictors of outcome were mechanical ventilation for >or=4 days in the neonatal period and behavior problems at 3 years. At 12 months of age, independent predictors of cognitive functioning were length of hospitalization and, at 3 years of age, the number of operative procedures. Mechanical ventilation was not significantly associated with cognitive functioning at previous stages of the study., Conclusions: These results show that, in early adolescence, children who required major neonatal surgery were performing less well academically compared with their peers. This information can be used to increase awareness of the difficulties these children may experience during childhood so that intervention can be directed appropriately, thereby lessening the risk of later educational problems. J Pediatr Surg 36:858-862., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The aim of this study was to characterize the type and incidence of complications related to colostomy formation in newborn infants with anorectal anomalies., Methods: The authors reviewed a 5-year (1994 to 1999) experience of a single institution in the management of neonates with high and intermediate anorectal anomalies who required colostomy at birth. Patients with colostomy still in place have been excluded from the study to maximize the chances of detecting colostomy-related complications., Results: There were 80 neonates with anorectal malformations, of whom, 49 (31 boys and 18 girls) were included in the study. The site of colostomy was sigmoid colon (n = 32), transverse colon (n = 7), and descending colon (n = 10). Thirty-nine colostomies were loop, and the remaining 7 were divided. The median birth weight was 2.96 kg (range, 1.46 to 3.88). The age at colostomy formation was 2 days (range, 1 to 210). Mechanical complications related to colostomy formation were observed in 16 infants (32%) with 3 infants having more than 1 mechanical complication. These included prolapse in 8 (50%), intestinal obstruction (adhesions, intussusception, and volvulus) in 7 (44%), and skin dehiscence in 3 (19%). One neonate had necrotizing enterocolitis (NEC) after colostomy formation. Urinary tract infection was observed after colostomy in 14 infants (29%). The incidence of urinary tract infection was not higher in infants who had loop colostomy (11 of 39, 28%) compared with infants who had divided colostomy (3 of 10, 30%). There were no differences in the incidence of colostomy-related complications and urinary tract infection between male and female infants. There were no deaths in this series., Conclusions: Formation of colostomy for anorectal anomalies should not be considered a minor procedure. In our experience the incidence of complications after colostomy formation is high. The incidence of urinary tract infections does not seem to be affected by the type of colostomy performed., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The aim of this study was to (1) characterize the source of additional medical information acquired by parents and (2) to verify how much information is correctly interpreted and remembered correctly., Methods: A cross-sectional prospective study was made of parents of 108 children with surgical diseases consecutively admitted to a children's hospital in 1999. The median age of the children was 3 years (range, 0.01 to 16), and that of parents was 34 years (range, 18 to 54). Parental knowledge and understanding of their child's illness, operation, potential operative risk, and long-term outcome were scored on the basis of (1) parents' self appraisal (questionnaire) and (2) objective evaluation (interview) performed by one investigator not involved in the child's care., Results: Parents' education included a university degree (15%), entry examination for university (13%), basic educational qualification (47%), and no educational qualification (15%). Additional medical information was obtained by 77% of the parents (63% from general practitioner, 46% from books, 31% from popular magazines, 24% from internet). Inadequate knowledge of illness and treatment was highlighted in 12% and 19% of the assessment made by interview, respectively, compared with 8% and 14% of the self appraisals. Inadequate knowledge of operative risk was higher in the assessment made by interview (42%) compared with self appraisal (9%; P <.0001). Similarly, inadequate knowledge of long-term outcome was higher in the interview (28%) compared with self appraisal (13%; P <.05). Acquisition of additional medical information irrespective of the source did not affect the accuracy of parental knowledge. Medical knowledge was more accurate in parents of children who required major operations and in those with higher educational qualifications (P <.05)., Conclusions: Parents commonly obtain additional medical information. However, this information does not necessarily improve parents' understanding of the child's operative risk and long-term problems. Parents' perception of having adequate medical information often is incorrect., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The aim of this study was to evaluate the clinical significance of low platelet count in the surgical management of neonates with necrotizing enterocolitis (NEC)., Methods: The clinical course of 58 consecutive neonates with advanced NEC (Bell's stages II or III) treated in the authors' hospital between 1995 and 1998 was reviewed. NEC in neonates who required operation was classified as isolated, multifocal, or pan-intestinal. Severe thrombocytopenia was defined as platelet count less than 100 x 10(9)/L. Rapid fall in platelet count was defined as fall greater than 150 x 10(9)/L within 24 hours to a level less than 100 x 10(9)/L., Results: Median birth weight was 1,564 g (range, 550 to 4,270) and gestational age was 31 weeks (range 23 to 41). Twenty-two neonates (38%) were below 1,000 g. Age at the onset of the disease was 13 days (range, 1 to 62). NEC was treated medically in 7 neonates (12%). Indications for operation included pneumoperitoneum in 23 neonates (45%), clinical deterioration in 19 (37%), and intestinal obstruction in 9 (18%). The nadir platelet count (lowest level during the course of disease) was lower in patients with stage III disease than in patients with stage II disease (P <.05). The greater the extent of the disease, the lower the platelet count (P =.012). The nadir platelet count was lower in infants who died than in survivors (P <.05). None of the patients with platelet count greater than 100 x 10(9)/L died. In predicting intestinal gangrene, severe thrombocytopenia has a sensitivity of 69%, specificity of 60%, and positive predictive value of 89%; rapid fall in platelet count has a sensitivity of 32%, specificity of 89%, and positive predictive value of 92%., Conclusions: (1) A platelet count less than 100 x 10(9)/L or a rapid fall in platelet count represent poor prognostic factors. (2) Monitoring the platelet count during the course of NEC is useful; however, it cannot be used in isolation to predict the extent of the disease or survival rate., (Copyright 2001 by W.B. Saunders Company.)

Background/purpose: Surgical neonates are at risk of sepsis-associated liver dysfunction. Hydrogen peroxide (H(2)O(2)) and nitric oxide (NO) are important mediators of sepsis, which impair neonatal hepatic metabolism. Glutamine has been shown to have beneficial effects on hepatocyte metabolism during neonatal sepsis. However, the molecular basis of these effects are unknown. The aim of this study was to test the hypotheses that (1) glutamine and its dipeptides counteract the inhibitory effect of septic mediators on neonatal hepatocyte oxygen consumption and (2) the effects of glutamine are specific and not shared by other amino acids. In addition, we wished to determine the metabolic pathways and mediators involved in the action of glutamine., Methods: Hepatocytes were isolated from suckling rats, and O(2) consumption measured polarographically. Study A: the ability of 10 mmol/L glutamine to reverse the inhibitory effects of 1.5 mmol/L H(2)O(2) and 300 micromol/L S-Nitroso-N-acetylpenicillamine (SNAP; a nitric oxide donor) on O(2) consumption was examined. Study B: the ability of other amino acids and dipeptides of glutamine to reverse the effects of H(2)O(2) was examined. Study C: various concentrations of glutamine were tested for their ability to reverse the H(2)O(2) inhibition of O(2) consumption. Study D: the mechanism of action of glutamine was examined by incubating hepatocytes with either an inhibitor of entry into the Krebs cycle or an inhibitor of glutathione synthesis. Study E: the ability of glutathione to reverse the inhibitory effects of H(2)O(2) was examined., Results: Study A: glutamine reversed the inhibition of hepatocyte O(2) consumption exerted by either H(2)O(2) or NO. Study B: glutamine dipeptides reversed the inhibition of hepatocyte O(2) consumption by H(2)O(2), but other amino acids did not. Study C: the counteracting effect of glutamine was proportional to the dose administered. Study D: blocking entry of glutamine into the Krebs cycle did not abolish the effects of glutamine, but blocking glutathione synthesis completely abolished the effect of glutamine. Study E: exogenous glutathione reversed the inhibitory effect of H(2)O(2) on hepatocyte O(2) consumption., Conclusions: This study found that glutamine and its dipeptides are unique in reversing the effects of septic mediators on neonatal rat liver oxidative metabolism. The effectiveness of glutamine appears to be mediated via glutathione synthesis. Addition of glutamine, glutamine dipeptides, or glutathione to total parenteral nutrition (TPN) may be beneficial in preventing liver damage in neonatal sepsis.

Background/purpose: Lipopolysaccharide (LPS) and cytokines produced during neonatal sepsis trigger free radical production, which eventually results in inhibition of liver metabolism. Studies in adults have indicated a hypermetabolic response to sepsis; however, evidence for a hypermetabolic response in neonates is equivocal. This study was carried out to determine whether LPS and cytokines can cause liver hypermetabolism in neonates., Methods: The initial bacterial insult and cytokine cascade were mimicked by the addition of lipopolysaccharide (Escherichia coli 055:B5), tumour necrosis factor (TNF-alpha), and interleukin-6 (IL6) during the isolation of hepatocytes by collagenase digestion from 11- to 13-day-old Wistar rats. Hepatocyte oxygen consumption was measured polarographically with cells respiring on palmitate (0.5 mmol/L). Myxothiazol, a specific inhibitor of mitochondrial respiration, was used to distinguish extra- and intramitochondrial oxygen consumption. Morphologic changes were assessed by electron microscopy., Results: The addition of LPS, TNF-alpha and IL6 during hepatocyte isolation resulted in a 10% decrease in cell yield (P <.05) compared with untreated controls; however, cell viability was unchanged (n = 31). Both total and extramitochondrial oxygen consumption were significantly greater in treated cells compared with untreated controls (P <.05, Student's t test). Electron microscopy indicated that LPS, TNF-alpha, and IL6 did not cause ultrastructural changes to hepatocytes., Conclusions: The increase in oxygen consumption was predominantly extramitochondrial and likely to be caused by increased oxygen requirement for cytosolic detoxification and repair purposes. This study shows that liver hypermetabolism metabolism can occur in response to LPS and cytokines. However, during in vivo neonatal sepsis, additional free radical damage may blunt this hypermetabolic response.

Background/purpose: Intestinal ischaemia-reperfusion (IR) can cause liver failure. The aims of this work were to study the effects of intestinal IR on liver energy metabolism and to evaluate the effects of moderate hypothermia., Methods: Intestinal IR (90-minute intestinal ischaemia plus 60-minute or 240-minute reperfusion) was achieved by clamping and unclamping the superior mesenteric artery in rats. Normothermia or moderate hypothermia (30 degrees to 33 degrees C) was maintained by adjusting the environmental temperature. The ratio of hepatic inorganic phosphate to adenosine triphosphate (ATP) was monitored continuously during intestinal IR using in vivo phosphorus ((31)P) magnetic resonance spectroscopy. Phosphorus metabolites also were measured in extracts prepared from freeze-clamped liver and intestine., Results: Mortality occurred exclusively during normothermic intestinal IR. A progressive increase in the hepatic inorganic phosphate to ATP ratio after normothermic intestinal IR was observed. Moderate hypothermia delayed this effect. Analysis of liver extracts confirmed above findings. However, there was no difference in intestinal phosphocreatine or ATP between normothermic and hypothermic rats undergoing intestinal IR., Conclusions: Intestinal IR at normothermia was associated with liver energy failure and high mortality rate. Moderate hypothermia ameliorated liver energy failure but did not attenuate intestinal energy failure after intestinal IR. Hypothermia may prove to be useful in the management of patients with intestinal IR injuries in the future.

Background/purpose: Hypothermia is common after major surgery in newborns and can be triggered by intraoperative fentanyl analgesia. Recent studies have found that fentanyl inhibits hepatocyte mitochondrial oxidative metabolism, which is proportional to thermogenesis. In adults it has been shown that amino acids have a thermogenic effect, although the biochemical basis of this phenomenon is not known. The aim of this study was to test the hypothesis that amino acids counteract the inhibition of neonatal hepatocyte oxygen consumption by fentanyl., Methods: Hepatocytes were isolated from suckling rats, and O2 consumption was measured polarographically. In experiment A hepatocytes were incubated with (1) palmitate alone (control), (2) palmitate plus fentanyl, (3) palmitate plus fentanyl plus amino acids, and (4) palmitate plus amino acids. In experiment B the effects of essential and nonessential amino acids were tested separately. In experiment C, to investigate whether the effect of amino acids is intramitochondrial, hepatocytes were incubated with amino acids plus inhibitors of mitochondrial respiration., Results: In experiment A, fentanyl significantly inhibited O2 consumption (P = .006). This inhibition was reversed by amino acids (P < .001). In experiment B, both essential and nonessential amino acids reversed the effect of fentanyl (P < .001). In experiment C, there was no difference in O2 consumption in the presence of myxothiazol among the groups indicating that amino acids affect intramitochondrial O2 consumption., Conclusions: (1) Amino acids abolish the inhibitory effect of fentanyl on hepatocyte oxidative metabolism. (2) Amino acids affect intramitochondrial O2 consumption and therefore thermogenesis. (3) Perioperative administration of amino acids in neonates may help to prevent hypothermia and its deleterious effects.

Purpose: This study explores the effects of ischemia-reperfusion on various metabolic aspects of the small intestine., Methods: Intestinal ischemia-reperfusion was obtained by clamping and unclamping the superior mesenteric artery in adult rats. Four groups of animals were studied: (A) sham operation for 150 minutes, (B) 90-minute intestinal ischemia, (C) 150-minute intestinal ischemia, and (D) 90-minute intestinal ischemia followed by 60-minute reperfusion. Body temperature was maintained at normothermia (36.5 to 37.5 degrees C). Concentrations of intestinal glucose, succinate, lactate, amino acids, phosphocholine (PC), glycerophosphocholine (GPC), choline, and phosphoenergetics were measured using magnetic resonance spectroscopy of freeze-clamped small intestine extracts., Results: Intestinal ischemia (groups B and C) alone caused a significant drop in glucose and phosphoenergetics but caused an increase in amino acids, succinate, and lactate. Ischemia and ischemia-reperfusion decreased PC and GPC but increased choline. After intestinal reperfusion (group D), no recovery of phosphoenergetics was observed, but there was partial recovery of glucose, succinate, lactate, and amino acids., Conclusions: There is no recovery of phosphoenergetics after 90 minutes of intestinal ischemia followed by 60 minutes of reperfusion. Partial recovery of glucose, succinate, lactate, and amino acids may reflect equilibration of these metabolites between damaged cells and extracellular fluid.

Background/purpose: Cervical, thoracic, and pelvic neuroblastomas are regarded as having a better outcome than abdominal primaries. The aim of the study was to analyze the results of treatment of pelvic neuroblastomas in our institution., Methods: The authors reviewed the records of 284 patients with neuroblastoma treated in our hospital during the period 1983 through 1998 and identified 17 (6%) with pelvic tumors. The revised International Neuroblastoma Staging System was used., Results: There were 6 patients with stage 1 disease, 8 with stage 2, 2 with stage 3, and 1 with stage 4 disease. Intraspinal extension of the tumor was present in 7 patients (41%). Except for one child with stage 4 disease, all patients underwent an attempt of tumor excision, and 6 had a complete resection. All 7 patients with intraspinal tumor survived. Permanent postoperative neurological complications occurred in 6 patients (35%). These included sciatic nerve palsy, urinary and fecal incontinence, neuropathic bladder, and leg weakness or nerve root injury L4-S1. Three of 17 patients died, but 1 fatality was unrelated to the tumor. The overall survival rate was 82% and was not influenced by the completeness of tumor resection., Conclusions: The survival of nonmetastatic pelvic neuroblastoma in our institution is good despite incomplete tumor resection. Intraspinal extension is not a negative prognostic factor. Considering the high incidence of permanent neurological damage after surgery and the generally favorable biological characteristics of these tumors, surgical treatment should not be overaggressive.

Background/purpose: The management of oesophageal atresia (OA) in association with a right-sided aortic arch (RAA) is challenging. It is controversial whether right or left thoracotomy is the most appropriate surgical approach. The aim of this study was to determine the prevalence of RAA in patients with OA and the most appropriate surgical approach., Methods: The case records of all the neonates with OA treated over an 18-year period (1980 through 1997) were reviewed. Patients with RAA were analysed with particular reference to the preoperative investigations, operative approach and findings, and postoperative complications., Results: Of the 476 case notes reviewed, 12 (2.5%) had a RAA, and 5 of these infants had major cardiac anomalies. The diagnosis was not suspected on preoperative chest x-ray in any of these infants. In only 1 of the 5 neonates who had a preoperative echocardiogram was RAA suspected. A right thoracic approach was performed in the 11 infants in whom a RAA was not suspected. Immediate conversion to a left thoracotomy with primary anastomosis was carried out in 2 infants. In 2 further infants, fistula ligation alone was performed because of a long gap atresia. Anastomosis via the right thoracotomy was performed in 7 infants. There were 3 anastomotic leaks, 1 intractable stricture, and 1 recurrent fistula. Three patients (25%) had a double aortic arch, and, as a result, a left thoracotomy had to be abandoned in 2. There were 3 deaths, 2 as a result of major cardiac anomalies and 1 related to prematurity and sepsis., Conclusions: RAA occurs in 2.5% of infants with OA. Preoperative chest x-ray and echocardiogram are unreliable as diagnostic modalities. Anastomosis via a right thoracotomy is associated with a 42% leak rate. The presence of a double aortic arch can make the oesophageal anastomosis via a left thoracotomy as difficult as via a right thoracotomy.

Background/purpose: Liver function is frequently impaired in neonates with sepsis. Nitric oxide (NO) is thought to be a mediator of organ dysfunction and liver oxidative metabolism during sepsis. The authors developed an in vitro model to investigate the effect of NO and the combined effect of NO plus H2O2 on neonatal hepatocyte oxidative metabolism., Methods: Hepatocytes were isolated from neonatal rats. Oxygen consumption was measured polarographically. In Study A, cells were exposed to S-Nitroso-N-acetylpenicillamine (SNAP), an NO donor, at various concentrations. In study B, myxothiazol and oligomycin, inhibitors of mitochondrial respiration, were added to investigate the site of action of NO. In study C, hepatocytes were incubated in the presence of both SNAP (300 micromol/L) and H2O2 (1.5 mmol/L). In study D, morphological alterations induced by NO and NO plus H2O2 were investigated by hepatocyte electron microscopy., Results: In study A, SNAP caused a dose-dependent decrease in oxygen consumption. A significant inhibition was reached at 300 micromol/L SNAP. In study B, the lack of further inhibition when SNAP was given together with myxothiazol indicates that NO acts intramitochondrially. Similarly, no further inhibition occurred when the NO donor was given together with oligomycin, suggesting that the effect of NO is mainly at the level of ATP synthase. In study C, concomitant addition of 300 micromol/L SNAP and 1.5 mmol/L H2O2 to hepatocytes caused further inhibition of oxygen consumption compared with either SNAP or H2O2 alone. In study D, mild alterations in hepatocyte morphology were noted in the presence of SNAP or SNAP plus H2O2., Conclusions: In neonatal hepatocytes, NO significantly inhibits mitochondrial oxygen consumption, possibly at the level of ATP synthase. The effect of NO is additive to that of H2O2. Morphological findings were consistent with these biochemical effects and suggest that NO and H2O2 are important mediators of liver damage during sepsis.

The authors report the case of a 12-year-old boy with cystic fibrosis, in whom a percutaneous endoscopic gastrostomy device migrated into the jejunum, forming a gastrojejunal fistula.

Background/purpose: Conjoined twins often have different body composition and growth rate before separation. This may be because of differences in energy metabolism. The aim of this study was to investigate the energy expenditure, body composition, and calorie intake of thoracopagus conjoined twins with shared hepatic circulation but separate gastrointestinal tracts., Methods: The twins were studied at two periods: (1) before separation (age, 73 days) and (2) after separation (age, 97 days). Calorie intake over the study periods was carefully documented. Respiratory gas exchange was measured by computerized indirect calorimetry. The postseparation weight ratio of twin A to twin B was used to approximate the preseparation weights. Body composition (total body fat) was calculated from skinfold thickness and anthropometric measurements., Results: The body composition of the twins was different: body weight and total body fat were higher in twin B. Resting energy expenditure and calorie intake were markedly different between the conjoined twins before separation. In both twins, the energy expenditure increased after separation. After separation, the resting energy expenditure of the twins was similar., Conclusions: This study illustrates the difference in energy metabolism in a set of thoracopagus conjoined twins. The authors speculate that twin A was supplying nutrients to twin B resulting in increased energy expenditure before separation. This would explain the lower calorie intake and higher fat mass of twin B.

Background/purpose: Surgical neonates are at risk for sepsis and liver dysfunction. These complications are more common in preterm neonates and in those who receive total parenteral nutrition. Elevated levels of reactive oxygen species (eg, hydrogen peroxide) have been reported in these "at-risk" patients and may be the mediators of liver impairment via their effect on oxidative energy metabolism. The aim of this study was to test the hypothesis that elevated levels of hydrogen peroxide (H2O2) impair neonatal liver oxidative energy metabolism., Methods: An in vitro model to test this hypothesis was developed in hepatocytes isolated from neonatal (11-day to 15-day) rats. The cells, respiring on palmitate (0.5 mmol/L in 2% bovine serum albumin), were exposed to H2O2. Oxygen consumption was measured polarographically. In experiment A, H2O2 was added to the cell preparation at different concentrations (0.5 mmol/L, 1 mmol/L, 1.5 mmol/L, 2 mmol/L) to assess the effect on oxygen consumption. In experiment B, H2O2 (2 mmol/L) was added to hepatocytes in the presence of inhibitors of mitochondrial respiration to define the site of action of H2O2. In experiment C, electron microscopy was performed on hepatocytes after incubation with 1 mmol/L and 2 mmol/L of H2O2., Results: In experiment A, H2O2 significantly reduced hepatocyte oxygen consumption at 1.5 and 2 mmol/L. In experiment B, in the presence of inhibitors of mitochondrial respiration, myxothiazol (inhibitor of substrate oxidation), and oligomycin (inhibitor of adenosine triphosphate (ATP) synthase), no further inhibition by H2O2 occurred, indicating that the effect of H2O2 was intramitochondrial and affecting the synthesis of ATP. In experiment C, microscopic alterations of mitochondria were noticed exclusively in hepatocytes incubated with 2 mmol/L H2O2., Conclusions: Results of this study demonstrate that H2O2 impairs neonatal liver oxidative metabolism. H2O2 probably directly inhibits ATP synthase. The authors hypothesize that H2O2 may play a role in the biochemical pathogenesis of liver dysfunction associated with sepsis. Identification of the precise target site of H2O2 may be valuable in directing therapy in septic neonates.

Purpose: The aim of this study was to evaluate the results of surgical treatment of necrotizing enterocolitis (NEC) according to the extent of disease and to establish if resection of the ileocecal valve represents a poor prognostic factor., Methods: The authors reviewed all cases of NEC (n = 161) treated in our hospital during the last 11 years; of these, 83 required surgical intervention. Definitions used by the authors include isolated, disease in a single intestinal segment; multifocal, disease in two or more intestinal segments; and pan-intestinal, majority of small and large bowel involved., Results: Twenty-five neonates had isolated NEC, 46 neonates had multifocal NEC, and 12 had pan-intestinal involvement. Survival rate was affected by the extent of intestinal involvement, the lowest survival rate (33%) being noticed in neonates with pan-intestinal involvement. In patients with isolated NEC, postoperative complications and survival rate were not affected by the modality of operative treatment. Conversely, in patients with multifocal NEC, survival rate was higher (85%) after resection and primary anastomosis compared with enterostomy (50%; P =.03). Resection of the ileocecal valve was not associated with increased morbidity and mortality., Conclusions: (1) Resection and primary anastomosis is a valid treatment option in both isolated and multifocal NEC. (2) Neonates with NEC adapt rapidly to the loss of the ileocecal valve.

Purpose: The aim of this study was to determine the long-term outcome of neonates who required extracorporeal membrane oxygenation (ECMO) for respiratory insufficiency associated with congenital diaphragmatic hernia (CDH)., Methods: All neonates born with CDH, who required ECMO (oxygenation index over 40 in the presence of maximal support) during a 6-year period, were reviewed. The median duration of follow-up for survivors was 14 months (range, 9 to 22 months)., Results: Of 72 neonates with CDH, 11 (15%) required ECMO. The median age at the start of ECMO was 2 days (range, 1 to 4); duration was 9 days (range, 4 to 17); post-ECMO ventilation was 27 days (range, 8 to 86); and stay in intensive care unit, 33 days (range, 8 to 133). Three patients survived and are not oxygen dependent. There were three early deaths at 9 to 16 days and five late deaths at a median of 6 months (range, 2 to 32 months) secondary to chronic lung disease (n = 3) and infection (n = 2). Four of the five patients with late deaths were oxygen dependent at discharge from hospital. Three of the late death patients and one survivor underwent a fundoplication for gastrooesophageal reflux., Conclusions: Neonates born with CDH and requiring ECMO had a significant late mortality. Follow-up for more than 1 year is indicated to assess the benefit of ECMO for this diagnosis. The authors report only three long-term survivors from 11 ECMO patients selected from a total of 72 neonates with CDH.

Purpose: The aim of this study was to evaluate the results of fundoplication performed in the first 4 months of life., Methods: Case-notes of 66 patients less than 4 months old who underwent fundoplication between January 1986 and July 1997 were reviewed. The mean length of follow-up was 24 months (range, 3 to 115 months). Diagnosis of gastrooesophageal reflux (GOR) was based on a barium meal in 55 patients or 24-hour pH monitoring in 23 patients., Results: Fundoplication was performed at a median age of 9 weeks (range, 3 to 15 weeks). Associated anomalies were identified in 56 infants and included oesophageal atresia or tracheooesophageal fistula (n = 19), lung abnormalities (n = 7), congenital diaphragmatic hernia (n = 4), and various other complex syndromes including neurological disorders (n = 26). Isolated GOR occurred in 10. Ninety percent of infants with isolated GOR improved clinically after the fundoplication compared with 64% of those with associated anomalies. Only 58% of infants in whom GOR developed after oesophageal atresia repair improved after fundoplication. Vomiting was reduced after fundoplication in 76% of infants, apnoea and/or respiratory symptoms improved in 66%, whereas failure to thrive incidence improved in only 38% of infants. Revision of the fundoplication was required in 16 (24%) patients. There were eight deaths, all of which occurred at least 6 months after the fundoplication., Conclusions: Fundoplication in early infancy is unsuccessful in a high proportion of patients. The highest incidence of failure and redo fundoplication occurred in infants with associated anomalies. The best results were achieved in infants with emesis and respiratory symptoms.

Background/purpose: Studies in human surgical neonates have shown that intraoperative fentanyl analgesia results in greater fall in perioperative body core temperature compared with morphine analgesia. The aim of the study was to compare in a neonatal animal model the biochemical effect of fentanyl and morphine on hepatocyte oxidative metabolism., Methods: Hepatocytes were isolated from suckling rats and the oxygen consumption from palmitate was measured polarographically. In experiment A, fentanyl and morphine within the respective analgesic serum ranges were added to hepatocytes to assess the effect on oxygen consumption. In experiment B, fentanyl was added to hepatocytes in the presence of inhibitors of mitochondrial respiration to investigate its site of action. In experiment C, hepatocytes were incubated with either fentanyl or morphine, centrifuged, and then examined ultrastructurally by electron microscopy., Results: In experiment A, fentanyl inhibited oxygen consumption by up to 40% (P < .01). Morphine inhibited oxygen consumption to a maximum of 25% (P < .01). In experiment B, in the presence of oligomycin, fentanyl increased the inhibition of oxygen consumption; however, in the presence of myxothiazol, no further inhibition by fentanyl occurred. In experiment C, mild ultrastructural alterations to hepatocytes were observed after incubation with fentanyl but not with morphine., Conclusions: This study demonstrates that therapeutic doses of two commonly used analgesic drugs impair neonatal hepatic oxidative metabolism. Fentanyl exerts a greater effect than morphine by diminishing liver oxygen consumption by up to 40%. The inhibitory effect of fentanyl occurs directly on the mitochondrial respiratory chain, either on substrate oxidation or on the thermogenic proton leak. The findings of this study are relevant to the perioperative management of surgical neonates.

Background/purpose: Although the survival rate for the infants with total colonic aganglionosis (TCA) has improved significantly, problems with the surgical management continue and the long-term consequences for growth and continence are poorly documented. The aim of this study was to review the experience in the management of 48 patients over a 17-year period (1980 to 1996)., Methods: The medical records of all patients were analyzed with particular emphasis on the number and type of surgical procedures, the attainment of anorectal continence, number of stools per day, and physical development., Results: There were 30 boys and 18 girls. Three (6%) patients died: one of sepsis, one of associated major congenital cardiac anomaly, and one of Moebius syndrome and brain stem dysfunction. Forty-one patients (85%) went on to undergo a pull-through procedure: 38 Duhamel with 13 having Martin modification and three with Soave procedure. Inappropriate surgery (47 procedures) were carried out in 19 (40%) patients before the definitive diagnosis; only four of these infants were admitted primarily to our unit. Thirteen patients had a stoma in aganglionic intestine. An ileostomy was closed in six infants before the diagnosis was established, and six had a previous "negative" laparotomy. Long-term follow-up was possible in 27 patients, of whom 19 (70%) required a total of 39 additional procedures. Ten patients underwent a total of 16 anal dilatations and six underwent sphincterotomy. A permanent stoma was necessary in six patients (two with Down's syndrome). Two patients with Martin modification required resection of the side-to-side anastomosis for intractable diarrhea. The number of the stools per day decreased yearly. Fecal incontinence was common at the 5-year follow up (82%). However, at 10 and 15 years, the rate of incontinence decreased to 57% and 33%, respectively. The proportion of patients below the second percentile for body weight were 25% at 5 years, 20% at 10 years, and 63% at 15 years of age. The equivalent statistics for body height were 15%, 0%, and 23%, respectively., Conclusions: Patients with TCA tend to undergo multiple procedures. Anorectal function improves gradually over time. There are no advantages of the Martin modification. In the long-term follow-up over half of the patients are below the second percentile for weight and one quarter are below the second percentile for height. Careful long-term follow-up is necessary for the patients with TCA.

Purpose: The aim of this study was to review the need for regular endoscopic biopsy of Barrett's esophagus in children., Methods: This was a retrospective case-notes review of 38 children with Barrett's esophagus treated between January 1982 and August 1997. The mean age at diagnosis was 6.3 years (range, 1 to 15 years). All had gastroesophageal reflux at diagnosis. Two patients were treated medically, and 36 underwent antireflux surgery (32 Nissen fundoplication, four Thal procedures). At follow-up, for a mean of 43 months (range, 0 to 13 years), 25 underwent repeated endoscopy and biopsies, two underwent further surgery and biopsies, and four underwent endoscopy only. Seven have had follow-up at their referring hospital., Results: In the 27 patients who underwent rebiopsy, there was continued evidence of Barrett's esophagus in 15. There was reversion to normal tissue in 10 patients, and mild esophagitis was present in two. There was no evidence of any dysplastic or malignant change in any patient. All cases that reverted to normal esophagus or mild esophagitis had previously undergone a Nissen fundoplication., Conclusions: Dysplastic degeneration and malignant change did not occur in any of the authors' patients. In addition, 12 patients with Barrett's esophagus reverted to normal. The authors therefore question whether regular endoscopic surveillance is necessary in children under 16 years of age.