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At least 19 recordsLinked to original sources

Long-term outcome of anorectal malformations: the patient perspective.

To assess the long-term outcome of surgery for anorectal malformations (ARM) from the patient's perspective. One hundred and sixty seven children were operated for ARM between 1982 and 2000. Disease impact questionnaires to assess both clinical and psychological outcomes were sent to 137 eligible families. Statistical analysis was performed using SPSS version 11.5. The response rate was 61% (n=84). Sixty six percent had soiling. There was no significant difference in the incidence of soiling between genders or between the younger child and adolescent. Soiling was significantly increased in high (86%) and intermediate (79%) compared to low (43%) malformation (P=0.001). Constipation was seen in 62% and abdominal pain in 49%, with no significant difference between malformation levels. Overall, 71% had associated anomalies. Although 44% had a documented urological abnormality, clinical significant problems were seen in only 30%. Eighty percent of the children had one or more behavioural problems and 15% expressed suicidal thoughts. ARM had a negative impact on the social life of the child in 52% and on family functioning in 50%. Soiling (P=0.000), presence of associated anomalies (P=0.001), constipation (P=0.005), level of ARM (P=0.015) and abdominal pain (P=0.039) correlated significantly with psychosocial morbidity. Despite these findings, 62% of adolescents and 71% of children below 12 years with their parents reported above average global hopefulness score, remaining hopeful for the future. Children with ARM have ongoing physical and social morbidity, indicating the need for continuing multidisciplinary review and support to optimise their quality of life.

Adolescent↗

Body fluid compartment changes following neonatal surgery.

The neonate is born with an excess of total body water (TBW) in the range of 75% to 85% of body weight (v 60% in the adult), which is due to a large extracellular fluid volume (ECF) of 40% to 50% of body weight (v 20% in the adult). In an attempt to define the changes that occur in TBW and ECF following neonatal surgery, the following prospective study was carried out. Twenty newborns with major congenital anomalies (gastroschisis, 10; esophageal atresia, 4; ileal atresia, 3; omphalocele, 2; and malrotation, 1) were studied weekly for 4 weeks following surgery while being maintained on a standard protocol of total parenteral nutrition (TPN). Total body water was measured using deuterium oxide, and extracellular fluid volume was assayed with sodium bromide; both were expressed as percent body weight (BW). Weight gain or maintenance was observed in all infants. TBW remained unchanged (from 85.4% to 83.0%), and ECF decreased from 51.2% to 36.7% during the observation period. The TBW in the ten gastroschisis infants decreased from 87.3% to 78.0% v the ten other newborns, in whom there was no change (84.0% to 85.0%). The ECF changes were more dramatic, decreasing from 51.6% to 32.3% in the gastroschisis infants, v 50.8% to 45.5% in the other neonates (P = .0156). There were no differences between the two groups in the intake and output of fluids. The following conclusions were drawn: (1) Following major surgery, newborns show a steady decrease in ECF and minimal change in TBW in spite of adequate weight gain while receiving TPN.(ABSTRACT TRUNCATED AT 250 WORDS)

Body Fluid Compartments↗

[Malrotation?].

Explore the source record for details and available documents.

Digestive System Abnormalities↗

A syndrome of multiple gastrointestinal atresias with intraluminal calcification. A report of a case and a review of the literature.

An infant with multiple gastrointestinal atresias from the stomach to the rectum is reported and the literature concerning this syndrome is reviewed. The syndrome has been reported, to date, exclusively in infants born to families with a French-Canadian background. The radiological hallmark of this syndrome is extensive calcification of intraluminal content between the areas of atresia which appears as rounded or oval homogeneous radiopacities on abdominal radiographs. Inheritance is thought to be autosomal recessive and the etiology is uncertain. All previously reported cases have died. The significance of biliary gas, seen in postoperative films, is discussed.

Adult↗

Total parenteral nutrition associated cholestasis: a predisposing factor for sepsis in surgical neonates?

Of 496 neonates and infants less than 1 year of age admitted to the paediatric surgical intensive care unit (PSICU) over a 5 year period (1983-1987), 94 required total parenteral nutrition (TPN) for more than 14 consecutive days, generally due to congenital anomalies of the digestive tract. Cholestasis occurred in 15 of them and 12 of these patients developed sepsis. In contrast, of the 79 patients on TPN that remained free from cholestasis, only 23 developed sepsis. The mortality rate for the TPNAC-group was substantially higher than for the group without TPNAC. It is suggested that development of TPNAC might lead to impairment of non-specific cellular immunity in neonates.

Bacterial Infections↗

Separation of omphalopagus conjoined twins using combined caudal epidural-general anesthesia.

PURPOSE: To describe the anesthetic management of newborn omphalopagus conjoined twins undergoing a series of diagnostic and surgical procedures which culminated in successful separation at one month of age. CLINICAL FEATURES: Evaluations of the extent of shared organ systems were carried out without the need for anesthesia. The twins were anesthetized twice, once for insertion of skin expanders, and later for surgical separation. Various airway management techniques were utilized. To facilitate surgical separation, caudal epidural catheters were inserted in an effort to provide both operative and post-operative analgesia. In addition, prior to the induction of anesthesia, the extent of cross-circulation between twins was assessed. CONCLUSION: Caudal epidural catheters can be used to provide both operative and post-operative analgesia. Early extubation, another benefit of regional analgesia, was not achieved because both twins developed respiratory failure in the immediate postoperative period. Testing for the extent of cross-circulation between twins proved valuable, allowing for detailed scripting of the complex induction sequence and airway management.

Anesthesia, Epidural↗

Late presentation of a duodenal web in a patient with situs inversus and apple peel jejunal atresia.

A 16-year-old girl presented with signs of proximal intestinal obstruction. In the neonatal period, surgical correction of an apple peel atresia had been performed, and she also had a situs inversus abdominalis. Revision of the anastomosis had been done when she was 3 years old. Contrast studies apparently again showed a stricture of the anastomosis, which was treated by stricturoplasty. Because of persistent obstruction, reexploration was done and revealed a duodenal membrane. Anastomotic strictures are very rare several years after the primary operation, so other causes of obstruction should be sought.

Adolescent↗

Intercellular adhesion molecule (ICAM-1) response after major neonatal surgery.

Surgical stress induces systemic endocrine-metabolic responses that influence the function of endothelial cells (EC) to cause various systemic reactions. Intercellular adhesion molecule (ICAM)-1 is an adhesion molecule that plays an important role in inflammation, and increased expression of ICAM-1 on EC is a reflection of EC activation. In this study, we investigated the ICAM-1 response to surgical stress in neonates undergoing major surgery. Fifteen neonates (mean age at surgery: 3.5 +/- 1.2 days) were divided into two groups according to indications for surgery: Group I: Congenital diaphragmatic hernia without persistent pulmonary hypertension (n = 5); Group II: Gastrointestinal surgery [n = 10: duodenal atresia (n = 3), intestinal atresia (n = 6), and esophageal atresia (n = 1)]. Serum samples were obtained preoperatively, immediately after completion of surgery (time zero), and 24, 48, 72, 96, and 120 h after surgery to measure ICAM-1 levels using an enzyme-linked immunosorbent assay, C-reactive protein (CRP), and white blood cell count (WBC). Postoperative recovery was uneventful in all cases. ICAM-1 levels in both groups increased significantly within 24 h of surgery (Group I: P = 0.0038, Group II: P = 0.0320). In Group I, ICAM-1 peaked 72 h postoperatively while in Group II it continued to rise until 96 h postoperatively. The difference between peak levels reached was not significant. CRP was first detected 24 h postoperatively in both groups and continued to increase until 48 h postoperatively. Again, the difference between peak levels reached was not significant. No significant changes in WBC were observed in either group. We found that ICAM-1 increases in response to surgical stress in neonates, although there was no significant difference in levels. However, surgical stress as represented by serum ICAM-1 would appear to last longer with intestinal surgery than with non-intestinal surgery. Further research is required to establish the usefulness of ICAM-1 as an easily detectable substance associated with endothelial damage that reflects the host's response to major surgical stress.

Biomarkers↗

The optimal management of malrotation diagnosed after infancy: a decision analysis.

BACKGROUND: The benefit of a prophylactic Ladd's procedure in older children and adults with malrotation is controversial. The purpose of this study was to determine the role of the Ladd's procedure in patients with asymptomatic malrotation diagnosed after infancy. METHODS: A Markov decision analysis was used to compare the quality adjusted life expectancy with and without a Ladd's procedure among patients with asymptomatic malrotation. Data obtained from the Nationwide Inpatient Sample were used to estimate the age-related probability of emergency surgery or volvulus among patients with malrotation. Estimates of the mortality of elective and emergency surgery, mortality of volvulus, and utilities of each health state were obtained from the literature. RESULTS: After infancy, the gain in quality adjusted life expectancy associated with a prophylactic Ladd's procedure was highest when asymptomatic malrotation was treated at 1 year old and steadily declined until asymptomatic malrotation was treated at 20 years old. An increasing advantage of observation over prophylactic surgery on life expectancy was observed after the second decade of life. A 2-fold increase in mortality risk for an elective Ladd's procedure decreased the age threshold to 14 years, whereas a 4-fold increase decreased the threshold to 7 years. These results were found to be robust by sensitivity analyses and Monte Carlo simulation. CONCLUSION: A Ladd's procedure should be considered for children diagnosed with asymptomatic malrotation, particularly those who are younger and with a low risk of postoperative mortality. The rare occurrence of midgut volvulus does not justify performing a prophylactic Ladd's procedure on most adults with malrotation.

Adolescent↗

Surgical treatment of malrotation after infancy: a population-based study.

PURPOSE: Because malrotation most commonly presents in infants, treatment recommendations for older children (>1 year) have been based on data obtained from small case series. The purpose of this study was to use a large national database to determine the clinical significance of older children presenting with malrotation to develop treatment recommendations for this group. METHODS: Records of children undergoing a Ladd's procedure were identified in the Kids' Inpatient Database, an administrative database that contains all pediatric discharges from 27 states during 2000. Patient characteristics, associated diagnoses, operations performed, and mortality were evaluated. Discharge weighting was used to obtain a national estimate of the number of children older than 1 year treated for malrotation. RESULTS: Two hundred nineteen older children (>1 and <18 years) undergoing a Ladd's procedure were identified in the database. One hundred sixty-four (75%) of these patients were admitted for treatment of malrotation, whereas most of the remaining 55 patients (25%) were admitted for another diagnosis and underwent a Ladd's procedure incidental to another abdominal operation. Seventy-five patients underwent a Ladd's procedure during an emergency admission. Thirty-one patients had volvulus or intestinal ischemia, 7 underwent intestinal resection, and 1 patient died. Based on case weightings, it was estimated that 362 older children underwent a Ladd's procedure for symptoms related to malrotation in 2000 in the United States (5.3 cases per million population). CONCLUSIONS: These findings provide support for performing a Ladd's procedure in older children with incidentally found malrotation to prevent the rare but potentially devastating complications of this anomaly.

Adolescent↗

Characteristics of protein sparing effect of total parenteral nutrition in the surgical infant.

To determine the best formula to prevent protein depletion, 31 surgical infants on intravenous (IV) diet were studied. The study was divided into two phases. Phase I diet included 5% glucose and 1.5% or 2% amino acid infusion; phase II diet consisted of 5% glucose and 1.5% or 2% aminoacid plus 10% Intralipid. In each phase, oxygen consumption, carbon dioxide production, and energy expenditure were determined. The utilization of carbohydrate, fat, and protein was calculated from urinary nitrogen excretion and nonprotein respiratory quotient. The mean caloric intake during phase I and phase II was 62 and 94 kcal/kg/d, respectively. A positive nitrogen balance was obtained in infants receiving fat-free total parenteral nutrition (TPN) with a mean protein intake of 2.6 g/kg/d and a mean energy intake exceeding the energy expenditure by 24%. Infusion of more calories as Intralipid (phase II) caused a significant reduction in protein oxidation, thus protein contribution to the energy expenditure and an increase in protein retention. We conclude that supplementation of low-calorie TPN diets with Intralipid increases protein sparing and is preferable to the administration of very high glucose loads.

Amino Acids↗

Metabolism of intravenous fat emulsion in the surgical newborn.

The metabolism of an intravenous (IV) fat emulsion was investigated by the combination of chemical balance and computerized indirect calorimetry techniques in 21 newborns (birth weight, 3.0 +/- 0.1 kg; mean +/- SE). All babies were appropriate for gestational age and received total parenteral nutrition after a major surgical procedure. The study was divided into two consecutive periods. Phase 1 consisted of infusion of 10% glucose and 2% amino acid solutions for 24 hours, and phase 2 involved the "Intralipid utilization test" (isocaloric and isovolemic infusion of Intralipid 10% for four hours). The caloric intake was 67.1 +/- 1.9 kcal/kg/d during both phases of the study. The resting energy expenditure was 44.8 +/- 1.6 and 46.5 +/- 1.8 kcal/kg/d during phases 1 and 2 respectively. During glucose/amino acid infusion, 12 patients oxidized endogenous fat, and de novo lipogenesis from glucose was observed in nine. During the Intralipid infusion, there was a significant and progressive decrease of carbon dioxide production, respiratory quotient, and carbohydrate utilization (oxidation plus conversion to fat). Net lipogenesis ended and fat utilization significantly increased. By the second hour of Intralipid infusion, 58% of energy expenditure was derived from fat oxidation. The drop in carbon dioxide production correlated positively with the decrease in carbohydrate utilization (r = .07; P less than .001). During the third and fourth hours of phase 2, the percentage of fat utilized was negatively correlated with the amount of fat given (r = -.07; P less than .01). The surgical neonate showed rapid metabolic adaptation to Intralipid infusion.(ABSTRACT TRUNCATED AT 250 WORDS)

Dietary Carbohydrates↗