Surgical Necrotizing Enterocolitis and Spontaneous Intestinal Perforation Lead to Severe Growth Failure in Infants.

OBJECTIVE: We aimed to determine the incidence of growth failure in infants with necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP) and whether initial laparotomy versus peritoneal drainage (PD) impacted the likelihood of growth failure. SUMMARY BACKGROUND DATA: Infants with surgical NEC and SIP have high mortality, and most have neurodevelopmental impairment and poor growth. Existing literature on growth outcomes for these infants is limited. METHODS: This is a preplanned secondary study of the Necrotizing Enterocolitis Surgery Trial dataset. The primary outcome was growth failure (Z-score for weight <-2.0) at 18 to 22 months. We used logistic regression, including diagnosis and treatment, as covariates. Secondary outcomes were analyzed using the Fisher exact or Pearson χ2 test for categorical variables and the Wilcoxon rank sum test or one-way ANOVA for continuous variables. RESULTS: Among 217 survivors, 207 infants (95%) had primary outcome data. Growth failure at 18 to 22 months occurred in 24/50 (48%) of NEC infants versus 65/157 (42%) SIP (P=0.4). The mean weight-for-age Z-score at 18 to 22 months in NEC infants was -2.05±0.99 versus -1.84±1.09 SIP (P=0.2), and the predicted mean weight-for-age Z-score SIP (Beta -0.27; 95% CI: -0.53, -0.01; P=0.041). Median declines in weight-for-age Z-score between birth and 18 to 22 months were significant in all infants but most severe (>2) in NEC infants (P=0.2). CONCLUSIONS: This first ever prospective study of growth outcomes in infants with surgical NEC or SIP demonstrates that growth failure is very common, especially in infants with NEC, and persists at 18-22 months.

Initial Laparotomy Versus Peritoneal Drainage in Extremely Low Birthweight Infants With Surgical Necrotizing Enterocolitis or Isolated Intestinal Perforation: A Multicenter Randomized Clinical Trial.

OBJECTIVE: The aim of this study was to determine which initial surgical treatment results in the lowest rate of death or neurodevelopmental impairment (NDI) in premature infants with necrotizing enterocolitis (NEC) or isolated intestinal perforation (IP). SUMMARY BACKGROUND DATA: The impact of initial laparotomy versus peritoneal drainage for NEC or IP on the rate of death or NDI in extremely low birth weight infants is unknown. METHODS: We conducted the largest feasible randomized trial in 20 US centers, comparing initial laparotomy versus peritoneal drainage. The primary outcome was a composite of death or NDI at 18 to 22 months corrected age, analyzed using prespecified frequentist and Bayesian approaches. RESULTS: Of 992 eligible infants, 310 were randomized and 96% had primary outcome assessed. Death or NDI occurred in 69% of infants in the laparotomy group versus 70% with drainage [adjusted relative risk (aRR) 1.0; 95% confidence interval (CI): 0.87-1.14]. A preplanned analysis identified an interaction between preoperative diagnosis and treatment group (P = 0.03). With a preoperative diagnosis of NEC, death or NDI occurred in 69% after laparotomy versus 85% with drainage (aRR 0.81; 95% CI: 0.64-1.04). The Bayesian posterior probability that laparotomy was beneficial (risk difference <0) for a preoperative diagnosis of NEC was 97%. For preoperative diagnosis of IP, death or NDI occurred in 69% after laparotomy versus 63% with drainage (aRR, 1.11; 95% CI: 0.95-1.31); Bayesian probability of benefit with laparotomy = 18%. CONCLUSIONS: There was no overall difference in death or NDI rates at 18 to 22 months corrected age between initial laparotomy versus drainage. However, the preoperative diagnosis of NEC or IP modified the impact of initial treatment.

Bariatric Surgery Needs a Seat at the Children's Table: Bridging the Perception and Reality of the Role of Bariatric Surgery in the Treatment of Obesity in Adolescents.

The long-term morbidity of obesity in adolescents is well recognized nationally and represents a major health concern for the population of the near future. Traditional medical management of obesity focuses on addressing behavioral modification, dietary and exercise programs, and, to a lesser degree, pharmaceuticals. Although these strategies are relatively effective, they suffer from the lack of sustained benefit, a high relapse rate, and, in case of pharmacotherapy, potentially dangerous adverse effects. Bariatric surgery in adolescents has often been characterized as a risky intervention with unknown long-term benefits. However, recent data establish that a sustained, clinically meaningful effect on weight loss, as well as a reduction in chronic morbidities related to obesity, can be achieved. The role of bariatric surgery as an accepted adjunctive strategy in the treatment of obesity in adolescents is becoming more recognized; however, a number of barriers exist that prevent the timely evaluation of adolescents with obesity for potential surgical intervention. We examine these barriers in light of recent advancements to help better define the role of bariatric surgery in the treatment of obesity in adolescent population.

Overfeeding-associated hyperglycemia and injury-response homeostasis in critically ill neonates.

BACKGROUND: Injury severity induces a proportionate acute metabolic stress response, associated with increased risk of hyperglycemia. We hypothesized that excess caloric delivery (overfeeding) during high stress states would increase hyperglycemia and disrupt response homeostasis. METHODS: Gestational age, daily weight, total daily caloric intake, serum C-reactive protein (CRP), prealbumin, and blood glucose concentrations in all acutely-injured premature NICU infants requiring TPN over the past 3years were reviewed. Injury severity was based on CRP and patients were divided into high (CRP ≥50mg/L) versus low (CRP <50mg/L) stress groups. Glycemic variability was used to measure disruption of homeostasis. RESULTS: Overall sample included N=563 patient days (37 patients; 42 episodes). High stress group pre-albumin levels negatively correlated with CRP levels (R=-0.62, p<0.005). A test of equal variance demonstrated significantly increased high stress glycemic variability (Ha:ratio>1, Pr(F>f)=0.0353). When high stress patients were separated into high caloric intake (≥70kg/kcal/day) versus low caloric intake (<70kg/kcal/day), maximum serum glucose levels were significantly higher with overfeeding (230.33±55.81 vs. 135.71±37.97mg/dL, p<0.004). CONCLUSION: Higher injury severity induces increased disruption of response homeostasis in critically ill neonates. TPN-associated overfeeding worsens injury-related hyperglycemia in more severely injured infants. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level II.

Vascular access for home intravenous therapy in children.

This review article describes the vascular access devices available for long-term vascular access in children, describes the procedures for implanting them and the sites available for this purpose, discusses the complications associated with catheter placement and how to avoid them, and compares the various catheter systems in terms of their advantages and disadvantages.

A Unique Case of Mediastinal Teratoma with Mature Pancreatic Tissue, Nesidioblastosis, and Aberrant Islet Differentiation: a Case Report and Literature Review.

Mediastinal teratomas with elements of mature pancreatic tissue are rare. Only a very few cases of pancreatic tissue with nesidioblastosis in teratoma have been reported. Here, we report a case of a 12-year-old male who presented with pleural effusion and was revealed to have a large anterior mediastinal mass. Biopsy of the mass revealed benign mature teratoma. After biopsy, the teratoma ruptured into the right thoracic cavity. It was then excised and sent to pathology for further evaluation. Preoperatively, there was no evidence of hyperinsulinemia or hypoglycemia. Postoperatively, there was no change in blood glucose levels. Histologically, the mass showed large areas of mature pancreatic tissue flanking a small intestine-like structure. Numerous endocrine cell islets, poorly defined groups of neuroendocrine cells and ductular-insular complexes characteristic of nesidioblastosis were dispersed in the exocrine pancreatic parenchyma. In addition, other parts of the tumor containing keratinizing squamous epithelium with cutaneous adnexal glands, small intestine, and bronchus including cartilage and respiratory epithelium were observed. Some islets contained two or more cell types while others were monophenotypic. Immunohistochemical staining showed pronounced expression of pancreatic polypeptide, moderate expression of somatostatin and insulin and nearly complete absence of glucagon-containing cells. The selective deletion of glucagon might hold clues to an important regulatory mechanism in pancreatic development.

Regulation of the cellular and physiological effects of glutamine.

Glutamine is the most abundant amino acid in humans and possesses many functions in the body. It is the major transporter of amino-nitrogen between cells and an important fuel source for rapidly dividing cells such as cells of the immune and gastrointestinal systems. It is important in the synthesis of nucleic acids, glutathione, citrulline, arginine, gamma aminobutyric acid, and glucose. It is important for growth, gastrointestinal integrity, acid-base homeostasis, and optimal immune function. The regulation of glutamine levels in cells via glutaminase and glutamine synthetase is discussed. The cellular and physiologic effects of glutamine upon the central nervous system, gastrointestinal function, during metabolic support, and following tissue injury and critical illness is also discussed.

The role of insulin-like growth factor I, growth hormone, and plasma proteins in surgical outcome of children with congenital heart disease.

OBJECTIVE: To evaluate postoperative serum concentrations of growth hormone (GH), insulin-like growth factor I (IGF-I), C-reactive protein (CRP), and prealbumin as predictors of clinical outcome as defined by the incidence of 30-day postoperative mortality, the postoperative length of pediatric intensive care unit (PICU) stay, and the risk of postoperative infection in infants of high surgical risk undergoing operative correction of congenital cardiac defects. DESIGN: Prospective, observational study. SETTING: PICU of a university hospital. PATIENTS: A high surgical risk group of 36 children admitted for elective cardiac surgery. INTERVENTION: Measures of serum levels of IGF-I, basal GH, prealbumin, and CRP. These parameters were followed from the hospital admission until the discharge from the PICU at specific time points: preoperative and on the second, fifth, and tenth postoperative days. MEASUREMENTS AND MAIN RESULTS: Surgical stress response was marked by an increase of GH and CRP levels and a fall in prealbumin levels on the second postoperative day. Prealbumin, CRP, and GH returned to preoperative levels on average 10 days following surgery; the values of IGF-I, which had decreased on the fifth day, remained below those values observed before the surgery. Patients whose PICU stay was 10 days. The sustained high CRP (>/= 8.4 mg/dL, p <.05) and GH (>/= 66 mIU/L, p <.03) values on the fifth day were associated with increased mortality in contrast with patients in whom the values were returning to preoperative levels. CONCLUSIONS: Serial monitoring of serum GH, IGF-I, CRP, and prealbumin levels may be useful as a means to a) stratify the acute metabolic response to surgically induced injury insult and b) predict clinical outcome as defined by the length of stay in the PICU and the likelihood of 30-day survival following open-heart surgery in infants.

Bile decompression in children with histopathological evidence of pre-existing liver cirrhosis.

Although it is agreed upon by most that adequate and timely bile decompression can preserve or even improve existing liver function much debate centers on whether pre-existing liver cirrhosis can also be reversed. To help answer this question we analyzed data on 47 children with choledochal cyst disease (CD) who underwent simultaneous liver biopsy during bile decompression surgery. We collected data on two groups of children with CD spanning two different time periods: January 1985 through November 1994 (Group A) and June 1995 through November 1999 (Group B). In Group A 37 children (16 boys and 21 girls ages 5 days to 10 years) underwent simultaneous liver biopsy during elective definitive surgery for CD. In Group B ten children (five boys and five girls age one month to 7 years) underwent liver biopsy twice: first during initial cyst decompression for acute obstruction and second during elective definitive surgery after resolution of acute disease. Degree of liver cirrhosis was based on a modified World Health Organization classification system (0-IV). In Group A 15/37 (40.5%) had significant liver cirrhosis at time of biopsy (III or IV) with altered liver function in all cases; eight of nine had normal liver function on follow-up, six were lost to follow-up. In Group B seven of ten (70%) had less liver cirrhosis on pathology at second operation with three unchanged; nine of ten (90%) regained normal liver function. We conclude that bile duct obstruction is the main cause of liver cirrhosis in children with CD. Adequate and timely bile decompression can restore normal liver function and even reverse severe cirrhosis.

Primary insertion of a silastic spring-loaded silo for gastroschisis.

Gastroschisis is traditionally managed by emergency primary closure, with a temporary silo reserved for large defects unable to be closed primarily. We recently have begun primary Silastic (Dow Coming, Midland, MI) spring-loaded silo (SLS) closure followed by elective closure and report our preliminary experience. A total of 15 infants (weight range, 2.1-13.5 kg) at 2 different institutions were treated by SC by 3 different surgeons between 1998 and 2002. A 3-, 4-, or 5-cm (ring diameter) silo was used depending on size of abdominal wall defect. Elective closure was performed in the operating room or at the bedside. Surgical parameters assessed included success of SLS, peak inspiratory pressures (PIPs) pre- and post-SLS closure, total time of staged closure with SLS, time to full feedings, and intra- and postoperative complications. Fifteen of 15 infants were successfully treated by SLS closure followed by elective closure. Two of 15 (13.3%) experienced temporary dislodgement of the silo prior to permanent closure. In both cases, the silo was safely reinserted at the bedside. Comparison of PIP values measured at various stages of SLS closure revealed no significant difference (P > 0.05). Mean times to final fascial closure (3.7 days) and full enteral feedings (22 days) were similar to historical controls obtained from the surgical literature. In 1 case where there was associated intestinal atresia, SLS closure was effective in permitting concomitant elective closure and re-establishment of bowel continuity. All children are alive and well at the time of this report. SLS closure permits safe, gentle, and gradual reduction of the exposed viscera leading to successful permanent abdominal wall closure. Respiratory embarrassment and hemodynamic instability associated with emergent (primary) closure of large abdominal wall defects can thus be avoided.

Thoracoscopic thymectomy in children with myasthenia gravis.

Although conservative medical management is the mainstay in the treatment of myasthenia gravis (MG), severest forms of the disease often require surgical thymectomy. Thoracoscopic thymectomy (TT) represents a minimally invasive alternative to traditional thymectomy via sternotomy. We present our preliminary experience with TT as definitive treatment for severe forms of MG. The charts of 5 children (4 girls and 1 boy; age range, 11-17 years) who underwent TT for MG were retrospectively reviewed. TT was typically performed via left thoracoscopy using 4- or 5-mm ports with 1 of the ports enlarged at the end of the procedure for specimen retrieval. Thymic veins were identified and ligated with surgical clips in all cases. Surgical parameters assessed were the following: operating time, intra- and postoperative complications, length of postoperative stay, and resolution of symptoms. Follow-up ranged from 6 months to 2 years. All 5 TTs were successfully completed. In 1 case, right-sided thoracoscopy was added to ensure complete gland excision. Surgical pathology in all cases demonstrated complete excision. Mean operating time was 121 minutes (range 88 minutes to 188 minutes). There were no intra- or postoperative complications. Length of postoperative stay averaged 1.6 days (range, 1 to 3 days). Four of 5 (80%) had clear resolution of symptoms with 1 showing minimal resolution at 6 months. Thoracoscopic thymectomy is a safe and potentially attractive alternative to traditional thymectomy via median sternotomy in severe forms of myasthenia gravis. Complete thymectomy, the goal of traditional surgical treatment for myasthenia gravis, can effectively by achieved via this minimally invasive technique.

Minimally invasive surgery for pediatric solid neoplasms.

The role of minimally invasive surgery (MIS) in children with solid neoplasms is slowly evolving. MIS appears to be an ideal way to obtain diagnostic information (i.e., tissue biopsy) in children with solid neoplasms, but its role as an ablative/curative technique is controversial. We examined the safety, reliability, and outcome of decisions made on the basis of MIS performed in children with solid neoplasms. A total of 28 children (19 boys and nine girls; age range, 14 months to 17 years) with solid neoplasms underwent 29 MIS procedures between July 1, 2000 and June 30, 2002. Complications, biopsy results, and outcomes were reviewed. Successful ablation via MIS was defined as clear microscopic margins on permanent pathology and no evidence of remnant disease on follow-up diagnostic radiological examination. There were 20 thoracoscopic and nine laparoscopic procedures. Laparoscopy included purely diagnostic without tissue biopsy or simply determination of resectability (two), incisional biopsy (two), and excisional biopsy (five; two adrenalectomy and three oophorectomy). Thoracoscopy included 15 lung biopsies and five biopsies of mediastinal masses. Diagnostic accuracy was 100 per cent in all cases. MIS as an ablative technique was successful in 10 of 10 cases. No children were found retrospectively to have been inadequately treated via MIS. We conclude that MIS can be used safely and successfully to diagnose children with suspicious solid neoplasms. Furthermore MIS may have a role as an ablative/curative technique in carefully selected circumstances.

Are CT scans obtained at referring institutions justified prior to transfer to a pediatric trauma center?

PURPOSE: To assess whether pediatric trauma patients initially evaluated at referring institutions met Massachusetts statewide trauma field triage criteria for stabilization and immediate transfer to a Pediatric Trauma Center (PTC) without pre-transfer CT imaging. METHODS: A 3-year retrospective cohort study was completed at our level 1 PTC. Patients with CT imaging at referring institutions were classified according to a triage scheme based on Massachusetts statewide trauma field triage criteria. Demographic data and injury profile characteristics were abstracted from patient medical records and our pediatric trauma registry. RESULTS: A total of 262 patients with 413 CT scans were reviewed from 2008 to 2011. 172 patients scanned (66%, 95% CI: 60%, 71%) met criteria for immediate transfer to a pediatric trauma center. Notably, 110 scans (27% of the total performed at referring institutions) were duplicated within four hours upon arrival to our PTC. GCS score <14 (45%) was the most common requirement for transfer, and CT scan of the head was the most frequent scan obtained (53%). CONCLUSION: The majority of pediatric trauma patients were subjected to CT scans at referring institutions despite meeting Massachusetts trauma triage guidelines that call for stabilization and immediate transfer to a pediatric trauma center without any CT imaging.

Management of patients with combined tracheoesophageal fistula, esophageal atresia, and duodenal atresia.

INTRODUCTION: Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge. PRESENTATION OF CASE: Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms. DISCUSSION: While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3-7 days under controlled circumstances. CONCLUSION: A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes.