Journal of Pediatric Surgery

International innovations in pediatric minimally invasive surgery: the Argentine experience

Marcelo Martinez-Ferro — 2012-05-01

Abstract: This is a presentation about innovations in pediatric minimally invasive surgery and a review of the Argentine experience. The most representative are (1) the thoracoscopic treatment of long gap esophageal atresia with novel techniques; (2) the nonsurgical and minimally invasive treatment of chest wall deformities, particularly of pectus carinatum; and (3) the use of magnetic surgical devices in classic laparoscopy and transumbilical surgery.

Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice

2012-05-01

Abstract: Background/Purpose: Survivors of congenital diaphragmatic hernia (CDH) have a high incidence of morbidity. Variability in follow-up practices between institutions may affect perception of disability and prevent population-based outcome analysis.Methods: A survey of follow-up practices at 16 centers within a population-based CDH network was performed. A descriptive analysis of outcomes (minimum 24 months postdischarge) of CDH survivors from the 2 largest centers was performed.Results: The nature of follow-up of CDH survivors was highly variable in 12 of 16 responding centers, ranging from ad hoc, community-based, and pediatrician-sponsored follow-up to a single perinatal center-based multispecialty CDH clinic. Outcomes at 24 to 36 months were reported from the 2 largest centers (n = 44). Among survivors, neurodevelopmental disability was most common (12/44; 27%) followed by gastrointestinal (9; 20.5%), pulmonary (5; 11.4%), musculoskeletal (5; 11.4%), and cardiac (2; 4.5%). Additional surgery was required in 17 patients (38.6%), including recurrent CDH repair in 7 (15.9%). Five patients (11.4%) had hearing loss. Among 41 children with available 24-month data, 32 (78%), 17 (41.5%), and 14 (34.1%) patients had weights below the 50th, 25th, and 3rd percentiles, respectively.Conclusion: Congenital diaphragmatic hernia survivorship is associated with significant disability. Standardization of follow-up practices is essential to enable population-based outcomes analysis.

Chylothorax after congenital diaphragmatic hernia repair: a population-based study

2012-05-01

Abstract: Purpose: Chylothorax is a recognized complication of congenital diaphragmatic hernia (CDH) repair. Our aims were to describe the frequency and outcomes of chylothorax and to seek predictors of chylothorax occurrence within a population-based CDH cohort.Methods: Records for patients with CDH born between 2006 and 2010 were abstracted from a national database and were compared according to presence/absence of postrepair chylothorax. Univariate, and where appropriate, multivariate analyses were performed for group comparisons and chylothorax outcome prediction.Results: Of 243 newborns with CDH surviving to repair, 11 (4.5%) developed a chylothorax. All were managed nonoperatively. Factors predictive of chylothorax outcome on multivariate analysis included need for preoperative transfusion (odds ratio, 13.2; 95% confidence interval, 2.1-83.7; P = .006) and preoperative high-frequency oscillatory ventilation (odds ratio, 7.1; 95% confidence interval, 1.6-31.2; P = .01). Preoperative vasopressor use was significant on univariate analysis only. The groups were comparable for survival, length of stay, and duration of ventilation, but chylothorax patients had prolonged total parenteral nutrition (53 vs 21 days, P = .006) and more central line days (46 vs 24 days, P = .03).Conclusions: Our data suggest that severity of preoperative cardiopulmonary derangement and not anatomical or technical factors predicts chylothorax occurrence after CDH repair.

Alterations of peroxisome proliferator-activated receptor γ and monocyte chemoattractant protein 1 gene expression in the nitrofen-induced hypoplastic lung

2012-05-01

Abstract: Background/Purpose: Peroxisome proliferator-activated receptor γ (PPARγ) plays a key role in normal lung development. Peroxisome proliferator-activated receptor γ messenger RNA (mRNA) is detectable at 18 days of gestation in fetal rat lungs, and levels peak just before birth. Peroxisome proliferator-activated receptor γ agonists are reported to stimulate lung development, whereas inhibition of PPARγ disrupts postnatal lung maturation. Monocyte chemoattractant protein 1 (MCP-1), which is inhibited by PPARγ, is reported to disrupt late lung morphogenesis. This study was designed to investigate the hypothesis that PPARγ expression is downregulated and that MCP-1 expression is upregulated during the late stages of lung development in nitrofen-induced hypoplastic lungs.Methods: Pregnant rats were treated with nitrofen or vehicle on D9. RNA was extracted from fetal lungs (D18 and D21), and relative mRNA expression levels of PPARγ and MCP-1 were determined by reverse transcriptase–polymerase chain reaction. Immunohistochemistry was performed to evaluate protein expression/distribution of PPARγ and MCP-1.Results: Relative mRNA expression levels of PPARγ were significantly downregulated in the nitrofen group compared with controls on D21, whereas MCP-1 levels were upregulated. Immunohistochemical study showed markedly decreased PPARγ and increased MCP-1 immunoreactivity in the nitrofen-induced hypoplastic lungs compared with controls on gestational day 21.Conclusion: Altered pulmonary gene expression of PPARγ and MCP-1 during late gestation may impair lung development and maturation, contributing to pulmonary hypoplasia in the nitrofen-induced congenital diaphragmatic hernia model.

Does earlier lobectomy result in better long-term pulmonary function in children with congenital lung anomalies? A prospective study

Yoko Naito, Alana Beres, Eveline Lapidus-Krol, Felix Ratjen, Jacob C. Langer — 2012-05-01

Abstract: Background: Management of asymptomatic congenital pulmonary airway malformations remains controversial when addressing the optimal timing of surgical resection. Neonatal resection is advocated by some based on the theory that earlier lobectomy results in greater compensatory lung growth. We examined whether age at lobectomy is correlated with better pulmonary outcomes as reflected by pulmonary function and exercise testing.Methods: Patients who had lobectomy for congenital pulmonary airway malformation between 1985 and 2002 were identified and underwent detailed clinical history, physical examination, pulmonary function testing (total lung capacity, forced vital capacity, forced expiratory volume in 1 second), and exercise testing (power, maximal oxygen uptake [Vo2max]).Results: Of 87 patients identified, 47 met the inclusion criteria, and 28 were tested prospectively. Age at the time of lobectomy ranged from 3 days to 56 months. There was no correlation between age at lobectomy and pulmonary function (total lung capacity, P = .408; forced vital capacity, P = .319; forced expiratory volume in 1 second, P = .174) or maximal work capacity (power, P = .280). There was a trend toward lower Vo2max in patients who had undergone lobectomy at an older age (Vo2max, P = .055).Conclusion: Most children undergoing lobectomy have normal long-term pulmonary function. We found no correlation between age at lobectomy and future pulmonary function. Cardiopulmonary exercise testing should be considered in evaluating functional outcome in these patients.

Thoracoscopic vs open resection of congenital lung lesions: a meta-analysis

Ahmed Nasr, Juan Bass — 2012-05-01

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

Pectus carinatum treatment in Canada: current practices

Sherif Emil, Jean-Martin Laberge, David Sigalet, Robert Baird — 2012-05-01

Abstract: Background: Multiple treatment options currently exist for the correction of pectus carinatum (PC). We performed a survey of Canadian pediatric surgeons to define current practices.Methods: All active members of Canadian Association of Paediatric Surgeons were surveyed online during winter 2011 through the Canadian Association of Paediatric Surgeons Web site. The survey assessed multiple facets of PC evaluation and treatment, with particular emphasis on the practice of bracing.Results: Forty-five active members (85%) responded, of whom 32 (71%) currently treat PC. Fifty-three percent of practices are low volume (<5 patients annually). In terms of preferred or most used treatment modality, 69% of surgeons used bracing, 25% performed Ravitch repairs, 3% performed open minimal cartilage resections, and 3% performed reverse Nuss procedures. Of 23 surgeons (72%) who used bracing, 83% used it for most or the patients. Fifty-seven percent judged their bracing results as good or excellent, and 74% felt that most or all patients braced were satisfied; 80% and 88% agreed or strongly agreed that bracing was generally preferable to surgical repair and that bracing should be first line treatment, respectively.Conclusions: Bracing is the preferred treatment for PC by most Canadian pediatric surgeons, despite lack of prospective outcome data. This presents an opportunity for a multicenter prospective study.

An innovative method of pediatric chest wall reconstruction using Surgisis and swinging rib technique

2012-05-01

Abstract: Purpose: Herein, we describe a new surgical approach for chest wall reconstruction using a native supporting rib and Surgisis.Methods: A retrospective review of 3 cases from 2 tertiary pediatric health care centers presenting with chest wall defects in the neonatal period was performed. Perioperative data were collected.Results: Two chest wall deformities were diagnosed at birth (Poland syndrome and cleft sternum). One patient was diagnosed prenatally with a mediastinal mass. The first infant had absent ribs 2 through 9. He underwent chest wall reconstruction at 4 weeks of life because of difficulty weaning from ventilation related to paradoxical breathing. The hamartoma of the second asymptomatic patient was removed at 6 weeks. The third patient's V-shaped sternal defect encompassed through the upper two thirds of the sternum and was repaired at 6 months of age with intraoperative transesophageal echocardiogram monitoring. In all cases, Surgisis (collagen matrix) was used as an onlay patch. In 2 cases, a swinging rib acted supportive. Neither patient had intraoperative complications.Conclusion: Surgisis is useful in pediatric chest wall reconstruction, particularly in combination with swinging ribs. The capacity for adaptation to the child's growth of this approach is crucial. Short-term safety is shown, but long-term assessment is required.

Effects of sodium hydroxide exposure on esophageal epithelial cells in an in vitro ovine model: implications for esophagus tissue engineering

2012-05-01

Abstract: Background: Esophagus tissue engineering holds promises for esophageal replacement after severe caustic injuries. The aim of this study was to determine whether viable esophageal epithelial cells could be isolated from an esophagus exposed to varying concentrations of alkali with regard to number, viability, and morphology during in vitro culture.Methods: Ovine esophagi were exposed to phosphate-buffered saline 2.5%, 15%, or 25% sodium hydroxide (NaOH). The effect of NaOH concentrations on epithelial damage was assessed histologically. Esophageal epithelial cells were then isolated, and cell count and viability were investigated. Finally, cell number, viability, and morphology of esophageal epithelial cells were determined for 24 days of in vitro culture.Results: Histologic analysis showed a progressive destruction of the epithelium proportional to increasing NaOH concentrations. Esophagi treated with phosphate-buffered saline and 2.5% NaOH showed significantly higher viable cell counts after isolation and culture in comparison with those treated with 15% to 5% NaOH.Conclusion: The evidence presented in this study indicates that epithelial biopsies from an esophagus exposed to low concentrations (2.5%) of NaOH will still yield large numbers of viable cells suitable for tissue engineering applications. In cases of exposure to higher concentrations (15%-25%), alternative cell sources for epithelial regeneration, such as stem cells, will be necessary for tissue engineering applications.

An analysis of early nonmortality outcome prediction in esophageal atresia

Abdullah Alshehri, Andrea Lo, Robert Baird — 2012-05-01

Abstract: Background: Advances in care for neonates with esophageal atresia (EA) has improved overall survival rates. Disease-specific prognostic scores for EA assess mortality risk without assessing patient morbidity. We undertook an analysis of these and generic scoring systems evaluating their ability to predict early nonmortality outcomes.Methods: We conducted a retrospective review of all patients with EA at our tertiary care children's hospital. Disease-specific (Spitz, Waterston, and Montreal) and generic prognostic scores (Score for Neonatal Acute Physiology II and Score for Neonatal Acute Physiology–Perinatal Extension) were calculated. Outcomes investigated included mortality and early nonmortality outcomes (leak, stricture, and recurrent fistula). These were assessed individually and as a composite “poor outcome” score. Correlations were sought, and receiver operating characteristic curves were generated.Results: Fifty patients were included for analysis, with 5 deaths (10%) in our series. Eight patients developed a postoperative leak, 18 developed stenosis requiring dilatation, and 2 developed refistulization. Overall, 51% of survivors had a poor composite outcome. Although no prognostic score achieved statistical significance, the generic scores outperformed the disease-specific scores in predicting early nonmortality outcomes.Conclusions: Postoperative morbidity remains common in patients with EA. Disease-specific, preexisting prognostic scoring systems do not delineate surviving patients at risk for early complications and appears to underperform when compared with generic prognostic scores.

Role of endoscopic retrograde cholangiopancreatography in diagnosis and management of congenital choledochal cysts: 28 pediatric cases

2012-05-01

Abstract: Background/purpose: Management of choledochal cysts consists of surgical excision and hepaticojejunal anastomosis. Endoscopic retrograde cholangiopancreatography (ERCP) can be used to resolve complications and to evaluate the biliary tract and pancreatobiliary duct junction. Our aim was to underline the importance of ERCP for optimal management.Methods: From 2005 to 2011, 28 patients were reviewed (21 female, 7 male; mean age, 5.71 years; range, 2-16 years). After imaging, all patients underwent elective ERCP and were referred for surgery.Results: Choledochal cyst was diagnosed at ultrasound and magnetic resonance cholangiopancreatography in all examined patients; common biliopancreatic duct was diagnosed in 3 (20%) of 15 patients at magnetic resonance cholangiopancreatography and in none at ultrasound. Endoscopic retrograde cholangiopancreatography showed choledochal cyst in all patients and common biliopancreatic duct in 19 (68%) of 28 patients. Twelve patients underwent sphincterotomy. All patients underwent surgical extrahepatic biliary tree resection and hepaticojejunal anastomosis. Mean period of hospitalization was 9.5 days (range, 6-13 days). No major complications related to ERCP were observed. Two patients needed postoperative ERCP for complications (pancreatitis during follow-up).Conclusions: In our pediatric experience, ERCP is feasible and safe. It can rule out other possible biliary tract anomalies and help plan the timing and choice of the appropriate surgical procedure.

Reduced aluminum contamination decreases parenteral nutrition associated liver injury

2012-05-01

Abstract: Purpose: Parenteral nutrition–associated cholestasis remains a significant problem, especially for the surgical neonates. Aluminum is a toxic element known to contaminate parenteral nutrition. We hypothesize that parenterally administered aluminum causes liver injury similar to that seen in parenteral nutrition–associated cholestasis.Methods: Twenty 3- to 6-day-old domestic pigs were divided into 5 equal groups. A control group received daily intravenous 0.9% NaCl. Each subject in experimental groups received intravenous aluminum chloride at 1500 μg kg−1 d−1 for 1, 2, 3, or 4 weeks. At the end of the study, blood was sampled for direct bilirubin and total bile acid levels. Liver, bile, and urine were sampled for aluminum content. Liver tissue was imaged by transmission electron microscopy for ultrastructural changes.Results: Transmission electron microscopy revealed marked blunting of bile canaliculi microvilli in all experimental subjects but not the controls. Serum total bile acids correlated with the duration of aluminum exposure. The hepatic aluminum concentration correlated with the duration of aluminum exposure.Conclusions: Parenterally infused aluminum resulted in liver injury as demonstrated by elevated bile acids and by blunting of the bile canaliculi microvilli. These findings are similar to those reported in early parenteral nutrition–associated liver disease.

Food aversion among patients with persistent hyperinsulinemic hypoglycemia of infancy

Saud Al-Shanafey, Hussain AlKhudhur — 2012-05-01

Abstract: Introduction: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of neonatal hypoglycemia. Feeding plays a major role in its management. We evaluated the magnitude of food aversion among our patients with PHHI.Methods: A retrospective medical record review was conducted for patients with PHHI managed during the last 15 years. Demographic and clinical data were retrieved, and descriptive data were generated.Results: Forty-two patients (18 were male and 24 were female) were managed for PHHI over the study period. Thirty-three patients had subtotal pancreatectomy, and 9 were managed medically, with a mean follow-up of 5 years. Nineteen patients (45%) had aversion to food, and 9 of them were developmentally delayed. Of 14 patients assessed by a speech pathologist, 5 had aversive sensory behavior, 4 had dysfunctional pharyngeal swallowing, and 5 had a normal assessment. Ten were managed with oral feeding, 4 had gastrostomy feedings, and 5 were managed with oral and nasogastric tube feeds for various periods.Conclusions: Food aversion is significantly prevalent among patients with PHHI. The presence of food aversion appears to be independent of developmental delay. Aggressive measures to manage food aversion could help in the management of patients with PHHI.

Single-incision laparoscopic splenectomy in children

Robert Bell, Trevor Boswell, Thomas Hui, Wendy Su — 2012-05-01

Abstract: Purpose: Single-incision laparoscopic surgery is being incorporated into the practices of many pediatric surgeons. Its superior cosmetic outcomes have resulted in increased patient- and parent-driven demand for the approach. This article describes a series of single-incision laparoscopic splenectomies (SSs) in children and compares outcomes with a historical series of standard laparoscopic splenectomies (LS).Methods: The medical records of patients who underwent standard splenectomy and SS at our institution between 2007 and 2010 were reviewed. Perioperative data were recorded. Descriptive comparisons were made between the SS and LS groups.Results: Seven patients underwent SS, and 4 underwent LS during the study period. Operative times for the SS group decreased as experience with the procedure accumulated and ultimately approached LS operative times. No conversions to a standard laparoscopic or open approach were required. No complications occurred. Length of stay and postoperative pain were similar in both groups. single-incision laparoscopic splenectomy provided excellent cosmetic outcomes.Conclusions: Single-incision laparoscopic splenectomy is feasible in children. Its main benefit is improved cosmesis. It can be performed without detrimental increases in operative times or patient discomfort. Given an increased demand for the approach, it remains a viable option in children.

Surgery for suspected rotation abnormality: selection of open vs laparoscopic surgery using a rational approach

Marvin Hsiao, Jacob C. Langer — 2012-05-01

Abstract: Background: Laparoscopy is increasingly used for children with suspected rotation abnormalities. However, indications for open and laparoscopic approaches are not well defined. We reviewed our experience with both open and laparoscopic approaches to develop a rational approach to these patients.Methods: Charts of all children undergoing surgery for a suspected rotation abnormality for 10 years were retrospectively reviewed.Results: There were 173 patients. Of 73 neonates presenting with suspected volvulus, 71 underwent initial laparotomy and 2 were converted from initial laparoscopy. Eighty percent underwent Ladd procedure, 64% had volvulus, and 2 died of midgut volvulus. Of 18 neonates presenting without suspected volvulus, 14 underwent initial laparotomy and 4 had a laparoscopic approach with 1 conversion to laparotomy. Seventy-eight percent underwent Ladd procedure, and 22% had volvulus. Of the 82 older patients, 37 underwent laparotomy and 45 had initial laparoscopy, 8 of which were converted. Sixty-seven percent underwent Ladd procedure, and 28% had volvulus. Postoperative complication rate, median time to full diet, and median hospital stay were comparable with those previously reported in the literature.Conclusion: Based on our results, we advocate open surgery for neonates with suspected volvulus. Laparoscopy represents an excellent alternative for older children and for neonates presenting without suspected volvulus.

Associated malformations and the “hidden mortality” of gastroschisis

2012-05-01

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

Evaluation of Surgisis for patch repair of abdominal wall defects in children

Alana Beres, Emily R. Christison-Lagay, Rodrigo L.P. Romao, Jacob C. Langer — 2012-05-01

Abstract: Background: Abdominal wall defects in children are not always amenable to primary repair and may require a patch. The ideal material has yet to be established. We sought to evaluate our experience using the bioabsorbable material Surgisis (Cook Surgical, Bloomington, IN) for abdominal closure.Methods: A retrospective chart review of abdominal wall defects repaired with Surgisis in our institution from 2000 to 2010 was performed. Data extracted included cause of defect, age at operation, possibility of skin coverage, recurrence, length of follow-up, and rate of wound infection.Results: Thirteen patients were identified. Cause of defect was gastroschisis (n = 2), ventral hernia after diaphragmatic hernia repair (n = 2), and omphalocele (n = 9). At median follow-up of 60 months (range, 10-90), 5 (38%) of 13 patients recurred, and 1 patient recurred twice. All recurrences required subsequent patch closure. Six instances of wound infection required antibiotics. None required patch removal. There was a trend toward more frequent recurrence among infants undergoing patch repair (3/4 recurrences in this group) than neonates (1/4 recurrences) or children older than 18 months (1/5 recurrences).Conclusion: Our data suggest that Surgisis is moderately successful in the repair of pediatric abdominal wall defects. We noted a trend toward a higher recurrence rate in infants. Further studies investigating timing of repair and alternative biosynthetic materials are warranted.

EphB2/B3 gene expression is down-regulated during early embryogenesis in the cadmium-induced omphalocele chick model

Takashi Doi, Prem Puri, John Bannigan, Jennifer Thompson — 2012-05-01

Abstract: Purpose: In the chick embryo, the administration of cadmium (Cd) induces omphalocele phenotype. The earliest histologic change in this model is observed in the somite 4 hours (H) post treatment, postulating that disruption of somite development in embryogenesis may cause omphalocele phenotype. EphB2 and EphB3 are involved in many embryonic developmental processes, including somitogenesis. EphB2−/−EphB3−/− double knockouts display omphalocele phenotype. We hypothesized that EphB2/B3 genes are down-regulated in the Cd chick model during the critical period of embryogenesis.Methods: After 60H incubation, chicks were harvested 1H, 4H, and 8H post treatment with saline or Cd and divided into control and Cd groups. Reverse transcriptase–polymerase chain reaction was performed to evaluate gene expression levels of EphB2/B3. Immunofluorescence confocal microscopy was performed to evaluate protein expression/distribution of EphB2/B3.Results: At 4H post treatment, the messenger RNA expression levels of EphB2/B3 were significantly down-regulated in the Cd group compared with controls (P < .05). The intensity of EphB2/B3 immunofluorescence was markedly diminished at 4H in the Cd-treated embryos, whereas strong immunoreactivity was observed in the somite in controls.Conclusion: Downregulation of EphB2/B3 during the narrow window of early embryogenesis may interfere with normal somitogenesis, preventing migration of embryonic body wall ventrally and thus causing omphalocele.

Predictors of failed enema reduction in childhood intussusception

Frankie B. Fike, Vincent E. Mortellaro, George W. Holcomb, Shawn D. St. Peter — 2012-05-01

Abstract: Background: Initial management of intussusception is enema reduction. Data are scarce on predicting which patients are unlikely to have a successful reduction. Therefore, we reviewed our experience to identify factors predictive of enema failure.Methods: A retrospective review of all episodes of intussusception over the past 10 years was conducted. Demographics, presentation variables, colonic extent of intussusceptions, and hospital course were collected. Extent of intussusception was classified as right, transverse, descending, and rectosigmoid. Episodes were grouped as success or failure of enema reduction and compared using the Student t test for continuous variables and χ2 test for dichotomous variables. Significance was P less than .05.Results: We identified 405 episodes of intussusception and 371 attempts at enema reduction. There were 285 successful enema reductions. There was no difference between groups in age; sex; or the presence of emesis, fever, or abdominal mass. The failed enema group was more likely to have had symptoms over 24 hours before presentation (P = .006), bloody diarrhea (P < .001), and lethargy (P < .001). The chance of success diminished with colonic extent (right, 88%; transverse, 73%; left, 43%; colorectal, 29%; P < .001).Conclusion: Predictors of failed enema reduction of intussusception include presence of symptoms over 24 hours, diarrhea, lethargy, and distal extent of intussusception.

Antibiotics administration before enema reduction of intussusception: is it necessary?

2012-05-01

Abstract: Background: Some centers advocate using antibiotics before enema reduction to prevent septic complications. Our objective was to determine whether using antibiotics before reduction provided any improvement in outcomes.Methods: With institutional review board approval, patients from 2 centers were compared: 1 where antibiotics were administered, and one where they were not. This retrospective cohort study from January 2005 to December 2010 evaluated demographic data, episodes of postreduction fever, hospital stay, and analgesia requirements.Results: One hundred eighteen patients were identified: 83 males (70.3%) and 35 females (29.7%). The median age was 24 months (range, 1-180). Fifty-six patients (57.7%) received antibiotics, whereas 41 (42.7%) did not. Twenty-one patients (17.8%) had missing data and were excluded. The incidence of fever postreduction was not statistically different between groups: 12.8% for those who received antibiotics vs 17.9% for those who did not (P = .7367). No adverse antibiotic reactions were reported. Average time to oral feeds was 7.3 vs 10.6 hours (P = .06), and the length of stay was 1.7 vs 1.4 days (P = .07).Conclusion: Although antibiotics are administered routinely in some centers, they appear of little value. Financial costs and potential adverse reactions must be considered. Further prospective evaluation is being conducted using a larger sample size to confirm these results.

Five-year outcomes after serial transverse enteroplasty in children with short bowel syndrome

Carol Oliveira, Nicole de Silva, Paul W. Wales — 2012-05-01

Abstract: Aim: The aim of this study is to report 5-year outcomes in patients who have received serial transverse enteroplasty (STEP) using clinical and biochemical assessments of intestinal function.Methods: Thirty-one STEP procedures have been performed at our institution. Twelve patients who received STEP before January 2005 were analyzed. Clinical and biochemical outcomes were performed pre-STEP and post-STEP on an annual basis. Paired t tests were used to compare post-STEP outcomes to pre-STEP values (P < .05 was significant).Main Results: There were 12 patients (3 females; median age, 5.5 months). Serial transverse enteroplasty resulted in mean increase in length of dilated bowel segment of 89% ± 26% and increase in total small bowel length of 46% ± 40%. Two patients received liver-intestinal transplants post-STEP (at 4 and 5 months), and 2 patients died of liver failure at 3 and 8 months. The remaining 8 of 12 patients all show stable intestinal absorptive capacity (data not shown). Of 8 patients, 7 were weaned off parenteral nutrition by 4 years. No patient has required repeat STEP or bowel tapering. Two patients developed staple line leak, and 1 patient developed gastrointestinal bleed from staple line ulcers.Conclusion: This report represents the largest series of STEP patients followed up beyond 5 years. Long-term clinical, biochemical, and radiologic outcomes appear to be robust.

The next step? Use of tissue fusion technology to perform the serial transverse enteroplasty—proof of principle

2012-05-01

Abstract: Background: Serial transverse enteroplasty (STEP) is an established procedure for intestinal lengthening and tapering. A gastrointestinal linear stapler is used to taper the bowel sequentially. We report preliminary experience with tissue fusion technology to perform STEP in a porcine model.Methods: Four weaned male pigs (mean age, 4 ± 0 weeks; mean weight, 6.8 ± 0.1 kg) first underwent a 60-cm reversed intestinal segment followed by a STEP 4 to 6 weeks later. The LigaSure Impact (Covidien, Valleylab, Tyco Healthcare Group LP, Boulder, CO) was used to perform the procedure. Animals were fed on postoperative day 2 and terminated 1 week later. Morphometric data were collected, and intestinal tissue was obtained for histology.Results: Mean bowel caliber of 5.1 ± 0.5 cm was tapered to 1.8 ± 0.3 cm post-STEP with a mean increase in the length of the dilated segment of 82% ± 20%. All animals tolerated enteral feeding, and all survived to termination on day 7. Histologic evaluation revealed the zone of tissue fusion to be 7.1 ± 1.5 mm. Masson trichrome, hematoxylin and eosin, and polarized picrosirius red stains demonstrated that the fusion zone was well healed with overlying granulation tissue.Conclusion: This is the first report of the successful application of radiofrequency energy to perform the STEP procedure in animals. Although further evaluation is required, tissue fusion devices may eventually provide an alternative to the linear stapler for the STEP procedure.

Strictureplasty and intestinal resection: different options in complicated pediatric-onset Crohn disease

2012-05-01

Abstract: Background/Purpose: Surgical resection or strictureplasty (SP) are different options for intestinal Crohn disease (CD) strictures. The aim of this article is evaluation of long-term outcome of SP and resection.Methods: From 1996 to 2011, 39 patients (23 male, 16 female) with symptomatic ileal and ileocolonic CD strictures resistant to medical/nutritional therapy and treated with surgery in 2 different surgical units were reviewed. The mean age at diagnosis was 11.82 years (range, 4-17 years). Mean age at surgery was 15.94 years (range, 4-24 years). Mean follow-up was 6.88 years (range, 0.5-15 years). Patients underwent resection (group A) or different SP techniques (group B).Results: Twenty patients underwent intestinal resection (ileal or ileocolonic resection), and 19 patients underwent SP (jejunal, ileal, or ileocolic). Early postsurgical complications were observed in 2 patients of group A. Follow-up of group A patients revealed that 1 patient needed emergency treatment after 8 months surgery because of adhesions and 1 patient developed recurrence treated with medical therapy. In the follow-up group B, 3 patients experienced disease recurrence, 2 of them at the site of previous surgery.Conclusions: At long-term follow-up, no significant difference in relapsing rate was observed between the 2 groups. Strictureplasty and resection represent an effective treatment of pediatric CD strictures. Strictureplasty could represent the first option for intestinal preservation.

Surgical management of gastrointestinal basidiobolomycosis in pediatric patients

Saud Al-Shanafey, Faten AlRobean, Ibrahim Bin Hussain — 2012-05-01

Abstract: Introduction: Gastrointestinal basidiobolomycosis (GIB) is a rare fungal disease. We reviewed our experience with this disease over the last 10 years, with special emphasis on the surgical aspects.Methods: A retrospective chart review was conducted for pediatric patients with GIB who were managed at our institution over the last 10 years. Demographic, clinical, and follow-up data were collected, and descriptive data were generated.Results: Nine patients with a median age of 7 years were managed for GIB over the study period. Six patients were managed surgically. Four had colonic and liver involvement, 1 had a left hepatic lesion, and 1 had a porta hepatic mass. One patient had multiple liver lesions and was managed medically, and 2 patients had disseminated disease and died shortly after presentation. Tissue biopsies confirmed the diagnosis, and all received antifungal treatment. Patients were followed up for a median of 6 years. All live patients are free of disease, and 4 are still on antifungal medications.Conclusions: Gastrointestinal basidiobolomycosis is an aggressive disease that requires early surgical intervention to avoid complications and hasten disease eradication. Long-term follow-up is warranted, given the significant potential for complications.

The impact of variation in access to care on the management of Hirschsprung disease

2012-05-01

Abstract: Introduction: Variation in access to care has a significant impact on the disease management process and outcomes. Variable access to care might have similar effects on the management of Hirschsprung disease (HD). However, such variation has not been highlighted.Materials and Methods: All patients referred to 3 academic centers (ACs) with HD were reviewed. Patient presentations, referral patterns, timing, and type of surgical intervention were compared between patients born in AC and those born in non-AC. Babies born with major congenital anomalies (MCAs) or total colonic HD were excluded.Results: Between 1998 and 2011, 129 patients were identified. After excluding 30 patients, 99 were split into 20 inborn patients (AC) and 79 outborn patients. Outborn patients more often presented with constipation (95% vs 65%, P = .001), whereas inborn patients presented with feeding intolerance or vomiting (75% vs 39%, P = .004). Outborn patients were diagnosed and had their pull-through (PT) at an older median age (in days) of 186 (1-2621) vs 4.5 (1-451) (P = .001) and 345 (11-2757) vs 92 (3-928) (P = .001), respectively. Moreover, inborn patients were more likely to undergo primary PT (75% vs 46%, P = .02) and avoid bowel diversion (95% vs 66%, P = .02).Conclusion: Variation in access to care can have a significant impact on the quality of care delivery in HD. Limited access to AC is associated with staged PT in addition to delay in the diagnosis and management.

Three-dimensional endoanal ultrasound and anorectal manometry in children with anorectal malformations: new discoveries

2012-05-01

Abstract: Background/Purpose: Fecal incontinence and constipation are common problems in follow-up of anorectal malformations (ARMs). We evaluated the anal sphincters using the 3-dimensional endoanal ultrasonography (3D-EAUS) and the anorectal manometry after ARMs repair.Methods: Seventeen patients, divided into 3 groups according to Wingspread classification, underwent anorectal manometry and 3D-EAUS. Clinical, manometric, and endosonographic scoring systems were used.Results: The average anal resting pressure (aARP) was significantly higher in low ARMs than in intermediate and high ARMs. The anal squeeze pressure was not statistically different between the 3 groups. Three-dimensional EAUS visualized internal anal sphincter (IAS) disruptions in 7 of 17 patients and absence of IAS in 6 of 17 children with high ARMs. Scars of the external anal sphincter were localized in low ARMs and generalized in the other groups. In the case of IAS disruption with aARP greater than 20 mm Hg, fecal incontinence and constipation improved with biofeedback and/or laxatives, whereas daily enemas were necessary in absence of IAS with aARP less than 20 mm Hg. Statistical correlation was observed between endosonographic and manometric findings and clinical outcomes.Conclusions: Lesions of the anal sphincter are common in ARMs. Three-dimensional EAUS and anorectal manometry ensure a complete assessment of the anal sphincter and could provide useful information to define the most appropriate treatments to improve the quality of life.

Parent health literacy and satisfaction with plain language education materials in a pediatric surgery outpatient clinic: a pilot study

2012-05-01

Abstract: Background: Although significant, the issue of health literacy (HL) among parents attending pediatric surgery outpatient clinics has received little attention.Purpose: The objectives of this study are to determine the HL skills of parents attending the pediatric surgery outpatient clinic at McMaster Children's Hospital and to describe parent satisfaction with plain language materials.Methods: This cross-sectional study was conducted at the pediatric surgery outpatient clinic at McMaster Children's Hospital. Using convenience sampling for 4 months, parents were recruited and interviewed regarding their demographic status. The Newest Vital Sign tool was used to assess HL. Feedback on the plain language education material was received.Results: Seventy-nine individuals were recruited, with a recruitment rate of 62%. Seventy-one percent had adequate HL. English as a first language and Canada as the place of birth were significantly correlated with adequate HL (r = 0.367, P < .001; r = 0.259, P < .05). Parents reported satisfaction with the plain language material, regardless of their HL level.Conclusion: Twenty-nine percent of parents showed inadequate HL, likely an underestimate owing to study limitations. Parents expressed satisfaction with the plain language material, emphasizing the need for clear, effective communication with patients and families. Future directions include evaluating staff knowledge of a universal precautions approach to health communication and the accessibility of plain language materials.

The intrathoracic kidney: should we fix it?

James J. Murphy, Gabriel Altit, Siham Zerhouni — 2012-05-01

Abstract: Background: Intrathoracic kidney is a rare congenital anomaly, with only 13 cases reported in the pediatric age group over the past 25 years. The relevant literature is limited to individual case reports or small case series with no follow-up data. Both operative and nonoperative management has been advocated. We report our experience in the management of children with an intrathoracic kidney as well as the efficacy of nonoperative management in select patients.Methods: Five cases of intrathoracic kidney were collected prospectively since 1992 and carefully followed up long term.Results: Two children presented with acute respiratory distress and underwent right diaphragmatic hernia repair and nephropexy. Incidental diagnosis of a left intrathoracic kidney was made in 3 children. Long-term follow-up has demonstrated normal function and development of these kidneys in all 5 children with no late bowel herniation in the nonoperative group.Conclusion: Intrathoracic kidney associated with bowel in the chest should undergo standard repair and nephropexy. An isolated intrathoracic kidney without evidence of bowel herniation can safely be observed. This is the largest pediatric series of intrathoracic kidney as well as the first to document the efficacy of nonoperative management with long-term follow-up.

Evaluation of a new pediatric intraosseous needle insertion device for low-resource settings

2012-05-01

Abstract: Background and Purpose: The Near Needle Holder (NNH) (Near Manufacturing, Camrose, Alberta, Canada) is a reusable tool to introduce a standard hollow needle for pediatric intraosseous (IO) infusion. We compared the NNH to the Cook Dieckmann (Cook Critical Care, Bloomington, IN) manual IO needle in a simulation setting.Methods: Study subjects were 32 physicians, nurses, and medical students participating in a trauma course in Guyana. After watching a training video and practicing under supervision, subjects were observed inserting each device into a pediatric leg model using a randomized crossover design. Outcome measures were time to successful insertion, technical complications, ease of use, and safety of each device.Results: The mean time for IO insertion (32 ± 13 seconds) was similar for both devices (P = .92). Subjects rated the NNH device equivalent in ease of use to the Cook IO needle but slightly lower in perceived safety to the user.Conclusions: After training, all subjects successfully inserted the NNH IO device in a simulation environment, and most rated it as easy to use and safe. The NNH is a significant advance because IO needles are often not available in emergency departments in developing countries. Further studies are needed to evaluate clinical effectiveness of the NNH.

Increased use of enoxaparin in pediatric trauma patients

Johanna R. Askegard-Giesmann, Sarah H. O'Brien, Wei Wang, Brian D. Kenney — 2012-05-01

Abstract: Purpose: Venous thromboembolism (VTE) in pediatric trauma patients has been reported from 0.7 to 4.2 patients per 1000 admissions. There are no clear guidelines for prophylactic anticoagulation in children. The purpose of this study was to examine the use of enoxaparin in pediatric trauma patients.Methods: The Pediatric Health Information System database was queried from 2001 to 2008 for patients 0 to 18 years with a primary diagnosis of trauma based on International Classification of Diseases, Ninth Revision, codes. Patients who received enoxaparin and/or diagnosed with VTE were identified using pharmacy and International Classification of Diseases, Ninth Revision, codes. Logistic regression was used to identify patient and hospital characteristics associated with VTE and enoxaparin use.Results: Among 260,078 pediatric trauma patients, 3195 were prescribed enoxaparin (1.23%), 2915 (1.12%) of whom were given enoxaparin without a diagnosis of VTE. The incidence of VTE remained stable (0.23%-0.28%), whereas the use of enoxaparin increased (0.75%-1.54%), especially in patients without VTE (0.65%-1.43%). Venous thromboembolism was significantly associated with pelvic fractures, intensive care unit stay, and central venous catheters (P = .017, P < .001, P < .001).Conclusions: Despite a stable VTE incidence, the use of enoxaparin significantly increased in pediatric trauma patients, suggesting that use of pharmacologic thromboprophylaxis is increasing in pediatric trauma centers.

Reduction of radiation exposure in pediatric patients with trauma: cephalic stabilization improves adequacy of lateral cervical spine radiographs

2012-05-01

Abstract: Introduction: Plain radiographs continue to play a role in cervical spine clearance. Inadequate radiographs commonly necessitate repeat x-rays or computed tomography imaging (10× radiation dose). We have used the technique of cephalic stabilization (CS) to improve the results of plain radiographs. Cephalic stabilization lateral radiographs are obtained, with one assistant applying traction to the arms while another placing fingers in the patient's ears and stabilizing the head. This study tests the hypothesis that CS improves visualization of the cervicothoracic junction during lateral cervical spine radiographs.Methods: A 2-year review of institutional pediatric trauma registry identified 46 patients with CS, matched 1:3 with controls. Randomized lateral radiographs were evaluated independently by 2 pediatric radiologists to determine adequate visualization of the craniocervical and cervicothoracic junctions. Reviewers were blinded to CS through image cropping.Results: The proportion of adequate visualization of the cervicothoracic junction was 0.85 for cases with stabilization and 0.60 for controls. Odds of obtaining adequate visualization with stabilization are 3.8 times those without stabilization (P = .001) and were even greater for patients younger than 13 years.Conclusions: Cephalic stabilization improves visualization of the cervicothoracic junction in lateral cervical spine radiographs and can reduce radiation exposure in patients who would otherwise require further imaging.

Television-related injuries in children—the British Columbia experience

Jessica Mills, Jeremy Grushka, Sonia Butterworth — 2012-05-01

Abstract: Purpose: In Canada, mortality from falling televisions (TVs) is the 15th leading cause of childhood death owing to injury. Frequency, characteristics, and outcomes of TV childhood injuries were examined to determine any at risk populations.Methods: All TV-related traumas at a tertiary children's hospital from 1997 to 2011 were identified using the Canadian Hospitals Injury Reporting and Prevention Program database and the hospital's trauma database. Charts of admitted patients were reviewed.Results: Analysis of 179 injuries (10-24 per year) revealed a high frequency of injury in the home and a preponderance of head and neck injuries. Toddlers were the most commonly injured age group. Eleven admitted patients were identified; 6 were admitted to intensive care unit with significant head injuries, 2 of whom required surgery. More than half of admitted patients were First Nations or recent immigrants. The length of stay for a ward vs intensive care unit admission was 1.3 days (range, <1-2 days) compared with 7.6 days (range, <1-20 days), respectively. One child had residual deficits requiring rehabilitation, but there were no mortalities.Conclusion: Injury severity appeared higher in patients from First Nations and recent immigrant families. Television injury would likely have been prevented by a securing device or support.

Vinyl glove ingestion in children: a word of caution

Gustavo Stringel, Michael Parker, Erin McCoy — 2012-05-01

Abstract: Background: Vinyl gloves when ingested will harden and develop sharp edges producing gastric bezoars; bowel obstruction; and, ultimately, perforation. We report 4 children with complications secondary to vinyl glove ingestion who required surgical intervention.Methods: A 3-year-old boy, a 13-year-old adolescent girl with Down syndrome, a 14-year-old adolescent girl, and a 15-year-old adolescent boy presented with bowel obstruction secondary to a bezoar caused by a vinyl glove. The adolescent girl with Down syndrome presented again at age 17 years with a large vinyl glove gastric bezoar. Three of the children had mental retardation, and 1 was a victim of child abuse. Three had laparoscopic-assisted removal of the vinyl glove bezoar, and 1 had laparotomy.Results: The 4 children recovered uneventfully. Two of the patients had unsuspected intestinal perforation. The caretakers denied awareness of the vinyl glove ingestion.Conclusions: Vinyl glove ingestion can cause intestinal obstruction and perforation. Vinyl gloves should be removed from the immediate proximity of mentally retarded patients or patients with pica. Most of the time, the finding of vinyl gloves as etiology of the obstruction or perforation is incidental. If the event is known or witnessed, prompt surgical intervention is generally recommended.

Feasibility of conducting a prospective cohort study in pediatric surgery: introducing the Caregiver Quality of Life of pediatric patients referred for feeding tube insertion (CARE) study

2012-05-01

Abstract: Introduction: High-quality prospective cohort studies are needed to answer research questions focused on prognosis. To determine the feasibility of conducting this type of research, a prospective cohort study focused on investigating health-related quality of life (HRQoL) of caregivers of children who require a gastrostomy (G) tube at the McMaster Children's Hospital was piloted from November 2009 to May 2011.Methods: Recruitment began after research ethics board approval. Feasibility data were collected on all caregivers of patients assessed for G tube placement by a pediatric surgeon including recruitment status, protocol compliance, and HRQoL. Data were collected at 1 month and 2 weeks before surgery and 2 weeks and 3, 6, 9, and 12 months after surgery. Health-related quality of life measures included the Short Form 36v2, the Caregiver Strain Index, and the Parent Experience of Childhood Illness.Results: A total of 117 G procedures were conducted, and 39 caregivers met eligibility criteria. Thirty-one caregivers were enrolled (79.5% recruitment rate), 8 parents were not interested, and 26 were missed. Protocol adherence to the follow-up schedule is high (91.3%).Conclusion: Conducting high-level research with long-term follow-up in pediatric surgery is challenging. Factors including low prevalence of patients and competing priorities for busy clinicians indicate that multicenter collaboration is essential to research success in this field.

The 43rd annual meeting of the Canadian Association of Pediatric Surgeons

Pramod S. Puligandla — 2012-05-01

The 43rd annual meeting of the Canadian Association of Pediatric Surgeons (CAPS) was held in our nation's capital at the Chateau Laurier in Ottawa, Ontario, Canada, from September 22 to 24, 2011. The hotel facilities were fabulous, and the quality of the program was superb, much to the credit of the program chair, Dr Sarah Bouchard, and her committee. Thirty-two oral articles and 33 posters were presented. Each presentation was of very high quality, and lively discussion often ensued, as is habit at CAPS. Arlene Ein was invaluable in ensuring the smooth transition of the different components of the meeting, both scientific and social. Local host Dr Juan Bass did an excellent job making sure that the conference attendees had a memorable and pleasurable visit to Ottawa.

Continuing Medical Education Program: Journal of Pediatric Surgery–CME featured articles in this issue

2012-05-01

Congenital absence of cartilaginous tracheal rings associated with esophageal atresia and trifurcated carina: a novel anomaly?

2012-05-01

Abstract: Tracheomalacia associated with esophageal atresia (EA) is a well-known condition. However, complete absence of tracheal rings (TRs) is extremely rare. Our aim is to describe a novel triad of conditions and to discuss the best treatment. An expremature male operated for EA presented with severe respiratory distress. The diagnosis of absent cartilage rings, suspected on bronchoscopy, was confirmed by optical coherence tomography. The absence of TRs was localized to a short tracheal segment, and the carina trifurcated into right upper lobe, right intermediate, and left main bronchus. The patient was treated with resection and anastomosis with a completely satisfactory course. Absence of TRs was previously reported by us in 2 other cases, both with associated EA and trifurcation of the carina. One child was treated with tracheostomy and the other with a stent, but the outcome was far from optimal. The patient with tracheostomy eventually underwent resection and anastomosis with tracheostomy closure. Congenital absence of TRs is extremely rare. Although localized, it is responsible for severe symptoms owing to complete tracheal collapse and may be misdiagnosed as tracheomalacia. In our experience, it has been associated with EA and trifurcated carina. Our limited experience suggests resection of the abnormal segment and tracheal anastomosis as the best treatment.

Pseudoaneurysm of the radial branch of the splenic artery with pancreatic pseudocyst in a child with recurrent acute pancreatitis: treatment with endovascular stent graft and cystogastrostomy

2012-05-01

Abstract: Splenic artery pseudoaneurysms (SAPs) are rare in children and usually follow abdominal trauma. Although pancreatitis is a well-known cause for SAP in adults, pancreatitis resulting in SAP has only sporadically been reported in children. Before the refinements of endovascular techniques for management of SAP, surgery used to be the mainstay of treatment, often resulting in splenectomy. Recent technical advancements, including development of smaller delivery systems and microcatheters, have made endovascular treatment feasible in children with SAP and increased chances of splenic preservation. In this article, we report a rare case of SAP associated with a pancreatic pseudocyst in a 5-year-old boy with recurrent acute pancreatitis that was managed by endovascular stent graft and cystogastrostomy.

Life-threatening hemorrhage from a congenital hemangioma caused by birth trauma

Meghan Nolan, Charles W. Hartin, Joelle Pierre, Doruk E. Ozgediz — 2012-05-01

Abstract: Pediatric surgeons frequently diagnose and treat vascular malformations. We present the case of a boy born with a large congenital hemangioma of the flank that ruptured during birth, resulting in life-threatening hemorrhage, requiring emergent excision. Prenatal diagnosis may help to identify such lesions, and pediatric surgeons must be ready to treat emergent complications of vascular malformations.

Gastric adenomyoma mimicking gastric duplication cyst in a 5-year-old girl

2012-05-01

Abstract: Gastric adenomyoma in children is a rare benign tumor composed of a mixture of duct-like epithelial structures with smooth muscle bundles. It has been considered as a subtype of ectopic pancreas because the epithelial component resembles that in pancreatic ducts. However, it is now recognized as an independent pathologic lesion. Gastric adenomyoma is usually asymptomatic but could cause obstruction, inflammation, ulceration, and malignant transformation. Hence, to achieve diagnostic certainty and to control symptoms, complete operative resection is recommended. To the best of our knowledge, this is the first case of gastric adenomyoma mimicking a gastric duplication cyst in children.

Morio Kasai: a remarkable impact beyond the Kasai procedure

Alejandro V. Garcia, Robert A. Cowles, Tomoaki Kato, Mark A. Hardy — 2012-05-01

Abstract: Morio Kasai is one of the most influential Japanese pediatric surgeons. He is best recognized in the United States for his pioneering efforts in the field of biliary atresia. His work revolutionized the treatment of infants born with biliary atresia throughout the world. Less is known about his remarkable impact in the fields of general surgery and pediatric surgery. This review highlights some of Dr Kasai's major contributions and highlights the influence he had in the establishment of pediatric surgery as a field.

Lipofibromatosis of the knee in a 19-month-old child

2012-05-01

Abstract: Lipofibromatosis is a rare benign fibrofatty tumor of childhood. The typical presentation of this tumor is as a poorly demarcated and slow-growing mass involving the subcutaneous or deep soft tissues. Lipofibromatosis was first described in 2000, and since then, a small number of cases have been reported in the literature. We report a case of a 19-month-old boy who presented with a swelling of the anterior aspect of the right knee since birth, which had increased in size out of proportion with his growth. Magnetic resonance imaging was extremely useful because it showed the lipomatous nature of the mass, narrowing the differential diagnosis to the pediatric fibrofatty soft tissue tumors. The histologic biopsy revealed the specific diagnosis of lipofibromatosis. We describe the radiologic and pathologic features of this entity and discuss the differential diagnosis in a young child with a fat-containing limb mass.

Glandular resection and central embedding in hypospadias repair—a novel modification of the Barcat technique

2012-05-01

Abstract: Purpose: Urethrocutaneous fistula and urethral or meatal stricture, to date, remain the commonest complications of hypospadias repair, with increased tissue tension being a major cause of failure. We developed a novel technique comprising glandular resection and central embedding of the neourethra to specifically address this critical issue. We report the results of the first 112 procedures, of which 7 cases were a secondary hypospadias repair.Materials and Methods: All patients who underwent hypospadias repair using the glandular resection and central embedding technique over a period of 10 years were included into this retrospective single-surgeon case series. An independent third-party observer carried out follow-up examinations including urometric evaluation.Results: The median follow-up was 37 months (range, 7-147 months). In the group of primary hypospadias repair, urethrocutaneous fistulas were observed in 4%, all requiring surgical revision, and meatal or urethral stricture were observed in 4% of the patients, treated either surgically or by dilatation. In 98% of the patients, the neomeatus was located at the tip of the glans. A cosmetically impeccable slitlike appearance of the meatus was achieved in 84%, and a cosmetically acceptable oval shape was achieved in 10% of the patients. Both maximum and average urinary flow rates were within the standard ranges for the respective age groups in 93.7% and 96%, respectively. There was no significant residual urine in 92% of the patients. In the group of secondary hypospadias repairs, both cosmetic and functional results were similar, and no complications were noted.Conclusions: Glandular resection and central embedding is a promising expansion of the repertoire of Barcat modifications. It allows safe embedding of the neourethra and glandular closure without detrimental tension. The cosmetic and functional results are encouraging.

Tapering duodenoplasty and gastrojejunostomy in the management of idiopathic megaduodenum in children

2012-05-01

Abstract: Purpose: The purpose of this study is to present the management of idiopathic megaduodenum in children.Methods: A retrospective analysis of 4 cases of megaduodenum admitted from 2005 to 2011 was performed evaluating clinical features, radiologic data, treatment, pathologic findings, and prognosis. The corresponding literature was reviewed.Results: The diagnosis of nonobstructive megaduodenum was confirmed by upper gastrointestinal contrast study, ultrasonography, and exploratory laparotomy. Treatment consisted of either tapering duodenoplasty with pylorus division and closure of the proximal stump plus Roux-en-Y gastrojejunostomy or tapering duodenoplasty with closure of the proximal stump and end-to-side gastrojejunostomy. On pathologic evaluation, neural and vascular structures appeared normal in all sections. All symptoms, including diarrhea, bloating, vomiting, and nausea, had resolved on follow-up, and all patients experienced rapid weight gain after their operation.Conclusions: Idiopathic megaduodenum without organic obstruction is a rare clinical condition. Massive dilatation confined to the duodenum was shown by upper gastrointestinal contrast studies and ultrasonography and can also be identified on antenatal ultrasonography. In children with megaduodenum, satisfactory results can be obtained by tapering duodenoplasty with proximal stump closure and gastrojejunostomy with either Roux-en-Y or end-to-side anastomosis.