Journal of Pediatric Surgery

Health care quality, access, cost, workforce, and surgical education: the ultimate perfect storm

Marshall Z. Schwartz — 2012-01-01

Abstract: The discussions on health care reform over the past two years have focused on cost containment while trying to maintain quality of care. Focusing on just cost and quality unfortunately does not address other very important factors that impact on our health care delivery system. Availability of a well-trained workforce, maintaining the sophisticated medical/surgical education system, and ultimately access to quality care by the public are critical to maintaining and enhancing our health care delivery system. Unfortunately, all five of these components are under at risk. Thus, we have evolving the ultimate perfect storm affecting our health care delivery system. Although not ideal and given the uniqueness of our population and their expectations, our current delivery system is excellent compared to other countries. However, the cost of our current system is rising at an alarming rate. Currently, health care consumes 17% of our gross domestic product. If our system is not revised this will continue to rise and by 2025 it will consume 48%. The dilemma, given the current state of our overall economy and rising debt, is how to address this major problem. Unfortunately, the Affordable Care Act, which is now law, does not address most of the issues and the cost was initially grossly under estimated. Furthermore, the law does not address the issues of workforce, maintaining our medical education system or ultimately, access. A major revision of our system will be necessary to truly create a system that protects and enhances all five of the components of our health care delivery system. To effectively accomplish this will require addressing those issues that lead to wasteful spending and diversion of our health care dollars to profit instead of care. Improved and efficient delivery systems that reduce complications, reduction of duplication of tertiary and quaternary programs or services within the same markets (i.e. regionalization of care), health insurance reform, and tort reform collectively could save hundreds of billion dollars per year! These changes may not be easy to accomplish politically but will be essential to save what is likely the best health care system in the world.

Notes on the early development of pediatric surgery in the United States

Judson G. Randolph — 2012-01-01

Abstract: This lecture addresses the leadership roles in American surgery.

Regenerative medicine strategies

Anthony Atala — 2012-01-01

Abstract: Applications of regenerative medicine technology may offer novel therapies for patients with injuries, end-stage organ failure, or other clinical problems. Currently, patients suffering from diseased and injured organs can be treated with transplanted organs. However, there is a severe shortage of donor organs that is worsening yearly as the population ages and new cases of organ failure increase. Scientists in the field of regenerative medicine and tissue engineering are now applying the principles of cell transplantation, material science, and bioengineering to construct biological substitutes that will restore and maintain normal function in diseased and injured tissues. The stem cell field is also advancing rapidly, opening new avenues for this type of therapy. For example, therapeutic cloning and cellular reprogramming may one day provide a potentially limitless source of cells for tissue engineering applications. While stem cells are still in the research phase, some therapies arising from tissue engineering endeavors have already entered the clinical setting successfully, indicating the promise regenerative medicine holds for the future.

The history of paediatric surgery in the United Kingdom and the influence of the national health service on its development

Lewis Spitz — 2012-01-01

Abstract: Details of the modern history of paediatric surgery in the United Kingdom with particular emphasis on the 3 main training centres in England in the 1960s to 1970s are discussed. The genesis of the National Health Service and of the British Association of Paediatric Surgeons and their influence on the establishment and siting of regional centres, education and training, and centralization of rare conditions is highlighted.

Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection

2012-01-01

Abstract: Background/Purpose: Congenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis.Method: A retrospective review of patients undergoing open fetal surgery or steroids for CPAM at our institution was performed. The primary outcome was survival.Results: A retrospective review of all patients referred to our institution with the diagnosis of CPAM was performed. Fetuses with predominantly microcystic CPAM and the presence of hydrops fetalis treated with steroid or surgery were included. Thirteen patients were treated with steroids, and 11 patients underwent open fetal surgery. In the steroid group 12 (92%) of 13 fetuses survived to delivery versus 9 (82%) of 11 in the open fetal surgery group. Only 5 (56%) of 9 of the patients in the open fetal surgery group survived to neonatal discharge compared to 10 (83%) of 12 in the steroid group.Conclusions: In the present retrospective study, improved survival was seen in fetuses with hydrops fetalis and predominantly microcystic CPAM treated with steroids when compared with open fetal surgery. Steroids should be considered for first-line therapy in these cases.

Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors

2012-01-01

Abstract: Purpose: The aim of this study was to identify the most accurate prenatal predictors of outcomes and need for fetal surgery for fetuses with high-risk lung masses and vascular tumors.Methods: The records of all fetuses with high-risk lung mass (congenital cystic adenomatoid malformation-volume ratio > 1.6 or findings of hydrops) and vascular tumor evaluated between July 2001 and March 2011 were reviewed retrospectively. Hydrops was defined as accumulation of fluid in 2 or more compartments.Results: Of fetuses with high-risk lung mass, hydrops was identified in 46% (11/24). Fetuses with hydrops and an abnormal echocardiogram (n = 8) demonstrated poor survival without fetal surgery (13%) compared with 100% survival in fetuses with hydrops and a normal echocardiogram (n = 3; P = .02). Of 21 fetuses with vascular tumor (11 sacrococcygeal and 8 cervical teratomas; 2 hemangioendotheliomas), hydrops was identified in 29% and an abnormal echocardiogram in 57%. All fetuses with hydrops had an abnormal echocardiogram and either died (n = 5) or required fetal surgery (n = 1). However, all fetuses with abnormal echocardiograms alone (n = 7) survived without fetal intervention.Conclusions: For fetuses with lung mass, an abnormal echocardiogram in the setting of hydrops is the best predictor of mortality and need for fetal surgery. For fetuses with vascular tumor, hydrops in the setting of high-output physiology best predicts demise and need for fetal surgery.

Tracheoesophageal displacement index and predictors of airway obstruction for fetuses with neck masses

2012-01-01

Abstract: Purpose: There are no established selection guidelines to determine which fetuses with giant neck masses may benefit from delivery using an ex utero intrapartum treatment procedure. The purposes of this study were to describe the tracheoesophageal displacement index (TEDI), a novel measurement of fetal airway displacement, and to correlate this measurement and other prenatal findings to the extent of airway obstruction at birth.Methods: The medical records of all fetuses with giant neck masses evaluated between 2001 and 2011 were reviewed. At birth, each infant's airway was categorized as uncomplicated or complicated. Prenatal variables were correlated with airway difficulty.Results: There were 24 fetuses with large neck masses (11 lymphatic malformations, 10 teratomas, 3 others). One fetus died in utero, and 3 underwent pregnancy termination. Variables associated with a complicated airway at birth included polyhydramnios, teratoma diagnosis, and tracheoesophageal displacement index. Tracheoesophageal displacement index greater than 12 correlated strongly with a complicated airway (area under the curve = 0.80). All fetuses classified with an uncomplicated airway (n = 7) had a diagnosis other than teratoma and normal amniotic fluid volume.Conclusion: In fetuses with giant neck masses, the presence of polyhydramnios, teratoma diagnosis, or tracheoesophageal displacement index greater than 12 are predictive of a complicated airway at birth. Our data suggest that fetuses without any of these findings may be delivered safely without an ex utero intrapartum treatment approach.

Long-term morbidity after fetal endoscopic surgery for severe twin-to-twin transfusion syndrome

2012-01-01

Abstract: Background/Purpose: Severe twin-to-twin transfusion syndrome (TTTS) leads to 80% to 100% dual mortality. Endoscopic laser coagulation of connecting vessels improves outcome to 80% survival of at least 1 twin. There is limited long-term follow-up of surviving TTTS patients. The aim of this study was to analyze gestational age-stratified, long-term morbidity in these patients.Methods: A retrospective case-control study of TTTS surviving patients (38 patients, 72% follow-up rate) from one center. Perinatal and pediatric records were reviewed, and outcomes were compared with published reports and gestational age-matched controls.Results: Forty percent (15/38) had at least 1 major sequela, all but 6 of which were fully resolved at a median follow-up of 4.4 years. There were no permanent cardiac, genitourinary, renal, or respiratory sequelae. All major complications were in patients born <29 weeks. There were no significant differences in complications between this cohort of patients and gestational age (GA)-matched control patients.Conclusions: The long-term morbidity of monochorionic twins after fetal laser surgery for severe TTTS is 13%. At a median follow-up of more than 4 years, these children fare no worse than gestational age-matched, non-operated twins and singletons. The degree of prematurity at birth is the best predictor of temporary or permanent sequela in this group of patients.

The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital diaphragmatic hernia

2012-01-01

Abstract: Purpose: We developed the congenital diaphragmatic hernia congenital prognostic index (CDH-CPI) to incorporate all known prognostic variables into a single composite index to improve prognostic accuracy. The purpose of this study is to examine the ability of the CDH-CPI to predict survival in patients with left-sided congenital diaphragmatic hernia and to determine if the index has a stronger correlation with survival than each of the individual components.Methods: A retrospective review of patients with left-sided congenital diaphragmatic hernia between 2004 and 2010 was conducted. Ten prenatal parameters of the CDH-CPI were collected, total score was tabulated, and patients stratified according to total score and survival.Results: Sixty-four patients with a prenatal diagnosis of left-sided congenital diaphragmatic hernia were identified. Patients with a CDH-CPI score of 8 or higher had a significantly higher survival than patients with a CDH-CPI score of lower than 8. The CDH-CPI has the strongest correlation with survival compared with the individual parameters measured. The CDH-CPI correlates with extracorporeal membrane oxygenation use, and 75% of patients with a score of 5 or lower were placed on extracorporeal membrane oxygenation.Conclusions: The CDH-CPI accurately stratifies survival in left-sided congenital diaphragmatic hernia. The amalgamation of 10 prenatal parameters of the CDH-CPI may be a better prenatal predictor than any single prognostic variable currently used.

Improved survival in venovenous vs venoarterial extracorporeal membrane oxygenation for pediatric noncardiac sepsis patients: a study of the Extracorporeal Life Support Organization registry

2012-01-01

Abstract: Background/Purpose: There are few studies comparing venoarterial (VA) and venovenous (VV) extracorporeal membrane oxygenation (ECMO) in pediatric noncardiac sepsis patients.Methods: Following approval, we reviewed the Extracorporeal Life Support Organization registry data from 1990 to 2008 for patients 0 to 18 years with a diagnosis of sepsis and without diagnosis of congenital heart disease. Survival to discharge was compared between VA and VV ECMO using χ2 analysis and multivariable logistic regression.Results: Four thousand three hundred thirty-two ECMO runs were reviewed, 3256 VA (75%) and 1076 VV (25%). A majority of VA modality was noted in each decade studied. Overall survival was 68% and was higher in VV (79%) than in VA ECMO (64%, P < .001). Survival decreased with increasing age (73% in newborns ≤1 month, 40% in children 1 month to 12 years, and 32% in adolescents >12 years, P < .001). VA ECMO had increased mortality risk after adjustment for age, use of vasoactive agents, and advanced respiratory support (odds ratio, 2.06; 95% confidence interval, 1.74-2.44; P < .001).Conclusions: These data demonstrate improved survival in VV vs. VA ECMO in select pediatric septic patients without congenital heart disease. When technically feasible, physicians should consider VV ECMO as first therapeutic choice in this patient population.

Extracorporeal membrane oxygenation cannulation trends for pediatric respiratory failure and central nervous system injury

2012-01-01

Abstract: Background: Guidelines regarding arterial cannula site and cannula site-specific risks of central nervous system (CNS) injury for pediatric patients requiring extracorporeal membrane oxygenation (ECMO) support are lacking. We reviewed cannulation trends for pediatric respiratory failure and evaluated CNS complication rates by cannulation site and mode of support.Methods: The Extracorporeal Life Support Organization (ELSO) registry was queried for all pediatric respiratory failure patients <18 years treated from 1993-2007. The primary outcome was radiographic evidence of CNS injury.Results: Venoarterial (VA) support was used in 62% of 2617 ECMO runs. The carotid artery was used in 93% of VA patients. Femoral artery use increased in patients >5 years of age and >20 kg. Venovenous (VV) ECMO was used in >50% of children >10 years. No significant difference was identified in CNS injury between carotid and femoral cannulation in any age group but the femoral group was small (4.4%). VA support was independently associated with increased odds of CNS injury compared to VV cannulation (OR, 1.6).Conclusion: VA ECMO is the most common mode of support in pediatric respiratory failure patients. Although no significant difference in CNS injury was noted between carotid and femoral artery cannulation, the odds of injury were significantly higher than VV support.

Utility of neuroradiographic imaging in predicting outcomes after neonatal extracorporeal membrane oxygenation

2012-01-01

Abstract: Background: The need for routine neuroimaging after extracorporeal membrane oxygenation (ECMO) and the optimal radiographic study remains unclear. We sought to evaluate the correlation between findings on head ultrasound (HUS) and magnetic resonance imaging (MRI) and determine the association of these findings to neurodevelopmental outcome.Methods: A retrospective review was performed (2003-2010) to identify neonates who had a MRI after ECMO. Each MRI was reviewed by a single pediatric neuroradiologist. Neurodevelopmental data was collected from the high-risk neonatal follow-up clinic.Results: Fifty neonates had a MRI (venoarterial 37, venovenous 13) after ECMO. HUS was abnormal in 24%, whereas MRI was abnormal in 62%. All infants with an abnormal HUS had an abnormal MRI, but an additional 50% of patients with a normal HUS had an abnormal MRI. Venoarterial ECMO was significantly associated with an abnormal MRI. Follow-up data was available for 26 neonates. The only predictor of abnormal neurodevelopment was the need for supplemental tube feeds at discharge.Conclusions: MRI identified significantly more abnormalities compared to routine HUS after neonatal ECMO. However, neither MRI nor HUS findings correlated with early neurodevelopmental outcome. Feeding ability at discharge was the overall best predictor of neurologic impairment in survivors.

Split abdominal wall muscle flap repair vs patch repair of large congenital diaphragmatic hernias

2012-01-01

Abstract: Purpose: Large congenital diaphragmatic hernias are commonly repaired with a prosthetic patch. We hypothesized that a split abdominal wall muscle flap would reduce the risk of recurrence.Methods: A retrospective review of neonates with congenital diaphragmatic hernia in whom primary repair was not possible was performed. Kaplan-Meier analyses and Cox proportional hazards modeling were performed.Results: Of 153 patients, 46 could not have repair with primary closure of the diaphragm. Thirty-three survived to discharge and were subjected to analysis for recurrence. Ten underwent repair with a patch, whereas 23 had a muscle flap (internal oblique and transversalis) patch. The groups were similar with regard to demographics, need for extracorporeal membrane oxygenation, repair on extracorporeal membrane oxygenation, and size of the defect. Fifty percent of patch repairs recurred with a median time of 0.5 years. Only one (4.3%) of the patients who had muscle flap patch developed a recurrence. This was significant on Kaplan-Meier analysis (P = .0009) and had a hazard ratio of 14.3 on Cox regression (P = .018). Median follow-up exceeded 4 years. No children required surgery for an abdominal wall hernia.Conclusions: The split abdominal wall muscle flap allows for closure of large congenital diaphragmatic hernia defects with autologous tissue. This approach is associated with significantly fewer recurrences than patch repairs.

Congenital lung anomalies: can we postpone resection?

2012-01-01

Abstract: Background/Purpose: The management of asymptomatic congenital lung lesions is controversial. It is unclear whether elective resection provides a significant benefit. We sought to determine whether early vs delayed resection of asymptomatic congenital lung malformations resulted in complications.Methods: Institutional billing records were queried for patients with lung malformations over a 10-year period. Medical records were reviewed for demographics, type of anomaly, symptoms, management, and procedural or disease-related complications.Results: Eighty-seven patients were identified. The diagnoses included congenital cystic adenomatoid malformation (41%), bronchogenic cyst (19.3%), sequestration (13.2%), and congenital lobar emphysema (12.0%). Fifty patients were observed for some period. Eleven became symptomatic, and 47 underwent resection at a mean age of 11 months. There was no difference in the type of resection, length of hospitalization, or complication rate between patients who underwent early vs delayed resection. There were no occurrences of malignancy or death.Conclusions: In our series, there was no difference in measurable outcomes between early and delayed resection of congenital lung lesions. These data provide some support for a management strategy that might include observation with delayed resection for asymptomatic patients.

Pyloromyotomy: randomized control trial of laparoscopic vs open technique

Sabina Siddiqui, R. Eric Heidel, Carlos A. Angel, Alfred P. Kennedy — 2012-01-01

Abstract: Purpose: Open pyloromyotomy remains as the criterion standard treatment for hypertrophic pyloric stenosis with the laparoscopic approach rapidly gaining adoption. We present a prospective, randomized trial between the 2 approaches.Methods: After institutional review board approval, 98 patients with hypertrophic pyloric stenosis were consecutively randomized to either open or laparoscopic pyloromyotomy. Postoperative and hospital course were evaluated by review of the hospital records and long-term follow-up with scripted telephone survey using Likert scales. The length of operating room time, surgical procedure, postoperative stay, time to refeeding, and complications were evaluated. Secondary outcomes of cosmetic results and parental satisfaction were determined.Results: Ninety-eight patients were enrolled during a 4-year period. There were no significant differences between 2 groups on all primary outcomes. There were 3 complications in the open group—a wound dehiscence, a surgical site infection, and a gastric serosal tear—and 2 complications in the laparoscopic group—mucosal perforation and a suture granuloma. In long-term follow-up on 72 patients (56 months), parents described significant cosmetic results with laparoscopic approach.Conclusions: There was no difference in operating time, hospital stay, or refeeding patterns between open and laparoscopic pyloromyotomy. The complication rates were similar between the 2 methods. However, long-term cosmetic results were significantly superior in the laparoscopic group.

A novel antireflux procedure: gastroplasty with restricted antrum to control emesis (GRACE)

2012-01-01

Abstract: Purpose: Nissen fundoplication is the most commonly performed operation to treat gastroesophageal reflux disease and vomiting in children with neurologic impairment. However, failure rates of Nissen fundoplication in this population are higher, and alternatives to Nissen fundoplication have technical and functional disadvantages. We hypothesize that the novel gastroplasty with restricted antrum to control emesis (GRACE) would be more effective than Nissen fundoplication at reducing emetic reflux.Methods: To compare the GRACE with Nissen fundoplication, 15 canine subjects were randomized to Nissen fundoplication or GRACE. All subjects underwent gastrostomy tube placement. Baseline gastric emptying, electrogastrography, and induced vomiting studies were performed. Nissen fundoplication or GRACE was then performed. Postoperatively, gastric emptying and vomiting studies were repeated.Results: Gastric emptying before and after antireflux procedures was not significantly different between groups. Both Nissen fundoplication (38%, P = .04) and GRACE (69%, P < .01) procedures prevented reflux compared with baseline. However, the GRACE procedure significantly reduced reflux when compared with Nissen fundoplication (P = .03).Conclusions: In this canine model, GRACE appears to be significantly more effective than Nissen fundoplication at reducing emetic reflux. This novel procedure preserves gastric function and is well tolerated. The GRACE procedure may provide an alternative to Nissen fundoplication as a primary or repeat antireflux procedure for children with neurologic impairment.

Agency for Healthcare Research and Quality pediatric indicators as a quality metric for surgery in children: do they predict adverse outcomes?

Daniel Rhee, Yiyi Zhang, Dominic Papandria, Gezzer Ortega, Fizan Abdullah — 2012-01-01

Abstract: Background/Purpose: The pediatric quality indicators (PDIs) were developed by the Agency for Healthcare Research and Quality to compare patient safety and quality of pediatric care. These are being considered for mandatory reporting as well as pay-for-performance efforts. The present study evaluates the PDIs' predictive value for surgical outcomes in children.Methods: A cross-sectional study was performed using nationwide inpatient data from 1988 to 2007. Patients younger than 18 years with an inpatient surgical procedure were included and evaluated for 10 PDIs. Odds ratios for mortality, increase in length of stay, and total charges were calculated using multivariate regression adjusting for age, sex, race, region, hospital type, and comorbidities.Results: A total of 1,964,456 pediatric discharges were included. Mortality rates were 5.4% for patients with at least 1 PDI and 0.6% for those with none. Multivariate analysis showed that occurrence of any PDI was associated with a 20% increased risk of mortality. The PDIs were associated with an increased length of stay and total hospital charges.Conclusion: The present study shows that PDIs are associated with increased mortality risk as well as increased hospital stay and total hospital charges. This provides positive evidence for the utility of these indicators as metrics for quality and patient safety.

Quality improvement and patient care checklists in intrahospital transfers involving pediatric surgery patients

2012-01-01

Abstract: Background: Intrahospital transfers are necessary but hazardous aspects of pediatric surgical care. Plan-Do-Study-Act processes identify risks during hospitalization and improve care systems and patient safety.Methods: A multidisciplinary team developed a checklist that documented patient data and handoffs for all intrahospital transfers involving pediatric surgical inpatients. The checklist summarized major clinical events and provided concurrent summaries by 3-month quarters (Q) over 1 year.Results: There were 903 intrahospital transfers involving 583 inpatients undergoing surgery. Total handoffs were documented in 436 (75% of 583), with greater than 1 handoff in 202 (46% of 436). Documented problems occurred in 31 transfers (3.4%), the most during Q1 (19/191; 9.9%). Incidence fell to 3.5% (9/260) in Q2, 0.4% (1/243) in Q3, and 1.0% (2/209) in Q4 (P < .001). Patient care issues (14/31; 45%) were most common, followed by documentation (10, 32%) and process problems (7, 23%). The quality improvement team was able to resolve patient instability during transport (5 in Q1, none in Q3, Q4) and poor pain control (3 in Q2, 1 in Q3, Q4). Of the patients, 3.2% had identified problems with patient care during intrahospital transfer.Conclusions: Plan-Do-Study-Act review emphasizes ongoing process analysis by multidisciplinary teams. Checklists reinforce communication and provide feedback on whether system goals are being achieved.

The jury is still out: changes in gastroschisis management over the last decade are associated with both benefits and shortcomings

Brent R. Weil, Charles M. Leys, Frederick J. Rescorla — 2012-01-01

Abstract: Purpose: Management of gastroschisis has shifted from early primary closure to preformed silo placement and delayed closure. We aimed to identify how closure techniques have changed and how outcomes have been affected.Methods: Records of patients undergoing gastroschisis closure at a single institution from 2000 to 2009 were reviewed. Patient characteristics and outcomes were collected and compared among those undergoing primary closure vs preformed silo placement. Outcomes were also compared in an era when primary closure predominated (2000-2002) vs one when primary silo predominated (2003-2009).Results: From 2000 to 2009, 203 patients underwent gastroschisis closure. Primary closure was performed in 50% of patients from 2000 to 2002 vs 12.3% from 2003 to 2009. Preformed silos were placed in 34.7% of patients from 2000 to 2002 vs 84.4% from 2003 to 2009. Patients treated from 2000 to 2002 experienced shorter hospital stays and shorter time to achievement of full enteral nutrition. Patients treated from 2003 to 2009 developed fewer ventral hernias and wound infections and required less ventilator days. Patients undergoing early primary closure developed ventral hernias at higher rates compared with those treated with preformed silos. Intensive care unit stay was longer for patients receiving preformed silos.Conclusion: Change in our management strategy has resulted in prolonged intensive care unit stay and time to full feeds but reduced postoperative hernias and wound infections.

Long-term neurodevelopmental outcomes in children born with gastroschisis: the tiebreaker

2012-01-01

Abstract: Purpose: We evaluated 2-year neurodevelopmental outcomes in children with gastroschisis.Methods: We reviewed the records of children with gastroschisis treated between August 2001 and July 2008. Children discharged from the neonatal intensive care unit were referred to the state-sponsored Developmental Tracking Infant Progress Statewide (TIPS) program. We reviewed TIPS assessments performed before age 2 years. School districts evaluated children referred by TIPS and determined their eligibility for early intervention services. Poor outcomes were defined as scores of “failure” or “moderate/high risk” on the screening assessment or enrollment in early intervention services by 2 years. Children with gastroschisis were compared with case-matched nonsurgical, nonsyndromic children of similar gestational age and birth weight.Results: One hundred five children were born with gastroschisis, and 46 were followed up with TIPS. There was no statistically significant difference in performance on screening assessments or in the rate of enrollment in early intervention services between the gastroschisis children and controls.Conclusions: Children born with gastroschisis have similar 2-year neurodevelopmental outcomes as nonsurgical, nonsyndromic neonatal intensive care unit children of similar gestational age and birth weight. Both groups of children have a higher rate of enrollment in early intervention than their healthy peers. These data suggest that neurodevelopmental outcomes in gastroschisis children are delayed secondary to prematurity rather than the presence of the surgical disease.

Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism

2012-01-01

Abstract: Purpose: To determine the outcome of patients who underwent pancreatic head resection and Roux-en-Y pancreaticojejunostomy to the remaining normal pancreatic body and tail for the treatment of a focal lesion in the pancreatic head causing congenital hyperinsulinism (HI).Methods: One hundred thirty-eight patients underwent pancreatic resection for focal HI between 1998 and 2010. Twenty-three patients in the group underwent pancreatic head resection and Roux-en-Y pancreaticojejunostomy.Results: There were 13 females and 10 males. Median age and weight at surgery were 8 weeks and 5.8 kg, respectively. Twenty-one patients had a near-total pancreatic head resection, and 2 patients had a pylorus-preserving Whipple procedure. The pancreaticojejunostomy anastomosis was performed with interrupted fine monofilament sutures such that the transected end of the pancreatic body was tucked within the end of the Roux-en-Y jejunal limb. Median hospital stay was 22 days. All patients were cured of HI.Conclusion: We conclude that pancreatic head resection with Roux-en-Y pancreaticojejunostomy is a safe and effective procedure for the treatment of the HI patient with a large focal lesion in the pancreatic head that is not amenable to local resection alone.

Development of an endoluminal intestinal lengthening capsule

Rebecca Stark, Mohanchandra Panduranga, Gregory Carman, James C.Y. Dunn — 2012-01-01

Abstract: Purpose: Prior studies demonstrated the ability of a spring to lengthen intestinal segments. We made two innovations to this device. First, we employed a degradable capsule to control the deployment of the spring. Second, we decreased the spring force to allow slower expansion of the intestinal segment.Methods: Nitinol springs with varying forces were compressed and placed in gelatin capsules. These capsules were coated with a degradable polymer and were placed in isolated segments of rat jejunum. Serial x-rays were used to determine the rate of spring expansion. Retrieved jejunal segments were analyzed histologically.Results: Using the polymer-coated capsule, the spring was reliably deployed between 24 and 48 hours. Intestinal segments were lengthened from 1.0 cm to 3.6 cm after 14 days. The optimal spring for the gradual expansion of jejunal segments had a spring constant of 0.0010 N/mm. Villus height was preserved, but crypt depth was significantly greater in the lengthened intestine.Conclusion: Use of a low-force spring resulted in a nearly four-fold lengthening of jejunal segments. The use of a polymer-coated capsule provided a reliable way to control the timing of spring deployment. This capsule may be useful for the endoscopic placement of the spring in patients with short bowel syndrome.

Evaluation of intestinal viability using 3–charge coupled device image enhancement technology in a pediatric laparoscopic appendectomy model

Maridelle B. Millendez, Nicole J. Crane, Eric A. Elster, Shawn D. Safford — 2012-01-01

Abstract: Background: Intraoperative laparoscopic determination of intestinal viability is currently limited to subjective visible cues. Adjunctive modalities are neither widely available nor practical. Three–charge coupled device (3-CCD) imaging directly correlates the amount of light detected by CCDs to tissue oxygenation. We hypothesize that application of 3-CCD image enhancement detects bowel ischemia in a pediatric laparoscopic appendectomy model.Methods: We recorded 10 laparoscopic appendectomies for appendicitis. Offline analysis involved selecting regions of interest (ROIs) in the appendix, adjacent colon, and nonappendiceal fat and calculating mean intensity values in selected images before and after division of the mesoappendix. The colon was used as a control, and the intensity values were normalized to fat.Results: As an indicator of decreased perfusion, the mean appendix ROI intensity values decreased over time (R2 = 0.92) compared with the colon mean ROI intensity values, which remained stable. There was a statistically significant difference between fat-normalized intensity values for ischemic and nonischemic appendix after 1 minute.Conclusion: We have demonstrated proof of principle for the determination of bowel ischemia using 3-CCD image enhancement. By quantitatively identifying areas of ischemia, this technique has the potential to significantly change the management of ischemic bowel in the future.

Epidural vs patient-controlled analgesia for postoperative pain after pectus excavatum repair: a prospective, randomized trial

2012-01-01

Abstract: Purpose: Management of postoperative pain is a challenge after the minimally invasive repair of pectus excavatum. Pain is usually managed by either a thoracic epidural or patient-controlled analgesia with intravenous narcotics. We conducted a prospective, randomized trial to evaluate the relative merits of these 2 pain management strategies.Methods: After obtaining permission/assent (Institutional Review Board no. 06 08 128), patients were randomized to either epidural or patient-controlled analgesia with fixed protocols for each arm. The primary outcome variable was length of stay with a power of .8 and α of .05.Results: One hundred ten patients were enrolled. There was no difference in length of stay between the 2 arms. A longer operative time, more calls to anesthesia, and greater hospital charges were found in the epidural group. Pain scores favored epidural for the few days and favored patient-controlled analgesia thereafter. The epidural catheter could not be placed or was removed within 24 hours in 12 patients (22%).Conclusions: There is longer operating room time, increase in calls to anesthesia, and greater hospital charges with epidural analgesia after repair of pectus excavatum. Pain scores favor the epidural approach early in the postoperative course and patient-controlled analgesia later.

Magnetic mini-mover procedure for pectus excavatum III: safety and efficacy in a Food and Drug Administration-sponsored clinical trial

2012-01-01

Abstract: Purpose: The magnetic mini-mover procedure (3MP) uses magnetic force to gradually remodel pectus excavatum deformity. A magnet is implanted on the sternum and coupled with an external magnetic brace. Under Investigational Device Exemption and Institutional Review Board approval, we performed a pilot study of safety, probable efficacy, and cost-effectiveness of this new treatment of an orphan disease using an implantable pediatric device.Methods: Ten otherwise healthy patients, ages 8 to 14 years, with severe pectus excavatum (pectus severity index [PSI] > 3.5) underwent 3MP treatment (mean, 18.8 ± 2.5 months). Safety was assessed by postimplant and postexplant electrocardiograms and monthly chest x-rays. Efficacy was assessed by change in pectus severity index as measured using pretreatment and posttreatment computed tomographic scan. Cost of 3MP was compared with that of standard procedures.Results: The 3MP device had no detectable ill effect. Device weld failure or malpositioning required revision in 5 patients. Average wear time was 16 h/d. Pectus severity index improved in patients in the early or mid puberty but not in patients with noncompliant chest walls. Average cost for 3MP was $46,859, compared with $81,206 and $81,022 for Nuss and Ravitch, respectively.Conclusion: The 3MP is a safe, cost-effective, outpatient alternative treatment for pectus excavatum that achieves good results for patients in early and midpuberty stages.

Optoelectronic plethysmography demonstrates abrogation of regional chest wall motion dysfunction in patients with pectus excavatum after Nuss repair

2012-01-01

Abstract: Purpose: We previously demonstrated that patients with pectus excavatum (PE) have significantly decreased chest wall motion at the pectus defect compared with the rest of the chest vs unaffected individuals and use abdominal respiratory contributions to compensate for decreased upper chest wall motion. We hypothesize that PE repair will reverse chest wall motion dysfunction.Methods: A prospective, institutional review board–approved study compared patients with PE before and after Nuss repair. Informed consent was obtained before motion analysis. Sixty-four patients with uncorrected PE ages 10 to 21 years underwent optoelectronic plethysmography analysis. Repeat analysis was performed in 42 patients 6 months postoperative (PO).Results: Volume of the chest wall and its subdivisions were calculated. Total chest wall volume at rest was significantly increased after repair and in each thoracic compartment. PO patients developed increased midline marker excursion at the pectus defect and significantly decreased excursion at the level of the umbilicus.Conclusions: Optoelectronic plethysmography kinematic analysis demonstrates that chest wall remodeling during Nuss repair results in increased thoracic volume. Chest wall motion dysfunction at the pectus defect is reversed after Nuss repair. Abdominal respiratory contributions are also markedly decreased. These findings may help to explain why patients with PE report an improvement in endurance after the Nuss procedure.

Lessons from a liver hemangioma registry: subtype classification

2012-01-01

Abstract: Purpose: A previously proposed classification of hepatic hemangioma postulated 3 types of lesions: focal, multifocal, and diffuse. A registry (www.liverhemangioma.org) was created to track patients to validate this classification scheme.Methods: Registry records entered between 1995 and 2010 were reviewed.Results: Of 121 patients with hepatic hemangioma, 119 were focal (n = 33), multifocal (n = 68), and diffuse (n = 18). Two patients shared features of multifocal and diffuse. The focal group had a balanced sex distribution, whereas multifocal and diffuse were more common in women (66.2% and 70.0%, respectively). Hepatic hemangioma was detected prenatally in 30% (9/30) of patients with focal disease. Detection was not possible in multifocal or diffuse hepatic hemangioma, indicating postnatal proliferation typical of common cutaneous infantile hemangioma. Cutaneous hemangiomas accompanied 77.4% (48/62) of multifocal hepatic hemangioma, 53.3% (8/15) of diffuse hepatic hemangioma, and 15.3% (4/26) of focal hepatic hemangioma. Hypothyroidism was documented in all (16/16) patients with diffuse hepatic hemangioma, 21.4% (9/42) with multifocal hepatic hemangioma, but in no patients with focal hepatic hemangioma (0/17).Conclusions: Analysis of the Liver Hemangioma Registry confirms that focal lesions are biologically distinct from multifocal and diffuse hepatic hemangioma. Feared diffuse hepatic hemangioma may evolve from previously undetected multifocal hepatic hemangioma. Screening ultrasounds in infants with multiple cutaneous infantile hemangioma may allow for earlier detection and therapy.

Circulating thyrotropin receptor messenger RNA for evaluation of thyroid nodules and surveillance of thyroid cancer in children

Jesse Gutnick, Oliver Soldes, Manjula Gupta, Mira Milas — 2012-01-01

Abstract: Purpose: The aim of this study was to determine the role of thyrotropin receptor messenger RNA as a novel blood test for evaluation of thyroid nodules and cancer in children.Methods: We reviewed thyroid disease management of patients younger than 18 years with thyrotropin receptor messenger RNA measurements identified from a prospective, institutional review board-approved registry during 2008 to 2010.Results: Thirty-nine thyrotropin receptor messenger RNA measurements were made in 21 female patients (12-17 years old; median, 16 years). Four patients with fine-needle aspiration only had benign thyroid cytology, and 3 of 4 had undetectable thyrotropin receptor messenger RNA. Seventeen patients underwent 22 thyroid operations. Preoperative thyrotropin receptor messenger RNA was measured in 9 patients: 3 of 4 with thyroid cancer had elevated levels and 3 of 5 with goiters undetectable. Postthyroidectomy surveillance (median, 16 months; range, 6-24) of 11 thyroid cancer patients (8 papillary, 3 follicular) showed that thyrotropin receptor messenger RNA was concordant with thyroglobulin in 14 (73%) of 19 measurements. In 3 (16%) of 19 measurements, thyrotropin receptor messenger RNA was the only blood test useful for disease assessment because of elevated antithyroglobulin antibodies. Overall, to predict thyroid cancer, thyrotropin receptor messenger RNA demonstrated 73% sensitivity, 82% specificity, 62% positive predictive value, 88% negative predictive value, and 79% accuracy.Conclusion: Thyrotropin receptor messenger RNA provides complementary evaluation to thyroglobulin and fine-needle aspiration for pediatric thyroid nodule management.

Novel zebrafish model reveals a critical role for MAPK in lymphangiogenesis

R. Dawn Fevurly, Sean Hasso, Alexander Fye, Steven J. Fishman, Joanne Chan — 2012-01-01

Abstract: Purpose: Lymphatic disorders are poorly understood with few animal models. We designed a novel assay to measure lymphatic development using transgenic zebrafish with fluorescently labeled endothelial cells. Two major branches of the vascular endothelial growth factor receptor (VEGFR) signaling pathway were examined: the MAPK and PI3K pathways.Methods: Direct visualization of lymphatic development was performed in control embryos or under chemical inhibition. Treatment involved a 6-hour pulse of inhibitor at 3 days postfertilization. Fish were analyzed for the presence of the thoracic duct (TD) at 4 days postfertilization (n > 100 specimens).Results: Thoracic duct formation was prevented using selective inhibitors against kinases (MAPK, PI3K/TOR, or VEGFR). These kinases were important for TD formation because the lymphatic vessel failed to form in most of treated animals. Remarkably, MAPK pathway inhibition most robustly reduced lymphangiogenesis, demonstrated by a lack of lymphatic endothelial cells.Conclusion: We conclude that MAPK pathway function downstream of the VEGFRs is crucial at the early stages of TD development. This study provides a novel animal model and a potential target pathway for further investigation. We suggest further examination of MAPK pathway deregulation as a potential mechanism underlying lymphatic disease in humans.

Rapamycin-induced tumor vasculature remodeling in rhabdomyosarcoma xenografts increases the effectiveness of adjuvant ionizing radiation

2012-01-01

Abstract: Purpose: Rapamycin inhibits vascular endothelial growth factor expression. Vascular endothelial growth factor is a tumor-elaborated protein that stimulates neovascularization. This inhibition can cause transient “normalization” of the generally dysfunctional tumor vasculature, resulting in improved tumor perfusion and oxygenation. We hypothesized that this may potentiate the antitumor effects of adjuvant ionizing radiation.Methods: Mice bearing orthotopic Rh30 alveolar rhabdomyosarcomas were treated with rapamycin (5 mg/kg intraperitoneally daily ×5). Tumors were then evaluated for changes in intratumoral oxygenation, perfusion, vessel permeability, and microvessel density. Additional tumor-bearing mice were treated with 5 doses of rapamycin, irradiation (4 Gy), or 5 doses of rapamycin with irradiation administered on the first or sixth day of rapamycin treatment.Results: Although tumor vessel permeability changed only minimally, microvessel density decreased (3153 ± 932 vs 20,477 ± 3717.9 pixels per high-power field), whereas intratumoral oxygenation increased significantly (0.0385 ± 0.0141 vs 0.0043 ± 0.0023 mm Hg/mm3) after 5 doses of rapamycin. Contrast-enhanced ultrasound demonstrated a significantly increased rate of change of signal intensity after 5 days of rapamycin, suggesting improved intratumoral perfusion. Tumor volume 14 days after treatment was smallest in mice treated with the combination of rapamycin given before irradiation.Conclusion: Combination therapy with rapamycin given before irradiation to normalize the tumor vasculature, thereby improving tumor oxygenation, increased the sensitivity of alveolar rhabdomyosarcoma xenografts to adjuvant irradiation.

A multicenter evaluation of the role of mechanical bowel preparation in pediatric colostomy takedown

2012-01-01

Abstract: Background: In response to studies in adults that have failed to demonstrate a benefit for mechanical bowel preparation in colonic surgery, we sought to evaluate the utility of mechanical bowel preparation in a multicenter, retrospective study of children who underwent colostomy takedown.Methods: The records of 272 children who underwent colostomy takedown at 3 large children's hospitals were reviewed, and the utilization of mechanical bowel preparation and perioperative antibiotics was noted. Length of stay and the incidences of wound, anastomotic, and other complications were compared.Results: A polyethylene glycol bowel prep was administered to 187 children. All subjects received perioperative, intravenous antibiotics, and 52% of those with bowel preps received preoperative oral antibiotics. Subjects in the bowel prep group had a significantly higher incidence of wound infection (P = .04) and longer length of stay (P = .05). Oral antibiotics did not affect outcome.Conclusions: The use of a mechanical bowel preparation in children before colostomy takedown was associated with a greater risk for wound infection, no protection from other complications, and a longer length of stay. This suggests that bowel preparation may be safely omitted in many children who undergo colonic surgery, thereby reducing cost and discomfort.

Hepatocyte growth factor and omega-3–enriched feeds have a synergistic effect on mucosal mass in an animal model of inflammatory bowel disease

Michael S. Katz, Keith A. Thatch, Marshall Z. Schwartz — 2012-01-01

Abstract: Background: Hepatocyte growth factor (HGF) decreases intestinal inflammation and cytokine levels in an animal model of inflammatory bowel disease (IBD). Luminal omega-3 (OM-3) is anti-angiogenic, reduces inflammation, and may decrease symptoms in patients with Crohn's disease. This study evaluates the synergism of HGF and OM-3.Methods: Twenty adult female transgenic HLA-B27 rats were divided into 4 groups: group 1: regular feeds, IV saline; group 2: OM-3–enriched feeds, IV saline; group 3: regular feeds, IV HGF (150 µg/kg per day); and group 4: OM-3–enriched feeds, IV HGF(150 µg/kg per day). Rats were killed at 14 days after pump placement. Small and large bowel mucosa was harvested, and DNA and protein were extracted and quantified. Statistical analysis was done by analysis of variance with post-hoc Tukey's HSD test.Results: Content of protein and DNA in the ileum were significantly increased by supplementation of HGF (P < .001, P < .01, respectively) alone. OM-3 significantly increased protein content but not DNA (P = .02, P = 0.3, respectively). Combined, they had a synergistic effect greater than either supplement alone (P = .0001, P = .002, respectively). In the colon, HGF and OM-3 did not significantly increase protein or DNA content individually or together.Conclusions: This is the first demonstration of the synergistic effect of a growth factor (HGF) and a dietary supplement (OM-3) in an immunologic model of IBD.

Pediatric chronic ulcerative colitis: does infliximab increase post–ileal pouch anal anastomosis complications?

2012-01-01

Abstract: Background: Total proctocolectomy with ileal pouch anal anastomosis (IPAA) is a common surgical approach to chronic ulcerative colitis (CUC). Preoperative use of Infliximab (IFX) has raised concern of increased postoperative complications. We sought to compare outcomes of pediatric patients (≤18 years) who were treated with IFX before IPAA to those who did not.Methods: Patients (≤18 years of age) who underwent IPAA from 2003 to 2008 for CUC were included, and their records were retrospectively reviewed for preoperative medications, operative technique, and 1-year postoperative complications (leak, wound infection, small bowel obstruction, pouchitis). Subjects were divided into 2 groups—those who received IFX preoperatively and those who did not.Results: Eleven patients received IFX preoperatively, and 27 children did not. All complications following IPAA were more frequent in the IFX group compared to controls (55% vs 26%). Small bowel obstruction was significantly higher in the IFX group (55% vs 7%). Long-term complications occurred in 64% of the IFX group and 61% of the controls.Conclusion: Children that were treated with IFX prior to IPAA suffered twice as many postoperative complications. Long-term outcomes are similar. Currently, we recommend colectomy with end ileostomy for patients that receive IFX within 8 weeks of colectomy for CUC.

Restorative proctocolectomy without diverting ileostomy in children with ulcerative colitis

Brian W. Gray, Robert A. Drongowski, Ronald B. Hirschl, James D. Geiger — 2012-01-01

Abstract: Purpose: The safety of performing a restorative proctocolectomy (RP) and J-pouch ileoanal anastomosis (IPAA) without diverting ileostomy for children with ulcerative colitis (UC) is a subject of extensive debate. Our goal was to examine pediatric outcomes of RP and IPAA without ileostomy.Methods: We performed a single-institution review of UC patients who had RP and IPAA with (+Ostomy) or without (−Ostomy) diverting ileostomy from 2002 to 2010. Surgeon and patient preference determined ileostomy decision. The study included 50 patients (28 +Ostomy, 22 –Ostomy).Results: Preoperative demographics were similar between 2 groups in age (13.5 ± 3.5 years –Ostomy, 14.3 ± 3 years +Ostomy), serum albumin (3.6 ± 0.7 –Ostomy, 3.6 ± 0.7 +Ostomy), body mass index (20.8 ± 6.9 −Ostomy, 21.3 ± 8.6 +Ostomy), and daily corticosteroid dose (22.4 ± 17.7 mg −Ostomy, 23.5 ± 13.7 mg +Ostomy). Operating time was less in –Ostomy with mean times of 6:22 ± 2:04 vs 9:07 ± 2:57. The –Ostomy group required fewer ileoanal anastomotic dilations per patient (0.4 ± 0.8 vs 1.4 ± 1.9). Functional outcomes were not significantly different regarding pouchitis episodes per patient (0.6 ± 1.1 –Ostomy, 0.6 ± 1.1 +Ostomy), daily bowel movements (5.5 ± 1.9 –Ostomy, 6.7 ± 4.0 +Ostomy), and daily postoperative loperamide dose (8.4 ± 4.3 mg –Ostomy, 6.8 ± 4.0 mg +Ostomy).Conclusion: Short- and long-term outcomes can be equivalent in patients with and without diverting ileostomy, but questions remain regarding patient selection and quality of life impact.

Is daily dilatation by parents necessary after surgery for Hirschsprung disease and anorectal malformations?

Sara J. Temple, Anna Shawyer, Jacob C. Langer — 2012-01-01

Abstract: Background/Purpose: Most surgeons recommend daily dilatation after surgery for Hirschsprung disease and anorectal malformations. Our goal was to critically evaluate the potential risks and benefits of this practice.Methods: A retrospective chart review was carried out of all children undergoing repair of Hirschsprung disease or anorectal malformation over 5 years. Patients with long segment Hirschsprung disease or anal stenosis were excluded.Results: There were 95 patients, of which 34 had Hirschsprung disease and 61 had an anorectal malformation. Postoperatively, 65 underwent routine dilatation by parents; and 30 underwent weekly calibration by the surgeon, with daily dilatation by the parents only if the anastomosis was felt to be narrow. Of the 30 children undergoing weekly calibration, only 5 (17%) developed late narrowing that required conversion to the daily parental dilatation approach. There were no significant differences between the 2 approaches with respect to stricture development, anastomotic disruption, perineal excoriation, or enterocolitis.Conclusion: Weekly calibration by the surgeon is associated with similar outcomes to daily dilatation by the parents. Because this approach is kinder to the parents and the child, it should be seriously considered for the postoperative management of children with Hirschsprung disease or anorectal malformations.

Pediatric hernia repair: 1-stop shopping

2012-01-01

Abstract: Purpose: Multiple visits for the evaluation, treatment, and follow-up of straightforward surgical problems are inconvenient, can result in lost work for the parents, and missed school for the child. We hypothesized that with proper previsit screening, patients with select diagnoses can be evaluated in an outpatient clinic setting and undergo operation the same day.Methods: Criteria were developed to identify straightforward referrals to our surgical practice for umbilical, epigastric, or inguinal hernias. Scripting was created to offer families the option of consultation and, if indicated, surgical treatment on the same day. Data collected included number of patients, cases performed, insurance status, and consultation reimbursement and surgical fees. Families were surveyed postoperatively.Results: Sixty-one patient candidates participated. The diagnosis and indication for surgery were confirmed in 56 (92%), of which 50 underwent repair the day of their consultation. Seventy-two percent of patients had commercial insurance, whereas 28% had Medicaid. The preoperative consultation fee was reimbursed in 39 (78%) of 50 encounters (57% Medicaid, 86% commercial). All surgical cases were reimbursed. Patient and family satisfaction was high.Conclusions: We conclude that it is feasible to provide same-day evaluation and service for straightforward pediatric hernias with acceptable financial reimbursement and high parent satisfaction.

Overnight observation in former premature infants undergoing inguinal hernia repair

2012-01-01

Abstract: Background: Overnight observation for apneic events is standard practice in former preterm infants. However, the literature supporting current protocols is dated. Therefore, we retrospectively evaluated the post-anesthetic risks in these patients.Methods: A retrospective review was conducted on former preterm infants admitted after an inguinal herniorrhaphy between 1/00 and 10/09. The protocol for overnight admission was for patients born before 37 weeks gestation who are less than 60 weeks post-conceptional age (PCA).Results: There were 363 patients, of which 23 were <40 weeks PCA (group 1), 244 were 40 to 49.9 weeks PCA (group 2), and 96 were 50 to 60 weeks PCA (group 3). Events registered by alarms occurred in 4 patients (1.1%), 2 from group 1 and 2 from group 2. In Group 1, one occurred during nasogastric tube placement and resolved spontaneously. In group 2, one was apnea-induced bradycardia that resolved spontaneously, and one was in a patient on home monitors with an event similar to home reports. There were no events in group 3.Conclusion: Conservative guidelines for overnight observation after inguinal hernia repair could be set for patients born before 37 weeks gestation who are under 50 weeks PCA.

Using robotic telecommunications to triage pediatric disaster victims

2012-01-01

Abstract: Purpose: During a disaster, hospitals may be overwhelmed and have an insufficient number of pediatric specialists available to care for injured children. The aim of this study was to determine the feasibility of remotely providing pediatric expertise via a robot to treat pediatric victims.Methods: In 2008, Los Angeles County held 2 drills involving telemedicine. The first was the Tri-Hospital drill in which 3 Los Angeles County hospitals, one being a pediatric hospital, participated. The disaster scenario involved a Metrolink train crash, resulting in a large surge of traumatic injuries. The second drill involved multiple agencies and was called the Great California Shakeout, a simulated earthquake exercise. The telemedicine equipment installed is an InTouch Health, Inc, Santa Barbara, CA robotic telecommunications system. We used mixed-methods to evaluate the use of telemedicine during these drills.Results: Pediatric specialists successfully provided remote triage and treatment consults of victims via the robot. The robot proved to be a useful means to extend resources and provide expert consult if pediatric specialists were unable to physically be at the site.Conclusion: Telemedicine can be used in the delayed treatment areas as well as for training first receivers to collaborate with specialists in remote locations to triage and treat seriously injured pediatric victims.

Parenteral nutrition–associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review

2012-01-01

Abstract: Objective: The aim of this study was to review evidence-based data addressing key clinical questions regarding parenteral nutrition–associated cholestasis (PNAC) and parenteral nutrition–associated liver disease (PNALD) in children.Data Source: Data were obtained from PubMed, Medicine databases of the English literature (up to October 2010), and the Cochrane Database of Systematic Reviews.Study Selection: The review of PNAC/PNALD has been divided into 4 areas to simplify one's understanding of the current knowledge regarding the pathogenesis and treatment of this disease: (1) nonnutrient risk factors associated with PNAC, (2) PNAC and lipid emulsions, (3) nutritional (nonlipid) considerations in the prevention of PNAC, and (4) supplemental medications in the prevention and treatment of PNAC.Results: The data for each topic area relevant to the clinical practice of pediatric surgery were reviewed, evaluated, graded, and summarized.Conclusions: Although the conditions of PNAC and PNALD have been well recognized for more than 30 years, only a few concrete associations and treatment protocols have been established.

Probiotic supplement reduces risk of necrotizing enterocolitis and mortality in preterm very low-birth-weight infants: an updated meta-analysis of 20 randomized, controlled trials

Quanzhen Wang, Jing Dong, Yimin Zhu — 2012-01-01

Abstract: Purpose: Necrotizing enterocolitis (NEC) is the most common acquired disease of the gastrointestinal tract in preterm infants, whereas probiotic supplementation might reduce NEC risk and potentially provide benefits to preterm infants. We performed an updated meta-analysis of all relevant randomized, controlled trials to assess the benefits of probiotic supplementation for preterm very low-birth-weight (VLBW) infants.Methods: We searched in PubMed, Embase, and Chinese BioMedical Literature Database (CBM) databases, and 20 randomized, controlled trials (a total of 3816 preterm VLBW infants) were finally included into this meta-analysis. Incidence and relative risk (RR) were calculated using a random-effects or fixed-effects model depending on the heterogeneity of the included studies.Results: Probiotic supplement was associated with a significantly decreased risk of NEC in preterm VLBW infants (RR = 0.33; 95% confidence interval [CI], 0.24-0.46; P < .00001). Risk of death was also significantly reduced in the probiotic group (RR = 0.56; 95% CI, 0.43-0.73; P < .0001). There was no difference in the risk of sepsis between the probiotic group and placebo group (RR = 0.90; 95% CI, 0.71-1.15; P = .40).Conclusions: Probiotic supplement can reduce risk of NEC and mortality in preterm VLBW infants. However, the optimum type of probiotic supplement and the long-term effects need further study.

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

2012-01-01

Trichoblastoma: management of a rare skin lesion

Kristen A. Zeller, Deborah F. Billmire — 2012-01-01

Abstract: A 15-year-old girl presented with a 2-year history of an enlarging parietal scalp lesion. Excisional biopsy revealed trichoblastoma, a benign secondary tumor arising from a nevus sebaceous. There is no consensus in the literature about the surgical management of nevus sebaceous in children owing to the low incidence of secondary malignancy. There is a 20% lifetime risk of transformation to a soft tissue neoplasm, most of which are benign; but malignant neoplasms have also been reported. At minimum, these lesions need to be followed clinically over the patient's lifetime if left in situ. Recurrence after excision is rare.

Gastroportal shunt for portal hypertension in children

2012-01-01

Abstract: Purpose: Extrahepatic portal venous obstruction is the most common cause of portal hypertension in children. The Rex shunt has been used successfully to treat patients with extrahepatic portal hypertension. In the conventional Rex shunt, the internal jugular vein is used as a venous graft. Inevitably, such a procedure requires neck exploration and sacrifice of internal jugular vein. The authors describe a novel adaptation of gastroportal shunt, successfully carried out in 8 children with extrahepatic portal hypertension.Methods: The mean age of the 8 patients (6 boys and 2 girls) was 66.6 months at the time of operation. All children had portal hypertension. Seven had a history of upper gastrointestinal bleeding, and 4 had splenomegaly and hypersplenism. Gastroportal shunt was performed in all patients. The left gastric vein was mobilized and anastomosed to left portal vein. In 1 patient, the left gastric vein was not of adequate length and required a venous graft (the inferior mesenteric vein). All patients were followed up for 3 to 20 months (median, 9 months).Results: The gastroportal shunt was successfully performed in all patients. The median operative time was 265 minutes (range, 205-360 minutes). Operative blood loss was 21 ± 7.4 mL, and the length of hospital stay varied from 9 to 19 days (median, 15 days). Intraoperative portal venous angiography demonstrated the patency of the shunt in all patients. Postoperatively, the complete blood count normalized, and the biochemistry tests were within reference range. Postoperative ultrasound confirmed shunt patency and satisfactory flow in the gastroportal shunt in each patient. The size of spleen decreased. There was no recurrence of variceal bleeding.Conclusions: The gastroportal shunt is an effective treatment of extrahepatic portal hypertension.

Simplified technique for minimally invasive repair of congenital diaphragmatic hernia using hollow-needle snare and transthoracic traction stitches

Ayman Al-Jazaeri — 2012-01-01

Abstract: Background: Minimally invasive (MI) congenital diaphragmatic hernia (CDH) repair can be challenging. Placing rib-anchoring stitches without creating skin incisions and closing wider defects are some of the difficulties. In Bochdaleck hernia repair, maintaining visceral reduction and minimizing pneumothorax use are additional obstacles. We describe the use of hollow-needle snares (HNS) and transthoracic traction stitches (TTS) to overcome these challenges.Methods: Hollow-needle snares is assembled by passing a prolene stitch through a hollow needle creating a retractable snare, which is used to extract the placed anchoring stitches by passing it over the ribs but through the same stitch's skin entrance site. In Bochdaleck hernia, the early placement of TTS using HNS can facilitate visceral reduction, patch lay down, and tension-free closure of possible residual V-shaped defects.Results: Between July 2009 and April 2011, we performed 10 consecutive MI CDH repairs for 9 patients, including 7 Bochdaleck and 3 Morgagni hernias. The median age was 8 days (range, 3-172 days), and the mean operative time was 148.5 ± 37.8 minutes for Bochdaleck hernia repairs. For Morgagni hernia, the median age was 18.3 months (range, 10.5-37 months), and the mean operative time was 100 ± 26.5 minutes. All cases were completed without conversion. One patient had a hernia recurrence and was repaired similarly, whereas the others had uneventful recovery at a median follow-up of 5.8 months (range, 1.1-23.7 months).Conclusion: Hollow-needle snare and TTS are simple and available tools that can facilitate MI repair of CDH. This initial experience demonstrates the technique's effectiveness and its excellent cosmetic outcomes.

A.J.A. Holland — 2012-01-01

The authors report their initial experience with propranolol, a nonselective β-blocker, used to treat subglottic hemangiomas (SGHs) in 10 patients referred to 1 of 2 pediatric teaching hospitals in Sydney, Australia, and Auckland, New Zealand. Subglottic hemangiomas remain uncommon but may cause airway compromise, with tracheostomy required in 39% in addition to traditional treatment with corticosteroids and/or surgical excision.

A.J.A. Holland — 2012-01-01

Although common, supracondylar fractures result in pulse loss in less than 14% of cases and a white pulseless hand in just 1%. Optimal management of a warm but pulseless hand after reduction remains controversial, as does the approach to clinic-radiologic review and the treatment of any subsequent stenosis.

Thomas A. Angerpointner — 2012-01-01

Self-filling, hydrogel-based osmotic tissue expanders have been successfully used for several years mainly in adult patients. The authors tested this novel device in pediatric plastic and reconstructive surgery. Fifty-three osmotic expanders were implanted following standard surgical principles in a total of 30 children and adolescents with burn scars, congenital nevi, alopecia, and foot deformities. All expanders reached their predicted volume within 6 weeks, and 51 (96.2%) produced a sufficient amount of additional skin for the intended coverage of the defect.

Sigmund H. Ein — 2012-01-01

This article was written by the surgeon-in chief (Dr Wright) and coauthored by the pediatrician-in-chief, vice president of corporate strategy and performance, and chief of research, all at the Hospital for Sick Children, Toronto, Ontario, Canada. As such, it may be both of some interest and value for other academic health science centers (AHSCs) or teaching hospitals. The authors wonder about “the future role for AHSC” and propose several strategies to enhance innovation (which they refer to as “that historical strength”). (1) The AHSC should consider turning to the private sector to implement tactics that have been shown to promote and sustain a culture of innovation. The authors say that the need for innovation has spread beyond research to many areas including education, managing institutions, and increasing efficiency. (2) Leadership has a key role to play in promoting innovation by creating the necessary structures, processes, and reward systems. (3) How people are organized may also have a substantially influence on creativity and innovation. Alternative models (matrix organizations) that focus on integration bring multiple disciplines together around a single theme. (4) The skills and attitudes of people working in AHSC will affect performance, culture, direction, and willingness to change. Multidisciplinary approaches are needed to create new approaches to problems. Creating a climate that encourages brainstorming and welcoming ideas will tend to bring out innovative ideas. (5) The organization must find ways to recognize and reward innovation across all activities. Innovation requires support, and individuals require time to be creative. Developing and evaluating innovative ideas will often benefit from seed money or innovative funds. (6) An enduring theme of research is placing offices of individuals or groups of researchers together, which strongly influences the probability of collaboration. Overall, the authors feel that “the time is ripe for innovation as a key strategy to preserve and enhance the clinical and academic mission of teaching hospitals.” However, they also state that although “the private sector has tested and implemented many strategies that have direct relevance to AHSC,” they have “yet to be tried in the academic setting.”

Federico G. Seifarth — 2012-01-01

Surgical repair of pectus excavatum (PE) in childhood is a well-established procedure. Previously used operative techniques to correct PE were largely based on the Ravitch technique. Since about 10 years, the minimally invasive repair of PE (MIRPE) by Nuss is well established. Conservative treatment with the vacuum bell to elevate the funnel in patients with PE represents a potential alternative to surgery in selected patients. A suction cup is used to create a vacuum at the anterior chest wall. Three different sizes of vacuum bell exist that are selected according to the individual patients' age. When creating the vacuum, the lift of the sternum is obvious and remains for a different period. The device should be used for a minimum of 30 minutes (twice per day) and may be used up to a maximum of several hours daily. One hundred thirty-three patients (110 men and 23 women) aged from 3 to 61 years (median, 16.21 years) used the vacuum bell for 1 to a maximum of 36 months. Computed tomographic scans showed that the device lifted the sternum and ribs immediately. In addition, this was confirmed thoracoscopically during the MIRPE procedure. One hundred five patients showed a permanent lift of the sternum for more than 1 cm after 3 months of daily application. Thirteen patients stopped the application and underwent MIRPE. Relevant side effects were not noted. The vacuum bell has proved to be an alternative therapeutic option in selected patients having PE. The initial results proved to be dramatic, but long-term results are, so far, lacking. Further evaluation and follow-up studies are necessary.

Thomas A. Angerpointner — 2012-01-01

The authors report on their experience with a modified Spitz procedure using a Collis gastroplasty for the repair of long-gap esophageal atresia (EA). The authors reviewed 5 cases of children with long-gap (5-6 vertebral bodies) EA. The repair was carried out 3 months after permanent monitoring, enteral nutrition via gastrostomy tube, and permanent aspiration of the proximal esophagus. The Collis gastroplasty was performed to create a gastric tube along the lesser gastric curvature using an Endo-GIA linear stapler. The gastric tube can be easily mobilized into the thorax after ligation of the gastric vessels and anastomosed to the proximal esophageal segment. There were no introperative complications. The median postoperative hospital stay was 39 days (range, 30-60 days) with a median follow-up of 20 months (range, 16-29 months). During the early follow-up period, a digestive occlusion occurred in 1 patient, and several months later, she had hiatal herniation. Other postoperative complications included anastomotic stricture (n = 2) treated by dolatations, gastroesophageal reflux (n = 3), and weight gain cirfiay (n = 3) requiring additional enteral nutrition in 2 cases. No mortality was seen in the series. The authors propose a modified Spitz procedure using a Collis gastroplasty in the primary repair of long-gap EA as an alternative option to esophageal replacement or elongation techniques. The complications noted are common complications of EA surgical repair.

A.J.A. Holland — 2012-01-01

Three boys with ultrasound-confirmed infantile hypertrophic pyloric stenosis presented with minimal electrolyte imbalance and mild clinical dehydration. All 3 had been treated for presumed gastroesophageal reflux with ranitidine for a period of 2 to 5 weeks. There was a limited biochemical response to “resuscitation” with half normal saline and 5% dextrose. Similar findings have been reported with patients on omeprazole, and 1 study has advocated cimetidine therapeutically in infantile hypertrophic pyloric stenosis to reduce the need for surgery.

Federico G. Seifarth — 2012-01-01

The aim of this study was to investigate the importance of the ileocaecal valve and its reconstruction in patients who are not having short bowel syndrome and Crohn disease. Case notes of 99 children with hemicolectomy and 24 children with terminal ileal resection were reviewed and sorted into 3 groups: group 1, ileocaecal valve resection (limited hemicolectomy); group 2, hemicolectomy; and group 3, terminal ileal resection between 10 and 25 cm. Patients with Crohn, short bowel syndrome, and incomplete follow-up were excluded. Chronic diarrhea was documented in 7 (27%) of 26 cases in group 1, 6 (26%) of 23 patients in group 2, and none of the 13 patients had diarrhea in group 3. Pearson χ2 test showed significant difference between groups 1 and 3 (P = .038) and between groups 2 and 3 (P = .043). However, there was no significant difference between groups 1 and 2 (P = .947). The authors conclude that chronic diarrhea is a significant complication after limited hemicolectomy not only in Crohn disease and but also in short bowel syndrome. This is likely to originate from the loss of the ileocecal valve itself rather than the loss of the ileal or colonic segment. These results justify attempts to reconstruct the ileocecal valve.

Federico G. Seifarth — 2012-01-01

A link between factors governing brain development and the development of the enteric nervous system is not surprising because both processes are largely controlled by the same or similar neural growth factors that are expressed at more or less in the same spatio-temporal time frame. Hirschsprung disease occurs as an isolated phenotype in 70% of cases but is associated with other congenital abnormalities and syndromic phenotypes in the remainder, with central nervous system (CNS) anomalies making up 6.78%. These associations may be underestimated and are possibly pathogenetically linked to genetic associations and probable gene-gene interaction. In this review, the author explores known syndromes and other enteric nervous system associations of Hirschsprung disease, looking at possible pathogenetic associations. He points out that borderline cognitive abilities, attention-deficit disorders, and possible epileptic seizures in patients with Hirschsprung disease should be fully investigated. The author recognizes that this group of patients remains a challenge from a clinical and functional management point of view and suggests possible management guidelines.

Federico G. Seifarth — 2012-01-01

The enteric nervous system (ENS) is a network of neurons and glia that lies within the gut wall. It is responsible for the normal regulation of gut motility and secretory activities. Hirschsprung disease (HD) is a congenital defect of the ENS characterized by an absence of ganglia in the distal colon. Intestinal neuronal dysplasia (IND) is a condition that clinically resembles HD, characterized by hyperganglionosis and giant and ectopic ganglia, resulting in intestinal dysmotility. Intestinal ganglioneuromatosis is characterized by hyperplasia and hypertrophy of enteric neuronal cells and causes chronic intestinal pseudoobstruction. Phosphatase and tensin homolog deleted on chromosome 10 (Pten) is a phosphatase that is critical for controlling cell growth, proliferation, and cell death. A recent study of Pten knockout mice showed evidence of ganglioneuromatosis in the ENS, suggesting a role for this protein in ENS development. Patients with ganglioneuromatosis have also been shown to have a decreased level of Pten expression in the colon. The aim of this study was to investigate Pten expression in the ENS of HD and patients with IND compared with healthy controls. Resected tissue from 10 patients with HD and 10 patients with IND type B was fixed and embedded in paraffin wax. Normal control colon tissue was obtained from 10 patients who underwent a colostomy closure for imperforate anus. Sections were cut, and immunohistochemistry was carried out using a Pten antibody. Results were analyzed by light microscopy. Staining showed that Pten was strongly expressed in ganglia of both the submucosal and myenteric plexus of normal and HD specimens from the ganglionic colon. Pten expression was significantly reduced in the giant ganglia in patients with IND in both the myenteric and submucosal plexuses compared with the healthy controls. Specimens from the aganglionic region of HD did not show Pten expression. This is the first study demonstrating a marked reduction of Pten expression in the myenteric and submucous plexuses of patients with IND. Neuronal Pten deficiency in IND may disrupt the chemical pathway associated with the proliferation and development of neuronal cells forming mature ganglia and thus cause motility dysfunction.

A.J.A. Holland — 2012-01-01

The authors report a male infant with a left-sided, infradiaphragmatic mixed pulmonary sequestration (PS) and type II congenital cystic adenomatoid malformation. The mass was detected as an isolated finding on a routine antenatal ultrasound scan at 19 weeks of gestation. Postnatal ultrasound confirmed the 3.5-cm mass that was associated with normal urinary catecholamines. At 3 months, the mass had increased in size to 5 cm, and a computed tomogram revealed that it was supplied by a single vessel from the celiac axis. A laparoscopic approach was unsuccessful because of the fibrotic nature of the mass, dense-surrounding adhesions, and inability to clearly visualize the feeding vessel. After conversion to an open procedure, the mass was excised and identified on subsequent histology. This case adds to the increasing number of mixed PS and congenital cystic adenomatoid malformations reported in the literature, suggesting a common embryological origin.

Thomas A. Angerpointner — 2012-01-01

The aim of this study was to report the authors' experience with tubularized incised plate repair of hypospadias without placement of a postoperative urethral stent in 41 cases.

Thomas A. Angerpointner — 2012-01-01

The aim of this study sparing was to explore the true efficacy and potential of advantages of lymphatic-sparing laparoscopic varicocelectomy (LSV) over lymphatic non-LSV (LNSV) in children and adolescents.

Federico G. Seifarth — 2012-01-01

Since 1999, complete primary repair of bladder exstrophy has represented a valid alternative in the treatment for patients with exstrophy, offering 1-stage reconstruction for all components of this malformation in newborns. Most cases are currently approached within 48/72 hours of life, and risk of vascular injury to penile glans and/or corpora has been reported with increased frequency with this procedure. The authors from the urology department of the Bambino Gesu' Children's Hospital in Rome/Italy report their initial experience with a delayed approach to complete repair, with the bladder plate left intact and taken care at home by the parents while awaiting for the patient to reach adequate weight. Delayed approach enabled them to preoperatively stimulate phallic size with testosterone, a treatment that was, so far, confined only to redo or failed cases. Six male patients with exstrophy were treated over a 3-year period (2007-2009). After initial workup, newborns were discharged home, with the bladder plate taken care by the parents. A weight of 4500 g was arbitrarily deemed satisfactory for surgery. While at home, patients underwent preoperative testosterone stimulation (testosterone enanthate, 4 biweekly administrations of 100 mg/m2 body surface). In each case, biopsies of bladder mucosa were taken at time of surgery. Complications, age at surgery, and increases in phallic size were extracted from clinical and surgical case notes. Weight at surgery ranged from 4510 to 5600 g. Age range was 43 to 91 days. Mean increase in phallic size after testosterone stimulation was 8.3 mm. Three complications were observed: 2 were suprapubic fistulas; of these, 1 closed spontaneously, and 1 required surgery subsequently. In 1 fascial dehiscence, emergency closure was needed. Hypospadias occurred in all patients. All histologic specimens demonstrated a mildly inflamed bladder mucosa. The authors conclude that delayed repair of bladder exstrophy is feasible in patients who have reached adequate weight and stabilization; if adequately cared for, the bladder plate shows minimal inflammation at the time of surgery. Deferring surgery also offers the advantages of preoperative testosterone stimulation and promotion of mother-infant relationship as well as the transfer to centers with adequate experience and proficiency in all aspects of bladder exstrophy reconstruction.

A.J.A. Holland — 2012-01-01

The authors report their experience with 61 consecutive fetuses with severe megacystis (MC) managed at a tertiary center in Victoria, Australia, between 1988 and 2008. Severe MC was defined as a fetal bladder surgical length greater than the gestational age plus 12 years, and nonsyndromic fetuses with multisystem anomalies were excluded. Of 61 singleton pregnancies, 52 were boys. Four fetuses had trisomy 18 and did not survive. Autopsy was not performed on 2 terminations, and 1 fetus was lost to placental abruption. The most common cause of MC was posterior urethral valves (n = 17) followed by Prune-belly syndrome (n = 12) in boys and urethral stenosis (n = 3) in girls.

A rare case of ovarian juvenile granulosa cell tumor without precocious pseudopuberty in an 11-month-old infant

2012-01-01

Ovarian juvenile granulosa cell tumors (JGCTs) in children younger than 1 year are exceptional and generally present with isosexual precocious pseudopuberty . We describe a new case of JGCT occurring in an 11-month-old girl, presenting without hormonal manifestations..

Announcements of Future Meetings

2012-01-01

Comparison of 5 intravenous lipid emulsions and their effects on hepatic steatosis in a murine model

Jonathan Meisel — 2012-01-01

In the article “Comparison of 5 intravenous lipid emulsions and their effects on hepatic steatosis in a murine model” (J Pediatr Surg. 2011;46(4):666-673), contained some incorrect data. The correct table is presented below.

Successful management of congenital bronchial stenosis using an expandable stent

2012-01-01

Abstract: Congenital bronchial stenosis is a very rare cause of neonatal dyspnea. Surgical management remains challenging in small children. We report successful implantation of a bronchial stent in a 3-month-old female infant presenting with congenital right bronchial stenosis and 18 months of follow-up. Use of stents in children remains controversial because of the problem of size mismatch as the child grows. Nevertheless, expandable stent implantation could be an interesting alternative to complex surgery for localized bronchial stenosis in neonates.

Esophageal bronchus in an infant—a rare cause of recurrent pneumonia

Ashwin P. Pimpalwar, Saif F. Hassan — 2012-01-01

Abstract: A 4-month-old female infant presented to our institute with recurrent pneumonia and was diagnosed with a right lower lobe esophageal bronchus. This congenital anomaly is extremely rare with very few cases reported in the literature. We describe the diagnostic workup and management that led to a successful outcome.

Solitary Langerhans cell histiocytosis arising from sternum: a case report

Tae Hwan Park, June-kyu Kim, Tae Yun Oh, Yun Joo Park — 2012-01-01

Abstract: Langerhans cell histiocytosis (LCH) is a rare idiopathic benign disease characterized by proliferation of Langerhans cells, most commonly in the skull. In extremely rare cases, the tumor can occur in the sternum. A 4-year-old girl presented to our institution with a firm, nontender mass in her anterior chest that had indolently enlarged to approximately 2 cm in diameter over the previous several months. Computed tomography, magnetic resonance imaging, and F18-flurodeoxy-glucose positron emission tomography computed tomography were performed for preoperative diagnosis, but the findings were inconclusive. Therefore, we performed surgical excision under general anesthesia, followed by microscopic and immunohistochemical analysis of the excised specimen. The mass was eventually diagnosed as a LCH arising from the sternum. No postoperative oncological treatment was given, and follow-up has continued for 1 year until the time of writing without any tumor recurrence. To our knowledge, only 10 cases of LCH arising from the sternum have been reported in the medical literature. Among them, surgical approaches including curettage and partial sternotomy were performed in only 6 cases. Without exception, all patients experienced excellent clinical outcomes. Therefore, additional clinical experiences are required. No standard treatment of choice for this disease currently exists. In our experience, curettage of the involved soft tissue mass and bone followed by appropriate reconstruction of the defect is considered a good option for the treatment of solitary LCH of the sternum. In addition, LCH should be considered in the differential diagnoses when a sharp delineated osteolytic mass is detected in the sternum.

Poland syndrome with extracorporeal intercostal liver herniation and thoracic myelomeningocele

2012-01-01

Abstract: Poland syndrome is characterized by hypoplastic unilateral chest wall structures. These chest wall deformities may be associated with upper extremity anomalies.The association of Poland syndrome with either intercostal liver herniation or a spinocerebral deformity has been described, but there is no report of both findings encountered simultaneously.This is the first report of a newborn child with Poland syndrome associated with an intercostal liver segment herniation and thoracic myelomeningocele with features of an Arnold-Chiari II cerebral malformation.

Ulcerative colitis of the neovagina as a postsurgical complication of persistent cloaca

2012-01-01

Abstract: Persistent cloaca is characterized by a common vestibule connected to the bladder, uterus, and rectum and a single perineal orifice to the exterior. Treatment can involve vaginal reconstruction with sigmoid/descending colon. We report a case of an 8-year-old girl who, as an infant, underwent vaginoplasty using sigmoid colon for persistent cloaca and developed a fulminant colitis that also affected the colonic neovagina 2 years after her reconstruction.

Partial nephrectomy for a large renal lymphatic malformation in a child presenting with hypertension

2012-01-01

Abstract: A 9-year-old girl had hypertension (systolic blood pressure of 125 mm Hg) noted at an annual well child visit. An ultrasound study demonstrated a large right renal cystic mass. A partial nephrectomy was performed. The surgical specimen was 9.7 × 9.4 × 6.4 cm and weighed 413.2 g. The tumor stained diffusely positive for smooth muscle actin and focally positive for factor VIII. Final histologic diagnosis was primary intrarenal lymphatic malformation. The case is unusual because of the presentation, size of the mass, as well as the therapeutic approach of partial nephrectomy.

Preduodenal portal vein, malrotation, and high jejunal atresia: a case report

Maciej Baglaj, Sylwester Gerus — 2012-01-01

Abstract: Preduodenal portal vein (PDPV) is a rare congenital anomaly. In most patients, it is associated with other congenital defects including situs inversus, malrotation, and biliary atresia or occurs as part of the heterotaxia syndrome or polysplenia syndrome. We describe a newborn affected by high jejunal atresia, malrotation, and a complex cardiac anomaly, in whom PDPV was diagnosed at early relaparotomy because of stenosis of the jejunal anastomosis. Occurrence of PDPV with intestinal atresia has not been previously reported in the literature.

2012-01-01

Journal of Pediatric Surgery

Health care quality, access, cost, workforce, and surgical education: the ultimate perfect storm

Notes on the early development of pediatric surgery in the United States

Regenerative medicine strategies

The history of paediatric surgery in the United Kingdom and the influence of the national health service on its development

Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection

Defining hydrops and indications for open fetal surgery for fetuses with lung masses and vascular tumors

Tracheoesophageal displacement index and predictors of airway obstruction for fetuses with neck masses

Long-term morbidity after fetal endoscopic surgery for severe twin-to-twin transfusion syndrome

The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital diaphragmatic hernia

Improved survival in venovenous vs venoarterial extracorporeal membrane oxygenation for pediatric noncardiac sepsis patients: a study of the Extracorporeal Life Support Organization registry

Extracorporeal membrane oxygenation cannulation trends for pediatric respiratory failure and central nervous system injury

Utility of neuroradiographic imaging in predicting outcomes after neonatal extracorporeal membrane oxygenation

Split abdominal wall muscle flap repair vs patch repair of large congenital diaphragmatic hernias

Congenital lung anomalies: can we postpone resection?

Pyloromyotomy: randomized control trial of laparoscopic vs open technique

A novel antireflux procedure: gastroplasty with restricted antrum to control emesis (GRACE)

Agency for Healthcare Research and Quality pediatric indicators as a quality metric for surgery in children: do they predict adverse outcomes?

Quality improvement and patient care checklists in intrahospital transfers involving pediatric surgery patients

The jury is still out: changes in gastroschisis management over the last decade are associated with both benefits and shortcomings

Long-term neurodevelopmental outcomes in children born with gastroschisis: the tiebreaker

Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism

Development of an endoluminal intestinal lengthening capsule

Evaluation of intestinal viability using 3–charge coupled device image enhancement technology in a pediatric laparoscopic appendectomy model

Epidural vs patient-controlled analgesia for postoperative pain after pectus excavatum repair: a prospective, randomized trial

Magnetic mini-mover procedure for pectus excavatum III: safety and efficacy in a Food and Drug Administration-sponsored clinical trial

Optoelectronic plethysmography demonstrates abrogation of regional chest wall motion dysfunction in patients with pectus excavatum after Nuss repair

Lessons from a liver hemangioma registry: subtype classification

Circulating thyrotropin receptor messenger RNA for evaluation of thyroid nodules and surveillance of thyroid cancer in children

Novel zebrafish model reveals a critical role for MAPK in lymphangiogenesis

Rapamycin-induced tumor vasculature remodeling in rhabdomyosarcoma xenografts increases the effectiveness of adjuvant ionizing radiation

A multicenter evaluation of the role of mechanical bowel preparation in pediatric colostomy takedown

Hepatocyte growth factor and omega-3–enriched feeds have a synergistic effect on mucosal mass in an animal model of inflammatory bowel disease

Pediatric chronic ulcerative colitis: does infliximab increase post–ileal pouch anal anastomosis complications?

Restorative proctocolectomy without diverting ileostomy in children with ulcerative colitis

Is daily dilatation by parents necessary after surgery for Hirschsprung disease and anorectal malformations?

Pediatric hernia repair: 1-stop shopping

Overnight observation in former premature infants undergoing inguinal hernia repair

Using robotic telecommunications to triage pediatric disaster victims

Parenteral nutrition–associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review

Probiotic supplement reduces risk of necrotizing enterocolitis and mortality in preterm very low-birth-weight infants: an updated meta-analysis of 20 randomized, controlled trials

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

Trichoblastoma: management of a rare skin lesion

Gastroportal shunt for portal hypertension in children

Simplified technique for minimally invasive repair of congenital diaphragmatic hernia using hollow-needle snare and transthoracic traction stitches

A rare case of ovarian juvenile granulosa cell tumor without precocious pseudopuberty in an 11-month-old infant

Announcements of Future Meetings

Comparison of 5 intravenous lipid emulsions and their effects on hepatic steatosis in a murine model

Successful management of congenital bronchial stenosis using an expandable stent

Esophageal bronchus in an infant—a rare cause of recurrent pneumonia

Solitary Langerhans cell histiocytosis arising from sternum: a case report

Poland syndrome with extracorporeal intercostal liver herniation and thoracic myelomeningocele

Ulcerative colitis of the neovagina as a postsurgical complication of persistent cloaca

Partial nephrectomy for a large renal lymphatic malformation in a child presenting with hypertension

Preduodenal portal vein, malrotation, and high jejunal atresia: a case report

Table of Contents