Advances in the Surgical Treatment of Gastroschisis.

Gastroschisis (GS) is a structural defect of the anterior abdominal wall, usually diagnosed antenatally, that occurs with a frequency of approximately 4 per 10,000 pregnancies. Babies born with GS require neonatal intensive care and surgical management of the abdominal wall defect soon after birth. Although contemporary survival rates for GS are over 90%, these babies are at risk for significant morbidity, and require 4 to 6 weeks of costly, resource-intensive care in specialized neonatal units. Much consideration has been given to how best to treat the abdominal wall defect of GS. The traditional approach, necessitated by a need to establish enteral feeding as quickly as possible, consists of early postnatal visceral reduction and sutured abdominal closure. Advances in neonatal nutritional support have enabled the development of surgical approaches, which permit gradual visceral reduction and delayed abdominal closure. In cases where early visceral reduction cannot be achieved, delayed closure enabled by the initial placement of a prosthetic silo has been a live-saving alternative. The development of preformed silos has simplified their use and led to an interest in treating all cases with a delayed closure philosophy. Most recently, a sutureless technique of abdominal closure has been reported, which has the benefit of avoiding general anesthesia and offers other outcome improvements over sutured closure of the defect. The debate over primary closure versus silo placement and delayed closure continues to receive much attention. The goal of this article is to review historical aspects of gastroschisis closure, and then focus on current surgical techniques, including the innovative sutureless closure, with an analysis of the comparative clinical effectiveness of these approaches to treatment of the abdominal wall defect in GS.

Management of the base of the appendix in pediatric laparoscopic appendectomy: clip, ligate, or staple?

Options for intracorporeal appendiceal stump closure span a variety of techniques including ligation using intra-corporeal knots, extra-corporeal knots, or an endo-loop (EL), closure with endoscopic clips (EC), or endoscopic stapled (ES) closure. The guiding principles are the need for secure, inert closure of the appendiceal base without injury to the appendiceal stump or cecum, with minimal risks of complication attributable to the closure technique. Safety and complication rates, as well as cost data, should guide the techniques used for pediatric laparoscopic appendectomy. Based on the literature available there is not a clear answer as to the best method for closing the appendiceal stump in pediatric patients, with each of the methods described providing safe closure. Many institutions and surgeons may favor a selective approach, with choice of closure determined by the condition of the appendix at laparoscopy.

Preclosure fluid resuscitation influences outcome in gastroschisis.

Optimal preclosure fluid resuscitation in gastroschisis (GS) is unknown. The purpose of our study was to evaluate effects of preclosure intravenous fluid resuscitation on GS outcome. Cases were accrued from a national GS database. Risk variables analyzed included gestational age (GA), birth weight (BW), neonatal illness severity score, and bolus fluid administration within 6 hours of neonatal intensive care unit admission. Outcomes analyzed included closure success, days of ventilation/total parenteral nutrition (TPN), and bacteremic episodes. Linear and logistic regression analyses were performed. Four hundred seven live-born GS cases were identified (362 with complete resuscitative fluids data). Mean BW, GA, and Score for Neonatal Acute Physiology-II score were 2562 ± 539 g, 36.17 ± 1.95 weeks, and 9.97 ± 12.65, respectively. One hundred sixty-two patients received no supplemental fluid, and 200 patients received a mean of 21.49 (0.81 to 134.81) mL/kg of intravenous fluid. Multivariate outcomes analyses demonstrated a significant, direct relationship between resuscitative volume and days of postclosure ventilation, TPN, length of hospital stay, and bacteremic episodes; specifically, every 17 mL/kg of fluid predicted one additional ventilation day (p = 0.002), TPN day (p = 0.01), and hospital day (p = 0.01) and 0.02 odds increase of an episode of bacteremia (p = 0.03). Judicious, preclosure fluid resuscitation is essential in early GS management. Excessive fluid is associated with several adverse survival outcomes.

Bowel-defect disproportion in gastroschisis: does the need to extend the fascial defect predict outcome?

BACKGROUND/PURPOSE: Validated outcome prediction for gastroschisis (GS) permits early risk stratification. The aim of our study was to determine whether the need for GS defect extension: (a) correlates with bowel injury severity at birth, and (b) predicts outcome. METHODS: A national dataset was used to study GS babies born between 2005 and 2010. The primary outcome was days of parenteral nutrition (PN). Outcomes were analyzed according to the need for fascial extension to facilitate closure or silo placement as follows: Group 1, no extension; Group 2A, extension <2 cm; Group 2B, extension >2 cm. Univariate and where appropriate, multivariate analyses were used. RESULTS: Of 507 cases, 402 had complete defect extension data: Group 1, 297 (73%); Group 2A, 67 (17%); Group 2B, 42 (10%). Group 2B patients had higher rates of atresia, perforation and severe matting (P = 0.001) and required more days on PN compared to Group 1 (63.0 ± 100.4 vs. 39.7 ± 44.5 days: CI 1.2-45.1; P = 0.03). Multivariate analysis revealed that the presence of atresia (P = 0.01) and surgical site (P = 0.001) or bloodstream (P = 0.001) infections were predictive of prolonged PN; however, the need for fascial extension was not. CONCLUSIONS: GS newborns who require fascial extension are more likely to have complicated GS and are at greater risk for adverse outcome, although it is not an independent predictor of the latter.

Endoloop versus endostapler closure of the appendiceal stump in pediatric laparoscopic appendectomy.

BACKGROUND: There is little information available to inform choice of technique for appendiceal stump control in pediatric laparoscopic appendectomy (LA). We compared complications (stump leak, intra-abdominal abscess formation [IAA], surgical site infection [SSI]) in children undergoing LA for perforated (PA) and nonperforated appendicitis (NPA) by technique of appendiceal stump control. METHODS: All children who underwent LA for confirmed acute appendicitis between 2006 and 2009 were reviewed. Choice of stump control (endoloop [EL] or endostapler [ES]) was determined by surgeon preference. Interactions between stump closure techniques and other potential confounders (intra-abdominal drain, irrigation, different antibiotic regimens) were explored using a logistic regression model. RESULTS: Of 242 patients undergoing LA, 57 (23.6%) had PA. In the PA group the appendiceal stump was closed with EL in 47 (82.5%) patients, while in the NPA group EL was used in 161 (87%) patients. Among PA patients, IAA was more common in the ES than the EL group (5 of 10 [50%] v. 6 of 47 [12.7%]). There was no significant difference in rates of SSI. Among NPA patients, there were no differences in rates of IAA or SSI. There were no stump leaks in either group. Logistic regression analysis confirmed the predictive effect of ES use on IAA formation in PA (adjusted odds ratio 7.09; 95% confidence interval 1.08-46.13; p = 0.042). CONCLUSION: Our data suggest that in most cases of PA, the appendiceal stump can be safely controlled with EL. Within the PA group, the higher rates of IAA seen in ES patients may be attributable to the quality of the appendiceal stump rather than the technique of closure.

Does operative experience during residency correlate with reported competency of recent general surgery graduates?

BACKGROUND: Identification of attributes of residency training that predict competency would improve surgical education. We hypothesized that case experience during residency would correlate with self-reported competency of recent graduates. METHODS: Aggregate case log data of residents enrolled in 2 general surgery programs were collected over a 12-month period and stratified into Surgical Council on Resident Education (SCORE) categories. We surveyed recent (< 5 yr) residency graduates on procedural competency. Resident case volumes were correlated with survey responses by SCORE category. RESULTS: In all, 75 residents performed 11 715 operations, which were distributed by SCORE category as follows: essential-common (EC) 9935 (84.8%), essential-uncommon (EU) 889 (7.6%) and complex 891 (7.6%). Alimentary tract procedures were the most commonly performed EC (2386, 24%) and EU (504, 56.7%) procedures. The least common EC procedure was plastic surgery (4, 0.04%), and the least common EU procedure was abdomen-spleen (1, 0.1%). The questionnaire response rate was 45%. For EC procedures, self-reported competency was highest in skin and soft tissue, thoracic and head and neck (each 100%) and lowest in vascular-venous (54%), whereas for EU procedures it was highest in abdomen-general (100%) and lowest in vascular-arterial (62%). The correlation between case volume and self-reported competency was poor (R = 0.2 for EC procedures). CONCLUSION: Self-reported competency correlates poorly with operative case experience during residency. Other curriculum factors, including specific rotations and timing, balance between inpatient and outpatient surgical experience and competition for cases, may contribute to procedural competency acquisition during residency.

Urgency of Esophageal Foreign Body Removal: Differentiation Between Coins and Button Cell Batteries.

OBJECTIVE: Coin-shaped button battery foreign bodies have a similar initial presentation to coin ingestion, but delayed retrieval of a battery from the esophagus can have devastating consequences. Variations in timing of retrieval for children with ingestion of coin foreign bodies have been reported. The study assesses the sensitivity and specificity of conventional and digital radiographs to differentiate button batteries from coin foreign bodies. STUDY DESIGN: 3B case control study. STUDY SETTING: Tertiary academic medical center. METHODS: A radiographic study of the 12 most common commercially available button batteries and 66 coins of varying international origins was performed. Foreign bodies were placed at the cervical esophagus of a cadaver, and anteroposterior (AP) and lateral conventional radiographs of the neck were obtained. Digital AP and lateral radiographs of standalone coins and batteries were also obtained. Images were blindly read by 2 otolaryngologists and 2 radiologists. Statistical analysis was performed to determine accuracy in identifying coins vs batteries. RESULTS: Using conventional radiographs to identify button batteries yielded a sensitivity of 0.88 and a specificity of 0.92 (positive predictive value [PPV] = 0.75, negative predictive value [NPV] = 0.97). Digital radiography yielded an overall sensitivity of 0.98 and specificity of 0.97 (PPV = 0.87, NPV = 0.99). Features of button batteries were only seen on AP conventional radiographs using reverse contrast. CONCLUSIONS: Neither conventional nor digital radiographic imaging had perfect accuracy in identifying coins vs batteries. Features of common disc batteries were identified, which may aid in diagnosis. With potential devastating consequences from retained battery in the esophagus, emergent removal of any possible disc battery foreign body should be considered.

Case Reports: Management Challenges in Pediatric Germ Cell Tumors.

Germ cell tumors in infants are most frequently extragonadal, benign, and amenable to surgical resection. An unusual feature of germ cell tumors is the potential coexistence of malignant with benign disease which makes it possible for patients with incompletely resected tumors to experience either a benign or malignant recurrence. A challenge to postoperative surveillance is the interpretation of serum alpha fetoprotein, a marker of malignancy, that is physiologically elevated during the first year of life. A rare subset of germ cell tumors occur in the retroperitoneum. Although the vast majority are benign, these tumors are often large and distort normal anatomy, and may demonstrate local invasiveness that increases risk of resection. The intent of these reports is to caution readers about these unusual features of germ cell tumors of infancy.

Transmission of Mycoba cterium tuberculosis to households of tuberculosis patients: a comprehensive contact tracing study.

BACKGROUND: Tuberculosis (TB) continues to be a major health problem in developing countries. Contact investigation is the most appropriate strategy to interrupt transmission and subsequent development of TB. METHODS: This cross-sectional study was conducted to assess the impact of contact screening on case-finding by using tuberculin skin test chest radiography. Contacts of smear-positive patients with pulmonary TB (index cases) were diagnosed and registered in our center during 2002 - 2004. Contacts, defined as household members living with index cases for >30 days, were screened by sputum examination, tuberculin skin test, and chest radiography. RESULTS: Sixty-eight patients with smear-positive pulmonary TB were considered as index cases. A total of 224 close contacts with index cases (an average of 3 contacts for each index case) were detected. Age among contacts ranged from 6 months to 74 years. Eighty-three percent of contacts were Iranians and 17% were Afghans. Abnormal radiographs were seen in 49.6% of contacts. Sixteen point five percent of contacts had a positive tuberculin skin test of >10 mm; 7.6% had a positive sputum smear. The mean +/- SD age of Iranian contacts (29.1 +/- 16.6 years) was significantly (P < 0.001) higher than that of Afghans (18.6 +/- 14.1 years). Cavitary formation, nodular pattern, and infiltration were found to have a strong association with a positive sputum smear for acid fast bacilli (100%, 100%, and 87%, respectively). CONCLUSION: The rate of TB in contacts was higher than other similar studies. Earlier detection and treatment of adults with TB could interrupt transmission and be a step towards eliminating childhood TB. Contact control and source-case investigations should be emphasized for TB control. Novel strategies are needed to maximize the number of contacts who are not only identified and evaluated, but also completely treated.

Trauma center variation in the management of pediatric patients with blunt abdominal solid organ injury: a national trauma data bank analysis.

BACKGROUND: Nonoperative management of hemodynamically stable children with Solid Organ Injury (SOI) has become standard of care. The aim of this study is to identify differences in management of children with SOI treated at Adult Trauma Centers (ATC) versus Pediatric Trauma Centers (PTC). We hypothesized that patients treated at ATC would undergo more procedures than PTC. METHODS: Patients younger than 18 years old with isolated SOI (spleen, liver, kidney) who were treated at level I-II ATC or PTC were identified from the 2011-2012 National Trauma Data Bank. The primary outcome measure was the incidence of operative management. Data was analyzed using multivariate logistic regression analysis. Procedures were defined as surgery or transarterial embolization (TAE). RESULTS: 6799 children with SOI (spleen: 2375, liver: 2867, kidney: 1557) were included. Spleen surgery was performed more frequently at ATC than PTC {101 (7.7%) vs. 52 (4.9%); P=0.007}. After adjusting for potential confounders (grade of injury, age, gender and injury severity score), admission at ATC was associated with higher odds of splenic surgery (OR: 1.5, 95% CI: 1.02-2.25; p=0.03). 11 and 8 children underwent kidney and liver operations respectively. TAE was performed in 17 patients with splenic, 34 with liver and 14 with kidney trauma. There was no practice variation between ATC and PTC regarding kidney and liver operations or TAE incidence. CONCLUSIONS: Operative management for SOI was more often performed at ATC. The presence of significant disparity in the management of children with splenic injuries justifies efforts to use these surgeries as a reported national quality indicator for trauma programs.

Children are safer in states with strict firearm laws: a National Inpatient Sample study.

BACKGROUND: Firearm control laws vary across the United States and remain state specific. The aim of this study was to determine the relationship between variation in states' firearm control laws and the risk of firearm-related injuries in pediatric population. We hypothesized that strict firearm control laws impact the incidence of pediatric firearm injury. METHODS: All patients with trauma Ecodes and those 18 years or younger were identified from the 2009 Nationwide Inpatient Sample. Individual states' firearm control laws were evaluated and scored based on background checks on firearm sales, permit requirements, assault weapon and large-capacity magazine ban, mandatory child safety lock requirements, and regulations regarding firearms in college and workplaces. States were then dichotomized into strict firearm laws (SFLs) and non-strict firearm laws (non-SFLs) state based on median total score. The primary outcome measure was incidence of firearm injury. Data were compared between the two groups using simple linear regression analysis. RESULTS: A total of 60,224 pediatric patients with trauma-related injuries across 44 states were included. Thirty-three states were categorized as non-SFL and 11 as SFL. Two hundred eighty-six (0.5%) had firearm injuries, of which 31 were self-inflicted. Mean firearm injury rates per 1,000 trauma patients was higher in the non-SFL states (mean [SD]: SFL, 2.2 [1.6]; non-SFL, 5.9 [5.6]; p = 0. 001). Being in a non-SFL state increased the mean firearm injury rate by 3.75 (ß coefficient, 3.75; 95% confidence interval, 0.25-7.25; p = 0.036). CONCLUSION: Children living in states with strict firearm legislation are safer. Efforts to improve and standardize national firearm control laws are warranted. LEVEL OF EVIDENCE: Prognostic study, level III.

New insight into the treatment of advanced differentiated thyroid cancer.

The vast majority of patients with differentiated thyroid cancer (DTC) are treated successfully with surgery and radioactive iodine ablation, yet the treatment of advanced cases is frustrating and largely ineffective. Systemic treatment with conventional cytotoxic chemotherapy is basically ineffective in most patients with advanced DTC. However, a better understanding of the genetics and biologic basis of thyroid cancers has generated opportunities for innovative therapeutic modalities, resulting in several clinical trials. We aim to delineate the latest knowledge regarding the biologic characteristics of DTC and to describe the available data related to novel targeted therapies that have demonstrated clinical effectiveness.

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

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

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

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

Traumatic pseudoaneurysms of the liver and spleen in children: is routine screening warranted?

BACKGROUND: Although blunt injury to the spleen and liver can lead to pseudoaneurysm formation, current surgical guidelines do not recommend follow-up imaging. Controversy exists regarding the clinical implications of these traumatic pseudoaneurysms as well as their management. METHODS: Retrospective review of children treated nonoperatively for isolated blunt liver and spleen trauma between 1991 and 2008 was undertaken. Patient demographics, grade of injury, and follow-up Doppler ultrasound results were obtained. RESULTS: Three hundred sixty-two children were identified. One hundred eighty-six of them had splenic injuries, and 10 (5.4%) developed pseudoaneurysms. They were associated with grade III (3/39 [8%]) and grade IV (7/41 [17%]) injuries. In 7 patients, the pseudoaneurysm thrombosed spontaneously. Angiographic embolization was required in 2 children, and one underwent emergency splenectomy for delayed hemorrhage. Of the 176 patients who had liver injuries, 3 (1.7%) developed pseudoaneurysms. All 3 were associated with grade IV injuries (3/11 [27%]). One child underwent early embolization, while 2 developed delayed hemorrhage requiring emergent treatment. CONCLUSIONS: Pseudoaneurysm development after blunt abdominal trauma is associated with high-grade splenic and liver injuries. Routine screening of this group of patients before discharge from hospital may be warranted because of the potential risk of life-threatening hemorrhage.

Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network.

BACKGROUND: Perinatal management of congenital diaphragmatic hernia (CDH) and gastroschisis (GS) remains nonstandardized and institution specific. This analysis describes practice and outcome variation across a national network. METHODS: A national, prospective, disease-specific database for CDH and GS was evaluated over 4 years. Centers were evaluated individually and defined as low (low-volume center [LVC]) or high (high-volume center [HVC]) volume based on case mean. RESULTS: Congenital diaphragmatic hernia. Two hundred fifteen liveborn cases were studied (mean, 14.3 cases/center) across 15 centers (8 LVCs and 7 HVCs). Significant interinstitutional practice variation was noted in rates of termination (0%-40%) and cesarean delivery (0%-61%). Centers demonstrated marked variation in ventilation strategies, vasodilator and paralytic use, timing of surgery, and rates of primary closure. Overall survival was 81.4% (LVC, 76.9%; HVC, 82.4%; P = .43). Gastroschisis. Four hundred sixteen cases were investigated (mean, 26 cases/center; range, 6-72) across 16 centers (10 LVCs and 6 HVCs). Cesarean delivery rates varied widely between centers (0%-86%) as did timing of closure (early vs delayed, 1%-100%). There was no difference in length of stay, days on total parenteral nutrition, and overall survival (94.3% vs 97.2%; P = .17) between LVCs and HVCs. CONCLUSIONS: The existence of perinatal practice and outcome variation for GS and CDH suggests targets for improved delivery of care and justifies efforts to standardize treatment on a national basis.

Perinatal management of congenital diaphragmatic hernia: when and how should babies be delivered? Results from the Canadian Pediatric Surgery Network.

PURPOSE: A prenatal diagnosis of congenital diaphragmatic hernia (CDH) enables therapeutic decision making during the intrapartum period. This study seeks to identify the gestational age and delivery mode associated with optimal outcomes. PATIENTS AND METHODS: A national data set was used to study CDH babies born between 2005 and 2009. The primary outcome was survival to discharge. Primary and secondary outcomes were analyzed by categorical gestational age (preterm, <37 weeks; early term, 37-38 weeks; late term, >39 weeks) by intended and actual route of delivery and by birth plan conformity, regardless of route. RESULTS: Of 214 live born babies (gestational age, 37.6 ± 4.0 weeks; birth weight, 3064 ± 696 g), 143 (66.8%) had a prenatal diagnosis and 174 (81.3%) survived to discharge. Among 143 prenatally diagnosed pregnancies, 122 (85.3%) underwent abdominal delivery (AD) and 21 (14.6%) underwent cesarean delivery (CS). Conformity between intended and actual delivery occurred in 119 (83.2%). Neither categorical gestational age nor delivery route influenced outcome. Although babies delivered by planned CS had a lower mortality than those delivered by planned AD (2/21 and 36/122, respectively; P = .04), this difference was not significant by multivariate analysis. Conformity to any birth plan was associated with a trend toward improved survival. CONCLUSION: Our data do not support advocacy of any specific delivery plan or route nor optimal gestational age for prenatally diagnosed CDH.