Endoscopic repair of tracheoesophageal fistulas: A contemporary multi-institutional case series and literature review.

OBJECTIVES: Recurrent and primary tracheoesophageal fistulas (TEFs) are a challenging surgical pathology to treat, as standard open surgical approaches are associated with high morbidity and mortality. As such, endoscopic modalities have gained interest as an alluring alternative, yet variable success rates have been reported in the literature. The aim of this study was to provide a contemporary update of the literature and describe our institutional experience with the bronchoscopic obliteration of recurrent and primary TEFs. METHODS: Retrospective chart review of all pediatric patients having undergone endoscopic TEF repair at two pediatric academic centers in Montreal, Canada and Lille, France between January 1, 2008 to December 31, 2020. RESULTS: 28 patients with TEFs (20 recurrent, 8 primary) underwent a total of 48 endoscopic procedures. TEF repair was performed under endoscopic guidance using various combinations of techniques, including fistula de-epithelialization (endoscopic brush, thulium laser, trichloroacetic acid-soaked pledgets or electrocautery), tissue adhesives, submucosal augmentation, esophageal clip and stenting. Successful closure was achieved in 16 patients (57 %), while 12 (43 %) required eventual open or thoracoscopic repair. The mean number of endoscopic procedures was 1.7. There were no major treatment-related complications such as pneumothorax, mediastinitis or death (mean follow-up 50.8 months). CONCLUSIONS: Endoscopic repair of recurrent or primary TEFs is a valuable component of our therapeutic armamentarium and may contribute to decreased surgical morbidity in this complex patient population. Families should be counselled that endoscopic results may be more modest than with open or thoracoscopic approaches, and multiple procedures may be required.

A minimally invasive hybrid procedure to correct pectus arcuatum.

BACKGROUND: Pectus arcuatum, also known as horns of steer anomaly or Currarino-Silverman Syndrome, is a distinct chest wall anomaly characterized by severe manubriosternal angulation, a shortened sternum, and mild pectus excavatum. The anomaly is typically repaired using open techniques, employing orthopedic fixation devices. Here, we report the results of a minimally invasive hybrid procedure to repair pectus arcuatum. METHODS: The procedure combines a standard Nuss procedure to correct the depressed sternum with a short upper chest (in boys) or inter-mammary (in girls) incision for bilateral subperichondrial resection of the upper costal cartilages, osteotomy, and correction of the manubrial angulation. The medical records of all patients who underwent the procedure over the last 10 years were reviewed. RESULTS: Five patients, 3 boys and 2 girls, aged 14 to 17 years, underwent the procedure. Three patients had their pectus bars removed 3-4 years after repair. Follow-up after correction ranged from 6 months to 7 years. Good correction resulted in all patients achieving recovery without complications and recurrence. To date, all patients have been satisfied with their results. CONCLUSIONS: The minimally invasive hybrid procedure adequately corrects pectus arcuatum with minimal scarring and high satisfaction.

The Impact of Educational Materials on Parental Anxiety and Productivity: A Clinical Trial in Pediatric Appendicitis.

OBJECTIVES: There is limited literature on how acute appendicitis, the most common acute children's surgical illness, affects the family. We conducted a prospective study to assess the impact of educational materials on parents' anxiety and productivity during the child's illness. METHODS: A quasi-experimental clinical trial was conducted among parents of children undergoing laparoscopic appendectomy. In Phase I, parents received the standard explanations at diagnosis and throughout the postoperative period. In Phase II, parents also received a comprehensive educational brochure on pediatric appendicitis at diagnosis. The primary outcome, parental preoperative anxiety, was assessed using the Amsterdam Preoperative Anxiety and Information Scale (APAIS). The secondary outcome, parental productivity, was evaluated through a post-recovery online questionnaire based on the Productivity and Disease Questionnaire (PRODISQ). Baseline characteristics and outcomes were compared between the two cohorts using t-tests, Mann-Whitney, chi-square, or Fischer's exact test as appropriate. RESULTS: Phases I and II included 67 and 66 families, respectively. Patient demographics and disease severity were similar between both groups. Of the 53 parents (80.3%) in Phase II who answered the postoperative questionnaire, most recommended the booklet (96.2%), as it decreased their stress (78.0%) and enhanced their understanding of appendicitis (94.1%). However, the two groups showed similar preoperative anxiety levels and postoperative productivity loss. CONCLUSIONS: Educational materials increased satisfaction with surgical care but did not mitigate the high parental preoperative anxiety levels and postoperative productivity loss. Additional research is required to elucidate interventions that may improve these important patient and family-centered outcomes. TYPE OF STUDY: Non-Randomized Clinical Trial. LEVEL OF EVIDENCE: II.

Postoperative pain following minimally invasive repair of pectus excavatum: A descriptive study.

BACKGROUND: Minimally Invasive Repair of Pectus Excavatum (MIRPE) is associated with significant postoperative pain. The objective of our study was to characterize the severity and duration of this pain, and to investigate possible associations with pectus severity. METHODS: We conducted a retrospective cohort study of pediatric patients who underwent MIRPE from January 2014 to April 2018. Pectus excavatum (PE) severity was determined with 3 indices measured from computed tomography: Depression Index (DI), Correction Index (CI), and Haller index (HI). Mean pain scores for every 6-hour period and the presence of pain and intake of analgesics during follow-up were extracted from the medical record. RESULTS: The cohort included 57 patients with a mean age of 15.9 ± 1.3 years. All 3 severity indices were positively correlated, with a correlation coefficient of 0.8 between the DI and CI. The requirement for 2 bars was significantly associated with higher indices (95% CI:0.18-0.63, p = 0.01). Pain was managed with thoracic epidural analgesia for all but one patient. Growth linear modeling identified five different pain trajectory subgroups of patients up to post-operative day 5. None of the tested predictors (age, gender, body image, physical activity level, DI, CI, HI, difference deformity-epidural level) were significantly associated with class membership. Persistent pain at one-year follow-up was present in 18% of patients, all with severe deformity (DI≥0.8). CONCLUSION: Pain trajectory and intensity after MIRPE can be classified into discrete patterns but are not influenced by PE severity. Severe deformity seems to predict persistent pain at one year.

The utility of echocardiography and pulmonary function testing in the preoperative evaluation of pectus excavatum.

PURPOSE: Echocardiography (ECHO) and pulmonary function testing (PFT) are routinely performed during the preoperative evaluation of pectus excavatum (PE). We hypothesized that these investigations may be performed selectively based on patient symptoms and pectus severity. METHODS: A retrospective review of all PE patients who underwent a Nuss procedure during a 15-year period (2004-2018) was conducted. Symptoms, clinical characteristics, ECHO, and PFT results were extracted from the medical chart. PE severity on computed tomography was measured using the Haller Index (HI) and Correction Index (CI), and reported as mean ± SEM. Logistic and linear regression assessed the ability of symptoms and indices to predict abnormal cardiopulmonary test results. RESULTS: Of 119 patients, 116 patients had symptom documentation, and 74 (64%) had one or more symptoms. HI and CI were 3.8 ± 1.0 and 31.6 ± 10.3, respectively. Of those with ECHO available (111), 14 (13%) were abnormal, and 12 of 14 required cardiology follow-up. Of those with PFT available (90), the results were abnormal in 15 (17%), including 9 (11%) obstructive, 4 (5%) restrictive, and 2 (2%) mixed. The presence of symptoms did not predict abnormal ECHO or PFT, but each standard deviation increase in the CI was associated with abnormal PFT and ECHO by a factor of 2.2 and 2.0 respectively. HI severity was only associated with ECHO. CONCLUSION: The rates of abnormal ECHO and PFT testing in PE patients are low, and do not correlate with symptoms. Routine ECHO is still recommended to detect anomalies requiring follow-up. Elevated CI severity may be used to guide selective PFT testing. LEVELS OF EVIDENCE: Retrospective Study, Level III.

Effect of transanastomotic feeding tubes on anastomotic strictures in patients with esophageal atresia and tracheoesophageal fistula: The Quebec experience.

PURPOSE: Recent studies have identified transanastomotic tubes (TATs) as a risk factor for the development of anastomotic strictures after repair of esophageal atresia with tracheoesophageal fistula (EATEF). We further investigated these findings in a multicenter study. METHODS: We conducted a retrospective cohort study at three university-affiliated hospitals in the province of Quebec. All patients with types C and D EATEF who underwent primary repair between January 1993 and August 2018 were included. Anastomotic stricture was defined as clinical symptoms of stricture with confirmation on esophagram or endoscopy. Multivariate logistic regression and the Wilcoxon Rank-Sum test were used to evaluate the primary outcome of stricture within one year of surgery and secondary outcome of duration of postoperative total parenteral nutrition (TPN). RESULTS: 244 patients were included, of which 234 (96%) were type C and 10 (4%) were type D. The anastomotic stricture rate at 1 year was 30%. TATs were utilized in 61% of patients. Thirty-six percent of patients with TATs developed a stricture within one year, as compared to 19% of patients without TATs (p = 0.005). TATs were associated with stricture on univariate analysis (OR 2.49, p = 0.004, 95% CI: 1.37-4.69). On multivariate analysis, after adjusting for gestational age, birth weight, leak, long gap, anastomotic tension, and daily acid suppression, patients with TATs had 2.72 times higher odds of developing a stricture as compared to patients without TATs (p = 0.006, 95% CI: 1.35-5.74). The median duration of TPN was 9 days in both groups (p = 0.139, IQR 6-14 in patients with TATs versus IQR 7-16 in patients without). CONCLUSION: Transanastomotic tubes are associated with a significantly higher risk of postoperative stricture following repair of esophageal atresia with tracheoesophageal fistula and do not shorten the duration of total parenteral nutrition. LEVEL OF EVIDENCE: III.

Musculoskeletal deformities after thoracic surgery in children: An observational long-term follow-up study.

PURPOSE: This study reports the incidence, severity, and predictors of musculoskeletal deformities (MD), including scoliosis and chest wall anomalies, following thoracic procedures in children. METHODS: Children younger than 14 years who had thoracic surgery between 1997 and 2012 and had no other predispositions to MD, underwent longitudinal follow-ups with dedicated musculoskeletal examination performed in an esophageal atresia, orthopedic, or research clinic. Incidence of MD was calculated, and logistic regression methods were used to determine independent predictors, including sex, gestational age, age at procedure, serratus anterior muscle division, and chest tube placement. RESULTS: The study cohort consisted of 104 patients followed for a median of 10.8 years (range 3-21). A total of 56 MD developed in 41 patients (39%), including scapular winging (24; 23%), scoliosis (17; 16%), and chest wall anomalies (15; 14%). The majority of MD were subclinical, with only 8 patients [8% (6 thoracotomies, 2 thoracoscopies)] requiring intervention. Among patients who underwent thoracotomies (93, 89%), serratus anterior muscle division was the only significant predictor of the development of MD [OR 8.9; 95% CI 2.8-32.6]. CONCLUSION: Musculoskeletal deformities develop in a significant proportion of children following thoracic surgery, but most are subclinical. A muscle-sparing technique decreases the incidence of these deformities. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: Level II.

Defining the critical pediatric surgical workforce density for improving surgical outcomes: a global study.

PURPOSE: Low- and middle-income countries (LMICs) have only 19% of the global surgical workforce yet see 80% of worldwide deaths from noncommunicable diseases. We aimed to interrogate the correlation between pediatric surgical workforce density (PSWD) and survival from pediatric surgical conditions worldwide. METHODS: A systematic review of online databases identified outcome studies for key pediatric surgical conditions (gastroschisis, esophageal atresia, intestinal atresia, and typhoid perforation) as well as PSWD data across low-income (LICs), middle-income (MICs), and high-income countries (HICs). PSWD was expressed as the number of PSs/million children under 15 years of age and we correlated this to surgical outcomes for our case series. RESULTS: PSWD ranged between zero (Burundi, The Gambia, and Mauritania) and 125.2 (Poland) across 86 countries. Outcomes for at least one condition were obtained in 61 countries: 50 outcomes in HICs, 52 in MICs and 8 in LICs. The mean survival in our case series was 42.3%, 69.4% and 91.6% for LICs, MICs, and HICs, respectively. A PSWD ≥4 PSs/million children under 15 years of age significantly correlated to odds of survival ≥80% (OR 16.8, p < 0.0001, 95% CI 5.66-49.88). Specifically in the studied LICs and MICs, increasing the PSWD to 4 would require training 1427 additional surgeons. CONCLUSION: Using a novel approach, we have established a benchmark for the scale-up of pediatric surgical workforce, which may support broader efforts to reduce childhood deaths from congenital disease. LEVELS OF EVIDENCE: 2c - Outcomes Research.

Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes.

PURPOSE: Effective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with postnatal outcomes at two tertiary pediatric referral centers. METHODS: Prenatal lung-head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24 and 32 weeks gestational age. Postnatal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted. RESULTS: Eighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values >45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair. CONCLUSIONS: This audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review. LEVEL OF EVIDENCE: IV.

Vacuum bell treatment of pectus excavatum: An early North American experience.

PURPOSE: Conservative treatment of pectus excavatum with a vacuum bell device may be an attractive alternative to surgical repair. We describe an early North American experience with this device. METHODS: Prospectively maintained chest wall clinic registries from two institutions were reviewed to identify pectus excavatum patients ≤21 years treated with the vacuum bell from 2013 to 2017. Multivariate linear regression was used to compare mean improvements in deformity-depth and Haller Index between groups of patients based on age and usage metrics (hours/day and days/week). RESULTS: Thirty-one patients with a median age of 14 years received treatment with the device. Mean follow-up duration was 18 months. Median depth and Haller Index at treatment onset were 2.3 cm and 3.9, respectively. Improvements in deformity-depth were superior with device usage >2 h/day (p < 0.01) and daily use (p < 0.01). After adjusting for compliance, younger age of treatment onset was associated with greater improvement in Haller Index but not deformity depth. CONCLUSION: Our prospective early North American experience found the vacuum bell to be a potential alternative to surgical treatment for pectus excavatum. Longer usage periods in a daily frequency are associated with best results. TYPE OF STUDY: Treatment study; case series with no comparison group. LEVEL OF EVIDENCE: Level IV.

Nebulized analgesia during laparoscopic appendectomy (NALA): A randomized triple-blind placebo controlled trial.

PURPOSE: Postoperative pain remains a considerable concern for patients and families. We assessed whether nebulized ropivacaine reduces morphine consumption and pain after laparoscopic appendectomy for uncomplicated appendicitis in children. METHODS: Patients 7-17 years old with uncomplicated appendicitis were randomized to ropivacaine (intervention arm) or saline nebulization (placebo arm) at the onset of laparoscopy. Nonconsenting individuals were treated with standard care and invited to provide clinical data (baseline arm). The primary outcome was in-patient morphine utilization. Secondary outcomes included pain scores at multiple time-points, markers of recovery, operative times, and surgeon satisfaction. The trial was registered (NCT02624089). RESULTS: Study enrollment was 116 patients over a 1-year period: Intervention (n = 43), Placebo (n = 39), Baseline (n = 34). No differences in baseline characteristics were noted between groups. No difference was noted in overall in-patient morphine consumption between randomized groups (0.31 vs. 0.35 mg/kg, p = 0.42) or between ropivacaine and baseline (0.31 vs. 0.277 mg/kg, p = 0.62). Although operative times were comparable between groups, 63% of surgeon respondents felt that nebulization obscured visualization. CONCLUSION: Nebulized ropivacaine did not reduce postoperative morphine consumption or pain scores after laparoscopic appendectomy for simple appendicitis in children. Given that it decreases visualization and likely increases costs, nebulized administration of intraperitoneal analgesia does not appear warranted in this context. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level I.

The utility of magnetic resonance imaging in the diagnosis and management of pediatric benign ovarian lesions.

BACKGROUND: The utility of magnetic resonance imaging (MRI) in the diagnosis and management of pediatric ovarian lesions has not been well defined. METHODS: A retrospective review of all girls who underwent MRI evaluation of ovarian masses during the period 2009-2015 was performed. The accuracy of MRI was evaluated by comparing results with surgical findings, pathology reports, and subsequent imaging. The influence of the MRI on the treatment plan was specifically explored. RESULTS: Eighteen girls, 12-17years of age, underwent 27 MRIs, subsequent to ultrasound identification of ovarian lesions. Of 9 neoplastic lesions diagnosed on MRI, 8 (89%) were confirmed by surgical and pathological findings. Of 18 functional lesions, 17 (94.4%) were confirmed pathologically or by resolution on subsequent imaging. Twenty MRI exams (74%) directly influenced the treatment plan, by leading to appropriate operative intervention in 9 and appropriate observation in 11. The extent of ovarian resection was guided by MRI findings in 8 of 9 (89%) neoplastic lesions. For characterizing lesions as neoplastic, the sensitivity, specificity, negative predictive value, positive predictive value, and accuracy of MRI were 89%, 94%, 94%, 89%, and 93% respectively. CONCLUSIONS: MRI can differentiate functional from neoplastic pediatric ovarian masses, and guide ovarian resection in appropriate cases. LEVEL OF STUDY: II.

Risk stratification in pediatric perforated appendicitis: Prospective correlation with outcomes and resource utilization.

PURPOSE: Despite a wide spectrum of severity, perforated appendicitis in children is typically considered a single entity in outcomes studies. We performed a prospective cohort study to define a risk stratification system that correlates with outcomes and resource utilization. METHODS: A prospective study was conducted of all children operated for perforated appendicitis between May 2015 and December 2016 at a tertiary free-standing university children's hospital. Surgical findings were classified into one of four grades of perforation: I. localized or contained perforation, II. Contained abscess with no generalized peritonitis, III. Generalized peritonitis with no dominant abscess, IV. Generalized peritonitis with one or more dominant abscesses. All patients were treated on a clinical pathway that involved all points of care from admission to final follow-up. Outcomes and resource utilization measures were analyzed using Fisher's exact test, Kruskal-Wallis test, One-way ANOVA, and logistic regression. RESULTS: During the study period, 122 patients completed treatment, and 100% had documented follow-up at a median of 25days after operation. Grades of perforation were: I, 20.5%; II, 37.7%; III, 10.7%; IV, 31.1%. Postoperative abscesses occurred in 12 (9.8%) of patients, almost exclusively in Grade IV perforations. Hospital stay, duration of antibiotics, TPN utilization, and the incidence of postoperative imaging significantly increased with increasing grade of perforation. CONCLUSION: Outcomes and resource utilization strongly correlate with increasing grade of perforated appendicitis. Postoperative abscesses, additional imaging, and additional invasive procedures occur disproportionately in patients who present with diffuse peritonitis and abscess formation. The current stratification allows risk-adjusted outcome reporting and appropriate assignment of resource burden. LEVEL OF EVIDENCE: I (Prognosis Study).

Standardization of care for pediatric perforated appendicitis improves outcomes.

BACKGROUND: The treatment of perforated appendicitis in children is characterized by significant variability in care, morbidity, resource utilization, and outcomes. We prospectively studied how minimization of care variability affects outcomes. METHODS: A clinical pathway for perforated appendicitis, in use for three decades, was further standardized in May 2015 by initiation of a disease severity classification, refinement of discharge criteria, standardization of the operation, and establishment of criteria for use of postoperative total parenteral nutrition, imaging, and invasive procedures. Prospective evaluation of all children treated for 20months on the new fully standardized protocol was conducted and compared to a retrospective cohort treated over 58months prior to standardization. Differences between outcomes before and after standardization were analyzed using regression analysis techniques to adjust for disease severity. RESULTS: Median follow-up time post discharge was 25 and 14days in the post- and prestandardization groups, respectively. Standardization significantly reduced postoperative abscess (9.8% vs. 17.4%, p=0.001) and hospital stay (p=0.002). Standardization reduced the odds of developing a postoperative abscess by four fold. CONCLUSION: Minimizing variability of care at all points in the treatment of perforated appendicitis significantly improves outcomes. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: Level II.

Position Paper of INoEA Working Group on Long-Gap Esophageal Atresia: For Better Care.

INoEA is the International Network of Esophageal Atresia and consists of a broad spectrum of pediatric specialties and patient societies. The working group on long-gap esophageal atresia (LGEA) set out to develop guidelines regarding the definition of LGEA, the best diagnostic and treatment strategies, and highlight the necessity of experience and communication in the management of these challenging patients. Review of the literature and expert discussion concluded that LGEA should be defined as any esophageal atresia (EA) that has no intra-abdominal air, realizing that this defines EA with no distal tracheoesophageal fistula (TEF). LGEA is considerably more complex than EA with distal TEFs and should be referred to a center of expertise. The first choice is to preserve the native esophagus and pursue primary repair, delayed primary anastomosis, or traction/growth techniques to achieve anastomosis. A cervical esophagostomy should be avoided if possible. Only if primary anastomosis is not possible, replacement techniques should be used. Jejunal interposition is proposed as the best option among the major EA centers. In light of the infrequent occurrence of LGEA and the technically demanding techniques involved to achieve esophageal continuity, it is strongly advised to develop regional or national centers of expertise for the management and follow-up of these very complex patients.

Congenital malformations and medical conditions associated with orofacial clefts in children in Burkina Faso.

BACKGROUND: Orofacial clefts are usually isolated cases but can be associated with other congenital malformations that are either recognised or unrecognised syndromes. The reported prevalence and pattern of such associated malformations, however, vary among studies. OBJECTIVES: To assess the frequencies and aetiologies of congenital malformations and associated medical conditions in children with orofacial clefts in Burkina Faso (Western Africa). METHODS: A retrospective descriptive study was carried out at the El Fateh-Suka Clinic in Ouagadougou, Burkina Faso. All children who attended surgery for the repair of a cleft lip and/or palate were included in this study. RESULTS: The frequency of congenital malformations associated with cleft lip and/or palate was 39/185 (21.1%). In the group with multiple congenital malformations of unknown origin (34 patients; 18.4%), 66.7% had cleft lip and palate, followed by isolated cleft lip (27.4%) and isolated cleft palate (5.9%). The digestive system (35.3%), the musculoskeletal system (19.6%), and eye, ear, face, and neck (15.7%) were the most affected systems. In the group of syndromic malformations (five patients; 2.7%), amniotic band syndrome (one patient), Van der Woode syndrome (one patient), Goltz syndrome (one patient), and holoprosencephaly (two patients) were identified. Medical conditions included anaemia (39.4%), infections (9.2%), malnutrition (7.5%), and haemoglobinopathies (4.3%). CONCLUSIONS: Congenital malformations and medical co-morbidities were frequent in children with OFCs. Further studies and a National Malformations Registry are needed to improve the comprehension of OFCs in Burkina Faso.

Outcome prediction in gastroschisis - The gastroschisis prognostic score (GPS) revisited.

PURPOSE: The GPS enables risk stratification for gastroschisis and helps discriminate low from high morbidity groups. The purpose of this study was to revalidate GPS's characterization of a high morbidity group and to quantify relationships between the GPS and outcomes. METHODS: With REB approval, complete survivor data from a national gastroschisis registry was collected. GPS bowel injury scoring was revalidated excluding the initial inception/validation cohorts (>2011). Length of stay (LOS), 1st enteral feed days (dFPO), TPN days (dTPN), and aggregate complications (COMP) were compared between low and high morbidity risk groups. Mathematical relationships between outcomes and integer increases in GPS were explored using the entire cohort (2005-present). RESULTS: Median (range) LOS, dPO, and dTPN for the entire cohort (n=849) was 36 (26,62), 13 (9,18), and 27 (20,46) days, respectively. High-risk patients (GPS≥2; n=80) experienced significantly worse outcomes than low risk patients (n=263). Each integer increase in GPS was associated with increases in LOS and dTPN by 16.9 and 12.7days, respectively (p<0.01). COMP rate was also increased in the high-risk cohort (46.3% vs. 22.8%; p<0.01). CONCLUSION: The GPS effectively discriminates low from high morbidity risk groups. Within the high risk group, integer increases in GPS produce quantitatively differentiated outcomes which may guide initial counseling and resource allocation. LEVEL OF EVIDENCE: IIb.

Determinants of outcomes in patients with simple gastroschisis.

PURPOSE: We analyzed the determinants of outcomes in simple gastroschisis (GS) not complicated by intestinal atresia, perforation, or necrosis. METHODS: All simple GS patients enrolled in a national prospective registry from 2005 to 2013 were studied. Patients below the median for total parenteral nutrition (TPN) duration (26days) and hospital stay (34days) were compared to those above. Univariate and multivariate logistic and linear regression analyses were employed using maternal, patient, postnatal, and treatment variables. RESULTS: Of 700 patients with simple GS, representing 76.8% of all GS patients, 690 (98.6%) survived. TPN was used in 352 (51.6%) and 330 (48.4%) patients for ≤26 and >26days, respectively. Hospital stay for 356 (51.9%) and 330 (48.1%) infants was ≤34 and >34days, respectively. Univariate analysis revealed significant differences in several patient, treatment, and postnatal factors. On multivariate analysis, prenatal sonographic bowel dilation, older age at closure, necrotizing enterocolitis, longer mechanical ventilation, and central-line associated blood stream infection (CLABSI) were independently associated with longer TPN duration and hospital stay, with CLABSI being the strongest predictor. CONCLUSIONS: Prenatal bowel dilation is associated with increased morbidity in simple GS. CLABSI is the strongest predictor of outcomes. Bowel matting is not an independent risk factor. LEVEL OF EVIDENCE: 2c.