Outcomes for pediatric acute lymphoblastic leukemia patients with intestinal perforation.

OBJECTIVES: Intestinal perforation during acute lymphoblastic leukemia (ALL) treatment in children is rare, but represents a severe complication with possible long-term consequences. In this study, we aim to provide an overview of the epidemiology and clinical characteristics of these patients; analyze surgical pathology findings for possible causes; and determine its impact on patients' therapy, nutritional status, and outcome. STUDY DESIGN: Historical chart review from January 2000 to October 2020 of children with ALL and intestinal perforation during therapy diagnosed at a single institution. Data collected included patient demographics, anthropometric measurements, ALL characteristics, diagnosis and surgery of intestinal perforation, pathology, adjustments to treatment plan, and outcome. RESULTS: Of 1840 ALL patients, 13 (0.7%) presented with intestinal perforation during treatment. Perforation occurred during induction phase in 91% of cases. Most patients underwent laparotomy with ostomy creation, and no patient died from the intervention or developed malnutrition. Pathology mainly revealed inflammation at the perforation site. Two samples showed leukemic infiltration and presence of microorganisms. Patients were able to resume ALL therapy in all cases. A total of eight patients (73%) were in first remission at last follow-up, with a median follow-up time of 42 months (interquartile range = 42). CONCLUSION: Early surgical intervention is a successful treatment approach for intestinal perforation in ALL patients. There is a clear predilection for induction phase in the occurrence of intestinal perforation in ALL patients. No specific cause was identified. Patients can receive bridging chemotherapy during surgical recovery and proceed with their treatment without apparent impact on outcome.

Mortality of Pediatric Surgical Lung Biopsies in Ontario, Canada, 2000-2019.

Rationale: Surgical lung biopsies are often required for the definitive diagnosis of nonmalignant pediatric diffuse lung diseases; however, the literature on mortality after surgical lung biopsy in pediatric patients is sparse. Objectives: To determine the 30-day postoperative mortality rate after surgical lung biopsies for nonmalignant lung disease in pediatric patients in Ontario, Canada, and to identify risk factors associated with mortality. Methods: We performed an observational cohort study using population-based health administrative data available from ICES in Ontario, Canada, from 2000 to 2019. Cases were identified using the Canadian Classification of Health Interventions. Inclusion criteria were first surgical lung biopsies between 2000 and 2019 and age <18 years. Individuals with lung cancer, lung transplant, or missing data were excluded. A multivariable logistic regression model with generalized estimating equation was used to estimate the 30-day odds of mortality after surgical lung biopsy and to identify patient characteristics associated with increased mortality while accounting for clustering by hospital. Results: We identified 1,474 pediatric patients who underwent surgical lung biopsy in Ontario between 2000 and 2019. The overall mortality rates decreased over the study duration from 6.6% (2000-2004) to 3.0% (2015-2019). The study cohort for multivariate analyses consisted of 1,342 patients who had complete data. The pediatric mortality 30 days after surgical lung biopsy was 5.1% but was <1% in elective cases. Risk factors for increased mortality included open surgical lung biopsy (vs. video-assisted) (odds ratio [OR], 13.13; 95% confidence interval [CI], 3.76, 45.87; P < 0.001), nonelective procedure (OR, 11.74; 95% CI, 3.51, 39.27; P < 0.001), younger age (<3 mo) (OR, 6.04; 95% CI, 2.40, 15.22; P < 0.001), and higher comorbidity score (OR, 1.15; 95% CI, 1.05, 1.26; P = 0.003). Conclusions: Pediatric mortality postsurgical lung biopsy is not insignificant, particularly in nonelective procedures. Other important risk factors to consider when pursuing pathologic diagnosis include surgical approach, younger age, and higher comorbidity.

Diagnosis and management of congenital diaphragmatic hernia: a 2023 update from the Canadian Congenital Diaphragmatic Hernia Collaborative.

OBJECTIVE: The Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative sought to make its existing clinical practice guideline, published in 2018, into a 'living document'. DESIGN AND MAIN OUTCOME MEASURES: Critical appraisal of CDH literature adhering to Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Evidence accumulated between 1 January 2017 and 30 August 2022 was analysed to inform changes to existing or the development of new CDH care recommendations. Strength of consensus was also determined using a modified Delphi process among national experts in the field. RESULTS: Of the 3868 articles retrieved in our search that covered the 15 areas of CDH care, 459 underwent full-text review. Ultimately, 103 articles were used to inform 20 changes to existing recommendations, which included aspects related to prenatal diagnosis, echocardiographic evaluation, pulmonary hypertension management, surgical readiness criteria, the type of surgical repair and long-term health surveillance. Fifteen new CDH care recommendations were also created using this evidence, with most related to the management of pain and the provision of analgesia and neuromuscular blockade for patients with CDH. CONCLUSIONS: The 2023 Canadian CDH Collaborative's clinical practice guideline update provides a management framework for infants and children with CDH based on the best available evidence and expert consensus.

Survival in very preterm infants with congenital diaphragmatic hernia and association with prenatal imaging markers: A retrospective cohort study.

OBJECTIVES: To describe the outcomes of preterm born infants with congenital diaphragmatic hernia (CDH; ≤32.0 weeks of gestation) and the associations between prenatal imaging markers and survival. DESIGN: Retrospective cohort study. SETTING: Multicentre study in large referral centres. POPULATION: Infants with an isolated unilateral CDH, live born at 32.0 weeks or less of gestation, between January 2009 and January 2020. METHODS: Neonatal outcomes were evaluated for infants that were expectantly managed during pregnancy and infants that underwent fetoscopic endoluminal tracheal occlusion (FETO) therapy, separately. We evaluated the association between prenatal imaging markers and survival to discharge. Prenatal imaging markers included observed to expected lung-to-head ratio (o/e LHR), side of the defect, liver position, stomach position grade, and observed to expected total fetal lung volume (o/e TFLV). MAIN OUTCOME MEASURE: Survival to discharge. RESULTS: We included 53 infants born at 30+4 (interquartile range 29+1 -31+2 ) weeks. Survival in fetuses expectantly managed during pregnancy was 48% (13/27) in left-sided CDH and 33% (2/6) in right-sided CDH. Survival in fetuses that underwent FETO therapy was 50% (6/12) in left-sided CDH and 25% (2/8) in right-sided CDH. The o/e LHR at baseline was positively associated with survival in cases expectantly managed during pregnancy (odds ratio [OR] 1.20, 95% CI 1.07-1.42, p < 0.01), but not in cases that received FETO therapy (OR 1.01, 95% CI 0.88-1.15, p = 0.87). Stomach position grade (p = 0.03) and o/e TFLV were associated with survival (p = 0.02); liver position was not (p = 0.13). CONCLUSIONS: In infants with CDH born at or before 32 weeks of gestation, prenatal imaging markers of disease severity were associated with postnatal survival.

Surgical management of acute life-threatening events affecting esophageal atresia and/or tracheoesophageal fistula patients.

BACKGROUND: Following surgical correction, many patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) present to the emergency department (ED) with acute airway complications. We sought to determine the incidence and risk factors for severe acute life-threatening events (ALTEs) in pediatric patients with repaired congenital EA/TEF and the outcomes of operative interventions. METHODS: A retrospective cohort chart review was performed on patients with EA/TEF with surgical repair and follow-up at a single centre from 2000 to 2018. Primary outcomes included 5-year ED visits and/or hospitalizations for ALTEs. Demographic, operative, and outcome data were collected. Chi-square tests and univariate analyses were performed. RESULTS: In total, 266 EA/TEF patients met inclusion criteria. Of these, 59 (22.2%) had experienced ALTEs. Patients with low birth weight, low gestational age, documented tracheomalacia, and clinically significant esophageal strictures were more likely to experience ALTEs (p < 0.05). ALTEs occurred prior to 1 year of age in 76.3% (45/59) of patients with a median age at presentation of 8 months (range 0-51 months). Recurrence of ALTEs after esophageal dilatation was 45.5% (10/22), mostly due to stricture recurrence. Patients experiencing ALTEs received anti-reflux procedures (8/59, 13.6%), airway pexy procedures (7/59, 11.9%), or both (5/59, 8.5%) within a median age of 6 months of life. The resolution and recurrence of ALTEs after operative interventions are described. CONCLUSION: Significant respiratory morbidity is common among patients with EA/TEF. Understanding the multifactorial etiology and operative management of ALTEs have an important role in their resolution. TYPE OF STUDY: Original Research, Clinical Research. LEVEL OF EVIDENCE: Level III Retrospective Comparative Study.

The long-term outcomes of symptomatic congenital lobar emphysema patients.

INTRODUCTION: Surgical (OP) management for symptomatic congenital lobar emphysema (CLE) is the standard of care with nonoperative (NOP) approach applied for asymptomatic cases. The aim of this study is to report the outcomes for NOP approach to the care of symptomatic CLE infants. METHODS: A retrospective study of CLE patients treated 2000-2021 at a single institution. Patients with CLE and respiratory symptoms were included. RESULTS: Overall, 23 children had symptomatic CLE, and 12 had NOP management. The median age at diagnosis was 38.5 days (50) in the NOP group versus 25 days (20) in the OP group (p = 0.31). There was no significant difference in the location of the involved lobe, term birth, postnatal diagnosis and gender, and both groups required noninvasive support in 33% of the cases. There was a trend towards higher frequency of oxygen support in the OP group preoperatively (89% vs. 42%, p = 0.07). The median length of stay was 14 days in the NOP group compared to a median postsurgery stay of 7.5 days in the OP group. In follow-up, there was no significant difference in respiratory readmission in first year of life, growth delay, treatment with asthma medication or body mass index in the NOP versus OP group. None of the children in the NOP group required surgery during follow-up. CONCLUSIONS: A NOP approach for symptomatic CLE infants can have favorable long-term outcomes. Further studies will be required to identify markers to aid in clinical decision-making.

Precision cut intestinal slices, a novel model of acute food allergic reactions.

BACKGROUND: Food allergy affects up to 10% of the pediatric population. Despite ongoing efforts, treatment options remain limited. Novel models of food allergy are needed to study response patterns downstream of IgE-crosslinking and evaluate drugs modifying acute events. Here, we report a novel human ex vivo model that displays acute, allergen-specific, IgE-mediated smooth muscle contractions using precision cut intestinal slices (PCIS). METHODS: PCIS were generated using gut tissue samples from children who underwent clinically indicated surgery. Viability and metabolic activity were assessed from 0 to 24 h. Distribution of relevant cell subsets was confirmed using single nucleus RNA sequencing. PCIS were passively sensitized using plasma from peanut allergic donors or peanut-sensitized non-allergic donors, and exposed to various stimuli including serotonin, histamine, FcɛRI-crosslinker, and food allergens. Smooth muscle contractions and mediator release functioned as readouts. A novel program designed to measure contractions was developed to quantify responses. The ability to demonstrate the impact of antihistamines and immunomodulation from peanut oral immunotherapy (OIT) was assessed. RESULTS: PCIS viability was maintained for 24 h. Cellular distribution confirmed the presence of key cell subsets including mast cells. The video analysis tool reliably quantified responses to different stimulatory conditions. Smooth muscle contractions were allergen-specific and reflected the clinical phenotype of the plasma donor. Tryptase measurement confirmed IgE-dependent mast cell-derived mediator release. Antihistamines suppressed histamine-induced contraction and plasma from successful peanut OIT suppressed peanut-specific PCIS contraction. CONCLUSION: PCIS represent a novel human tissue-based model to study acute, IgE-mediated food allergy and pharmaceutical impacts on allergic responses in the gut.

Risk factors for prolonged mechanical ventilation in neonates following gastrointestinal surgery.

Background: Prolonged mechanical ventilation (MV) should be avoided in neonates. Noninvasive ventilation (NIV) can facilitate weaning from MV but has risks for patients immediately following foregut surgery due to the potential risk of anastomotic leak. We evaluated the risk factors for prolonged MV following intestinal surgery in neonates. Methods: We retrospectively reviewed 253 neonates undergoing intestinal surgery in 2017-2018 to identify risk factors for prolonged MV, and determine the correlation between NIV and anastomotic leak in a tertiary neonatal intensive care unit that performs the greatest number of neonatal surgeries in Ontario. Results: The most common diagnoses were necrotizing enterocolitis/spontaneous intestinal perforation (NEC/SIP) 21%, intestinal atresia 16%, esophageal atresia/tracheoesophageal fistula 14%, ano-rectal malformation 13%, malrotation/volvulus 11%, gastroschisis 9% and omphalocele 4%. The median (IQR) duration of MV post-surgery was 3 (1-8) days with 25.7 % (n=65) of neonates on MV for >7 days. Compared to infants on MV post-surgery for ≤7 days, those with MV>7 days were of lower gestational age, birth weight and weight at surgery, but a higher proportion underwent stoma creation, had a longer duration of opioid administration and higher rates of moderate to severe bronchopulmonary dysplasia (BPD) and mortality (P<0.05). Generalized linear regression analysis showed lower gestational age (GA) and longer opioid administration were associated with longer duration of MV (P<0.001), but indication for surgery, weight at surgery and stoma creation didn't correlate with longer duration of MV (P>0.05). Of the 122 patients handled by one-stage resection with primary anastomosis, 22.1% (n=27) received NIV with 74.1% (n=20) commenced on NIV after 7 days post-surgery, anastomotic leak was detected in 2.5 % (3/122) patients and didn't correlate with NIV. Conclusions: Lower GA and longer opioid administration were risk factors for prolonged MV in neonates following intestinal surgery. Further research is needed to investigate modifiable practices around pain assessment/ventilation in these patients, and the correlation between NIV and anastomotic leak.

Ileal lactobezoar in extreme premature infant complicated by intestinal perforation: A case report.

INTRODUCTION AND IMPORTANCE: Lactobezor is a rare complication that has been reported more in the stomach, however it may be located anywhere in the intestine. CASE REPORT: Reported here, is a case of ileal lactobezoar which was complicated by perforation and was mimicking necrotizing enterocolitis in presentation, ex preterm (26 weeks) male infant who presented at day of life 18th (2 days after BM fortification) with hemodynamic instability and intestinal perforation, which was diagnosed by Abdominal X-ray and Ultrasound necessitating urgent laparotomy. CLINICAL DISCUSSION: Laparotomy revealed an area of ileal perforation and an inspissated mass which was confirmed to be lactobezoar by pathology, ileostomy was performed. The baby had an acute postoperative status of hypovolemic shock which was managed clinically, then was restarted on feeds, and the stoma was reversed 9 weeks later. CONCLUSION: Lactobezoar, although rare, but numbers increased especially with the rise in numbers of extremely preterm infants worldwide, it most commonly presents later in life but in some cases, such as our case it may happen in 1st 2-3 weeks after birth and may cause significant complications as perforation making its differentiation from common GI problems in neonates as NEC more challenging.

Fetal Meconium Peritonitis - Prenatal Findings and Postnatal Outcome: A Case Series, Systematic Review, and Meta-Analysis.

PURPOSE: To describe the postnatal outcome of fetal meconium peritonitis and identify prenatal predictors of neonatal surgery. METHODS: We retrospectively reviewed all fetuses with ultrasound findings suspicious for meconium peritonitis at a single center over a 10-year period. A systematic review and meta-analysis were then performed pooling our results with previous studies assessing prenatally diagnosed meconium peritonitis and postnatal outcome. Prenatal sonographic findings were analyzed to identify predictors for postnatal surgery. RESULTS: 34 cases suggestive of meconium peritonitis were diagnosed at our center. These were pooled with cases from 14 other studies yielding a total of 244 cases. Postnatal abdominal surgery was required in two thirds of case (66.5 %). The strongest predictor of neonatal surgery was meconium pseudocyst (OR [95 % CI] 6.75 [2.53-18.01]), followed by bowel dilation (OR [95 % CI] 4.17 [1.93-9.05]) and ascites (OR [95 % CI] 2.57 [1.07-5.24]). The most common cause of intestinal perforation and meconium peritonitis, found in 52.2 % of the cases, was small bowel atresia. Cystic fibrosis was diagnosed in 9.8 % of cases. Short-term neonatal outcomes were favorable, with a post-operative mortality rate of 8.1 % and a survival rate of 100 % in neonates not requiring surgery. CONCLUSION: Meconium pseudocysts, bowel dilation, and ascites are prenatal predictors of neonatal surgery in cases of meconium peritonitis. Fetuses with these findings should be delivered in centers with pediatric surgery services. Though the prognosis is favorable, cystic fibrosis complicates postnatal outcomes.

Airway Clearance in Tracheomalacia.

Airway clearance is an essential part of airway maintenance to ensure the airway lumen is protected against particulate and infectious insults. The mechanisms involved in airway clearance include intrinsic structural and cellular components that can be impaired or inhibited through developmental defects and surgical interventions. Tracheomalacia is a developmental defect of the airway that can contribute to the mechanical failure to clear the airway. This chapter will review the mechanisms of airway clearance and the processes that can impair this vital process.

Prenatally diagnosed omphaloceles: Report of 92 cases and association with Beckwith-Wiedemann syndrome.

OBJECTIVE: Describe the prevalence, perinatal and long-term outcomes of Beckwith-Wiedemann syndrome (BWS) among prenatally detected omphaloceles. METHODS: All prenatally diagnosed omphaloceles from 2010 to 2015 within a single tertiary care centre were identified. An echocardiogram and detailed fetal ultrasound were performed, and amniocentesis was offered with karyotype/microarray analysis and BWS molecular testing. Perinatal, neonatal, and long-term outcomes were retrieved for BWS cases. RESULTS: Among 92 omphaloceles, 62 had additional anomalies. Abnormal karyotypes were identified in 23/62 (37%) non-isolated and 2/30 (7%) isolated cases. One BWS case (5%) was identified among non-isolated omphaloceles and six BWS cases (37.5%) were identified among isolated omphaloceles after exclusion of aneuploidy. Among 19 BWS cases, 21% were conceived by ART. All omphaloceles underwent primary closure. Prenatally, macrosomia and polyhydramnios were seen in 42%. Macroglossia and nephromegaly were more commonly detected postnatally. Preterm birth occurred in 10/19 (53%) cases and cesarean deliveries were performed in 7/19 (40%) cases. Overall mortality was 20% (4/19). Embryonal tumors were diagnosed in 2/16 (12.5%) children, and neurodevelopmental outcomes were normal in 9/12 (75%) survivors. CONCLUSIONS: After excluding aneuploidy, BWS was identified in 37.5% and 5% of isolated and non-isolated omphaloceles, respectively. Omphaloceles were small-moderate size with good long-term surgical and neurodevelopmental outcomes when isolated.

Preoperative delineation of pulmonary fissural anatomy at multi-detector computed tomography in children with congenital pulmonary malformations and impact on surgical complications and postoperative course.

BACKGROUND: Delineation of the anatomy and integrity of the pulmonary fissures at CT is important because anomalous or incomplete fissures might increase the risk of surgery and of postoperative complications. OBJECTIVE: To preoperatively evaluate the integrity of the pleural fissures in children with congenital lung malformations and determine whether anomalous fissural anatomy is a risk factor for a more complicated surgery and postoperative course. MATERIALS AND METHODS: We reviewed preoperative multi-detector CT scans of consecutive children who underwent open or thoracoscopic resection of a congenital pulmonary malformation from 2008 to 2018, to determine the integrity of the fissural anatomy, and compared these findings with the surgical report. We correlated postoperative factors including operating room time, days in hospital and chest tube with the operating room documented fissural integrity. RESULTS: We saw a significant association between the radiologically determined fissural integrity at CT and the operative findings independently for the right, left and both lungs combined (P<0.001). The sensitivity of CT to determine fissural integrity was 76.9%, specificity 95.2%, positive predictive value 95.2%, negative predictive value 76.9%, and accuracy 85.1%. There was a statistically significant association between size of the pulmonary malformation and the integrity of the fissure(s) (P=0.024). Larger lesions also resulted in a significantly longer hospitalization (P=0.024). CONCLUSION: Chest CT showed high accuracy for delineating fissural anatomy in children with congenital pulmonary malformations, with a good interobserver correlation. Incomplete lung fissures were found more often in children with larger congenital pulmonary malformations. In addition, larger lesions were associated with longer hospital stays. Therefore, children with incomplete fissures may have a longer postoperative course. Analysis of the fissural anatomy should be included in the CT report.

Fetal Sclerotherapy for Hydropic Congenital Cystic Adenomatoid Malformations of the Lung Refractory to Steroids: A Case Report and Review of the Literature.

Microcystic congenital cystic adenomatoid malformations (CCAM), when associated with hydrops, carry a dismal prognosis. Options for treatment are limited and experimental, including antenatal corticosteroids, open fetal surgery, laser ablation and, more recently, sclerotherapy. We describe a case of a large, predominantly microcystic CCAM in a hydropic fetus treated successfully with direct interstitial injection of a sclerosant agent (3% sodium tetradecyl sulfate) at 23+3 weeks gestation, after multiple failed courses of steroids. Elective thoracoscopic right lower lobectomy was performed at 1 year of life and there have been no respiratory or other medical morbidities since. A literature review of fetal lung masses treated with sclerosants antenatally reveals that sclerotherapy may represent a novel treatment option for large hydropic microcystic CCAMs, which are unresponsive to corticosteroids. Further studies are required to evaluate the utility and safety of fetal sclerotherapy, as this may represent an alternative minimally invasive treatment option to fetal lobectomy.

Outcomes of multi-gestational pregnancies affected by esophageal atresia - tracheoesophageal fistula.

BACKGROUND: Contemporary outcomes of infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) from multi-gestational pregnancies compared to those of singleton pregnancies have not been reported. METHODS: A single-center retrospective review of EA/TEF patients born from 1999 to 2013 was performed. Patient demographics, gestational age (GA), birth weight, associated anomalies, requirement for gastrostomy tube and mortality were reviewed. RESULTS: Singleton EA/TEF patients outnumbered those from multi-gestational pregnancies nearly 10:1 (214 vs 22 patients). EA/TEF patients from multi-gestational pregnancies were more likely to be premature (77% vs. 32%), have lower birth weight (mean 1766 g vs. 2695 g), have associated duodenal atresia (18% vs. 6%) and require gastrostomy tube (41% vs. 33%) for feeding challenges compared to EA/TEF singletons. Mortality was also significantly greater for multi-gestational EA/TEF patients compared to singleton EA/TEF patients (18% vs. 6%). CONCLUSION: EA/TEF infants from multi-gestational pregnancies have greater clinical complexity and mortality than singleton EA/TEF patients. Parents of EA/TEF multi-gestational infants should be appropriately counseled and supported.

Management of ovarian lesions diagnosed during infancy.

PURPOSE: Prior studies of infant ovarian cysts have recommended intervention for those larger than 4 cm. We reviewed the natural history and features of those managed operatively versus nonoperatively. METHODS: A retrospective study was performed of ovarian lesions in children <1 year-old from 2000 to 2014. RESULTS: Forty patients were identified. Twenty-eight (70%) underwent operative management, while 12 (30%) were managed conservatively, including one undergoing aspiration. The mean age at surgery was 125 days. All but one patient (96%) had evidence of antenatal torsion intraoperatively or on final pathology. All resected lesions were benign. Ultrasound findings more common in those undergoing surgery included intracystic debris (p < 0.001), fluid-fluid or fluid-debris levels (p = 0.002), absence of Doppler flow (p = 0.014), solid components (p = 0.04), and calcifications (p = 0.001). Cysts managed nonoperatively had an average diameter of 2.5 cm, compared to 5.1 cm in the operative group (p < 0.001). Three of the lesions managed nonoperatively had a diameter greater than 3.5 cm (mean 5.1 cm) and were followed for an average of 153 days until resolution. CONCLUSIONS: The majority of infant ovarian lesions were excised, although none were malignant. Aspiration or observation of larger cysts was feasible and safe. Ovarian cysts in this age group should be considered for nonoperative management and closely followed. LEVEL OF EVIDENCE: IIC.

Requirement and Duration of Tube Feed Supplementation among Congenital Diaphragmatic Hernia Patients.

PURPOSE: Oral feeds pose a challenge for congenital diaphragmatic hernia (CDH) infants. Tube feed (TF) supplementation may be required to support the achievement of normal growth. The aim of this study was to determine the duration and factors associated with TF use in CDH infants at our institution. METHODS: A single centre retrospective chart review was performed for CDH-born infants who underwent repair between 2000 to 2013 (REB #1000053124). Patient demographics, perinatal management, and feeding status of infants with at least 1-year follow-up were reviewed. RESULTS: Of 160 CDH infants, 32 (20%) were discharged on partial or complete TF, and an additional 5 (3.1%) patients started TF post discharge. CDH infants with TF were more likely to have initial arterial blood pH < 7.25, patch repair, ECMO support, and prolonged ICU stay (p < 0.05). Time to TF discontinuation did not differ significantly between those partially or fully TF at discharge. Twelve patients (33.3%) remained TF at their last known follow-up. CONCLUSION: High risk CDH patients are likely to require TF to support their nutritional intake. Parents and caregivers need to be informed and properly supported. Long-term monitoring of CDH patient oral intake, growth, and development will be required. LEVEL OF EVIDENCE/TYPE OF STUDY: Level III Retrospective Study.

Vocal cord dysfunction following esophageal atresia and tracheoesophageal fistula (EA/TEF) repair.

BACKGROUND: The purpose of this study was to determine risk factors and long-term outcomes in patients with esophageal atresia +/-tracheoesophageal fistula (EA/TEF) with vocal cord dysfunction (VCD) owing to recurrent laryngeal nerve (RLN) injury. METHOD: A retrospective chart review was performed for EA/TEF patients repaired at our institution from 1999 to 2014 (REB #1000032265). RESULTS: Of 197 patients, 22 (11.2%) were diagnosed with VCD by indirect laryngoscopy following EA/TEF repair. Aspiration was documented on video swallow study for 21 patients, and as a result, 13 required thickened feeds and 8 required gastrostomy tube feeds. Of the 16 H-type TEF patients, 8 (50%) had VCD. Following discharge, 20 (90.9%) patients with VCD eventually tolerated full feeds orally without aspiration but only 8 (36.4%) had documented recovery of vocal cord movement at long-term follow up (mean 452 days). Overall, patients with VCD were more likely to have feeding modifications, increased exposure to radiological studies, and increased frequency of Otolaryngology follow-up compared to EA/TEF patients without VCD. CONCLUSION: EA/TEF patients are at risk for VCD. Clinical improvement did not always correlate with recovery of VC motion. Strategies to minimize RLN damage will improve outcomes and quality of life for EA/TEF patients. LEVEL OF EVIDENCE: Level III.

Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia.

PURPOSE: The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors. METHODS: A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance. RESULTS: Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group. CONCLUSION: Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood.

Pulmonary hypertension in congenital diaphragmatic hernia patients: Prognostic markers and long-term outcomes.

Prenatal observed/expected lung-to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients. METHODS: A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5years of age. RESULTS: Of 153 newborns, 123 survived (80.4%), 58 (37.9%) had prenatal O/E LHR, and 42 (27.5%) had postnatal ECHO results. High mortality risk neonates (O/E LHR ≤45%) correlated with higher right ventricular systolic pressure (RVsp) at birth. Generally PH resolved by age 5years. LPS results did not change over time (p>0.05) regardless of initial PH severity, suggesting that PH resolution did not correlate with increased ipsilateral lung perfusion to offload the right ventricle. CONCLUSION: Prenatal prognostic markers correlated with initial PH severity for CDH newborns, but PH resolved over time despite fixed perfusion bias to the lungs. These results suggest favorable PH outcomes for CDH patients who survive beyond infancy. TYPE OF STUDY: Retrospective Cohort Study. LEVEL OF EVIDENCE: 3b.