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Small bowel obstruction (SBO) is a known complication after congenital diaphragmatic hernia (CDH) repair, which can require surgery and even extensive bowel resection causing short bowel syndrome (SBS). We investigate whether specific bowel rotation and fixation can be used as a predictor for SBO including volvulus.A retrospective review of 256 CDH survivors following repair from 2003 to 2020 was performed. Operative notes and upper gastrointestinal series (UGI) were screened to determine the rotation and fixation of the bowel. Primary outcomes included SBO occurrence, SBO treated surgically, and volvulus. For statistical analysis Fisher's exact test was utilized.Twenty-two (9%) patients presented with SBO and majority, 19 (86%), required surgery. Adhesion were observed in 10 (45%), recurrence in 5 (23%), and extensive volvulus leading to SBS in 3 (14%). Both rotation and fixation were recorded in 117 (46%). Presence of left CDH with malrotation and nonfixation was a significant predictor for SBO requiring surgery (P0.05 vs all other groups). All 3 patients with extensive volvulus had left CDH with nonfixed bowel (100%), however only 1 had malrotation (33%).Malrotation and nonfixation are associated with increased SBO in CDH. Normal rotation is not protective and patients are still at risk for volvulus resulting in SBS. SBO requiring surgical intervention is common in CDH. Bowel rotation and fixation are important determinants that, should be routinely documented and education about the risk of SBO should be included in family counseling.Level IV - Case Series.

To characterize the prevalence, associations, management, and outcomes of supraventricular tachycardia (SVT) in neonates with congenital diaphragmatic hernia (CDH).Retrospective chart and cardiology code review within a cohort of patients with CDH was used to define a subpopulation with atrial arrhythmia. SVT mechanisms were confirmed by electrocardiogram analysis. Cox proportional hazard regression identified risk factors for SVT and association with clinical outcomes.Medical Surgical ICU in a single, tertiary center, Boston Children's Hospital.Eligible patients included neonates presenting with classic Bochdalek posterolateral CDH between 2005 and 2017, excluding newborns with Morgagni hernia or late diagnoses of CDH (28 d).None.SVT arose in 25 of 232 neonates with CDH, (11%); 14 of 25 infants (56%) had recurrent SVT; atrioventricular node-dependent tachycardia was the most frequent mechanism (32%). The majority (71%) of SVT episodes received intervention. Nine patients (36%) received preventative antiarrhythmic medications. SVT was associated with lower Apgar score at 1 min, structural heart disease, larger defect size, extracorporeal membrane oxygenation (ECMO) support, and prostaglandin therapy for ductal patency as well as hospital stay greater than or equal to 8 weeks and use of supplemental oxygen at discharge.SVT can occur in neonates with CDH and frequently requires treatment. Odds of occurrence are increased with greater CDH disease severity, ECMO, and prostaglandin use. In unadjusted logistic regression analysis, SVT was associated with adverse hospital outcomes, underscoring the importance of recognition and management in this vulnerable population.

RationaleCongenital diaphragmatic hernia (CDH) is characterized by incomplete closure of the diaphragm and lung hypoplasia. The pathophysiology of lung defects in CDH is poorly understood.ObjectivesTo establish a translational model of human airway epithelium in CDH for pathogenic investigation and therapeutic testing.MethodsWe developed a robust methodology of epithelial progenitor derivation from tracheal aspirates of newborns. Basal stem cells (BSCs) from CDH patients and preterm and term, non-CDH controls were derived and analyzed by bulk RNA-sequencing, ATAC-sequencing, and air-liquidinterface differentiation. Lung sections from fetal human CDH samples and the nitrofen rat model of CDH were subjected to histological assessment of epithelial defects. Therapeutics to restore epithelial differentiation were evaluated in human epithelial cell culture and the nitrofen rat model of CDH.Measurements and Main ResultsTranscriptomic and epigenetic profiling of CDH and non-CDH basal stem cells reveals a disease-specific, proinflammatory signature independent of severity or hernia size. In addition, CDH basal stem cells exhibit defective epithelial differentiationin vitrothat recapitulates epithelial phenotypes found in fetal human CDH lung samples and fetal tracheas of the nitrofen rat model of CDH. Furthermore, steroid treatment normalizes epithelial differentiation phenotypes of human CDH basal stem cellsin vitroand in nitrofen rat tracheasin vivo.ConclusionsOur findings have identified an underlying proinflammatory signature and BSC differentiation defects as a potential therapeutic target for airway epithelial defects in CDH.

Pediatric functional constipation (FC) may require invasive evaluations [like colon manometry (CM)] and surgical interventions [including diverting ostomy (DO)]. We evaluated the utility of CM in guiding surgery after DO.Children with medically refractory FC undergoing an ostomy were included. Institutional Review Board approval was obtained for this retrospective study. Demographics and CM variables [high amplitude propagating contractions (HAPCs)] were recorded. Outcome measures: response to ostomy closure defined as successful if no need for further surgery after ostomy closure, and improvement on baseline CM after ostomy. A CM-guided ostomy closure algorithm was developed based on previous studies. We evaluated the association between response to ostomy closure and demographics, ostomy indication and CM improvement, and evaluated the role of CM predicting response using algorithm.A total of 60 children underwent ostomy for FC (median age: 7.1 years, range 0.15-23.6 years, 50% female). Ostomy was closed in 30 patients and deemed successful in 23 of 30. CM was performed in 42 of 60 patients before ostomy and in 29 of 30 before ostomy closure. We found no association between ostomy outcome and age, gender, weight, imaging studies, follow-up time, time with ostomy, HAPCs, and CM improvement. We found an association between failed response and ostomy indication of antegrade colonic enemas (ACE) failure ( P = 0.026) and successful response when ostomy closure was guided by algorithm ( P = 0.03).DO is a useful intervention in selected children with medically refractory FC, improving colon motility in most. CM can successfully guide the timing and type of ostomy closure. Larger studies are needed to further validate our findings.

Introduction: Prenatal suprarenal lesions represent diverse pathologies. This study investigated prenatal imaging features and regression patterns associated with specific lesion diagnoses. Methods: This is a multicenter retrospective review of fetuses with prenatally diagnosed suprarenal lesions between 2001 and 2019. Prenatal ultrasound and MRI characteristics, postnatal imaging, and clinical course were reviewed. Prenatal imaging findings were compared by the most common diagnoses and regression patterns. Results: Forty-four fetuses were prenatally diagnosed with suprarenal lesions. Diagnoses included pulmonary sequestration (n = 12; 27.3%), adrenal hemorrhage (n = 12; 27.3%), upper quadrant cyst (including 2 duplication cysts, 1 splenic cyst, and 3 indeterminate cysts), neuroblastoma (n = 4), adrenal hyperplasia (n = 3), bilateral adrenal calcifications (n = 1), and indeterminate lesions (n = 6). Sequestrations were uniformly left-sided (100 vs. 50%; p = 0.014) and diagnosed earlier in gestation than adrenal hemorrhages (p = 0.025). Sequestrations were also significantly more likely to have a prenatal feeding vessel (p = 0.005), low T1 MRI signal (p = 0.015), and no MRI blood products (p = 0.018) compared to adrenal hemorrhages. When comparing all 44 patients, a prenatal feeding vessel and low T1 signal on prenatal MRI were significantly associated with lesion persistence (p = 0.003; p = 0.044). Discussion/Conclusion: Imaging findings on prenatal ultrasound and MRI aid in the diagnosis of suprarenal lesions, including differentiating pulmonary sequestrations and adrenal hemorrhages.

Objectives Given significant focus on improving survival for "high-risk" congenital diaphragmatic hernia, there is the potential to overlook the need to identify risk factors for suboptimal outcomes in "low-risk" congenital diaphragmatic hernia cases. We hypothesized that early cardiac dysfunction or severe pulmonary hypertension were predictors of adverse outcomes in this "low-risk" congenital diaphragmatic hernia population. Design This is a retrospective cohort study using data from the Congenital Diaphragmatic Hernia Study Group registry. "Low-risk" congenital diaphragmatic hernia was defined as Congenital Diaphragmatic Hernia Study Group defect size A/B without structural cardiac and chromosomal anomalies. Examined risk factors included left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension on the first postnatal echocardiogram. The primary outcome was composite adverse events, defined as either death, extracorporeal membrane oxygenation utilization, oxygen requirement on day 30 of life, or hospitalization greater than or equal to 8 weeks. Multivariable adjustment was performed with logistic regression and inverse probability weighting. Setting Neonatal index hospitalization for congenital diaphragmatic hernia. Patients "Low-risk" congenital diaphragmatic hernia infants born between January 2015 and December 2018. Interventions First postnatal echocardiogram performed within 24 hours from birth. Measurements and main results Seven-hundred seventy-eight patients were identified as "low-risk" congenital diaphragmatic hernia. Left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension were present in 10.8%, 20.5%, and 57.5%, respectively. The primary outcome occurred in 21.3%. Death occurred in 3.0% and 9.1% used extracorporeal membrane oxygenation. On unadjusted analysis, all three risk factors were associated with the primary outcome. On all multivariable adjustment methods, left ventricular dysfunction and severe pulmonary hypertension remained significant predictors of adverse outcomes while right ventricular dysfunction no longer demonstrated any effect. Conclusions Early left ventricular dysfunction and severe pulmonary hypertension are independent predictors of adverse outcomes among "low-risk" congenital diaphragmatic hernia infants. Early recognition may lead to interventions that can improve outcome in this at-risk cohort.

Gastroschisis is a congenital abdominal wall defect that, when simple, has excellent overall outcomes. However, morbidity increases with prematurity. A staged approach to closure is often needed until the infant can tolerate definitive repair. We demonstrate the novel use of cadaveric skin allograft as a defect patch, exploiting a tolerant neonatal immune system for long-term durable coverage. A 580-g, 26-week-gestation boy was born with gastroschisis. Primary closure was not possible, necessitating staged closure. After initial silo placement, neither the fascia nor the skin could be closed. Therefore, cadaveric skin was utilized for coverage: there was 100% take, no wound care needs, and no acute rejection. He was discharged at 4 months tolerating full feeds. At 6 months signs of rejection ultimately manifested, and he underwent uneventful elective graft excision and fascial closure. We offer this as a useful option for management of staged gastroschisis closure in an extremely premature infant.

This retrospective cohort study compares the natural history of patients with extralobar sequestrations (ELS) who do not undergo intervention with those who undergo resection to assess the safety of non-operative management. 126 patients with pulmonary sequestrations or congenital pulmonary airway malformations born between 1999 and 2016 were identified. 49 patients had ELS on postnatal imaging, but two were excluded for associated congenital diaphragmatic hernia. Demographic and clinical data were retrospectively reviewed, with phone follow-up for non-operative patients with no records for > 1 year. Statistical analysis was by Fisher’s exact test or Wilcoxon signed-rank test (two-tailed p

Introduction: Survivors of congenital diaphragmatic hernia (CDH) are at risk of pulmonary hypertension (PH) due to pulmonary hypoplasia and other pulmonary vascular abnormalities. Hypothesis: Exercise stress echocardiography (ESE) may detect PH, not apparent on echocardiography at rest, in children and young adults with repaired CDH. Methods: Single center retrospective cohort study of all CDH survivors who underwent ESE at Boston Children’s Hospital (January 2006 – June 2020). Severity of PH was assessed at baseline and after exercise. Patients were assigned to one of two categories based on right ventricular pressure: group 1: no or mild PH after exercise; and group 2: moderate or severe PH after exercise (right ventricular pressure ≥ 60 mmHg or ≥ ½ systemic blood pressure). Results: During the study period 84 CDH patients (70% male) underwent 173 ESE with a median age 8.1 (4.8 – 19.1) years at first ESE. Sixty-four patients were classified as group 1, 11 as group 2, and 9 were unable to be classified due to inconclusive ESE. Moderate to severe PH after exercise was found in 8 (10%) patients with no or mild PH at rest. Risk factors for PH after exercise included larger CDH defect size, patch repair, use of extracorporeal membrane oxygenation, and supplemental oxygen at discharge in univariate analysis (Table 1). Exercise and pulmonary function test parameters associated with a group 2 test included higher VE/VCO2 slope, lower peak oxygen saturation, and lower percent predicted FEV1 and FEV1/FVC ratio. Nine of 11 group 2 patients had a change in management based on ESE. PH was found in all 5 group 2 patients with cardiac catheterization after ESE, including 3 patients with no or mild PH on baseline ESE. Conclusions: Among long term CDH survivors, ESE identified 10% with moderate-severe exercise induced PH indicating ongoing pulmonary vascular abnormalities. Further studies are needed to optimally define screening and treatment for patients with repaired CDH.

Aim of Study Congenital Diaphragmatic Hernia (CDH) is associated with lung hypoplasia and pulmonary hypertension. Many patients receive care in specialty centers requiring air travel upon discharge and for subsequent follow-up care. Premature infants can experience significant hypoxia in flight, but this has not been studied in the CDH population. This report describes our center's experience with simulated altitude testing among CDH patients. Methods In a single center retrospective cohort study, CDH patients who underwent a High Altitude Simulation Test (HAST) from 2006-2019 were analyzed. HAST simulates increased altitude by reducing oxygen tension to an FIO2 of 0.15. Patients were tested only when flight was anticipated. Patients requiring oxygen were challenged on their baseline requirement. To pass, patients had to maintain oxygen saturation >90%, and 94% if diagnosed with pulmonary hypertension. Supplemental oxygen was titrated as needed to achieve this goal. Results Of twenty patients tested, only six (30%) passed on their first attempt. Ten (50%) eventually passed, after an average of 3.2 additional attempts over 1.8 years. No patient passed initially who utilized ECMO support, diaphragmatic agenesis, or had elevated right ventricular pressure on echocardiogram. All patients achieved the targeted spO2 with supplemental oxygen. Conclusion CDH patients experience hypoxia when exposed to the simulated hypobaric nature of air travel and therefore may become hypoxic in flight, requiring oxygen supplementation. Disease severity seems to correlate with risk of in-flight hypoxia. This data suggests that CDH patients should be screened to assess their need for supplemental oxygen to ensure safe air travel.

OBJECTIVE To determine if risk-adjusted survival of patients with congenital diaphragmatic hernia (CDH) has improved over the last 25 years within centers that are long-term, consistent participants in the CDH Study Group (CDHSG). SUMMARY BACKGROUND DATA The CDHSG is a multicenter collaboration focused on evaluation of infants with CDH. Despite advances in pediatric surgical and intensive care, CDH mortality has appeared to plateau. Herein, we studied CDH mortality rates amongst long-term contributors to the CDHSG. METHODS We divided registry data into five-year intervals, with Era 1 (E1) beginning in 1995, and analyzed multiple variables (operative strategy, defect size, and mortality) to assess evolution of disease characteristics and severity over time. For mortality analyses, patients were risk stratified using a validated prediction score based on 5-minute Apgar (Apgar5) and birth weight. A risk-adjusted, observed to expected (O:E) mortality model was created using E1 as a reference. RESULTS 5,203 patients from 23 centers with ≥22 years of participation were included. Birth weight, Apgar5, diaphragmatic agenesis, and repair rate were unchanged over time (all p > 0.05). In E5 compared to E1, minimally invasive and patch repair were more prevalent, and timing of diaphragmatic repair was later (all p < 0.01). Overall mortality decreased over time: E1 (30.7%), E2 (30.3%), E3 (28.7%), E4 (26.0%), E5 (25.8%) (p = 0.03). Risk-adjusted mortality showed a significant improvement in E5 compared to E1 (OR 0.78, 95% CI 0.62-0.98; p = 0.03). O:E mortality improved over time, with the greatest improvement in E5. CONCLUSIONS Risk-adjusted and observed-to-expected CDH mortality have improved over time.

Purpose Prenatal occurrence and timing of appearance of associated features in Beckwith-Wiedemann syndrome (BWS) are unknown. We reviewed our BWS patients with serial fetal imaging and correlated these with postnatal findings. Methods All BWS patients with fetal ultrasound (US) or magnetic resonance imaging (MRI) from 2000 to 2016 were reviewed to determine the presence of polyhydramnios, placentamegaly, macrosomia, macroglossia, retrognathia, omphalocele, visceromegaly, and hemihypertrophy. These observations were correlated with postnatal findings. Data were analyzed by Mann-Whitney U test. Results Nine BWS patients underwent 42 fetal imaging studies with median of five (range of two to six) studies per patient between 13 and 35 weeks gestation. All prenatal findings were confirmed postnatally with complete concordance. All patients with omphalocele were detected early in gestation but other postnatal findings less predictably so. All omphaloceles were small, and were found significantly earlier in gestation than macrosomia (P = 0.004) and macroglossia (P = 0.012). Visceromegaly and retrognathia were less frequent, with no significant differences in median gestational age from omphalocele when prenatally identified. Conclusions In BWS, omphalocele is the most common prenatal finding and routinely observed in early gestation with 100% accuracy. Associated findings of macrosomia, macroglossia, visceromegaly, and retrognathia, when present, are detected later in gestation. Imaging in later gestation may reveal additional abnormalities that support a BWS diagnosis.

Although the quality of online health information (OHI) for adult surgical conditions is well described, the availability of quality OHI for pediatric surgical conditions, and the comparison to that of adult surgical OHI, remains undefined. Medical and lay terms for 15 pediatric and 15 adult surgical conditions were searched using Google in English. The Health on the Net Foundation, a non-governmental OHI accreditation body, designates approval for quality websites. We compared the role of patient population while controlling for disease incidence (pediatric vs. adult), term complexity (medical vs. lay), and order (earlier vs. later listing of websites) on availability of quality OHI among the first 100 websites for each term. Among the first 100 websites, the adjusted mean number of quality websites was 11.80 for pediatric vs. 17.92 for adult medical search terms, and 13.27 for pediatric vs. 18.20 for adult lay search terms (P

Introduction: Several fetal therapies involve repeated amniotic fluid intervention. We hypothesize that a minimally invasive approach can be used to safely implant an intrauterine catheter infusion system in a fetal ovine model for chronic use during pregnancy. Method: Five pregnant sheep underwent operation between gestational days 110 and 115 (term 145 days). A Codman® implantable infusion pump was adapted for intrauterine use. The chamber was placed in the maternal flank and the tunneled catheter laparoscopically inserted into the amniotic cavity, secured with a pursestring. Three had an additional uterine anchoring suture. Ewes were sacrificed after natural delivery, and the uterus underwent gross and microscopic analyses. Results: There were no maternal mortalities, abortions, or preterm labor. Pumps were accessed and remained functional throughout gestation. Four ewes delivered healthy term lambs; the other delivered twins with failure to progress and demise. On necropsy, catheters secured with an anchoring suture remained in place, while the other 2 dislodged during labor. There was no chorioamnionitis by culture or histology. Conclusion: Laparoscopically placed intra-amniotic infusion catheters were implanted safely and remained functional until delivery in an ovine model. This novel approach has promise in providing safe, durable amniotic fluid access for the potential treatment of fetal disease.

Infants prenatally suspected of having a choledochal cyst (CDC) typically undergo ultrasound imaging shortly after birth. This study sought to evaluate features on the initial postnatal ultrasound (IPU) that could identify newborns at risk for early complications.Following IRB approval, patients from four US fetal centers with prenatal suspicion for CDC and postnatal imaging from 2000 to 2017 were reviewed. Imaging and clinical courses were assessed.Forty-two patients had prenatal ultrasounds suspicious for CDC. Nineteen (45.2%) were excluded due to diagnostic revision (In newborns with prenatally diagnosed CDC, a cyst size ≥ 4.5 cm on IPU is associated with symptom development during the first month after birth and therefore early cyst excision is recommended.

Esophageal atresia (EA) is a congenital anomaly occurring in 2.3 per 10,000 live births. Due to advances in prenatal imaging, EA is more readily diagnosed, but data on the associated genetic diagnoses, other anomalies, and postnatal outcome for fetuses diagnosed prenatally with EA are scarce. We collected data from two academic medical centers (n = 61). Our data included fetuses with suspected EA on prenatal imaging that was confirmed postnatally and had at least one genetic test. In our cohort of 61 cases, 29 (49%) were born prematurely and 19% of those born alive died in the first 9 years of life. The most commonly associated birth defects were cardiac anomalies (67%) and spine anomalies (50%). A diagnosis was made in 61% of the cases; the most common diagnoses were vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula with esophageal atresia, radial or renal dysplasia, and limb anomalies association (43%, although 12% met only 2 of the criteria), trisomy 21 (5%), and CHARGE syndrome (5%). Our findings suggest that most fetuses with prenatally diagnosed EA have one or more additional major anomaly that warrants a more comprehensive clinical genetics evaluation. Fetuses diagnosed prenatally appear to represent a cohort with a worse outcome.

The Spitz classification for esophageal atresia with/without tracheoesophageal fistula (EA/TEF) predicts mortality. This study evaluates the contemporary relevance of the Spitz classification and investigates predictors of morbidity.EA/TEF patients born between 1995 and 2018 at two centers were retrospectively reviewed. Clinical variables including sex, prenatal diagnosis, birth weight, prematurity, major congenital heart disease (MCHD), and pre-operative mechanical ventilation (POMV) were collected. Index admission composite morbidity was considered positive if: length-of-stay90th percentile (139 days), ventilation days90th percentile (24 days), and/or gastrostomy was used for long-term feeding. Multivariable regression determined predictors of index admission mortality and composite morbidity. A composite morbidity predictive algorithm was created. ROC curves evaluated model discrimination.Of 253 patients, 13 (5.1%) experienced index admission mortality. Of the patients not suffering mortality, 74 (31.6%) experienced composite morbidity. Only MCHD predicted mortality (p = 0.001); birth weight did not (p = 0.173). There was no difference between the Spitz classification and MCHD alone in predicting mortality risk (p = 0.198); both demonstrated very good discrimination. Prenatal diagnosis, POMV, prematurity, and male sex predicted composite morbidity risk (p 0.001; p = 0.008; p = 0.009; p = 0.05). An algorithm incorporating these predictors demonstrated good discrimination (AUC = 0.784; 95% CI: 0.724, 0.844).The Spitz classification maintains contemporary relevance for mortality risk, though birth weight can be de-emphasized. A new morbidity risk algorithm is proposed for early postnatal counseling.Prognosis study.Level IV.

Percent predicted lung volume (PPLV)15% on fetal MRI predicts high-risk CDH. Potential changes in PPLV throughout gestation and impact on risk stratification are unknown. We reviewed CDH patients with serial fetal MRIs to follow PPLV and determine correlation with postnatal outcomes.CDH patients with serial fetal MRIs from 2005 to 2015 were included. We recorded prenatal MRI gestational age (GA) and PPLV, postnatal ECMO use, and survival. Data were analyzed by logistic regression and Fisher's exact test.57 patients had 127 fetal MRI studies. PPLV decreased from mean 25.4% to 19.6% between GA 22.1 and 32.6weeks. A steeper decline in PPLV, regardless of final PPLV, was independently predictive of higher ECMO use (p=0.046) and death (p=0.045). All patients with first PPLV15% remained high-risk with poor outcomes. Of those with first PPLV15%, 31% dropped below 15%, having similar ECMO use as the high-risk cohort, but trending toward greater survival (p=0.09). Those with first and final PPLV15% had significantly less ECMO use (p=0.015) and greater survival (p0.001) than the high-risk cohort.On average, PPLV decreases throughout gestation in fetuses with CDH. Serial MRI is recommended for those with initial PPLV15%, as clinical outcomes tend to mirror the lowest PPLV.Treatment study LEVEL OF EVIDENCE: III.

In high-risk congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before extracorporeal membrane oxygenation (ECMO) can be initiated. We previously examined ex utero intrapartum treatment (EXIT)-to-ECMO in our most severe CDH patients, but demonstrated no survival advantage. We now report morbidity outcomes in survivors of this high-risk cohort to determine whether EXIT-to-ECMO conferred any benefit.All CDH survivors with15% predicted lung volume (PPLV) from September 1999 to December 2010 were included. We recorded prenatal imaging, defect size, and pulmonary, nutritional, cardiac, and neurodevelopmental outcomes.Seventeen survivors (8 EXIT-to-ECMO, 9 non-EXIT) had an average PPLV of 11.7%. Eight of 9 non-EXIT received ECMO within 2days. There were no significant defect size differences between groups, mostly left-sided (13/17) and type D (12/17). Average follow-up was 6.7years (0-13years). There were no statistically significant differences in outcomes, including supplemental oxygen, diuretics, gastrostomy, weight-for-age Z scores, fundoplication, pulmonary hypertension, stroke or intracranial hemorrhage rate, CDH recurrence, and reoperation. No survivor in our cohort was neurologically devastated. All had mild motor and/or speech delay, which improved in most.In this pilot series of severe CDH survivors, EXIT-to-ECMO confers neither significant survival nor long-term morbidity benefit.Level III treatment study.

OBJECTIVE To determine the optimal timing of congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) cannulation. SUMMARY BACKGROUND DATA The timing of CDH repair after ECMO cannulation remains a controversial topic due to studies with low power or strong selection bias. METHODS This is a 2-aim retrospective cohort study based on the CDH Study Group registry for the period of 2007-2017. Aim 1-Compare On versus After ECMO repair. Aim 2-Compare Early versus Late repair on ECMO. In order to minimize selection bias and account for non-repairs, subjects in each aim were stratified into study groups based on their treatment center's characteristics. In each aim, the study groups were matched based on propensity score (PS). The main outcomes included mortality rate and incidence of non-repair. RESULTS In aim 1, 136 patients remained in each group after PS matching. Compared to the After ECMO group, patients in the On ECMO group demonstrated a lower mortality rate, hazard ratio (HR) 0.54 (0.38, 0.77) (P < 0.001), and lower incidence of non-repair, 5.9% versus 33.8% (P < 0.001). In aim 2, 77 patients remained in each group after PS matching. Compared to the Late group, Early repair of CDH on ECMO was associated with a lower mortality rate, HR 0.51 (0.33, 0.77) (P = 0.002), and lower incidence of non-repair, 9.1% versus 44.2% (P < 0.001). CONCLUSIONS The approach of early repair after ECMO cannulation is associated with improved survival compared to delayed surgical correction.

To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH).This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model.During the study period, 212 patients had at least one V/Q study. The average ipsilateral V/Q of the cohort increased over time (P .01), an effect driven by progressive reduction in relative perfusion (P = .012). A higher V/Q ratio was correlated with lower body mass index percentile (P .001) and higher probability of poor functional status (New York Heart Association class III or IV) (P = .045).In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.

Jejunoileal atresia (JIA) is a congenital defect that can result in significant loss of bowel length. The traditional classification of JIA was first proposed by Grosfeld and includes 4 subtypes. Among these, type IIIB, or apple-peel atresia, is characterized by a proximal atretic jejunum and a distal segment of spiraled bowel that terminates at the cecum. Owing to this anatomy, patients with type IIIB JIA are at increased risk for short bowel syndrome and intestinal failure. In this report, we described the case of a neonate with a prenatal diagnosis of JIA. At exploration, she was initially found to have a type IIIB atresia. However, instead of terminating at the cecum, the distal spiraled segment was followed by 75 cm of normal small bowel and mesentery. Surgical correction proceeded with minimal resection and primary anastomosis. She recovered well from this procedure, tolerated full enteral nutrition by mouth, and displayed good weight gain at outpatient follow-up. Owing to the unique anatomy of the gastrointestinal tract in this case report, we propose the addition of a new class of JIA, type IIIC, to better reflect its prognostication and surgical management.

This study describes trends in emergency department visits for ingestions by children of small neodymium magnets between 2009 and 2019, before, during, and after a 2014 Consumer Product Safety Commission rule restricting their sales.

Complex tracheobronchial obstruction and malacia can be associated with conotruncal and aortic arch anomalies. A circumflex aortic arch composed of a left aortic arch and right descending aorta is an extremely rare anomaly that can severely affect the distal trachea and mainstem bronchi, resulting in severe respiratory symptoms. We report the case of a patient with circumflex aortic arch and severe left mainstem bronchial obstruction and malacia in which the external aortic compression and residual bronchomalacia were addressed with direct bronchial and tracheal intervention.

Survivorship of patients with congenital diaphragmatic hernia (CDH) has created a unique cohort of children, adolescents and adults with complex medical and surgical needs. Morbidities specific to this disease benefit from multi-specialty care, and the long term follow up of these patients offers a tremendous opportunity for research and collaboration. Herein we aim to offer an overview of the challenges that modern CDH survivors face, and include a risk-stratified algorithm as a general guideline for a multi-specialty follow up program.

OBJECTIVE: To analyze longitudinal trends of pulmonary function test (PFT) in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic. STUDY DESIGN: This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in one second (FEV(1)pp), percent predicted forced vital capacity (FVCpp), and FEV(1)/FVC over time. RESULTS: Of 268 CDH patients that survived to discharge, 119 had at least one PFT study. FEV(1)pp (P

Purpose Children failing medical management for severe constipation and/or fecal incontinence may undergo surgical intervention for antegrade enema administration. We present a modification of the laparoscopic-assisted percutaneous endoscopic cecostomy (LAPEC) procedure that allows primary placement of a skin-level device. Methods A single-institution retrospective review was performed from 2009 to 2015. In the modified technique the colonoscope is advanced to the cecum, cecal suspension sutures are placed under laparoscopic visualization, and percutaneous needle puncture of the cecum is performed under direct laparoscopic and endoscopic visualization. A skin-level cecostomy tube is then placed over a guide wire. Patient characteristics and 30-day results were analyzed by Fisher's exact test. Results Fifty-two patients underwent attempted LAPEC. Successful LAPEC using both laparoscopic and endoscopic guidance was achieved in 46 (88.5%). A MIC-KEY device was placed in 38. Corflo PEG tube placement was necessary in 14 due to high BMI (mean 28.4). Colonoscopy failed to reach the cecum in 6 and laparoscopy alone was utilized to achieve successful tube placement. Cecostomy site infections occurred in 3 (5.8%), only in those undergoing PEG placement using a pull technique (p Conclusion Primary placement of a skin-level device was successful in the majority of patients undergoing cecostomy tube placement for bowel management utilizing antegrade colonic enemas. This technique avoids a second anesthesia for tube conversion. Visualization via colonoscopy with the use of cecal suspension sutures is recommended. High BMI necessitates initial placement of a PEG tube and complications exclusively occurred in this group. Type of Study Clinical. Level of Evidence IV Case series study.

To determine (1) whether fetal care paediatric (FCP) and maternal-fetal medicine (MFM) specialists harbour differing attitudes about pregnancy termination for congenital fetal conditions, their perceived responsibilities to pregnant women and fetuses, and the fetus as a patient and (2) whether self-perceived primary responsibilities to fetuses and women and views about the fetus as a patient are associated with attitudes about clinical care.Mail survey of 434 MFM and FCP specialists (response rates 60.9% and 54.2%, respectively).MFMs were more likely than FCPs to disagree with these statements (all p values0.005): (1) 'the presence of a fetal abnormality is not an appropriate reason for a couple to consider pregnancy termination' (MFM : FCP-78.4% vs 63.5%); (2) 'the effects that a child born with disabilities might have on marital and family relationships is not an appropriate reason for a couple to consider pregnancy termination' (MFM : FCP-80.5% vs 70.2%); and (3) 'the cost of healthcare for the future child is not an appropriate reason for a couple to consider pregnancy termination' (MFM : FCP-73.5% vs 55.9%). 65% MFMs versus 47% FCPs disagreed that their professional responsibility is to focus primarily on fetal well-being (p0.01). Specialists did not differ regarding the fetus as a separate patient. Responses about self-perceived responsibility to focus on fetal well-being were associated with clinical practice attitudes.Independent of demographic and sociopolitical characteristics, FCPs and MFMs possess divergent ethical sensitivities regarding pregnancy termination, pregnant women and fetuses, which may influence clinical care.

OBJECTIVES:The expansion of pediatric-based fetal care raises questions regarding pediatric specialists’ involvement in pregnancies when maternal conditions may affect pediatric outcomes. For several such conditions, we compared pediatric and obstetric specialists’ attitudes regarding whether and when pediatrics consultation should be offered and their views about seeking court authorization to override maternal refusal of physician recommendations.METHODS:We used a mail survey of 434 maternal-fetal medicine specialists (MFMs) and fetal care pediatric specialists (FCPs) (response rate: MFM, 60.9%; FCP, 54.2%).RESULTS:FCPs were more likely than MFMs to indicate that pediatric counseling should occur before decisions regarding continuing or interrupting pregnancies complicated by maternal alcohol abuse (FCP versus MFM: 63% vs 36%), cocaine abuse (FCP versus MFM: 60% vs 32%), use of seizure medications (FCP versus MFM: 62% vs 33%), and diabetes (FCP versus MFM: 56% vs 27%) (all P < .001). For all conditions, MFMs were more than twice as likely as FCPs to think that no pediatric specialist consultation was ever necessary. FCPs were more likely to agree that seeking court interventions was appropriate for maternal refusal to enter a program to discontinue cocaine use (FCP versus MFM: 72% vs 33%), refusal of azidothymidine to prevent perinatal HIV transmission (80% vs 41%), and refusal of percutaneous transfusion for fetal anemia (62% vs 28%) (all P < .001).CONCLUSIONS:Pediatric and obstetric specialists differ considerably regarding pediatric specialists’ role in prenatal care for maternal conditions, and regarding whether to seek judicial intervention for maternal refusal of recommended treatment.

We report late-onset hypertrophic pyloric stenosis in a 17-year-old female. She presented with abdominal pain and an episode of upper gastrointestinal hemorrhage and subsequently developed gastric outlet obstruction. Work-up revealed circumferential pyloric thickening, delayed gastric emptying, and a stenotic, elongated pyloric channel. Biopsies showed benign gastropathy, negative for Helicobacter pylori, without eosinophilic infiltrates. Botulinum toxin injection provided limited relief. Diagnostic laparoscopy confirmed the hypertrophic pylorus and we performed laparoscopic pyloromyotomy. The patient tolerated the procedure well and had complete symptom resolution at 1-year follow-up. Hypertrophic pyloric stenosis is a rare cause of gastric outlet obstruction in adolescents and may be managed successfully with laparoscopic pyloromyotomy.

Purpose In the most severe cases of congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before advanced therapies such as extracorporeal membrane oxygenation (ECMO) can be initiated. We have previously examined the use of the ex utero intrapartum treatment (EXIT) to ECMO procedure (EXIT with placement on ECMO) in high-risk infants and reported a survival advantage. We report our experience with EXIT to ECMO in a more recent cohort of our patients with most severe CDH. Methods Every patient with less than 15% predicted lung volume during January 2005 to December 2010 was included. We obtained data on prenatal imaging, size and location of the defect, and survival. Results Seventeen high-risk infants were identified. All 17 (100%) received ECMO and required a patch. Six children were delivered by EXIT to ECMO, and only 2 (33%) survived. An additional patient was delivered by EXIT to intubation with ECMO on standby and died. Of the 10 children who did not receive EXIT, 5 (50%) survived. Conclusions No clear survival benefit with the use of the EXIT to ECMO procedure was demonstrated in this updated report of our high-risk CDH population. The general application of EXIT to ECMO for CDH is not supported by our results.

Background/Purpose Severe neonatal pulmonary hypoplasia incurs mortality rates approaching 71% to 95%. We sought to determine the utility of serial amnioinfusions through a subcutaneously implanted intraamniotic catheter to prevent pulmonary hypoplasia in fetal obstructive uropathy. Methods Fetal lambs (n = 32) were divided into 3 groups. Group I (n = 12) underwent a sham operation, group II (n = 15) underwent a complete urinary tract obstruction via ligation of the urachus and urethra with a subcutaneous tunneled intraamniotic port-a-cath without amnioinfusions, and group III (n = 5) underwent a creation of a complete urinary tract obstruction with a port-a-cath as described in group II with serial amnioinfusions. Lung tissue was analyzed by lung volume to body weight ratios and stereology. Statistical analysis was performed by analysis of variance and Bonferroni comparisons ( P Results Obstructed fetuses grossly had smaller lungs than treated and control animals. Lung volume to body weight ratios were statistically significant between groups. Airspace fractions were comparable between groups I and III (average = 0.53 and 0.55, respectively), although both were significantly greater than group II (average = 0.48) ( P = .049). Conclusions Serial amnioinfusions through an intraamniotic port-a-cath prevented pulmonary hypoplasia in an ovine model of complete obstructive uropathy. The use of an easily accessible device for amnioinfusions may be a viable option to treat oligohydramnios.

We aimed to determine whether the profile of matrix metalloproteinase (MMP) activity in fetal urine correlates with the degree of kidney damage in the setting of congenital obstructive uropathy.Fetal lambs underwent either a sham operation or creation of a complete urinary tract obstruction. Necropsies were performed before term, when urinary MMP profiling was performed by zymography; and kidney damage was assessed histologically by multiple semiquantitative analyses and histomorphometric measurements.There was a significant correlation between inner medullary thickness and MMP-9 (P = .005) and 63-kd MMP-2 (P = .019) activities. In like manner, the only MMPs associated with kidney fibrosis were MMP-9 and 63-kd MMP-2. Matrix metalloproteinase-9 activity was a highly significant independent predictor of the total combined kidney fibrosis score (P.001) as well as of higher fibrosis grades in each of 6 kidney areas analyzed (all with P.01). The activity of 63-kd MMP-2 correlated significantly with higher fibrosis in select areas.In a fetal ovine model, urinary MMP activity correlates with the degree of kidney damage. The presence of MMP-9 (in particular) and that of 63-kd MMP-2 are independent predictors of severity. Prenatal urinary MMP profiling may enhance patient stratification and counseling in the setting of congenital obstructive uropathy.

A healthy 9-month-old boy presented with a 1-month history of cough. A chest x-ray showed a linear metallic foreign body (FB) lying transversely in the posterior mediastinum. Computed tomographic scan confirmed the location and size of the metallic FB and also revealed a large pericardial effusion that was corroborated by echocardiogram. The patient underwent a right thoracotomy revealing a normal esophagus without mediastinitis and a 12-mm needle in the posterior mediastinum embedded in the pericardium with the sharp end abutting the left atrium. The needle was extracted uneventfully, and his postoperative course was unremarkable. The diagnosis and treatment for this case are discussed along with a review of the literature.

Segmental dilatation of the ileum (SDI), also termed ileal dysgenesis or segmental megaileum, is characterized by a sharply defined and markedly dilated segment of the ileum flanked by normal-caliber afferent and efferent bowel.1 Segmental dilatation of the ileum is a rare condition, with only 125 published cases, 1-16 It commonly presents during the neonatal period or childhood with an abdominal mass and symptoms including abdominal pain, bowel obstruction, anemia, and gastrointestinal bleeding. Segmental dilatation of the ileum is definitively treated by surgical resection. We report a case of SDI in a male neonate whose prenatal and postnatal imaging revealed a cystic abdominal mass. To our knowledge, the prenatal appearance of SDI has not been reported previously.

Objective: The uninterrupted passage of amniotic fluid through the gastrointestinal tract is hypothesized to influence both intestinal and overall fetal somatic development. The effect of in utero esophageal ligation (EL) and therefore the exclusion of AF on somatic growth, small intestinal (SI) morphology and proliferation, and the expression of the glucose transporter sodium-glucose cotransporter 1 (SGLT-1) in both normal and intrauterine growth-retarded (IUGR) fetal rabbits were evaluated. Methods: Thirteen pregnant New Zealand white rabbits underwent surgery on day 24 of their normal 31-day gestation. Ipsilateral normal and IUGR fetuses underwent EL; the contralateral normal and IUGR fetuses underwent cervical exploration only forming 4 study groups (control-normal, control-IUGR, EL-normal and EL-IUGR). Rabbits were killed on day 31. Small intestinal villus height was measured, and epithelial cell proliferation was deter mined by proliferating cell nuclear antigen staining. Sodium-glucose cotransporter 1 messenger RNA (mRNA) and protein expressions were analyzed. Statistical analysis was performed using 2-way analysis of variance. Results: Esophageal ligation reduced fetal weight in IUGR by 15% and in normal by 10%. Villus height was significantly reduced in IUGR versus normal in both control and EL (control, P = 0.01; EL, P = 0.05). Intrauterine growth-retarded fetuses had reduced SI proliferation versus normal in both control and EL. Sodium-glucose cotransporter 1 mRNA production in EL fetuses was equal to control fetuses. Esophageal ligation-normal and EL-IUGR fetuses exhibited reduced protein levels and decreased staining for SGLT-1 in villus enterocytes. Conclusions: Amniotic fluid exclusion by in utero EL reduced fetal weight. Small intestinal proliferation was not affected by EL. Although SGLT-1 mRNA and protein were produced in all 4 groups, exposure of the fetal gastrointestinal tract to amniotic fluid appears necessary for proper brush border expression of nutrient transporter proteins.

Aim: The purpose of the present study was to determine the potential maturational effects of maternal dexamethasone administration on fetal upper gastrointestinal motility. Methods: Nineteen pregnant rabbits were randomized into two groups (DEX and CONT) and studied on either day 24, 27 or 30 of pregnancy (term 31 days). The DEX group received intramuscular dexamethasone (DEX) 2 days before study and the controls received saline. Under ultrasound guidance, a needle was percutaneously inserted into each fetal stomach and fluorescein, labeled with color-coded microspheres, was injected. Two hours later, the length of fluorescein travel throughout the small intestine was measured. The percent motility was calculated by dividing the length of fluorescein travel by the total fetal small intestinal length × 100%. Results: All maternal and fetal rabbits survived. On day 24 the length of fluorescein travel and the percent motility of the DEX group were significantly longer than controls. There were no differences on either day 27 or 30. Fetal growth was significantly suppressed by a single course of DEX at all gestational ages. Conclusions: Fetal rabbit gastrointestinal motility is significantly increased after prenatal steroids are given early in the last trimester (day 24 of a 31-day gestation). Although beneficial in end-organ maturation, the timing of prenatal steroid administration is crucial to minimize its potential detrimental effects on fetal somatic growth.

Intrauterine growth retardation (IUGR) affects almost 10% of infants born in the United States. It may be responsible for delayed gastrointestinal function and is an important cause of perinatal morbidity and mortality. The New Zealand White rabbit provides an optimal model for the study of naturally occurring IUGR. At term, birth weight is determined by fetal position within the bicornuate uterus. The small intestinal disaccharidase enzymes are indicators of bowel maturity and function. To examine potential differences in disaccharidase development between normal and IUGR fetuses, this rabbit model was investigated. Jejunum was harvested at multiple stages in rabbit development including the third trimester fetus, neonate, and adult. Lactase, maltase, and sucrase enzyme activity, as well as total protein content, was determined. Results were analyzed by the 2-tailed t test and ANOVA. Lactase activity appeared in the mid-third trimester, peaked in the early neonatal period, then declined to adult levels. Maltase activity appeared in the early third trimester and gradually rose to adult levels. Sucrase remained at trace levels until the mid-neonatal period, reaching adult levels by weaning. Both lactase and maltase activity were depressed in IUGR fetuses compared with their normal littermates. This pattern of disaccharidase depression continued into the neonatal period until catch-up growth occurred at 2 wk when levels equalized. This report describes differential small intestinal disaccharidase development between normal and growth-retarded rabbit fetuses in a naturally occurring model of IUGR.

During fetal hypoxic stress, blood flow is shunted from nonvital to life-preserving organs, including the heart and brain. Reduced oxygen to the small intestine (SI) induces mucosal injury and may contribute to neonatal necrotizing enterocolitis (NEC). As little is known about the relationship between fetal hypoxia and GI motility, we assessed potential effects in a rabbit model. Twenty-one pregnant rabbits were randomized into two groups, hypoxia (Hyp) and control (Cont). Seven litters were studied at Gestational Days 24, 27, and 30 of their normal 31-day gestation. Under ultrasound guidance each fetal stomach was percutaneously accessed. Fluorescein, labeled with color-coded microspheres for precise fetal identification, was injected. Hyp rabbits breathed 11% oxygen for 1 h after recovery from anesthesia; Cont rabbits breathed room air. Two hours after injection, fetuses were delivered and weighed. The SI was harvested, the length recorded, and the distance fluorescein traveled measured by UV light optical density. Results were analyzed by the unpaired Student test. All injected fetuses (N = 167) survived. The length fluorescein traveled was shorter in Hyp than Cont at all gestational days studied (P < 0.01): Day 24, Hyp = 6.7 +/- 2.0 vs Cont = 8.4 +/- 2.1 cm; Day 27, Hyp = 10.1 +/- 2.9 vs Cont = 19.1 +/- 4.4 cm; and Day 30, Hyp = 16.8 +/- 3.5 vs Cont = 23.1 +/- 5.2 cm. The percentage motility, defined as the length of fluorescein travel divided by total SI length, was also significantly less at all gestational days. Fetal rabbit GI motility was significantly decreased by maternal hypoxia during the last third of gestation. Hypoxia-induced reduction in GI motility may contribute to neonatal NEC.