Congenital Diaphragmatic Hernia Research Articles

Sex-specific differences in the severity of pulmonary hypoplasia in experimental congenital diaphragmatic hernia and implications for extracellular vesicle-based therapy.

Amniotic fluid stem cell extracellular vesicles (AFSC-EVs) hold regenerative potential to treat hypoplastic lungs secondary to congenital diaphragmatic hernia (CDH). This study aims to investigate sex-specific differences in pulmonary hypoplasia severity and responses to AFSC-EV administration in an experimental CDH mouse model. C57BL/6J dams were fed with nitrofen + bisdiamine (left-sided CDH) or olive oil only (control) at embryonic day (E) 8.5. Lungs were dissected (E18.5), grown ex vivo and treated with medium ± AFSC-EVs that were collected via ultracentrifugation and characterized (nanoparticle tracking analysis, electron microscopy, Western blotting). Pulmonary hypoplasia was assessed via mean linear intercept (MLI). Gene and protein expression changes (Cd31, Enos, Il1b, TNFa) were measured via RT-qPCR and immunofluorescence. Pups were genotyped for Sry. Experimental CDH showed a male predominance without sex differences for pulmonary hypoplasia severity, fetal lung vascularization, and inflammation. AFSC-EV administration led to improved lung growth (decreased MLI), improved fetal lung vascularization (increased Cd31 and Enos), and decreased fetal lung inflammation (Il1b, TNFa). There was no sex-specific response to AFSC-EV administration. This study shows sex-independent impaired lung growth, vascularization and fetal lung inflammation in a CDH mouse model. Antenatal administration of AFSC-EVs reverses aspects of pulmonary hypoplasia secondary to CDH independent of the biological sex.

Congenital partial diaphragmatic eventration presenting with Chilaiditi’s sign: a case report

BackgroundChilaiditi’s sign is an incidental radiographic finding, associated with intestinal disposition located between liver and right diaphragm. It is considered as an acquired rather than a congenital condition and the prevalence ranges from 1.18% to 2.4% according to recent adult retrospective studies. The aspects of this rare entity with regards to a 7-month-old male initially misdiagnosed as diaphragmatic hernia is discussed.Case presentationA 4-month-old Caucasian male was misdiagnosed with a congenital diaphragmatic hernia owing to previous hospitalization with complaints of respiratory tract infection. On admission 3 months later, he was free of any signs and symptoms of intestinal obstruction or respiratory distress. Thorax computed tomography revealed Chilaiditi’s sign. A diagnostic laparoscopy was regarded necessary to evaluate the anatomical details. The most prominent finding was the lack of muscle fibers and almost transparent appearance of the medial aspect of the partially eventrated right hemidiaphragm. Owing to delicate anatomical presentation, diaphragmatic plication was considered hazardous. The patient is doing well and under follow-up.ConclusionsIt is obvious that Chilaiditi’s sign is not always a completely incidental finding of no consequence, and may indicate an underlying congenital diaphragmatic pathology, clearly defined by laparoscopic evaluation in this case.

Robot-assisted congenital diaphragmatic hernia repair in adults: A case series.

Congenital diaphragmatic hernia (CDH) is a rare condition predominantly affecting neonates, with only a few cases remaining undetected until adulthood. Surgical repair is the primary treatment approach for adults with confirmed CDH. Traditionally, these procedures include laparotomy, thoracotomy, and minimally invasive techniques such as thoracoscopy and laparoscopy. However, only a few cases of robotic diaphragmatic hernia repair have been reported in recent decades. The patients, aged 31 and 71 years, presented with atypical symptoms of chest tightness and fever. Imaging studies revealed a left-sided Bochdalek CDH in 1 patient and a right-sided Morgagni CDH in the other. The patients were diagnosed as CDH in adult with different symptoms. Both patients received robot-assisted diaphragmatic hernia repair at our institution. The patients received robotic-assisted diaphragmatic hernia repair with acceptable surgery outcome and safety. There was no complication or recurrence. This case series indicates that the robotic transabdominal approach for CDH repair in adults can be an optimal minimally invasive approach for selected patients, demonstrating adequate surgical safety and favorable outcomes.

Predicting neonatal mortality prior to discharge from hospital in prenatally diagnosed left congenital diaphragmatic hernia.

To evaluate the association of standardized prenatal imaging parameters and immediate neonatal variables with mortality prior to discharge in infants with isolated left congenital diaphragmatic hernia (LCDH), and to compare the performance of ultrasound- and magnetic resonance imaging (MRI)-based severity grading for the prediction of neonatal mortality. This was a retrospective study of infants with prenatally diagnosed isolated LCDH referred to a single tertiary center between 2008 and 2020. Fetuses with right or bilateral congenital diaphragmatic hernia, additional major structural anomaly or known genetic condition, as well as cases that underwent fetal intervention or declined postnatal intervention, were excluded. Ultrasound and MRI images were reviewed retrospectively. Univariable and multivariable analyses were performed, incorporating prenatal and immediate neonatal factors to analyze the association with neonatal mortality prior to discharge, and a prediction calculator was generated. The performance of ultrasound and that of MRI for the prediction of neonatal mortality were compared. Of 253 pregnancies with fetal CDH, 104 met the inclusion criteria, of whom 77 (74%) neonates survived to discharge. Seventy-five fetuses underwent both prenatal ultrasound and MRI. On multivariable analysis, observed/expected (o/e) lung-to-head ratio and o/e total fetal lung volume were associated independently with neonatal death (adjusted odds ratio, 0.89 (95% CI, 0.83-0.95) and 0.90 (95% CI, 0.84-0.97), respectively), whereas liver position was not. There was no significant difference in predictive performance between using ultrasound and MRI together (area under the receiver-operating-characteristics curve (AUC), 0.85 (95% CI, 0.76-0.93)) compared with using ultrasound alone (AUC, 0.81 (95% CI, 0.72-0.90); P = 0.19). The addition of neonatal parameters (gestational age at birth and small-for-gestational age) did not improve model performance (AUC, 0.87 (95% CI, 0.80-0.95)) compared with the combined ultrasound and MRI model (P = 0.22). There was poor agreement between severity assessment on ultrasound and MRI (Cohen's κ, 0.19). Most discrepancies were seen among cases deemed to be non-severe on ultrasound and severe on MRI, and outcomes were more consistent with MRI-based prognostication. In fetuses with prenatally diagnosed isolated LCDH, mortality prediction using standardized ultrasound and MRI measurements performed reasonably well. In cases classified as non-severe on ultrasound, MRI is recommended, as it may provide more accurate prognostication and assist in the determination of candidacy for fetal intervention. © 2024 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Toll-like receptors ligand immunomodulators for the treatment congenital diaphragmatic hernia

BackgroundCongenital diaphragmatic hernia (CDH) is a rare disease that affects the development of the diaphragm, leading to abnormal lung development. Unfortunately, there is no established therapy for CDH. Retinoic acid pathways are implicated in the ethology of CDH and macrophages are known to play a role in repairing organ damage.MethodsWe have analyzed the effect of several Toll like receptor (TLR) ligands in the nitrofen-induced CDH model in pregnant rats widely used to study this disease and in the G2-GATA4Cre;Wt1fl/fl CDH genetic mice model. Morphometric and histological studies were carried out. Immune cell infiltration was assayed by immunochemistry and immunofluorescence and retinoic pathway gene expression analyzed in vivo and in vitro in macrophages.ResultsWe found that administering a single dose of atypical TLR2/4 ligands (CS1 or CS2), 3 days after nitrofen, cured diaphragmatic hernia in 73% of the fetuses and repaired the lesion with complete diaphragm closure being on the other hand nontoxic for the mothers or pups. Moreover, these immunomodulators also improved pulmonary hypoplasia and alveolar maturation and vessel hypertrophy, enhancing pulmonary maturity of fetuses. We also found that CS1 treatment rescued the CDH phenotype in the G2-GATA4Cre;Wt1fl/fl CDH genetic mice model. Only 1 out of 11 mutant embryos showed CDH after CS1 administration, whereas CDH prevalence was 70% in untreated mutant embryos. Mechanistically, CS1 stimulated the infiltration of repairing M2 macrophages (CD206+ and Arg1+) into the damaged diaphragm and reduced T cell infiltration. Additionally, those TLR ligands induced retinol pathway genes, including RBP1, RALDH2, RARα, and RARβ, in the affected lungs and the diaphragm and in macrophages in vitro.ConclusionsOur research has shown that TLR ligand immunomodulators that influence anti-inflammatory macrophage activation can be effective in treating CDH, being nontoxic for the mothers or pups suggesting that those TLR ligands are a promising solution for CDH leading to orphan drug designation for CS1. The immune system of the fetus would be responsible for repairing the damage and closure of the hernia in the diaphragm and enhanced proper lung development after CS1 treatment.

Could a Two-Staged Repair Be the Solution to the Dilemma of Repair Timing for Severe Congenital Diaphragmatic Hernia Requiring Extracorporeal Membrane Oxygenation?

Congenital diaphragmatic hernia (CDH) remains a significant challenge, particularly in severe cases with persistent pulmonary hypertension (PPHN) and hypoplastic lungs and heart. For patients unresponsive to conventional therapies, ECMO is required. While the surgical repair is relatively simple, determining the optimal timing for surgery in patients requiring ECMO is particularly challenging. This review explores the dilemma of surgical timing and proposes a two-staged approach: a reduction in herniated organs and the creation of a silo to relieve abdominal pressure before initiating ECMO, with defect closure following ECMO decannulation. Studies support pre-, on-, and post-ECMO repair, each with its own risks and benefits. Pre-ECMO repair may enhance ECMO efficacy by relieving organ compression but poses risks due to instability. Post-ECMO repair is safer but may result in losing the chance to repair. On-ECMO repair has significant hemorrhage risks, but early repair with careful anticoagulation management is currently recommended. Recently, the author reported a successful case using a two-staged approach-reducing herniated organs and creating a silo before ECMO, followed by defect closure after ECMO decannulation-which suggests a potential alternative strategy for managing severe CDH. A two-staged approach may offer a solution for severe CDH patients requiring ECMO.

Utility of urinary NT-proBNP in congenital diaphragmatic hernia: a prospective pilot study.

Echocardiography is the reference standard for diagnosing pulmonary hypertension (PH) and cardiac dysfunction (CD) in congenital diaphragmatic hernia (CDH). The use of an adjunct non-invasive biomarker would be invaluable. Plasma N-terminal brain Natriuretic Peptide (NT-proBNP) has been evaluated as a biomarker in CDH. Our objective was to investigate the utility of urinary NT-proBNP as an adjunct biomarker in CDH. Prospective observational study of neonates with CDH. Urinary NT-proBNP and its ratio to urinary creatinine (UNBCR) were analyzed at 24 h of life (HOL). Spearman's correlation was used to quantify the association between the urinary NT-proBNP with corresponding plasma concentrations. Logistic regression and receiver operating characteristic (ROC) curve analyses were used to examine urinary NT-proBNP and UNBCR's association with need for ECMO. The Kruskal-Wallis test was used to compare urinary NT-proBNP concentrations with echocardiography parameters of PH and CD. Urinary NT-proBNP and UNBCR positively correlated with plasma concentrations. Patients with high right ventricular (RV) pressures had higher urinary NT-proBNP concentrations. Both urinary NT-proBNP and UNBCR concentrations increased with worsening left and right ventricular dysfunction on first postnatal echocardiogram. We report the feasibility of urinary NT-proBNP as a non-invasive biomarker to assess PH and CD in CDH. Non-invasive biomarkers are an important area of research in neonates as they have the potential to decrease the need for blood sampling, which may cause pain, stress, and induce iatrogenic anemia. Use of non-invasive biomarkers could be especially useful in congenital diaphragmatic hernia (CDH) who may need Extra-Corporeal Life Support (ECLS) for their management. We evaluated the potential utility of the urinary NT-proBNP in CDH. Plasma NT-proBNP has been studied in neonates with CDH; however, urinary NT-proBNP has not been explored, making this the first study to examine its potential as a biomarker.

Congenital Diaphragmatic Hernia With Poor Clinical Outcome: Key Lessons To Be Learned

Congenital Diaphragmatic Hernia With Poor Clinical Outcome: Key Lessons To Be Learned

Burden and mortality of congenital gastrointestinal anomalies: insights from a nationwide cohort study.

Improved perioperative care has enhanced survival in children with congenital gastrointestinal conditions and abdominal wall defects (AWD). However, epidemiological and surgical outcomes in developing nations are still scarce. Our aim was to assess the burden and mortality of common congenital gastrointestinal anomalies and AWD in Malaysia, and their influencing factors. Using the Global PaedSurg study protocol with permission, we performed a prospective cohort study on children presenting for the first time between October 2021 and April 2022 with these conditions: Esophageal atresia (EA), congenital diaphragmatic hernia (CDH), intestinal atresia, gastroschisis, exomphalos, anorectal malformation (ARM) and Hirschsprung's disease. We compared mortality and 30-day outcome data across different geographical regions in Malaysia. There were 228 patients with 242 study conditions (EA n = 28, CDH n = 36, intestinal atresia n = 49, gastroschisis n = 12, exomphalos n = 8, ARM n = 77, Hirschsprung's disease n = 32). Our mortality rate was 8.8%; 60% of these were CDH patients. Factors significantly associated with mortality were CDH diagnosis, central venous access requirement, higher American Society of Anesthesiologists (ASA) score, blood transfusion and ventilation requirement. Diagnosis of CDH is the most important predictor for sepsis on arrival and mortality, therefore measures should be taken for early recognition and aggressive management.

Ventricular Dysfunction in Patients With Congenital Diaphragmatic Hernia Who Die After Repair

BackgroundQuantitative echocardiographic (echo) measures of ventricular function predict mortality in pediatric pulmonary hypertension (PH), but studies in congenital diaphragmatic hernia (CDH)-related PH are limited. Few studies report quantitative echo data beyond the first week of life in CDH non-survivors. MethodsA single-center retrospective, cross-sectional, cohort study included CDH patients born between January 2013 and April 2022 who survived to surgical repair but died during the neonatal hospitalization. Quantitative measures of right (RV) and left ventricular (LV) size and function including tricuspid annular plane systolic excursion Z-score (TAPSEZ), RV fractional area change (FAC), RV/LV ratio, LV eccentricity index, LV M-mode dimensions, and RV/LV systolic strain were performed offline on the last echocardiogram before death. Data were compared between patients who died ≤30 days after repair (“early”) vs. >30 days after repair (“late”) using the Wilcoxon rank sum test. ResultsTwenty-five (11 early, 14 late) deceased patients had echo images available for analysis. LV size by end-diastolic dimension Z-score was smaller in patients who died early vs. late after repair [-3.03 (−3.93, −2.51) vs. −0.24 (−2.11, 0.53), p = 0.021]. There were trends toward worse RV function (TAPSEZ, RVFAC, RV global and free wall strain) and LV function (apical 4 chamber strain) in patients who died early vs. late after repair. ConclusionThese preliminary findings support future study of the impact of ventricular hypoplasia and dysfunction on mortality and opportunities for risk stratification based on quantitative echo findings in CDH. Level of EvidenceCohort study, 4.

Clinical features and genetic analysis of child with Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 6 due to variant of DNA2 gene

To explore the genetic etiology for a child with Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 6 (PEOA6). A child who had attended the Women and Children's Hospital Affiliated to Ningbo University on 7 August, 2023 was selected as the study subject. Clinical data of the child were analyzed retrospectively. The child and her parents were subjected to whole exome sequencing (WES), and candidate variant was verified by Sanger sequencing and bioinformatic analysis. This study was approved by the Women and Children's Hospital Affiliated to Ningbo University (Ethics No. EC2020-048). The child, a 7-year-old female, had presented with limb muscle pain, amyosthenia, significantly increased creatine kinase, congenital diaphragmatic hernia and recurrent respiratory tract infections. WES revealed that the she has harbored a heterozygous c.1590G>C (p.L530F) variant of the DNA2 gene, which was verified to have a de novo origin by Sanger sequencing. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c.1590G>C was rated as a likely pathogenic variant (PS2+PM2_Supporting+PP3). The c.1590G>C (p.L530F) variant of the DNA2 gene probably underlay the PEOA6 in this child.

Use of Low-Molecular-Weight Heparin for Right Atrial Thrombus Following Extracorporeal Membrane Oxygenation in Neonates after Surgical Repair of Congenital Diaphragmatic Hernia: A Case Report and Review of the Literature

Some critically ill neonates with congenital diaphragmatic hernia (CDH) require extracorporeal membrane oxygenation (ECMO) during the perioperative period. Neonates on ECMO face a significantly increased risk of thrombotic events. Thrombosis management varies across centers and may include anticoagulation, thrombolysis, or thrombectomy. We present our experience using low molecular weight heparin (LMWH) to treat a right atrial thrombus (RAT) following ECMO in a neonate with CDH.

Utility of the oxygenation index in management of congenital diaphragmatic hernia: a report from a Thai University Surgical Centre

BackgroundOxygenation index (OI) is associated with severity of newborn pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH). Higher OI may indicate worst degree(s) of PH.ObjectivesThis study reports OI dynamic(s) over the first 72 h of life and its correlation with (1) perioperative morbidity and (2) CDH mortality.MethodsMedical records of inborn CDH babies during 2002–2022 were examined. OI on Days (s) 1–3 and perioperative OI trends were recorded. Operation (primary vs patch repair) and survival rates (%) were studied.ResultsFifty-five CDH newborns (54.5% male: 45.5% female)—mean birth GA 37.5 ± 2.7 wks. had a mean birth weight 2813 ± 684 g with prenatal diagnosis in 32.7% cases. 52/55 (94.5%) were intubated at birth and HFOV deployed in 29 (55.8%). Those requiring HFOV had higher OI on DOL1 (24.8 ± 17 vs 10.3 ± 11.5; p < 0.05), DOL 2 (26.3 ± 22.9 vs 6.7 ± 12.1; p < 0.05) and DOL 3 (21.9 ± 33.8 vs 5.5 ± 9.3; p = 0.04). Operation was undertaken in 36/55 (65.5%). Preoperative mortality group had significant higher OI on DOL 2 (42.1 ± 21.0 vs 14.9 ± 9.3; p = 0.04). CDH defects were—Type A N = 27 (75%), Type B N = 7 (19.4%) and Type C N = 2 (5.6%). Overall mortality was 40% (22/55). Statistically significant OI trends were recorded in non-survival vs. survival groups on DOL 1 (31.6 ± 16.8. vs 10.5 ± 9.0; p < 0.05, DOL 2 (38.1 ± 21.9 vs 6.3 ± 7.1; p < 0.05), and DOL 3 (38.8 ± 39.4; p = 0.012).ConclusionsOI dynamics are highly predictive for accurate monitoring of CDH cardiorespiratory physiology and crucially may guide ventilatory management as well as timing of surgery.

Clinical challenges and management of late presenting congenital diaphragmatic hernia mimicking tension pneumothorax in a child: a case report and review of literatures

BackgroundCongenital diaphragmatic hernia(CDH) is a rare congenital anomaly characterized by herniation of abdominal contents into thoracic cavity through a defect in diaphragm. While commonly diagnosed prenatally or in neonatal period, late-presenting CDH can occur and may mimic other thoracic emergencies such as tension pneumothorax, complicating diagnosis and management.Case presentationA two-year old male black child from Ethiopia presented to the emergency department with sudden onset of acute respiratory distress. Initial clinical assessment and chest radiography suggested a diagnosis of tension pneumothorax due to the presence of significant mediastinal shift and apparent pleural air. Despite insertion of chest tube, the child’s condition did not improve, raising suspicion of alternative diagnosis. Careful observation of initial chest x-ray and subsequent chest ultrasound revealed a left sided congenital diaphragmatic hernia with herniation of stomach and intestine into thoracic cavity compressing the left lung and causing mediastinal shift. After the diagnosis of CDH was confirmed, the child was stabilized and emergent surgical repair performed. Postoperative recovery was uneventful, and the child was discharged with no significant long-term complications.ConclusionThis case underscores the importance of considering CDH in the differential diagnosis of acute respiratory distress in a child. It highlights the diagnostic challenges and potential risks of emergency interventions based on initial misdiagnosis. Even if x -ray looks like typical of tension pneumothorax, it showed giant cystic air filled structure pushing the mediastinal structure to contralateral side with loss of left diaphragmatic outline which raised suspicion of congenital cystic lung mass or congenital diaphragmatic hernia. Advanced imaging and high index of suspicion are crucial for accurate diagnosis and timely management, ultimately improving patient outcomes. Consideration of alternative diagnosis when our initial intervention with insertion of chest tube fail to provide symptom improvement in suspected pneumothorax should raise suspicion of congenital diaphragmatic hernia like in our case.

Congenital diaphragmatic hernia and cleft lip and palate: looking for a common genetic etiology.

Congenital diaphragmatic hernia (CDH) and cleft lip and/or palate (CL/P) are inborn closure defects. Genetic factors in and outcomes for patients with both anomalies (CDH+CL/P) remain unclear. We aimed to investigate associated genetic aberrations, prevalence of, and outcomes for, CDH+CL/P. Data from Congenital Diaphragmatic Hernia Study Group (CDHSG) registry were collected. CL/P prevalence in CDH patients was determined. Genetic abnormalities and additional malformations in CDH+CL/P were explored. Patient characteristics and outcomes were compared between CDH+CL/P and isolated CDH (CDH-) using Fisher's Exact Test for categorical, and t-test or Mann-Whitney U-test for continuous, data. p < 0.05 was considered statistically significant. Genetic anomalies in CDH+CL/P included trisomy 13, 8p23.1 deletion, and Wolf-Hirschhorn syndrome (4p16.3 deletion). CL/P prevalence in CDH was 0.7%. CDH+CL/P had lower survival rates than CDH-, a nearly fourfold risk of death within 7days, were less supported with extracorporeal life support (ECLS), had higher non-repair rates, and survivors had longer length of hospital stay. Genetic anomalies, e.g. trisomy 13, 8p23.1 deletion, and Wolf-Hirschhorn syndrome, are seen in patients with the combination of CDH and orofacial clefts. CL/P in CDH patients is rare and associated with poorer outcomes compared to CDH-, influenced by goals of care decision-making.

Seguridad y eficacia de un programa de ejercicio terapéutico en jóvenes intervenidos de hernia diafragmática congénita

Introduction and objectivePatients with congenital diaphragmatic hernia (CDH) can have up to 40 times more frequency of muskuloskeletal deformities and decreased perception of physical activity tan their pairs. The objective of this study is to evaluate the safety and efficacy of an individualized exercise program in late adolescents and young adults with repaired CDH, as well as a description of their basal status. Material and methodsNon randomized prospective trial of 13 patients with repaired CDH between 1997-2005. An initial physical exploration and a pre-post assessment of bioimpedance (BIA), dynamometry, maximal inspiratory and expiratory pressure (MIP/MEP), 6-minute walk test (6MWT), physical activity level (IPAQ) and quality of life (QoL) was made. The training program last for 4 weeks. For the statistical analysis, the Student's t test for paired samples and Wilcoxon test were used. Results77% (n=10) were male with a mean age of 19.23±2.13 years. In baseline BIA, 62% (n=8) had truncal sarcopenia that improved in −0.43±0.58, and P=.016. MIP, MEP, 6MWT and QoL tests increased by −7.27±8.26 cmH2O, P=.008; −11.91±10.20 cmH2O, P=.002; −70.63±17.88 m, P=.001; −42,19±26.79, P=.00 respectively. The IPAQ did not change significantly (P=0.86), however the time dedicated to muscle strengthening increased. No adverse effects were reported. ConclusionsA personalized rehabilitation program is safe and could improve the respiratory muscle strength and truncal sarcopenia as well as the submaximal effort capacity in late adolescents and young adults with repaired CDH.

Diagnostic confusion caused by tension gastrothorax – Case series

Abstract The presence of the stomach in the thorax is called gastrothorax. If excessive dilatation occurs due to a valve mechanism in the stomach which then is herniated into the thorax, it is called tension gastrothorax and the situation can be life-threatening. In late presentation, congenital diaphragmatic hernias or traumatic diaphragmatic hernias, if the stomach located in the thorax is severely distended, it can be confused with tension pneumothorax or pleural effusion/empyema. We presented tension gastrothorax in two patients with distended stomach in the thorax, in which tension pneumothorax and pleural effusion/empyema-like images were created, based on plain X-rays taken at the first admission in the emergency department, which may cause diagnostic confusion. The tension gastrothorax can cause mediastinal shift, which can lead to respiratory distress and eventually cardiac arrest secondary to obstructive shock. In case of mediastinal shift, it should reduce the tension of the stomach located in the thorax by inserting a nasogastric catheter, and if this fails, by aspiration with a needle.

Sildenafil as Bridge Therapy for Inhaled Nitric Oxide in Preterm Neonates

OBJECTIVE Inhaled nitric oxide (iNO) is a mainstay of treatment for infants with persistent pulmonary ­hypertension. However, abrupt discontinuation of inhaled nitric oxide can result in rebound pulmonary ­hypertension. The objective of this analysis is to describe the use of sildenafil to facilitate the weaning from iNO in preterm neonates. METHODS This retrospective chart review identified all infants who were receiving iNO and subsequently received sildenafil between 2017 and 2021. Neonates were included if they met the following criteria: gestational age at birth less than 34 weeks, receiving iNO, and started on sildenafil with the indication to facilitate weaning or discontinuation of iNO. Patients were excluded if they had major congenital anomalies, including congenital heart disease or congenital diaphragmatic hernia. RESULTS We identified 7 neonates with a gestational age range of 22 5/7 weeks to 31 0/7 weeks and birth weight range of 545 to 910 g with previously failed attempts at iNO weaning. The most common starting dose for sildenafil was 0.125 mg/kg intravenously every 8 hours or 0.25 mg/kg enterally every 8 hours. Four infants were able to discontinue iNO within 48 hours of sildenafil initiation, 1 patient discontinued iNO within 5 days, and 1 patient within 10 days of sildenafil initiation. One patient experienced weaning failure from iNO despite initiation of sildenafil. No adverse events, such as hypotension or deaths, were reported in any of the 7 infants. CONCLUSIONS Sildenafil facilitated weaning off iNO in most preterm neonates evaluated without adverse side effects.

Cutting into the NICU: Improvements in Outcomes for Neonates with Surgical Conditions.

The Children's Hospitals Neonatal Consortium (CHNC), established in 2010, seeks to improve care for infants with medically and surgically complex conditions who are cared for in level IV regional children's hospital NICUs across North America. Through patient-level individual data collection, comparative benchmarking, and multicenter quality improvement work, CHNC has contributed to knowledge and improved outcomes, leveraging novel collaborations between and across institutions. Focusing on antenatal and inpatient care for infants with surgical conditions including congenital diaphragmatic hernia, gastroschisis, and necrotizing enterocolitis, we summarize the progress made in these infants' care. We highlight the ways in which CHNC has enabled multidisciplinary and multicenter collaborations through the facilitation of diagnosis-specific focus groups, which enable comparative observations of outcomes through quality improvement and research initiatives. Finally, we review the importance of postbirth hospitalization needs of these infants and the application of telemedicine in this population.

Oral feeding practices in medically complex infants receiving prolonged high-flow nasal cannula support: A retrospective cohort study.

To characterise the feeding profile and care pathway for infants receiving prolonged high-flow nasal cannula (HFNC) respiratory support for management of a chronic condition at one facility from January to December 2021. Data regarding medical history, HFNC admission details (reason for HFNC, HFNC duration, flow rate), feeding outcomes and speech pathology care were collected from electronic records of HFNC-dependent infants (requiring HFNC ≥2-3 L/kg for ≥5 consecutive days). Infants with acute respiratory conditions (e.g. bronchiolitis) were excluded. This study included 24 participants (median corrected age at admission 5.3 weeks, range -6 to 18.6). Of these, 15 (60%) had a condition/s that affected more than one body system (e.g. congenital diaphragmatic hernia), requiring the care of multiple specialities. Median length of HFNC use was 37.5 days (range 11-188). Twenty (83.3%) infants were referred for speech pathology (SLP) input while on HFNC support. For those referred, frequency of SLP input was variable (0-3 sessions/week), and HFNC support requirements were the most common barrier to SLP intervention (n = 9, 45%). Twelve (54.5%) infants demonstrated improvement in their primary feeding method by discharge; however, only two (9.1%) infants were discharged on full oral feeds. This study demonstrates variability in oral feeding management in infants with prolonged HFNC-dependence at our centre. Respiratory support with HFNC was identified as a barrier to progressing oral feeding. Further research is required to determine if oral feeding can be safely undertaken in this cohort. This is imperative to ensure that long-term feeding outcomes are not negatively impacted by current practice.