A novel approach for repair of right sided congenital diaphragmatic hernia and hepatopulmonary fusion.

Right sided congenital diaphragmatic hernia (CDH) associated with hepatopulmonary fusion (HPF) is a rare congenital anomaly in which the herniated liver is fused with lung parenchyma. We discuss the case of an infant with right-sided CDH and HPF found on index operation for repair of right-sided CDH. Due to the high incidence of vascular anomalies associated with HPF the decision was made to close the patient and get further imaging to characterize the HPF before returning to the operating room for definitive repair. We describe a novel and successful operative approach where the liver was left entirely fused to the lung and the liver was partially brought down from the chest and was plicated to the diaphragm form a seal between the parietal and pleural cavities to repair the CDH.

Diagnostic challenges in late-presentation congenital diaphragmatic hernia: A case study of a 10-month-old with respiratory symptoms.

Congenital diaphragmatic hernia (CDH) is a developmental disorder in which the diaphragm, the muscle that separates the chest from the abdomen, does not close during prenatal development, allowing abdominal organs to herniate into the chest cavity. It occurs mainly on the left side (80%-85% of cases). CDH is often identified during prenatal assessment. However, instances of late-presenting CDH beyond infancy are exceedingly uncommon, contributing to frequent misdiagnosis and delayed therapeutic intervention. We present a case of a 10-month-old female with an uneventful antenatal and perinatal history who presented with respiratory distress and multiple episodes of vomiting. Her vital signs were stable upon arrival, but she was sent to the PICU due to hypoactivity, reduced oral intake, and agitation. After an urgent CT scan, a herniation of the small and large bowel loops into the right hemithorax was discovered, along with a defect in the right hemidiaphragm. This resulted in a pleural effusion on the right side, a partially collapsed left lung, and a mediastinal shift to the left. The diaphragmatic hernia was corrected through a lateral thoracotomy at the sixth rib with multiple interrupted sutures, and a chest tube was then inserted into the pleural space above the diaphragm following a smooth reduction of the bowl. This case highlights the importance of early diagnosis, appropriate clinical investigation, and treatment. A good prognosis can be anticipated by promptly discovering and examining the condition.

Delayed presentation of congenital diaphragmatic hernia: A report of 2 cases.

Late presentation of congenital diaphragmatic hernia (CDH) presents usually after the neonatal period and often misdiagnosed for other respiratory pathologies. It is crucial to differentiate late presentation of CDH from other potential causes of respiratory distress and gastrointestinal symptoms. Herein, we present 2 cases of delayed presentation of congenital diaphragmatic hernia in infants. Initially, both cases were managed as respiratory conditions in outpatient settings, with no significant improvement. The correct diagnosis was eventually made through radiological evaluation at our tertiary centre, leading to successful surgical management. Delayed presentation of CDH beyond the neonatal period is rare, owing to the wide spectrum of clinical manifestations. Early diagnosis and surgical management are crucial to reduce morbidity and mortality, making a high index of suspicion essential for timely intervention.

Patterns and Outcomes of Epoprostenol Use in Infants with Congenital Diaphragmatic Hernia Requiring Extracorporeal Life Support.

OBJECTIVE: To describe our experience utilizing epoprostenol for pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS). STUDY DESIGN: We retrospectively reviewed infants diagnosed with CDH who required ECLS at our institution from 2013 to 2023. Data collected included demographics, disease characteristics, medication administration patterns, and hospital outcomes. We first compared infants who received intravenous epoprostenol and those who did not. Among infants who received epoprostenol, we compared survivors and nonsurvivors. χ² test/Fisher's exact and Mann-Whitney tests were used, with significance defined at P < .05. RESULTS: Fifty-seven infants were included; 40 (70.2%) received epoprostenol. Infants receiving epoprostenol had lower observed/expected total fetal lung volume (O/E TFLV) on magnetic resonance imaging (20 vs 26.2%, P = .042) as well as higher prenatal frequency of liver-up (90 vs 64.7%, P = .023) and "severe" classification (67.5 vs 35.3%, P = .007). Survival with and without epoprostenol was comparable (60% vs 64%, P = .23). Of those receiving epoprostenol, both survivors and nonsurvivors had similar prenatal indicators of disease severity. Most (80%) of hernia defects were classified as type C/D and 68% were repaired <72 hours after ECLS cannulation. The median age at initiation of epoprostenol was day of life 6 (IQR: 4, 7) in survivors and 8 (IQR: 7, 16) in nonsurvivors (P = .012). Survivors had shorter ECLS duration (11 vs 20 days, P = .049). Of nonsurvivors, refractory PH was the cause of death for 13 infants (81%). CONCLUSIONS: In infants with CDH requiring ECLS, addition of epoprostenol appears promising and earlier initiation may affect survival.

Case Report: A "senior" with serpentine-like syndrome-treatment of combined brachyoesophagus, intrathoracic stomach and cervical rachischisis.

Serpentine-like syndrome, characterized by the combination of intrathoracic stomach, a notably short esophagus anomaly, splenic abnormalities, and cervical spine malformations, has been associated with a high mortality rate since its identification in 2008. This report presents the case of a remarkable patient who recently celebrated her fifth birthday, marking her as the oldest documented individual with this syndrome to date. Highlighting the significance of comprehensive evaluations for concurrent malformations, the report discusses potential treatment modalities and challenges inherent in managing patients with this intricate syndrome. A comprehensive review of previously published cases is provided, comparing surgical interventions, causes of death, and age at the time of demise. This report underscores the importance of ongoing research and collaborative efforts to optimize outcomes for individuals afflicted with serpentine-like syndrome.

Optimizing Congenital Diaphragmatic Hernia Repair on ECMO: Evaluating the Risk of Bleeding.

BACKGROUND: Institutions lack consensus on the management of patients with congenital diaphragmatic hernia (CDH) who are repaired on extracorporeal membrane oxygenation (ECMO). Our study aimed to evaluate risk factors associated with bleeding complications in patients with CDH repaired on ECMO. METHODS: A single-institution retrospective review evaluated all patients with CDH who underwent on-ECMO repair between January 2005 and December 2023. A significant bleeding complication post-repair was defined as bleeding necessitating re-operation. The association between preoperative factors and bleeding complications was evaluated. RESULTS: Forty-six patients were included. Bleeding complications developed in 11/46 (24%) patients. Birthweight (2.5 vs. 3.2 kg, p = 0.02), platelet count <100/mm3 (64% vs. 29%, p = 0.04), elevated blood urea nitrogen (BUN; 24.5 vs. 17.5 mg/dL, p = 0.05), and older age at repair (8 vs. 5 days, p = 0.04) were associated with bleeding. In univariate analysis, patients with platelets under 100/mm3 were more likely to develop a bleeding complication (OR = 4.4, p = 0.04). Patients who experienced a significant bleeding event experienced increased ECMO days (12 vs. 7 days, p < 0.01), ventilator days (31 vs. 18 days, p < 0.05), and lower survival to discharge (36% vs. 74%, p = 0.03). CONCLUSION: Among CDH patients undergoing repair on ECMO, those with lower birth weight, platelet counts under 100/mm3, elevated BUN, and older age at repair had an increased risk of a significant bleeding complication, resulting in more ECMO and ventilator days and higher mortality. Patients undergoing on-ECMO repair should have platelet count transfused to greater than 100/mm3. Patients at high risk for bleeding may benefit from early repair on ECMO. LEVEL OF EVIDENCE: Level III.

Case Report: Thoracoscopic treatment of infradiaphragmatic pulmonary sequestration and intrathoracic kidney associated with congenital diaphragmatic hernia.

Background: Congenital pulmonary sequestration is a rare lung anomaly that can be classified as intralobar pulmonary sequestration or extralobar lung sequestration (ELS). Infradiaphragmatic pulmonary sequestration is a rare type of ELS. Furthermore, intrathoracic kidney (ITK) is a rare disease that can be associated with a congenital diaphragmatic hernia (CHD) in 0.25% of cases. We report the first case of infradiaphragmatic pulmonary sequestration and ITK associated with CDH in a child. Case report and management: The patient, male, aged 6 months, visited our hospital 2 months prior due to shortness of breath. Based on chest ultrasonography and enhanced computed tomography (CT) examination, infradiaphragmatic pulmonary sequestration and ITK were considered to be associated with CDH. The patient was admitted to our hospital for treatment. After admission, his blood pressure was 85/61 mmHg, there was no hematuria or proteinuria, creatinine was 14 µmol/L, and urea nitrogen was 2.96 mmol/L, all of which showed no abnormalities. A complete preoperative examination was performed prior to surgical treatment. Thoracoscopy revealed that the right kidney had herniated into the chest cavity on the posterolateral side of the diaphragm. The right kidney was returned to the abdominal cavity, the hernia sac was opened, and a bright red lesion tissue with clear boundaries and an abnormal blood vessel supply was observed. After cutting off the abnormal blood vessels, LigaSure TM was used to remove the diseased tissue, and the renal fat sacs and renal tissue were visible. Intermittent suturing of the hernia ring was performed to seal the diaphragmatic hernia. Postoperative pathological examination revealed infradiaphragmatic pulmonary sequestration. The postoperative recovery of the patient was smooth, and a chest CT scan at 2 months showed that the right kidney had returned to the abdominal cavity and the right diaphragm was in the normal position. Conclusion: Infradiaphragmatic pulmonary sequestration and ITK associated with CDH is extremely rare. A diagnosis and appropriate surgical planning can be developed using enhanced CT. For infradiaphragmatic pulmonary sequestration located at the top of the hernia sac in CHD, thoracoscopic resection of the infradiaphragmatic pulmonary sequestration and repair of the diaphragmatic hernia is feasible and effective.

Intraoperative surgical complications of open surgery for congenital diaphragmatic hernia: a multicenter, observational study in Japan.

PURPOSE: This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). METHODS: We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients were obtained from the database of the Japanese CDH Study Group. RESULTS: Among 1,111 neonates with left or right CDH, 852 underwent open surgery (laparotomy or thoracotomy). Of these 852 neonates, 51 had the following surgical complications: organ injury (n = 48; 6% of open surgeries); circulatory failure caused by changes in the organ location (n = 2); and skin burns (n = 1). Injured organs included the spleen (n = 30; 62% of organ injuries), liver (n = 7), lungs (n = 4), intestine (n = 4), adrenal gland (n = 2), and thoracic wall (n = 2). Fourteen of the patients who experienced organ injury required a blood transfusion (2% of open surgeries). The adjusted odds ratio of splenic injury for patients with non-direct closure of the diaphragm was 2.2 (95% confidence interval, 1.1-4.9). CONCLUSION: Of the patients who underwent open surgery for CDH, 2% experienced organ injury that required a blood transfusion. Non-direct closure of the diaphragmatic defect was a risk factor for splenic injury.

Observed-to-expected lung-area-to-head-circumference ratio on ultrasound examination vs total fetal lung volume on magnetic resonance imaging in prediction of survival in fetuses with left-sided diaphragmatic hernia.

OBJECTIVE: To assess and compare the value of antenatally determined observed-to-expected (O/E) lung-area-to-head-circumference ratio (LHR) on ultrasound examination vs O/E total fetal lung volume (TFLV) on magnetic resonance imaging (MRI) examination to predict postnatal survival of fetuses with isolated, expectantly managed left-sided congenital diaphragmatic hernia (CDH). METHODS: This was a multicenter retrospective study including all consecutive fetuses with isolated CDH that were managed expectantly in Mannheim, Germany, and in five other European centers, that underwent at least one ultrasound examination for measurement of O/E-LHR and one MRI scan for measurement of O/E-TFLV during pregnancy. All MRI data were centralized, and lung volumes were measured by two experienced operators blinded to the pre- and postnatal data. Multiple logistic regression analyses were performed to examine the effect on survival at hospital discharge of various perinatal variables, including the center of management. In left-sided CDH with intrathoracic herniation of the liver, receiver-operating-characteristics (ROC) curves were constructed separately for cases from Mannheim and the other five European centers and were used to compare O/E-TFLV and O/E-LHR in the prediction of postnatal survival. RESULTS: From Mannheim, 309 patients were included with a median gestational age (GA) at ultrasound examination of 29.6 (range, 19.7-39.1) weeks and median GA at MRI examination of 31.1 (range, 18.0-39.9) weeks. From the other five European centers, 116 patients were included with a median GA at ultrasound examination of 26.7 (range, 20.6-37.6) weeks and median GA at MRI examination of 27.7 (range, 21.3-37.9) weeks. Regression analysis demonstrated that the survival rates at discharge were lower in left-sided CDH (odds ratio (OR), 0.349 (95% CI, 0.133-0.918), P = 0.033) and those with intrathoracic liver (OR, 0.297 (95% CI, 0.141-0.628), P = 0.001), and higher with increasing O/E-TFLV (OR, 1.123 (95% CI, 1.079-1.170), P < 0.001), advanced GA at birth (OR, 1.294 (95% CI, 1.055-1.588), P = 0.013) and when birth occurred in Mannheim (OR, 7.560 (95% CI, 3.368-16.967), P < 0.001). Given the difference in survival rate between Mannheim and the five other European centers, ROC curve comparisons between the two imaging modalities were presented separately. For cases of left-sided CDH with intrathoracic herniation of the liver, pairwise comparison showed no significant difference between the area under the ROC curves for the prediction of postnatal survival between O/E-TFLV and O/E-LHR in Mannheim (mean difference = 0.025, P = 0.610, standard error = 0.050), whereas there was a significant difference in the other European centers studied (mean difference = 0.056, P = 0.033, standard error = 0.056). CONCLUSIONS: In fetuses with left-sided CDH and intrathoracic herniation of the liver, the predictive value for postnatal survival of O/E-TFLV on MRI examination and O/E-LHR on ultrasound examination was similar in one center (Mannheim), but O/E-TFLV had better predictive value compared to O/E-LHR in the five other European centers. Hence, in these five European centers, MRI should be included in the diagnostic process for left-sided CDH. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Comparative Transcriptome Analysis of Human and Mouse Canalicular Lungs in Fetal Diaphragmatic Hernia.

BACKGROUND: The nitrofen model of congenital diaphragmatic hernia (CDH) is widely used in translational research. However, the molecular pathways associated with pulmonary hypoplasia in this model compared to the human CDH phenotype have not been well described. The aim of this study was to investigate differentially expressed genes (DEG) and signaling pathways in early stage fetal lungs in mouse and human CDH. METHODS: CDH lung tissue was obtained from human fetuses (21-23 weeks gestation) and nitrofen mouse pups (E15.5). NovaSeq Flowcell RNA-seq was performed to evaluate differentially expressed transcriptional and molecular pathways (DEGs) in fetal mice with CDH, compared with age-matched normal mouse lungs and human CDH samples. RESULTS: There were thirteen overlapping DEGs in human and mouse CDH lung samples compared to controls. These genes were involved in extracellular matrix, myogenesis, cilia, and immune modulation pathways. Human CDH was associated with an upregulation of collagen formation and extracellular matrix reorganization whereas mouse CDH was associated with an increase in muscular contraction. The most common cell types upregulated in human and mouse CDH samples were ciliated airway cells. CONCLUSIONS: This study highlights the unique gene transcriptional patterns in early fetal mouse and human lungs with CDH. These data have implications when determining the translational potential of novel therapies in CDH using nitrofen-based animal models. LEVEL OF EVIDENCE: Level IV. STUDY TYPE: Basic science/case series.

Foreign Body Ingestion Ending Up in Late-Presenting Morgagni Hernia Diagnosis: A Case Report.

Morgagni hernia (MH), also known as a retrosternal or parasternal hernia, is a rare type of congenital diaphragmatic hernia (CDH) characterized by a defect in the anterior diaphragm. Patients with late-diagnosed MH typically present with vague gastrointestinal or respiratory symptoms. In some instances, MH is incidentally identified through chest X-rays performed for other reasons, such as foreign body ingestion, as illustrated in our presented case. We present a case of a delayed congenital diaphragmatic hernia of the Morgagni type in a two-year-old boy with a history of foreign body ingestion and severe abdominal pain. Diagnostic imaging, including chest radiograph and computed tomography (CT) scan, confirmed the diaphragmatic defect. Surgical repair, performed laparoscopically, resulted in an uncomplicated postoperative course and a favorable long-term outcome.

Evaluation of Hyponatremia in Infants on Vasopressin Therapy.

OBJECTIVE: Vasopressin has systemic vasoconstrictive yet pulmonary vasodilatory effects, making it an ideal agent for hypotension management in infants with congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension. The side effects of vasopressin in this population, such as hyponatremia, are understudied. This study aims to characterize the effect of vasopressin on sodium concentrations in infants with and without CDH. METHODS: This was a retrospective review of patients who received vasopressin while admitted to a level IV neonatal intensive care unit. The primary outcome was the incidence of hyponatremia (blood sodium <135 mmol/L) during vasopressin therapy. Secondary outcomes included time to hyponatremia, dose and duration of vasopressin, incidence of severe hyponatremia (blood sodium <125 mmol/L), and hypertonic saline use. Both blood serum and blood gas sample sodium concentrations were used to compare CDH vs non-CDH patients. RESULTS: The average difference between baseline and lowest blood sodium was significant for both CDH and non-CDH patients for all samples (p < 0.001). There was no significant difference in the primary outcome, nor in the secondary outcomes of time to hyponatremia or duration of vasopressin infusion. The average dose of vasopressin was higher in the CDH vs non-CDH group (p = 0.018). The incidences of severe hyponatremia and hypertonic saline use were greater in the CDH vs non-CDH group for patients who had blood serum sodium samples collected (p = 0.049 and p = 0.033, respectively). CONCLUSIONS: This study showed that severe hyponatremia occurred more frequently in CDH vs non-CDH patients. Extreme caution is necessary when managing total body sodium in patients with CDH.

A Case Report of a Diaphragmatic Defect Developing Into a Late-Presenting Congenital Diaphragmatic Hernia With Severe Respiratory Failure.

Diaphragmatic hernia is a congenital malformation, often discovered in the neonatal period, and its occurrence in adults is very rare. This patient, who was completely asymptomatic until the age of 62, had developed an intestinal obstruction and went into respiratory failure after surgery for an external auditory canal carcinoma. He was subsequently diagnosed with a late-presenting congenital diaphragmatic hernia (CDH), thus requiring surgical treatment. Anesthesiologists and critical care physicians should keep in mind the possibility of CDH as well as diaphragmatic relaxation when an unexplained elevation of the diaphragm is observed perioperatively.

MED12 Loss-of-Function Variants as a Cause of Congenital Diaphragmatic Hernia in Females With Hardikar Syndrome and Nonspecific Intellectual Disability.

Mediator complex subunit 12 (MED12) is required for the assembly of the kinase module of Mediator, a regulatory complex that controls the formation of the RNA polymerase II-mediated preinitiation complex. MED12-related disorders display unique gender-specific genotype-phenotype associations and include X-linked recessive Opitz-Kaveggia syndrome, Lujan-Fryns syndrome, Ohdo syndrome, and nonspecific intellectual disability in males predominantly carrying missense variants, and X-linked dominant Hardikar syndrome and nonspecific intellectual disability in females known to predominantly carry de novo nonsense/frameshift and nonsense/missense variants, respectively. MED12 was previously identified as a low-penetrance candidate gene for non-isolated congenital diaphragmatic hernia (CDH+). At the time, however, there was insufficient evidence to confirm this association. In a clinical database search, we identified 18 individuals who were molecularly diagnosed with MED12-related disorders by exome or genome sequencing, including eight missense, four frameshift, two nonsense, and one splice variant. Nine of these variants have not been previously reported. Two females with nonspecific intellectual disability were found to carry a de novo frameshift variant, indicating that potentially truncating variants causing nonspecific intellectual disability are not limited to nonsense variants. Notably, CDH was reported in three out of seven females with Hardikar syndrome or nonspecific intellectual disability but was not reported in males with MED12-related disorders. These results suggest that pathogenic MED12 variants are a cause of CDH+ in females with Hardikar syndrome and nonspecific intellectual disability.

Left-Sided Intra-thoracic Ectopic Kidney With Symptomatic Bochdalek Hernia: A Case Report.

A congenital defect in the diaphragm, known as a Bochdalek hernia (BH), is a condition that allows herniation of the abdominal viscera into the thorax. BH is the most common type of congenital diaphragmatic hernia (CDH) and is typically detected on the left side. An ectopic kidney is a rare condition. An intra-thoracic ectopic kidney is an extremely uncommon condition. In adult patients, the presence of BH with an intra-thoracic kidney is extremely uncommon and is often a finding discovered unintentionally. A 51-year-old male patient presented to the outpatient unit of the pulmonology department. He stated that he had been suffering symptoms such as coughing, wheezing, and breathing difficulties for one year. A chest X-ray showed a well-defined radio-opaque lesion in the lower left zone. A computed tomography (CT) scan of the chest demonstrated a defect in the posterolateral region of the left hemidiaphragm, as well as herniation of the left kidney and retroperitoneal fat in the left hemithorax. The intra-thoracic ectopic kidney was found to be normal in size and showed normal attenuation and enhancement, with the contrast being promptly excreted into the pelvicalyceal system during CT urography. Due to the hernia's small size and lack of abnormalities on CT urography, the patient was recommended a conservative treatment. A follow-up examination was performed on the patient annually. Throughout the follow-up period, there was not a single episode of kidney-related issues. To avoid unwanted image-guided biopsies and surgical procedures, it is imperative to look for intra-thoracic kidneys in patients presenting with a thoracic mass or an elevated hemi-diaphragm.

Beyond Bronchopulmonary Dysplasia: A Comprehensive Review of Chronic Lung Diseases in Neonates.

In neonates, pulmonary diseases such as bronchopulmonary dysplasia and other chronic lung diseases (CLDs) pose significant challenges due to their complexity and high degree of morbidity and mortality. This review discusses the etiology, pathophysiology, clinical presentation, and diagnostic criteria for these conditions, as well as current management strategies. The review also highlights recent advancements in understanding the pathophysiology of these diseases and evolving strategies for their management, including gene therapy and stem cell treatments. We emphasize how supportive care is useful in managing these diseases and underscore the importance of a multidisciplinary approach. Notably, we discuss the emerging role of personalized medicine, enabled by advances in genomics and precision therapeutics, in tailoring therapy according to an individual's genetic, biochemical, and lifestyle factors. We conclude with a discussion on future directions in research and treatment, emphasizing the importance of furthering our understanding of these conditions, improving diagnostic criteria, and exploring targeted treatment modalities. The review underscores the need for multicentric and longitudinal studies to improve preventative strategies and better understand long-term outcomes. Ultimately, a comprehensive, innovative, and patient-centered approach can enhance the quality of care and outcomes for neonates with CLDs.

Bochdalek's hernia in adults: A unique case with gastric volvulus and intrathoracic kidney.

INTRODUCTION AND IMPORATNCE: Introduction: Bochdalek's hernia (BH) is a congenital diaphragmatic hernia predominantly diagnosed in the pediatric population but infrequently found in adults. This paper presents a unique case of an adult patient with a left-sided BH accompanied by gastric volvulus and an intrathoracic kidney. CASE OF PRESENTATION: A 21-year-old male presented with abdominal pain and vomiting. An MDCT scan revealed a twisted stomach, spleen, and kidney herniated into the chest due to left diaphragmatic eventration. Surgery involved untwisting the stomach, relocating the organs, and removing the hernia sac. DISCUSSION: Bochdalek hernias (BHs) are rare conditions in which abdominal organs move into the chest due to defects in the diaphragm. BH usually occurs on the left side and can be triggered by factors such as pregnancy, obesity, or trauma. Symptoms can vary from abdominal pain to chest discomfort, and diagnosis can be challenging. Imaging tests such as CT scans are essential for accurate diagnosis. In adults, the BH can contain various organs, such as the spleen and kidney. Rarely, BH can be associated with an ectopic kidney located inside the chest cavity. In some cases of BH, there is a risk of complications such as gastric volvulus, where the stomach twists on itself, leading to potentially serious symptoms such as severe abdominal pain and vomiting. CONCLUSION: This case underscores the severe risks of BH in adults, such as gastric twisting and blockage, necessitating urgent surgery. Timely diagnosis and surgical intervention are crucial for preventing life-threatening outcomes. More research is needed to improve the management of this rare condition.

Long-term follow-up in congenital diaphragmatic hernia.

With improvements in initial care for patients with congenital diaphragmatic hernia (CDH), the number of CDH patients with severe disease who are surviving to discharge has increased. This growing population of patients faces a unique set of long-term challenges, multisystem adverse outcomes, and post-intervention complications requiring specialized multidisciplinary follow-up. Early identification and intervention are essential to mitigate the potential morbidity associated with these challenges. This manuscript outlines a general framework for long-term follow-up for the CDH patient, including cardiopulmonary, gastrointestinal, neurodevelopmental, surgical, and quality of life outcomes.

Surgical approaches to congenital diaphragmatic hernia.

Surgical repair of the diaphragm is essential for survival in congenital diaphragmatic hernia (CDH). There are many considerations surrounding the operation - why the operation matters, optimal timing of repair and its relation to extracorporeal life support (ECLS) use, minimally invasive versus open approaches, and strategies for reconstruction. Surgery is both affected by, and affects, the physiology of these infants and is an important factor in determining long-term outcomes. Here we discuss the evidence and provide insight surrounding this complex decision making, technical pearls, and outcomes in repair of CDH.

Ventilator strategies in congenital diaphragmatic hernia.

This review focuses on contemporary mechanical ventilator practices used in the initial management of neonates born with congenital diaphragmatic hernia (CDH). Both conventional and non-conventional ventilation modes in CDH are reviewed. Special emphasis is placed on the rationale for gentle ventilation and the current evidence-based clinical practice guidelines that are recommended for supporting these fragile infants. The interplay between CDH lung hypoplasia and other key cardiopulmonary elements of the disease, namely a reduced pulmonary vascular bed, abnormal pulmonary vascular remodeling, and left ventricular hypoplasia, are discussed. Finally, we provide insights into future avenues for mechanical ventilator research in CDH.