-
BMC Pediatrics Mar 2021To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated...
BACKGROUND
To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors.
METHODS
Children with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Main bronchus diameters and lung volumes (total, left/right) were measured. According to total lung volume (TLV) relative to body surface area, children were grouped into TLV ≥ 50 group and TLV < 50 group and the associations with perinatal-neonatal factors were analyzed.
RESULTS
Of the 28 children (mean age, 6.2 ± 0.2 years) with left-sided CDH, 7 (25%) had abnormal pulmonary function, of whom 6 (87%) showed restrictive patterns. All pulmonary functions except FEF25-75% were worse than those in matched healthy control group. Worse pulmonary function was significantly associated with small head and abdominal circumferences at birth. The mean TLV was 1339.1 ± 363.9 mL and LLV/TLV was 47.9 ± 2.5 mL. Children with abnormal pulmonary function were more likely to have smaller lung volumes. In multivariate analysis, abdominal circumference at birth was significantly associated with abnormal lung volume.
CONCLUSIONS
A quarter of children with repaired CDH showed abnormal pulmonary function. Small abdominal circumference at birth was associated with abnormal pulmonary function and lower TLV. .
Topics: Child; Female; Gestational Age; Hernias, Diaphragmatic, Congenital; Humans; Infant; Infant, Newborn; Lung; Lung Volume Measurements; Pregnancy
PubMed: 33706730
DOI: 10.1186/s12887-021-02586-3 -
Frontiers in Surgery 2021We aimed to investigate the safety and effectiveness of laparoscopic repair for treating chronic traumatic diaphragmatic hernia (CTDH). In this retrospective analysis,...
We aimed to investigate the safety and effectiveness of laparoscopic repair for treating chronic traumatic diaphragmatic hernia (CTDH). In this retrospective analysis, we included 23 cases with CTDH underwent laparoscopy in our hospital between June 2015 and October 2019 was performed. The patient characteristics were recorded. We compared the diameter of hernia ring, surgery duration, intraoperative bleeding volume, means of repairing, as well as the follow-up data. All the patients underwent laparoscopic diaphragmatic hernia repair, without conversion to laparotomy or thoracotomy. The operation time ranged from 60 min to 200 min (mean, 108.04 ± 42.93 min). The blood loss volume ranged from 10 to 300 ml (mean volume, 63.48 ± 71.69 ml). The postoperative hospital stayed ranged from 5 to 15 days (mean, 6.22 ± 2.11 days). The patients were followed up for 1-50 months (mean, 17.5 ± 10.90 months). No recurrence of diaphragmatic hernia was found. Laparoscopic repair of CTDH is featured by fast recovery, high security, and effectiveness. Reducing the hernia contents and close of the hernia ring are crucial for the surgery that is performed based on the size and location of the diaphragmatic hernia.
PubMed: 34336921
DOI: 10.3389/fsurg.2021.706824 -
Frontiers in Bioengineering and... 2023Pulmonary hypertension associated with congenital diaphragmatic hernia (CDH) is a critical factor in determining prognosis. We propose that intra-amniotic sildenafil...
Pulmonary hypertension associated with congenital diaphragmatic hernia (CDH) is a critical factor in determining prognosis. We propose that intra-amniotic sildenafil administration is an effective prenatal therapy for CDH-induced pulmonary hypertension. To assess the efficacy of this treatment, we administered sildenafil to nitrofen-induced congenital diaphragmatic hernia fetuses and control fetuses via an intra-amniotic injection after a laparotomy on the pregnant dam at either E13.5 or E15.5. Intra-amniotic sildenafil treatment attenuated peripheral vascular muscularization, enhanced pulmonary blood flow, and increased the ratio of pulmonary artery size to aortic size in congenital diaphragmatic hernia fetuses after both E13.5 and E15.5 treatments. E13.5-treated congenital diaphragmatic hernia fetuses showed a higher and more prolonged expression of cyclic guanosine monophosphate (cGMP)-dependent protein kinase and more production of vascular endothelial growth factor, resulting in a significant improvement in lung architecture. The E13.5-treated congenital diaphragmatic hernia fetuses also had an increase in lung weight-to-body weight ratio and an improved fetal survival. Intra-amniotic sildenafil treatment did not show any detectable negative effects in control fetuses. Intra-amniotic sildenafil treatment for rats attenuates CDH-induced pulmonary hypertension and enhanced peripheral pulmonary blood flow. Moreover, early intervention may be preferable to better accelerate lung development and improve prognosis. Direct sildenafil administration via an intra-amniotic injection may be a promising option in congenital diaphragmatic hernia prenatal therapy.
PubMed: 37545896
DOI: 10.3389/fbioe.2023.1195623 -
Nature Medicine Mar 2024Isolation of tissue-specific fetal stem cells and derivation of primary organoids is limited to samples obtained from termination of pregnancies, hampering prenatal...
Isolation of tissue-specific fetal stem cells and derivation of primary organoids is limited to samples obtained from termination of pregnancies, hampering prenatal investigation of fetal development and congenital diseases. Therefore, new patient-specific in vitro models are needed. To this aim, isolation and expansion of fetal stem cells during pregnancy, without the need for tissue samples or reprogramming, would be advantageous. Amniotic fluid (AF) is a source of cells from multiple developing organs. Using single-cell analysis, we characterized the cellular identities present in human AF. We identified and isolated viable epithelial stem/progenitor cells of fetal gastrointestinal, renal and pulmonary origin. Upon culture, these cells formed clonal epithelial organoids, manifesting small intestine, kidney tubule and lung identity. AF organoids exhibit transcriptomic, protein expression and functional features of their tissue of origin. With relevance for prenatal disease modeling, we derived lung organoids from AF and tracheal fluid cells of congenital diaphragmatic hernia fetuses, recapitulating some features of the disease. AF organoids are derived in a timeline compatible with prenatal intervention, potentially allowing investigation of therapeutic tools and regenerative medicine strategies personalized to the fetus at clinically relevant developmental stages.
Topics: Pregnancy; Female; Humans; Hernias, Diaphragmatic, Congenital; Amniotic Fluid; Prenatal Care; Lung; Organoids
PubMed: 38438734
DOI: 10.1038/s41591-024-02807-z -
African Journal of Paediatric Surgery :... 2023Despite all the advances, the mortality rate of congenital diaphragmatic hernia (CDH) ranges from 30% to 60% for isolated CDH and as high as 89% when they are associated...
INTRODUCTION
Despite all the advances, the mortality rate of congenital diaphragmatic hernia (CDH) ranges from 30% to 60% for isolated CDH and as high as 89% when they are associated with additional structural or chromosomal anomalies. Hence, a study was conducted to evaluate the factors contributing to the mortality of neonates treated for CDH or the eventration of diaphragm.
MATERIALS AND METHODS
A retrospective study was conducted in the department of paediatric surgery at a tertiary centre. The neonates admitted with a diagnosis of CDH or eventration requiring surgery, between March 2013 and March 2021, were included in the study.
RESULTS
A total of 123 neonates were included in the study. The variables, earlier median age at presentation (1 [1-23] vs. 3 [1-28]; P < 0.001; Mann-Whitney U-test), preterm birth (10/79 vs. 0/44; P = 0.01; Fischer's exact test), inborn (68/79 vs. 27/44; P = 0.002; Chi-square test), weight ≤2 kg (18/79 vs. 1/44; P = 0.003; Chi-square test), central cyanosis at presentation (21/79 vs. 1/44; P < 0.001; Chi-square test), antenatal detection (47/79 vs. 14/44; P = 0.003; Chi-square test) and earlier mean age at surgery (3.66 ± 1.47 vs. 7.66 ± 6.88; P < 0.001; Independent sample t-test) were associated with increased mortality. On multinominal logistic regression analysis, the factors preterm (odd's Ratio [OR] =4.735; P = 0.03), weight ≤2 kg (OR = 5.081; P = 0.02), central cyanosis at presentation (OR = 6.969; P = 0.008) and antenatal detection (OR = 7.471; P = 0.006) were found to be independently associated with increased mortality in CDH/eventration.
CONCLUSION
The factors: prematurity, weight <2 kg, cyanosis at presentation and antenatal diagnosis were independently associated with increased mortality in neonates with CDH/eventration requiring surgery.
Topics: Child; Infant, Newborn; Humans; Female; Pregnancy; Hernias, Diaphragmatic, Congenital; Retrospective Studies; Premature Birth; Cyanosis
PubMed: 36960499
DOI: 10.4103/ajps.ajps_165_21 -
Pediatric Critical Care Medicine : a... May 2023We aimed to determine the prevalence of electrographic seizures and associated odds of adverse outcomes of electrographic seizures in neonates with congenital...
OBJECTIVES
We aimed to determine the prevalence of electrographic seizures and associated odds of adverse outcomes of electrographic seizures in neonates with congenital diaphragmatic hernia (CDH) receiving extracorporeal membrane oxygenation (ECMO).
DESIGN
Retrospective, descriptive case series.
SETTING
Neonatal ICU (NICU) in a quaternary care institution.
PATIENTS
All neonates with CDH receiving ECMO undergoing continuous electroencephalographic monitoring (CEEG) and follow-up between January 2012 and December 2019.
INTERVENTIONS
None.
MEASUREMENTS AND MAIN RESULTS
All eligible neonates with CDH receiving ECMO underwent CEEG (n = 75). Electrographic seizures occurred in 14 of 75 (19%): they were exclusively electrographic-only in nine of 14, both electrographic-only and electroclinical in three of 14, and electroclinical only in two of 14. Two neonates developed status epilepticus. We identified an association between presence of seizures, rather than not, and longer duration of initial session of CEEG monitoring (55.7 hr [48.2-87.3 hr] vs 48.0 hr [43.0-48.3 hr]; p = 0.001). We also found an association between presence of seizures, rather than not, and greater odds of use of a second CEEG monitoring (12/14 vs 21/61; odds ratio [OR], 11.43 [95% CI, 2.34-55.90; p = 0.0026). Most neonates with seizures (10/14), experienced their onset of seizures more than 96 hours after the start of ECMO. Overall, the presence of electrographic seizures, compared with not, was associated with lower odds of survival to NICU discharge (4/14 vs 49/61; OR 0.10 [95% CI 0.03 to 0.37], p = 0.0006). Also, the presence of seizures-rather than not-was associated with greater odds of a composite of death and all abnormal outcomes on follow-up (13/14 vs 26/61; OR, 17.5; 95% CI, 2.15-142.39; p = 0.0074).
CONCLUSIONS
Nearly one in five neonates with CDH receiving ECMO developed seizures during the ECMO course. Seizures were predominantly electrographic-only and when present were associated with great odds of adverse outcomes. The current study provides evidence to support standardized CEEG in this population.
Topics: Humans; Infant, Newborn; Extracorporeal Membrane Oxygenation; Hernias, Diaphragmatic, Congenital; Retrospective Studies; Seizures; Prevalence; Intensive Care Units, Neonatal; Electroencephalography
PubMed: 37140337
DOI: 10.1097/PCC.0000000000003197 -
The Canadian Veterinary Journal = La... Feb 2023Data on dogs and cats that underwent surgery for a traumatic diaphragmatic hernia were retrospectively collected and analyzed, with an objective to identify factors that...
Data on dogs and cats that underwent surgery for a traumatic diaphragmatic hernia were retrospectively collected and analyzed, with an objective to identify factors that influenced the survival rate. Forty-nine dogs and 48 cats were included. The predominant respiratory clinical sign was dyspnea, and the interval from trauma to development of clinical signs did not influence patient survival. Concurrent orthopedic and/or soft tissue injuries were identified in 48.45% of the animals. Intraoperative complications occurred in 14 dogs and 5 cats, and postoperative complications in 7 dogs and 6 cats. Intraoperative death occurred in 6.2% and postoperative death in 8.3% from 1 h to 10 d after surgery. Animals that received surgical treatment within 48 h after diagnosis had a lower risk of death. Conversely, concurrent injuries and intraoperative and postoperative complications were the main factors associated with a higher risk of death.
Topics: Cats; Dogs; Animals; Retrospective Studies; Treatment Outcome; Cat Diseases; Dog Diseases; Hernia, Diaphragmatic; Postoperative Complications
PubMed: 36733640
DOI: No ID Found -
Cirugia Pediatrica : Organo Oficial de... Jul 2022The advances made in the surgical and postnatal treatment of congenital diaphragmatic hernia (CDH) have considerably improved patient survival, but morbidity remains...
OBJECTIVE
The advances made in the surgical and postnatal treatment of congenital diaphragmatic hernia (CDH) have considerably improved patient survival, but morbidity remains significant. The objective of this study was to analyze the effect these sequels have on the health-related quality of life (HRQL) of adolescents and young adults who have survived CDH, and to compare it with that of the general population.
MATERIALS AND METHODS
A transversal descriptive study of patients diagnosed with CDH in our institution from 1997 to 2004 was carried out. Survival, location, hernia size, herniated organs, need for extracorporeal membrane oxygenation, and mechanical ventilation time were analyzed. In addition, a comparative study of the current HRQL of survivors was conducted using the SF-36 survey (36-Item Health Survey Short Form), which assessed physical function, physical role, body pain, general health, vitality, social function, emotional role, and mental health. Data of 24 healthy adolescents was used as a control group.
RESULTS
Of the 29 survivors (70.7%), 21 were successfully contacted, and 16 responded to the survey. They all claimed their overall quality of life was good or very good. The group of adolescents who underwent CDH surgical repair had better results in the vitality (p= 0.001) and mental health (p<0.05) areas, but the overall HRQL score and the remaining health areas were similar. No significant differences were found regarding diaphragmatic size or need for ECMO.
CONCLUSION
According to adolescent survivors who underwent CDH surgical repair, their quality of life is similar to that of other individuals of their age. Our results are encouraging and may prove useful for future parents of CDH patients.
Topics: Adolescent; Diaphragm; Extracorporeal Membrane Oxygenation; Hernias, Diaphragmatic, Congenital; Humans; Quality of Life; Retrospective Studies
PubMed: 35796083
DOI: 10.54847/cp.2022.03.14 -
The Turkish Journal of Pediatrics 2023Late-presenting congenital diaphragmatic hernia occurs beyond the neonatal period, and is relatively rare, presenting with nonspecific respiratory and gastrointestinal...
BACKGROUND
Late-presenting congenital diaphragmatic hernia occurs beyond the neonatal period, and is relatively rare, presenting with nonspecific respiratory and gastrointestinal symptoms.
CASE
We report a rare case of late-presenting congenital diaphragmatic hernia in a 7-year-old girl, who presented with abdominal pain, shortness of breath and fever on admission. Work-up revealed intrathoracic gastric perforation, acute pancreatitis and septic shock with a diaphragmatic defect. Due to the high content of amylase in pleural effusion, we suspected the presence of a pancreaticopleural fistula, and we were also puzzled whether the gastric perforation was caused by a pleural indwelling catheterization, but this was ruled out. We about performed a laparotomy to reposition the herniated organs, repair the hernia and the gastric perforation, and undergo the gastrostomy. The girl had an uneventful post-operative recovery.
CONCLUSIONS
Late-presenting congenital diaphragmatic hernias are often misdiagnosed. Clinicians should combine multiple imaging modalities to make a definite diagnosis and perform surgery as soon as possible to avoid severe complications.
Topics: Infant, Newborn; Female; Child; Humans; Hernias, Diaphragmatic, Congenital; Acute Disease; Pancreatitis; Abdominal Pain; Tomography, X-Ray Computed
PubMed: 37853980
DOI: 10.24953/turkjped.2022.512 -
Cureus Jan 2024In this case report, we describe a rare presentation of diaphragmatic hernia in a pediatric patient presenting with syncope. Congenital diaphragmatic hernia (CDH) is a...
In this case report, we describe a rare presentation of diaphragmatic hernia in a pediatric patient presenting with syncope. Congenital diaphragmatic hernia (CDH) is a developmental discontinuity of the diaphragm that causes the abdominal viscera to herniate into the thoracic cavity. It is usually diagnosed shortly after birth and is often associated with pulmonary hypoplasia and pulmonary hypertension, causing life-threatening conditions, or it could be completely asymptomatic. Syncope is induced by various conditions such as cerebrovascular disease, arrhythmia, hypoglycemia, anemia, epilepsy, and autonomic nervous disorder.
PubMed: 38318536
DOI: 10.7759/cureus.51715