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The New England Journal of Medicine Jul 2021
Topics: Fetal Diseases; Fetus; Hernias, Diaphragmatic, Congenital; Humans
PubMed: 34106554
DOI: 10.1056/NEJMe2107446 -
ANZ Journal of Surgery Nov 2020Parahiatal hernia is a less common form of diaphragmatic hernia, with the defect lateral to the crus and oesophageal hiatus, and the unfamiliarity of this disease would... (Review)
Review
BACKGROUND
Parahiatal hernia is a less common form of diaphragmatic hernia, with the defect lateral to the crus and oesophageal hiatus, and the unfamiliarity of this disease would cause confusion to general surgeons. In the present study, the literature on parahiatal hernia were reviewed, the clinical profile of this disease, as well as our own experience is presented and analysed.
METHODS
In the present study, a thorough identification of all published reports on parahiatal hernias was made, together with our own cases, the available data were summarized, analysed and discussed.
RESULTS
A total of 27 cases of parahiatal hernias were identified since 1987. Among them, 19 cases were primary parahiatal hernias, and eight cases were secondary or acquired parahiatal hernias. None of the 27 cases were pre-operatively diagnosed, and the majority of them were pre-operatively diagnosed as paraoesophageal hernias. Detailed treatment data were available in 26 of the 27 cases. Three patients received open surgery, and 23 patients were treated with laparoscopic procedures. Suture repair was used in 12 cases, and 14 cases were repaired with mesh reinforcement. In addition, two cases underwent partial gastrectomy, stomach suture was performed in another two cases.
CONCLUSION
Patients with parahiatal hernia have a high risk of developing hernia incarceration or gastric vovulus. Laparoscopic treatment of parahiatal hernia is feasible and safe in the majority cases. Surgeons should be aware of this disease when performing paraoesophageal hernia repair, as parahiatal hernias may occur with or without previous diaphragmatic surgery.
Topics: Diaphragm; Hernia, Hiatal; Hernias, Diaphragmatic, Congenital; Herniorrhaphy; Humans; Laparoscopy; Surgical Mesh
PubMed: 32356615
DOI: 10.1111/ans.15926 -
World Journal of Emergency Surgery :... Jul 2023Diaphragmatic hernia (DH) presenting acutely can be a potentially life-threatening condition. Its management continues to be debatable. (Review)
Review
BACKGROUND
Diaphragmatic hernia (DH) presenting acutely can be a potentially life-threatening condition. Its management continues to be debatable.
METHODS
A bibliographic search using major databases was performed using the terms "emergency surgery" "diaphragmatic hernia," "traumatic diaphragmatic rupture" and "congenital diaphragmatic hernia." GRADE methodology was used to evaluate the evidence and give recommendations.
RESULTS
CT scan of the chest and abdomen is the diagnostic gold standard to evaluate complicated DH. Appropriate preoperative assessment and prompt surgical intervention are important for a clinical success. Complicated DH repair is best performed via the use of biological and bioabsorbable meshes which have proven to reduce recurrence. The laparoscopic approach is the preferred technique in hemodynamically stable patients without significant comorbidities because it facilitates early diagnosis of small diaphragmatic injuries from traumatic wounds in the thoraco-abdominal area and reduces postoperative complications. Open surgery should be reserved for situations when skills and equipment for laparoscopy are not available, where exploratory laparotomy is needed, or if the patient is hemodynamically unstable. Damage Control Surgery is an option in the management of critical and unstable patients.
CONCLUSIONS
Complicated diaphragmatic hernia is a rare life-threatening condition. CT scan of the chest and abdomen is the gold standard for diagnosing the diaphragmatic hernia. Laparoscopic repair is the best treatment option for stable patients with complicated diaphragmatic hernias. Open repair is considered necessary in majority of unstable patients in whom Damage Control Surgery can be life-saving.
Topics: Humans; Diaphragm; Hernias, Diaphragmatic, Congenital; Tomography, X-Ray Computed; Thorax; Hernia, Hiatal; Thoracic Injuries
PubMed: 37496073
DOI: 10.1186/s13017-023-00510-x -
Best Practice & Research. Clinical... Jul 2019Congenital diaphragmatic hernia is characterized by failed closure of the diaphragm, thereby allowing abdominal viscera to herniate into the thoracic cavity and... (Review)
Review
Congenital diaphragmatic hernia is characterized by failed closure of the diaphragm, thereby allowing abdominal viscera to herniate into the thoracic cavity and subsequently interfering with normal lung development. At birth, pulmonary hypoplasia leads to respiratory insufficiency and persistent pulmonary hypertension (PHT), that is lethal in up to 32% of patients. In isolated cases, the outcome may be predicted prenatally by medical imaging and advanced genetic testing. In those fetuses with a predicted poor outcome, fetoscopic endoluminal tracheal occlusion may be offered. This procedure is currently being evaluated in a global randomized clinical trial (www.TOTALtrial.eu). We are currently investigating alternative strategies including transplacental sildenafil administration to reduce the occurrence of persistent PHT.
Topics: Female; Fetal Therapies; Genetic Testing; Hernias, Diaphragmatic, Congenital; Humans; Liver; Lung; Pregnancy; Stomach; Ultrasonography, Prenatal
PubMed: 30772144
DOI: 10.1016/j.bpobgyn.2018.12.010 -
Seminars in Perinatology Feb 2020Congenital Diaphragmatic Hernia (CDH) is a birth defect that is characterized by lung hypoplasia, pulmonary hypertension and a diaphragmatic defect that allows... (Review)
Review
Congenital Diaphragmatic Hernia (CDH) is a birth defect that is characterized by lung hypoplasia, pulmonary hypertension and a diaphragmatic defect that allows herniation of abdominal organs into the thoracic cavity. Although widely unknown to the public, it occurs as frequently as cystic fibrosis (1:2500). There is no monogenetic cause, but different animal models revealed various biological processes and epigenetic factors involved in the pathogenesis. However, the pathobiology of CDH is not sufficiently understood and its mortality still ranges between 30 and 50%. Future collaborative initiatives are required to improve our basic knowledge and advance novel strategies to (prenatally) treat the abnormal lung development. This review focusses on the genetic, epigenetic and protein background and the latest advances in basic and translational aspects of CDH research.
Topics: Animals; Disease Models, Animal; Epigenesis, Genetic; Fetal Therapies; Fetoscopy; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; MicroRNAs; Prenatal Diagnosis; Stem Cell Transplantation; Trachea; Translational Research, Biomedical; Vascular Remodeling
PubMed: 31427115
DOI: 10.1053/j.semperi.2019.07.009 -
Seminars in Pediatric Surgery Aug 2019Congenital diaphragmatic hernia (CDH) is a condition that results from incomplete diaphragm formation during embryogenesis. The diaphragmatic defect allows for... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a condition that results from incomplete diaphragm formation during embryogenesis. The diaphragmatic defect allows for herniation of abdominal viscera into the chest, and the resulting pulmonary hypoplasia and pulmonary hypertension can lead to cardiorespiratory failure in the neonatal period. There is a wide spectrum of disease severity in CDH, and while advances in neonatal care and the introduction of extracorporeal membrane oxygenation have improved outcomes in many cases, the most severe defects are still associated with high morbidity and mortality. Improvements in prenatal diagnostic and prognostic capabilities have created an opportunity to select high risk patients for fetal intervention. Three decades of refinements in the fetal surgical therapy for CDH have led to the current technique of Fetoscopic Endoluminal Tracheal Occlusion (FETO). Herein, we review the current considerations for selecting patients for fetal intervention, and the contemporary fetal surgical operation for CDH, FETO, with a focus on early outcomes and ongoing studies.
Topics: Clinical Trials as Topic; Female; Fetoscopy; Fetus; Hernia; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Liver; Lung Volume Measurements; Magnetic Resonance Imaging; Pregnancy; Prognosis; Severity of Illness Index; Ultrasonography, Prenatal
PubMed: 31451175
DOI: 10.1053/j.sempedsurg.2019.07.001 -
Journal of Pediatric Surgery Sep 2022Emanuel Syndrome (ES), a rare chromosomal disorder caused by a supernumerary chromosome 22 derivative (der(22)t(11;22)), was identified in a fetus with congenital...
BACKGROUND
Emanuel Syndrome (ES), a rare chromosomal disorder caused by a supernumerary chromosome 22 derivative (der(22)t(11;22)), was identified in a fetus with congenital diaphragmatic hernia (CDH) at our fetal center. We aimed to identify a precedent for clinical care and patient outcomes to guide family decision-making.
METHODS
This non-funded and non-registered study queried the entire CDH Registry (CDHR) including >10,000 patients since 1995 and conducted a systematic literature review for patients with concomitant ES and CDH.
RESULTS
Literature review captured 12 citations and identified 9 patients with CDH+ES from over 400 known ES cases. Given the rarity of the disease and to reduce bias, there were no exclusion criteria aside from non-English language. Of these 9, two underwent surgical CDH repair with neither surviving. The CDHR identified 6 patients with ES, all reported after 2013 and prenatally diagnosed. Median estimated gestational age was 39 weeks (range 37-40) and median birth weight was 2.72 kg (range 2.4-3.4 kg). 3 patients died within the first few postnatal days; surgical repair was not offered due to "anomalies" and "pulmonary hypertension" in two and one family chose comfort measures. The other 3 patients underwent surgical repair, and 2 were supported with ECMO. Two patients survived to discharge, incurring surgical comorbidities associated with severe CDH including gastrostomy dependence, tracheostomy, and CDH recurrence.
CONCLUSIONS
ES patients with CDH have potential to tolerate repair and survive to discharge, however with significant additional morbidity combined with severe challenges inherent to ES. This represents the largest series of patients with CDH and ES to date.
LEVEL OF EVIDENCE
IV (Case series with no comparison group).
Topics: Chromosome Disorders; Cleft Palate; Extracorporeal Membrane Oxygenation; Heart Defects, Congenital; Hernias, Diaphragmatic, Congenital; Humans; Infant; Intellectual Disability; Muscle Hypotonia; Retrospective Studies
PubMed: 34865829
DOI: 10.1016/j.jpedsurg.2021.11.005 -
Archives of Disease in Childhood. Fetal... Sep 2022Congenital diaphragmatic hernia (CDH) is a complex malformation characterised by a triad of pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a complex malformation characterised by a triad of pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. Much of the mortality and morbidity in CDH is largely accounted for by PH, especially when persistent beyond the neonatal period and refractory to available treatment. Gentle ventilation, haemodynamic optimisation and pulmonary vasodilation constitute the foundations of neonatal treatment of CDH-related PH (CDH-PH). Moreover, early prenatal diagnosis, the ability to assess severity and the developmental nature of the condition generate the perfect rationale for fetal therapy. Shortcomings of currently available clinical therapies in combination with increased understanding of CDH pathophysiology have spurred experimental drug trials, exploring new therapeutic mechanisms to tackle CDH-PH. We herein discuss clinically available neonatal and fetal therapies specifically targeting CDH-PH and review the most promising experimental treatments and future research avenues.
Topics: Female; Fetal Therapies; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Infant, Newborn; Lung; Pregnancy; Prenatal Diagnosis
PubMed: 34952853
DOI: 10.1136/archdischild-2021-322617 -
The Veterinary Clinics of North... Jul 2024Laparoscopic herniorrhaphy provides a feasible minimally invasive treatment option for dogs with peritoneal-pericardial hernias with careful case selection. This article... (Review)
Review
Laparoscopic herniorrhaphy provides a feasible minimally invasive treatment option for dogs with peritoneal-pericardial hernias with careful case selection. This article describes the techniques, instrumentation, and challenges associated with laparoscopic peritoneal-pericardial diaphragmatic hernia repair.
Topics: Animals; Laparoscopy; Dogs; Dog Diseases; Hernia, Diaphragmatic; Herniorrhaphy; Pericardium
PubMed: 38519368
DOI: 10.1016/j.cvsm.2024.02.003 -
The New England Journal of Medicine Apr 2021
Topics: Arrhythmias, Cardiac; Calcinosis; Colon, Transverse; Drainage; Female; Hernia, Diaphragmatic; Humans; Middle Aged; Pericarditis, Constrictive; Pericardium; Tomography, X-Ray Computed
PubMed: 33793148
DOI: 10.1056/NEJMicm2028463