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Thoracic Surgery Clinics May 2024The diaphragm is a critical musculotendinous structure that contributes to respiratory function. Disorders of the diaphragm are rare and diagnostically challenging.... (Review)
Review
The diaphragm is a critical musculotendinous structure that contributes to respiratory function. Disorders of the diaphragm are rare and diagnostically challenging. Herein, the author reviews the radiologic options for the assessment of the diaphragm.
Topics: Humans; Diaphragm; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Hernia, Diaphragmatic
PubMed: 38705659
DOI: 10.1016/j.thorsurg.2024.02.002 -
European Journal of Pediatric Surgery :... Aug 2019Congenital diaphragmatic hernia (CDH) is a devastating disease that still carries a high mortality and morbidity rate. Poor outcomes for fetuses and infants with CDH are... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a devastating disease that still carries a high mortality and morbidity rate. Poor outcomes for fetuses and infants with CDH are mainly related to pulmonary hypoplasia (PH) and pulmonary vascular remodeling that leads to pulmonary hypertension (PHTN). Over the last five decades, research efforts have focused on modeling CDH not only to study the pathophysiology of the diaphragmatic defect, pulmonary hypoplasia, and pulmonary hypertension, but also to identify therapies that would promote lung growth and maturation, and correct vascular remodeling. As CDH is a multifactorial condition whose etiology remains unknown, there is not a single model of CDH, rather several ones that replicate different aspects of this disease. While small animals like the mouse and the rat have mainly been used to uncover biological pathways underlying the diaphragmatic defect and poor lung growth, larger animals like the lamb and the rabbit models have been instrumental for pursuing medical and surgical interventions. Overall, the use of animal models has indeed advanced our knowledge on CDH and helped us test innovative therapeutic options. For example, the lamb model of CDH has been the paradigm for testing fetal surgical procedures, including tracheal occlusion, which has been translated to clinical use. In this review, we outline the induction protocols of CDH in animals with the use of chemicals, dietary changes, genetic alterations, and surgical maneuvers, and we describe the studies that have translated experimental results to the bedside.
Topics: Animals; Disease Models, Animal; Fetal Therapies; Hernias, Diaphragmatic, Congenital; Humans; Translational Research, Biomedical
PubMed: 31365938
DOI: 10.1055/s-0039-1693993 -
Surgical Endoscopy May 2022Diaphragmatic hernia (DH), congenital or traumatic, is uncommon but sometimes can lead to a serious surgical emergency. There are no clinical guidelines or approved... (Review)
Review
BACKGROUND
Diaphragmatic hernia (DH), congenital or traumatic, is uncommon but sometimes can lead to a serious surgical emergency. There are no clinical guidelines or approved recommendations for the management of this condition, and most data are from retrospective, single-institution series. The aim is to analyze the management of the DH at our institution and review the indications for laparoscopic repair.
METHODS
A retrospective serie of patients diagnosed of DH with surgical treatment at our institution between 2009 and 2019. Literature review was carried out to establish the current indications of laparoscopic repair in each type of DH.
RESULTS
Surgery was carried out in 15 patients with DH, 5 congenital and 10 traumatic hernias. Traumatic hernias were classified as acute (n = 2) and chronic (n = 8). 53.4% of all cases (8 patients) required urgent surgery using an abdominal approach (5 open and 3 laparoscopic) and elective surgery was performed in 46.6% of all cases (7 patients) with an abdominal approach (3 open and 4 laparoscopic) and 2 patients with a combined approach. Primary repair was performed in 4 patients (26.6%), closure and mesh reinforcement in 9 cases (60%) and only mesh placement in 2 patients (13.4%). Postoperative morbidity and mortality were 20% and 0%, respectively. No recurrences were detected.
CONCLUSIONS
DH may pose different scenarios which require urgent or elective surgical treatment. Laparoscopic approach may be a first option in elective surgery; and in emergency setting taking into account hemodynamic stability and associated injuries.
Topics: Adult; Hernia, Diaphragmatic, Traumatic; Hernias, Diaphragmatic, Congenital; Herniorrhaphy; Humans; Laparoscopy; Retrospective Studies; Surgical Mesh
PubMed: 34312729
DOI: 10.1007/s00464-021-08651-3 -
Seminars in Fetal & Neonatal Medicine Aug 2022Congenital diaphragmatic hernia (CDH) contributes to neonatal morbidity and mortality worldwide. Pulmonary hypertension (PH) is a key component of CDH pathophysiology... (Review)
Review
Congenital diaphragmatic hernia (CDH) contributes to neonatal morbidity and mortality worldwide. Pulmonary hypertension (PH) is a key component of CDH pathophysiology and critical consideration for management and therapeutic options. PH associated with CDH has traditionally been attributed to pulmonary vascular maldevelopment and associated lung parenchymal hypoplasia, leading to pre-capillary increase in pulmonary vascular resistance (PVR). However, there is increasing recognition that left ventricular hypoplasia, dysfunction and elevated end diastolic pressure may contribute to post-capillary pulmonary hypertension in CDH patients. The interplay of these mechanisms and associated dysfunction in the right and left ventricles results in variable hemodynamic phenotypes in CDH. Clinical assessment of individual phenotype may help guide personalized management strategies, including effective use of pulmonary vasodilators and extra-corporeal membrane oxygenation. Ongoing investigation of the underlying mechanisms of PH in CDH, and efficacy of physiology-based treatment approaches may support improvement in outcomes in this challenging condition.
Topics: Echocardiography; Heart Ventricles; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Lung
PubMed: 35995665
DOI: 10.1016/j.siny.2022.101383 -
Annals of the American Thoracic Society Nov 2020
Topics: Heart Ventricles; Hernias, Diaphragmatic, Congenital; Humans; Natriuretic Peptide, Brain; Risk Assessment; Ventricular Function, Right
PubMed: 33124910
DOI: 10.1513/AnnalsATS.202008-1029ED -
Revista Colombiana de Obstetricia Y... Sep 2023To report a case of prenatal diagnosis of ectopic intrathoracic kidney with diaphragmatic hernia managed surgically after birth, and to conduct a review of the...
OBJECTIVES
To report a case of prenatal diagnosis of ectopic intrathoracic kidney with diaphragmatic hernia managed surgically after birth, and to conduct a review of the literature on prenatal diagnosis of ectopic intrathoracic kidney and perinatal prognosis.
MATERIAL AND METHODS
We report the case of a 28-week fetus in which, on ultrasound imaging, a mass was observed displacing the heart and lung in the right hemithorax, which was was confirmed by magnetic resonance (MR) to be an ectopic intrathoracic kidney (ITEK). After birth, the neonate was approached by laparoscopy to place a mesh in continuity with the diaphragm, leaving the kidney in the abdomen, with good evolution. A search was conducted in the PubMed, Embase and Cochrane databases for cohorts, case reports and case series of prenatal diagnosis of intrathoracic kidney in the fetus. Information was retrieved regarding design, population, imaging diagnosis, treatment and prognosis.
RESULTS
The search identified 8 studies that met the inclusion criteria, reporting a total of 8 cases. Ultrasound diagnosis showed ectopic intrathoracic kidney associated with diaphragmatic hernia in all the subjects. Fetal magnetic resonance imaging (MRI) was also used in 5 cases.
CONCLUSIONS
Ectopic intrathoracic kidney is a congenital abnormality amenable to prenatal diagnosis. Survival after corrective surgery performed in the neonatal period is common. There is a paucity of publications, limited to case reports, regarding the prenatal diagnosis of this condition.
Topics: Pregnancy; Infant, Newborn; Female; Humans; Prenatal Diagnosis; Hernia, Diaphragmatic; Hernia; Kidney
PubMed: 37937910
DOI: 10.18597/rcog.4020 -
The Journal of Pediatrics Aug 2019
Topics: Female; Hernia, Diaphragmatic; Humans; Infant; Kidney Diseases; Radiography, Thoracic; Tomography, X-Ray Computed
PubMed: 31128884
DOI: 10.1016/j.jpeds.2019.04.036 -
The American Surgeon Nov 2023Giovanni Morgagni remains an eminent figure in the field of pathological anatomy. Born in Forli, Italy, he excelled as a child. He entered medical school at the age of...
Giovanni Morgagni remains an eminent figure in the field of pathological anatomy. Born in Forli, Italy, he excelled as a child. He entered medical school at the age of 16 years old in Bologna. By the age of 31 he held the chair position at the University of Padua. During his tenure, he discovered many anatomical and pathological findings, with the most widely known discovery being the Morgagni Hernia. Morgagni first described this eponymic hernia in an adult stonecutter during an autopsy. In addition to his many discoveries, his most esteemed written contribution to the field of medicine came in the form of a five-volume book titled , in which he correlated cadaveric anatomy and symptomatology revealed upon autopsy. He remained on faculty at the University of Padua for over five decades until his death in 1771.
Topics: Italy; History, 18th Century; Humans; Anatomy; Hernia, Diaphragmatic
PubMed: 33886389
DOI: 10.1177/00031348211011108 -
Archives of Disease in Childhood Aug 2019Survival rates for congenital diaphragmatic hernia (CDH) are increasing. The long-term outcomes of CDH survivors were compared with a healthy control group to assess the...
OBJECTIVE
Survival rates for congenital diaphragmatic hernia (CDH) are increasing. The long-term outcomes of CDH survivors were compared with a healthy control group to assess the morbidity for guidance of antenatal counselling and long-term follow-up programmes.
PARTICIPANTS AND DESIGN
Participants born with CDH in Western Australia 1993-2008 were eligible with matched controls from the general population. Participants had comprehensive lung function tests, echocardiogram, low-dose chest CT scan and completed a Strengths and Difficulties Questionnaire (SDQ) and quality of life (QOL) questionnaire.
RESULTS
34 matched case-control pairs were recruited. Demographic data between groups were similar. Cases were smaller at follow-up (weight Z-score of -0.2vs0.3; p=0.03; height Z-score of -0.3vs0.6; p=0.01). Cases had lower mean Z-scores for forced expiratory volume in 1 s (FEV) (-1.49 vs -0.01; p=0.004), FEV/forced vital capacity (-1.92 vs -1.2; p=0.009) and forced expiratory flow at 25-75% (FEF25-75) (-1.18vs0.23; p=0.007). Cases had significantly worse respiratory mechanics using forced oscillation technique. Subpleural triangles architectural distortion, linear opacities and scoliosis on chest CT were significantly higher in cases. Prosthetic patch requirement was associated with worse lung mechanics and peak cough flow. Cases had significantly higher rates of gastro-oesophageal reflux disease (GORD) and GORD medication usage. Developmental delay was significantly higher in cases. More cases had a total difficulties score in the high to very high range (25% vs 0%, p=0.03) on the SDQ and reported lower objective QOL scores (70.2 vs 79.8, p=0.02).
CONCLUSION
Survivors of CDH may have significant adverse long-term medical and psychosocial issues that would be better recognised and managed in a multidisciplinary clinic.
Topics: Adolescent; Case-Control Studies; Child; Child Health Services; Echocardiography; Female; Gestational Age; Hernias, Diaphragmatic, Congenital; Humans; Male; Quality of Life; Respiratory Function Tests; Surveys and Questionnaires; Survivors; Tomography, X-Ray Computed; Western Australia
PubMed: 30877092
DOI: 10.1136/archdischild-2018-316091 -
The Journal of Pediatrics Apr 2020
Topics: Hernias, Diaphragmatic, Congenital; Humans; Infant; Respiration; Survivors; Ventricular Dysfunction, Left
PubMed: 31812295
DOI: 10.1016/j.jpeds.2019.11.013