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Paediatric Respiratory Reviews Mar 2021To develop a clinical guideline for structured assessment and uniform reporting of congenital lung abnormalities (CLA) on Computed Tomography (CT)-scans. (Review)
Review
OBJECTIVES
To develop a clinical guideline for structured assessment and uniform reporting of congenital lung abnormalities (CLA) on Computed Tomography (CT)-scans.
MATERIALS AND METHODS
A systematic literature search was conducted for articles describing CT-scan abnormalities of congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE) and bronchogenic cyst (BC). A structured report using objective features of CLA was developed after consensus between a pediatric pulmonologist, radiologist and surgeon.
RESULTS
Of 1581 articles identified, 158 remained after title-abstract screening by two independent reviewers. After assessing full-texts, we included 28 retrospective cohort-studies. Air-containing cysts and soft tissue masses are described in both CPAM and BPS while anomalous arterial blood supply is only found in BPS. Perilesional low-attenuation areas, atelectasis and mediastinal shift may be found in all aforementioned abnormalities and can also be seen in CLE as a cause of a hyperinflated lobe. We have developed a structured report, subdivided into five sections: Location & Extent, Airway, Lesion, Vascularization and Surrounding tissue.
CONCLUSIONS
CT-imaging findings in CLA are broad and nomenclature is variable. Overlap is seen between and within abnormalities, possibly due to definitions often being based on pathological findings, which is an unsuitable approach for CT imaging. We propose a structured assessment of CLA using objective radiological features and uniform nomenclature to improve reporting.
Topics: Child; Cystic Adenomatoid Malformation of Lung, Congenital; Humans; Lung; Pulmonary Atelectasis; Respiratory System Abnormalities; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 32178987
DOI: 10.1016/j.prrv.2019.12.004 -
Journal of Thoracic Disease Jan 2023
PubMed: 36794129
DOI: 10.21037/jtd-22-1420 -
Pneumologie (Stuttgart, Germany) Jun 2020An 80-year old female was referred to our hospital with left internal carotid artery stenosis and a childhood history of hemoptysis.
HISTORY
An 80-year old female was referred to our hospital with left internal carotid artery stenosis and a childhood history of hemoptysis.
INVESTIGATIONS AND DIAGNOSIS
The ECG showed 2nd degree Mobitz atrio-ventricular block. The chest x-ray and computerized tomography identified a shift of the mediastinum and the heart to the left. The left lung was completely destroyed whilst the right lung was enlarged and crossed the midline. Pulmonary function tests revealed a moderate restrictive ventilation disorder. The diagnosis of autopneumonectomy was based on patient history together with radiological findings.
TREATMENT AND COURSE
A pacemaker was implanted with two stimulation electrodes via a left cephalic venous cutdown. A carotid endarterectomy was also performed without any complication.
CONCLUSION
After autopneumonectomy, postpneumonectomy like syndrome may occur in very rare cases, whereupon operative treatment is mandatory. Any respiratory infections should be treated with antibiotics. Pacemaker electrode placement via the subclavian vein is contraindicated due to the risk of a catastrophic pneumothorax.
Topics: Aged, 80 and over; Carotid Stenosis; Endarterectomy, Carotid; Female; Hemoptysis; Humans; Lung; Lung Diseases; Pacemaker, Artificial; Pneumonectomy; Respiratory Function Tests; Subclavian Vein; Treatment Outcome; Venous Cutdown
PubMed: 32557508
DOI: 10.1055/a-1148-8770 -
Fetal and Pediatric Pathology Oct 2022One-third of fetal soft tissue tumors are malignant and include congenital fibrosarcoma (CF). We report two fetal CFs arising in the posterior mediastinum. In case 1,...
One-third of fetal soft tissue tumors are malignant and include congenital fibrosarcoma (CF). We report two fetal CFs arising in the posterior mediastinum. In case 1, the CF resulted in a mediastinal shift, extensive infiltration of the tumor around adjacent structures, pulmonary hypoplasia, pleural effusion, and rapid growth. The pregnancy was terminated. Case 2 had multiple intrathoracic masses, thoracic hypoplasia, pleural effusion, and fetal death. Both were diagnosed as fibrosarcoma at fetopsy. Although congenital CF tends to be locally aggressive with a low metastatic rate, it tends to grow rapidly and the tumor location can affect fetal survival. In Case 1, the tumor demonstrated locally aggressive behavior whereas multiple distant metastases such as lung, liver, adrenals, and left eye were detected in Case 2. The tumor was directly responsible for intrauterine fetal demise in the second case.
Topics: Female; Fetus; Fibrosarcoma; Humans; Mediastinum; Pleural Effusion; Pregnancy; Soft Tissue Neoplasms
PubMed: 34622726
DOI: 10.1080/15513815.2021.1988010 -
Praxis Nov 2022Pulmonary Opacities - What Lies Beneath? Abstract: Pulmonary opacities are among the most common findings that general practitioners and internists have to interpret in...
Pulmonary Opacities - What Lies Beneath? Abstract: Pulmonary opacities are among the most common findings that general practitioners and internists have to interpret in everyday life. Conventional chest x-rays are still important, but computed tomograms often provide additional information. Patient history, clinical examination but also additionally collected laboratory findings are important prerequisites for the interpretation of imaging studies. Likewise, radiological patterns should be recognized and correctly described. The density, distribution to one or both sides, basal or apical, unifocal or multifocal, also the involvement of the interstitial tissue, bronchioles, the alveolar space and pleura can provide decisive differential diagnostic information. Space-occupying or shrinking processes may be suspected on behalf of the course of pleural lines. Tumours may be differentiated from shrinking lung volume as seen in atelectasis by shift of the mediastinum or the shape of pleural lines. Occasionally control images can support the interpretation of the radiological results.
Topics: Humans; Pleura; Diagnostic Imaging
PubMed: 36415984
DOI: 10.1024/1661-8157/a003932 -
Translational Pediatrics Apr 2024In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development... (Review)
Review
In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25-30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24-48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.
PubMed: 38715680
DOI: 10.21037/tp-23-602 -
Life (Basel, Switzerland) Dec 2023Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature.... (Review)
Review
BACKGROUND
Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature. The occasional necessity for pneumonectomy is still supported by limited evidence.
METHODS
A systematic review of the literature was conducted using the PubMed search engine, focusing on UPIPA cases that received pneumonectomy. Thirty-one pertinent articles were selected and included in the analysis. A case reported from our institution was included in the analysis.
RESULTS
We found 25 adults and seven children affected by UPIPA who received an indication for pneumonectomy, plus an additional case that was reported by our institution. Among adult patients, the predominant indication was hemoptysis (57%), followed by suspected or confirmed lung cancer (23%). Approximately 46% of surgical procedures were classified as urgent or emergent. Postoperative complications were observed in 36% of cases, with no recorded mortality. In pediatric cases, pneumonectomy was primarily a life-saving intervention, performed urgently or emergently in 75% of instances. A possible late complication in pediatric patients involves a mediastinal shift leading to respiratory distress, which may be mitigated using an inflatable prosthesis.
CONCLUSIONS
Pneumonectomy achieves complete resolution of UPIPA symptoms. In the adult population, its primary indication is hemoptysis, with procedures conducted in both elective and urgent/emergent settings. Despite a mortality rate of zero, a notable proportion of patients may experience postoperative complications. In pediatric cases, the clinical presentation varies more extensively, and pneumonectomy is typically reserved for life-threatening situations, emphasizing the need for careful patient selection.
PubMed: 38137929
DOI: 10.3390/life13122328 -
Journal of Clinical Medicine Jan 2024Fetal magnetic resonance imaging (MRI) is broadly used as a method for assessing prognosis in congenital diaphragmatic hernia (CDH). In addition to the extent of lung...
OBJECTIVE
Fetal magnetic resonance imaging (MRI) is broadly used as a method for assessing prognosis in congenital diaphragmatic hernia (CDH). In addition to the extent of lung hypoplasia, determined by measuring the lung volume, cardiac impairment due to pulmonary hypertension and left cardiac hypoplasia is decisive for the prognosis. The percentage area of left ventricle (pALV) describes the percentage of the inner area of the left ventricle in relation to the total area, whereas the mediastinal shift angle (MSA) quantifies the extent of cardiac displacement. The prognostic value of pALV and MSA should be evaluated in terms of survival, the need for extracorporeal membrane oxygenation (ECMO) therapy, and the development of chronic lung disease (CLD).
METHODS
In a total of 122 fetal MRIs, the MSA and pALV were measured retrospectively and complete outcome parameters were determined regarding survival for all 122 subjects, regarding ECMO therapy in 109 cases and about the development of CLD in 78 cases. The prognostic value regarding the endpoints was evaluated using logistic regression and ROC analysis.
RESULTS
The MSA was significantly higher in children who received ECMO therapy ( = 0.0054), as well as in children who developed CLD ( = 0.0018). ROC analysis showed an AUC of 0.68 for ECMO requirement and 0.77 with respect to CLD development. The pALV showed a tendency towards higher levels in children who received ECMO therapy ( = 0.0824). The MSA and the pALV had no significant effect on survival (MSA: = 0.4293, AUC = 0.56; pALV: = 0.1134, AUC = 0.57).
CONCLUSIONS
The MSA determined in fetal MRI is a suitable prognostic parameter for ECMO requirement and CLD development in CDH patients and can possibly be used as a supplement to the established parameters.
PubMed: 38202274
DOI: 10.3390/jcm13010268 -
European Radiology Mar 2024To develop a mediastinal shift angle (MSA) measurement method applicable to right-sided congenital diaphragmatic hernia (RCDH) in fetal MRI and to validate the...
OBJECTIVES
To develop a mediastinal shift angle (MSA) measurement method applicable to right-sided congenital diaphragmatic hernia (RCDH) in fetal MRI and to validate the predictive value of MSA in RCDH.
METHODS
Twenty-seven fetuses with isolated RCDH and 53 controls were included in our study. MSA was measured on MRI axial image at the level of four-chamber view of the fetal heart. The angle between the sagittal midline landmark line and the left boundary landmark line touching tangentially the lateral wall of the left ventricle was used to quantify MSA for RCDH. Appropriate statistical analyses were performed to determine whether MSA can be regarded as a valid predictive tool for postnatal outcomes. Furthermore, predictive performance of MSA was compared with that of lung area to head circumference ratio (LHR), observed/expected LHR (O/E LHR), total fetal lung volume (TFLV), and observed/expected TFLV (O/E TFLV).
RESULTS
MSA was significantly higher in the RCDH group than in the control group. MSA, LHR, O/E LHR, TFLV, and O/E TFLV were all correlated with postnatal survival, pulmonary hypertension (PH), and extracorporeal membrane oxygenation (ECMO) therapy (p < 0.05). Value of the AUC demonstrated good predictive performance of MSA for postnatal survival (0.901, 95%CI: (0.781-1.000)), PH (0.828, 95%CI: (0.661-0.994)), and ECMO therapy (0.813, 95%CI: (0.645-0.980)), which was similar to O/E TFLV but slightly better than TFLV, O/E LHR, and LHR.
CONCLUSIONS
We developed a measurement method of MSA for RCDH for the first time and demonstrated that MSA could be used to predict postnatal survival, PH, and ECMO therapy in RCDH.
CLINICAL RELEVANCE STATEMENT
Newly developed MRI assessment method of fetal MSA in RCDH offers a simple and effective risk stratification tool for patients with RCDH.
KEY POINTS
• We developed a measurement method of mediastinal shift angle for right-sided congenital diaphragmatic hernia for the first time and demonstrated its feasibility and reproducibility. • Mediastinal shift angle can predict more prognostic information other than survival in right-sided congenital diaphragmatic hernia with good performance. • Mediastinal shift angle can be used as a simple and effective risk stratification tool in right-sided congenital diaphragmatic hernia to improve planning of postnatal management.
Topics: Pregnancy; Female; Humans; Hernias, Diaphragmatic, Congenital; Lung; Lung Volume Measurements; Reproducibility of Results; Hypertension, Pulmonary; Magnetic Resonance Imaging; Risk Assessment; Ultrasonography, Prenatal; Retrospective Studies
PubMed: 37644150
DOI: 10.1007/s00330-023-10178-1 -
Japanese Journal of Clinical Oncology Mar 2021Locally advanced non-small cell lung cancer, especially mediastinal lymph node metastasis-positive stage IIIA-N2 cancer, is a heterogeneous disease state characterized... (Review)
Review
Locally advanced non-small cell lung cancer, especially mediastinal lymph node metastasis-positive stage IIIA-N2 cancer, is a heterogeneous disease state characterized by anatomically locally advanced disease with latent micrometastases. Thus, surgical resection or radiotherapy alone has historically failed to cure this disease. During the last three decades, persistent efforts have been made to develop a suitable treatment modality to overcome these problems using chemotherapy and/or radiotherapy with surgical resection. However, the role of surgical resection remains unclear, and the standard treatment for stage IIIA-N2 disease is concurrent chemoradiotherapy. In general, adjuvant chemotherapy is indicated for completely resected pathological stage IB disease or lymph node metastasis-positive pathological stage II or IIIA disease. Platinum-based doublet cytotoxic chemotherapy is currently the standard regimen. Additionally, post-operative radiotherapy might be indicated for post-operatively proven mediastinal lymph node metastasis; i.e. clinical N0-1 and pathological N2 disease. With the remarkable progression that has recently been made in the field of chemotherapy, such as advances in molecular targeting agents and immune checkpoint inhibitors, the basic policy of chemotherapy has been shifting to personalized treatment based on the individual patient's oncogene driver mutation status, immune status and other parameters. The same trend is being seen in the treatment of stage IIIA-N2 disease. We should consider the past and upcoming results of several clinical trials to optimize the coming era of personalized treatment.
Topics: Carcinoma, Non-Small-Cell Lung; Clinical Trials, Phase III as Topic; Combined Modality Therapy; Humans; Induction Chemotherapy; Lung Neoplasms; Neoplasm Staging
PubMed: 33506253
DOI: 10.1093/jjco/hyaa249