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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 -
Heart, Lung & Circulation Dec 2020Pneumonectomy in the adult patient is associated with a mortality of 1-9%. Death is often due to post pneumonectomy pulmonary oedema (PPPO). The use of balanced chest... (Observational Study)
Observational Study
BACKGROUND
Pneumonectomy in the adult patient is associated with a mortality of 1-9%. Death is often due to post pneumonectomy pulmonary oedema (PPPO). The use of balanced chest drainage system (BCD) in the setting of post pneumonectomy has been reported to be of benefit in the prevention of PPPO. This study seeks to compare the incidence of PPPO in patients who underwent pneumonectomy and whose empty pleural space was managed either with CRD or BCD.
METHODS
This retrospective observational cohort study involved 98 patients who were operated on by one surgeon at Liverpool Hospital, Sydney, Australia from 1997 to 2019. The patients were divided into two groups according to the era in which they had their pneumonectomy. Group 1 consisted of 18 patients managed with clamp-release drainage between 1997 and 2002. Group 2 consisted of 80 patients managed with balanced chest drainage between 2003 and 2019. The primary outcomes of interest were the development of PPPO and death. Demographic and clinico-pathological variables between the groups were compared including whether the phrenic nerve was sacrificed, volume of infused intraoperative fluid, duration of single lung ventilation, intraoperative tidal volumes, agents of anaesthetic induction and maintenance, mean urine output in the first 4 postoperative hours, institution of a postoperative 1.5 L fluid restriction, total chest drainage, day of chest drain removal, presence of radiological postoperative mediastinal shift, post-pneumonectomy pulmonary oedema and death. Group characteristics were compared using t-test and chi-squared for continuous and categorical variables respectively. Univariate and multivariate analysis was also undertaken using the Firth method of logistic regression for rare occurrences in a stepwise fashion.
RESULTS
Through univariate analysis, balanced chest drainage, postoperative fluid restriction and intraoperative fluid infusion showed significant effect on PPPO. Through multivariate analysis, balanced chest drainage was found to have independent protective value for PPPO and mortality.
CONCLUSION
Compared with clamp-release drainage, balanced chest drainage results in a lower incidence of post-pneumonectomy pulmonary oedema and death.
Topics: Chest Tubes; Drainage; Female; Follow-Up Studies; Humans; Male; Middle Aged; Pneumonectomy; Postoperative Complications; Pulmonary Edema; Retrospective Studies; Thorax
PubMed: 32327311
DOI: 10.1016/j.hlc.2020.03.006 -
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 -
European Radiology Apr 2018To prospectively evaluate usefulness of the apparent diffusion coefficient (ADC) in differentiating anterior mediastinal lymphoma from nonsuppressing normal thymus on...
Nonsuppressing normal thymus on chemical-shift MR imaging and anterior mediastinal lymphoma: differentiation with diffusion-weighted MR imaging by using the apparent diffusion coefficient.
OBJECTIVES
To prospectively evaluate usefulness of the apparent diffusion coefficient (ADC) in differentiating anterior mediastinal lymphoma from nonsuppressing normal thymus on chemical-shift MR, and to look at the relationship between patient age and ADC.
METHODS
Seventy-three young subjects (25 men, 48 women; age range, 9-29 years), who underwent chemical-shift MR and diffusion-weighted MR were divided into a normal thymus group (group A, 40 subjects), and a lymphoma group (group B, 33 patients). For group A, all subjects had normal thymus with no suppression on opposed-phase chemical-shift MR. Two readers measured the signal intensity index (SII) and ADC. Differences in SII and ADC between groups were tested using t-test. ADC was correlated with age using Pearson correlation coefficient.
RESULTS
Mean SII±standard deviation was 2.7±1.8% for group A and 2.2±2.4% for group B, with no significant difference between groups (P=.270). Mean ADC was 2.48±0.38x10mm/s for group A and 1.24±0.23x10mm/s for group B. A significant difference between groups was found (P<.001), with no overlap in range. Lastly, significant correlation was found between age and ADC (r=0.935, P<.001) in group A.
CONCLUSIONS
ADC of diffusion-weighted MR is a noninvasive and accurate parameter for differentiating lymphoma from nonsuppressing thymus on chemical-shift MR in young subjects.
KEY POINTS
• SII cannot differentiate mediastinal lymphoma from nonsuppressing normal thymus at visual assessment • ADC is useful for distinguishing nonsuppressing normal thymus from mediastinal lymphoma • ADC is more accurate than transverse-diameter and surface-area in this discrimination • ADC of normal thymus is age dependent and increases with increasing age.
Topics: Adolescent; Adult; Age Factors; Child; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Female; Humans; Lymphoma; Magnetic Resonance Imaging; Male; Mediastinal Neoplasms; Mediastinum; Prospective Studies; Thymus Gland; Young Adult
PubMed: 29143106
DOI: 10.1007/s00330-017-5142-z -
Indian Journal of Pediatrics Jul 2015Acute respiratory distress is one of the most common reason for emergency visits in children under 5 y of age. An accurate understanding of the epidemiology of these... (Review)
Review
Acute respiratory distress is one of the most common reason for emergency visits in children under 5 y of age. An accurate understanding of the epidemiology of these diseases, identification of risk factors and etiology is critical for successful treatment and prevention of related mortality. The cause of acute respiratory distress varies in etiology, and hence is amenable to different treatment modalities. Depending on the predominant symptoms and signs, a child presenting to the clinician can be divided into six groups, viz., stridor; cough, fever and difficulty in breathing or fast breathing; wheezing; mediastinal shift with severe respiratory distress; slow or irregular breathing in absence of any pulmonary sign; and respiratory distress with cardiac findings. A detailed history followed by a thorough clinical examination and laboratory evaluation assisted by imaging modalities if indicated, helps to establish the exact cause of respiratory distress in the child. Early recognition and prompt institution of appropriate management or referral can significantly improve the outcome of this illness. This article offers clinicians a brief update on the general management guidelines of respiratory distress in pediatric patients. Specific treatment depends on the exact cause, however croup and acute severe asthma have been discussed in this article.
Topics: Adolescent; Airway Obstruction; Asthma; Child; Child, Preschool; Croup; Diagnosis, Differential; Humans; Respiratory Distress Syndrome
PubMed: 25257964
DOI: 10.1007/s12098-014-1559-4 -
Fetal Diagnosis and Therapy 2017To evaluate the efficiency of thoracoamniotic shunts for drainage of macrocystic-type congenital cystic adenomatoid malformation (CCAM). (Review)
Review
OBJECTIVE
To evaluate the efficiency of thoracoamniotic shunts for drainage of macrocystic-type congenital cystic adenomatoid malformation (CCAM).
SUBJECTS AND METHODS
This was a retrospective study of 12 fetuses with a large thoracic cyst treated with thoracoamniotic shunting between 2004 and 2014 in a tertiary fetal therapy center. Medline was searched to identify cases of CCAM treated with thoracoamniotic shunting.
RESULTS
In all cases the thoracic cyst was associated with major mediastinal shift, the CCAM volume ratio (CVR) was >1.6, and in eight cases there was associated hydrops. Shunt insertion was successfully carried out in all cases at a median gestational age of 24 weeks (range 18-34). In 10 cases there was live birth at a median age of 38 weeks (range 35-41), but in two hydropic fetuses there was intrauterine death. A literature search identified a total of 98 fetuses with CCAM treated with thoracoamniotic shunting between 1987 and 2016. In the combined data from the previous and the current study, the survival rate was 77% (53 of 69) for hydropic and 90% (37 of 41) for nonhydropic fetuses.
CONCLUSIONS
The role of thoracoamniotic shunting in macrocystic lung lesions associated with hydrops is well accepted. Intrauterine intervention is also likely to be beneficial in the subgroup of nonhydropic fetuses with a CVR >1.6.
Topics: Catheterization; Cystic Adenomatoid Malformation of Lung, Congenital; Female; Humans; Pregnancy; Retrospective Studies; Thoracotomy; Ultrasonography, Prenatal
PubMed: 27489957
DOI: 10.1159/000448095 -
Endocrine-related Cancer Sep 2016The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of... (Review)
Review
The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of an endocrine tumor associated with the vagus nerve. This rare, neural crest tumor constitutes the second most common site of hereditary head and neck paragangliomas (HNPGLs), most often in relation to mutations in the succinate dehydrogenase complex subunit D (SDHD) gene. The treatment paradigm for VPGL has progressively shifted from surgery to abstention or therapeutic radiation with curative-like outcomes. Parathyroid tissue and parathyroid adenoma can also be found in close association with the vagus nerve in intra or paravagal situations. Vagal parathyroid adenoma can be identified with preoperative imaging or suspected intraoperatively by experienced surgeons. Vagal parathyroid adenomas located in the neck or superior mediastinum can be removed via initial cervicotomy, while those located in the aortopulmonary window require a thoracic approach. This review particularly emphasizes the embryology, molecular genetics, and modern imaging of these tumors.
Topics: Cranial Nerve Neoplasms; Humans; Paraganglioma; Parathyroid Neoplasms; Vagus Nerve; Vagus Nerve Diseases
PubMed: 27406876
DOI: 10.1530/ERC-16-0241 -
Clinical Case Reports Mar 2022Mediastinal shift and chest wall retraction limit the indication of a double lung transplant in patients with complete destruction of lung parenchyma. We report outcomes...
Mediastinal shift and chest wall retraction limit the indication of a double lung transplant in patients with complete destruction of lung parenchyma. We report outcomes as well as preoperative and operative management in two patients with significant lung volume reduction and mediastinal shift due to bronchiectasis of the right lung.
PubMed: 35990927
DOI: 10.1002/ccr3.5515 -
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 -
The Journal of Maternal-fetal &... Oct 2022Congenital-pulmonary-airway-malformation (CPAM) and bronchopulmonary-sequestration (BPS) are rare conditions. The objective of this study was to describe the sonographic...
BACKGROUND
Congenital-pulmonary-airway-malformation (CPAM) and bronchopulmonary-sequestration (BPS) are rare conditions. The objective of this study was to describe the sonographic characteristics and prenatal course of fetal lung mass.
METHODS
A retrospective study on 20 pregnancies with CPAM and BPS diagnosed during 2018-2018 was performed. Data evaluated included gestational age (GA) at diagnosis, size of lesion, CPAM-volume-ratio (CVR), associated sonographic findings and pregnancy outcome.
RESULTS
Fourteen cases of CPAM and six cases of BPS were evaluated. The average GA at diagnosis was 22 weeks'-gestation (range 17-26). 66% were macrocystic-multicystic. Five cases of BPS (71.4%) had other structural anomalies. None of the fetuses with CPAM had additional anomalies. Four pregnancies (20%) underwent termination due to severe hydrops fetalis. Two cases of CPAM (15%) had spontaneous resolution by 30 week's-gestation; three cases (20%) continued to grow during pregnancy and all the rest reached their largest size at 25-28 week's-gestation. Most cases delivered at term (average 37.6 range 32.2-41.4). Two children had lobectomy at 11 and 12 months of life.
CONCLUSIONS
Careful follow-up during pregnancy may result in term delivery of adequate-for-gestational-age newborns, with no need for admission to the NICU, with only 10% need for lobectomy during the first year of life.
Topics: Bronchopulmonary Sequestration; Child; Cystic Adenomatoid Malformation of Lung, Congenital; Female; Humans; Infant, Newborn; Lung; Pregnancy; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 33108914
DOI: 10.1080/14767058.2020.1836149