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1.
Int J Mol Sci ; 22(21)2021 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-34769246

RESUMO

The inflammatory response plays a central role in the complications of congenital pulmonary airway malformations (CPAM) and severe coronavirus disease 2019 (COVID-19). The aim of this study was to evaluate the transcriptional changes induced by SARS-CoV-2 exposure in pediatric MSCs derived from pediatric lung (MSCs-lung) and CPAM tissues (MSCs-CPAM) in order to elucidate potential pathways involved in SARS-CoV-2 infection in a condition of exacerbated inflammatory response. MSCs-lung and MSCs-CPAM do not express angiotensin-converting enzyme 2 (ACE2) and transmembrane serine protease 2 (TRMPSS2). SARS-CoV-2 appears to be unable to replicate in MSCs-CPAM and MSCs-lung. MSCs-lung and MSCs-CPAM maintained the expression of stemness markers MSCs-lung show an inflammatory response (IL6, IL1B, CXCL8, and CXCL10), and the activation of Notch3 non-canonical pathway; this route appears silent in MSCs-CPAM, and cytokine genes expression is reduced. Decreased value of p21 in MSCs-lung suggested no cell cycle block, and cells did not undergo apoptosis. MSCs-lung appears to increase genes associated with immunomodulatory function but could contribute to inflammation, while MSCs-CPAM keeps stable or reduce the immunomodulatory receptors expression, but they also reduce their cytokines expression. These data indicated that, independently from their perilesional or cystic origin, the MSCs populations already present in a patient affected with CPAM are not permissive for SARS-CoV-2 entry, and they will not spread the disease in case of infection. Moreover, these MSCs will not undergo apoptosis when they come in contact with SARS-CoV-2; on the contrary, they maintain their staminality profile.


Assuntos
Células-Tronco Mesenquimais/metabolismo , Anormalidades do Sistema Respiratório , SARS-CoV-2/fisiologia , Transcriptoma , COVID-19/genética , COVID-19/metabolismo , COVID-19/patologia , Estudos de Casos e Controles , Células Cultivadas , Perfilação da Expressão Gênica , Interações Hospedeiro-Patógeno/genética , Humanos , Lactente , Pulmão/anormalidades , Pulmão/metabolismo , Pulmão/patologia , Masculino , Células-Tronco Mesenquimais/patologia , Células-Tronco Mesenquimais/virologia , RNA-Seq , Anormalidades do Sistema Respiratório/genética , Anormalidades do Sistema Respiratório/patologia , Anormalidades do Sistema Respiratório/virologia
2.
Pediatr Surg Int ; 37(9): 1273-1280, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34213588

RESUMO

PURPOSE: To examine the association between the MSCT quantitative measurements of congenital lung malformations (CLM) and the selection of surgical approaches (lobectomy vs. lung-sparing surgery). METHODS: This retrospective study evaluated CLM surgical cases at our institution from 2016 to 2018. MSCT quantitative measurements were generated by a semi-automated approach: the volume of the lesion (Vlesion), the volume of the lesion-involved lobe (Vlobe), the volume of the lesion-involved lung (Vlung) and the volume of the total lung (Vtotal lung). The proportions of Vlesion to Vlobe (Plesion/lobe), Vlesion to Vlung (Plesion/lung), and Vlesion to V total lung (Plesion/total lung) were calculated. We used Logistics regression to examine whether quantitative measurements were associated with the selection of surgical approaches. RESULTS: 151 patients were included (median age at surgery 6 months). 82 patients underwent lung-sparing surgery, and 69 patients underwent lobectomy. Vlesion (OR 1.51, 95% CI 1.09-2.07), Plesion/lobe (OR 1.78, 95% CI 1.16-2.72), Plesion/lung (OR 1.63, 95% CI 1.13-2.35), and Plesion/total lung (OR 1.58, 95% CI 1.12-2.22) were positively associated with the selection of lobectomy. CONCLUSION: The application of quantified MSCT analysis may provide insight into the quantitative characteristics of CLM, which could be potentially useful for surgical approach selection.


Assuntos
Pneumonectomia , Anormalidades do Sistema Respiratório , Computadores , Humanos , Pulmão/diagnóstico por imagem , Pulmão/cirurgia , Anormalidades do Sistema Respiratório/cirurgia , Estudos Retrospectivos
3.
Pediatr Pulmonol ; 56(9): 2903-2910, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34196512

RESUMO

PURPOSE: To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven combined congenital lung lesion consisting of bronchial atresia (BA) and congenital pulmonary airway malformation (CPAM) in children. MATERIALS AND METHODS: All pediatric patients (age ≤ 18 years) with a known pathological diagnosis of a combined BA-CPAM congenital lung lesion, who underwent thoracic MDCT studies from January 2011 to January 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung, including nodule, mass, cyst, ground-glass opacity, and consolidation. When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), borders (well-circumscribed vs. ill-defined), contrast enhancement pattern (nonenhancement vs. enhancement), and location (laterality, and lobar distribution) were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics. RESULTS: Eighteen contrast-enhanced thoracic MDCT studies from 18 individual pediatric patients (8 males (44%) and 10 females (56%); mean age: 4.9 months; SD: 2.6; range: 1-10 months) with a pathological diagnosis of combined BA-CPAM congenital lung lesion comprised the final study population. The most frequent MDCT finding of combined BA-CPAM congenital lung lesion in children was a solitary (18/18; 100%), well-circumscribed (18/18; 100%), both solid and cystic (17/18; 94%) lesion with nonenhancing (17/17; 100%) nodule, reflecting the underlying BA component, adjacent to a well-circumscribed multicystic mass (18/18; 100%), representing the underlying CPAM component. This combined congenital lung lesion occurred in all lobes with similar frequency. There was almost perfect interobserver κ agreement between the two independent reviewers for detecting abnormalities on thoracic MDCT studies (k = 0.98). CONCLUSION: The characteristic thoracic MDCT findings of a combined BA-CPAM congenital lung lesion are a solitary, well-circumscribed solid and multicystic mass, with a nonenhancing nodule, reflecting the BA component, adjacent to a cystic mass, representing the CPAM component. Accurate recognition of these characteristic MDCT findings of combined BA-CPAM congenital lung lesion has great potential to help differentiate this combined congenital lung lesion from other thoracic pathology in children.


Assuntos
Broncopatias , Malformação Adenomatoide Cística Congênita do Pulmão , Anormalidades do Sistema Respiratório , Adolescente , Criança , Feminino , Humanos , Lactente , Pulmão/diagnóstico por imagem , Masculino , Tomografia Computadorizada Multidetectores , Anormalidades do Sistema Respiratório/diagnóstico por imagem
4.
Eur J Pediatr ; 180(8): 2687-2691, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34086104

RESUMO

An azygos lobe is a rare anatomic variant of the lung which may be misdiagnosed as other pathological conditions. There is a dearth of information on the clinical characteristics of children with azygos lobe. This study aims to summarize the clinical features of children with azygos lobe, which may be helpful to improve recognition and clinical care of those patients. Clinical findings of the children with imaging findings of azygos lobe were collected and analyzed. In this study, 50 children with azygos lobe were included. A total of 28% cases were found to have azygos lobe incidentally. The remaining 72% cases were diagnosed when they presented with respiratory symptoms including cough (36%), fever (34%), wheezing (18%), dyspnea (14%), and cyanosis (8%). Congenital heart disease, Down syndrome, and other respiratory malformations were also found in a small proportion of these patients. Only 7 (14%) patients suffered from azygos lobe infection and were clinically cured after reasonable anti-infective treatment. Compared with the non-infected group, no valuable risk factors were found to be related to azygos lobe infection. All children who had azygos lobe infections responded to appropriate antibiotics therapy. There was no evidence that an azygos lobe was associated with recurrent infections.Conclusion: The clinical characteristics of children with azygos lobe are nonspecific and diagnosis relies on chest imaging. Some congenital abnormalities may be complicated with azygos lobe. It is reasonable to keep watch over those patients without symptoms. What is Known: • An azygos lobe is a congenital variation of the lung. • Azygos lobe can mimic various pathological conditions leading to misdiagnosis and inappropriate treatment in adults, which presents challenges during thoracic surgical interventions. What is New: • Azygos lobe in children may be complicated with other congenital abnormalities. • There is no evidence that azygos lobe is associated with recurrent infections. • After appropriate anti-infective treatment, those children with azygos lobe infections could be clinically cured.


Assuntos
Pneumopatias , Anormalidades do Sistema Respiratório , Adulto , Veia Ázigos/diagnóstico por imagem , Criança , Humanos , Pulmão/diagnóstico por imagem , Estudos Retrospectivos
5.
Int J Pediatr Otorhinolaryngol ; 146: 110762, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33992970

RESUMO

BACKGROUND: Microtia is a congenital malformation of the external ear often with one or more associated congenital anomalies. The purpose of this study was to identify the characteristics and prevalence of respiratory anomalies in patients with microtia, and clarify the importance of this association in the perioperative period of patients' external ear reconstruction surgery. METHODS: Data were collected from 923 microtia patients between August 2017 and December 2020 in the Department of Auricular Reconstruction at the Plastic Surgery Hospital of Peking Union Medical College. Co-occurring respiratory anomalies were detected using chest computed tomography plus three-dimensional reconstruction and Chest X-ray. Physical examination was performed to assess the severity and type of microtia by trained clinicians. Fisher's exact test was used to analyze the relation between laterality of pulmonary underdevelopment and microtia type. RESULTS: Among the 923 participants enrolled in the study, we identified 21 cases (2.3%) having respiratory system anomalies, consisting of 6 cases with pulmonary underdevelopment (28.6% of all anomalies of respiratory system detected), 2 cases with tracheal bronchus (9.5%), 1 case with tracheal diverticula (4.8%), 11 cases with lung bullae(52.4%), and 1 case with pulmonary azygos lobe (4.8%). The laterality of pulmonary underdevelopment was related to the type of microtia (difference between types, p < 0.05), as patients with concha-type remnant ear had pulmonary underdevelopment ipsilaterally. CONCLUSIONS: This study represents the first detailed and thematic study of a association featured by microtia and respiratory anomalies. Characteristics and prevalence of respiratory anomalies was observed in a Chinese clinical microtia population. Early diagnosis of associated respiratory malformations had practical clinical significance for microtia patients, plastic surgeons and anesthesiologists. Future studies are required to improve understanding of this association and its cause.


Assuntos
Microtia Congênita , Anormalidades do Sistema Respiratório , Cirurgia Plástica , China/epidemiologia , Microtia Congênita/epidemiologia , Orelha Externa , Humanos , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Anormalidades do Sistema Respiratório/epidemiologia
6.
Pediatr Radiol ; 51(11): 2027-2037, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33988754

RESUMO

BACKGROUND: Tracheal atresia causes some secondary changes (dilated trachea, flattened/inverted diaphragm, hyperintense and hyperinflated lungs). They can be reduced if a high airway fistula is present. OBJECTIVE: This study evaluated fetal MR images of tracheal atresia and the secondary changes, focusing on the presence of a fistula. MATERIALS AND METHODS: We assessed fetal MR images of tracheal atresia without fistula (n=4, median 26 weeks), tracheal atresia with fistula (n=4, median 33 weeks) and controls (n=30, median 32 weeks). We evaluated airway obstruction using true-positive rate in tracheal atresia and false-positive rate in controls indicating they are likely normal variants. Tracheal diameter, craniocaudal-anteroposterior ratio of the right hemidiaphragm, lung-to-liver signal intensity ratio, and cardiothoracic ratio were compared among the three groups using the Kruskal-Wallis test followed by pairwise comparison using the Mann-Whitney U test. RESULTS: True-positive rate was 100% in tracheal atresia, while false-positive rate was 20% in controls. The Kruskal-Wallis test showed differences among groups in craniocaudal-anteroposterior ratio and cardiothoracic ratio (P<0.001) but not in tracheal diameter (P=0.256) or lung-to-liver signal intensity ratio (P=0.082). The pairwise comparison in craniocaudal-anteroposterior ratio and cardiothoracic ratio showed differences between controls and tracheal atresia without fistula (P<0.01) and with fistula (P<0.05). CONCLUSION: Fetal MRI is useful for the diagnosis of tracheal atresia, and detection of airway obstruction is essential. Lower craniocaudal-anteroposterior ratio and cardiothoracic ratio might be reliable measures even if a fistula is present.


Assuntos
Obstrução das Vias Respiratórias , Atresia Esofágica , Anormalidades do Sistema Respiratório , Fístula Traqueoesofágica , Humanos , Imageamento por Ressonância Magnética , Traqueia/diagnóstico por imagem
8.
Pediatr Pulmonol ; 56(7): 2164-2168, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33760393

RESUMO

INTRODUCTION: Congenital bronchial atresia (CBA) is a rare airway malformation. No management guidelines exist because of limited evidence: treatment, surgical or conservative, is based on consensus and opinion. OBJECTIVE: To review the experience of a pediatric tertiary center and provide additional data about nonsurgical management of CBA and its outcomes after a structured follow-up, and to help formulate appropriate evidence-based guidelines. METHODS: A retrospective analysis of clinical, radiological, surgical, and pathological data of all pediatric patients with suspicion of CBA referred to the surgical department of the Bambino Gesù children's hospital of Rome between December 2013 and 2019, along with a review of the literature. RESULTS: Among the 18 children initially included in the study, 2 were lost to follow-up after radiological diagnosis, 4 underwent surgery for radiological suspicion of other pulmonary malformations. The final population is composed of 12 conservatively managed patients. At the end of the follow-up (median: 29 months, range 3-61), 1 patient (8%) was symptomatic. CONCLUSION: Conservative management for CBA appears to be safe. Surgery should be reserved for patients with symptomatic or complicated cases.


Assuntos
Broncopatias , Anormalidades do Sistema Respiratório , Criança , Tratamento Conservador , Hospitais Pediátricos , Humanos , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Anormalidades do Sistema Respiratório/cirurgia , Estudos Retrospectivos
9.
BMJ Case Rep ; 14(2)2021 Feb 22.
Artigo em Inglês | MEDLINE | ID: mdl-33619150

RESUMO

We reported here a boy aged 5 years who presented for the evaluation of recurrent croup since infancy. On chest examination, breath sounds were reduced throughout the right lung field with a shifting of the trachea and cardiac apex to the right side. The chest radiograph showed a small right lung with decreased vascularity, hyperinflated left lung and mediastinum shifted towards the right side. Flexible bronchoscopy revealed tracheomalacia with left bronchomalacia due to external pulsatile compression. In CT angiogram, the right pulmonary artery (PA) was absent with dilated left PA. Echocardiography did not show any features of pulmonary arterial hypertension (PAH). Since the child was growing well, and there was no limitation of activity and evidence of PAH, he was managed conservatively and kept on follow-up. Though unilateral absent PA is a rare condition, it should be suspected in children with unilateral hypoplastic lung.


Assuntos
Broncomalácia , Crupe , Anormalidades do Sistema Respiratório , Broncoscopia , Criança , Pré-Escolar , Crupe/diagnóstico , Humanos , Masculino , Artéria Pulmonar
10.
Eur Arch Otorhinolaryngol ; 278(10): 3605-3611, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33388986

RESUMO

PURPOSE: Congenital nasal obstruction can be a significant cause of respiratory distress in the newborn, given that they are considered to be obligate nasal breathers. Several different causes have been described, which can be broadly classified as anatomical/malformative, non-tumoral masses and cysts, benign and malignant neoplasia, inflammatory/infectious, traumatic/iatrogenic, and miscellaneous. The purpose of this review is to provide updated and useful clinical information for teams involved in neonatal care, especially in a hospital setting. METHODS: A review of the available literature was performed. Studies were sourced from PubMed with searching of relevant headings and sub-headings and cross-referencing. RESULTS: The most common etiology is inflammatory, which can have different precipitating factors or be idiopathic, a condition known as neonatal rhinitis. On the other hand, some less frequent but nonetheless relevant conditions causing severe nasal obstruction include choanal atresia, midnasal stenosis, and pyriform aperture stenosis. Some cystic lesions, such as dacryocystoceles with intranasal mucocele, can also produce significant obstruction. Diagnosis usually requires a nasal endoscopy and in some cases imaging such as computed tomography. Management includes different medical and surgical strategies and will greatly depend on the etiology and the severity of symptoms. CONCLUSION: Congenital nasal obstruction can be a significant cause of respiratory distress in the newborn. The wide spectrum of differential diagnoses requires a thorough knowledge of nasal anatomy, physiology, and pathology; as well as different management strategies.


Assuntos
Atresia das Cóanas , Obstrução Nasal , Anormalidades do Sistema Respiratório , Rinite , Humanos , Recém-Nascido , Obstrução Nasal/diagnóstico , Obstrução Nasal/etiologia , Nariz
11.
BMC Pulm Med ; 21(1): 2, 2021 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407289

RESUMO

BACKGROUND: Congenital bronchial atresia is a rare pulmonary abnormality characterized by the disrupted communication between the central and the peripheral bronchus and is typically asymptomatic. Although it can be symptomatic especially when infections occur in the involved areas, fungal infections are rare complications in patients with bronchial atresia. We report a case of congenital bronchial atresia complicated by a fungal infection. CASE PRESENTATION: A 30-year-old man with no previous history of immune dysfunction was brought to a nearby hospital and diagnosed with a left lung abscess. Although antimicrobial treatment was administered, it was ineffective, and he was transferred to our hospital. Since diagnostic imaging findings and bronchoscopy suggested congenital bronchial atresia and a fungal infection, he was treated with voriconazole and surgical resection was subsequently performed. A tissue culture detected Aspergillus fumigatus and histopathological findings were compatible with bronchial atresia. After discharge, he remained well and voriconazole was discontinued 5 months after the initiation of therapy. CONCLUSION: Bronchial atresia is a rare disease that is seldom complicated by a fungal infection, which is also a rare complication; however, physicians should consider fungal infections in patients with bronchial atresia who present with infections resistant to antimicrobial treatment.


Assuntos
Aspergilose/microbiologia , Aspergillus fumigatus/isolamento & purificação , Brônquios/anormalidades , Abscesso Pulmonar/microbiologia , Anormalidades do Sistema Respiratório/complicações , Adulto , Aspergilose/patologia , Aspergilose/terapia , Brônquios/cirurgia , Broncoscopia , Humanos , Abscesso Pulmonar/patologia , Abscesso Pulmonar/cirurgia , Masculino , Radiografia Torácica , Anormalidades do Sistema Respiratório/diagnóstico , Tomografia Computadorizada por Raios X , Voriconazol/uso terapêutico
12.
J Thorac Cardiovasc Surg ; 161(3): 1112-1121.e7, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33419543

RESUMO

OBJECTIVES: Airway anomalies are common in children with cardiac disease but with an unquantified impact on outcomes. We sought to define the association between airway anomalies and tracheal surgery with cardiac surgery outcomes using the Society of Thoracic Surgery Congenital Heart Surgery Database. METHODS: Index cardiac operations in children aged less than 18 years (January 2010 to September 2018) were identified from the Society of Thoracic Surgery Congenital Heart Surgery Database. Patients were divided on the basis of reported diagnosis of an airway anomaly and subdivided on the basis of tracheal lesion and tracheal surgery. Multivariable analysis evaluated associations between airway disease and outcomes controlling for covariates from the Society of Thoracic Surgery Congenital Heart Surgery Database Mortality Risk Model. RESULTS: Of 198,674 index cardiovascular operations, 6861 (3.4%) were performed in patients with airway anomalies, including 428 patients (0.2%) who also underwent tracheal operations during the same hospitalization. Patients with airway anomalies underwent more complex cardiac operations (45% vs 36% Society of Thoracic Surgeons/European Association for Cardiothoracic Surgery Congenital Heart Surgery Mortality category ≥3 procedures) and had a higher prevalence of preoperative risk factors (73% vs 39%; both P < .001). In multivariable analysis, patients with airway anomalies had increased odds of major morbidity and tracheostomy (P < .001). Operative mortality was also increased in patients with airway anomalies, except those with malacia. Tracheal surgery within the same hospitalization increased the odds of operative mortality (adjusted odds ratio, 3.9; P < .0001), major morbidity (adjusted odds ratio, 3.7; P < .0001), and tracheostomy (adjusted odds ratio, 16.7; P < .0001). CONCLUSIONS: Patients undergoing cardiac surgery and tracheal surgery are at significantly higher risk of morbidity and mortality than patients receiving cardiac surgery alone. Most of those with unoperated airway anomalies have higher morbidity and mortality, which makes it an important preoperative consideration.


Assuntos
Procedimentos Cirúrgicos Cardíacos/mortalidade , Cardiopatias Congênitas/cirurgia , Anormalidades do Sistema Respiratório/cirurgia , Procedimentos Cirúrgicos Torácicos/mortalidade , Traqueia/cirurgia , Adolescente , Fatores Etários , Canadá , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Humanos , Lactente , Masculino , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Anormalidades do Sistema Respiratório/mortalidade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Procedimentos Cirúrgicos Torácicos/efeitos adversos , Fatores de Tempo , Traqueia/anormalidades , Traqueia/diagnóstico por imagem , Resultado do Tratamento , Estados Unidos
13.
J Pediatr Surg ; 56(4): 788-794, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33012559

RESUMO

BACKGROUND: The purpose of this review is to provide ECMO outcome data for medical personnel who counsel families of patients with pulmonary hypoplasia (PH), often secondary to renal abnormalities. We report diagnoses and outcomes associated with PH in neonates that were treated with ECMO over the past 35 years. METHODS: Retrospective cohort study using the ELSO database for neonates born between 1981 and 2016 with a primary or secondary diagnosis of PH. Five patient groups were created based on ICD-9 codes. Mortality rates were compared and trends over time were investigated. RESULTS: Thirty-three percent of the 1385 patients survived to discharge. Congenital diaphragmatic hernia (CDH) patients had significantly higher mortality than PH patients secondary to renal dysplasia (p < 0.001). Mortality decreased significantly over time for all groups (p < 0.001). The proportion of patients alive at discharge increased over time for CDH patients (p < 0.001), whereas survival decreased for patients with PH secondary to renal dysplasia (p = 0.012). CONCLUSIONS: Neonates with PH that require ECMO have high mortality rates, which have generally decreased over the past 35 years; however, mortality for neonates with PH secondary to renal dysplasia continues to increase. We speculate that the apparent rise in mortality for these patients is because of changes in patient selection subsequent to improvements in non-ECMO ventilatory support. LEVEL OF EVIDENCE: II.


Assuntos
Oxigenação por Membrana Extracorpórea , Hérnias Diafragmáticas Congênitas , Anormalidades do Sistema Respiratório , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Recém-Nascido , Estudos Retrospectivos
14.
Ann Thorac Surg ; 112(1): 315-325, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33159867

RESUMO

BACKGROUND: The aim of this study was to determine the prevalence and anatomic features of major tracheobronchial anomalies. METHODS: Major electronic databases were systematically searched to identify eligible studies. Data were extracted and pooled into a meta-analysis. The primary outcome was the prevalence of major tracheobronchial anomalies, specifically tracheal bronchus (TB) and accessory cardiac bronchus (ACB). Secondary outcomes included the origin and types of TB and ACB. RESULTS: A total of 27 studies (n = 119,695 patients) were included. A TB was present in 0.99% (95% confidence interval, 0.67 to 1.37) of patients, and an ACB was present in 0.14% (95% confidence interval, 0.09 to 0.20). The overall prevalence of TB was higher in imaging than in operative studies (1.81% vs 0.82%). It was also higher in pediatric (2.55%) than in adult studies (0.50%). Patients with other congenital anomalies were 15 times more likely to have a TB (odds ratio 14.89; 95% confidence interval, 7.09 to 31.22). The most common origin of TBs was from the trachea (81.42%), primarily from the right side (96.43%). The most common origin of ACBs was from the intermediate bronchus (74.32%). The ACBs terminated as blind-ending diverticulum in two thirds of cases. CONCLUSIONS: Major tracheobronchial anomalies are present in approximately 1% of the population, although the prevalence is higher among pediatric patients and patients with accompanying congenital anomalies. Although rare, major tracheobronchial anomalies can be associated with significant respiratory morbidities and present challenges during airway management in surgical and critical care patients. Establishing a preoperative diagnosis of these variations is essential for planning and implementing an appropriate airway management strategy to minimize attendant complications.


Assuntos
Brônquios/anormalidades , Traqueia/anormalidades , Adulto , Brônquios/anatomia & histologia , Criança , Humanos , Prevalência , Anormalidades do Sistema Respiratório/epidemiologia
15.
Asian J Endosc Surg ; 14(1): 109-111, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32495500

RESUMO

Bronchial abnormalities are rare, and they are infrequently associated with pulmonary vascular abnormalities. It is important to identify such abnormal anatomical structures before lobectomy of the lung under thoracoscopy. There have been only three reports on video-assisted thoracoscopic lobectomy in patients with anomalous bronchi and vascular abnormalities to date. Here, we present a report of video-assisted thoracoscopic right upper lobectomy performed in a patient with bronchial and vascular abnormalities. Both preoperative imaging and intraoperative findings revealed a displaced anomalous B2 bronchus arising from the bronchus intermedius, as well as abnormal distribution of the aberrant vein V2 draining into vein V6 . It is critical to understand the precise anatomical structures preoperatively to perform video-assisted thoracoscopic pulmonary lobectomy safely.


Assuntos
Adenocarcinoma de Pulmão/cirurgia , Neoplasias Pulmonares/cirurgia , Pneumonectomia/métodos , Anormalidades do Sistema Respiratório/cirurgia , Cirurgia Torácica Vídeoassistida , Malformações Vasculares/cirurgia , Adenocarcinoma de Pulmão/diagnóstico por imagem , Idoso , Brônquios/anormalidades , Brônquios/diagnóstico por imagem , Brônquios/cirurgia , Feminino , Humanos , Pulmão/anormalidades , Pulmão/irrigação sanguínea , Pulmão/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagem , Malformações Vasculares/diagnóstico por imagem
17.
Pediatr Pulmonol ; 56(3): 678-685, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-32735399

RESUMO

Pulmonary growth abnormality (PGA) is a common type of diffuse lung disease in infants. Although the histologic and radiographic features of PGA have been described in the literature in varying detail, the clinical spectrum of disease has not. The array of case series and case reports has led to a clinical picture that could be confusing to clinicians. We describe three subsets of PGA, including its association with the histologic marker of pulmonary interstitial glycogenosis, and its common association with pulmonary hypertension. We propose a new approach to what we consider an increasingly broad array of different disease entities.


Assuntos
Displasia Broncopulmonar/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Doenças Pulmonares Intersticiais/diagnóstico , Pulmão/anormalidades , Mutação , Criança , Pré-Escolar , Feminino , Filaminas/genética , Doença de Depósito de Glicogênio/complicações , Humanos , Hipertensão Pulmonar/complicações , Lactente , Recém-Nascido , Pulmão/patologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Alvéolos Pulmonares/patologia , Anormalidades do Sistema Respiratório/complicações , Tomografia Computadorizada por Raios X
18.
Cleft Palate Craniofac J ; 58(5): 628-636, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33054357

RESUMO

INTRODUCTION: Nasal duplication is a rare congenital deformity with many subtypes including supernumerary nostril. The challenge of surgical correction is to achieve nasal symmetry and restore nasal airflow. However, there is no defined protocol for treatment, especially with regard to presurgical therapy. METHODS: We performed a review of literature of studies reporting on patients with supernumerary nostril to complete this review. We then report on a patient with supernumerary nostril who was treated with nostril expansion therapy prior to surgical intervention. RESULTS: We identified 59 cases of nostril duplication. Because of the rarity of the condition, treatment protocols varied greatly. For our patient, preoperative nasal appliance therapy was implemented for 3 months prior to surgical intervention. Patient was followed-up regularly for 1 year. DISCUSSION: Although literature on nasal duplication is scarce, there is a general agreement that early intervention has psychological, anatomic, and functional benefits to the patient. In our case report, nostril expansion therapy was easy to implement and facilitated surgical reconstruction, resulting in aesthetic outcome and expanded airway 1 year postoperatively.


Assuntos
Anormalidades do Sistema Respiratório , Rinoplastia , Estética Dentária , Humanos , Cavidade Nasal/cirurgia , Nariz/diagnóstico por imagem , Nariz/cirurgia
19.
Pediatr Clin North Am ; 68(1): 1-24, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33228926

RESUMO

Congenital bronchopulmonary malformations are relatively common and arise during various periods of morphogenesis. Although some are isolated or sporadic occurrences, others may result from single gene mutations or cytogenetic imbalances. Single gene mutations have been identified, which are etiologically related to primary pulmonary hypoplasia, lung segmentation defects as well as pulmonary vascular and lymphatic lesions. Functional defects in cystic fibrosis, primary ciliary dyskinesias, alpha-1-antitrypsin deficiency, and surfactant proteins caused by gene mutations may result in progressive pulmonary disease. This article provides an overview of pediatric pulmonary disease from a genetic perspective.


Assuntos
Pneumopatias/congênito , Pneumopatias/genética , Anormalidades do Sistema Respiratório/genética , Criança , Pré-Escolar , Doenças do Tecido Conjuntivo/congênito , Doenças do Tecido Conjuntivo/genética , Humanos , Lactente , Recém-Nascido , Erros Inatos do Metabolismo/genética
20.
Neumol. pediátr. (En línea) ; 16(1): 41-47, 2021. tab, ilus
Artigo em Espanhol | LILACS | ID: biblio-1284223

RESUMO

Congenital pulmonary airway malformations (CPAM) are rare and occur due to a failure in lung embryological development. They are classified according to their pathological characteristics and their anatomical origin. They can occur from the antenatal period to adulthood, can be associated with hydrops fetalis, respiratory distress, recurrent infections, or in an otherwise asymptomatic patient. In this article we carry out a scope review of the literature to answer frequent questions of the clinical teams in charge of patients with CPAM, such as the antenatal approach, the different types of surgeries and the management of asymptomatic patients. Although the indication for surgery is clear in symptomatic patients, little is known about its natural history of this condition, including the possibility of spontaneous resolution and the development of complications or neoplasm. So, the treatment of asymptomatic patients continues to be controversial. Therapeutic decisions must be made by multidisciplinary teams with the informed participation of parents and patients. In our opinion, considering the excellent results of minimally invasive surgery, its low incidence of complications, and practically zero mortality when performed by experienced groups, it seems reasonable to consider elective resection of a MCVAP in asymptomatic patients.


Las malformaciones congénitas de la vía aérea pulmonar (MCVAP) son infrecuentes y ocurren debido a una falla en el desarrollo embriológico pulmonar. Se clasifican de acuerdo con sus características patológicas y a su origen anatómico. Se pueden presentar desde el periodo antenatal hasta la adultez, asociarse a cuadros de hidrops fetal, distrés respiratorio, infecciones recurrentes, o como un hallazgo en pacientes asintomáticos. En este artículo realizamos una revisión bibliográfica exploratoria para responder dudas frecuentes de los equipos clínicos a cargo de pacientes con MCVAP, como el enfrentamiento antenatal, los distintos tipos de cirugía y su abordaje, y el manejo de pacientes asintomáticos. Si bien la indicación de cirugía es clara en pacientes sintomáticos, poco se conoce acerca de su historia natural, incluyendo la posibilidad de resolverse de forma espontánea, de complicarse o de evolucionar hacia el desarrollo de una neoplasia, por lo que el tratamiento de pacientes asintomáticos continúa siendo controversial. Las decisiones terapéuticas deben ser tomadas por equipos multidisciplinarios con la participación informada de los padres y de los pacientes. En nuestra opinión, considerando los excelentes resultados de la cirugía mínimamente invasiva, su baja incidencia de complicaciones y prácticamente nula mortalidad, al ser realizada por grupos con experiencia, nos parece razonable plantear la resección electiva de una MCVAP en un paciente asintomático.


Assuntos
Humanos , Criança , Anormalidades do Sistema Respiratório/cirurgia , Pneumonectomia , Cuidado Pré-Natal , Imageamento por Ressonância Magnética , Toracotomia , Radiografia Torácica , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Anormalidades do Sistema Respiratório/classificação , Anormalidades do Sistema Respiratório/embriologia , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Risco , Pulmão/anormalidades
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