Surgical Management Of Congenital Thoracic Disorders: A 15-Year Center Experience.

INTRODUCTION: Congenital thoracic disorders represent a spectrum of fetal lung bud development abnormalities, which may affect breathing capacity and quality of life. We aim to evaluate the impact of surgery in the treatment of 4 major congenital conditions. MATERIALS AND METHODS: We performed a retrospective cohort analysis of patients who underwent surgical treatment in our tertiary center, from 2007 to 2022. RESULTS: Over the 15-year period, we treated 33 patients, with a male predominance of 55%. 22 patients (67%) were asymptomatic. When symptomatic, the recurrence of respiratory infections was the most common clinical presentation (18%). In 13 patients (39%), diagnosis was achieved through fetal ultrasonography. This study encompassed 13 patients with pulmonary sequestration (39%), 11 patients with bronchogenic cysts (33%), 7 patients with congenital pulmonary airway malformation (21%) and 2 patients with congenital lobar emphysema (6%). Considering solely lung malformation conditions, we accounted 22 patients with a median age of 3 [1-67] years-old. Surgery comprised bilobectomy (9%), lobectomy (77%), lobectomy with wedge resection (5%), segmentectomy (5%) and wedge resection (5%). Concerning bronchogenic cysts, we treated 11 patients with a median age of 19 [14-66] years-old. We identified 1 hilar, 1 intrapulmonary and 9 mediastinal lesions, of which 4 were paraesophageal, 4 were subcarinal and 1 was miscellaneous. Overall, surgery was conducted by thoracotomy in 61% of patients, VATS in 33% and RATS in 6%. The median drainage time was 3 [1-40] days and median hospital stay was 4 [1-41] days. There were no cases of mortality. Ensuing, 94% of patients experienced clinical improvement after surgery. CONCLUSION: Early diagnosis of congenital thoracic malformations increased considerably with the improvement in imaging technology and prenatal screening. Treatment may include expectant conservative treatment. However, in selected cases, surgery may play an important role in symptomatic control and prevention of disease progression.

The ten-year evaluation of clinical characteristics in congenital lung anomaly in pediatrics; a retrospective study in North of Iran.

INTRODUCTION: Congenital lung anomalies (CLA) are a group of anomalies, including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestrations (BPS), congenital lobar emphysema (CLE), and bronchogenic cysts (BC). The prevalence of these rare anomalies has risen in recent years, according to various population-based studies due to advances in fetal ultrasound technology. METHOD: This retrospective study examines the diagnosis of CLA, and was conducted on 72 patients between March 2014 and March 2024 at Taleghani Pediatric Hospital in Gorgan, Iran. RESULT: The average age was 18.8 ± 30.3 months, with the majority being boys (62.5%). Most participants had CCAM (41.7%), followed by CLE (18.1%), BPS (16.7%), pulmonary hypoplasia (9.7%), BC (8.3%), and hybrid lesion (5.6%). The majority of patients were Fars (62.5%), and the average hospitalization days was 9.4 ± 4.5 days. Cardiac anomalies were observed in 19.4% of the patients. 62 patients (86.1%) exhibited respiratory symptoms, and prenatal screening during pregnancy led to the diagnosis in 51 patients (70.8%). Most patients had left lung anomalies (43; 59.7%), and the majority (90.3%) survived. There is a statistically significant relation between needed for surgical treatment and patients' type of pulmonary lesions (p-value: 0.02). In addition, there was a significant relation between the Fars ethnicity and the presence of cardiac anomalies (p-value: 0.04). CONCLUSION: Some CLAs remain undiagnosed or untreated due to the rare nature of congenital lung anomalies. Nevertheless, improvements in ultrasound and other imaging methods will make diagnosing and managing these anomalies during the prenatal period more prevalent, resulting in enhanced understanding.

Congenital Rare Abnormal Shapes of Lungs in Relation to Abnormal Lobes and Fissures and Its Clinical Implications.

BACKGROUND: Detailed anatomical knowledge of congenital abnormalities of the lungs is essential for proper evaluation and treatment of respiratory diseases, identification of bronchopulmonary segments during lobectomies and surgical resection, and identification and interpretation of their variable radiological imaging. AIM: Accordingly, the aim of this study was designed to evaluate the prevalence of congenital abnormal shapes of lungs in relation to abnormal lobes and fissures. MATERIALS AND METHODS: This study was carried out on 270 isolated lung specimens (right lungs - 135 and left lungs - 135) from 135 formalin-embalmed middle-aged human cadavers irrespective of gender. RESULTS: Out of 270 lung specimens following congenital abnormalities were reported - (1) three lungs (1.1%) had uunusual different abnormal shapes, such as typical snail shell-shaped right lung with single abnormal circular fissure, bearded axe-shaped left lung with huge cardiac notch, and single-lobed classical pointed hat-shaped right lung, (2) one right lung (0.37%) and 9 left lungs (6.67%) showed complete absence of fissures, (3) among 9 single-lobed left lungs, one had split apex separated by a left subclavian groove with the absence of lingula and cardiac notch, (4) cardiac notch and ligula were noted absently in 3 left lungs (2.36%), and (5) one right lung (0.37%) showed five lobes with complete one oblique and three incomplete horizontal fissures. CONCLUSION: To the best of our knowledge, such abnormalities of the lungs have not been cited in the recent medical literature. Awareness of these abnormalities is necessary to avoid complications during various radiodiagnostic procedures or cardiothoracic surgeries.

Résumé Contexte:Une connaissance anatomique détaillée des anomalies congénitales des poumons est essentielle pour une évaluation et un traitement appropriés des maladies respiratoires, l'identification des segments broncho-pulmonaires lors des lobectomies et de la résection chirurgicale, l'identification et l'interprétation de leur imagerie radiologique variable.Objectif:En conséquence, l'objectif de cette étude a été conçu pour évaluer la prévalence des formes anormales congénitales des poumons en relation avec les lobes et les fissures anormaux.Matériels et méthodes:cette étude a été réalisée sur 270 échantillons pulmonaires isolés (poumons droits - 135 et poumons gauches - 135) provenant de 135 cadavres humains d'âge moyen embaumés au formol, quel que soit leur sexe.Résultats:Sur 270 échantillons pulmonaires suite à des anomalies congénitales, 1. Trois poumons (1,1 %) présentaient des formes anormales différentes et inhabituelles, telles qu'un poumon droit typique en forme de coquille d'escargot avec une seule fissure circulaire anormale, un poumon gauche en forme de hache barbu avec une énorme encoche et chapeau pointu classique unilobé en forme de poumon droit. 2. Un poumon droit (0,37 %) et 9 poumons gauches (6,67 %) ont montré une absence totale de fissures. 3. Parmi 9 poumons gauches monolobés, un avait un sommet fendu séparé par un sillon sous-clavier gauche avec absence de lingula et d'encoche cardiaque. 4. L'encoche cardiaque et la ligule ont été notées absentes dans 3 poumons gauches (2,36 %). 5. Un poumon droit (0,37 %) a montré cinq lobes avec une fissure complète oblique et trois fissures horizontales incomplètes.Conclusion:A notre connaissance, de telles anomalies des poumons n'ont pas été citées dans la littérature médicale récente. La connaissance de ces anomalies est nécessaire pour éviter les complications lors de diverses procédures de radiodiagnostic ou de chirurgies cardiothoraciques.

Congenital Chest Lesions and Interventions.

Anomalies of the fetal chest require advanced imaging with ultrasound and MR imaging as well as expertise on the part of the interpreting pediatric radiologist. Congenital diaphragmatic hernia and congenital lung malformation are the most frequently seen, and in both conditions, the radiologist should provide both detailed anatomic description and measurement data for prognostication. This article provides a detailed approach to imaging the anatomy, in-depth explanation of available measurements and prognostic value, and keys to identifying candidates for fetal intervention. Less common congenital lung tumors and mediastinal and chest wall masses are also reviewed.

Hepatopulmonary fusion in congenital diaphragmatic hernia: successful management of a lethal variant.

Congenital diaphragmatic hernia (CDH) is a congenital anomaly involving the herniation of intra-abdominal contents into the thoracic cavity. Hepatopulmonary fusion (HPF), an exceedingly rare subtype mainly associated with right-sided CDH, presents unique diagnostic and therapeutic challenges. This case report describes a male infant with right-sided CDH complicated by HPF. The intricate anatomical anomaly involved the fusion of the right lung to the liver, posing challenges during surgical separation. The patient experienced postoperative complications, including prolonged ventilation, tracheostomy and pulmonary issues, which led to a prolonged hospital stay. Intraoperative challenges stem from the absence of demarcation between lung and liver tissues and abnormal vascular structures. In summary, managing HPF in right-sided CDH necessitates a customised, multidisciplinary approach to optimise patient outcomes, highlighting the need for ongoing research to refine understanding and treatment strategies.

[Surgery for complicated accessory lobe of the azygos vein]. / Khirurgiya oslozhnennoi dobavochnoi doli neparnoi veny.

Congenital anomalies of respiratory system are quite diverse and not all of them are subject to surgical treatment. One example is accessory lobe of the azygos vein. This anomaly usually has no clinical manifestations and requires only follow-up, as well as attention in surgery on the right half of the chest for some other disease. This situation changes when complications occur, for example, purulent-inflammatory process. Therapy is not always effective, and lung tissue destruction requires surgical treatment. Progressive destruction complicates diagnosis and choosing surgical tactics. We present a rare case of severe purulent-inflammatory complication with abscess in accessory lobe of v. azygos. Anatomical abnormalities following this congenital pulmonary anomaly can cause difficulties in surgeries for other intra-thoracic diseases. The situation is especially relevant for thoracoscopic access. This report will be useful for radiologists, pulmonologists and thoracic surgeons.

Interstitial Lung Abnormalities: Current Understanding.

Interstitial lung abnormalities (ILAs) are incidental findings on computed tomography scans, characterized by nondependent abnormalities affecting more than 5% of any lung zone. They are associated with factors such as age, smoking, genetic variants, worsened clinical outcomes, and increased mortality. Risk stratification based on clinical and radiological features of ILAs is crucial in clinical practice, particularly for identifying cases at high risk of progression to pulmonary fibrosis. Traction bronchiectasis/bronchiolectasis index has emerged as a promising imaging biomarker for prognostic risk stratification in ILAs. These findings suggest a spectrum of fibrosing interstitial lung diseases, encompassing from ILAs to pulmonary fibrosis.

Hydrops fetalis caused by a complex congenital heart defect with concurrent hypoplasia of pulmonary blood vessels and lungs visualized by micro-CT in a French Bulldog.

BACKGROUND: Hydrops fetalis (HF) is fluid accumulation in fetus body cavities and subcutaneous tissue. The condition has been described in various farm and companion animal species, including dogs. Most of cases result from a heart defect. Exact nature of this defect is rarely clarified. CASE PRESENTATION: A newborn, male French bulldog puppy with severe HF underwent a full anatomopathological examination to diagnose the primary cause of HF. Based on the anatomopathological examination, fetal ultrasound, and micro-computed tomography, transposition of the great arteries with hypoplasia of the ascending aorta, aortic arch interruption, ostium secundum atrial septal defect, severe tricuspid valve dysplasia, as well as hypoplasia of pulmonary vessels and lungs were diagnosed. CONCLUSIONS: This is the first report of HF caused by severe, complex congenital heart defects with concurrent pulmonary vessel and lung hypoplasia.

Single-Center Incidence and Patterns of Stroke in Early Renal Anhydramnios After Serial Amnioinfusions.

The Renal Anhydramnios Fetal Therapy (RAFT) trial is a study of serial amnioinfusions to prevent lethal neonatal pulmonary hypoplasia from early renal anhydramnios. Infant neurologic outcomes were not originally evaluated. We describe the high incidence of stroke observed among infants in the treatment arm of the trial at our center.

Outcome of fetal congenital pulmonary malformations: a systematic review and meta-analysis.

OBJECTIVES: To report the outcome of fetuses with a prenatal diagnosis of congenital lung malformation (CLM) diagnosed on ultrasound by performing a comprehensive assessment of these outcomes through a systematic review and meta-analysis. CONTENT: CLMs are a heterogeneous group of anomalies that involve the lung parenchyma and its bronchovascular structures. Their presentation and evolution are variable, from entirely asymptomatic lesions with sonographic regression in utero to hydropic fetuses requiring fetal therapy, intrauterine death or neonatal morbidity. A systematic review was conducted in Medline, Embase and Cochrane databases including studies on fetuses with CLM diagnosed prenatally in order to report the in-utero natural history of these lesions. Thirty-nine studies (2,638 fetuses) were included in the final review. SUMMARY: Regression/reduction in size of the lung lesion during pregnancy was reported in 31 % of cases, while its increase in 8.5 % of cases. Intra-uterine death complicated 1.5 % of pregnancies with fetal CLM, while neonatal and perinatal death were 2.2 and 3 %, respectively. Neonatal morbidity occurred in 20.6 % of newborns with CLM; 46 % had surgery, mainly elective. In fetuses with CLM and hydrops, fetal/perinatal loss occurred in 42 %. Assessment of the role of fetal therapy in improving the outcomes of pregnancies complicated by CLM was hampered by the small number of included cases and heterogeneity of type of interventions. OUTLOOK: Fetuses with CLM prenatally diagnosed have a generally favorable outcome. Conversely, there is a low quality of evidence on the actual role of fetal therapy in improving the outcome of fetuses presenting with these anomalies.

Early Lung Ultrasound Scores in Neonates With Respiratory Distress - A Cross-Sectional Study From South India.

OBJECTIVE: To estimate the lung ultrasound (LUS0) scores within 6 hours of birth in neonates with respiratory distress (RD) and assess its ability to predict the severity of RD. METHODS: This single-center cross-sectional study included all neonates admitted with RD during the study period for whom a LUS was performed within 6h of birth. LUS0 scoring was done by dividing the lung fields into 3 fields on either side and a score from 0 to 3 per field (maximum score 18). We excluded neonates with congenital heart disease, congenital anomalies of chest/lung, chromosomal anomalies and if the operator for LUS0 was not available. ROC curves were constructed for estimating the cut-off LUS0 score for the severity of RD in terms of the following six outcomes: fraction of inspired oxygen (FiO2) requirement >50% during first 3 days of life, need for invasive ventilation on day 3 of life, Silverman-Anderson score >7, surfactant requirement, radiological grades of RDS, and death. RESULTS: The median (IQR) LUS0 scores were significantly higher in neonates with greater severity of RD in terms of FiO2 requirement >50% during first 3 days of life [12.0, (5.0, 14.0)], need for invasive ventilation on day 3 of life [12.0 (7.5, 12.5)], Silverman-Anderson score ≥ 7 in preterm [9.5, (6.0, 12.0)], surfactant requirement [11.5, (4.0, 12.5)], radiological grades of RDS [10.0, (4.0, 12.0)], and death [12.0, (7.0, 15.0)]. In logistic regression analysis, with continuous LUS0 scores as covariates, the odds ratio significantly increased for every unit increase in LUS0 score. CONCLUSION: Early LUS0 scores can predict the prognosis and severity of neonatal RD.

Non-tuberculous mycobacterial pulmonary infection presenting in a patient with unilateral pulmonary artery agenesis.

People who have structural or developmental lung disease are more likely to develop non-tuberculous mycobacterial infections. We present the case of a young man in his 30s who had unilateral pulmonary artery agenesis on the right side and presented with a 6-month history of productive cough and fever. His CT scan showed nodular and cavitating lesions on the right side, and sputum analysis confirmed infection with Mycobacterium chimaera He had to undergo modifications in his treatment, including a change from rifampicin to rifabutin due to drug interactions and his amikacin had to be stopped due to signs of vestibular toxicity. Using a multidisciplinary approach, we were able to formulate an appropriate drug regimen for him, and he is now under regular follow-up with infectious diseases and respiratory medicine.

[Congenital pulmonary malformations : Diagnosis and treatment]. / Kongenitale Lungenfehlbildungen : Diagnostik und Therapie.

PERFORMANCE: Congenital pulmonary malformations (CPM) are rare and can be associated with high morbidity. Clinical presentation, diagnostic procedures, imaging, and therapy of CPM are discussed. ACHIEVEMENTS: Today, most CPM can be diagnosed prenatally by ultrasound. Postnatally, respiratory symptoms up to respiratory failure and recurrent lower respiratory tract infection are typical findings. Due to low diagnostic accuracy of chest x­ray in CPM, all children with prenatal diagnosis of CPM or postnatally suspected CPM should undergo cross-sectional imaging. PRACTICAL RECOMMENDATIONS: Based on imaging alone, the various subtypes of CPM cannot be definitively differentiated, which is why histological confirmation remains the gold standard. Surgical resection is the standard of care with minimally invasive procedures increasingly being employed. In certain situations, a watch-and-wait approach is possible.

The RNA-binding protein quaking is upregulated in nitrofen-induced congenital diaphragmatic hernia lungs at the end of gestation.

BACKGROUND: The RNA-binding protein Quaking (QKI) increases during epithelial-to-mesenchymal transition and its expression is controlled by microRNA-200 family members. Here, we aimed to describe the expression of QKI in the developing lungs of control and nitrofen-induced congenital diaphragmatic hernia lungs (CDH). METHODS: To investigate the expression of QKI, we dissected lungs from control and nitrofen-induced CDH rats on embryonic day 15, 18, 21 (E15, E18, E21). We performed immunofluorescence (IF) and quantitative reverse transcription PCR (RT-qPCR) for QKI expression. Additionally, we assessed Interleukin-6 (IL-6) abundance using IF. RESULTS: On E21, IF showed that the abundance of all three QKI isoforms and IL-6 protein was higher in CDH lungs compared to control lungs (QKI5: p = 0.023, QKI6: p = 0.006, QKI7: p = 0.014, IL-6: p = 0.045, respectively). Furthermore, RT-qPCR data showed increased expression of QKI5, QKI6, and QKI7 mRNA in E21 nitrofen lungs by 1.63 fold (p = 0.001), 1.63 fold (p = 0.010), and 1.48 fold (p = 0.018), respectively. CONCLUSIONS: Our data show an increase in the abundance and expression of QKI at the end of gestation in nitrofen-induced CDH lungs. Therefore, a disruption in the regulation of QKI during the late stage of pregnancy could be associated with the pathogenesis of abnormal lung development in CDH.