Thoracic ultrasound: a review of the state-of-the-art.

Thoracic ultrasound (TUS) is a tool that has become increasingly essential in the daily practice of thoracic medicine. Driven by the need to assess patients during the COVID-19 pandemic, there has been an increase in the use of point-of-care TUS, which has demonstrated several benefits, either as a complement to clinical decision-making for diagnosis or as a real-time guide for procedures, whether as a predictor or measure of treatment response. Here, we present a review of TUS, based on the most recent scientific evidence, from equipment and techniques to the fundamentals of pulmonary ultrasound, describing normal and pathological findings, as well as focusing on the management of lung disease and guidance for invasive thoracic procedures at the bedside. Finally, we highlight areas of perspective and potential lines of research to maintain interest in this valuable tool, in order to improve the diagnostic process and expand the treatment arsenal.

Drug-induced lung disease: a narrative review.

Drug-induced lung disease (DILD) encompasses a broad, highly heterogeneous group of conditions that may occur as a result of exposure to numerous agents, such as antineoplastic drugs, conventional or biological disease-modifying antirheumatic drugs, antiarrhythmics, and antibiotics. Between 3% and 5% of prevalent cases of interstitial lung diseases are reported as DILDs. The pathogenesis of lung injury in DILD is variable, multifactorial, and often unknown. Acute presentation is the most common, can occur from days to months after the start of treatment, and ranges from asymptomatic to acute respiratory failure. The CT patterns are varied and include ground-glass opacities, organizing pneumonia, and diffuse alveolar damage. Notably, there are no clinical manifestations or CT patterns specific to DILD, which makes the diagnosis quite challenging and necessitates a high index of suspicion, as well as the exclusion of alternative causes such as infection, cardiac-related pulmonary edema, exacerbation of a preexisting ILD, and neoplastic lung involvement. Discontinuation of the offending medication constitutes the cornerstone of treatment, and corticosteroid treatment is usually necessary after the onset of clinical manifestations. The prognosis varies widely, with high mortality rates in severe cases. A history of medications related to pulmonary toxicity in patients with new-onset respiratory symptoms should prompt consideration of DILD as a potential underlying cause.

Intercostal Lung Hernias Presenting After Minimally Invasive Cardiac Surgery.

INTRODUCTION: With the introduction of minimally invasive cardiac surgery, more commonly cases of lung herniation are starting to appear. Acquired lung hernias are classified as postoperative, traumatic, pathologic, and spontaneous. Up to 83% of lung hernias are intercostal. Herein, we describe patients presenting with intercostal lung hernias following minimally invasive cardiac surgery at a single center in Medellín, Colombia. METHODS: We conducted a retrospective search of all patients presenting with intercostal lung hernias secondary to minimally invasive cardiac surgery at our clinic in Medellín since the beginning of our program, from 2010 to 2022. Mini-sternotomies were excluded from our study. We reviewed the incision type and other possible factors leading to intercostal lung hernia development. We also describe the approach taken for these patients. RESULTS: From 2010 up until 2022, 803 adult patients underwent minimally invasive cardiac surgeries through a mini-thoracotomy. At the time of data retrieval, nine patients presented with intercostal lung hernias at the previous incision site. Five hernias (55%) were from right 2nd intercostal parasternal mini-thoracotomies for aortic valve surgeries. Four hernias (45%) were from right 4th intercostal lateral mini-thoracotomies for mitral valve surgeries. Our preferred repair technique is a video-assisted thoracoscopic mesh approach. CONCLUSION: Minimally invasive cardiac surgical approaches are becoming more routine. Proper wound closure is critical in preventing lung hernias. Additionally, timely diagnosis and opportune hernia surgery using video-assisted thoracoscopic mesh repair can prevent further complications.

Neumatocele en paciente pediátrico. ¿Cuándo debemos actuar? / Pneumatocele in a pediatric patient. When should we act?

El neumatocele es una lesión cavitada llena de aire de carácter adquirido que se encuentra en el interior del parénquima pulmonar. Aunque las causas pueden variar, el origen infeccioso bacteriano es lo más frecuente. Los cambios en los serotipos de neumococo y el aumento de las neumonías necrotizantes observado en las últimas décadas hacen de este tipo de lesiones algo cada vez más frecuente. Es importante conocer la evolución esperable, así como también saber qué paciente se beneficia de intervención para evitar secuelas a largo plazo y complicaciones graves. En este artículo se exponen las causas, epidemiología, orientación diagnóstica y una propuesta de manejo para el neumatocele.

A pneumatocele is an air-filled cavitary lesion of acquired nature located within the pulmonary parenchyma. Although causes can vary, bacterial infectious origin is the most common. Changes in pneumococcal serotypes and the increase in necrotizing pneumonia observed in recent decades have made these lesions increasingly frequent. It is important to know the expected evolution and to identify which patients would benefit from intervention to prevent long-term sequelae and severe complications. This article exposes the causes, epidemiology, diagnostic approach, and a management proposal for pneumatocele.

Recomendaciones para el manejo de la enfermedad pulmonar post tuberculosis / Recommendations for the Management of Post Tuberculosis Lung Disease

Introducción: Los esfuerzos de la lucha contra la tuberculosis (TB) se centran habitualmente en un diagnóstico precoz y un tratamiento eficaz y oportuno para romper la cadena de transmisión de Mycobacterium tuberculosis. Sin embargo, en los últimos años, coincidiendo con la asociación sindémica TB/COVID-19, han aparecido cada vez más evidencias sobre las graves secuelas clínicas, funcionales y psicosociales que puede ocasionar la TB, condición que se ha definido como enfermedad pulmonar post-tuberculosis (PTLD). Aproximadamente, un tercio de los pacientes que sobreviven a la TB se enfrentan a esto, incluyendo síntomas respiratorios persistentes con exacerbaciones episódicas, insuficiencia respiratoria crónica, trastornos emocionales y desafíos psico-sociales que impactan negativamente en la calidad de vida y enfrentan un alto costo catastrófico. Objetivo: Proporcionar un modelo compartido, orientador y científicamente válido para diagnosticar, evaluar y tratar en forma oportuna a los pacientes con PTLD (prevención, diagnóstico, tratamiento y posible rehabilitación). Metodología: Es una investigación documental que incluye revisiones sistemáticas, meta-análisis, estudios observacionales y de las directrices existentes en los últimos años al respecto, sumado a una evaluación por expertos en el tema, con el propósito de adaptarlas a las condiciones locales de cada país latinoamericano. Conclusiones: Considerando la carga mundial, particularmente, latinoamericana de TB, y la carga estimada de la PTLD, se considera urgente el desarrollo de un consenso sobre este tema. Creemos que las recomendaciones de ALAT proporcionarán la base para la formulación y adopción de directrices nacionales para el manejo del PTLD en Amé- rica Latina.

Introduction: Efforts to combat tuberculosis (TB) usually focus on early, rapid diagnosis and effective treatment to break the chain of transmission of Mycobacterium tuberculosis. However, in the last few years, coinciding with the syndemic TB/COVID-19 association, more and more evidence has proved the serious clinical, functional and psycho-social sequelae that TB can cause. This condition has been defined as Post-Pulmonary Disease Tuberculosis (PTLD) and it affects approximately one-third of the patients who survive TB, facing persistent respiratory symptoms with episodic exacerbations, chronic respiratory failure, emotional disorders and psychosocial challenges that negatively impact their life quality, meaning a high catastrophic cost. Objective: Provide a shared, guiding and scientifically valid model to promptly diagnose, evaluate and treat patients with PTLD (prevention, diagnosis, treatment and possible rehabilitation). Methodology: It is documentary research that includes systematic reviews, meta-analysis, observational studies and the guidelines that have existed in recent years in this regard, added to an evaluation by experts, with the purpose of adapting them to local conditions of each Latin American country. Conclusions: Considering the global and, particularly, the Latin American burden of TB, and the estimated burden of PTLD, the development of a consensus document on this topic is urgent. Therefore, we think ALAT recommendations will provide the basis for the formulation and adoption of national specific guidelines for the management of PTLD in Latin America.

Evaluation of variants in the ENTPD1 and ENTPD2 genes in athletic horses with exercise-induced pulmonary haemorrhage.

BACKGROUND: Exercise-induced pulmonary haemorrhage (EIPH) in athletic horses is characterized by the presence of blood from the lungs in the tracheobronchial tree after intense exercise. Despite the high prevalence of EIPH in horses, the primary aetiology remains unknown. Variants in the genes encoding CD39 and CD39L1 (ENTPD1 and ENTPD2, respectively) were previously reported as potential genetic causes involved in EIPH pathogenesis. However, the role of these variants in haemostatic functions is unknown. RESULTS: To investigate the association between EIPH and missense variants in the ENTPD1 (rs1152296272, rs68621348, and rs68621347) and ENTPD2 genes (rs782872967), 76 Thoroughbred horses diagnosed with EIPH and 56 without clinical signs of EIPH (control group) by trachea-bronchial endoscopy were genotyped. The rs1152296272 and rs68621347 variants were linked, which explained why the same results were found in all horses. Approximately 96% and 95% of the EIPH and control horses, respectively, carried at least one nonreference allele for these variants. In contrast, 100% of the control horses and 96% of the EIPH horses were homozygous for the reference allele for the rs68621348 variant. In the EIPH group, 1.5% of the horses were homozygotes and 24% were heterozygous for the nonreference allele of the rs782872967 variant. In the control group, the nonreference allele of this variant was observed only in heterozygotes (16%). There were no significant differences between groups for any of the variants. CONCLUSIONS: The variants previously described in the genes encoding the CD39 and CD39L1 enzymes were highly present in the studied population. However, no association was found between the occurrence of EIPH and the presence of these variants in Thoroughbred horses in this study.

[Shrinking lung syndrome associated with systemic lupus erythematosus]. / Síndrome del pulmón encogido asociado a lupus eritematoso sistémico.

INTRODUCTION: Shrinking lung syndrome (SLS) is a rare and less-known manifestation of systemic lupus erythematosus (SLE). The aim of this study is to describe the demographic, clinical, functional, imaging characteristics, and treatment received in a cohort of patients diagnosed with SLS. METHODS: Clinical records were reviewed retrospectively in a cohort of patient with SLE and SLS followedup prospectively between 2007 to 2023 in the Rheumatology and Neumonology Units. RESULTS: Eleven SLS patients (10.3%) of 107 SLE patients were diagnosed. They were 81.8% female and 18.2% male. Median age was 30 years old (RIQ = 25-75% [25.5-41.5]). Clinical symptoms were dyspnea (72%), pleuritic pain (36%), but 27.3% of patients were asymptomatic at diagnosis. Respiratory functional evaluation was, median FEV1 / FVC : 86% (RIQ = 82-90), median FVC: 61% (RIQ = 38.5-71), median DLCO: 65% (RIQ = 48-69) and median DLCO/VA: 95% (RIQ = 89-106). High-resolution computed tomography of thorax (HRCT) showed atelectasis (54.5%), diafragmatic elevation (27.3%), normal (27.3%) and pleural effusion (9%). Patients were treated with systemic corticosteroids (100%); in 72.7% of them, associated to other immunosupressives: methotrexate (36%), mycophenolate (27.3%), hydroxychloroquine (27.3%) and azatioprine (18.2%). CONCLUSION: SLS prevalence was 10.3%, higher than reported in other studies of SLE. Dyspnea was the most clinical symptom. Suspicion of moderate restriction were determined with mild deterioration of DLCO. Atelectasis was the most frequent HCRT sign. Immunosupressive treatment was prescribed in all patients with systemic corticosteroids associated to other drugs.

Introducción: El síndrome de pulmón encogido (SPE) es una manifestación rara y poco conocida del lupus eritematoso sistémico (LES). El objetivo es describir las características demográficas, manifestaciones clínicas, funcionales, imagenológicas y tratamiento recibido en una cohorte de pacientes diagnosticados con SPE. Métodos: Se examinaron las historias clínicas de una cohorte de pacientes con LES seguidos prospectivamente desde 2007 al 2023 por los Servicios de Reumatología y Neumotisiología. Resultados: De 107 pacientes diagnosticados con LES, se hallaron 11 pacientes con SPE (10.3%), 81.8% fueron mujeres y 18.2 % varones; edad (mediana): 30 años (RIQ 25-75% = 25.5-41.5). Presentaron disnea 72%, dolor pleurítico 36%, y asintomáticos 27.3% al momento del diagnóstico. Funcionalmente presentaron FEV1 / FVC = 86% mediana, (RIQ = 82-90), FVC mediana 61% (RIQ= 38.5-71), DLCO mediana: 65% (RIQ = 48­69) y DLCO/VA mediana: 95% (RIQ = 89-106). Se observó en la tomografía de tórax de alta resolución (TACAR) atelectasias (54.5%), elevación del diafragma (27.3%), normalidad (27.3%) y derrame pleural (9%). Recibieron tratamiento con corticoides sistémicos (100%), asociados a otros inmunosupresores en 72.7%: metotrexato (36%), micofenolato (27.3%), hidroxicloroquina (27.3%) y azatioprina (18.2%). Conclusión: La prevalencia de SPE fue 10.3%, más alta que lo reportado en otras series de casos de LES. La disnea fue el síntoma principal de consulta. Los pacientes presentaron funcionalmente sospecha de restricción moderada y leve caída de la DLCO. La atelectasia fue el signo más frecuente en la TACAR. El tratamiento inmunosupresor fue indicado en todos los pacientes con corticoides sistémicos asociados usualmente a otros inmunosupresores.

Ultrasound evaluation in the postoperative period for surgical treatment of thoracic infectious and inflammatory diseases.

OBJECTIVE: This study aims to evaluate the role of TUSG in the postoperative period and the detection of early complications after surgical treatment, pulmonary resection, or decortication for infectious and inflammatory thoracic diseases, comparing with the standard method (Chest Radiography ‒ CXR). METHODS: Prospective non-randomized self-controlled study. Twenty-one patients over 16 years of age have undergone surgical treatment of inflammatory and infectious lung diseases. These patients were followed up with CXR and TUSG (performed on the 1st and 3rd postoperative days and/or after the chest tube removal). RESULTS: Both exams demonstrated similar results regarding their ability to safely predict the adequate moment for chest drain removal. TUSG allowed chest drain removal in 30% of cases and CXR in 34%. Statistical analysis demonstrates that both exams have similar capabilities in detecting postoperative changes in the pleural space. However, the authors report that TUSG is statistically more accurate in detecting subcutaneous emphysema than CXR (p = 0.037, Kappa [κ = 0.3068]). The analysis of other parameters showed no statistical difference. CONCLUSION: The authors conclude that TUSG in trained hands is equivalent to CXR in searching for postoperative complications regarding the surgical treatment of infectious and inflammatory thoracic diseases and can be used as a complement, and not a substitute, to CXR, when CCT is not feasible, or a more urgent diagnosis is needed.

Cell Proliferation and Apoptosis-Key Players in the Lung Aging Process.

Currently, the global lifespan has increased, resulting in a higher proportion of the population over 65 years. Changes that occur in the lung during aging increase the risk of developing acute and chronic lung diseases, such as acute respiratory distress syndrome, chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, and lung cancer. During normal tissue homeostasis, cell proliferation and apoptosis create a dynamic balance that constitutes the physiological cell turnover. In basal conditions, the lungs have a low rate of cell turnover compared to other organs. During aging, changes in the rate of cell turnover in the lung are observed. In this work, we review the literature that evaluates the role of molecules involved in cell proliferation and apoptosis in lung aging and in the development of age-related lung diseases. The list of molecules that regulate cell proliferation, apoptosis, or both processes in lung aging includes TNC, FOXM1, DNA-PKcs, MicroRNAs, BCL-W, BCL-XL, TCF21, p16, NOX4, NRF2, MDM4, RPIA, DHEA, and MMP28. However, despite the studies carried out to date, the complete signaling pathways that regulate cell turnover in lung aging are still unknown. More research is needed to understand the changes that lead to the development of age-related lung diseases.

[Cholestasis, delirium and pulmonary nocardiosis in a man with systemic lupus erythematosus of late onset]. / Colestasis, delirium y nocardiosis pulmonar en un varón con lupus eritematoso sistémico de inicio tardío.

A case is presented of a 64-year-old male patient who was admitted because of delirium, jaundice, a pattern of cholestasis in the liver profile and a right lung mass in the context of a constitutional syndrome and weight loss in the last eight months. The lung mass was punctured and the culture of the obtained material developed white colonies, identified by mass spectrometry (MALDI-TOF) as Nocardia cyriacigeorgica. Regarding the clinical diagnosis, it was considered as systemic lupus erythematosus (SLE), on the basis of fulfilling 8 criteria according to SLICC 2012 group, and 24 points according to EULAR/ACR 2019. The liver biopsy showed a mixt cellular infiltrate in portal spaces, with absence of interphase hepatitis and presence of peripheral ductular reaction. These findings were interpreted as liver compromise relate to SLE. Delirium was also considered as a neurological manifestation related to SLE on the basis of ruling out other causes. After being treated with antibiotics and documenting a reduction in the size of the lung mass he received cyclophosphamide in intravenous pulses, achieving normalization of his liver profile and his state of consciousness, and a progressively weight recovering. A year after he was in good health. The report of this case is justified because of the rare presenting form of late onset SLE, as well as the concomitant pulmonary nocardiosis in the absence of previous immunosuppressant treatment.

Se presenta el caso de un varón de 64 años que fue internado por delirium asociado a ictericia con patrón de colestasis en el hepatograma, y una masa en el pulmón derecho en el contexto de pérdida de peso y síndrome constitucional de 8 meses de evolución. Se realizó punción de la masa pulmonar cuyo cultivo desarrolló colonias blanquecinas identificadas como Nocardia cyriacigeorgica por espectrometría de masas (MALDI-TOF MS). Se llegó al diagnóstico de lupus eritematosos sistémico (LES) por presentar 8 de los criterios de acuerdo con el grupo SLICC 2012 y 24 puntos de acuerdo a los criterios EULAR/ACR 2019. La biopsia hepática mostró leve y variable infiltrado inflamatorio mixto en espacios porta, con ausencia de hepatitis de interfase y presencia de reacción ductular periférica. Se interpretaron estos hallazgos como vinculados a hepatopatía por LES. El delirium fue interpretado como afectación neurológica por LES en base al descarte de otras enfermedades. Recibió tratamiento antibiótico y tras constatarse reducción del tamaño de la masa pulmonar se administraron pulsos de ciclofosfamida intravenosa. Evolucionó favorablemente, con normalización del hepatograma y el estado de conciencia, y recuperación del peso en forma progresiva. Al año se lo encontró en buen estado de salud. Justifica el reporte del caso la rara forma de presentación del LES de comienzo tardío, así como la nocardiosis pulmonar concomitante sin tratamiento inmunosupresor previo.

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.

Fluid therapy and pulmonary complications in abdominal surgeries: randomized controlled trial.

BACKGROUND: There is no consensus on the most effective strategy for Postoperative Pulmonary Complication (PPC) reduction. This study hypothesized that a Goal-Directed Fluid Therapy (GDFT) protocol of infusion of predetermined boluses reduces the occurrence of PPC in patients undergoing elective open abdominal surgeries when compared with Standard of Care (SOC) strategy. METHODS: Randomized, prospective, controlled study, conducted from May 2012 to December 2014, with ASA I, II or III patients undergoing open abdominal surgeries, lasting at least 120 min, under general anesthesia, randomized into the SOC and the GDFT group. In the SOC, fluid administration was according to the anesthesiologist's discretion. In the GDFT, the intervention protocol, based on bolus infusion according to blood pressure and delta pulse pressure, was applied. Patients were postoperatively evaluated by an anesthesiologist blinded to the group allocation regarding PPC incidence, mortality, and Length of Hospital Stay (LOHS). RESULTS: Forty-two patients in the SOC group and 43 in the GDFT group. Nineteen patients (45%) in the SOC and 6 in the GDFT (14%) had at least one PPC (p = 0.003). There was no difference in mortality or LOHS between the groups. Among the patients with PPC, four died (25%), compared to two deaths in patients without PPC (3%) (p = 0.001). The LOHS had a median of 14.5 days in the group with PPC and 9 days in the group without PPC (p = 0.001). CONCLUSION: The GDFT protocol resulted in a lower rate of PPC; however, the LOHS and mortality did not reduce.

Infants with neonatal Chronic Lung Disease are associated with delayed auditory conduction in the rostral brainstem after term.

AIMS: Very Low Birthweight (VLBW) infants with neonatal Chronic Lung Disease (CLD) have been found to have functional impairment of the brainstem auditory pathway at term. This study investigated the functional status of the brainstem auditory pathway in VLBW infants with CLD after term for any abnormality. METHODS: Fifty-two VLBW infants were recruited at 50 weeks of Postconceptional Age: 25 with neonatal CLD and 27 without CLD. None had any other major complications to minimize confounding effects. Brainstem Auditory Evoked Responses were studied at 21‒91/s click rates. RESULTS: Compared with those without CLD, VLBW infants with CLD had relatively shorter latencies of BAER waves I and III, associated with a slightly lower BAER threshold. Wave V latency and I‒V interpeak interval did not differ significantly between the two groups of infants. The I‒III interval in infants with CLD was shorter than in those without CLD at 91/s clicks. However, the III‒V interval was significantly longer than in those without CLD at all click rates (all p < 0.05). There were no significant differences in the amplitudes of BAER wave components between the two groups of infants. CONCLUSIONS: The main BAER abnormality in VLBW infants with CLD was a prolonged III‒V interval. Auditory conduction is delayed or impaired at more central regions of the brainstem in CLD infants. After term central auditory function is adversely affected by neonatal CLD. Monitoring post-term change is required to provide valuable information for post-term care of CLD infants.

Pathological analysis and etiological assessment of pulmonary lesions and its association with pleurisy in slaughtered pigs.

The intensification of pig farming has posed significant challenges in managing and preventing sanitary problems, particularly diseases of the respiratory complex. Monitoring at slaughter is an important control tool and cannot be overstated. Hence, this study aimed at characterizing both macroscopical and microscopical lesions and identifying the Actinobacillus pleuropneumoniae (APP), Mycoplasma hyopneumoniae (Mhyo), and Pasteurella multocida (PM) associated with pleurisy in swine. For this, a selected slaughterhouse in São Paulo State underwent a thorough examination of carcasses on the slaughter line, followed by lung sampling. The carcasses and lungs underwent macroscopical examination and were classified according to the score of pleurisy and lung samples were allocated into five groups, being: G0: score 0 - no lesions; G1: score 1; G2: score 2; G3: score 3; and G4: score 4. In total, 217 lung fragments were collected, for the histopathological evaluation and detection of the following respiratory pathogens: APP, Mhyo, and PM by qPCR. The results demonstrated that Mhyo and APP were the most prevalent etiological agents (single and co-identification) in lung samples, in different scores of pleurisies, while bronchopneumonia and bronchus-associated lymphoid tissue (BALT) hyperplasia lesions were the most frequent histopathological findings. Positive correlations were found between the quantification of APP DNA with 1) the score of pleurisy (R=0.254); 2) with the score of lung consolidation in all lung lobes (R=0.181 to R=0.329); and 3) with the score of lung consolidation in the entire lung (R=0.389). The study brings relevant information regarding the main bacterial pathogens associated with pleurisy in pigs and helps with understanding the relationship between the abovementioned pathogens and their impact on the respiratory health of pigs.

Presentación de 11 pacientes con el diagnóstico de agenesia e hipoplasia pulmonar diagnosticados en un periodo de 24 años y revisión de la literatura / Presentation of 11 patients diagnosed with pulmonary agenesis and hypoplasia over a period of 24 years and a review of the literature

La agenesia pulmonar (AgP), la aplasia pulmonar (AP) e hipoplasia pulmonar (HP) son malformaciones congénitas poco comunes. En la AgP, no se desarrollan el bronquio ni el pulmón; en la AP, hay un bronquio rudimentario sin parénquima pulmonar; y en la HP, uno o ambos pulmones presentan un tamaño reducido debido a trastornos en su crecimiento. Las causas de la AgP, AP y HP pueden ser tanto primarias como secundarias, predominando estas últimas. Entre ellas se incluyen: oligohidroamnios, anomalías esqueléticas, enfermedades neuromusculares, hernia diafragmática, malformaciones vasculares, cardiopatías complejas, genopatías y cromosomopatías. El rango de manifestaciones varía desde pacientes asintomáticos hasta aquellos con dificultad respiratoria neonatal de leve a severa. Con el tiempo, algunos pacientes pueden experimentar neumonías recurrentes y progresar hacia una enfermedad pulmonar crónica (EPC). La imagenología juega un papel crucial en el diagnóstico. El pronóstico está fuertemente influenciado por la presencia de otras malformaciones congénitas. Generalmente, el enfoque terapéutico es conservador. Este artículo detalla la presentación clínica y la evolución a lo largo de 24 años de 11 pacientes diagnosticados con AgP o HP.

Pulmonary agenesis (AgP), aplasia (AP), and hypoplasia (HP) are rare congenital malformations. In AgP, there is no development of the bronchus or lung; in AP, a rudimentary bronchus is present without lung parenchyma; and in HP, one or both lungs are reduced in size due to growth disorders. The causes of AgP, AP, and HP can be either primary or secondary, with the latter being more common. Examples include oligohydramnios, skeletal anomalies, neuromuscular diseases, diaphragmatic hernia, vascular malformations, complex heart diseases, genopathies, and chromosomal disorders. The spectrum of manifestations ranges from asymptomatic patients to those with mild to severe neonatal respiratory distress. Over time, some patients may experience recurrent pneumonias and progress to chronic lung disease (CLD). Imaging studies are crucial for diagnosis. The prognosis primarily depends on the presence of other congenital malformations. The treatment approach is generally conservative. This article describes the clinical presentation and evolution over 24 years of 11 patients diagnosed with AgP or HP.