ABSTRACT
INTRODUCTION AND SIGNIFICANCE: Congenital lobar emphysema (CLE) is a rare but important lung malformation typically discovered in the newborn period. Some rare cases are reported in adults. It can present with various respiratory symptoms. Diagnosis relies primarily on chest CT scans, and the main treatment is surgery. CASE PRESENTATION: We present a case of a young girl with recurrent respiratory infections who was misdiagnosed with a pneumothorax. A chest tube was inserted. Later, CLE was identified, and the affected lung lobe was surgically removed. CLINICAL DISCUSSION: This case highlights the importance of including CLE in the differential diagnosis for hyperinflation of a lung lobe. A CT scan is crucial for confirmation. CONCLUSION: Congenital lobar emphysema is a rare disease that primarily affects children. Most children with CLE experience symptoms and require surgery. In adults, CLE is uncommon, and surgery is based on the severity of symptoms and radiological findings.
ABSTRACT
This case report discusses the diagnostic challenge of congenital lobar emphysema (CLE) in a three-month old infant with severe respiratory distress. The infant was initially misdiagnosed and managed as a case of pneumothorax. This case highlights the importance of CT scans as a diagnostic tool for early diagnosis and lifesaving management of CLE. It also signifies the need for adequate funds and infrastructure in the health care system especially in rural areas of developing countries like Pakistan.
Subject(s)
Pulmonary Emphysema , Tomography, X-Ray Computed , Humans , Infant , Pulmonary Emphysema/diagnostic imaging , Pulmonary Emphysema/congenital , Pneumothorax/diagnostic imaging , Pneumothorax/diagnosis , Male , Diagnosis, DifferentialABSTRACT
There are many congenital anomalies of the lung, some of which have no clinical symptoms and are detected incidentally, while others, particularly in the neonatal and infant period, are recognized by their typical signs, symptoms, and radiological appearance. Some congenital lung anomalies are so important that they can cause the death of the patient if not diagnosed and treated early. Classification of congenital lung anomalies is difficult since these anomalies may be related to the airway, arterial and venous vascular system, pulmonary parenchyma, and primitive anterior intestinal anomalies from which the lung originates, and some anomalies may have several etiologic origins. In this review, all subgroups of congenital pulmonary malformations will be discussed.
ABSTRACT
There has been an increased recognition of a subset of congenital lobar emphysema (CLE), termed congenital sublobar hyperinflation (CSLH), which may affect only a segment of lung as opposed to an entire lobe. This is an uncommon variant for which there is a paucity of information in published literature. The majority of CLE are managed surgically. Current literature suggests non-operative management for CSLH. However, there has been slow adoption of non-operative management and there is not a well-established observation pathway. A retrospective review of all pediatric patients diagnosed with CSLH at a single institution was performed from 2017 to 2023 to determine if this variant may be safely managed with observation. A total of 10 patients were identified. Of these, three patients had consolidation on cross-sectional imaging; therefore, operative intervention was undertaken given diagnostic uncertainty. All patients managed observationally remained asymptomatic. This case series validates non-operative management for patients with asymptomatic CSLH.
Subject(s)
Pulmonary Emphysema , Humans , Retrospective Studies , Pulmonary Emphysema/congenital , Pulmonary Emphysema/therapy , Pulmonary Emphysema/diagnosis , Pulmonary Emphysema/surgery , Female , Male , Infant , Child, Preschool , Watchful Waiting , Child , Infant, Newborn , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Lung , Female , Humans , Infant, Newborn , Male , Lung/abnormalities , Lung/diagnostic imaging , Lung/surgery , Lung Diseases/diagnosis , Lung Diseases/therapy , Lung Diseases/congenital , Lung Diseases/diagnostic imaging , Respiratory System Abnormalities/diagnosis , Respiratory System Abnormalities/therapy , Respiratory System Abnormalities/surgery , Tomography, X-Ray Computed , Ultrasonography, PrenatalABSTRACT
Recurrent wheezing is very common in infants. When these symptoms appear early without a free interval, a pulmonary malformation should be investigated. Congenital lobar emphysema is a rare abnormality of the lower respiratory tract. Here, we report a case series of six cases of congenital lobar emphysema between 2015 and 2023. Clinical and radiological data were collected according to an operating sheet previously established in our pediatric pneumo-allergology unit. They all had recurrent wheezing and dyspnea. Chest radiography and chest CT were consistent with the diagnosis of congenital lobar emphysema. All patients had lobectomy without complications.
ABSTRACT
BACKGROUND: Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. METHODS: We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment. RESULTS: The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization. CONCLUSIONS: In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.
INTRODUCCIÓN: Las malformaciones pulmonares congénitas son poco frecuentes en niños. El objetivo de este estudio fue describir las características clínicas, imagenológicas y tratamiento de los pacientes con esta patología. MÉTODOS: Se llevó a cabo un estudio descriptivo y retrospectivo con datos de los pacientes con malformaciones pulmonares congénitas diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú) entre enero 2010 y diciembre 2020. Se describieron las características clínicas, imagenológicas, el tipo de malformación pulmonar congénita y el tratamiento. RESULTADOS: La muestra fue de 70 pacientes. La mediana de edad fue 29 meses, la relación masculino/femenino fue 1.4. En la tomografía de tórax se encontró compromiso parenquimal en 50 (71.4%) casos, y compromiso mixto (parenquimal y vascular) en 18 (25.7%). La malformación congénita de la vía aérea pulmonar se observó en 39 (55.7%) casos, seguida del quiste broncogénico en 10 (14.3%), secuestro pulmonar intralobar en 9 (12.9%) y secuestro pulmonar extralobar en 7 (10%). La lobectomía fue realizada en 61 (87.1%) casos, la quistectomía en 5 (7.1%), segmentectomía en 2 (2.9%) y embolización en 2 (2.9%). La complicación posquirúrgica más frecuente fue la neumonía en 9 (12.9%) casos. La mediana de estancia hospitalaria fue de 26 días. Ningún paciente falleció durante la hospitalización. CONCLUSIONES: En nuestra institución, la malformación pulmonar congénita más frecuente fue la malformación congénita de la vía aérea pulmonar, y la lobectomía el procedimiento quirúrgico más comúnmente realizado. Las malformaciones pulmonares congénitas representan un grupo diverso de trastornos del desarrollo pulmonar con variados patrones imagenológicos y manifestaciones clínicas.
Subject(s)
Bronchopulmonary Sequestration , Respiratory System Abnormalities , Humans , Child , Female , Male , Infant, Newborn , Bronchopulmonary Sequestration/diagnosis , Bronchopulmonary Sequestration/epidemiology , Bronchopulmonary Sequestration/surgery , Hospitals, Pediatric , Peru/epidemiology , Retrospective Studies , Respiratory System Abnormalities/diagnosis , Respiratory System Abnormalities/epidemiology , Respiratory System Abnormalities/surgery , LungABSTRACT
Abstract Background: Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. Methods: We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment. Results: The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization. Conclusions: In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.
Resumen Introducción: Las malformaciones pulmonares congénitas son poco frecuentes en niños. El objetivo de este estudio fue describir las características clínicas, imagenológicas y tratamiento de los pacientes con esta patología. Métodos: Se llevó a cabo un estudio descriptivo y retrospectivo con datos de los pacientes con malformaciones pulmonares congénitas diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú) entre enero 2010 y diciembre 2020. Se describieron las características clínicas, imagenológicas, el tipo de malformación pulmonar congénita y el tratamiento. Resultados: La muestra fue de 70 pacientes. La mediana de edad fue 29 meses, la relación masculino/femenino fue 1.4. En la tomografía de tórax se encontró compromiso parenquimal en 50 (71.4%) casos, y compromiso mixto (parenquimal y vascular) en 18 (25.7%). La malformación congénita de la vía aérea pulmonar se observó en 39 (55.7%) casos, seguida del quiste broncogénico en 10 (14.3%), secuestro pulmonar intralobar en 9 (12.9%) y secuestro pulmonar extralobar en 7 (10%). La lobectomía fue realizada en 61 (87.1%) casos, la quistectomía en 5 (7.1%), segmentectomía en 2 (2.9%) y embolización en 2 (2.9%). La complicación posquirúrgica más frecuente fue la neumonía en 9 (12.9%) casos. La mediana de estancia hospitalaria fue de 26 días. Ningún paciente falleció durante la hospitalización. Conclusiones: En nuestra institución, la malformación pulmonar congénita más frecuente fue la malformación congénita de la vía aérea pulmonar, y la lobectomía el procedimiento quirúrgico más comúnmente realizado. Las malformaciones pulmonares congénitas representan un grupo diverso de trastornos del desarrollo pulmonar con variados patrones imagenológicos y manifestaciones clínicas.
ABSTRACT
Congenital lobar emphysema (CLE) is caused by airway defects resulting in air trapping and hyperinflation of the affected lobe. Case reports of families affected with CLE imply a genetic etiology. However, the genetic contributions have not been well-described. We present a case of CLE in a monozygotic twin brother with respiratory distress diagnosed with right upper lobe (RUL) CLE and treated with a lobectomy. His asymptomatic twin brother was screened prophylactically, found to have RUL CLE and subsequently underwent a lobectomy. Our report provides further evidence supporting the genetic predisposition and potential benefit of early screening for CLE in comparable scenarios.
ABSTRACT
Congenital lobar emphysema (CLE) is a rare developmental lung disorder characterized by lobar hyperinflation secondary to bronchopulmonary obstruction. Half of patients are symptomatic at birth, with many requiring urgent or emergent surgical resection to treat respiratory distress. Meanwhile, patients achieving late childhood or adolescence without symptoms usually never require surgery. We present a case of a 26 year old otherwise healthy female with known CLE who developed massive hemoptysis and required urgent videoscopic (VATS) resection of her right lung upper lobe. We know of no other report of CLE causing life-threatening bleeding at any age, and herein review pathology and pathophysiology of the condition.
Subject(s)
Hemoptysis , Pulmonary Emphysema , Humans , Infant, Newborn , Female , Child , Adult , Hemoptysis/surgery , Hemoptysis/complications , Pulmonary Emphysema/complications , Pulmonary Emphysema/surgery , Pulmonary Emphysema/pathology , Lung/surgery , DyspneaSubject(s)
Bronchogenic Cyst , Pulmonary Emphysema , Infant , Humans , Bronchogenic Cyst/complications , Bronchogenic Cyst/diagnostic imaging , Bronchogenic Cyst/surgery , Respiratory Sounds/etiology , Pulmonary Emphysema/complications , Pulmonary Emphysema/diagnostic imaging , Pulmonary Emphysema/congenital , Bronchoscopy/adverse effectsABSTRACT
Congenital lung malformations represent a spectrum of lesions, each with a distinct cause and tailored clinical approach. This article will focus on the following malformations: congenital pulmonary airway malformations, formally known as congenital cystic adenomatoid malformations, bronchopulmonary sequestration, congenital lobar emphysema, and bronchogenic cyst. Each of these malformations will be defined and examined from an embryologic, pathophysiologic, and clinical management perspective unique to that specific lesion. A review of current recommendations in both medical and surgical management of these lesions will be discussed as well as widely accepted treatment algorithms.
Subject(s)
Bronchopulmonary Sequestration , Cystic Adenomatoid Malformation of Lung, Congenital , Lung Diseases , Pulmonary Emphysema , Respiratory System Abnormalities , Humans , Cystic Adenomatoid Malformation of Lung, Congenital/diagnostic imaging , Cystic Adenomatoid Malformation of Lung, Congenital/surgery , Bronchopulmonary Sequestration/diagnostic imaging , Bronchopulmonary Sequestration/surgery , Pulmonary Emphysema/surgery , Pulmonary Emphysema/congenital , Respiratory System Abnormalities/diagnosis , Respiratory System Abnormalities/surgery , Lung/abnormalitiesABSTRACT
Congenital lobar overinflation (CLO) is a rare congenital pulmonary abnormality characterized by progressive overexpansion of a lobe(s) exerting mass effect on the remaining lobes and the mediastinum. Bilateral involvement is extremely rare and has been reported only a handful of times. We present a case of bilateral congenital lobar overinflation in a term female neonate managed with single-step bilateral lobectomies.
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Introduction and importance: Congenital Lobar Emphysema is a rare cystic lesion of the lung which may be misdiagnosed and managed as pneumonia or pneumothorax. Case presentation: We presented a case of a congenital cystic lung malformation, the case demonstrated a 29-day-old boy who presented with respiratory distress. He was initially diagnosed as pneumonia and was given unnecessary antibiotic treatment in another hospital without improvement. Subsequently, the diagnosis of congenital lobar emphysema of the left upper lobe was made on the basis of the clinical and radiologic features. The condition wasn't detected before birth. He underwent a successful left thoracotomy with left upper lobe lobectomy. Clinical discussion: Congenital Lobar Emphysema poses a challenge in diagnosis. It may mimic other causes of respiratory distress. Conclusion: Congenital Lobar Emphysema requires a high index of clinical and radiological suspicion to make an early diagnosis so that timely treatment will be offered.
ABSTRACT
Congenital lobar emphysema (CLE) is defined as the hyperinflation of pulmonary lobes due to obstruction of the flow of air via a known or unknown etiology, which causes pressure symptoms in the adjacent organs. CLE is mainly diagnosed in the neonatal period, and very few adult cases have been reported. Here we report a 34-year-old male with muscular dystrophy who was diagnosed with CLE on examination. He underwent a right lower lobectomy via 3-portal completely video-assisted thoracoscopic surgery, and his symptoms improved. Thoracoscopic surgery helped preserve the respiratory muscles and led to the improvement of respiratory function in this patient.
Subject(s)
Pulmonary Emphysema/congenital , Thoracic Surgery, Video-Assisted/methods , Adult , Humans , Male , Muscular Dystrophies/complications , Pulmonary Emphysema/surgeryABSTRACT
INTRODUCTION: Congenital lobar emphysema (CLE), a rare developmental lung malformation, involves the hyperaeration of one or more lung lobes caused by partial obstruction and occurs at a rate of 1/20,000-30,000 live births. Here, we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated by surgical or non-surgical (conservative) approaches at our center and compared our results with those in the literature. METHODS: We examined the clinical, radiological, and bronchoscopy findings of 20 patients with CLE aged 0-18 years at our center between 2013 and 2020. In addition, we examined the symptoms and findings recorded during the patients' follow-up in this retrospective descriptive study. RESULTS: The median age of 20 patients with CLE at diagnosis was 3.2 years (range 1 day-17 years). Respiratory distress and mediastinal shift were more prominent in patients who underwent surgery, and they were diagnosed at an earlier age compared with patients who were followed up conservatively (p = 0.001, 0.049, 0.001, respectively). Neither the pulmonary lobe involvement nor the bronchoscopy findings were found to be indicative of surgery. DISCUSSION: We observed that respiratory distress and mediastinal shift were more prominent in patients with a diagnosis of CLE who underwent surgery compared with patients who were conservatively followed up. Moreover, we observed that those who underwent surgery were diagnosed with CLE at an earlier age. In line with the literature, the pulmonary symptoms and CLE-related imaging findings in our study were reduced during conservative follow-up.
Subject(s)
Conservative Treatment , Pulmonary Emphysema , Adolescent , Child , Child, Preschool , Follow-Up Studies , Humans , Infant , Infant, Newborn , Pulmonary Emphysema/congenital , Pulmonary Emphysema/diagnostic imaging , Pulmonary Emphysema/surgery , Retrospective StudiesABSTRACT
We report a 4-month-old baby presenting with bilateral congenital lobar emphysema. A two-staged bilateral lobectomy was planned, but bilateral lobectomy had to be performed as a single-staged procedure. Data are scarce on the appropriate approach to children with bilateral involvement. Both single-staged and two-staged procedures have shown variable success.
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This is a case report of a child who had acyanotic congenital heart disease - ventricular septal defect (VSD) and a patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension. The child underwent open-heart surgery - VSD closure with PDA ligation - and six months later was re-admitted for congenital lobar emphysema of the right middle lobe. He underwent successful right middle lobectomy of the lung six months after cardiac surgery under a one-lung ventilation technique in which application of fiber-optic bronchoscope made the surgery safer and more suitable.
ABSTRACT
Congenital lobar emphysema (CLE) is a developmental anomaly of the lower respiratory tract characterized by hyperinflation of one or more pulmonary lobes in the absence of extrinsic bronchial obstruction. We present a case of a 24-year-old male, nonsmoker who presented with shortness of breath and severe left sided chest pain. A chest x-ray was significant for a very large left-sided pneumothorax and chest CT showed lobar emphysematous changes. Video assisted thoracoscopic surgery (VATS) and lobectomy was subsequently performed after persistence of pneumothorax despite chest tube insertion and conservative management. Surgical pathology of resected specimen showed chronic emphysematous changes with patchy chronic organizing pneumonitis. Histology showed advanced emphysematous changes of pulmonary parenchyma consistent with congenital lobar emphysema. This finding combined with features seen on computed tomography of the chest led to the diagnosis of congenital lobar emphysema. This case demonstrated that CLE can be a cause of tension pneumothorax in adults in rare cases.
