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.

A three month old infant with severe respiratory distress.

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.

A Review of Management of Congenital Sublobar Hyperinflation at a Single Institution.

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.

Management of Congenital Lung Malformations.

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.

Rare interstitial lung disease in a preterm neonate with congenital lobar emphysema.

Congenital lobar emphysema (CLE)/congenital alveolar overdistension/congenital lobar overinflation or infantile lobar emphysema is a rare developmental anomaly of the lower respiratory tract which is characterised by hyperinflation of one or more of the pulmonary lobes. Histopathology may be variable, which may show abnormality in the cartilage, granulation tissue, mucosal folds, etc. We report a rare underlying histopathology in a preterm neonate with CLE. This entity referred to as pulmonary interstitial glycogenosis (PIG) is a group of heterogeneous lung disease affecting the lung parenchyma and is characterised by the presence of glycogen laden cells in the lung interstitium. This impairs the gas exchange and typically manifest as tachypnoea, retractions, hypoxia and increased respiratory support. Our case report highlights the association of CLE and PIG and a review of literature. One should always have a detailed histopathology in children presenting with CLE to rule out PIG.

Outcome of paediatric lobectomy by thoracotomy - a single centre, six-year experience.

This short report highlights the patient outcome of lung resections for congenital and acquired lung lesions in children who presented to the Liaquat National Hospital, Karachi, from January 2013 to April 2019. Common indications were congenital pulmonary airway malformation (CPAM), congenital lobar emphysema (CLE), and bronchiectasis. Two patients died whereas, 21 were discharged home. Lung resection can be performed safely in children. Early diagnosis and surgery may improve the outcome.

Congenital Lobar Emphysema in a Kitten with Concomitant Hiatal Hernia and Nutritional Secondary Hyperparathyroidism.

A 2 mo old domestic shorthair kitten was presented for acute respiratory distress and severe ambulatory difficulties. Thoracic radiography revealed hyperinflation of the left cranial lung lobe and a mass with soft-tissue/gas opacity in the caudal mediastinum, leading to the suspicion of congenital lung lobe emphysema and hiatal hernia. Decreased bone radiopacity and suspected pathological fractures were also present. Complete clinicopathological analyses showed significant ionized hypocalcemia and suspicion of secondary hyperparathyroidism related to an inadequate diet. Lung lobectomy and reduction of the hiatal hernia following a median sternotomy and a cranial laparotomy were performed. IV and oral supplementation of calcium led to a full recovery and improvement in the kitten's walking. A histopathological analysis revealed pulmonary emphysema associated with hypoplastic and irregular bronchial cartilage. Congenital lobar emphysema is a rare disease in both humans and animals. This is the first veterinary report describing a kitten affected by congenital lobar emphysema combined with a hiatal hernia and additionally complicated by secondary nutritional hyperparathyroidism with a good long-term outcome.

Completely Video-assisted Thoracoscopic Lobectomy for Congenital Lobar Emphysema in a Young Adult.

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.

Surgery versus conservative management in congenital lobar emphysema: follow up and indicators for surgery.

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.

[Congenital Malformations of the Lung - an Overview]. / Angeborene Fehlbildungen der Lunge ­ eine Übersicht.

Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. The management of asymptomatic lesions remains subject to debate, but early elective surgery is generally recommended to avoid respiratory and infectious complications at a later time point.We here provide a comprehensive review in which we present causes, clinical presentation and therapeutic options for the most prominent congenital malformations of the airways and lung parenchyma.

Enfisema pulmonar congênito: relato de caso / Congenital pulmonary enphysema: case report

Introdução: O enfisema pulmonar congênito (EPC) é uma doença rara, possuindo uma incidência de 1:20-30 mil nascimentos, é mais comum no sexo masculino do que no feminino, em uma razão de 3:1 e sua etiologia permanece desconhecida. Um terço dos casos são sintomáticos ao nascer e praticamente todos são diagnosticados nos primeiros seis meses de vida. Relato de Caso: Recém-nascido (RN) do sexo masculino, evoluiu com desconforto respiratório precoce, sendo encaminhado ao centro de terapia intensiva. Após uso de continuous positive airway pressure (CPAP) e cateter nasal de oxigênio de 12 horas, resultou em bom padrão respiratório e boa saturação. Após realização de tomografia computadorizada, foi diagnosticado o EPC. Conclusão: O EPC é uma patologia rara e deve ser suspeitado em RN com desconforto respiratório, atribuindo-se importância aos vários diagnósticos diferenciais possíveis. Apesar da etiologia incerta, é de fácil diagnóstico e possui opções de manejo clínico e cirúrgico.

Introduction: Congenital pulmonary emphysema (EPC) is a rare disease, with an incidence of 1:20-30 thousand births, it is more common in males than in females, in a ratio of 3:1 and its etiology remains unknown. One third of the cases are symptomatic at birth and practically all are diagnosed in the first six months of life. Case Report: Newborn (NB) male, developed early respiratory distress, being referred to the intensive care unit. After using continuous positive airway pressure (CPAP) and a 12-hour oxygen nasal catheter, it resulted in a good breathing pattern and good saturation. After performing computed tomography, EPC was diagnosed. Conclusion: EPC is a rare pathology and should be suspected in newborns with respiratory distress, with importance being given to the various possible differential diagnoses. Despite its uncertain etiology, it is easy to diagnose and has options for clinical and surgical management.

Fatal congenital lobar emphysema in a puerpera: a case report and literature review.

BACKGROUND: Congenital lobal emphysema (CLE) is a developmental lung abnormality usually diagnosed in the neonatal period and is rarely observed in adults. Adults with CLE are usually asymptomatic and only a small fraction may present with coughing, recurrent pneumonia and respiratory distress. In imaging studies, the most frequently affected lobe of CLE is the left upper lobe, followed by the right middle lobe. However, multilobar involvement with severe mediastinal shift is extremely rare. CASE PRESENTATION: We report a case of fatal CLE in a 28-year-old puerpera with postpartum respiratory failure. Chest computed tomography (CT) revealed emphysema of the right upper, middle and lower lobes resulting in adjacent atelectasis. Hyperinflation of the right upper lobe crossed the midline, leading to a deviation of the mediastinal structure to the left hemithorax and severe compression of the left lung. CONCLUSIONS: Early and timely diagnosis of CLE with routine follow-up is necessary for patients. CLE, especially with multilobar involvement or mediastinal shift, could be life-threatening and should be promptly and aggressively treated to prevent severe complications.

Bronchopulmonary dysplasia in extremely premature infant with congenital lobar emphysema: a case report.

BACKGROUND: Congenital lobar emphysema (CLE) is a congenital pulmonary cystic disease, characterized by overinflation of the pulmonary lobe and compression of the surrounding areas. Most patients with symptoms need an urgent surgical intervention. Caution and alertness for CLE is required in cases of local emphysema on chest X-ray images of extremely premature infants with bronchopulmonary dysplasia (BPD). CASE PRESENTATION: Here, we report a case of premature infant with 27 + 4 weeks of gestational age who suddenly presented with severe respiratory distress at 60 days after birth. Chest X-ray and computed tomography (CT) indicated emphysema in the middle lobe of the right lung. The diagnosis of CLE was confirmed by histopathological examinations. CONCLUSIONS: Although extremely premature infants have high-risk factors of bronchopulmonary dysplasia due to their small gestational age, alertness for CLE is necessary if local emphysema is present. Timely pulmonary CT scan and surgical interventions should be performed to avoid the delay of the diagnosis and treatment.

Congenital Lobar Emphysema: Perioperative Evaluation and Management.

BACKGROUND: Congenital lobar emphysema (CLE) is a lung malformation characterized by overdistension and air trapping in the affected lobe. It is one of the causes of neonatal and infantile respiratory distress. This study aimed to evaluate our experience regarding perioperative and surgical management in children with CLE. METHODS: A retrospective observational study was done for all CLE patients who underwent surgery at Menoufia University Hospital. Perioperative data collected included demographic, clinical, and radiological findings, as well as operative and postoperative data. RESULTS: We included 30 neonates and infants who suffered from CLE between January 2013 and December 2020; the mean age was 111.43 ± 65.19 days, and 21 were males. All cases presented with respiratory distress; 19 had cyanosis, and 15 had recurrent pneumonia and fever. Plain chest x-ray and computed tomography (CT) revealed emphysema in all cases. Lobectomy was done in all cases; the mean age at surgery was 147.58 ± 81.49 days. Postoperative complications occurred in 5 patients, and 2 of them needed mechanical ventilation. The follow-up duration ranged from 3 months to 1 year (except 1 case lost to follow-up after 3 months), and all patients were doing well. CONCLUSION: CLE is a rare bronchopulmonary malformation that requires a high index of clinical suspicion, especially in persistent and recurrent infantile respiratory distress. Chest CT is the most useful diagnostic modality. Early management of CLE improves outcome and prevents life-threatening complications. Surgical management is the treatment of choice in our center, without recorded mortality.

Surgical management of congenital lobar emphysema associated with CHD.

Congenital lobar emphysema is often associated with CHD in early infancy; however, the surgical strategy for this condition is still controversial. We report three successful cases of emphysematous lung lobectomy before the surgical repair of associated CHD. Aggressive lobectomy preceding cardiac interventions is advised when the management of congenital lobar emphysema is difficult.