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.

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.

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.

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.

One-Stage Bilateral Lobectomy in an Infant with Bilateral Congenital Lobar Emphysema.

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.

Congenital Lobar Hyperinflation - A rare anomaly misdiagnosed as bronchiolitis: A case report.

Congenital Lobar Hyperinflation is an overinflation of one or more than one lobes of the lung and is caused by an abnormal development of broncho-pulmonary segment. We report a case of a 2-month-old female who presentedin outpatient department with complaints of fever, cough and difficulty in breathing. This case was seen in January, 2018. Congenital Lobar Hyperinflation was diagnosed on chest x-rays and Computed Tomography (CT ) scan. Immediate surgical excision (lobectomy) of the affected lobe was conducted.

Congenital Lobar Overinflation: A Rare Enigmatic Lung Lesion on Prenatal Ultrasound and Magnetic Resonance Imaging.

OBJECTIVES: To report the ultrasound (US) features in prenatal cases of suspected congenital pulmonary airway malformation or unspecified lung lesions with a final surgical pathologic diagnosis of congenital lobar overinflation (CLO). METHODS: Institutional Review Board-approved radiology and clinical database searches from 2001 to 2017 were performed for prenatally diagnosed lung lesions with a final diagnosis of CLO. All patients had detailed US examinations in addition to magnetic resonance imaging (MRI). Size, echotexture, and vascularity were assessed with US, and the signal and vascularity were assessed with MRI. Follow-up prenatal US scans, postnatal imaging, and postnatal outcomes were reviewed. RESULTS: The study population consisted of 12 patients. The median gestational age was 23.3 weeks. The median congenital pulmonary airway malformation volume-to-head circumference ratio was 0.66. Lesion locations were 6 in the lower lobes (4 right and 2 left), 5 in the upper lobes (3 left and 2 right), and 1 in the right middle lobe. The texture was homogeneously echogenic relative to the normal lung in 100% with no visualized macrocysts. Hypervascularity by color Doppler US was observed in 5 cases (41.7%). A T2 hyperintense lung lesion was identified by MRI in 12 of 12 cases (100%), with elongated vessels identified in 11 of 12 cases (91.7%). All 12 cases had pathologically proven CLO. CONCLUSIONS: Congenital lobar overinflation should be considered in cases of prenatal echogenic lung lesions without macrocysts or classic findings of bronchial atresia. Hypervascularity may be an important imaging feature of a subset of CLO. Most cases become less conspicuous, decrease in size without overt hydrops, and are asymptomatic postnatally.

Clinical presentation and outcome in congenital pulmonary malformation: 25 year retrospective study in Thailand.

BACKGROUND: Congenital pulmonary malformations (CPM) are a group of rare abnormal lung development lesions that can have various presentations. The aim of this study was to define the differences in the clinical presentations of CPM in neonates, infants, and children, and to review the outcomes. METHODS: A retrospective study was conducted at a tertiary care hospital in southern Thailand between 1992 and 2016. RESULTS: Fifty-four patients were diagnosed with CPM, and the median age at onset was 1.7 months (IQR, 0.03-10 months). There were 33 cases (61.1%) of congenital pulmonary airway malformations, two (3.7%) of bronchogenic cyst, eight of (14.8%) congenital lobar emphysema, seven of (13.0%) pulmonary sequestrations, and four of (7.4%) congenital lung cysts. Twenty patients under 1 month old and 16 patients who were 1-12 months old had symptoms of respiratory distress. In contrast, 13 patients >1 year old had symptoms of pulmonary infection. There were significant differences in the numbers of patients who had cyanosis (P = 0.006), cough (P < 0.001), and fever (P < 0.001) between the three age groups. Thirty-eight patients (70%) required surgical treatment involving lobectomy (78.9%). Median follow-up duration was 28.1 months (IQR, 3.7-9.4 months). Nine of 10 patients had abnormal lung function tests, and 80.6% of patients had no subsequent limitations in physical activities. CONCLUSIONS: Respiratory distress is the important clinical feature in neonates and infants, whereas the signs of pulmonary infection usually occur in children >1 year old. Good outcomes usually occur after surgery but need long-term follow up including lung function assessment.

Variability of presentation and surgical approach in Congenital Cystic Lesions of Lung: A retrospective study in children presenting in Mayo Hospital.

The objective was to study the clinical presentation and surgical outcome in children with congenital cystic lesions of the lung. The medical records of 11 patients operated in the department of paediatric surgery, from January 2014 to December 2017, were evaluated retrospectively. Median age was 18 months (1-108).Respiratory distress was seen in 5 (45.4%) patients, recurrent chest infections in 4 (36.4%) patients and only 2 (18.2%) presented after birth. One patient of congenital lobar emphysema was misdiagnosed as pneumothorax and four patients of recurrent chest infection had been misdiagnosed as pulmonary tuberculosis. All patients underwent lateral thoracotomy. There was no mortality, median length of hospital stay was 4 days (4-5) and only one patient needed postoperative ventilation. On follow up, 10 (90.9%) patients had attained normal level of physical activities. We conclude that increasing awareness of these lesions can prevent misdiagnosis and unnecessary tube thoracostomy and anti-tubercular therapy in children.

A review of congenital lung malformations with a simplified classification system for clinical and research use.

PURPOSE: Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. MATERIALS AND METHODS: We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. RESULTS: The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. CONCLUSIONS: Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.

Congenital lobar emphysema.

Congenital lobar emphysema is a rare variety of congenital malformation of lung characterized by over distension of a lobe of the lung due to partial obstruction of the bronchus. It is one of the rare causes of infantile respiratory distress requiring surgical resection of affected lobe. We are reporting the case of an 8-week-old baby who presented with respiratory distress related to CLE affecting the left upper lobe. Lobectomy was performed under general anaesthesia followed by an uneventful recovery.

Risk of respiratory syncytial virus infection in infants with congenital cystic lung disease.

BACKGROUND: Congenital cystic lung disease (CCLD), which includes congenital cystic adenomatoid malformation, bronchopulmonary sequestration, and congenital lobar emphysema, has been reported to increase the risk of recurrent respiratory infection. In particular, respiratory syncytial virus (RSV) causes severe lower respiratory tract disease in high-risk infants. The objective of this study was to investigate the risk of severe RSV infection in infants with CCLD. METHODS: Infants antenatally diagnosed as having CCLD and admitted to a neonatal intensive care unit at the National Center for Child Health and Development in Tokyo between September 2002 and October 2011 were included in this study. We investigated retrospectively whether the infants were hospitalized with RSV infection by 24 months of age using their medical records. RESULTS: Forty-eight infants were antenatally diagnosed as having CCLD. Of the 48 infants, four (8.3%) were hospitalized with RSV infection by 24 months of age. CONCLUSIONS: Infants with CCLD have increased risk of severe RSV infection.

Congenital lobar emphysema: A modified approach to anesthetic management.

Congenital lobar emphysema (CLE) is a potentially reversible, though life-threatening cause of respiratory distress in neonates. It is a rare developmental anomaly of the lower respiratory tract. A 10-month-old child presented with fever, cough and difficulty in breathing. Respiratory system examination revealed tachypnea with intercostal retraction, decreased breath sounds over the left upper lobe and room air saturation of 95%. Chest radiograph and computed tomography showed hyperinflated left upper zones with mediastinal shift. Left thoracotomy, followed by left upper lobectomy was performed under general anesthesia with one lung ventilation. Oxygen saturation, blood pressure, electrocardiogram and capnogram were continuously monitored. During anesthesia, conventionally positive pressure ventilation is avoided until the diseased lobe is isolated, however we adopted gentle manual ventilation maintaining the airway pressure before thoracotomy as described by Coté and Payne et al. Successful anesthetic management of CLE can be achieved by proper understanding of pathophysiology, good perioperative monitoring, and adopting novel management strategies.

Recurrent pneumonia in a 2-year-old: A rare case of congenital lobar emphysema.

INTRODUCTION AND SIGNIFICANCE: Emphysema is an uncommon but important condition that often appears in the neonatal period. Diagnosis is based on CT, which identifies the affected lung lobe, which is treated with complete surgical resection. CASE PRESENTATION: We present a case of a child who had been suffering for about a year from recurrent respiratory infections without arriving at a clear and correct diagnosis. He was evaluated by us and diagnosed correctly despite the difficulty of distinguishing it from pneumothorax. The final treatment was surgical removal. CLINICAL DISCUSSION: Emphysema is considered one of the important conditions that should be considered as a differential diagnosis if there is clear hyperinflated in the pulmonary lobe. The evaluation is mainly done through CT to reach the correct diagnosis and treatment. CONCLUSION: Congenital lobar emphysema is a rare condition that primarily affects children. The majority of children with CLE experience symptoms and necessitate surgery.

A rare case of congenital lobar emphysema presenting as recurrent pneumonia in early adulthood: A case report and literature review.

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.

A rare case of symptomatic congenital lobar emphysema in an adolescent female who underwent expectant management of a congenital pulmonary malformation: a case report.

Background: Asymptomatic congenital pulmonary airway malformations (CPAMs) and congenital lobar emphysema (CLE) may safely be observed, though little is known about how many patients later require surgery. The management of these lesions remains controversial, as limited research exists on which patients later require surgery. Notably, there are few reported cases where patients become symptomatic and require definitive treatment beyond childhood. Case Description: We present the rare case of a 17-year-old female who developed dyspnea on exertion associated with pre-syncope, following diagnosis of a CPAM at birth. She had undergone surveillance until 2 years of age. The decision to proceed with a left upper lobectomy via thoracotomy was made, for treatment of her symptoms. During the surgery, one lung ventilation suggested that oxygenation had likely limited to the patient's healthy lung, prior to intervention. The surgery was uncomplicated, and her recovery was uneventful. Pathology revealed CLE. Conclusions: This case highlights that congenital lung malformations should remain in the differential diagnosis of patients presenting in adolescence or adulthood with new onset respiratory symptoms. For patients with a history of untreated congenital lung pathology, it is important to consider the presence of adhesions and loss of domain in perioperative planning. This case also highlights the ongoing need for research on the prognostication of these lesions to better inform surveillance.

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.