Congenital lobar emphysema concurrent with pneumothorax and pneumomediastinum in a dog.

A two-year-old castrated male Pomeranian dog was referred with the chief complaints of coughing and subcutaneous emphysema. On physical examination, the crepitant areas were palpable. When auscultated, the right chest was absent of respiratory sound, while the sound of the opposite side was enhanced. Radiographs presented pneumothorax and pneumomediastinum. On computed tomography, hypoattenuated bulla-like lesion at right middle lung lobe and trapped air in mediastinum were shown. After patient stabilization, surgery for excision of affected lobe was performed. During follow-up period, there were no recurrence and complication on radiographic examination. Based on clinical and pathological findings, the dog was diagnosed as congenital lobar emphysema.

Enfisema Lobar Congénito, reporte de dos casos / Congenital Lobar Emphysema, Two cases report

El enfisema lobar congénito es una entidad poco común. No existe una etiología clara y se ha descrito que la causa más común es la displasia del cartílago bronquial. El diagnóstico clínico es difícil de realizar en tanto que la tomografía multidetector, así como el estudio histopatológico pueden definir el diagnóstico. Presentamos los casos clínicos de dos neonatos de sexo masculino con clínica similar a la de una neumonía.Describimos el proceso con el cual llegamos a la conclusión diagnóstica, su manejo, pronóstico y una breve revisión de la literatura.

Congenital lobar emphysema is a rare entity; there is no clear etiology and it has been described that the most common cause is the bronchial cartilage dysplasia. Clinical diagnosis is difficult to perform and multidetector computed tomography either the histopathological study can define the diagnosis. We present two cases of two male neonates with similar symptomatology than pneumonia. We describe the process that lead us to the diagnostic conclusion, management, prognostic and we present a brief review of the literature.

VATS for congenital lobar emphysema: a case report.

UNLABELLED: Congenital lobar emphysema (CLE) is a rare congenital lung disease consisting in overinflation of a pulmonary lobe. Adult onset of CLE is therefore unusual, often presented with mild symptoms. The authors report a very uncommon case of congenital segmental emphysema diagnosed in a 21-year-old non-smoking man because of recurrent right pneumothorax. Indication to pulmonary resection was established according to functional limitation, radiological findings of right upper lobe segmental emphysema with corresponding bronchial agenesia, scintigraphic result of extremely reduced ventilation and perfusion of lung emphysematous area and recurrency of pneumothorax. The intervention was carried out by 3-portal video-assisted thoracic surgery (VATS) using single-lung ventilation leading to determine precisely how much lung to resect thanks to the obvious and clear-cut distinction between functioning and non functioning parenchyma of the upper lobe. A stapler wedge resection by VATS was thus obtained, that, as far as the author's knowledge, it is the first case of endoscopic parenchymal sparing resection in CLE. Even though congenital lobar emphysema is rare, clinical awareness of this condition is important for early diagnosis and effective surgical treatment that in this case led to favourable RESULTS: The VATS procedure seems to be an advantageous approach.

Congenital lobar emphysema: differential diagnosis and therapeutic approach.

BACKGROUND: Congenital lobar emphysema (CLE) is a rare anomaly of lung development that usually presents in the neonatal period with respirator distress and pulmonary lobar hyperinflation. It is commonly confused with pneumothorax. The aim of the present paper was to review the authors' experience in order to emphasize the importance of differential diagnosis with pneumothorax. METHODS: Children with CLE treatment at Department of Thoracic Surgery, Dicle University School of Medicine, Turkey, between January 1993 and June 2004, were reviewed. RESULTS: Ten children consisting of six boys and four girls (age range, 6 h-12 months) had CLE. Major presenting symptoms were tachypnea(n = 100%) and respiratory distress in (n = 80%). On chest radiograph, emphysema was seen in all patients, and shift-herniation to the opposite lung, atelectasis were observed. Computed tomography was performed in all patients, which indicated emphysema in the affected lobes in all cases. Pulmonary perfusion scan was performed in two patients, showing loss of perfusion in the affected lobe. The most common affected lobe was the left upper lobe (50%). In the present series, three patients were mistakenly diagnosed as pneumothorax and intercostal drains were inserted in the emergency department. Eight patients underwent lobectomy, and postoperative course was uneventful. Two patients were followed conservatively. Emphysema was detected in all pathological specimens. One patient was lost to follow up. Mean follow-up duration of all patients was 26.8 +/- 29.24 months (range, 1-89 months). CONCLUSIONS: CLE is established on combined clinical, radiological and scintigraphic imaging. Surgical excision of the affected lobe is the appropriate treatment. Particularly, differential diagnosis should be made between CLE and pneumothorax.

HRCT in small and large airways diseases.

High-resolution computed tomography (HRCT) is being increasingly used in the diagnostic work-up of paediatric patients with large and small airways disease due to its ability to provide valuable information far beyond that of other non-invasive investigations. This article highlights the key HRCT appearances of a range of conditions involving the airways in children, and where relevant, the role of HRCT in assessment of disease severity and monitoring of disease progression.

Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology.

Congenital anomalies of the chest are an important cause of morbidity in infants, children, and even adults. The evaluation of affected patients frequently requires multiple imaging modalities to diagnose the anomaly and plan surgical correction. The authors analyze and illustrate practical aspects of certain common and uncommon congenital anomalies affecting the tracheobronchial tree, lung, and mediastinum, with emphasis on radiologic manifestations. Other thoracic anomalies such as rib anomalies and vascular rings are discussed when they are associated with anomalies of the tracheobronchial tree. The usefulness of the various imaging modalities in the diagnosis and treatment of these conditions is also evaluated. Specific topics addressed include tracheal conditions such as tracheal stenosis, tracheomalacia, tracheal bronchus, tracheal atresia, and bronchogenic cyst; anomalies of the lung such as lung underdevelopment (agenesis and hypoplasia), scimitar syndrome, congenital cystic adenomatoid malformation, congenital lobar emphysema, and pulmonary sequestration; esophageal anomalies such as esophageal atresia, tracheoesophageal fistula, and esophageal duplications; and vascular rings. The embryologic and pathologic basis of the radiologic findings are discussed in appropriate cases. Differential diagnoses, as well as pitfalls and diagnostic difficulties, are included.

[Congenital lobular emphysema. Eight case reports]. / L'emphyseme lobaire congenital. A propos de 8 cas.

We have conducted a retrospective study about 8 infants having CLE and who were hospitalised for 11 years in the Pediatric department of Sfax university hospital (1989-1999). The average age of these patients having revealing symptoms ranges from birth to 8 months, with an average age of 2 months and 3 weeks. During the neo-natal period (< 1 month), the disease was found among 35.5% of the patients. The discovery circumstances are represented by a permanent dyspnea in 4 cases, repetitive bronchopneumopathies with paroxystic dyspnea in 2 cases, a prolonged bronchopneumopathy in one case and a whooping cough in one case. The pre-operatory diagnosis was suspected on the chest-radiography in all cases and on the chest scanner in 7 cases. All patients have undergone a surgical treatment. The anatomy-pathological exam has confirmed the diagnosis in all cases. The immediate post-operatory results were simple in all the 8 cases and the long-term evolution has shown minor respiratory and orthopedic defects only in one patient aged 8 at present. The CLE is a lung-malformation often responsible for serious respiratory problems. The symptomatic forms should be operated very early because the ulterior "prognosis" depends on the patient's age at the moment of the surgery.

Combined sequestration, bronchogenic cyst, and dysgenetic lung simulating congenital lobar emphysema.

We report the case of a newborn with bronchopulmonary sequestration, bronchogenic cyst, and dysgenetic lung that radiographically simulated congenital lobar emphysema. We discuss congenital malformations of the lungs and the concept of a continuum in the development of pulmonary anomalies.

Enfisema lobar congénito: reporte de nueve casos y revisión de la literatura / Congenital lobar emphysema: report of nine cases and review of the literature

El enfisema lobar congénito (ELC), es una causa importante de insuficiencia respiratoria en los primeros meses de la vida. Los datos clínicos más importantes son taquipnea, cianosis intermitente y asimetría torácica debido al abombamiento del hemitórax afectado. La radiografía de tórax revela un área hiperinsuflada, los lóbulos adyacentes están comprimidos, el contenido mediastinal puede estar desplazado y el pulmón contralateral parcialmente colapsado. La información sobre procedimientos diagnósticos y el tratamiento del ELC en la edad pediátrica es limitado. Presentamos nueve casos con diagnóstico de ELC vistos durante los últimos 10 años en el Instituto Nacional de Enfermedades Respiratorias.

Alpha 1-antitrypsin-deficiency-related emphysema.

BACKGROUND: A congenital cause of emphysema resulting from alpha 1-antitrypsin (A1AT) deficiency affects 1 in 2500 individuals and could account for emphysema in 2 percent of all persons with emphysema. Individuals aged 30 to 45 years with chronic shortness of breath and coughing could have A1AT deficiency. METHODS: Using the key words "alpha 1-antitrypsin deficiency," "chronic obstructive pulmonary disease," and "emphysema," the MEDLINE files were searched from 1985 to the present. Data from articles published before 1985 were accessed from cross-reference of the recent articles. RESULTS AND CONCLUSIONS: Unlike smoker's emphysema, A1AT deficiency is panacinar, appears in middle-aged patients, and is more severe at the lung bases. Chronic bronchitis, mucous hypersecretion, and liver disease, as well as a family history of emphysema, are associated conditions. Clinical management includes the avoidance of smoking and atmospheric pollution. Also available is purified, functional human A1AT in quantities large enough for intravenous replacement or augmentation therapy. Future treatment for the disease includes synthetic elastase inhibitors and an aerosolized formulation of A1AT, which is currently under investigation.

Congenital lobar emphysema requiring surgery in adult life.

We report a case of congenital lobar emphysema requiring surgery in early adult life to relieve respiratory symptoms of late onset. Regional lung function studies helped to predict the outcome of surgery which was successful in relieving symptoms.

Congenital lobar emphysema.

The aetiology of congenital lobar emphysema is not always evident. In the group with demonstrable check-valve mechanism, which allows the air to enter but not to leave the lung, there is either internal stenosis or external compression of the bronchus. When no cause can be found, the condition is called idiopathic, although in some cases alveolar fibrosis has been demonstrated, the check-valve mechanism being in these cases at an alveolar level. In the small group of rare cases of bronchial atresia, air which enters through a collateral ventilation cannot be removed by the same route; in these case too, the check-valve mechanism exists at the alveolar level. Five cases of "congenital lobar emphysema" are presented. One case showed no bronchial anomaly; another case showed an increase in interstitial connective tissue in the lung; tow cases showed hypoplasia or absence of bronchial cartilage; in one case, bronchial atresia was found at operation. Infants show a typical symptomatology of dyspnoea and cyanosis, and a typical chest X-ray with unilateral radiolucency and a delicate lung pattern, collapse of surrounding lung tissue, and mediastinal hernia. In older children, the diagnosis is made either incidently or following a complication. The condition is usually found in the left upper and the right middle lobe. Treatment is surgical and consists of resection of the emphysematous segments.