Congenital lobular emphysema. It is characterized by emphysematous expansion of a pulmonary lobe with dyspnea and cyanosis that progress slowly or rapidly until severe respiratory distress appears.
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- 1 Pathological anatomy and pathogenesis
- 2 Clinical picture
- 3 Diagnosis
- 4 Roentgenographic signs
- 5 Treatment
- 6 Recommendations
- 7 Forecast
- 8 Sources
Pathological anatomy and pathogenesis
In the operation or at the autopsy, there is the expansion of a pulmonary lobe, generally the upper one, less often the middle of the right side and never the lower ones, which acquires a considerable volume. In general, it has a pinkish and fluffy appearance, similar, according to one author, to a pink “soufflée”. After excision, it retains the appearance of hyperdistension. On microscopic examination, distension of most of the alveoli is found , with coalescence of many of them due to the rupture of the septa and subpleural blisters.
Careful post mortem studies by Reid et al. Have shed light on the variety of findings in this condition. In some infants, the tissue is normal with respect to the number of alveoli and airways, but it is simply overstretched, as by a mucus plug; in others, the number of airways and alveoli is reduced (a hypoplastic overstretched lobe). Radiographically they appear very translucent, but not overstretched. Rarely a lobe can show normal airways, but an excess of alveoli, the so-called polyalveolar lobe, can.
Finally, the atresia of a bronchus can make possible the overdistension of some segments, but little exit of air, causing radiolucency of segments of the lung .
Many lung injuries have been claimed to be the cause of excessive gradual insufflation. Bronchial abnormalities have been reported: folds of the mucosa, decrease, absence or flaccidity of the bronchial cartilages and stenosis due to inflammation, cysts or mucus plugs.
In some cases, extrabronchial compression has been found by aberrant blood vessels, especially by the ductus arteriosus or its residual fibrous cord.
Symptoms generally occur in the newborn period (25 to 33 percent in the newborn period, 50 percent per month, and almost all at 6 months of age).
Progressive respiratory distress may develop in some or be more gradual or insidious in others. The severity depends on the size of the affected lobe, the compression of the lung tissue and the deviation of the newborn. Typically there is transient neonatal tachypnea , increased respiratory effort, and frequently cyanosis. In moderate cases there may be growth failure, recurrent pneumonia and food rejection.
The physical examination revealed breathlessness and wheeze frequently. Lung sounds are decreased near the area compromised with hypersority to percussion. If there is mediastinal displacement, the heartbeat is deviated. Other malformations such as cardiovascular (14 percent), kidney, gastrointestinal, and skin malformations can occur.
The first symptoms appear in a few cases at the moment of birth and in some during the first week; in most they appear between the first week and the first month of life. Its start between the first and the sixth month is exceptional; It is, therefore, an eminently neonatal disease .
In general, the disease begins with attacks of dyspnea and cyanosis, manifestations that soon acquire a continuous and serious character. Complaining expiration is not infrequently observed; vomiting and cough rarely occur .
On examination, general dyspnea was found with painful inspiration, whining or prolonged expiration and wheezing, and an area of hyperresonance was discovered in the upper lobe or in an entire lobe, with great cardiac and mediastinal deviation to the opposite side to that of hyperresonance.
Excessive expansion and clarity in a lobe, usually the upper right or left or right middle, which often exceeds the midline in front of the heart . In the adjacent lobes is observed atelectasis . The heart is deviated to the opposite side to that of the hyper-distended lobe and is generally pushed back by the herniated lung.
Immediate intervention is indicated if the infant has a severe respiratory disorder. After a chest x-ray has been performed , it is preferable that a child surgeon and an otolaryngologist collaborate, the latter to perform an endoscopy on the operating table and the surgeon to perform, if necessary, an exploratory taracotomy.
It is advisable to practice a bronchoscope in those asymptomatic infants in whom a lobular emphysema is accidentally discovered, we have personally preferred to follow the evolution of some infants with this lung condition who, without being operated, have remained asymptomatic.
The perspective after the intervention is generally excellent. Few functional assessments have been made, but DeMuth and Sloan, in 1966 , discovered some persistent decrease in mean expiratory volume in two children , and suggested that the disease is not always limited to the more clearly affected lobes