New pathological findings in emphysema of childhood: 3. Unilateral congenital emphysema with hypoplasia--and compensatory emphysema of contralateral lung

Abstract

A quantitative study is here reported on the lungs of a child of 9 months with unilateral emphysema associated with hypoplasia, and with compensatory emphysema in the contralateral lung. The left lung was judged to show congenital hypoplasia because of the reduced number of bronchi and arteries, its small volume, and a total alveolar number less than that normally found at birth. This last was due mainly to reduction in the number of acini, the intra-acinar number seeming little affected. As the alveoli were eight times the normal volume, the case was considered to be one of congenital hypoplastic emphysema.The right lung was evidently normal at birth, since it had a normal number of bronchi, arteries, and alveoli. The lung was increased in volume due to an increase in alveolar volume-an example of compensatory emphysema.This case is discussed with relation to two previously reported cases of lobar emphysema and other cases in the literature. It is emphasized that a quantitative analysis of the state of bronchi, alveoli, and blood vessels is important to establish the nature of the pathological change. A preliminary classification based on the anatomical features is offered.The degree of growth of airways, alveoli, and arteries in cases of childhood lobar emphysema has recently been assessed quantitatively. In one case the condition was due to a 'polyalveolar' lobe (Hislop and Reid, 1970) and in another to overinflation of a normally developed lobe . In the first paper (Hislop and Reid, 1970) This baby was born on 17 March, 1965. She had a cleft lip and anterior palate. She was seen at 6 months for repair of the lip but only a limited repair was considered possible because of her poor physical condition. At this time she was thought to have an enlarged left ventricle with a loud systolic murmur at the left sternal edge together with a murmur at the apex interpreted as being due to increased blood flow.The radiograph on 3 September, 1965 ( Fig. 1) was in partial expiration, the left dome being low compared with the right, suggesting air trapping in the left lung, although the heart was not displaced: the left lung was hypertransradiant and avascular, these changes being least marked in the medial part of the left upper lobe where the blood flow seemed better but still not normal. The right lung was plethoric, the heart and pulmonary trunk enlarged, and the aortic knuckle small. The apical lower lobe was partially collapsed. Angiography confirmed that the vessels to the right lung were enlarged with dilatation and tortuosity mainly of the proximal intrapulmonary vessels. No medium entered the left lung; an infracristal septal defect was seen.

Persistent hypoplasia of the lung after repair of congenital diaphragmatic hernia.

“…A similar increase has been seen in the right lung in a case with a congenital hypoplastic left lung (Henderson, Hislop, and Reid, 1971). Since airway development is complete in the normal fetus by 16 weeks' gestation (Bucher and Reid, 1961), it seems unlikely that there would be any new growth of airways after birth, and none was evident in the present case.…”

Pulmonary hypoplasia in babies with severe rhesus isoimmunisation: A quantitative study

“…In the other three cases, however, the alveolar differentiation had continued normally despite reduction in airway number but the alveoli were abnormal in size, in one larger and too few in number, in the others smaller than normal. In a case, with congenital reduction in the number of airways, alveolar development in each acinus was normal (Henderson, Hislop and Reid, 1971). Alveolar immaturity has been described in some cases of congenital diaphragmatic hernia, renal malformations and anencephaly (Reale and Esterly, 1973).…”

Polialveolose: patogênese do enfisema lobar congênito?

“…Esse fato explica a hipocaptação registrada pela cintilografia de perfusão e, de maneira semelhante, o "achado" de diminuição da cartilagem pelos autores que utilizaram esse método 3,5,14,15 . Poucos trabalhos 7,15,16,21,22,[24][25][26] pesquisaram a presença de lobo polialveolar em situações de enfisema lobar.…”

“…Usaram a contagem radial para avaliar o número de alvéolos dentro do ácino e compararam com Emery e Mithal 10 . Fizeram uma contraprova, utilizando os mesmos métodos numa torção de brôn-quio, que mimetizava o quadro de enfisema lobar congênito 21 e num enfisema compensatório 22 . Em ambos casos, a contagem foi normal, encontraram diferença no tamanho do alvéolo mas não no núme-ro.…”