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The lung following repair of congenital diaphragmatic hernia


, : The lung following repair of congenital diaphragmatic hernia. Journal of Pediatrics 90(3): 405-414

To determine the effects of the pulmonary hypoplasia present at birth in infants with congenital diaphragmatic hernia upon subsequent development of the lung, 19 patients who had undergone surgical repair before the age of 1 yr were studied at ages 6-18 yr. Total lung capacity and vital capacity averaged 99% of predicted value. Diffusing capacity for CO was normal. Forced expiratory volume in 1 s averaged 89% of predicted value and 80% of vital capacity. Total respiratory system conductance and maximum expiratory flow volume curves obtained during air and He-O2 breathing were normal. 133Xe radiospirometry performed in 9 patients revealed equal distribution of lung volumes on the 2 sides. Ventilation to the hernia side was reduced in only 2 patients. Blood flow to the hernia side was reduced in all 9 patients. Chest radiographs supported the physiologic observations. These findings are consistent with the persistence of a reduction in the number of branches or generations of pulmonary arteries and bronchi on the side of the hernia. Since a substantial part of the vascular resistance resides in peripheral vessels, this developmental abnormality influences the distribution of pulmonary blood flow, although it has little effect on tests reflecting airway resistance or the distribution of ventilation.

Accession: 006712401

PMID: 839333

DOI: 10.1016/s0022-3476(77)80702-6

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Related references

Wilcox, D.T.; Glick, P.L.; Karamanoukian, H.; Rossman, J.; Morin, F.C.; Holm, B.A., 1994: Pathophysiology of congenital diaphragmatic hernia. V. Effect of exogenous surfactant therapy on gas exchange and lung mechanics in the lamb congenital diaphragmatic hernia model. The aim of this study was to assess the impact of surfactant deficiency on the pathophysiology of congenital diaphragmatic hernia (CDH). Pregnant ewes were operated on at 80 days of gestation for creation of a diaphragmatic hernia in the lambs. Tw...

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Freyschuss, U.; Lännergren, K.; Frenckner, B., 1984: Lung function after repair of congenital diaphragmatic hernia. To study lung development after repair of congenital diaphragmatic hernia 20 patients operated on between 1960 and 1976 were followed-up at 6-22 years of age. All had had an uneventful postoperative course, had no concomitant disease and were subj...

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Benoist M.R.; Cloup I.; Jean R.; Rufin P.; Scheinmann P.; Paupe J., 1982: Lung function after surgical repair of congenital diaphragmatic hernia. Goetz, M H And O B Stur (Ed ) Progress in Respiration Research, Vol 17 Paediatric Respiratory Disease; 2nd Annual Meeting Of The European Paediatric Respiratory Society, Baden/Vienna, Austria, Oct 9-12, 1980 Ix+306p S Karger: Basel, Switzerland; New York, N Y , Usa Illus P244-251, 1981

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Hislop, A.; Reid, L., 1976: Persistent hypoplasia of the lung after repair of congenital diaphragmatic hernia. Quantitative analysis has been used to assess growth in a lung from an infant aged 21/2 months in whom a diaphragmatic hernia was repaired at birth. The lungs had been abnormally small at birth but at 21/2 months were of normal volume. Alveoli had...

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