Congenital Lobar Emphysema

The term congenital lobar emphysema was used by Robertson and James (1951) to denote infantile emphysema when pulmonary infection or intrabronchial foreign bodies are absent. Although in most instances only one lobe is affected, a few patients have emphysema of more than one lobe or subsequently develop emphysema of a second lobe (Kernan, 1944; Ehrenhaft and Taber, 1953; Sloan, 1953; Potts, Holinger and Rosenblum, 1954; Morse and Gladding, 1955; Holzel, Bennett, and Vaughan, 1956; Nelson, 1957; Contro, Miller, White, and Potts, 1958a and b; Sherman, 1959). However, as these multilobar cases are exceptional and as many authors have used the term congenital lobar emphysema, its adoption for this relatively specific clinical condition seems advisable. Typical cases clinically exhibit either remittent or progressive dyspnoea and cyanosis, associated with severe unilateral emphysema and displacement of the mediastinum to the opposite side. Wheezing chest sounds are frequent and the temperature may be elevated. In several cases the condition of the infant is so critical that urgent surgical thoracic decompression by lobectomy is necessary. The symptoms often arise at or shortly after birth; their onset is rarely delayed beyond the sixth month. In the present paper one case is described with a method of histological investigation which was found to be useful in elucidating its basic pathology.