Congenital Absence of a Lung Diagnosed before Death

Congenital absence of a lung is looked upon as a rare occurrence. To substantiate this claim, a review of the literature reveals only 38 authenticated cases since Haberlein (2) reported the first example in 1787. The rarity of this condition is further borne out by the fact that only 3 of the recorded cases were diagnosed before death and in a number of instances aplasia of the lung was not suspected but was found at routine autopsy following death from trauma. Hurwitz and Stephens (5), reporting a case in 1937, tabulated details of the 34 cases recorded up to that time, with references to the original reports. The accompanying table brings their review to date. The feeling persists, however, that aplasia of the lung occurs much more frequently than is generally suspected and the possibility of such an occurrence should be considered in differential diagnosis of diseases of the chest, particularly in what appears to be a persistent atelectasis. One normally functioning lung is compatible with and sufficient for life and all ordinary needs, provided the lung remains healthy. The decrease in respiratory reserve is often offset by an increase in pulmonary tissue in the opposite lung, and instances have been reported where six lobes have been present. Compatibility of a single lung with life is further borne out by the fact that 11 of the eases reported were in patients twenty years of age or older. Herrero (4) reported a case in a man of sixty-five. Van Loon and Diamond's (7) patient was a child three and one-half years old who at the present time is still alive. Schneider (6) recognizes three main types of pulmonary agenesis. The first type is a true aplasia with no trace of lung, bronchus, or vascular supply on the affected side. The second type is characterized by a small out-pouching from the trachea, with rudimentary bronchus. In the third type the bronchus is fully formed but ends in a fleshy mass of areolar tissue of varying amount. The different types of pulmonary agenesis may be explained on an embryological basis. The lungs arise at the end of the fifth week as two bronchial buds which evaginate from the primordial trachea. These buds branch, forming the future bronchial tree. Failure of the lung to develop may be due to an abnormality of the anlage of the lung or interference with its development or to regressive changes occurring at any time during the development of an anlage that had a normal beginning. The left lung seems to be absent more frequently than the right lung. The incidence of the condition is about the same in either sex. The following case is the second of congenital absence of a lung, seen in this hospital in the last six years (1). Both were diagnosed before death. Case History J. M., male, aged six .months, was admitted to Children's Mercy Hospital, Kansas City, Mo., Nov. 22, 1942, with a history of a cold, with cough and some cyanosis, of eight days' duration.