Unilateral lobar pulmonary agenesis in sibs

EDITOR—Pulmonary agenesis is an extremely rare congenital malformation which has been classified morphologically by the extent to which bronchopulmonary tissue is absent. Spencer1 divided pulmonary agenesis into (1) bilateral complete agenesis, (2) unilateral agenesis with (a) complete absence of bronchi, (b) rudimentary bronchus present but no pulmonary tissue, or (c) poorly developed main bronchus with poorly organised parenchyma, and (3) lobar agenesis. The incidence of this rare condition is not known. According to the calculations of Mardini and Nyhan2it may range between 0.0034 and 0.0097%. Familial pulmonary agenesis is even more uncommon. We found only two published reports of affected sibs.3 4 One pair of sibs had agenesis of the right upper and middle lobes3 and the other pair suffered from bilateral agenesis.