Report of a case with congenital absence of the spleen and laevocardia.

Congenital absence of the spleen is a rare anomaly. The first case to be described in infancy is that of Martin (1826). At necropsy a partial situs inversus of the viscera and congenital malformations of the heart were found. Polhemus and Schafer (1952) reviewed the literature up to 1952 and found 12 cases of congenital absence of the spleen in infants. To these they added four cases of their own. In all cases congenital malformations of the heart were also present. They consisted of pulmonary stenosis or atresia, anomalies of the great vessels and atrioventricularis communis. In eight of the 16 cases there was partial or total situs inversus of the viscera, while the heart was on the left side. This syndrome is known as laevocardia. In a recent article Bush and Ainger (1955) found five additional cases of congenital absence of the spleen in infants in the literature, to which they added one of their own. It has been possible for us to ascertain the presence of laevocardia in only two of these cases, the authors' own patient and one of the patients described by Gasser and Willi (1952), the remaining bibliography not being available to us. Bush and Ainger (1955) drew attention to the characteristic blood picture of their patient which permitted the ante-mortem diagnosis of the absence of the spleen. The picture is similar to the one observed in patients who have undergone splenectomy and is characterized by the presence of Howell-Jolly bodies and Heinz bodies in the red cells, normoblastaemia, target cells, and siderocytes. The only two other cases of splenic agenesis diagnosed before death are those of Gasser and Willi (1952). The diagnosis was made on the basis of the haematological findings. In reviewing the literature on splenic agenesis we have come across two other cases in infants, recently described by Baumann (1954). Both had an associated partial situs inversus of the viscera and anomalies of the heart. The position of the heart was normal in one of the cases. The number of established cases of splenic agenesis in infants is therefore 24 up to the present day. In 11 of them at least laevocardia was present. In adults splenic agenesis has usually been an isolated finding although in some other minor congenital anomalies have also been present. According to Bush and Ainger (1955) 10 adult cases have been reported in the literature. The purpose of this paper is to present another case of splenic agenesis which was suspected before death on the basis of the blood picture and to call attention to the frequency with which laevocardia is associated with this anomaly.