Anatomic and functional asplenia—Absence of the splenic image during 99mTc-sulfur colloid scintigraphy

Abstract

A 2860-g term female infant was admitted to the University of California, Davis, Medical Center, Sacramento, Calif., Neonatal Intensive Care Unit for evaluation of congenital heart disease. The infant was the product of a spontaneous uncomplicated delivery that followed a routine prenatal course and routine delivery. One and five minute Apgars were 7 and 9, respectively. Physical examination showed a term infant with acrocynosis at rest and central cyanosis with crying. A grade 3/6 systolic murmur was heard at the right sternal border and the PMI and heart sounds were in the right chest. A chest x-ray showed dextrocardia (Fig. 1). Cardiac catheterization revealed dextrocardia with a single large ventricle and an atrial septal defect. A 99roTe-sulfur colloid liver-spleen scan was obtained (Fig. 2). The asplenia syndrome first described by Ivemark in 1955 occurs in association with a variable constellation of congenital cardiovascular, pulmonary, and abdominal anomalies/'2 During embryogenesis, at the 31-36 day of human embryo gestation, there is the appearance of splenic primordia, fusion of atrioventricular canal cushions, septation of the conotruncus, lobation of the lungs, and rotation of the gastrointestinal tract; 3 hence, the association of asplenia, congenital anomalies of the heart, right-sided trilobed pulmonary symmetry, malrotation of the intestine, and symmetrical liver.' .2 Failure to image the spleen on 99roTe-sulfur colloid scanning may relate to either anatomic or functional asplenia. 4 ~4