Intestinal obstruction in asplenia syndrome: report of three cases.

Congenital absence of spleen is rare. When it does occur, it may be associated with a characteristic group of anomalies of the cardiovascular and gastrointestinal system. Fifteen neonates of asplenia syndrome were seen in our hospital from April 1989 to November 1992, three of these fifteen cases were associated with intestinal obstruction and malrotation. All of the three neonates were noted to have cyanosis, heart murmur and abdominal distention soon after birth. Absence of spleen, heterotaxia and complex cardiac malformations were detected by sonography. Howell-Jolly bodies were found in their peripheral blood smear. The barium examinations of gastrointestinal tract showed intestinal obstruction and malrotation. All of them died in the first month of life. One of them received an autopsy.

Persistent truncus arteriosus associated with interrupted aortic arch: report of two cases.

Two neonates with a combination of truncus arteriosus and interruption of the aortic arch, so-called type A4 of the Van Praagh classification, are reported. Both presented as tachypnea, poor appetite and increasing cyanosis during the first week of life. The combination of these defects significantly increases the surgical risk. Di-George syndrome was noted in one patient, who presented with hypocalcemia and T cell dysfunction. Autopsy confirmed this diagnosis.