Surgery for arteriovenous fistulas and dilated vessels in the right lung after the Glenn procedure.

A superior vena cava-right pulmonary artery (SVC-RPA) anastomosis was constructed in a 2-year-old boy with tetralogy of Fallot. Ten years later and 5 years after "corrective" surgery without removal of the shunt, cyanosis and heart failure developed. Stereocineangiography and lung scanning revealed arteriovenous fistulas and dilated vessels in the right lung. The SVC-RPA anastomosis was taken down, the SVC being reimplanted in the right atrium and the RPA end being closed with a few stitches. Neither lobectomy nor pneumonectomy was performed. Immediately after the operation and during a follow-up period of almost 2 years, the boy has remained asymptomatic. Whenever a correction is planned in a patient with SVC-RPA anastomosis, the vessels of the right lung should be examined by scanning and angiography. If important arteriovenous fistulas do exist, the affected lung should be excluded from the pulmonary artery circulation.

The 9p- deletion syndrome. Report of a patient with a 46, XX, 9P- constitution due to a paternal t(9p-;15+) translocation.

A new case of the 9p- chromosome-deletion syndrome is described. The 9p-chromosome, identified by the G-, R-, Q- and G11-banding techniques, showed mainly a deletion of bands p23 and p24. Routine chromosome analysis and banding studies in the parents revealed normal chromosomes in the mother and a balanced t (9p-; 15q+) translocation in the father. The main clinical features of the proband are narrow cranium, prominent forehead, flat occiput, hyperteloris, flat bridge of the nose, long upper lip, micrognathia, low-set and abnormal ears, short, broad neck, wide-set nipples, systolic murmur, umbilical hernia, diastasis musculi recti, short arms and broad thumbs, equinovarus adductus, hypotonia and psychomotor retardation. These clinical findings are compared with those of the three 9p- cases found in the literature.