The TACRD association is distinct from VACTERL association--a case report

Tracheal agenesis is a rare malformation. A case of tracheal agenesis with associated ventricular septal defect, gastric hypoplasia, hiatus hernia, duodenal atresia, isomerism of the lungs, hypoplastic spleen with polysplenia, Meckel's diverticulum and fused crossed ectopia of the kidneys is presented. This case fits into a newly proposed TACRD association, an extremely rare condition composed of tracheal agenesis, cardiac, renal and duodenal malformations. It is distinct from the more common VACTERL association of which tracheo-esophageal fistula and not tracheal agenesis is a part. TACRD association has complex internal malformations and lacks the external skeletal manifestations of VACTERL association. Awareness amongst pathologists conducting neonatal autopsies is necessary for correct identification of this condition.