VACTERL-association: case report

Abstract

Background: VACTERL is a rare association of congenital abnormalities with incidence 1/10 000 to 1/40 000 live births. VACTERL association is typically defined by the presence of at least three of the following congenital malformations: vertebral defects, anal  atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. We are presenting a case report of natural delivery of live born full-term fetus with VACTERL association. Our aim is to emphasize the role of prenatal diagnostics of fetal malformations in order to avoid deliveries of polymalformative fetuses with poor prognosis and mental trauma of the parents. Methods: A 18-year-old primiparous woman in 38th gestational week was admitted in the department because of spontaneous rupture of amniotic membranes and irregular uterine activity. History of the patient indicated insufficient monitoring during the pregnancy. Ultrasound examination showed hypotrophic and polymalformative fetus. The amount of amniotic fluid was increased despite of definite rupture of amniotic sac. Presence of only two umbilical vessels was established. Results: Regardless of the manifestation of fetal distress during the delivery, medical council decided that cesarean section was unjustified because of unsecure prognosis of the newborn and the labor should be completed vaginally. Hypotrophic male with multiple congenital malformations was born that required surgical intervention in the postpartum period. Lethal outcome occurred due to multiple organ failure. Conclusions: The prognosis of VACTERL is determined by the number, severity, and nature of abnormalities present. The ultrasound can identify not only the defects but also specify the prognosis of the fetus.