Ndolo Josephine,  Crane Gabriella,  Hernanz-schulman Marta

Final Pr. ID: Poster #: CR-001

Introduction
Scimitar syndrome and subdiaphragmatic total anomalous pulmonary venous connections (TAPVC) are congenital pulmonary vascular anomalies that are not typically diagnosed on routine abdominal sonography. We present three cases of ultrasound diagnosis of neonates with subdiaphragmatic pulmonary veins diagnosed on abdominal ultrasound; to the best of our knowledge, this is the first reported case of abdominal ultrasound diagnosis of previously unknown scimitar syndrome.

Case reports
Case 1: 16 day old girl born at term with cardiac dextroposition and right lung hypoplasia. On abdominal ultrasound, a large vascular structure was seen draining into the inferior vena cava (IVC) near the hepatic confluence, arising in lung above the diaphragm. Doppler confirms venous flow into the IVC (Figure 1); an arterial aortic branch with pulmonary spectral Doppler signature, extended into the base of the right lung. The diagnosis of scimitar syndrome was made, confirmed several weeks later with CT angiography.

Case 2: One day old girl born at 29 weeks gestation had a prenatal diagnosis of complex congenital heart disease. Abdominal ultrasound demonstrated asplenia. As part of the complex, the examination demonstrated the common pulmonary vein draining into the left portal vein, decompressing into the heart via the ductus venosus, with color and spectral demonstration of flow direction and velocity. (Figure 2) Closure of the ductus venosus contributed to the demise of this child who was inoperable due to her extreme prematurity and her other congenital cardiac lesions.

Case 3: Eight day old term infant with multiple congenital anomalies. An abdominal ultrasound for heterotaxy evaluation demonstrated a large anomalous vessel arising in the chest between the descending aorta and esophagus posteriorly, and the atrium anteriorly, descending into the abdomen draining into the portal vein, with a partially obstructing membrane near the insertion point. (Figure 3). The TAPVC was repaired on day 9 of life with direct anastomosis to the left atrium.

Conclusion:
In conclusion, subdiaphragmatic vascular components of thoracic anomalies should be recognized at abdominal sonography by pediatric radiologists, who may be the first to both recognize and diagnose these conditions. Read More

Authors:  Ndolo Josephine , Crane Gabriella , Hernanz-schulman Marta

Keywords:  total anomalous pulmonary venous connection (TAPVC), Scimitar syndrome, ultrasound