EP07.13: Ultrasound imaging in unusual anatomical variant of fetal congenital diaphragmatic hernia

In congenital diaphragmatic hernia (CDH) there are certain patterns of abdominal organs' position in fetal thorax, which can be differentiated during US exam at the standard 4-chamber view of the heart. Unusual location of abdominal organs requires comparison with intraoperative findings. 37y.o. woman, G2P1, referred to our specialised tertiary centre at 34 weeks because of late diagnosis of fetal CDH. US scan revealed SGA fetus (AC 5th centile) with dextroposition of the heart, intestine, spleen and left kidney in left hemitorax, “liver-down”, stomach low in abdomen, near urinary bladder. Spleen was visualised as rounded hypoechoic structure 22x23mm located close to anterior thoracic wall, although typically in CDH spleen is found posteriorly, in front of thoracic aorta or close to right lung. Around the spleen there were more echoic unidentifiable structures. At the 4-chamber view left lung was not visualised, right lung was visualised and measured, lung to head ratio (LHR) by area method 2.0, o/e LHR 64.1%; lung to thorax transverse area ratio 0.14. Final pre-op prenatal diagnosis: “Left-sided CDH with herniation of intestine, kidney, spleen to the chest. Moderate/severe hypoplasia of the left lung, mild/moderate hypoplasia of the right lung. Suspicion for combined defect of diaphragm. SGA fetus”. Term Caesarean section delivery: newborn 2700 gr, Apgar 4/5, operated on the 2nd day of life. Surgery revealed a non-communicating defect of diaphragm 40x25 mm, with a “sack” that contained spleen, loops of small and large intestine, left kidney and partially pancreas. There were multiple adhesions between intestinal loops, and especially spleen with hernia sac; abdominal organs must have been separated from the sac, what caused mild surficial spleen hemorrhage, and needed additional hemostasis measures. After the sac resection small functioning left lung was seen in upper thorax. Post-op period uncomplicated, on day 15th infant transferred from surgical NICU to recovery ward.