A Case of Neonatal Splenogonadal Fusion Associated with Unusual Malformations

Abstract

The patient was a Japanese male newborn infant whose delivery was at term and uneventful, but who had severe hypoxia at birth. X-ray examination revealed a left diaphragmatic hernia. Peromelia and micrognathia were not recognized. At operation, a posterolateral defect in the left diaphragm was repaired and we found an incidental splenic anomaly. A splenic elongation arised from the normal spleen, and tapared downward to the left intra-abdominal testis. He expired four hours after surgery because of cardiopulmonary insufficiency. An autopsy confirmed the dignosis of splenogonadal fusion associated with congenital diaphragmatic hernia, left ventricular hypoplasia of the heart and male pseudohermaphroditism. Splenogonadal fusion (SGF) is a rare but benign anomaly. To our knowledge, 38 cases of SGF in infancy and children have been reported in the world literature during the past twenty years. In summarizing of the literature, we found only two cases of SGF associated with congenital diaphragmatic hernia. Our case is the first report of the continuous type of neonatal SGF in Japan.