Background: Enterolithiasis is an uncommon finding of intraluminal calcified meconium. Whereas extraluminal calcifications are commonly reported and usually indicate intrauterine intestinal perforation with intraperitoneal extravasation of meconium (meconium peritonitis), true intraluminal calcifications of meconium in newborns are rare.
Case report: We report a case of a newborn boy with pneumothorax and pneumoperitoneum because of a transmediastinal air leakage who unfortunately died on the fifth postnatal day after a pneumopericard. The baby had lung hypoplasia and a hypoplastic thorax. Oligohydramnion was present because of urethral agenesis and anal atresia with rectourinary fistula. Enterolithiasis was found, distributed from the transverse to the rectosigmoid colon. Enterolithiasis was not diagnosed prenatally in this boy, and any of those preexisting pathologies were therefore not assumed before birth except the suspicion for urethral valves because of oligohydramnion.
Discussion: Approximately 48 cases of human neonatal enterolithiasis have been described in the medical literature. The etiology of enterolithiasis appears to be intraluminal mixture of meconium und alkaline urine--as it appears in anal atresia with enterourinary fistula. Most cases of enterolithiasis are associated with major urogenital and intestinal malformations--especially VACTERL association. With support of modern high-resolution imaging devices, enterolithiasis can be detected antenatally. We suppose that prenatal diagnosis of enterolithiasis is a warning sign of underlying pathologies.