Un caso raro de malformación del tubo neural: síndrome de hendidura notocordal

a 33 years old woman, gravida ii, para i, was referred at 27 weeks gestation with the diagnosis of fetal ascites. ultrasound report showed, polyhydramnios, fetal abdomen distended occupied by a large liquid mass initially impressed as fetal ascites, echogenic bowel is also consistent with meconium peritonitis and polymicrogiria. in a subsequent ultrasonography was observed column defect on the sacrum where emerges a cystic mass equivalent to the known "anterior spina bifida", others previous ultrasound findings were confirmed. cesarean section was performed at 33 weeks gestation, obtaining male live newborn, 2 820 g and 45 cm in length, with apgar score of 6 y 7 at 1 and 5 minutes, respectively. the newborn presented transient distress respiratory syndrome and symptoms of intestinal obstruction which was resolved surgically. operative findings: intestinal atresia, and confirming the split notochord syndrome. the newborn died of sepsis. no autopsy was performed. commentary: the ultrasound findings depend on the form of the lesion, but often are abdomino-thoracic cysts, myelomeningocele and spina bifida. the prognosis is usually ominous, but depends on the extent of the lesions and associated anomalies.