Resumen

GONZALEZ DE CHIRIVELLA, Xiomara; BETANCOURT, Zuray  y  GONZALEZ, Mariángela. Un caso raro de malformación del tubo neural: síndrome de hendidura notocordal. Rev Obstet Ginecol Venez [online]. 2009, vol.69, n.4, pp.269-272. ISSN 0048-7732.

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.

Palabras clave : Split notochord syndrome; Meconial peritonitis; Intestinal atresia; Fetal ascites; Polyhydramnios.