抄録
In 1976, Berdon et al. described a new type of neonatal intestinal obstruction. They reported five newborn girls who presented with marked abdominal distension and bilious vomiting with specific clinical features including dilated small bowel and rnicrocolon without anatomic obstruction, short intestine and malrotation, defective intestinal peristalsis, megacystis without vesicoureteral reflux, and hydronephrosis or dilated ureter. Pathorogical studies show almost normal ganglion cells in both dilated and narrowed areas of the intestine. Recently we encountered a patient with this condition. A 4-day-old girl underwent a tube-ileostomy for intestinal obstruction described above. But the ileostomy drainage was minimal. The patient required continuous intravenous hyperalimentations. She could survive for 46 postoperative days.