Senyüz O F, Büyükünal C, Danişmend N, Erdoğan E, Ozbay G, Söylet Y
Department of Pediatric Surgery, Cerrahpasa Medical School, University of Istanbul, Turkey.
J Pediatr Surg. 1989 May;24(5):453-6. doi: 10.1016/s0022-3468(89)80400-2.
Extensive aganglionosis very close to the ligament of Treitz and total intestinal aganglionosis are rare forms of Hirschsprung's disease. In these cases, nutrition, fluid, and electrolyte balance are a problem. Although the myectomy-myotomy technique of Ziegler appears hopeful, no effective surgical method has been put into the practice for the treatment of these rare forms of Hirschsprung's disease. We report four cases with extensive intestinal aganglionosis, one of which was total intestinal aganglionosis with involvement of the stomach.
非常靠近Treitz韧带的广泛性无神经节症和全肠道无神经节症是先天性巨结肠症的罕见形式。在这些病例中,营养、液体和电解质平衡是个问题。尽管齐格勒的肌切除术-肌切开术技术似乎有希望,但尚无有效的手术方法用于治疗这些罕见形式的先天性巨结肠症。我们报告了4例广泛性肠道无神经节症病例,其中1例为累及胃部的全肠道无神经节症。