Lassmann G
Neurological Institute, University of Vienna, Austria.
Acta Neuropathol. 1987;74(3):281-6. doi: 10.1007/BF00688193.
A woman of 36 was admitted to hospital because of griping abdominal pain which occurred especially during the night when turning in bed. She had a history of constipation and bloating since birth. Irrigoscopy revealed megacolon extending from the middle of the transverse colon to the rectum. A rectal biopsy excluded Hirschsprung's disease. When surgery was performed, megacolon combined with malrotation of the colon was found, only the ascending colon being attached to the abdominal wall. Hemicolectomy was performed. In the megacolon, a cropolith, the size of a foetal head, was found which along with malrotation of the large intestine had caused the woman's symptoms. The nerve supply of the ascending colon was normal but in the megacolon there was evidence of delayed migration of ganglion nerve cells and varying degrees of degeneration of ganglion nerve cells and intraganglionic nerve fibres. Malrotation of the colon associated with megacolon and delayed migration of ganglion nerve cells and degeneration of nerve elements has not been reported previously.
一名36岁女性因腹部绞痛入院，这种疼痛尤其在夜间翻身时出现。她自出生以来就有便秘和腹胀的病史。灌肠造影显示巨结肠从横结肠中部延伸至直肠。直肠活检排除了先天性巨结肠症。手术时发现巨结肠合并结肠旋转不良，仅升结肠附着于腹壁。进行了半结肠切除术。在巨结肠中发现了一个胎儿头部大小的粪石，它与大肠旋转不良共同导致了该女性的症状。升结肠的神经供应正常，但在巨结肠中存在神经节神经细胞迁移延迟以及神经节神经细胞和神经节内神经纤维不同程度退变的证据。此前尚未有关于巨结肠合并结肠旋转不良以及神经节神经细胞迁移延迟和神经元件退变的报道。
