Long-Distance Type (Hirschsprung´s Disease)

Operative preparation of a 7-month-old girl with chronic disturbance of intestinal transit. On the right the whole colon is displayed, on the left 20 cm of the terminal ileum. The diagnosis is a long-distance Hirschsprung's disease with total involvement of the colon and partial involvement of the terminal ileum = Zuelzer Wilson syndrome. In the neonatal period the patient had an ileostomy following an ileus. At 7 months of age an ileoanal anastomosis was performed followig resection of the aganglionic intestine. The mother of the girl already had a similar type of Hirschsprug's disease.