Delayed Maturation of Ganglion Cells (Differential Diagnosis)

obst_16a_n.jpg: Operative findings in a newborn with a subacute to chronic ileus. At the top there is the transversely running ileum whose diameter is twice the size of the terminal ileum on the right side of the patient at the bottom. The preparation (skeletization of the mesentery) has already been performed at the transitional zone (= site of change of intestinal calibre). Because a rapid evaluation of the intestinal biopsies did not reveal any useful findings, a temporary ileostomy was performed under the impression of a long-distance aganglionosis. The final histology showed mature ganglion cells in the wall orally of the trasition zone and immature ganglion cells in the terminal ileum. obst_16b_n.jpg: Operative findings some months later. The intestinum of the dissected proximal ileostoma has now a normal diameter and is of the same size as that of the visible intestinal loops of the terminal ileum. During the past months there has been a gradually improved feeding ability through the distal ileostoma by administering the thin stool of the proximal stoma, and clinical and radiological normalization of the transit from the distal ileostoma to the anus. The biopsies performed during dissection of both ileostomas and re-anastomosing the proximal with the distal part of the ileum showed a maturation of the intramural ganglion cells in the terminal ileum and in the colon. Final diagnosis: Disturbance of transit of the whole colon and the terminal ileum in the neonatal period and early infancy due to a disorder of innervation by an immaturity of the intramural ganglion cells.