ABSTRACT
In addition to the classified types of dysganglionosis, certain non-classified dysganglionoses (NCD) (types 1-4) were introduced by Meier-Ruge in 1992. Clinical data on these conditions are limited. Among 134 children with intestinal dysganglionoses (ID) treated from 1979 to 1999, 12 were identified to have a NCD. Their clinical course is presented. The existence of mild ID (type 1) is difficult to demonstrate. Current definitions and data on clinical relevance are not convincing. An indication for surgical treatment is not present. Isolated hypogenesis of the submucous plexus (SMP) (type 2, n = 8) is clinically a more severe kind of intestinal neuronal dysplasia type B and often requires early surgical intervention, but not resection. When associated with aganglionosis, its recognition is important for surgical strategy, to avoid complicated clinical courses, which are frequent if total or nearly-total resection is not performed. Hypogenesis of the myenteric plexus (MP) (type 5, n = 1) has received little attention so far. The sporadic appearance of heterotopic nerve cells of the SMP in the mucosa (type 3, n = 1) is physiologic; clusters of such cells, however, are probably of pathologic value, especially in combination with other types of ID in the same patient. Heterotopic nerve cells of the MP (type 4, n = 3) in the circular and longitudinal muscle layers are highly pathologic. This clearly-defined type is of major clinical relevance and requires complete resection. A severe disturbance of the migration process is the underlying cause. To simplify the terminology of IDs, a grading system based on the anatomic structures and clinical findings is proposed: innervation disturbances of the mucosa (grade I) are of limited clinical significance. Isolated malformations of the SMP (grade II) may require an enterostomy, but do not require resection except in certain cases associated with distal aganglionosis. Dysganglionosis of the MP (grade III) usually exhibits more severe symptoms and resection is indicated, especially with associated hypo- or aganglionosis. In aganglionic bowel (grade IV) resection is mandatory.
