Updated results on intestinal neuronal dysplasia (IND B): Meier-Ruge WA, Ammann K, Bruder E, et al. Eur J Pediatr Surg 2004 (December);14:384-391

Abstract

Intestinal neuronal dysplasia (IND B) is still a subject of controversy. The aim of this paper was to review the present state of knowledge on IND B. A summary is given of the technical and diagnostic criteria which have to be considered to achieve a reliable diagnosis. The available therapeutic procedures are additionally discussed. Between 1992 and 2001, 3984 colonic mucosal biopsies from 1328 children were investigated. Nerve cell staining was performed on native tissue sections: 15 μm thick cryostat sections which, after spreading and drying, have a final thickness of 4-5 μm, with dehydrogenase reactions. The biopsies were taken 8-10 cm above the dentate line with a sufficient amount of submucosa. The criteria for IND B is 15-20% submucosa) giant ganglia with more than 8 nerve cells on 30 sections of a single biopsy. The diagnosis of IND B is quantitative. A diagnosis of IND B was made in 51 Hirschsprung resections, and in 92 children with chronic constipation (6% and 2.3% incidence, respectively). Up to the 14th year of life, most children with isolated IND B can be treated conservatively due to the delayed maturation of the enteric nervous system which is characteristic for IND B. Only children with additional hypo-plastic hypoganglionosis were treated surgically. Children with HD and IND B proximal to the aganglionosis often exhibited post-operative disturbances of intestinal motility when a disseminated IND B was present. Ganglioneuromatosis (MEN2B) must be clearly differentiated from IND B. The clinical course in IND B depends on the extent of impaired bowel innervation, the severity of motility affection and the coexistence of HD. Conservative management of isolated IND B is possible in most children. In individual cases, however, a transient enterostomy or segmental resection is unavoidable.