Intestinal neuronal dysplasia (IND) type B is a disease of the submucosal plexus of intestine manifesting chronic intestinal obstruction or severe chronic constipation. IND is one of intestinal dysganglionoses and clinically closely associated with Hirschsprung's disease. Until recently, it is not fully clear whether IND is a congenital malformation or an acquired secondary condition related to some gastrointestinal problems. However, recently published data and consensus reports have enhanced our understanding of the pathogenesis and management of IND. The aim of this paper was to review the current state of knowledge regarding the controversial issues of IND including the etiology, classification, diagnostic criteria, and available therapeutic intervention.
Child ; Colon/*innervation/radiography ; Constipation/etiology ; Enteric Nervous System/*abnormalities ; Ganglia/pathology ; Gastrointestinal Motility ; Hirschsprung Disease/pathology ; Humans ; Immunohistochemistry ; Intestinal Diseases/*diagnosis/pathology ; Intestinal Mucosa/pathology

Autonomic Nervous System Diseases ; metabolism ; pathology ; surgery ; Colon ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Enteric Nervous System ; abnormalities ; pathology ; Hirschsprung Disease ; pathology ; Humans ; Infant, Newborn ; Intestinal Diseases ; metabolism ; pathology ; surgery ; Male ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism

OBJECTIVETo investigate the relationship between the morphological features of different types of neuronal intestinal malformations (NIM) and their postoperative complications.

METHODSThe data of morphological and clinical features of 324 cases with NIM were analyzed retrospectively.

RESULTSIn all 324 patients, 210 cases were Hirschsprung's disease (HD), 38 intestinal neuronal dysplasia (IND), 45 mixed HD/IND, 8 hypoganglionosis, 22 combined HD/hypoganglionosis and 1 immaturity of ganglion cells. The percentages of normal neuron in bowel of different NIM were 88.1%, 24.4%, 18.4%, 4/8, 27.7% and 0/1 in HD, HD/IND, IND, hypoganglionosis, HD/hypoganglionosis and immaturity of ganglion cells respectively. There were totally 46 cases complicated with recurrent postoperative enterocolitis (EC). Incidence of recurrent postoperative EC in HD patients was 6.7% while in IND/HD and IND patients was 35.6% and 28.9%, respectively. Incidences of EC in cases with the residual IND margins and with the normal margins were 38.2% and 8.7%, respectively. Incidence of EC in cases with transanal endorectal pull-through procedure and with transabdominal procedure was 18.0% and 8.3%, respectively. Nine cases underwent another procedure because of severe persistent constipation or EC after operation, including 4 cases HD/IND, 1 case IND, 3 cases HD and 1 case HD/hypoganglionosis.

CONCLUSIONSNeuron distribution is inconsistent with pathology of NIM. Postoperative EC are rare in the patients only with isolated HD. Furthermore, margins with residual IND and transanal endorectal pull-through procedure are risk factors to recurrent EC. However, the extension of excision about IND is uncertain and need further study.

Adolescent ; Child ; Child, Preschool ; Digestive System Abnormalities ; complications ; pathology ; surgery ; Enteric Nervous System ; abnormalities ; pathology ; Female ; Hirschsprung Disease ; complications ; pathology ; surgery ; Humans ; Infant ; Male ; Postoperative Complications ; etiology ; Retrospective Studies