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Objective Intestinal neuronal dysplasia type B (IND B) is one of the gastrointestinal motility disorders with a defined malformation of the parasympathetic submucous and myenteric ganglia. The clinical presentation of IND B is variable, ranging from intestinal obstruction in the neonatal period to acute or chronic constipation in childhood. Methods Between 1993 and 1996, 105 patients (49 females and 56 males) were treated for constipation, and in all of them an IND type B was confirmed histopathologically. Twenty-two neonates, 42 infants to 6 months of age (38% of them were premature, and 5% had additional malformations), and 41 patients to the age of 4 years were included in this study. All 105 patients had been treated conservatively. Treatment consisted of diet in all patients, cisapride in 70% of them, laxatives in 52%, and repeated anal dilatations in 12% of the patients. The mean duration of their treatment lasted from 3 months to 10 months (mean, 6 months). Results The clinical follow-up 5 to 9 years later in 89 of the 108 (85%) patients showed daily defecation in 80% of them and every second day in 14% of them. Only 5 (6%) patients experience recurrent constipation, which responds well to diet and laxatives. Conclusions In young patients, constipation related to IND B can be treated successfully by conservative treatment regimens, including diet, laxatives, and prokinetic drugs.

Background Laparoscopy may reduce postoperative pain and hospital stay, compared with laparotomy. The use of laparoscopic surgery to obtain full-thickness intestinal biopsies in children has not been previously reported. Methods Eleven children aged 1.6 to 19 years (median, 4.5 years) underwent laparoscopic full-thickness biopsy of the stomach, small bowel, colon, or a combination thereof. Each procedure used one 12-mm and two 5-mm ports. Results Eight children with obstructive symptoms after a pull-through for Hirschsprung disease underwent multiple colon and small bowel biopsies (range, 3-6; median, 5); intestinal neuronal dysplasia was found in two. Two patients with cystic fibrosis had diffuse colonic narrowing; a diagnosis of enzyme-induced fibrosing colonopathy was made in one and nonspecific inflammation was found in the other. One child had a thickened stomach, and a gastroscopic-directed full-thickness biopsy revealed plasmacytoma. Nine of the 11 patients had a previous laparotomy, and ports were placed through preexisting scars. Median hospital stay was 2 days. No patient required more than 24 hours of narcotics. There were no leaks, and no other morbidity or mortality occurred. None of the patients required conversion to an open procedure. Biopsy results significantly affected treatment for each patient. Conclusions Laparoscopic full-thickness intestinal biopsy is safe and effective for a variety of gastrointestinal problems in children. This technique is associated with a short hospital stay, minimal pain, and a very low risk of complications and can be performed even in patients who have had a previous laparotomy.

Background Colonic manometry helps discriminate functional and behavioral causes for childhood constipation from colonic neuromuscular disease. Methods Of 375 colonic manometries performed for clinical indications, 12 could not be interpreted because of chronic colonic dilation. Based on colonic manometries that showed either no contractions or an absence of the gastrocolonic response or an absence of high-amplitude propagating contractions, the authors recommended diverting colostomies or ileostomies in 12 chronically constipated children (mean age, 4 years; range, 2-14 years, 5 boys). Before study, medical treatment was ineffective in all children. These children had persistently dilated colons with pathologic diagnoses of intestinal neuronal dysplasia (n = 4), hypoganglionosis (n = 2), hollow visceral myopathy (n = 1), and normal (n = 5). Results Six to 30 months after diversion, the authors restudied all the children. Eleven of 12 diverted colons were no longer dilated. In two patients, abnormal motility involving the entire colon was unchanged from the initial study, small bowel motility was abnormal, and we recommended no further surgery. In two cases, the colon remained abnormal but small bowel motility was normal, and we recommended subtotal colectomy and ileoproctostomy. In four cases, the left colon remained abnormal, but the right colon was normal, and we recommended reanastomosis after left hemicolectomy. In four cases, motility in the diverted colons was normal, including a gastrocolonic response and high-amplitude propagating contractions, and the authors recommended reanastomosis. Defecation problems resolved in 10 of 12 when followed up 5 to 30 months after treatment. Conclusion These data suggest that in some cases of intractable childhood constipation associated with colonic distention, temporary diversion improved colonic motility. Colonic manometry may be used to predict which patients will benefit from resection or reanastomosis.

BACKGROUND German pathologists have developed a consensus for histological features of intestinal neuronal dysplasia. METHODS A blind reevaluation of ganglionic suction rectal biopsies from infants and children who initially presented with symptoms of intestinal dysmotility was made. RESULTS 84 of 411 specimens had sufficient depth of submucosa for adequate assessment. Questionnaires or clinical interviews were employed 3-5 years after biopsy in these 84 patients to assess the relationship between histological changes and persistent symptomology. Eighteen children were lost to follow-up, 4 others had Hirschsprung's disease the study biopsy specimen having been taken from the pulled-through bowel after surgical resection of the aganglionic segment. The remaining 62 patients were divided into three groups. There were six patients in group A (both obligatory criteria) and 28 in group B (nonessential, or just one of the obligatory criteria), and 28 in group C (normal appearances). On follow-up, two of the 28 (7%) in group B, and six of the 28 (21%) in group C had persistent dysmotility symptoms. CONCLUSIONS Histological criteria of the consensus of German Pathologists for intestinal neuronal dysplasia was unhelpful in predicting the clinical outcome and therefore, should not influence clinical management. As one of the obligatory criteria, hyperplasia of the submucosal plexus was significantly more common in neonates (< 4 weeks), it is concluded that this is an age-related variation.

Four hundred ninety-eight acetylcholinesterase-stained suction rectal biopsies from 456 children were examined to assess the occurrence of "isolated" or "primary" intestinal neuronal dysplasia at Children's Hospital of Pittsburgh. Cases of proven Hirschsprung's disease were excluded. In 38 biopsies from 38 patients, we found a mild to moderate increase in mucosal acetylcholinesterase staining and abundant submucosal ganglion cells (large, irregular ganglia or at least five ganglia per HPF). This group was clinically heterogeneous with a frequent history of prematurity and small left colon/meconium plug syndrome, protein/formula intolerance, or obstructive anatomic gastrointestinal abnormalities. We feel that isolated "intestinal neuronal dysplasia," as diagnosed by suction rectal biopsy and the above criteria, is a descriptive biopsy appearance. Rather than characterizing a unique disease entity, it is encountered in a variety of clinical situations. Based on our observations and review of the literature, we therefore conclude that "intestinal neuronal dysplasia" not associated with Hirschsprung's disease, neurofibromatosis, or multiple endocrine neoplasia syndrome has yet to be well defined in clinical and histopathologic terms. The histologic diagnosis, at this time, should be reserved for those rare, florid cases of parasympathetic hyperganglionosis that are documented by adequate tissue sampling.