Neuronal intestinal dysplasia in pediatric patients has similar clinical symptoms and often similar radiologic findings to those of Hirschsprung's disease. Yet neuronal intestinal dysplasia shows hyperplasia of the myenteric plexus for the pathology, and it requires different treatment compared with Hirschsprung disease. This disease has been reported many times in Europe but, to date, only one case has been reported in the radiologic literatures in Korea. We report here on a case of neuronal intestinal dysplasia that involved the entire colon in a two-month-old boy, and we include the radiographic findings.
Colon ; Europe ; Hirschsprung Disease ; Humans ; Hyperplasia ; Infant* ; Korea ; Male ; Myenteric Plexus ; Neurons* ; Pathology

Neuronal intestinal dysplasia in pediatric patients has similar clinical symptoms and often similar radiologic findings to those of Hirschsprung's disease. Yet neuronal intestinal dysplasia shows hyperplasia of the myenteric plexus for the pathology, and it requires different treatment compared with Hirschsprung disease. This disease has been reported many times in Europe but, to date, only one case has been reported in the radiologic literatures in Korea. We report here on a case of neuronal intestinal dysplasia that involved the entire colon in a two-month-old boy, and we include the radiographic findings.
Colon ; Europe ; Hirschsprung Disease ; Humans ; Hyperplasia ; Infant* ; Korea ; Male ; Myenteric Plexus ; Neurons* ; Pathology

Gastrointestinal stromal tumors (GISTs) are rarely noted in association with neurofibromatosis-1 (NF-1, von Recklinghausen disease) as an individual gastrointestinal manifestation. We report here a case of multiple GISTs with an abundant skeinoid fiber in the jejunum of a 43-year-old woman diagnosed as NF-1. Histologically, the tumors were composed of uniform spindle-shaped cells with a fascicular pattern, almost indistinguishable from the histology characteristic of usual GISTs. However, multiple synchronous tumor occurrence, abundant skeinoid fiber, and presence of microscopic miniatures of stromal tumors are additional characteristic features of this case.
Adult ; Female ; Human ; Intestinal Neoplasms/*pathology ; Jejunum/innervation/*pathology ; Myenteric Plexus/pathology ; Neurofibromatosis 1/*pathology ; Stromal Cells/*pathology

Intestinal neuronal dysplasia(IND) is a disease characterized clinically by symptoms of intestinal obstruction and pathologically by hyperplasia of the submucosal and myenteric plexuses with formation of giant ganglia. Chronic intestinal pseudo-obstruction is a clinical diagnosis, composed of myopathic form and neuropathic form, and normal intestinal histology. Intestinal neuronal dysplasia is a neuropathic form of chronic intestinal pseudo-obstruction. The clinical presentation and the course of IND is very variable. We experienced a case of intestinal neuronal dysplasia in a 5 year-old boy who had suffered from recurrent abdominal pain and vomiting for 3 years. Small bowel series showed multiple intestinal dilatations and delayed excretion of contrast media. He underwent exploratory laparotomy. However no mechanical causes for markedly dilated intestine were found and he received loop ileostomy. However, he suffered from recurrent vomiting and abdominal pain. So, he received repair-operaton. The pathology of surgical specimen showed hyperplasia of the submucosal and myenteric plexuses with giant ganglia. We report this case with a brief review of the related literatures.
Abdominal Pain ; Child, Preschool ; Contrast Media ; Diagnosis ; Dilatation ; Ganglia ; Humans ; Hyperplasia ; Ileostomy ; Intestinal Obstruction ; Intestinal Pseudo-Obstruction ; Intestines ; Laparotomy ; Male ; Myenteric Plexus ; Neurons* ; Pathology ; Vomiting

BACKGROUND/AIMS: Previous studies from Korea have described chronic intestinal pseudo-obstruction (CIPO) patients with transition zone (TZ) in the colon. In this study, we evaluated the pathological characteristics and their association with long-term outcomes in Korean colonic pseudo-obstruction (CPO) patients with TZ. METHODS: We enrolled 39 CPO patients who were refractory to medical treatment and underwent colectomy between November 1989 and April 2016 (median age at symptoms onset: 45 [interquartile range, 29–57] years, males 46.2%). The TZ was defined as a colonic segment connecting a proximally dilated and distally non-dilated segment. Detailed pathologic analysis was performed. RESULTS: Among the 39 patients, 37 (94.9%) presented with TZ and 2 (5.1%) showed no definitive TZ. Median ganglion cell density in the TZ adjusted for the colonic circumference was significantly decreased compared to that in proximal dilated and distal non-dilated segments in TZ (+) patients (9.2 vs 254.3 and 150.5, P < 0.001). Among the TZ (+) patients, 6 showed additional pathologic findings including eosinophilic ganglionitis (n = 2), ulcers with combined cytomegalovirus infection (n = 2), diffuse ischemic changes (n = 1), and heterotropic myenteric plexus (n = 1). During follow-up (median, 61 months), 32 (82.1%) TZ (+) patients recovered without symptom recurrence after surgery. The presence of pathological features other than hypoganglionosis was an independent predictor of symptom recurrence after surgery (P = 0.046). CONCLUSIONS: Hypoganglionosis can be identified in the TZ of most Korean CPO patients. Detection of other pathological features in addition to TZ-associated hypoganglionosis was associated with poor post-operative outcomes.
Cell Count ; Colectomy ; Colon ; Colonic Pseudo-Obstruction ; Cytomegalovirus Infections ; Eosinophils ; Follow-Up Studies ; Ganglion Cysts ; Humans ; Intestinal Pseudo-Obstruction ; Korea ; Male ; Myenteric Plexus ; Pathology ; Recurrence ; Ulcer

Nitric oxide (NO) is a non-adrenergic, non-cholinergic neurotransmitter found in the enteric nervous system that plays a role in a variety of enteropathies, including inflammatory bowel disease. Alteration of nitrergic neurons has been reported to be dependent on the manner by which inflammation is caused. However, this observed alteration has not been reported with acetic acid-induced colitis. Therefore, the purpose of the current study was to investigate changes in nitrergic neuromuscular transmission in experimental colitis in a rat model. Distal colitis was induced by intracolonic administration of 4% acetic acid in the rat. Animals were sacrificed at 4 h and 48 h postacetic acid treatment. Myeloperoxidase activity was significantly increased in the acetic acid-treated groups. However, the response to 60 mM KCl was not significantly different in the three groups studied. The amplitude of phasic contractions was increased by Nomega-nitro-L-arginine methyl ester (L-NAME) in the normal control group, but not in the acetic acid-treated groups. Spontaneous contractions disappeared during electrical field stimulation (EFS) in normal group. However, for the colitis groups, these contractions initially disappeared, and then reappeared during EFS. Moreover, the observed disappearance was diminished by L-NAME; this suggests that these responses were NO-mediated. In addition, the number of NADPH-diaphorase positive nerve cell bodies, in the myenteric plexus, was not altered in the distal colon; whereas the area of NADPH-diaphorase positive fibers, in the circular muscle layer, was decreased in the acetic acidtreated groups. These results suggest that NO-mediated inhibitory neural input, to the circular muscle, was decreased in the acetic acid-treated groups.
Acetic Acid/toxicity ; Animals ; Colitis/chemically induced/*pathology/*physiopathology ; Colon/drug effects/enzymology/*innervation/pathology ; Indicators and Reagents/toxicity ; Male ; Muscle Contraction/drug effects ; Muscle, Smooth/drug effects/metabolism ; Myenteric Plexus/pathology ; NADPH Dehydrogenase/metabolism ; NG-Nitroarginine Methyl Ester/pharmacology ; Neuromuscular Junction/drug effects/*metabolism ; Nitrergic Neurons/drug effects/*metabolism ; Nitric Oxide/*metabolism ; Peroxidase/metabolism ; Potassium Chloride/pharmacology ; Rats ; Rats, Sprague-Dawley