Hirschsprung disease (HD) is a congenital absence of nerve cells (ganglions) in a segment of the intestinal wall, leading to its obstruction. Diagnosis of BG in children is a labor-intensive process, on which surgical treatment tactics depend. The concentration of patients in large surgical centers with equipped diagnostic departments makes it possible to develop the most adequate examination plan while minimizing the risk of diagnostic errors. The aim of the study was to analyze methods for diagnosis of HD in children; a retrospective analysis of the records of patients hospitalized in three medical organizations in Moscow (n=201) in 2017–2019 was carried out. The first group – children with confirmed diagnosis (n=152), the second group – patients with suspicion of BG with unsatisfactory results of previous operative treatment, postoperative complications (n=49). Results: in the 1st group, irrigography was performed before hospitalization in 118 patients (77,6%). In the hospitals, irrigography was performed (repeated) for 109 (71,7%) children. 25 (16,4%) patients showed discrepancies in interpretation of the results. Thus, irrigography was applied to all patients. Intestine biopsy before hospitalization was performed in 79 patients (52%), in the hospitals – performed/repeated in 50 patients (32,9%). There were discrepancies in the interpretation of histological findings in 8 patients (18,6 per cent). Thus, in the diagnosis of HD in children, biopsies were used (before hospitalization and/or during hospitalization) in 89 patients (58,6%). Anorectal manometry was performed to 3 (2%) children. In the 2nd group a histological examination was performed (before and during hospitalization) in 41 patients (84%), anorectal manometry – in 15 patients (31%), irrigography – in all children. Correlation analysis did not reveal any relationship between the HD variant and manifestation symptoms (Spearman's correlation coefficient was –0,232 at p<0,05. Conclusion: all medical teams began examining children with suspected HD with irrigography. Various intestinal biopsies were performed in 58,6% of cases. Anorectal manometry is currently practically not used in the diagnosis of HD in children, although it is a promising non-invasive method with high sensitivity and specificity.
Introduction. The pathology of the enteric ganglia can lead to different diseases (Hirschsprungs, neuronal intestinal dysplasia, ganglioneuromatosis, and Chagasse). Causes of acquired dysganglionosis remained unclear. Some authors hypothesized that pathology of the enteral nervous system may be secondary to intestinal ischemia.
Aim. To investigate the intestinal function and histological changes of the colon in rats with chronic ischemia.
Materials and methods. A total of 20 Sprague Dawley rats underwent surgery (ligation of the terminal mesenteric vessels next to the descending colon). The appetite of animals were checked, and stool were collected after the procedure. Reoperation was performed after 7 (n = 1), 9 (n = 2), 12 (n = 2), 14 (n = 1), 21 (n = 1), 42 (n = 1), 53 (n = 1), and 62 (n = 1) days. The diameter of the colon and changes of the serosa were visualized. In the experimental group, two samples biopsy was performed (ischemic and normal colon).
Results. Functional changes were observed in 90% of rats after the ligation of mesenteric vessels (constipation/impact, softening stool/diarrhea, and hemocolitis). Colonic stenosis of the ischemic area in 30% was detected. 70% animals have the intestinal dilatation above the ischemic segment (partial bowel obstruction). Necrosis of the ischemic colon was observed in 20%. Spontaneous fixation of the omentum to the ischemic segment was found in 40% animals. A microscopically inflamed infiltration of the mucosa in the ischemic zone (70%) and in normal colon (50%) was revealed in the ligation group. The number of the enteric ganglia decreased in the ischemic segment.
Conclusion. Functional disorders (colitis and obstruction) and morphological changes (inflammation and ganglion cells pathology) were found in rats with chronic mesenteric ischemia.
In this article, we report a rare case of anorectal malformation (anal stenosis) in combination with anterior communicating rectal tubular duplication in a 10-month-old boy. The patient presented with constipation, bloating, anal narrowing, and an additional opening in the anterior semicircle of the anal sphincters. His diagnosis was confirmed by contrast-enhanced computed tomography. Surgical treatment was conducted by stages and included colostomy, rectal duplication removal with anterior sphincteroplasty, and stoma closure. One year postoperatively, the child had regular stools, no fecal incontinence, and a good cosmetic result of the operation. We emphasized late diagnosis of this congenital malformation. In case of an additional opening in the anterior semicircle of the anus, it is necessary to exclude colonic duplication using visualization methods. Digital rectal examination is recommended to exclude anal stenosis in such patients. Key words: anorectal malformations, anal fistula, colonic duplication
The article presents the current methods of postmortem morphometric study great vessels fetuses and infants, taking into account the requirements and technical capabilities in vivo diagnosis of congenital diseases of the cardiovascular system - MRI, echocardiography and other.
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