Surgery for Hirschsprung's disease is associated with high rate of morbidity, in the form of either constipation or incontinence or a combination of the two. This study investigates the mechanisms responsible for incontinence and/or constipation following the pull-through operation for Hirschsprung's disease. There were 19 children (15 boys and 4 girls), who at the time of study; 16 had undergone Duhamel, 1 Rehbein, and 2 Soave operation. We classified patients according to their symptoms into 3 groups: Group A was incontinent of faeces; Group B was constipated and incontinent of faeces, and Group C was constipated only. The median age at referral was 6 years, and the median period after operation was 5 years. All patients were investigated by intestinal transit study, endoanal sonography and anorectal manometry. Group A had normal or rapid transit study, as opposed to Groups B and C, who had delayed-transit study. On endoanal sonography, all children had an intact internal and an external anal sphincter, below the level of pull-through operation. The anorectal manometry showed a significantly lower resting anal pressure in the incontinent Group A as compared to the constipated children with or without incontinence in Group B or C (38 mmHg versus 57 or 66 mmHg respectively). The rectal pressure was also significantly higher in children in Group A as compared to those in Group B or C (71 mmHg versus 42 or 36 mmHg). The ratio of rectal/anal pressure was higher in incontinent children in Group A, as compared to constipated children in Group B or C. Therefore, constipation can be caused by high anal resting pressure and a weak rectal peristalsis, while faecal incontinence can be secondary to poor compliance and elevated rectal pressure in the presence of normal or low anal sphincter resting pressure. Aperients are the mainstay of treatment of constipation, however, children with incontinence are more difficult to treat. We did not attempt to define the pattern of nerve plexus because of poor results of revision operation for residual hypoganglionic segment and intestinal neuronal dysplasia. Treatment of these children can become more rational, if furnished with detailed functional studies. We advocate investigation of the anorectal function at an early stage in symptomatic children after surgery for Hirschsprung's disease, and less invasive treatment should be considered before embarking on major surgery.
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http://dx.doi.org/10.1007/s00383-002-0897-x | DOI Listing |
Stem Cell Res Ther
January 2025
Department of Pediatric Surgery, Qilu Hospital of Shandong University, Jinan, China.
Background: Understanding how enteric neural crest cells (ENCCs) differentiate into neurons is crucial for neurogenesis therapy and gastrointestinal disease research. This study explores how magnesium ions regulate the glycolytic pathway to enhance ENCCs differentiation into neurons.
Materials And Methods: We used polymerase chain reaction, western blot, immunofluorescence, and multielectrode array techniques to assess magnesium ions' impact on ENCCs differentiation.
World J Pediatr Surg
December 2024
Department of General Surgery, Boston Children's Hospital, Boston, Massachusetts, USA.
Hirschsprung disease (HSCR) is the most common congenital motility disorder of the intestine, characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional bowel obstruction. Short-segment Hirschsprung disease (SS-HSCR) accounts for the majority of cases, with surgical resection being the cornerstone of treatment. Despite advances in surgical techniques, considerable variability exists in practice regarding the timing of surgery, the choice of technique, and the length of aganglionic rectal cuff to resect.
View Article and Find Full Text PDFCurr Pediatr Rev
January 2025
Pediatric Surgery Division, Department of Surgery/Genetics Working Group/Translational Research Unit, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, 55281, Indonesia.
Background: Hirschsprung disease (HSCR) is a multifactorial disorder due to the enteric nervous system (ENS) development failure. At least 35 genes have been responsible for HSCR, including EDNRB and EDN3. Here, we aimed to determine the EDRNB and EDN3 expressions effects in HSCR subjects.
View Article and Find Full Text PDFPediatr Gastroenterol Hepatol Nutr
January 2025
Department of Pediatric Surgery, Faculty of Medicine, Cairo University, Cairo, Egypt.
Purpose: Research proved the beneficial effect of Zinc on human health and Gastrointestinal tract inflammatory diseases. We propose that zinc would be of value in children with Hirschsprung's disease (HD) undergoing elective pull-through surgery. This study was carried out to determine the influence of preoperative zinc intake on postoperative outcomes, especially the hospital length of stay in patients diagnosed with HD as a primary outcome.
View Article and Find Full Text PDFEcotoxicol Environ Saf
January 2025
Department of Pediatric Surgery, Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou 563000, China; Department of Pediatric Surgery, Guizhou Children Hospital, Zunyi, Guizhou 563000, China. Electronic address:
Nonylphenol (NP), a ubiquitous environmental contaminant used as a surfactant in industrial production and classified as an endocrine disruptor, could interfere hormone secretion and exhibit neurotoxicity in organisms. Hirschsprung's disease (HSCR), one of the most frequently observed congenital malformations of the digestive system, arises mainly due to the failure of enteric neural crest cells to migrate to the distal colon during embryonic development. However, the effects of NP exposure on HSCR are largely unknown.
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