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  • 1
    ISSN: 1437-9813
    Keywords: Neuronal intestinal dysplasia ; Hirschsprung's disease ; Colon sonography ; Colon motility ; Colon biopsy, diagnostic
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Twenty-four cases of concomitant Hirschsprung's disease (HD) and neuronal intestinal dysplasia (NID) are presented. The clinical picture is characterized by the early and acute onset of HD symptoms. The diagnosis is established by means of rectal and colonic biopsies. Open biopsies during laparotomy should be taken without injury to the mucosa. Early surgical therapy consists of extended resection of the aganglionic segment and the colon affected by NID up to the splenic flexure. Complications are imminent if the aganglionosis masks the symptoms of NID and, accordingly, only the aganglionic segment is resected. As an alternative, postponed resection of the aganglionic segment alone is proposed once the NID-affected bowel develops functional maturation. In children who do not show an improvement of colon dysmotility, however, extended resection is recommended at the age of 4 years. In follow-up studies of colon motility, functional colon sonography is used.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 2 (1987), S. 352-358 
    ISSN: 1437-9813
    Keywords: Neuronal intestinal dysplasia ; Colon motility ; Colon sonography ; Colostomy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Findings in 23 children with neuronal intestinal dysplasia (NID) are presented. Twelve children had Hirschsprung's disease that masked the existence of proximal NID. In all cases the diagnosis was established by histological-histochemical examination. Amelioration of symptoms by conservative treatment alone was achieved in 4 children; in 13 cases colostomy was necessary. In 6 patients colon resection was performed: 2 suffered from both colitis and obstruction; the other 4 were treated for obstruction, multiple perforations, persistant adynamia of the left colon, and fistulation. In 9 patients the colostomy was closed. Four of 6 children who did not have sonographic confirmation of motility had complications due to colon dysmotility after closure of their normally functioning colostomy. Therapeutic measures are based exclusively on clinical and functional parameters. The clinical picture dictates the emergency measures (colostomy, colon resection, and colectomy). Normal colostomy function is not a sufficient criterion of normal colon motility. Functional sonography of the colon is included in the planning of further therapy. Indications, timing, and extent of colon resection can be reliably determined using the sonographic method.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 2 (1987), S. 81-85 
    ISSN: 1437-9813
    Keywords: Sphincter ani internus ; Aganglionosis ; Neuronal intestinal dysplasia (type B) ; Sphincter achalasia ; Biopsy diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Enzyme-histotopochemical studies of parasympathetic innervation in the internal anal sphincter muscle of 73 children with anal sphincter achalasia indicated that in Hirschsprung's disease and type B neuronal intestinal dysplasia (NID) the internal sphincter displays a pattern of changes analogous to that seen in the wall of the rectum. In Hirschsprung's disease the internal sphincter is also aganglionic, and its parasympathetic fibres exhibit increased acetylcholinesterase (ACE) activity in NID there is moderately increased ACE activity, with ganglionic neurons embedded singly or in groups in the thick afferent parasympathetic fibers. The innervation defect may be confined to the sphincter, or aganglionosis of the sphincter may be associated with proximal NID.
    Type of Medium: Electronic Resource
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