Hirschsprungs sjukdom - ovanlig medfödd tarmsjukdom. Nya operationsmetoder och principer för behandling av postoperativa besvär
Engelsk titel: Hirschsprung disease - a rare congenital intestinal disease. New surgical techniques and therapeutic principles of postoperative problems
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Författare:
Christofferson R
;
Zetterlind L
;
Lindblad K
;
Gustafson E
Email: rolf.christofferson@kbh.uu.se
Språk: Swe
Antal referenser: 18
Dokumenttyp:
Översikt
UI-nummer: 09041616
Sammanfattning
There are approximately twenty new cases of Hirschsprung's disease diagnosed annually in Sweden. The surgical principle is still resection of the aganglionic segment, but the original three stage procedure is being replaced by a one stage transanal endorectal pull-through procedure in the neonatal period. This procedure seems to be as safe and exhibits similar or better outcome in terms of incidence of enterocolitis, obstructive symptoms, or soiling at follow-up. New therapeutic alternatives for these inherently Hirschsprung-related problems are oral sodium cromoglycate, which may reduce the incidence of enterocolitis, construction of an appendicostomy for antegrade continence enemas reducing soiling, and injections of botulinum toxin A in the internal anal sphincter, which seem to relieve obstructive symptoms. Experimental data suggest that enteric neuronal stem cells may differentiate to enteric neurons, which may be a future therapeutic option. Since Hirschsprung's disease is uncommon, we emphasize the need for national clinical practice guidelines and establishment of a national database for monitoring and for prospective, randomized clinical trials.