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Symptoms, diagnosis, and therapy of neuronal intestinal dysplasia masked by Hirschsprung's disease

Report of 24 cases

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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.

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Authors and Affiliations

  1. Servicio de Cirurgia Infantil, Hospital E. Deformes, Valparaiso, Chile

    B. Fadda

  2. Klinik und Poliklinik für Kinderchirurgie, Universitätsklinik Mainz, Langenbeckstrasse 1, D-6500, Mainz, Germany

    G. Pistor & S. Hofmann-von Kap-herr

  3. Division of Neuropathology, Department of Pathology, UNiversity of Basle, CH-4003, Basle, Switzerland

    W. Meier-Ruge

  4. Institut für Kinderpathologie, Universitätsklinik Mainz, Langenbeckstrasse 1, D-6500, Mainz, Germany

    H. Müntefering

  5. Departamento de Patologia, Universidad de Valparaiso, Valparaiso, Chile

    R. Espinoza

Authors
  1. B. Fadda
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  2. G. Pistor
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  3. W. Meier-Ruge
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  4. S. Hofmann-von Kap-herr
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  5. H. Müntefering
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  6. R. Espinoza
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Fadda, B., Pistor, G., Meier-Ruge, W. et al. Symptoms, diagnosis, and therapy of neuronal intestinal dysplasia masked by Hirschsprung's disease. Pediatr Surg Int 2, 76–80 (1987). https://doi.org/10.1007/BF00174177

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  • DOI: https://doi.org/10.1007/BF00174177

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