Gastrointestinal neuromuscular pathology: Guidelines for histological techniques and reporting on behalf of the Gastro 2009 International Working Group

Charles H. Knowles, Roberto De Giorgio, Raj P. Kapur, Elisabeth Bruder, Gianrico Farrugia, Karel Geboes, Michael D. Gershon, John Hutson, Greger Lindberg, Joanne E. Martin, William A. Meier-Ruge, Peter J. Milla, Virpi V. Smith, Jean Marie Vandervinden, Béla Veress, Thilo Wedel

Research output: Contribution to journalArticlepeer-review

142 Scopus citations


The term gastrointestinal neuromuscular disease describes a clinically heterogeneous group of disorders of children and adults in which symptoms are presumed or proven to arise as a result of neuromuscular, including interstitial cell of Cajal, dysfunction. Such disorders commonly have impaired motor activity, i.e. slowed or obstructed transit with radiological evidence of transient or persistent visceral dilatation. Whilst sensorimotor abnormalities have been demonstrated by a variety of methods in these conditions, standards for histopathological reporting remain relatively neglected. Significant differences in methodologies and expertise continue to confound the reliable delineation of normality and specificity of particular pathological changes for disease. Such issues require urgent clarification to standardize acquisition and handling of tissue specimens, interpretation of findings and make informed decisions on risk-benefit of full-thickness tissue biopsy of bowel or other diagnostic procedures. Such information will also allow increased certainty of diagnosis, facilitating factual discussion between patients and caregivers, as well as giving prognostic and therapeutic information. The following report, produced by an international working group, using established consensus methodology, presents proposed guidelines on histological techniques and reporting for adult and paediatric gastrointestinal neuromuscular pathology. The report addresses the main areas of histopathological practice as confronted by the pathologist, including suction rectal biopsy and full-thickness tissue obtained with diagnostic or therapeutic intent. For each, indications, safe acquisition of tissue, histological techniques, reporting and referral recommendations are presented.

Original languageEnglish (US)
Pages (from-to)271-301
Number of pages31
JournalActa neuropathologica
Issue number2
StatePublished - 2009


  • Enteric myopathy
  • Enteric neuropathy
  • Hirschsprung disease
  • Histopathology
  • Interstitial cells of Cajal
  • Intestinal pseudo-obstruction
  • Suction rectal biopsy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience


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