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Pathology of the Gut Motility Disorders: Hirschsprung’s Disease

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Surgical Pathology of the Gastrointestinal System

Abstract

Intestinal motility disorders are clinical conditions associated with impaired peristalsis which occurs in the absence of any demonstrable luminal/mechanical cause. They most often recognized in the infants but may present at any age presenting with constipation and may get complicated by diarrhea due to superadded microbial overgrowth (enterocolitis), sometimes encopresis (fecal soiling) and result in failure to thrive. These conditions primarily involve ganglion cells, nerves, and smooth muscle fibers and hence, they are grouped together. Hirschsprung’s disease (HD) is the commonest of all of these surgically treatable conditions while the other conditions are rare and hence, HD is considered here as the prototype to discuss gut motility disorders. HD mandates high-quality clinical evidence along with standardized quality evaluation methodologies in the diagnostic front including histopathology for a final diagnosis and surgical management.

This chapter highlights the overview of the disease, its pathophysiology and clinicopathologic features on one end and on the other, details the basic concept of handling rectal mucosal and surgical colonic biopsies/resections in a general pathology laboratory set-up and details clues to interpret them for a definitive diagnosis and guide the surgeon at various stages in the surgical management of a child with Hirschsprung’s disease.

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The authors agree to the fact that all biopsy procedures and surgical resections were performed after taking informed consent from respective patients as per the individual Institutional policies, which also includes consent for publishing the unidentified clinical images for publication or research purposes. The authors also declare no conflict of interest.

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  1. Translational Research Lab for Gut Motility Disorders, Department of Pathology, St. John’s Medical College, St. John’s National Academy of Health Sciences, Bangalore, Karnataka, India

    Usha Kini

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  1. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Prasenjit Das

  2. Department of Pathology, Al Sabah Hospital, Kuwait City, Kuwait

    Kaushik Majumdar

  3. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Siddhartha Datta Gupta

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Kini, U. (2022). Pathology of the Gut Motility Disorders: Hirschsprung’s Disease. In: Das, P., Majumdar, K., Datta Gupta, S. (eds) Surgical Pathology of the Gastrointestinal System. Springer, Singapore. https://doi.org/10.1007/978-981-16-6395-6_10

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