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Intestinal microvillous dystrophy: a variant of microvillous inclusion disease or a new entity?

F Raafat1, N J Green, K A Nathavitharana

  • 1Department of Histopathology, Children's Hospital, Ladywood Middleway, Birmingham, UK.

Human Pathology
|November 1, 1994
PubMed

Insights

Intestinal microvillous dystrophy presents a spectrum of disorders causing severe infant diarrhea. Diagnosis involves jejunal biopsy, with poor prognosis and need for genetic counseling in affected families.

Area of Science:

  • Gastroenterology
  • Pediatric Pathology
  • Genetics

Background:

  • Microvillous inclusion disease (MVID) is a known cause of infant secretory diarrhea.
  • A spectrum of microvillous disorders may exist, potentially underrecognized.
  • This study investigates three cases with features distinct from classical MVID.

Observation:

  • Three patients, including siblings, presented with intestinal microvillous dystrophy.
  • Clinical presentation was delayed compared to typical MVID.
  • Jejunal biopsies showed altered brush border staining (PAS, alkaline phosphatase) and abnormal microvilli on electron microscopy.

Findings:

  • Cases exhibited thinning or absence of brush border staining.
  • Electron microscopy revealed poorly developed, haphazard microvilli with intracytoplasmic vesicles, but no classical inclusions.
  • The condition leads to fatal intractable secretory diarrhea in infants.

Implications:

  • Intestinal microvillous dystrophy represents a distinct entity within microvillous disorders.
  • Autosomal recessive inheritance is suggested by familial occurrence.
  • Genetic counseling is crucial for families with affected infants due to the poor prognosis.

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