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Microvillous inclusion disease with abundant vermiform, electron-lucent vesicles

Douglas A Weeks1, Craig W Zuppan, Richelle L Malott

  • 1Department of Pathology, Oregon Health and Science University, Portland, Oregon 97239, USA. weeksd@ohsu.edu

Ultrastructural Pathology
|January 8, 2004
PubMed

Insights

Microvillous inclusion disease diagnosis can be challenging. Electron microscopy may reveal varied ultrastructural findings beyond classic inclusions, expanding the disease spectrum.

Area of Science:

  • Gastroenterology
  • Pediatric Pathology
  • Electron Microscopy

Background:

  • Congenital secretory diarrhea presents a diagnostic challenge in infants.
  • Microvillous inclusion disease (MVID) is a rare cause of intractable diarrhea.
  • Diagnosis typically relies on identifying characteristic microvillous inclusions via electron microscopy.

Observation:

  • A 3-month-old infant with secretory diarrhea showed villous atrophy and abundant PAS-positive material in enterocytes.
  • Initial electron microscopy attempts failed to detect definitive microvillous inclusions.
  • Electron microscopy revealed large aggregates of electron-lucent, vermiform membranous vesicles.

Findings:

  • A third electron microscopy session identified sparse microvillous inclusions.
  • The observed vesicles corresponded to the PAS-positive material seen on histology.
  • These findings suggest an expanded spectrum of ultrastructural pathology in MVID.

Implications:

  • Diagnostic criteria for MVID may need to encompass a broader range of ultrastructural features.
  • Challenging cases may require multiple, meticulous electron microscopy examinations.
  • Understanding the varied ultrastructural presentation aids in accurate diagnosis and management of congenital secretory diarrhea.

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