Small intestine & ampulla
Malabsorption
Microvillus inclusion disease
Author: Hanni Gulwani, M.B.B.S.
Last author update: 1 August 2012
Last staff update: 18 November 2020
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Microvillus inclusion disease small bowel
Table of Contents
Definition / general | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Electron microscopy description | Electron microscopy imagesCite this page: Gulwani H. Microvillus inclusion disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/smallbowelmicrovillusinclusiondisease.html. Accessed March 28th, 2023.
Definition / general
- Also called congenital or familial microvillous atrophy
- Disorder of intestinal brush border that causes intractable watery diarrhea with steatorrhea in infants
- Patients require total parental nutrition and rarely live beyond age 2 years
- Villous atrophy may be due to apoptotic cell loss (Hum Pathol 2000;31:1404)
Treatment
- Small bowel transplant
Microscopic (histologic) description
- Severe villous abnormality with crypt hypoplasia, resembling celiac sprue but without lymphocytosis
- Increased enterocyte apoptosis and proliferation, bubbly vacuolated apical cytoplasm with extensive or patchy absence of brush border, absence of inflammation (Ultrastruct Pathol 2010;34:327)
Microscopic (histologic) images
Images hosted on other servers:
Various images
Positive stains
- CD10 (Am J Surg Pathol 2002;26:902), PAS, polyclonal CEA, alkaline phosphatase (cytoplasmic staining vs. linear brush border staining in normals)
- Vacuoles - PAS, CEA
- Cytoplasmic CD10 staining of absorptive colonocytes can aid in the diagnosis in situations where only colonic biopsy could be obtained (Am J Surg Pathol 2010;34:970)
Electron microscopy description
- Abnormal microvillus structures at luminal border of enterocytes
- Apical intracytoplasmic inclusions lined by microvilli
Electron microscopy images
Images hosted on other servers:
Neonatal congenital microvillus atrophy
