Program Operations Manual System (POMS)
TN 53 (08-22)
DI 23022.453 Microvillus Inclusion Disease – Child
COMPASSIONATE ALLOWANCES INFORMATION
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MICROVILLUS INCLUSION DISEASE
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ALTERNATE NAMES
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Congenital Enteropathy; Congenital Familial Protracted Diarrhea; Congenital Microvillus
Atrophy; Davidson’s Disease; Familial Protracted Enteropathy; Microvillus Familial
Enteropathy; Microvillus Atrophy; Microvillous Inclusion Disease; MVID
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DESCRIPTION
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Microvillus Inclusion Disease (MVID) is a rare congenital intestinal disorder that primarily affects newborns. MVID manifests
in the first hours or days of life with chronic watery diarrhea that increases in
frequency with food intake, causing malnutrition and dehydration. Fewer than 100 cases
have been documented.
MVID is caused by a mutation in the Myo5b gene and follows an autosomal recessive
inheritance pattern. The Myo5b gene regulates proteins in the epithelial lining of
the intestines. In infants with MVID, this gene is non-functional; as a result, intestinal
microvilli, structures which aid in the absorption of nutrients, are deformed or entirely
absent.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: A diagnosis of MVID can be confirmed through electronic microscope observation of
an intestinal tissue sample, or through genetic testing. Other conditions such as
lactose intolerance and familial chloride diarrhea must be ruled out before performing
a biopsy.
Physical findings: Symptoms of MVID include:
ICD-9: 751.5
ICD-10: P78.3; Q43.8
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PROGRESSION
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MVID typically leads to developmental delay, failure to thrive, irreversible damage
to the liver and kidneys, and often results in death in infancy. Most children with
MVID do not survive past early childhood.
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TREATMENT
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There is no cure for MVID. Parenteral (intravenous) nutrition is the most common form
of treatment, but it is not a viable long-term option due to risk of infection and
organ damage.
Intestine transplantation has more favorable outcomes. However, donor organs are not
widely available and lifelong treatment is necessary even with a successful transplant.
Other treatment is symptomatic and supportive.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Electron microscopic analysis of intestinal tissue sample; and
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Results of molecular genetic testing for mutation of Myo5b.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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105.08
105.10
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Equals
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105.07
105.09
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Requirement for parenteral feeding equals listing.
Small intestine transplantation, if performed, equals listing.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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