Program Operations Manual System (POMS)
TN 32 (08-20)
COMPASSIONATE ALLOWANCES INFORMATION
|
HYDRANENCEPHALY
|
ALTERNATE NAMES
|
Hydroanencephaly
|
DESCRIPTION
|
Hydranencephaly is a rare brain malformation in which the brain’s cerebral hemispheres do not develop,
and are replaced by sacs filled with cerebrospinal fluid. Hydranencephaly is considered
an extreme form of porencephaly (a disorder characterized by a cyst or cavity in the
cerebral hemispheres), and may be caused by intrauterine strokes or infections, or
maternal trauma. Hydranencephaly should not be confused with hydrocephalus, which
is an increased accumulation of cerebrospinal fluid within the ventricles.
|
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
|
Diagnostic testing: The diagnosis is established by imaging such as MRI, CT, after ruling out conditions
that are amenable to treatment. Preliminary diagnosis can be made in utero by ultrasound,
but must be confirmed after birth.
Physical
findings: Infants with hydrancephaly appear normal at birth, but within a few weeks, they may
show:
-
-
•
Abnormal breathing patterns; and
-
Other features developing over time may include:
-
-
-
-
-
•
Spastic quadriparesis (paralysis); and
-
ICD-9:
742.3
ICD-10:
Q04.3
|
PROGRESSION |
Hydranencephaly develops after the 12th week of pregnancy. The prognosis is generally poor, and many children die before
the age of 1 year.
|
TREATMENT
|
There is no definitive treatment for hydraencephaly. Treatment is symptom-specific
and supportive.
|
SUGGESTED PROGRAMMATIC
ASSESSMENT*
|
Suggested MER for Evaluation:
-
•
Clinical history and examination that describes the diagnostic features of the impairment;
-
•
Cranial MRI or CT scan; and
-
•
Developmental assessment.
|
Suggested Listings for Evaluation:
|
DETERMINATION
|
LISTING
|
REMARKS
|
Meets
|
110.08 A
|
|
Equals
|
|
|
* 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.
|