Zika virus disease; ZVD; CZS

Congenital Zika syndrome (CZS) is a pattern of birth defects caused by in utero infection of the fetal brain with theZika virus.

Diagnostic testing:

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  Positive Zika virus NAAT (Nucleic Acid Amplification Test) and IgM in the blood and CSF of the infant within a few days after birth; and

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  Positive plaque reduction neutralization testing (PRNT) at birth.

Physical findings: The physical findings below are usually seen in Zika syndrome but may also be seen in other congenital infections of the fetal brain.

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  Severe microcephaly (abnormally small head);

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  Decreased brain tissue with a specific pattern of brain damage, including subcortical calcifications;

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  Damage to the back of the eye, including macular scarring and focal retinal pigmentary mottling;

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  Congenital contractures, such as clubfoot or arthrogryposis; and

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  Hypertonia restricting body movement soon after birth.

Other findings for CZS may include:

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  Growth retardation;

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  Progressive, irreversible intellectual disability;

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  Brain atrophy and asymmetry;

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  Abnormally formed or absent brain structures;

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  Hydrocephalus (accumulation of cerebrospinal fluid within the brain);

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  Neuronal migration disorders;

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  Excessive and redundant scalp skin;

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  Hyperreflexia (overactive or overresponsive reflexes);

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  Irritability;

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  Tremors;

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  Seizures;

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  Brainstem dysfunction;

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  Dyspagia (difficulty swallowing);

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  Chorioretinal atrophy in the macula;

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  Optic nerve hypoplasia, cupping, and atrophy;

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  Other retinal lesions;

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  Iris colobomas (holes in the iris);

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  Congenital glaucoma;

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  Microphthalmia (abnormally small eyes);

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  Lens subluxation;

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  Cataracts; and

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  Intraocular calcifications.

ICD-9: 066.3

ICD-10: A92.5

A small percentage of children born with microcephaly have normal intelligence. Most children have issues with walking, communicating, coordination, and speech issues; and need constant attention and care.

There is no current treatment available for CZS. Some of the intervention programs like occupational therapy, speech assistance, and special needs help can provide some help.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Positive CZS-specific immunological testing;

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  Neuroimaging (cranial ultrasound, computed tomography, or magnetic resonance imaging) any time after birth showing microcephaly; and

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  Brainstem auditory evoked response (BAER) to document hearing impairment.

Suggested Listings for Evaluation:

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