Unifying Concepts

Features of Congenital Zika Syndrome

The majority (50 to 80%) of Zika virus (ZIKV) infections are asymptomatic. Symptomatic ZIKV infection has an incubation period of 3 to 14 days and is a mild illness, with a duration of up to 1 week, that manifests as a rash, low-grade fever, arthralgia and myalgia, and conjunctivitis. Complications are infrequent, but when they occur, they are severe and may be fatal.

The ZIKV causes a spectrum of fetal and birth defects that extends beyond microcephaly and is distinct from other congenital infections in that its pathologic manifestations are restricted primarily to the central nervous system.

Features of Congenital Zika Syndrome

Lesion Type Manifestations
Structural lesions
Fetal brain disruption sequence* Severe microcephaly, premature closure of fontanels, collapsed skull, overlapping sutures, redundant scalp skin
Brain abnormalities Cortical atrophy with decreased myelination, cerebellar hypoplasia Neuronal migration disorder – lissencephaly, agyria, pachygyria, polymicrogyria, heterotopia, dysgenesis of corpus callosum
Calcifications, mainly subcortical*
Ventriculomegaly, increased posterior fossa and pericerebral spaces
Ocular abnormalities Pigmented retinal mottling*, chorioretinal atrophy*, macular scarring, glaucoma, optic nerve atrophy and abnormalities, intraocular calcifications
Microphthalmia, anophthalmia
Iris coloboma, lens subluxation, cataract
Congenital contractures Arthrogryposis, talipes equinovarus, hip dislocation
Intrauterine growth restriction
Functional lesions
Pyramidal or extrapyramidal abnormalities* Body tone abnormalities (mainly hypertonia), swallowing disorder, movement abnormalities (dyskinesia, dystonia), hyperexcitability, impatient crying, sleep disorders
Visual impairment (strabismus, nystagmus, vision loss)
Hearing loss or deafness
Developmental delay

* Lesions are rarely observed in other congenital infections.



  1. Musso D, Ko AI, Baud D. Zika Virus Infection – After the Pandemic. N Engl J Med. 2019 Oct 10;381(15):1444-1457. [Medline]
  2. Lebov JF, Brown LM, MacDonald PDM, Robertson K, Bowman NM, Hooper SR, Becker-Dreps S. Review: Evidence of Neurological Sequelae in Children With Acquired Zika Virus Infection. Pediatr Neurol. 2018 Aug;85:16-20. [Medline]


Created Oct 11, 2019.

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