Prenatal Ultrasound and MRI in the Diagnosis of a Rare Fetal Dysplastic Megalencephaly Caused by AKT1 Gene Variant.
Chunying Li, Xiaoyu Song, Jifang Qian et al.
✦ AI-curated · Sources linked
Case in brief
Prenatal ultrasound and MRI identified progressive fetal macrocephaly with abnormal cortical development, raising concern for dysplastic megalencephaly. Whole-exome sequencing detected a somatic AKT1 c.49G>A variant, confirming the diagnosis. After counseling, the pregnancy was terminated.
What made this case unusual
The prenatal imaging findings were linked to a somatic AKT1 variant, identifying an mTOR pathway disorder as the cause of fetal dysplastic megalencephaly.
Diagnostic / clinical pearl
In suspected fetal dysplastic megalencephaly, ultrasound and MRI findings can be complemented by genetic analysis to establish the diagnosis.