PMC9709204_01
Electroencephalogram findings. (A) Low amplitude fast waves superimposed over diffused slow wave activity. (B) Hypsarrhythmia. EEG findings improved dramatically at 2 months after the surgery (C, D).
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F1
EEG pattern (Figure 1), the patient was diagnosed with infantile epileptic spasms syndrome. Epileptic spasms were manifested as rhythmic fast waves in the left temporal and occipital region. An EEG performed 2 months after surgery showed a significant improvement (Figure 1).
Prenatal magnetic resonance images. (1) T2-weighted image at 25 weeks of gestation. (2) T2-weighted image at 35 weeks of gestation. Axial (A, B) and coronal (D) images showed symmetrically dilated lateral ventricles, and corpus callosum agenesis (black arrow); sagittal (C) images showed abnormal morphology of the right ventricle.
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F2
Furthermore, fetal brain magnetic resonance imaging (MRI) was performed at 25 and 35 weeks, describing symmetrically dilated lateral ventricles, corpus callosum agenesis, and abnormal morphology of the right ventricle (Figure 2).
Postnatal magnetic resonance images. The brain MRI showed dilated ventricles, corpus callosum agenesis, and subependymal pseudocyst 3 days after birth (A). A large mass located in the posterior horn of the right ventricle with T2 heterogeneously hyperintense and T1 hypointense at 6 months (B) and 10 months (C) after birth with progression. T2- and T1-weighted images showed a Normal appearing choroid plexus and porencephalic cavity 2 weeks after surgery (D).
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However, a large mass in the posterior horn of the right lateral ventricle with T2 heterogeneously hyperintense and T1 hypointense was observed in the brain MRI scan and considered to be a CPP (Figure 3).
Clinical course of the patient and identification of a choroid plexus papilloma in the panel, the patient's mother was admitted to our institution for prenatal counseling due to fetal ventriculomegaly at 22 weeks of gestation. Amniocentesis showed a Normal karyotype and chromosomal microarray analysis. Fetal infection results were within Normal limits. fetal brain magnetic resonance imaging was performed at 25 and 35 weeks, describing symmetrically dilated lateral ventricles, progressive enlargement (from 10 mm at 22 weeks to 14 mm at 35 weeks), corpus callosum agenesis (blue arrow), and abnormal morphology of the right ventricle (subpanels A, B). A diagnosis of non-isolated ventriculomegaly was made and a female was delivered vaginally with Normal physical and neurological examinations. Brain MRI 3 days after birth showed dilated left ventricle, corpus callosum agenesis, and subependymal pseudocyst (subpanel C). At 5 months of age, epileptic spasms and developmental delay emerged, and hypsarrhythmia was evident on electroencephalogram (subpanels G and H). Levetiracetam and valproic acid failed to control the seizures. Routine laboratory tests and whole-exome sequencing were unremarkable. A large mass located in the posterior horn of the right ventricle with T2 heterogeneously hyperintense and T1 hypointense at 6 months (subpanel D) and 10 months (subpanel E) after birth with progression. A large mass was removed through a right parietal craniotomy and the histopathologic examination showed the mass was a choroid plexus papilloma. T2- and T1-weighted images showed a Normal appearing choroid plexus and porencephalic cavity 2 weeks after surgery (subpanel F). The child was maintained postoperatively on anticonvulsants and no seizures occurred. Throughout follow-up, he showed continuing developmental improvement. EEG findings improved dramatically at 2 months after the surgery (subpanel I).
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The detailed clinical course of the patient is shown in Figure 4.