Prenatal Diagnosis and Management of a Huge Infratentorial/Supratentorial Multiloculated Arachnoid Cystic Malformation in an Infant
Pediatric Neurology
A 23-year-old woman had a normal fetal ultrasound at 16 weeks' gestation. A second ultrasound at 25 weeks revealed a cystic brain malformation in the fetus. A fetal magnetic resonance image (MRI) at 28 weeks showed the presence of a huge infratentorial/supratentorial multiloculated arachnoid cystic malformation and hydrocephalus (A) . Although the prognosis was highly uncertain, the possibility of blindness, endocrine abnormalities, and severe developmental delay was discussed with the family. At 38 weeks, an infant boy was delivered via elective cesarian section with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Other than macrocephaly with full fontanelles, the physical examination was normal and without evidence of diabetes insipidus. A postdelivery MRI found the cystic malformation to have enlarged when compared with the fetal study (B). A craniotomy was performed to fenestrate this multiloculated cystic malformation . Histology of the submitted tissue was arachnoid. No problems were encountered postoperatively, and the infant was discharged home where growth and development progressed normally and the head circumference diminished by 50%. At 6 months an MRI (C) showed an increase in size of the third ventricle and both lateral ventricles when compared with the MRI performed just after the fenestration. As continued enlargement was expected, a second operative procedure was performed consisting of an endoscopic cyst fenestration and third ventriculostomy. At 9 months of age, an MRI (D) showed a decrease in the size of the cyst and lateral ventricles. At 1 year of age, his cognitive function appears mildly delayed. His vision is grossly normal; ocular motility is full, conjugate, and free of nystagmus. Tone and strength are normal and without focal deficit.
Original Article: http://www.pedneur.com/article/PIIS0887899413001355/abstract?rss=yes
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