Schizencephaly, type II

Fabrice Cuillier L. Lagarde

Article Published: Apr 29, 2008

Fabrice Cuillier, MD*; L. Lagarde, MD**.

* Department of Gynecology, Félix Guyon’Hospital, 97400 Saint-Denis, Ile de la Réunion, France;
** Department of Gynecology, Hôpital , 97400 Saint-Paul, Ile de la Réunion, France.

Case report

A 20-year-old woman was referred to our unit at 26 weeks for a targeted ultrasound examination due to colpocephaly. It was a first pregnancy of a nonconsanguineous couple. Her first sonography screening, performed at 13 weeks, was normal (nuchal translucency 0,5 mm, CRL 60 mm), and so was her triple test at 16 weeks.
Our examination found a normal fetal biometry, consistent with 26th week of gestational age, but the fetus presented with several anomalies:

Dilatation of the lateral and third cerebral ventricles accompanied with the type II of schizencephaly;
Normal corpus callosum;
Bilateral club feet.

MRI was practiced at 30 weeks and the anomalies were confirmed.

Serological tests (Toxoplasmosis, Rubeola, CMV, Herpes and Chikungunya) were normal. The parents opted for the interruption of the pregnancy at 32 weeks, but refused the autopsy.

Images 1, 2: Transverse scans of the fetal head showing communication between the enlarged lateral ventricles medially due to presence of the cleft affecting the sylvian fissure. The lateral clefting connected the lateral ventricle and subarachnoid space is not clearly visible on these images.

Images 3, 4: Transverse scans of the fetal head showing communication between the enlarged lateral ventricles medially due to presence of the cleft affecting the sylvian fissure. The lateral clefting connected the lateral ventricle (more distal to the probe) and subarachnoid space is visible on the image 3.

Images 5, 6: Coronal scans of the fetal head showing dilatation of the third cerebral ventricle. The image 6 shows lateral cleft connecting lateral ventricle and subarachnoid space (fingers).