Complications
Because of location, a cavum velum interpositum is not usually related to complications, but mass effect was reported with ventriculomegaly, hydrocephaly and macrocephaly [3, 6, 8, 9, 10].
Progression
The gestational age at the time of diagnosis varies between 19 and 36 weeks. The reported diameter is from 9 to 30 mm. Among cases detected prenatally, some decreased in size, some lesions were stable and some enlarged slightly during pregnancy. The cavum velum interpositum may undergo spontaneous resolution after delivery [1-3, 5, 6, 10, 12, 13].
Prognosis
The prognosis in prenatally diagnosed cavum velum interpositum is ususally good [3, 4, 12]. The prognosis associated with cavum velum interpositum cysts is uncertain and may be related to the size of the cyst. Psychotic disorders, low-pressure hydrocephalus, neurodevelopmental delays, epilepsy and multiple cranial neuropathies have been reported in patients with large cavum velum interpositum cysts [1, 3, 14].
Clinical importance
It is suggested that the cavum velum interpositum is a normal developmental phase of the cerebral midline and usually disappears with brain maturation by the end of the first year of life and, hence, has no pathological meaning. But it is not certain whether cavum velum interpositum cysts represent a simple variant of the norm or are developmental malformations since some neurodevelopmental complications are also reported. An arachnoid cyst of the velum interpositum can cause symptoms similar to those seen with a third ventricular mass [6, 9, 11].
Management
Careful fetal neurosonography is mandatory to rule out associated anomalies which can influence postnatal outcome. When the diagnosis of isolated cavum velum interpositum cyst is certain, no additional prenatal investigations are necessary and an expectant approach is recommended with monthly sonographic examinations until delivery. Due to the reported association of large cavum velum interpositum cysts and adverse pediatric outcome, postnatal sonographic follow-up with serial examinations at delivery and at 6 and 12 months is recommended [3, 5].
Differential diagnosis
The differential diagnosis of midline intracranial cystic lesions is a diagnostic challenge. The localization of internal cerebral veins is of great importance in the differential diagnosis that include:- an arachnoid cyst (most of them are supratentorial, and an arachnoid cyst at this location is extremely rare: only one third of cases are located in the posterior fossa. An arachnoid cyst is located below the internal cerebral veins. Median diameter of an arachnoid cyst is 40 mm (range, 10–80 mm). Associated findings are rather common);- dilated cavum septi pellucidi (located anterior to the foramen of Monro, between the frontal horns; also triangular, but with the apex pointing posteriorly);- cavum vergae (is superior and anterior to the columns of the fornix in the midsagittal view, rectangular in cross-section (parallel wall), located superior to the columns of the fornices, which are displaced inferiorly. Internal cerebral veins are the most important landmark that helps in differentiating. Whereas CV lies above these veins, cavum velum interpositum encloses them at its lower and lateral borders);- an arachnoid cysts of the quadrigeminal cistern (located below the internal cerebral veins; may compress the tectum of the midbrain; usually eccentric, and not triangular in cross section);- a vein of Galen aneurysm (color Doppler demonstrates arterio-venous malformation);- a pineal cyst (ovoid or spherical, located below the internal cerebral veins. abutting the colliculi [1- 5, 8-10].
Treatment
Fetuses with progressive hydrocephalus may require ventriculoperitoneal shunting. Surgical fenestration of the cyst is sometimes needed in case of hydrocephalus result from an arachnoid cyst of the velum interpositum [3, 6, 13].
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