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In the present paper, the authors described difficulties in employing diagnostic imaging in differentiating between type II (open-lip) schizencephaly and much more common intracranial fluid spaces of a different origin (arachnoid cysts and hydrocephalus).
The method of choice in diagnostic imaging of schizencephaly is MRI. CT is also useful, but to a lesser degree, since it provides poorer images of the gray matter, which are the key factor in differentiating between the malformation and other fluid-associated CNS abnormalities. Schizencephaly may be also diagnosed in prenatal or postnatal ultrasonography, but this is true for type II (open-lip) only.
A particular problem in differentia diagnosis is posed by open-type schizencephaly, which in medical imaging is seen as a fluid-filled space. Inappropriate interpretation of the image may result in inappropriate further management. In cases when imaging studies demonstrate intracranial fluid-filled spaces, especially when they are situated in the middle cranial fossa, differential diagnosis needs to take into consideration several pathologies. In the majority of cases, in this location, arachnoid cysts are seen. A very rare congenital pathology is schizencephaly. Differentiation between these two malformations poses a significant diagnostic problem. Inasmuch as therapeutic management of symptomatic forms of arachnoid cysts consists in a surgical intervention, in case of schizencephaly, the value of the method is doubtful and the modality is additionally associated with consequences in the form of postoperative complications.
The most differentiating important element in imaging studies is the presence of heterotrophic gray matter that lines the margins of the cleft in case of schizencephaly and absence of such a lining in arachnoid cysts or fluid-filled spaces with other background. Moreover, arachnoid cysts may cause a mass effect manifested as displacement of the longitudinal fissure of the brain, compression of the ventricular system, or local obliteration of brain sulci and fissures, what is not evident in schizencephaly. In rare instances of arachnoid cysts, thinning and bulging of cranial bones are seen in areas where they adhere to the cyst.
Initial diagnostic MRI results for both groups are summarized in Table 3. PCNSL lesions were generally localized supratentorially (66.7%). PCNSL affected both white and grey matter, basal ganglia involvement was present in 55.6%, and cortical grey matter was affected in 51.9%. Cortical grey matter was involved by both enhancing and non-enhancing tumorous infiltration in 37.1% of cases, only enhancing portion was present in 3.7% of cases, only non-enhancing infiltration was seen in 11.1% of cases. Solitary affection of white matter was found only in 7.4%; in 3.8% an isolated involvement of basal ganglia was present and in one case (1.9%) solitary infiltration of hypothalamus or vestibular nerve was found. At time of initial MRI, PCNSL appeared as multiple infiltrative lesions in 51.9% of cases, as a solitary infiltrative lesion in 20.4% of cases and as a diffuse infiltrative affection in 24.1% (Fig. 1). Solitary demarcated lesions were rare (3.7%). PCNSL enhanced homogenously (64.8%), vasogenic perifocal edema was present in most cases (92.6%), and diffusion restriction was detected in 97% of cases (Fig. 2). Optic nerves and tracts were infiltrated in 42.6% of cases (Fig. 3). Other cranial nerves were affected in 5.6% of PCNSL cases; in one case (1.9%) the optic and trigeminal nerve was affected, and in one case (1.9%) the optic nerves and both auditory and facial nerves were involved and in one case (1.9%) solitary infiltration of the auditory nerve was present without other brain lesions (Fig. 4a, b). PCNSL typically reached the surface of the brain (87%) with meningeal infiltration present in 35.2% of cases and ependymal infiltration in 53.7% of cases. Signs of bleeding (5.6%) were rare.
Our MRI findings in GBM are in agreement