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Vascular anomaly, lipoma, and polymicrogyria associated with schizencephaly: developmental and diagnostic insights. Illustrative case

Kevin K. Kumar, Angus Toland, Nancy Fischbein, Martha Morrell, Jeremy J. Heit, Donald E. Born, and Gary K. Steinberg

BACKGROUND

Schizencephaly is an uncommon central nervous system malformation. Intracranial lipomas are also rare, accounting for approximately 0.1% of brain “tumors.” They are believed to be derived from a persistent meninx primitiva, a neural crest–derived mesenchyme that develops into the dura and leptomeninges.

OBSERVATIONS

The authors present a case of heterotopic adipose tissue and a nonshunting arterial vascular malformation arising within a schizencephalic cleft in a 22-year-old male. Imaging showed right frontal gray matter abnormality and an associated suspected arteriovenous malformation with evidence of hemorrhage. Brain magnetic resonance imaging revealed right frontal polymicrogyria lining an open-lip schizencephaly, periventricular heterotopic gray matter, fat within the schizencephalic cleft, and gradient echo hypointensity concerning for prior hemorrhage. Histological assessment demonstrated mature adipose tissue with large-bore, thick-walled, irregular arteries. Mural calcifications and subendothelial cushions suggesting nonlaminar blood flow were observed. There were no arterialized veins or direct transitions from the arteries to veins. Hemosiderin deposition was scant, and hemorrhage was not present. The final diagnosis was consistent with ectopic mature adipose tissue and arteries with meningocerebral cicatrix.

LESSONS

This example of a complex maldevelopment of derivatives of the meninx primitiva in association with cortical maldevelopment highlights the unique challenges from both a radiological and histological perspective during diagnostic workup.

Open access

Frontal intradiploic encephalocele in a 44-year-old male patient: illustrative case

Baran Atli, Sebastian Rath, Johannes Burtscher, Johannes A. Hainfellner, and Simon Hametner

BACKGROUND

Encephaloceles are protrusions of the cerebral tissue through a skull defect. They occur mostly in children and very rarely in adults.

OBSERVATIONS

The authors present a case of a 44-year-old man presenting with a first-time generalized seizure. Computed tomography of the head showed bone destruction associated with a right frontal lesion. Magnetic resonance imaging scans demonstrated a largely isointense lesion in the intradiploic space that contained small, hyperintense nodular components and showed a low to moderate contrast agent enhancement.

LESSONS

The patient underwent resection, during which the histological examination found the lesion to be an intradiploic encephalocele. The patient had an uneventful postoperative course with a cessation of seizures. The imaging and neuropathological findings as well as a literature review, together with a discussion on the etiology of intradiploic encephaloceles, are contained in this report.