[Arachnoid cyst, hypoplasic temporal lobe and facial anomalies: a neurocristopathy].
The authors report on a patient which presented at birth facial anomalies similar to those of facial alcoholic syndrome (i.e. high forehead, wide nasal bridge, upturned nose, flat philtrum), low set ears, short neck. Successive-ly, also an arachnoid right temporo-polar cyst with hypoplasic right temporal lobe was diagnosed. They think that cerebral cyst, hypoplastic temporal lobe and facial anomalies are congenital. By the origin of facial and leptomeningeal structures from the neural crests, the authors conclude that facial, arachnoid and cerebral anomalies depend from the derangement of the neural crest development and that this complex syndrome is a neurocristopathy which can be ascribed to dysneurulation.
Berio
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Dipartimento di Scienze Pediatriche G. De Toni, Cattedra di Pediatria dell'Università di Genova, Genova, Italia.
Piazzi
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D000015:Abnormalities, Multiple / Q000175:diagnosis; D000328:Adult; D016080:Arachnoid Cysts / Q000150:complications* / Q000175:diagnosis; D005146:Facial Asymmetry / Q000150:complications* / Q000175:diagnosis; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009432:Neural Crest / Q000002:abnormalities; D013702:Temporal Lobe / Q000002:abnormalities*