title: Combination of Sturge-Weber Syndrome and Trigonocephaly.
abstract: Regarded singly, both Sturge-Weber syndrome and trigonocephaly are rare congenital disorders. The cardinal features of Sturge-Weber syndrome are facial cutaneous capillary malformation (port-wine stain), leptomeningeal angiomatosis, and glaucoma. Premature closure of the metopic suture results in trigonocephaly. However, to the best of our knowledge, the diagnosis of a combination of both Sturge-Weber syndrome and trigonocephaly has not as yet been reported. This brief clinical study thus presents a patient with the unusual findings of a Sturge-Weber syndrome and simultaneous trigonocephaly induced by premature metopic synostosis. Thus, the rare combination of a port-wine stain involving the first division of the trigeminal nerve with the diagnosis of a craniosynostosis justifies the indication of a prophylactic magnetic resonance imaging acquisition before craniofacial surgeries, in order to prevent seizures and stroke-like episodes triggered by the surgical intervention.
authors.affiliation: *Department of Oral and Maxillofacial Surgery †Department of Neurosurgery, University of Heidelberg, Im Neuenheimer Feld 400, Heidelberg, Germany.
authors.forename: Oliver
authors.initials: O
authors.lastname: Ristow
authors.forename: Christian
authors.initials: C
authors.lastname: Freudlsperger
authors.forename: Moritz
authors.initials: M
authors.lastname: Berger
authors.forename: Heidi
authors.initials: H
authors.lastname: Bächli
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hoffmann
authors.forename: Michael
authors.initials: M
authors.lastname: Engel
mesh_terms: D000015:Abnormalities, Multiple; D003398:Craniosynostoses / Q000175:diagnosis*; D003937:Diagnosis, Differential; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed