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Combination of Sturge-Weber syndrome and trigonocephaly

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 tr...

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Bibliographische Detailangaben
Hauptverfasser: Ristow, Oliver (VerfasserIn) , Freudlsperger, Christian (VerfasserIn) , Berger, Moritz (VerfasserIn) , Bächli, Heidi (VerfasserIn) , Hoffmann, Jürgen (VerfasserIn) , Engel, Michael (VerfasserIn)
Dokumenttyp: Article (Journal) Editorial
Sprache:Englisch
Veröffentlicht: [October 2016]
In: The journal of craniofacial surgery
Year: 2016, Jahrgang: 27, Heft: 7, Pages: e671-e673
ISSN:1536-3732
DOI:10.1097/SCS.0000000000003055
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1097/SCS.0000000000003055
Verlag, lizenzpflichtig, Volltext: https://journals.lww.com/jcraniofacialsurgery/Fulltext/2016/10000/Combination_of_Sturge_Weber_Syndrome_and.121.aspx
Volltext
Verfasserangaben:Oliver Ristow, MD, DDS, Christian Freudlsperger, MD, DDS, Moritz Berger, MD, DDS, Heidi Bächli, MD, Jürgen Hoffmann, MD, DDS, and Michael Engel, MD, DDS

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520 |a 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. 
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