Combination of Sturge-Weber Syndrome and Trigonocephaly. Issue 7 (October 2016)

• Record Type: Journal Article
• Authors: Ristow, Oliver; Freudlsperger, Christian; Berger, Moritz; Bächli, Heidi; Hoffmann, Jürgen; Engel, Michael
• Is Part Of: Journal of craniofacial surgery. Volume 27:Issue 7(2016)
• Page Start: e671
• Page End: e673
• Publication Date: 2016-10
• Subjects: Craniosynostosis -- metopic synostosis -- Sturge-Weber syndrome -- trigonocephaly; Facial bones -- Surgery -- Periodicals; Skull -- Surgery -- Periodicals; Face -- Surgery -- Periodicals; Surgery, Plastic -- Periodicals; 617.52
• DOI: 10.1097/SCS.0000000000003055
• Languages: English
• ISSNs: 1049-2275

Abstract

Abstract : 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.