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Abstract #70084 Published in IGR 18-2
Combination of Sturge-Weber Syndrome and Trigonocephaly
Ristow O; Freudlsperger C; Berger M; Bächli H; Hoffmann J; Engel MJournal of Craniofacial Surgery 2016; 27: e671-e673
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.
*Department of Oral and Maxillofacial Surgery †Department of Neurosurgery, University of Heidelberg, Im Neuenheimer Feld 400, Heidelberg, Germany.
Full articleClassification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
