Abnormal venous drainage in syndromic craniosynostosis and the role of CT venography

Objective Venous hypertension is emerging as a significant contributor to intracranial pressure in children with syndromic craniosysnostosis. This is associated with jugular foramen stenosis or atresia and with the development of collateral emissary veins. We demonstrate how computed tomography venography can document the prevalence of these emissary veins and how their visualisation plays an important role in operative planning. Materials and methods Patients with known syndromic craniosynostosis underwent computed tomography venography as part of their routine pre-operative evaluation. The venous drainage pattern was examined in these patients, with a special note of the presence of abnormal venous pathways. Results Eleven patients were recruited into the study from ages 3 months to 22 years. All had a diagnosis of syndromic craniosynostosis with six Crouzon’s, four Pfeiffer’s and one patient with Crouzon’s and acanthosis nigricans. Nine of 11 patients had demonstrable evidences of transosseous venous drainage through an identifiable abnormal emissary vein. Four of 11 had a transosseous route as the main mechanism of drainage for the cerebral venous system. Conclusions Patients with syndromic craniosynostosis often demonstrate abnormal venous anatomy, which can have serious consequences on craniofacial surgery, especially when a posterior decompression is being considered. Based on these findings, the authors assert that those children with some syndromic craniosynostosis being considered for surgery should undergo venographic studies as part of their pre-operative evaluation.