Spring-assisted cranial vault expansion in the setting of multisutural craniosynostosis and anomalous venous drainage: case report

Abstract

Patients with multisutural craniosynostosis can develop anomalous venous connections between the intracranial sinuses and cutaneous venous system through enlarged emissary veins. Cranial vault remodeling in this subset of patients carries the risk of massive intraoperative blood loss and/or occlusion of collateral draining veins leading to intracranial venous hypertension and raised intracranial pressure, increasing the morbidity of cranial expansion. The authors report the use of spring-mediated expansion as a technique for cranial reconstruction in which the collateral intracranial venous drainage system can be preserved. A patient with bilateral lambdoid, sagittal, and unicoronal synostosis presented for cranial reconstruction. A tracheostomy and ventriculoperitoneal shunt were placed prior to intervention. At the time of reconstruction, a Luckenschadel skull abnormality and Chiari malformation Type I were present. A preoperative CT venogram demonstrated large collateral superficial occipital veins, small bilateral internal jugular veins, and hypoplastic jugular foramina. Collateral flow from the transverse and sigmoid sinuses through large occipital emissary veins was seen. Spring-mediated cranial vault expansion was performed with care to preserve the large collateral veins at the occipital midline. Four springs were placed at each lambdoid and the posterior and anterior sagittal sutures following 1-cm strip suturectomies. Removal of the springs was performed 2 months postoperatively. Cranial vault expansion was performed without disturbing the aberrant intracranial/extracranial venous collateral system. Estimated blood loss was 150 ml. A CT scan obtained 3 months postoperatively showed resolution of the Luckenschadel deformity and a 40% volumetric increase in the skull compared with the preoperative CT. Patients with anomalous venous drainage patterns and multisutural synostosis can undergo spring-mediated cranial vault expansion while preserving the major emissary veins draining the intracranial sinuses. Risks of blood loss, intracranial venous hypertension, and increased intracranial pressure may be decreased compared with traditional techniques of repair.