title: A computer-assisted diagnostic and treatment concept to increase accuracy and safety in the extracranial correction of cranial vault asymmetries.
abstract: OBJECTIVE
  Proteus syndrome is described as a progressive, asymmetric, disproportional overgrowth of various parts of the body. The theory of somatic mosaicism is widely accepted to be the cause of this disease. Affected patients present very heterogeneous symptoms, but in about 30% craniofacial deformities are the leading clinical features. Because no causal therapy exists, treatment options are limited to surgical improvement of functional constraints.
  
  
  METHODS
  A computer-assisted method was used to increase the accuracy and safety of bone removal in the extracranial correction of cranial vault asymmetries. Descriptions of the diagnosis, preoperative planning, and intraoperative management of craniofacial dysmorphia caused by Proteus syndrome in a 6-year-old boy are presented. After computed tomography-based generation of a virtual 3-dimensional (3D) model of the patient and a haptic stereolithographic model to display the special pathology, flow-sensitized 4-dimensional magnetic resonance imaging was performed to clarify the properties of vascular formation inside the hyperostosis. To transfer the mathematically optimized preoperative planning of a new skull shape to the patient, a surgical guide was fabricated by rapid manufacturing. Intraoperative 3D real-time navigation was installed as an additional visualization and security feature.
  
  
  RESULTS
  The surgery could be performed safely and quickly. Postoperative imaging showed that the surgical plan was realized with high accuracy.
  
  
  CONCLUSIONS
  This newly developed and validated method can be successfully implemented in the operating room environment.
authors.affiliation: Department of Craniomaxillofacial Surgery, University Hospital Basel, University of Basel, Basel, Switzerland. pjuergens@uhbs.ch
authors.forename: Philipp
authors.initials: P
authors.lastname: Juergens
authors.forename: Jörg
authors.initials: J
authors.lastname: Beinemann
authors.forename: Maarten
authors.initials: M
authors.lastname: Zandbergen
authors.forename: Stefan
authors.initials: S
authors.lastname: Raith
authors.forename: Christoph
authors.initials: C
authors.lastname: Kunz
authors.forename: Hans-Florian
authors.initials: HF
authors.lastname: Zeilhofer
mesh_terms: D002648:Child; D002985:Clinical Protocols; D003198:Computer Simulation; D017076:Computer-Aided Design; D019465:Craniofacial Abnormalities / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000295:instrumentation / Q000379:methods; D021621:Imaging, Three-Dimensional; D008297:Male; D008953:Models, Anatomic; D010027:Osteotomy / Q000379:methods; D010347:Patient Care Planning; D016715:Proteus Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D011859:Radiography; D019651:Plastic Surgery Procedures / Q000379:methods*; D015203:Reproducibility of Results; D012886:Skull / Q000601:surgery*; D025321:Surgery, Computer-Assisted; D016896:Treatment Outcome