A computer-assisted diagnostic and treatment concept to increase accuracy and safety in the extracranial correction of cranial vault asymmetries

J Oral Maxillofac Surg. 2012 Mar;70(3):677-84. doi: 10.1016/j.joms.2011.02.046. Epub 2011 Jul 31.

Abstract

Purpose: 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.

Materials and 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.

Conclusion: This newly developed and validated method can be successfully implemented in the operating room environment.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Validation Study

MeSH terms

  • Child
  • Clinical Protocols
  • Computer Simulation
  • Computer-Aided Design
  • Craniofacial Abnormalities / complications
  • Craniofacial Abnormalities / diagnostic imaging
  • Craniofacial Abnormalities / surgery*
  • Humans
  • Image Processing, Computer-Assisted / instrumentation
  • Image Processing, Computer-Assisted / methods
  • Imaging, Three-Dimensional*
  • Male
  • Models, Anatomic
  • Osteotomy / methods
  • Patient Care Planning
  • Plastic Surgery Procedures / methods*
  • Proteus Syndrome / complications
  • Proteus Syndrome / diagnostic imaging
  • Proteus Syndrome / surgery*
  • Radiography
  • Reproducibility of Results
  • Skull / surgery*
  • Surgery, Computer-Assisted*
  • Treatment Outcome