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

[proteus syndrome]

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 .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 (3 D ) 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 3 D real-time navigation was installed as an additional visualization and security feature .The surgery could be performed safely and quickly . Postoperative imaging showed that the surgical plan was realized with high accuracy .This newly developed and validated method can be successfully implemented in the operating room environment .

Diseases
Validation

Diseases presenting "but in about 30%" symptom

proteus syndrome

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