{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,3,27]],"date-time":"2023-03-27T02:40:11Z","timestamp":1679884811890},"reference-count":26,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2011,9]]},"abstract":"<jats:p>Craniometaphyseal dysplasia is an extremely rare, genetic bone-remodeling disorder. Comparable to osteopetrosis, fibrous dysplasia, and other infrequent conditions, craniometaphyseal dysplasia is characterized by progressive diffuse hyperostosis of the neuro- and viscerocranium. Affected patients present with a pathognomonic dysmorphia: macrocephalus, hypertelorism, bulky facial skeleton, and a prominent mandible. Progressive thickening and petrification of the craniofacial bones can continue throughout life, often resulting in neurological symptoms due to obstruction of the cranial nerves in the foramina and therefore immediately requiring neurosurgical interventions to avoid persistent symptoms with severe impairment of function. Treatment is largely infeasible given the lack of suitable tools to perform a craniotomy through the gross calvarial bone.<\/jats:p>\n<jats:p>In this paper, the authors present a complete process chain from the CT-based generation of an individual patient's model displaying his pathology to optimized preoperative planning of the skull's shape with a thickness of about 6\u20137 mm. For concise verification of the surgical plan in an operating room environment, a 3D real-time navigation prototype system was utilized. To guarantee realization of the surgery in a reasonable time frame, the mechanical tools were preoperatively selected for optimizing the ablation rate in porcine and bovine bone, which were comparable to that in the patient. This process chain was developed in a modular way, so that it could be easily adopted completely or partially for other surgical indications.<\/jats:p>\n<jats:p>A 21-year-old man was treated according to this sophisticated concept. Skull bone more than 50 mm thick in some regions was reduced to physiological thickness. The patient was thus in a stage that neurosurgical interventions could be performed with a regular risk within a reasonable time of treatment.<\/jats:p>","DOI":"10.3171\/2011.3.jns101517","type":"journal-article","created":{"date-parts":[[2011,4,15]],"date-time":"2011-04-15T18:39:11Z","timestamp":1302892751000},"page":"528-535","source":"Crossref","is-referenced-by-count":4,"title":["Enabling an unimpeded surgical approach to the skull base in patients with cranial hyperostosis, exemplarily demonstrated for craniometaphyseal dysplasia"],"prefix":"10.3171","volume":"115","author":[{"given":"Philipp","family":"Juergens","sequence":"first","affiliation":[{"name":"Department of Cranio-Maxillofacial Surgery, University Hospital Basel, University of Basel;"},{"name":"Hightech Research Center of Cranio-Maxillofacial Surgery, University of Basel;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Javier","family":"Ratia","sequence":"additional","affiliation":[{"name":"Biomaterial Science Center, University of Basel;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00f6rg","family":"Beinemann","sequence":"additional","affiliation":[{"name":"Hightech Research Center of Cranio-Maxillofacial Surgery, University of Basel;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zdzislaw","family":"Krol","sequence":"additional","affiliation":[{"name":"Hightech Research Center of Cranio-Maxillofacial Surgery, University of Basel;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kurt","family":"Schicho","sequence":"additional","affiliation":[{"name":"University Hospital of Cranio-Maxillofacial and Oral Surgery, Medical University of Vienna, Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christoph","family":"Kunz","sequence":"additional","affiliation":[{"name":"Department of Cranio-Maxillofacial Surgery, University Hospital Basel, University of Basel;"},{"name":"Hightech Research Center of Cranio-Maxillofacial Surgery, University of Basel;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hans-Florian","family":"Zeilhofer","sequence":"additional","affiliation":[{"name":"Department of Cranio-Maxillofacial Surgery, University Hospital Basel, University of Basel;"},{"name":"Hightech Research Center of Cranio-Maxillofacial Surgery, University of Basel;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephan","family":"Zimmerer","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University Hospital Basel, University of Basel, Switzerland; and"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-jns101517","series-title":"J Craniomaxillofac Surg","first-page":"391","article-title":"Craniofacial hyperostoses in Proteus syndrome\u2014a case report","volume":"32","author":"Adolphs","year":"2004"},{"key":"b2-jns101517","series-title":"Neurol India","first-page":"97","article-title":"Craniofacial surgery for craniometaphyseal dysplasia","volume":"54","author":"Ahmad","year":"2006"},{"key":"b3-jns101517","series-title":"J Pediatr","first-page":"541","article-title":"Infantile cortical hyperostoses","volume":"29","author":"Caffey","year":"1946"},{"key":"b4-jns101517","series-title":"Proc R Soc Med","first-page":"347","article-title":"Infantile cortical hyperostosis; a review of the clinical and radiographic features","volume":"50","author":"Caffey","year":"1957"},{"key":"b5-jns101517","series-title":"Arch Otolaryngol Head Neck Surg","first-page":"566","article-title":"Skull base manifestations of Camurati-Engelmann disease","volume":"136","author":"Carlson","year":"2010"},{"key":"b6-jns101517","series-title":"Cir Cir","first-page":"437","article-title":"Cranial vault reconstruction using computer-designed polyetheretherketone (PEEK) implant: case report","volume":"77","author":"Chac\u00f3n-Moya","year":"2009"},{"key":"b7-jns101517","series-title":"IEEE Trans Inf Technol Biomed","first-page":"274","article-title":"A new system for computeraided preoperative planning and intraoperative navigation during corrective jaw surgery","volume":"11","author":"Chapuis","year":"2007"},{"key":"b8-jns101517","series-title":"J Perinatol","first-page":"241","article-title":"Bilateral choanal narrowing as a presentation of craniometaphyseal dysplasia","volume":"17","author":"Cheung","year":"1997"},{"key":"b9-jns101517","series-title":"J Neurosurg","first-page":"140","article-title":"Pigmented villonodular synovitis of the temporomandibular joint: a rare tumor of the temporal skull base","volume":"109","author":"Day","year":"2008"},{"key":"b10-jns101517","series-title":"Neurosurgery","first-page":"960","article-title":"Foramen magnum decompression for cervicomedullary encroachment in craniometaphyseal dysplasia: case report","volume":"41","author":"Day","year":"1997"},{"key":"b11-jns101517","series-title":"Am J Med Genet A","first-page":"2335","article-title":"Cranio-meta-diaphyseal dysplasia: 25 year follow-up and review of literature","volume":"152A","author":"Dhar","year":"2010"},{"key":"b12-jns101517","series-title":"Am J Med Genet","first-page":"245","article-title":"Temporal aspects in craniometaphyseal dysplasia: autosomal recessive type","volume":"76","author":"El\u00e7ioglu","year":"1998"},{"key":"b13-jns101517","series-title":"Am J Med Genet","first-page":"482","article-title":"Mapping of the autosomal recessive (AR) craniometaphyseal dysplasia locus to chromosome region 6q21-22 and confirmation of genetic heterogeneity for mild AR spondylocostal dysplasia","volume":"95","author":"Iughetti","year":"2000"},{"key":"b14-jns101517","series-title":"J Oral Maxillofac Surg","first-page":"2167","article-title":"Computer simulation and rapid prototyping for the reconstruction of the mandible","volume":"67","author":"Juergens","year":"2009"},{"key":"b15-jns101517","series-title":"Am J Med Genet A","first-page":"870","article-title":"Three novel mutations in the ANK membrane protein cause craniometaphyseal dysplasia with variable conductive hearing loss","volume":"152A","author":"Kornak","year":"2010"},{"key":"b16-jns101517","series-title":"J Craniofac Surg","first-page":"768","article-title":"Treatment strategies for fibrous dysplasia","volume":"20","author":"Kusano","year":"2009"},{"key":"b17-jns101517","series-title":"Oral Surg Oral Med Oral Pathol Oral Radiol Endod","first-page":"e23","article-title":"Craniometaphyseal dysplasia: a case report","volume":"107","author":"Lamazza","year":"2009"},{"key":"b18-jns101517","series-title":"Acta Neurochir (Wien)","first-page":"79","article-title":"Acquired encephaloceles and epilepsy in osteopetrosis","volume":"149","author":"Mandl","year":"2007"},{"key":"b19-jns101517","series-title":"Br J Plast Surg","first-page":"144","article-title":"Autosomal dominant craniometaphyseal dysplasia with atypical features","volume":"55","author":"McKay","year":"2002"},{"key":"b20-jns101517","series-title":"Dentomaxillofac Radiol","first-page":"262","article-title":"Craniometaphyseal dysplasia associated with obstructive sleep apnoea syndrome","volume":"33","author":"Mintz","year":"2004"},{"key":"b21-jns101517","series-title":"J Neurosurg","first-page":"804","article-title":"Bilateral jugular foraminal stenosis in a patient with benign osteopetrosis associated with pseudotumor cerebri syndrome. 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