{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T06:09:55Z","timestamp":1789711795701,"version":"4.0.1"},"reference-count":48,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2008,3]]},"abstract":"<jats:p>\u2713Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by disruption of normal collagen formation resulting in varying degrees of skeletal vulnerability, ligamentous laxity, and scleral discoloration. Children with OI may suffer from complex neurosurgical problems affecting the brain and spine. The authors sought to determine the neurosurgical implications of OI in a cohort of patients treated at a quaternary care center for pediatrics.<\/jats:p>\n<jats:p>The authors reviewed the case histories of 10 children with OI treated by the neurosurgical service at the Hospital for Sick Children in Toronto between January 1988 and March 2007. The cases of 4 of these children are highlighted in the article. The most common neurosurgical conditions encountered in this cohort included macrocephaly in 5 patients, subdural hematoma in 3 patients, epidural hematoma in 2 patients, and hydrocephalus in 3 patients. Basilar invagination and spinal fractures were observed in 20% of the cohort. Although some patients could be treated nonoperatively, several required craniotomy for clot evacuation, decompression, and spinal fixation for fracture or basilar invagination, and cerebrospinal fluid shunt insertion.<\/jats:p>\n<jats:p>Neurosurgical conditions affecting patients with OI include macrocephaly, the development of an acute intracranial hematoma after often minimal trauma, the development of chronic subdural fluid collections that may require drainage, hydrocephalus (both communicating and noncommunicating), basilar invagination, and subaxial spinal fractures. Surgery may be complicated in some children because of the underlying bone fragility and bleeding diathesis commonly observed in patients with OI.<\/jats:p>","DOI":"10.3171\/ped\/2008\/1\/3\/229","type":"journal-article","created":{"date-parts":[[2008,2,29]],"date-time":"2008-02-29T18:54:07Z","timestamp":1204311247000},"page":"229-236","source":"Crossref","is-referenced-by-count":36,"title":["Neurosurgical implications of osteogenesis imperfecta in children"],"prefix":"10.3171","volume":"1","author":[{"given":"Deanna","family":"Sasaki-Adams","sequence":"first","affiliation":[{"name":"Division of Neurosurgery, Department of General Surgery, University of North Carolina School of Medicine, Chapel Hill, North Carolina; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhaya","family":"Kulkarni","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, The Hospital for Sick Children, The University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Rutka","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, The Hospital for Sick Children, The University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Dirks","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, The Hospital for Sick Children, The University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Taylor","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, The Hospital for Sick Children, The University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James M.","family":"Drake","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, The Hospital for Sick Children, The University of Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-0010229","series-title":"AJNR Am J Neuroradiol","first-page":"748","article-title":"Abnormalities in the cerebral arterial system in osteogenesis imperfecta","volume":"24","author":"Albayram","year":"2003"},{"key":"b2-0010229","series-title":"Annalen fur die gesammte Heilkunde","first-page":"1","article-title":"Merkw\u00fcrdige fragilit\u00e4t der Knochen ohne dyskrasische Ursache als krankhafte Eigenth\u00fcmlichkeit dreier Geschwister","volume":"4","author":"Axmann","year":"1831"},{"key":"b3-0010229","series-title":"Pathophysiology of Disease: An Introduction to Clinical Medicine","first-page":"2","article-title":"Genetic disease","author":"Barsh","year":"2005"},{"key":"b4-0010229","series-title":"Spine","doi-asserted-by":"publisher","first-page":"E506","DOI":"10.1097\/00007632-200111010-00022","article-title":"Spondylolisthesis in osteogenesis imperfecta due to pedicle elongation: report of two cases","volume":"26","author":"Basu","year":"2001"},{"key":"b5-0010229","series-title":"J Bone Joint Surg Am","doi-asserted-by":"publisher","first-page":"925","DOI":"10.2106\/00004623-197860070-00009","article-title":"The spine in osteogenesis imperfecta","volume":"60","author":"Benson","year":"1978"},{"key":"b6-0010229","series-title":"Clin Orthop Relat Res","first-page":"147","article-title":"The spine and surgical treatment in osteogenesis imperfecta","volume":"159","author":"Benson","year":"1981"},{"key":"b7-0010229","series-title":"Curr Opin Pediatr","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1097\/00008480-200012000-00016","article-title":"Osteogenesis imperfecta: perspectives and opportunities","volume":"12","author":"Byers","year":"2000"},{"key":"b8-0010229","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"2603","DOI":"10.1212\/WNL.43.12.2603","article-title":"Communicating hydrocephalus, basilar invagination, and other neurologic features in osteogenesis imperfecta","volume":"43","author":"Charnas","year":"1993"},{"key":"b9-0010229","series-title":"Connect Tissue Res","doi-asserted-by":"publisher","first-page":"S23","DOI":"10.3109\/03008209509116828","article-title":"Neurologic profile in osteogenesis imperfecta","volume":"31","author":"Charnas","year":"1995"},{"key":"b10-0010229","series-title":"J Med Genet","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1136\/jmg.33.3.193","article-title":"Arachnoid cyst and chronic subdural haematoma in a child with osteogenesis imperfecta type III resulting from the substitution of glycine 1006 by alanine in the pro alpha 2(I) chain of type I procollagen","volume":"33","author":"Cole","year":"1996"},{"key":"b11-0010229","series-title":"Clin Orthop Relat Res","first-page":"40","article-title":"Operative treatment of spine deformity in osteogenesis imperfecta","volume":"139","author":"Cristofaro","year":"1979"},{"key":"b12-0010229","series-title":"Spine","doi-asserted-by":"publisher","first-page":"E191","DOI":"10.1097\/01.brs.0000157471.44284.a2","article-title":"Surgical management of severe cervical kyphosis with myelopathy in osteogenesis imperfecta: a case report","volume":"30","author":"Daivajna","year":"2005"},{"key":"b13-0010229","series-title":"Neurochirurgia (Stuttg)","first-page":"180","article-title":"Acute extradural haematoma following trivial trauma in a case of osteogenesis imperfecta","volume":"28","author":"Diaz","year":"1985"},{"key":"b14-0010229","series-title":"Descriptionem et Casus Aliquot Osteomalaciae Sistens. Dissertatio Medica","author":"Ekman","year":"1788"},{"key":"b15-0010229","series-title":"Scand J Haematol","first-page":"177","article-title":"Haemostatic studies in osteogenesis imperfecta","volume":"33","author":"Evensen","year":"1984"},{"key":"b16-0010229","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"116","DOI":"10.1016\/S0090-3019(82)80033-5","article-title":"Basilar impression and platybasia in osteogenesis imperfecta tarda","volume":"17","author":"Frank","year":"1982"},{"key":"b17-0010229","series-title":"Clin Nucl Med","doi-asserted-by":"publisher","first-page":"1055","DOI":"10.1097\/00003072-199512000-00003","article-title":"Multiple flexion-stress vertebral end-plate fractures. An osteogenesis imperfecta presentation","volume":"20","author":"Frater","year":"1995"},{"key":"b18-0010229","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1016\/j.pediatrneurol.2004.07.011","article-title":"Subdural hematoma as clinical presentation of osteogenesis imperfecta","volume":"32","author":"Groninger","year":"2005"},{"key":"b19-0010229","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"782","DOI":"10.1227\/00006123-199011000-00016","article-title":"The operative management of basilar impression in osteogenesis imperfecta","volume":"27","author":"Harkey","year":"1990"},{"key":"b20-0010229","series-title":"Blood","doi-asserted-by":"publisher","first-page":"500","DOI":"10.1182\/blood.V39.4.500.500","article-title":"Platelet function and pyrophosphates in osteogenesis imperfecta","volume":"39","author":"Hathaway","year":"1972"},{"key":"b21-0010229","series-title":"J Bone Joint Surg Am","doi-asserted-by":"publisher","first-page":"212","DOI":"10.2106\/00004623-199602000-00007","article-title":"Vertebral body shape as a predictor of spinal deformity in osteogenesis imperfecta","volume":"78","author":"Ishikawa","year":"1996"},{"key":"b22-0010229","series-title":"J Neurosurg Spine","doi-asserted-by":"publisher","first-page":"237","DOI":"10.3171\/spi.2004.1.2.0237","article-title":"Multiple vertebral fractures in osteogenesis imperfecta treated by vertebroplasty. Case illustration","volume":"1","author":"Kas\u00f3","year":"2004"},{"key":"b23-0010229","series-title":"Anesthesiology","doi-asserted-by":"publisher","first-page":"1011","DOI":"10.1097\/00000542-200210000-00039","article-title":"Osteogenesis imperfecta, perioperative bleeding, and desmopressin","volume":"97","author":"Keegan","year":"2002"},{"key":"b24-0010229","series-title":"Pediatr Pathol","doi-asserted-by":"publisher","first-page":"367","DOI":"10.3109\/15513818809041570","article-title":"Hydrocephalus of intrauterine onset in perinatally lethal osteogenesis imperfecta: clinical, sonographic, and pathologic correlations","volume":"8","author":"Knisely","year":"1988"},{"key":"b25-0010229","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"361","DOI":"10.3171\/jns.2006.105.3.361","article-title":"Skull base abnormalities in osteogenesis imperfecta: a cephalometric evaluation of 54 patients and 108 control volunteers","volume":"105","author":"Kovero","year":"2006"},{"key":"b26-0010229","series-title":"Trait\u00e9 de l'Anatomie Pathologique","first-page":"204","article-title":"De la fragilit\u00e9 des os, ou do l'ost\u00e9opsathyrose","volume":"Vol 2","author":"Lobstein","year":"1833"},{"key":"b27-0010229","series-title":"J Med Genet","doi-asserted-by":"publisher","first-page":"382","DOI":"10.1136\/jmg.39.6.382","article-title":"Testing for osteogenesis imperfecta in cases of suspected non-accidental injury","volume":"39","author":"Marlowe","year":"2002"},{"key":"b28-0010229","series-title":"J Clin Pathol","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1136\/jcp.49.8.627","article-title":"Causes of death in osteogenesis imperfecta","volume":"49","author":"McAllion","year":"1996"},{"key":"b29-0010229","series-title":"Spine","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1097\/00007632-198606000-00025","article-title":"A three-level fracture of the axis in a patient with osteogenesis imperfecta. A case report","volume":"11","author":"Meyer","year":"1986"},{"key":"b30-0010229","series-title":"Spine","doi-asserted-by":"publisher","first-page":"E224","DOI":"10.1097\/00007632-200204150-00025","article-title":"Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report","volume":"27","author":"Nakamura","year":"2002"},{"issue":"1 Suppl","key":"b31-0010229","series-title":"J Neurosurg","first-page":"57","article-title":"Epidural hematoma formation following trivial head trauma in a child with osteogenesis imperfecta. Case report","volume":"106","author":"Parmar","year":"2007"},{"key":"b32-0010229","series-title":"J Bone Joint Surg Br","first-page":"233","article-title":"Basilar impression in osteogenesis imperfecta. A report of three cases in one family","volume":"66","author":"Pozo","year":"1984"},{"key":"b33-0010229","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1227\/00006123-198307000-00014","article-title":"Acute bilateral extradural hematomas in a case of osteogenesis imperfecta congenita","volume":"13","author":"Pozzati","year":"1983"},{"key":"b34-0010229","series-title":"Clin Orthop Relat Res","first-page":"113","article-title":"Osteogenesis imperfecta as a differential diagnosis of pathologic burst fractures of the spine. A case report","volume":"289","author":"Rao","year":"1993"},{"key":"b35-0010229","series-title":"Lancet","doi-asserted-by":"publisher","first-page":"1377","DOI":"10.1016\/S0140-6736(04)16051-0","article-title":"Osteogenesis imperfecta","volume":"363","author":"Rauch","year":"2004"},{"key":"b36-0010229","series-title":"Metabolic Bone Disease and Clinically Related Disorders","article-title":"Osteogenesis imperfecta","author":"Rowe","year":"1997"},{"key":"b37-0010229","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"950","DOI":"10.3171\/jns.1997.86.6.0950","article-title":"Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management","volume":"86","author":"Sawin","year":"1997"},{"key":"b38-0010229","series-title":"Am J Emerg Med","doi-asserted-by":"publisher","first-page":"298","DOI":"10.1016\/0735-6757(87)90355-X","article-title":"Nontraumatic subdural hematoma in a patient with osteogenesis imperfecta and renal failure","volume":"5","author":"Sayre","year":"1987"},{"key":"b39-0010229","series-title":"Am J Med","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1016\/0002-9343(57)90016-5","article-title":"Hemorrhagic disease in osteogenesis imperfecta; study of platelet functional defect","volume":"22","author":"Siegel","year":"1957"},{"key":"b40-0010229","series-title":"Lancet","first-page":"1041","article-title":"Classification of osteogenesis imperfect","volume":"1","author":"Sillence","year":"1978"},{"key":"b41-0010229","series-title":"J Med Genet","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1136\/jmg.16.2.101","article-title":"Genetic heterogeneity in osteogenesis imperfecta","volume":"16","author":"Sillence","year":"1979"},{"key":"b42-0010229","series-title":"Am J Forensic Med Pathol","doi-asserted-by":"publisher","first-page":"54","DOI":"10.1097\/00000433-198903000-00014","article-title":"Fatal intraoperative hemorrhage during spinal fusion surgery for osteogenesis imperfecta","volume":"10","author":"Sperry","year":"1989"},{"key":"b43-0010229","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1227\/00006123-198803000-00029","article-title":"Infantile chronic subdural hematoma with local protrusion of the skull in a case of osteogenesis imperfecta","volume":"22","author":"Tokoro","year":"1988"},{"key":"b44-0010229","series-title":"Eur Spine J","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1007\/s00586-003-0574-3","article-title":"Osteogenesis imperfecta in childhood: effects of spondylodesis on functional ability, ambulation and perceived competence","volume":"13","author":"Tolboom","year":"2004"},{"key":"b45-0010229","series-title":"Arch Neurol","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1001\/archneur.1986.00520020044016","article-title":"Neurologic correlates of osteogenesis imperfecta","volume":"43","author":"Tsipouras","year":"1986"},{"key":"b46-0010229","series-title":"Tabulae ad Illustrandam Embryogenesin Hominis et Mammalium, tam Naturalem quam Abnormen","author":"Vrolik","year":"1849"},{"key":"b47-0010229","series-title":"Clin Orthop Relat Res","first-page":"6","article-title":"Osteogenesis imperfecta: historical background","volume":"159","author":"Weil","year":"1981"},{"key":"b48-0010229","series-title":"J Bone Joint Surg Br","first-page":"184","article-title":"Paraplegia in osteogenesis imperfecta. A case report","volume":"65","author":"Ziv","year":"1983"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/1\/3\/article-p229.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/1\/3\/article-p229.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/1\/3\/article-p229.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T14:46:17Z","timestamp":1681569977000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/1\/3\/article-p229.xml"}},"subtitle":["Report of 4 cases"],"short-title":[],"issued":{"date-parts":[[2008,3]]},"references-count":48,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3171\/ped\/2008\/1\/3\/229","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,3]]}}}