Specific entities affecting the craniocervical region: osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management of basilar impression.

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
Arnold H Menezes

Abstract

Osteogenesis imperfecta (OI) is an inheritable disorder of bone development caused by defective collagen synthesis. The attendant basilar impression or secondary basilar invagination is uncommon but can be devastating. Fifty-two patients with osteochondrodysplasia (28 with OI, six with Hajdu-Cheney syndrome, six with Paget's disease, and 12 with spondyloepiphyseal dysplasia) with basilar impression were evaluated between 1985 and 2005. The male/female ratio in this cohort was 1:1. The mean age at presentation was 12.2 years. Symptoms and signs included headache, lower cranial nerve dysfunction, dysphagia, respiratory embarrassment, weakness, and ataxia. In the earlier part of the series (1985-1995), all patients with hydrocephalus were shunted and a ventral transoral decompression made for ventral compression of the pontomedullary junction followed by a dorsal occipitocervical fusion. As a result of this evaluation, it was felt that most patients would benefit by early bracing after the hydrocephalus was shunted if it existed. However, 20% of patients still required an anterior ventral decompression and the occipitocervical fusion. The results showed that the fusions were stable but over a period of time, there was progressive ...Continue Reading

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Citations

Dec 17, 2009·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Heidi ArponenJanna Waltimo-Sirén
Jun 15, 2011·Nature Reviews. Endocrinology·Antonella ForlinoJoan C Marini
Aug 10, 2011·Neurosurgery·Hormuzdiyar H DasenbrockJean-Paul Wolinsky
Dec 3, 2014·Pediatric Clinics of North America·Jennifer HarringtonAndrew Howard
Jan 20, 2012·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Heidi ArponenJanna Waltimo-Sirén
Aug 20, 2010·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Moira S CheungFrank Rauch
Mar 7, 2019·Journal of Children's Orthopaedics·R M CasteleinUNKNOWN EPOS Spine Study Group
Sep 8, 2018·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·T SwezeyL L Tosi
Jul 22, 2020·Current Opinion in Pediatrics·Nipith CharoenngamMichael F Holick
Dec 21, 2021·Journal of Neurological Surgery. Part A, Central European Neurosurgery·Ricardo Vieira BotelhoJosé Marcus Rotta

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