Bone Health

Synonyms and related keywords: hyperlordosis, lordosis, kyphosis, scoliosis, congenital scoliosis, sacral agenesis, lumbosacral agenesis, cervical spine anomalies, basilar impression, invagination, occipitalization of the atlas, assimilation of the atlas into the occipital bone, occipitocervical synostosis, odontoid anomalies, Klippel-Feil syndrome, Trisomy 21 syndrome, Down syndrome, deletion 5p syndrome, chromosomal number 5 syndrome, Kabuki syndrome, Noonan syndrome, Turnerlike syndrome, Aarskog syndrome, cervico-oculo-acoustic syndrome, Wildervanck syndrome, MURCS association, VACTERL association, Jarcho-Levin syndrome, spondylothoracic dysplasia, proteus syndrome

Congenital deformities of the spine are caused by anomalous vertebral development in the embryo. Minor malformations of the spine are seldom apparent and often are identified only on routine chest films (see Image 1). The more severe congenital malformations that result in progressive scoliosis are even less common than are idiopathic scolioses (see Image 2). Congenital anomalies of the spine may be simple and benign, causing no spinal deformity, or they may be complex, producing severe spinal deformity or even cor pulmonale or paraplegia. The 3 major patterns of congenital spinal deformity are hyperlordosis, kyphosis, and scoliosis. Congenital scoliosis may have marked rotation (kyphoscoliosis) or take the form of true kyphoscoliosis, in which rotation is not a major component of the deformity.
Synonyms and related keywords: hyperlordosis, lordosis, kyphosis, scoliosis, congenital scoliosis, sacral agenesis, lumbosacral agenesis, cervical spine anomalies, basilar impression, invagination, occipitalization of the atlas, assimilation of the atlas into the occipital bone, occipitocervical synostosis, odontoid anomalies, Klippel-Feil syndrome, trisomy 21 syndrome, Down syndrome, deletion 5p syndrome, chromosomal number 5 syndrome, Kabuki syndrome, Noonan syndrome, Turnerlike syndrome, Aarskog syndrome, cervico-oculo-acoustic syndrome, Wildervanck syndrome, MURCS association, VACTERL association, VACTERL syndrome, Jarcho-Levin syndrome, spondylothoracic dysplasia, proteus syndrome
Letts & Jawadi 2007

Overview of causes and kinds of spinal deformities.

Wheeless' Textbook of Orthopaedics
this type of fixation is now outdated, and the text is maintained for historical context

Case Presentation at Dupont Institute 1996

Presentation 2004: Conclusions: Non-dystrophic curves maybe treated by posterior fusion alone achieving 60% Cobb correction and 55% AVT correction. Close observation should be maintained for the appearance of dystrophic features and deterioration of correction. Dystrophic curves should be treated early and aggressively by two/three stage apical vertebrectomy, grafting and posterior spinal fusion. In this series 68% coronal Cobb and 63% AVT correction was achieved post-operatively. Complications can be expected with scoliosis associated with more than 50 degrees of kyphosis.

Information on brace biomechanics, compliance and considerations as well as specific commercial braces.

Google Search for site:www.ejbjs.org dystrophic (curvature OR scoliosis) (neurofibroma OR neurofibromatosis)

Search of JBJS B site for articles on scoliosis in NF. Search string site:www.jbjs.org.uk dystrophic (curvature OR scoliosis) (neurofibroma OR neurofibromatosis)

Vertebral and Craniofacial abnormalities. NORD Database (Goldenhar Syndrome)
Synonyms of Oculo-Auriculo-Vertebral Spectrum * FAV * Facio-Auriculo-Vertebral Spectrum * First and Second Branchial Arch Syndrome * Goldenhar-Gorlin Syndrome * OAV Spectrum * OAVS * Oculo-Auriculo-Vertebral Dysplasia Disorder Subdivisions * Goldenhar Syndrome * Hemifacial Microsomia (HFM) * Oculo-Auriculo-Vertebral Disorder

Ch 20 from Disabled Village Children: A guide for community health workers, rehabilitation workers, and families

Surgical Correction of Dystrophic Spinal Curves in Neurofibromatosis: A Review of 56 Patients.
Spine. 24(21):2247, November 1, 1999. (abstract) Parisini, Patrizio MD *; Di Silvestre, Mario MD *; Greggi, Tiziana MD *; Paderni, Stefania MD *; Cervellati, Stefano MD *; Savini, Romolo MD *+
Conclusions. The severe dystrophic curve with anterior vertebral scalloping always requires combined anterior and posterior stabilization, particularly in younger patients, even if the sagittal curves have not become pathologic by the time of presentation.

from Neurosurgical Focus Posted 02/19/2003 Sanjay N. Misra, M.D., Howard W. Morgan, M.D.
Abstract The authors review the management of thoracolumbar kyphotic deformity in cases of achondroplasia. The presence of angular thoracolumbar kyphosis in achondroplasia is well recognized. In children this is initially a nonfixed deformity that persists, however, in more than 10% of individuals and becomes a fixed thoracolumbar kyphotic deformity. Additionally, with the coexistent spinal canal stenosis, neurological damage can occur and manifest as spinal cord or cauda equina compression. The nature of this condition, the natural history, and management options are discussed. Anatomical and biomechanical factors relevant to the condition are specifically highlighted. Avoidance of pitfalls in the management of these patients is discussed for both pediatric and adult patients.

(V) = vertebral abnormalities (A) = anal atresia (C) = cardiac (heart) defects (T) = tracheoesophageal fistula (E) = esophageal atresia (R) = renal (kidney) abnormalities (L) = limb abnormalities NORD Database
Synonyms of VACTERL Association
* VACTERLS association
* VATER Association
* VATERS association