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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Segmental spinal dysgenesis (SSD)
Definitions
- Complex congenital spinal malformation
- Localized segmental agenesis or dysgenesis of lumbar or thoracolumbar spine, spinal cord
- Distal spinal osseous architecture usually normal [unless concurrent caudal regression syndrome (CRS)]
- Complex congenital spinal malformation
IMAGING
General Features
Imaging Recommendations
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Cacciola F et al: Segmental spinal dysgenesis associated with occult dysraphism: Considerations on management strategies. J Craniovertebr Junction Spine. 8(2):144-8, 2017
- Eibach S et al: Unjoined primary and secondary neural tubes: junctional neural tube defect, a new form of spinal dysraphism caused by disturbance of junctional neurulation. Childs Nerv Syst. 33(10):1633-47, 2017
- Morell SM et al: Segmental spinal dysgenesis: a report of early surgical intervention and outcome in a 14-month-old child. Childs Nerv Syst. 33(2):381-4, 2017
- Pavlova OM et al: Surgical treatment of thoracolumbar segmental spinal dysgenesis: Optimal type of fusion. World Neurosurg. 106:551-6, 2017
- Lenke LG et al: Complications after 147 consecutive vertebral column resections for severe pediatric spinal deformity: a multicenter analysis. Spine (Phila Pa 1976). 38(2):119-32, 2013
- Pahwa S et al: Segmental spinal dysgenesis: a rare malformation of the spinal cord. Neurol India. 58(2):334-5, 2010
- Emmanouilidou M et al: Spinal segmental dysgenesis: presentation of a rare spinal congenital abnormality. Neuroradiol J. 21(3):388-92, 2008
- Bristol RE et al: Segmental spinal dysgenesis: report of four cases and proposed management strategy. Childs Nerv Syst. 23(3):359-64, 2007
- Fratelli N et al: Prenatal diagnosis of segmental spinal dysgenesis. Prenat Diagn. 27(10):979-81, 2007
- Ofiram E et al: Segmental spinal dysgenesis: case report of a 50-year follow-up after surgery at age 3 years: case report. Spine (Phila Pa 1976). 31(2):E59-61, 2006
- Zana E et al: An atypical case of segmental spinal dysgenesis. Pediatr Radiol. 35(9):914-7, 2005
- Erol B et al: Congenital scoliosis and vertebral malformations: characterization of segmental defects for genetic analysis. J Pediatr Orthop. 24(6):674-82, 2004
- Rossi A et al: Imaging in spine and spinal cord malformations. Eur J Radiol. 50(2):177-200, 2004
- Buyse G et al: Segmental spinal cord hypoplasia and meningocele with preservation of medullary function: case report. Surg Neurol. 59(6):505-7; discussion 508, 2003
- Desai K et al: Congenital thoracic cord segmental amyelia: a rare manifestation of segmental spinal dysgenesis. Pediatr Neurosurg. 38(2):102-6, 2003
- Tortori-Donati P et al: Segmental spinal dysgenesis: neuroradiologic findings with clinical and embryologic correlation. AJNR Am J Neuroradiol. 20(3):445-56, 1999
- Dias MS et al: The embryogenesis of congenital vertebral dislocation: early embryonic buckling? Pediatr Neurosurg. 29(6):281-9, 1998
- Hughes LO et al: Segmental spinal dysgenesis: a report of three cases. J Pediatr Orthop. 18(2):227-32, 1998
- Achiron R et al: Second-trimester ultrasonographic diagnosis of segmental vertebral abnormalities associated with neurological deficit: a possible new variant of occult spinal dysraphism. Prenat Diagn. 16(8):760-3, 1996
- Faciszewski T et al: Segmental spinal dysgenesis. A disorder different from spinal agenesis. J Bone Joint Surg Am. 77(4):530-7, 1995
- Scott RM et al: Segmental spinal dysgenesis. Neurosurgery. 22(4):739-44, 1988
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Segmental spinal dysgenesis (SSD)
Definitions
- Complex congenital spinal malformation
- Localized segmental agenesis or dysgenesis of lumbar or thoracolumbar spine, spinal cord
- Distal spinal osseous architecture usually normal [unless concurrent caudal regression syndrome (CRS)]
- Complex congenital spinal malformation
IMAGING
General Features
Imaging Recommendations
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Cacciola F et al: Segmental spinal dysgenesis associated with occult dysraphism: Considerations on management strategies. J Craniovertebr Junction Spine. 8(2):144-8, 2017
- Eibach S et al: Unjoined primary and secondary neural tubes: junctional neural tube defect, a new form of spinal dysraphism caused by disturbance of junctional neurulation. Childs Nerv Syst. 33(10):1633-47, 2017
- Morell SM et al: Segmental spinal dysgenesis: a report of early surgical intervention and outcome in a 14-month-old child. Childs Nerv Syst. 33(2):381-4, 2017
- Pavlova OM et al: Surgical treatment of thoracolumbar segmental spinal dysgenesis: Optimal type of fusion. World Neurosurg. 106:551-6, 2017
- Lenke LG et al: Complications after 147 consecutive vertebral column resections for severe pediatric spinal deformity: a multicenter analysis. Spine (Phila Pa 1976). 38(2):119-32, 2013
- Pahwa S et al: Segmental spinal dysgenesis: a rare malformation of the spinal cord. Neurol India. 58(2):334-5, 2010
- Emmanouilidou M et al: Spinal segmental dysgenesis: presentation of a rare spinal congenital abnormality. Neuroradiol J. 21(3):388-92, 2008
- Bristol RE et al: Segmental spinal dysgenesis: report of four cases and proposed management strategy. Childs Nerv Syst. 23(3):359-64, 2007
- Fratelli N et al: Prenatal diagnosis of segmental spinal dysgenesis. Prenat Diagn. 27(10):979-81, 2007
- Ofiram E et al: Segmental spinal dysgenesis: case report of a 50-year follow-up after surgery at age 3 years: case report. Spine (Phila Pa 1976). 31(2):E59-61, 2006
- Zana E et al: An atypical case of segmental spinal dysgenesis. Pediatr Radiol. 35(9):914-7, 2005
- Erol B et al: Congenital scoliosis and vertebral malformations: characterization of segmental defects for genetic analysis. J Pediatr Orthop. 24(6):674-82, 2004
- Rossi A et al: Imaging in spine and spinal cord malformations. Eur J Radiol. 50(2):177-200, 2004
- Buyse G et al: Segmental spinal cord hypoplasia and meningocele with preservation of medullary function: case report. Surg Neurol. 59(6):505-7; discussion 508, 2003
- Desai K et al: Congenital thoracic cord segmental amyelia: a rare manifestation of segmental spinal dysgenesis. Pediatr Neurosurg. 38(2):102-6, 2003
- Tortori-Donati P et al: Segmental spinal dysgenesis: neuroradiologic findings with clinical and embryologic correlation. AJNR Am J Neuroradiol. 20(3):445-56, 1999
- Dias MS et al: The embryogenesis of congenital vertebral dislocation: early embryonic buckling? Pediatr Neurosurg. 29(6):281-9, 1998
- Hughes LO et al: Segmental spinal dysgenesis: a report of three cases. J Pediatr Orthop. 18(2):227-32, 1998
- Achiron R et al: Second-trimester ultrasonographic diagnosis of segmental vertebral abnormalities associated with neurological deficit: a possible new variant of occult spinal dysraphism. Prenat Diagn. 16(8):760-3, 1996
- Faciszewski T et al: Segmental spinal dysgenesis. A disorder different from spinal agenesis. J Bone Joint Surg Am. 77(4):530-7, 1995
- Scott RM et al: Segmental spinal dysgenesis. Neurosurgery. 22(4):739-44, 1988
